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A Unique Case of the Transformation of a Hepatic Leiomyoma into Leiomyosarcoma with Pancreatic Metastases: Review of the Literature with Case Presentation. REPORTS 2021. [DOI: 10.3390/reports5010002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Abstract
Primary hepatic leiomyoma (PHL) is a rare entity, with very few cases reported in the literature. Even more rarely, until now practically undescribed, is the transformation of a hepatic leiomyoma into leiomyosarcoma with pancreatic metastases. Here, we report a single case of the progression of PHL in primary hepatic leiomyosarcoma, with clinical–surgical and histopathological features, and we conducted a review of the literature of related cases that can be found.
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Positron emission tomography in multiple sclerosis - straight to the target. Nat Rev Neurol 2021; 17:663-675. [PMID: 34545219 DOI: 10.1038/s41582-021-00537-1] [Citation(s) in RCA: 32] [Impact Index Per Article: 8.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/30/2021] [Indexed: 02/08/2023]
Abstract
Following the impressive progress in the treatment of relapsing-remitting multiple sclerosis (MS), the major challenge ahead is the development of treatments to prevent or delay the irreversible accumulation of clinical disability in progressive forms of the disease. The substrate of clinical progression is neuro-axonal degeneration, and a deep understanding of the mechanisms that underlie this process is a precondition for the development of therapies for progressive MS. PET imaging involves the use of radiolabelled compounds that bind to specific cellular and metabolic targets, thereby enabling direct in vivo measurement of several pathological processes. This approach can provide key insights into the clinical relevance of these processes and their chronological sequence during the disease course. In this Review, we focus on the contribution that PET is making to our understanding of extraneuronal and intraneuronal mechanisms that are involved in the pathogenesis of irreversible neuro-axonal damage in MS. We consider the major challenges with the use of PET in MS and the steps necessary to realize clinical benefits of the technique. In addition, we discuss the potential of emerging PET tracers and future applications of existing compounds to facilitate the identification of effective neuroprotective treatments for patients with MS.
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Djokic M, Hadzialjevic B, Luzar B, Trotovsek B. Primary Hepatic Leiomyoma in a Healthy Middle-Aged Woman: Literature Review and Case Report. Front Surg 2021; 8:691674. [PMID: 34195223 PMCID: PMC8236603 DOI: 10.3389/fsurg.2021.691674] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/06/2021] [Accepted: 05/17/2021] [Indexed: 11/13/2022] Open
Abstract
Introduction: Primary hepatic leiomyoma (PHL) is a rare benign hepatic tumor with unclear pathogenesis. It more commonly occurs in immunosuppressed patients, while only 24 cases have been described among immunocompetent individuals. To date, only one successful preoperative diagnosis of PHL has been achieved. Case Presentation: Here we report a case of PHL in a middle-aged woman with no history of immunosuppression. Preoperative diagnosis of PHL was established using ultrasound-guided fine needle trucut biopsy (FNTB). Nevertheless, due to the growing nature of tumor and patient's symptoms, we proceeded with surgical resection, which confirmed the diagnosis of PHL. At 6-month follow up, the patient is in good condition with no evidence of tumor recurrence. Conclusions: PHL is an uncommon tumor that should be considered in the differential diagnosis of rare liver tumors. Image guided FNTB appears to be effective in achieving preoperative diagnosis of PHL. Surgical resection, however, remains both diagnostic and curative in the management of PHL.
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Affiliation(s)
- Mihajlo Djokic
- Department of Abdominal Surgery, University Medical Centre Ljubljana, Ljubljana, Slovenia
- Department of Surgery, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
| | - Benjamin Hadzialjevic
- Department of Abdominal Surgery, University Medical Centre Ljubljana, Ljubljana, Slovenia
| | - Bostjan Luzar
- Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
| | - Blaz Trotovsek
- Department of Abdominal Surgery, University Medical Centre Ljubljana, Ljubljana, Slovenia
- Department of Surgery, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
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Hatano Y, Ideta T, Hirata A, Hatano K, Tomita H, Okada H, Shimizu M, Tanaka T, Hara A. Virus-Driven Carcinogenesis. Cancers (Basel) 2021; 13:2625. [PMID: 34071792 PMCID: PMC8198641 DOI: 10.3390/cancers13112625] [Citation(s) in RCA: 36] [Impact Index Per Article: 9.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/30/2021] [Revised: 05/22/2021] [Accepted: 05/25/2021] [Indexed: 12/13/2022] Open
Abstract
Cancer arises from the accumulation of genetic and epigenetic alterations. Even in the era of precision oncology, carcinogens contributing to neoplastic process are still an important focus of research. Comprehensive genomic analyses have revealed various combinations of base substitutions, referred to as the mutational signatures, in cancer. Each mutational signature is believed to arise from specific DNA damage and repair processes, including carcinogens. However, as a type of carcinogen, tumor viruses increase the cancer risk by alternative mechanisms, including insertional mutagenesis, viral oncogenes, and immunosuppression. In this review, we summarize virus-driven carcinogenesis to provide a framework for the control of malignant cell proliferation. We first provide a brief overview of oncogenic viruses and describe their implication in virus-related tumors. Next, we describe tumor viruses (HPV, Human papilloma virus; HBV, Hepatitis B virus; HCV, Hepatitis C virus; EBV, Epstein-Barr virus; Kaposi sarcoma herpesvirus; MCV, Merkel cell polyoma virus; HTLV-1, Human T-cell lymphotropic virus, type-1) and tumor virus-related cancers. Lastly, we introduce emerging tumor virus candidates, human cytomegalovirus (CMV), human herpesvirus-6 (HHV-6) and adeno-associated virus-2 (AAV-2). We expect this review to be a hub in a complex network of data for virus-associated carcinogenesis.
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Affiliation(s)
- Yuichiro Hatano
- Department of Tumor Pathology, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan; (H.T.); (A.H.)
| | - Takayasu Ideta
- Department of Gastroenterology, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan; (T.I.); (M.S.)
- Department of Laboratory Medicine, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan
| | - Akihiro Hirata
- Laboratory of Veterinary Pathology, Joint Department of Veterinary Medicine, Faculty of Applied Biological Sciences, Gifu University, Gifu 501-1194, Japan;
| | - Kayoko Hatano
- Department of Obstetrics and Gynecology, Gifu University Hospital, Gifu 501-1194, Japan;
| | - Hiroyuki Tomita
- Department of Tumor Pathology, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan; (H.T.); (A.H.)
| | - Hideshi Okada
- Department of Emergency and Disaster Medicine, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan;
| | - Masahito Shimizu
- Department of Gastroenterology, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan; (T.I.); (M.S.)
| | - Takuji Tanaka
- Department of Diagnostic Pathology (DDP) and Research Center of Diagnostic Pathology (RC-DiP), Gifu Municipal Hospital, Gifu 500-8513, Japan;
| | - Akira Hara
- Department of Tumor Pathology, Gifu University Graduate School of Medicine, Gifu 501-1194, Japan; (H.T.); (A.H.)
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5
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Lau K, Hsu Y, Lin Y, Chen K. Role of surgery in treating epstein-barr virus-associated smooth muscle tumor (EBV-SMT) with central nervous system invasion: A systemic review from 1997 to 2019. Cancer Med 2021; 10:1473-1484. [PMID: 33576167 PMCID: PMC7940242 DOI: 10.1002/cam4.3770] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/02/2020] [Revised: 01/15/2021] [Accepted: 01/21/2021] [Indexed: 12/18/2022] Open
Abstract
Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) is a rare mesenchymal tumor occurred almost exclusively in immunocompromised hosts. This article provides a systematic review of literature under PRISMA guideline on clinical features, treatment modalities, roles of surgical intervention, and outcomes of all 65 reported EBV-SMTs with central nervous system (CNS) invasion. Over 95% of reported cases were immunocompromised, while human immunodeficiency virus infection and post-organ transplantation were the most commonly associated underlying causes (near 90%). Despite a heterogeneous follow-up period, a 1-year survival rate of 76.0% and 5-year survival rate of 59.6% may support the indolent and non-deadly nature of EBV-SMT even with CNS invasion. Immune survey and reconstruction should be conducted for every patient with CNS EBV-SMT. Surgical resection is mostly adopted as primary treatment to obtain diagnosis and relieve compressive effect. A total resection of tumor may be beneficial if tumor was symptomatic and had intracranial invasion.
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Affiliation(s)
- Ka‐Wei Lau
- College of MedicineChang Gung UniversityTaoyuanTaiwan
| | - Yu‐Wei Hsu
- College of MedicineChang Gung UniversityTaoyuanTaiwan
| | - Yin‐Ting Lin
- College of MedicineChang Gung UniversityTaoyuanTaiwan
| | - Ko‐Ting Chen
- Department of NeurosurgeryChang Gung Memorial Hospital at LinkouTaoyuanTaiwan
- PhD. Program in Biomedical EngineeringChang Gung UniversityTaoyuanTaiwan
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6
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Coletta D, Parrino C, Nicosia S, Manzi E, Pattaro G, Oddi A, D'Annibale M, Marino M, Grazi GL. Primary leiomyoma of the liver in an immunocompetent patient. Intractable Rare Dis Res 2020; 9:251-255. [PMID: 33139985 PMCID: PMC7586883 DOI: 10.5582/irdr.2020.03075] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/12/2023] Open
Abstract
Primary leiomyoma of the liver (PLL) is a rare benign tumor occurring in immunosuppressed people. From 1926 less than fifty cases are reported in the scientific literature and about half are in immunocompetent patients. Etiology of this kind of lesion is not yet well known. We report a case of primary hepatic leiomyoma in a 60-year-old immunocompetent woman. The patient presented with lipothymia with unexpected vomiting. She underwent an ultrasound (US), and a computed tomography (CT) scan that revealed the presence of a single, solid lesion about 9 cm located between the S5 and S8 segment of the liver. It showed a well-defined, heterogeneous hypodensity with internal and peripheral enhancement and various central hypoattenuating areas and no wash-out in the portal and the late phases. Because of her symptoms and the risk of malignancy, the patient underwent a surgical liver resection. Histological diagnosis was primary leiomyoma of the liver. The patient had an uneventful recovery and was discharged after 7 days. At 30 months follow-up there were no symptoms and no evidence of disease. Leiomyoma of the liver is a rare benign neoplasm of which clinical symptoms are nonspecific and the exact radiological diagnosis still remains a challenge for radiologists. Etiology is still unclear and usually PLL represents an incidental diagnosis. Surgery plays a primary role not only in the treatment algorithm, but also in the diagnostic workout.
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Affiliation(s)
- Diego Coletta
- Hepatopancreatobiliary Surgery, IRCCS - Regina Elena National Cancer Institute, Rome, Italy
- Emergency Department - Emergency and Trauma Surgery Unit, Umberto I University Hospital, Sapienza University of Rome, Rome, Italy
- Address correspondence to:Diego Coletta, Hepatopancreatobiliary Surgery, IRCCS - Regina Elena National Cancer Institute, Via Elio Chianesi 53, 00144 Rome, Italy; Emergency Department - Emergency and Trauma Surgery Unit, Umberto I University Hospital, Sapienza University of Rome,Viale del Policlinico 155 ,00161 Rome , Italy. E-mail:
| | - Chiara Parrino
- Hepatopancreatobiliary Surgery, IRCCS - Regina Elena National Cancer Institute, Rome, Italy
| | - Simone Nicosia
- Hepatopancreatobiliary Surgery, IRCCS - Regina Elena National Cancer Institute, Rome, Italy
| | - Emy Manzi
- Hepatopancreatobiliary Surgery, IRCCS - Regina Elena National Cancer Institute, Rome, Italy
| | - Giada Pattaro
- Hepatopancreatobiliary Surgery, IRCCS - Regina Elena National Cancer Institute, Rome, Italy
| | - Andrea Oddi
- Hepatopancreatobiliary Surgery, IRCCS - Regina Elena National Cancer Institute, Rome, Italy
| | - Marco D'Annibale
- Hepatopancreatobiliary Surgery, IRCCS - Regina Elena National Cancer Institute, Rome, Italy
| | - Mirella Marino
- Department of Pathological Anatomy, IRCCS - Regina Elena National Cancer Institute, Rome, Italy
| | - Gian Luca Grazi
- Hepatopancreatobiliary Surgery, IRCCS - Regina Elena National Cancer Institute, Rome, Italy
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Matin RN, Ieremia E. Cutaneous Epstein-Barr virus-associated smooth muscle tumor in immunosuppression. J Cutan Pathol 2020; 48:325-329. [PMID: 33245561 DOI: 10.1111/cup.13849] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/19/2020] [Revised: 08/10/2020] [Accepted: 08/12/2020] [Indexed: 12/28/2022]
Abstract
A 36-year-old renal transplant recipient presented 15 months post-transplantation with a cutaneous spindle cell neoplasm with features of smooth muscle differentiation treated with local excision. 1.4 years later, a magnetic resonance imaging liver scan with gadolinium demonstrated multiple bilobar enhancing hepatic lesions, in keeping with metastases. A core biopsy revealed morphological appearances similar to the previous cutaneous spindle cell neoplasm. Epstein-Barr virus early RNA (EBER) in situ hybridization demonstrated strong diffuse staining of both cutaneous and liver tumor cells for EBER indicative of Epstein-Barr virus (EBV) infection. This is a rare presentation of multifocal EBV-associated smooth muscle tumors first presenting in the skin in an adult renal transplant recipient, which, despite being multifocal and involving the liver, may confer a better prognosis than predicted.
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Affiliation(s)
- Rubeta N Matin
- Dermatology Department, Oxford University Hospitals NHS Foundation Trust, Oxford, UK
| | - Eleni Ieremia
- Department of Cellular Pathology, John Radcliffe Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, UK
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Bruce-Brand C, Mohamed N, Botes S, Bates WD. Concurrent Diffuse Large B-Cell Lymphoma and Epstein-Barr Virus-Associated Smooth Muscle Tumour in the Small Bowel of an HIV-Positive Adult-a Case Report and Review of the Literature. J Gastrointest Cancer 2020; 50:572-577. [PMID: 29297137 DOI: 10.1007/s12029-017-0046-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Affiliation(s)
- C Bruce-Brand
- Tygerberg Hospital, University of Stellenbosch, Cape Town, South Africa.
| | - N Mohamed
- Tygerberg Hospital, University of Stellenbosch, Cape Town, South Africa
| | - S Botes
- Tygerberg Hospital, University of Stellenbosch, Cape Town, South Africa
| | - W D Bates
- Tygerberg Hospital, University of Stellenbosch, Cape Town, South Africa
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9
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Fraga J, Caetano Oliveira R, Terracciano L, Silva MR, Cipriano MA. Hepatic Myxoid Leiomyoma: A Very Rare Tumor. GE-PORTUGUESE JOURNAL OF GASTROENTEROLOGY 2019; 27:352-355. [PMID: 32999907 DOI: 10.1159/000504762] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/28/2019] [Revised: 11/07/2019] [Indexed: 12/31/2022]
Abstract
Introduction Mesenchymal tumors of the liver are rare, and in this group, myxoid leiomyomas are even rarer. So far, only 2 cases have been reported in the literature. Case Presentation We aim to report the case of a 16-year-old female with a large lesion on the right hepatic lobe, grossly composed of gelatinous and heterogeneous tissue. Discussion Histological evaluation revealed a benign mesenchymal neoplasm with expansive growth, paucicellular, with monotonous and dispersed spindle and ovoid cells, positive for α-smooth actin and h-caldesmon, without atypia or mitoses, consistent with the diagnosis of primary myxoid leiomyoma.
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Affiliation(s)
- João Fraga
- Pathology Department, Coimbra University Hospital, Coimbra, Portugal
| | | | | | - Mário Rui Silva
- Pathology Department, Coimbra University Hospital, Coimbra, Portugal
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Brown DA, Deep NL, Driscoll CL, Link MJ, Jentoft ME, Daniels DJ. Synchronous Epstein-Barr virus-associated skull base and adrenal smooth-muscle tumors in an 8-year-old girl with recent Epstein-Barr virus infection. J Neurosurg Pediatr 2018; 22:283-287. [PMID: 29905497 DOI: 10.3171/2018.3.peds17609] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Epstein-Barr virus-associated smooth-muscle tumors are rare tumors seen in immunocompromised patients. Most cases occur in the context of AIDS and organ transplantation, and very rarely in the setting of congenital immunodeficiency, with only 5 case reports of the latter published so far in the literature. The authors report the case of a previously healthy 8-year-old girl with headaches and precocious puberty who was found to have a large skull base lesion. There was a synchronous left adrenal lesion. She underwent resection of the skull base lesion and a left adrenalectomy. Thorough evaluation for immunodeficiency was negative for a known congenital immunodeficiency syndrome. She had a short course of intravenous immunoglobulin and has had no recurrence of disease or new lesions in the 17 months since presentation. Continued surveillance for the development of opportunistic infections and new or recurrent lesions is warranted in this case. Repeat surgery for surgically accessible tumors or chemoradiation would be recommended for any additional lesions.
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Affiliation(s)
| | | | | | | | - Mark E Jentoft
- 3Clinical and Anatomic Pathology, Mayo Clinic, Rochester, Minnesota
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11
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Epstein Barr virus-associated smooth muscle tumour (EBV-SMT) of the urinary bladder. Urol Case Rep 2017; 16:89-91. [PMID: 29226094 PMCID: PMC5705795 DOI: 10.1016/j.eucr.2017.11.015] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/17/2017] [Revised: 10/23/2017] [Accepted: 11/17/2017] [Indexed: 11/29/2022] Open
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12
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Primary leiomyoma of the liver: a review of a rare tumour. HPB SURGERY : A WORLD JOURNAL OF HEPATIC, PANCREATIC AND BILIARY SURGERY 2014; 2014:959202. [PMID: 25505821 PMCID: PMC4253698 DOI: 10.1155/2014/959202] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/11/2014] [Revised: 10/20/2014] [Accepted: 10/30/2014] [Indexed: 12/27/2022]
Abstract
Context. Primary leiomyoma of the liver is a rare tumour with uncertain pathogenesis with similar presentation with other tumours of the liver. Little is known about its clinical course. Objectives. To review the literature for case reports of primary leiomyoma of the liver. Methods. Extensive literature search was carried out for case reports of primary leiomyoma of the liver. Results. A total of 36 cases of primary leiomyoma of the liver were reviewed. The mean age of presentation is 43 years with slight female sex affectation; females accounted for 55.6% of the cases reported in the literature. The average size of the tumour is 8.7 cm. 34.4% of the cases reviewed were incidental finding with the mean follow-up time of 33 months with most cases reporting no evidence of disease. Conclusions. Primary leiomyoma of the liver is very rare tumour with complex pathogenesis which remains largely unknown. Imaging of the tumour does not allow for a tissue specific diagnosis; hence histological review of the tissue specimen and immunohistochemical stains are imperative for diagnosis. Surgical resection is both diagnostic and curative. The diagnosis of primary leiomyoma of the liver should be considered as a differential in the management of liver tumours.
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Choi HS, Jung CW, Cho SY, Kim SB, Park S. Primary myxoid leiomyoma of the liver. KOREAN JOURNAL OF PATHOLOGY 2014; 48:54-7. [PMID: 24627696 PMCID: PMC3950236 DOI: 10.4132/koreanjpathol.2014.48.1.54] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 12/05/2012] [Revised: 02/21/2013] [Accepted: 02/22/2013] [Indexed: 11/17/2022]
Abstract
Herein, we report a case of primary myxoid leiomyoma of the liver. A 60-year-old woman complained of upper abdominal fullness. Computed tomography showed a solid tumor (8 cm) in the liver. The patient underwent right hepatectomy and histological findings from the resected specimen revealed scattered bland spindle cells in a background of exuberant myxoid material. The tumor cells were immunoreactive for smooth muscle actin and desmin. No other lesions were found elsewhere in the body. Thus, the tumor was diagnosed as a primary myxoid leiomyoma of the liver.
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Affiliation(s)
- Hee Seung Choi
- Department of Pathology, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences, Seoul, Korea
| | - Chang Won Jung
- Department of Pathology, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences, Seoul, Korea
| | - Soo Youn Cho
- Department of Pathology, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences, Seoul, Korea
| | - Sang Bum Kim
- Department of Surgery, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences, Seoul, Korea
| | - Sunhoo Park
- Department of Pathology, Korea Cancer Center Hospital, Korea Institute of Radiological and Medical Sciences, Seoul, Korea
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Luo XZ, Ming CS, Chen XP, Gong NQ. Epstein-Barr virus negative primary hepatic leiomyoma: Case report and literature review. World J Gastroenterol 2013; 19:4094-4098. [PMID: 23840159 PMCID: PMC3703201 DOI: 10.3748/wjg.v19.i25.4094] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/21/2013] [Accepted: 05/17/2013] [Indexed: 02/06/2023] Open
Abstract
Primary hepatic leiomyoma is a neoplasm of mesenchymal origin and occurs only rarely. Secondary to benign smooth muscle proliferation, it is usually found in adult women and is associated with Epstein-Barr virus (EBV) infection. Here, we report the 29th case of primary hepatic leiomyoma with its unique features related to diagnosis, treatment and developmental biology. A 48-year-old man, with an immunocompromised status, complained of pain in the upper quadrant of the abdomen. Serological analysis indicated no presence of hepatitis virus, no human immunodeficiency virus, and no EBV infection. The levels of α-fetoprotein and carcinoembryonic antigen were normal. A mass was detected in segment III of the hepatic lobe by ultrasonography and an abdominal computed tomography scan. Endoscopy had negative findings. Exploratory laparotomy found no existing extrahepatic tumor and left lateral lobectomy was performed. Pathological examination showed the mass to be a typical leiomyoma. The cells were positive for α-smooth muscle actin and desmin, and negative for the makers of gastrointestinal stromal tumor (GIST), including CD117, CD34 and DOG1 (discovered on GIST1). In situ hybridization revealed negative status for EBV-encoded small RNA. After left lateral lobectomy, the patient was not given chemotherapy or radiotherapy. During a 2-year follow-up, no sign of local recurrence or distant metastasis was observed. In conclusion, we report a rare case of primary hepatic leiomyoma in a male patient without EBV infection. Hepatic resection was curative. This case presents data to expand our knowledge concerning the complex and heterogeneous nature of primary liver leiomyoma, indicating that EBV infection is important but neither necessary nor sufficient for the development of primary liver leiomyoma.
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15
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Sarcomas other than Kaposi sarcoma occurring in immunodeficiency: interpretations from a systematic literature review. Curr Opin Oncol 2012; 24:537-46. [PMID: 22729152 DOI: 10.1097/cco.0b013e328355e115] [Citation(s) in RCA: 35] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
PURPOSE OF REVIEW In immunodeficiency, an increased sarcoma risk is confirmed for Kaposi's sarcoma. Whether rates of other sarcoma subtypes are elevated in the setting of immunodeficiency is not known. We therefore reviewed published case reports on HIV and AIDS patients and organ transplant recipients with sarcomas. For comparison, we assessed sarcomas in the U.S. general population using Surveillance Epidemiology End Results (SEER) data. RECENT FINDINGS A total of 176 non-Kaposi sarcoma were identified, 75 in people with HIV and AIDS and 101 in transplant recipients. Leiomyosarcomas (n = 101) were the most frequently reported sarcomas, followed by angiosarcomas (n = 23) and fibrohistiocytic tumors (n = 17). Leiomyosarcomas were reported with two age peaks, in children and young adults. Epstein-Barr virus (EBV) was detected in the tumor cells in 85 and 88% of leiomyosarcomas in HIV-infected people and transplant recipients, respectively. Angiosarcomas and fibrohistiocytic tumors were most frequently reported in men. Among kidney transplant recipients, 20% of sarcomas arose at the site of an arteriovenous fistula. In comparison, leiomyoscarcomas, angiosarcomas, and fibrohistiocytic tumors comprised 16.9, 3.8, and 18.7% of sarcomas in the U.S. general population. SUMMARY Leiomyosarcoma and angiosarcoma may occur disproportionately in immunodeficiency. Leiomyosarcomas appear causatively linked to EBV, whereas angiosarcomas might be correlated with an arteriovenous fistula. Additional studies are necessary to understand the contribution of immunodeficiency to the cause of these sarcomas.
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Santos I, Valls C, Leiva D, Serrano T, Martinez L, Ruiz S. Primary hepatic leiomyoma: case report. ACTA ACUST UNITED AC 2011; 36:315-7. [PMID: 20959979 DOI: 10.1007/s00261-010-9648-y] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Abstract
The patient is a previously healthy 28-year-old woman with incidentally detected focal liver lesion. On CT, the tumor showed brisk arterial enhancement and persistent hyperenhancement on portal and delayed phases. Histological study showed spindle cells without atypia and immunohistochemical study was consistent with leiomyoma.
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Affiliation(s)
- Idoia Santos
- Department of Radiology, Bellvitge University Hospital, Barcelona, Spain
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17
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Tetzlaff MT, Nosek C, Kovarik CL. Epstein-Barr virus-associated leiomyosarcoma with cutaneous involvement in an African child with human immunodeficiency virus: a case report and review of the literature. J Cutan Pathol 2011; 38:731-9. [PMID: 21623869 DOI: 10.1111/j.1600-0560.2011.01721.x] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
Epstein-Barr virus (EBV)-associated smooth muscle tumors (SMTs) are infrequently encountered lesions restricted to immunocompromised patients. However, they represent the second most common tumor in children with human immunodeficiency virus (HIV) infection. We report a case of a progressively enlarging abdominal mass with cutaneous involvement in an HIV-infected, 4-year-old African girl in Malawi with clinical acquired immunodeficiency syndrome on highly active antiretroviral therapy. Analysis of an excisional specimen revealed a well-differentiated leiomyosarcoma and subsequent studies revealed diffuse nuclear positivity for Epstein-Barr virus early RNAs in lesional cells. We present a report of this case and provide a summary of the literature regarding SMTs in pediatric HIV-infected patients. In addition, we draw attention to the cutaneous manifestations of SMTs in immunosuppressed populations. We highlight EBV-related SMTs as a potential cutaneous complication of HIV infection in children and immunosuppressed patients populations as a whole.
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Affiliation(s)
- Michael T Tetzlaff
- Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia, PA 19104, USA
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Petrilli G, Lorenzi L, Paracchini R, Ubiali A, Schumacher RF, Cabassa P, Facchetti F. Epstein-Barr virus-associated adrenal smooth muscle tumors and disseminated diffuse large B-cell lymphoma in a child with common variable immunodeficiency: a case report and review of the literature. Int J Surg Pathol 2011; 22:712-21. [PMID: 21454372 DOI: 10.1177/1066896911399901] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
This article reports the clinical and the histological features in a 7-year-old girl affected by common variable immunodeficiency (CVID) who developed multiple Epstein-Barr virus-associated tumors, represented by bilateral adrenal smooth muscle tumors (EBV-SMT) and multifocal diffuse large B-cell lymphoma. The EBV-SMTs showed features compatible with a benign or at least a low-malignant potential neoplasm. A peculiar feature observed in both EBV-SMTs was the occurrence of numerous lymphocytes intermingled with the spindle cells, which consisted of CD3+ CD5+ T-cells, with a predominant cytotoxic CD8+ component. Interestingly, EBV status differed in the neoplasms, since the EBV-SMTs were negative for LMP1 and positive for EBER, whereas the B-cell lymphoma expressed both EBV markers. Furthermore, EBV-LMP1 deletion was positive only in the EBV-SMTs, thus indicating that these tumors were the consequence of 2 distinct, EBV-dependent transformations. Similarly, lymphocyte clonality assay also showed different clonal bands in different sites (skin and nasal cavity), suggesting the development of intratumoral mutations. Finally, the authors review all 127 previously reported EBV-SMT, with discussion of their clinical and pathological features.
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19
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Purgina B, Rao UNM, Miettinen M, Pantanowitz L. AIDS-Related EBV-Associated Smooth Muscle Tumors: A Review of 64 Published Cases. PATHOLOGY RESEARCH INTERNATIONAL 2011; 2011:561548. [PMID: 21437186 PMCID: PMC3062098 DOI: 10.4061/2011/561548] [Citation(s) in RCA: 54] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 12/06/2010] [Accepted: 01/01/2011] [Indexed: 11/20/2022]
Abstract
The number of reported cases of smooth muscle tumor (SMT) arising in patients with AIDS has been increasing since the mid-1990s. The aim of this study is to characterize the epidemiology, clinical manifestations, pathologic features, prognosis and, management of Epstein-Barr virus-related SMT (EBV-SMT) in patients with AIDS. An English language literature search identified 53 articles including 64 reported cases of EBV-SMT. The majority of these reports involved patients who were young, severely immunosuppressed, and had multifocal tumors. The central nervous system was the most common site to be involved. Histologically, tumors had smooth muscle features and were immunoreactive for muscle markers and all but two tumors demonstrated the presence of EBV by either immunohistochemistry, in situ hybridization, and/or PCR. While mitoses and/or necrosis were used to separate leiomyoma from leiomyosarcoma, these features did not correlate with clinical outcome. Treatment included primarily resection, and less often radiotherapy, chemotherapy and highly active antiretroviral therapy (HAART). Overall, EBV-SMTs appear to have variable aggressiveness and clinical outcome and may exhibit a more favorable prognosis compared to conventional leiomyosarcoma. Tumor-related death from EBV-SMT occurred in only 4 of 51 patients.
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Affiliation(s)
- Bibianna Purgina
- Department of Pathology, Presbyterian-Shadyside Hospital, University of Pittsburgh Medical Center, Pittsburgh, PA 15232, USA
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20
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Kalil AN, Ferreira MT, Ressler F, Zettler C. Leiomioma hepático em paciente imunocompetente. Rev Col Bras Cir 2009; 36:362-3. [DOI: 10.1590/s0100-69912009000400017] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/20/2006] [Accepted: 06/18/2006] [Indexed: 11/22/2022] Open
Affiliation(s)
- Antonio Nocchi Kalil
- Complexo Hospitalar Santa Casa de Porto Alegre; Faculdade Federal de Ciências Médicas de Porto Alegre, BR
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21
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Neves AM, Thompson G, Carvalheira J, Trindade JC, Rueff J, Caetano JM, Casey JW, Hermouet S. Detection and quantitative analysis of human herpesvirus in pilocytic astrocytoma. Brain Res 2008; 1221:108-14. [PMID: 18565499 DOI: 10.1016/j.brainres.2008.05.009] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/28/2008] [Revised: 05/05/2008] [Accepted: 05/05/2008] [Indexed: 10/22/2022]
Abstract
We investigated the hypothetical role of human herpesviruses (HHVs) in tumour formation of the cerebellum. Thirty-five samples of pilocytic astrocytoma and 10 control samples of cerebellum from patients who died of unrelated diseases were examined. Presence of the 8 known HHVs was first studied using specific real-time quantitative Polymerase Chain Reaction (qPCR) targeting viral DNA polymerase. HHV's DNA polymerase was found present in 20 samples (7 controls, 13 astrocytomas) and was absent in 25 samples (3 controls, 22 astrocytomas). DNA polymerase of Epstein-Barr Virus (EBV) was present in 16 samples, 7/10 controls (70%) and 9/35 astrocytomas (26%). HHV-1 and Varicella-Zoster virus were detected only twice and HHV-2, Cytomegalovirus, HHV-7 and HHV-8, only once. HHV-6 was not detected. In all cases, the gene copy numbers of DNA polymerase were low (<100/100 ng DNA). A second approach was to search for novel HHVs, using consensus-degenerated hybrid oligonucleotide primers (CODEHOP) PCR: no sequence indicative of a new HHV was detected. In summary, EBV was the most frequent HHV detected in pilocytic astrocytoma, but at very low levels. According to the actually accepted threshold the results suggest that EBV cannot be considered responsible for tumorigenesis of pilocytic astrocytoma.
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Affiliation(s)
- Ana M Neves
- Department of Microbiology and Immunology, Cornell University, Ithaca, NY, USA.
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22
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Sprangers B, Smets S, Sagaert X, Wozniak A, Wollants E, Van Ranst M, Debiec-Rychter M, Sciot R, Vanrenterghem Y, Kuypers DR. Posttransplant Epstein-Barr virus-associated myogenic tumors: case report and review of the literature. Am J Transplant 2008; 8:253-8. [PMID: 18184312 DOI: 10.1111/j.1600-6143.2007.02054.x] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/25/2023]
Abstract
Epstein-Barr virus (EBV) has been implicated in the pathogenesis of different types of malignancies. While nonmelanoma skin cancers, lymphomas and Kaposi sarcomas are the most frequently reported malignancies after solid organ transplantation, EBV-associated smooth muscle tumors (EBV-SMT) after transplantation are rare and thus far only 18 cases in kidney recipients have been reported. A case of a 51-year-old kidney transplant recipient diagnosed with EBV-SMT is reported together with a review of the literature.
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Affiliation(s)
- B Sprangers
- Department of Nephrology, University Hospital Gasthuisberg, Leuven, Belgium
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23
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Atluri S, Neville K, Davis M, Robertson KA, Marshalleck FE, O'Malley DP, Buckley RH, Nelson RP. Epstein-Barr-associated leiomyomatosis and T-cell chimerism after haploidentical bone marrow transplantation for severe combined immunodeficiency disease. J Pediatr Hematol Oncol 2007; 29:166-72. [PMID: 17356396 DOI: 10.1097/mph.0b013e31803b95b3] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
BACKGROUND The clinical course of Epstein-Barr virus (EBV)-associated smooth muscle tumors is variable and there are no reports in patients with mixed T-cell chimerism after bone marrow transplantation (BMT). OBSERVATIONS A child with X-linked severe combined immunodeficiency disease developed multiple renal and pulmonary leiomyomata 8 years after haploidentical BMT. Epstein-Barr viral DNA was detectable in the blood and in situ hybridization for EBV-encoded RNAs was positive in the tumor. The tumors have been radiographically stable, chimerism remains mixed, and plasma EBV DNA has been repeatedly negative for over 2 years after donor lymphocyte infusion. CONCLUSIONS EBV-associated smooth muscle tumors may occur in patients who are partially reconstituted after BMT for severe combined immunodeficiency and may not require surgery or chemotherapy.
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Affiliation(s)
- Srilatha Atluri
- Department of Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA
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24
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Paolo WF, Nosanchuk JD. Adrenal infections. Int J Infect Dis 2006; 10:343-53. [PMID: 16483815 PMCID: PMC7110804 DOI: 10.1016/j.ijid.2005.08.001] [Citation(s) in RCA: 56] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/23/2005] [Revised: 08/01/2005] [Accepted: 08/08/2005] [Indexed: 11/18/2022] Open
Abstract
Adrenal infections are an important but under-recognized clinical entity. The adrenal gland can be infected by a myriad of pathogens including fungi, viruses, parasites, and bacteria. Infection can directly or indirectly cause tissue damage and alteration in endocrine function. Direct damage occurs via microbial replication and local production of toxic compounds, such as endotoxins. Indirect damage results from alterations in the regulation of a host's immunologic and endocrine mediators in response to damage by a microbe at a distant site. Variations in pathogen tropism, adrenal anatomy, and host immune integrity contribute to the progression of active disease and discernable adrenal dysfunction. Early recognition and intervention in the case of adrenal infection can significantly improve outcome, demonstrating the need for increased clinical suspicion in the appropriate clinical setting.
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25
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Hudnall SD, Ge Y, Wei L, Yang NP, Wang HQ, Chen T. Distribution and phenotype of Epstein-Barr virus-infected cells in human pharyngeal tonsils. Mod Pathol 2005; 18:519-27. [PMID: 15696119 DOI: 10.1038/modpathol.3800369] [Citation(s) in RCA: 93] [Impact Index Per Article: 4.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Abstract
Although Epstein-Barr virus (EBV) is often found in human tonsils, it remains to be precisely determined in what cells and microenvironment the virus is present. Although generally regarded as a B lymphotropic virus, EBV is associated with non-B-cell tumors, for example, NK/T-cell lymphoma, carcinoma, and leiomyosarcoma. To provide a basis for understanding the origin and biology of EBV-infected non-B cells, the immunophenotype of all EBV-infected cells in reactive human tonsils was determined by subjecting tonsil sections to dual/triple EBER in situ hybridization and immunohistochemistry with monoclonal antibodies to T cells (CD3, CD4, CD8, CCR3), B cells (CD20), plasma cells (CD138), natural killer (NK) cells (PEN5), and epithelial cells (cytokeratin), as well as frozen section immunostaining with antibodies to EBV latent proteins EBNA1, EBNA2, LMP1, and EBV early protein BZLF1. Most tonsils contained nearly equal numbers of EBNA1- and LMP1-positive cells (latency program) while only a few contained EBNA2-positive cells (growth program). More than 1000 EBER-positive cells from six tonsils were detected in the interfollicular zone (59%), tonsillar crypts (26%), and follicles (15%). Most (82%) EBER-positive cells are CD20-positive B cells, 7% are CD3-positive T cells, and 11% are cells of indeterminate lineage, often with plasmacytoid morphology. However, no EBER-positive plasma cells were identified. Rare EBER-positive NK cells and EBER/BZLF1-positive epithelial cells were identified. The direct demonstration of EBV within rare T cells, NK cells, and epithelial cells in reactive human tonsils provide a basis for further understanding of the origin of EBV-associated tumors of non-B-cell type.
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Affiliation(s)
- S David Hudnall
- Department of Pathology and Laboratory Medicine, University of Texas Medical Branch, Galveston, TX 77555-0741, USA.
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26
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Sclabas GM, Maurer CA, Wente MN, Zimmermann A, Büchler MW. Case report: hepatic leiomyoma in a renal transplant recipient. Transplant Proc 2002; 34:3200-2. [PMID: 12493419 DOI: 10.1016/s0041-1345(02)03563-7] [Citation(s) in RCA: 21] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Affiliation(s)
- G M Sclabas
- Department of Visceral and Transplantation Surgery, The University of Bern, Bern, Switzerland.
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27
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Chang JYF, Wang S, Hung CC, Tsai TF, Hsiao CH. Multiple Epstein-Barr virus-associated subcutaneous angioleiomyomas in a patient with acquired immunodeficiency syndrome. Br J Dermatol 2002; 147:563-7. [PMID: 12207602 DOI: 10.1046/j.1365-2133.2002.04818.x] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Tumours of smooth muscle origin, either solitary or multiple, are occasionally found in immunocompromised patients, particularly in children with acquired immunodeficiency syndrome (AIDS). Most of the reported AIDS-associated leiomyomatous neoplasms have been found in the visceral organs, and the tumour cells all possessed the Epstein-Barr virus (EBV) genome. Here we present a 32-year-old-man with AIDS who developed three skin nodules on his lower left extremity. No other tumorous lesions were found using computed tomography scans. Two of the three nodules were resected for pathological examination. Histologically, both tumours were well circumscribed and located in the subcutis. The tumours were composed of interlacing fascicles of spindle-shaped cells with prominent vasculature and lymphocytic infiltration. No pleomorphism, mitosis or necrosis was seen. Immunohistochemically, the tumour cells were reactive to smooth muscle actin and desmin. Angioleiomyoma was diagnosed. EBV-encoded small RNAs were also demonstrated in the nucleus of the tumour cells by in situ hybridization but no EBV receptor (CD21) or latent membrane protein (LMP)-1 was found in the tumour cells. No human herpesvirus (HHV)-8 genome was detected in the lesion using polymerase chain reaction analysis. The results of this study indicated that EBV containing subcutaneous angioleiomyoma was another neoplasm that must be considered in patients with human immunodeficiency virus infection who develop skin nodules. The role of EBV in the pathogenesis of this unique neoplasm is still unknown.
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Affiliation(s)
- J Y-F Chang
- Department of Pathology, National Taiwan University Hospital, no. 7 Chung-Shan South Road, Taipei, Taiwan
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28
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Cheuk W, Li PCK, Chan JKC. Epstein-Barr virus-associated smooth muscle tumour: a distinctive mesenchymal tumour of immunocompromised individuals. Pathology 2002; 34:245-9. [PMID: 12109785 DOI: 10.1080/00313020220131309] [Citation(s) in RCA: 44] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
immunosuppressed patients are predisposed to the development of smooth muscle tumours which show near consistent association with Epstein-Barr virus (EBV). This report describes a 37-year-old patient with acquired immunodeficiency syndrome who initially presented with two masses in the liver. Image-guided core biopsy revealed a spindle cell tumour with histological and immunological features of smooth muscle neoplasm which was shown by in situ hybridisation for EBV early RNAs to be EBV-associated. The literature on this uncommon entity is critically reviewed and the differential diagnosis is also discussed.
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Affiliation(s)
- W Cheuk
- Department of Pathology and Medicine, Queen Elizabeth Hospital, Kowloon, Hong Kong.
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29
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Jenson HB, Gulley ML, Puri P. Absence of Epstein-Barr virus in smooth muscle cells of idiopathic hypertrophic pyloric stenosis. Arch Pathol Lab Med 2001; 125:361-3. [PMID: 11231484 DOI: 10.5858/2001-125-0361-aoebvi] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
CONTEXT The etiology of idiopathic hypertrophic pyloric stenosis (IHPS) is unknown. Epstein-Barr virus (EBV) infects smooth muscle cells and is associated with leiomyomas and leiomyosarcomas of immunocompromised persons, including persons with the acquired immunodeficiency syndrome. OBJECTIVE To determine whether EBV is causally associated with IHPS. DESIGN Biopsy samples of the pylorus were obtained from 10 infants with projectile vomiting and pyloric hypertrophy on ultrasound, with confirmation of hypertrophy at the time of pyloromyotomy. The presence of EBV infection was tested by in situ hybridization for EBV-encoded RNA 1 (EBER1) in smooth muscle cells of IHPS. SETTING Biopsy specimens were obtained from children treated for IHPS at a tertiary referral hospital and were tested in a clinical molecular diagnostics laboratory. RESULTS All of the 10 smooth muscle biopsies were negative for EBER1. Cellular U6 RNA was detected in all smooth muscle samples, confirming that the RNA in the specimens was intact and capable of detection by in situ hybridization. CONCLUSIONS The absence of EBER1 in 10 cases of clinically diagnosed and histopathologically confirmed cases of IHPS effectively excludes EBV infection of smooth muscle cells as a causal factor in the pathogenesis of IHPS.
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Affiliation(s)
- H B Jenson
- Department of Pediatrics , The University of Texas Health Science Center at San Antonio, 78229-3900, USA
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30
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Kaminski AM, Cameron DC, French MA. Leiomyoma of the oesophagus in an HIV-infected patient. AUSTRALASIAN RADIOLOGY 2001; 45:49-51. [PMID: 11259973 DOI: 10.1046/j.1440-1673.2001.00874.x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Leiomyomas of the oesophagus are uncommon, and have not been reported in patients infected with the human immunodeficiency virus (HIV). A case of an oesophageal leiomyoma in an adult infected with HIV is presented.
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Affiliation(s)
- A M Kaminski
- Department of Radiology, Royal Perth Hospital, Perth, Western Australia, Australia
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31
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Citow JS, Kranzler L. Multicentric intracranial smooth-muscle tumor in a woman with human immunodeficiency virus. Case report. J Neurosurg 2000; 93:701-3. [PMID: 11014553 DOI: 10.3171/jns.2000.93.4.0701] [Citation(s) in RCA: 22] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
A 31-year-old woman with acquired immunodeficiency syndrome (AIDS) was found to harbor both a pulmonary smooth-muscle tumor and an intracranial extraaxial smooth-muscle tumor of the parasellar region. The frequency of smooth-muscle tumors (leiomyoma and leiomyosarcoma) has increased with AIDS, but much more so in children than in adults. Only nine cases of human immunodeficiency virus-related smooth-muscle tumors have been previously reported in adults, and only one of these was located intracranially.
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Affiliation(s)
- J S Citow
- Section of Neurosurgery, The University of Chicago Hospitals, Illinois, USA.
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32
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Fosslien E. Biochemistry of cyclooxygenase (COX)-2 inhibitors and molecular pathology of COX-2 in neoplasia. Crit Rev Clin Lab Sci 2000; 37:431-502. [PMID: 11078056 DOI: 10.1080/10408360091174286] [Citation(s) in RCA: 187] [Impact Index Per Article: 7.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Several types of human tumors overexpress cyclooxygenase (COX) -2 but not COX-1, and gene knockout transfection experiments demonstrate a central role of COX-2 in experimental tumorigenesis. COX-2 produces prostaglandins that inhibit apoptosis and stimulate angiogenesis and invasiveness. Selective COX-2 inhibitors reduce prostaglandin synthesis, restore apoptosis, and inhibit cancer cell proliferation. In animal studies they limit carcinogen-induced tumorigenesis. In contrast, aspirin-like nonselective NSAIDs such as sulindac and indomethacin inhibit not only the enzymatic action of the highly inducible, proinflammatory COX-2 but the constitutively expressed, cytoprotective COX-1 as well. Consequently, nonselective NSAIDs can cause platelet dysfunction, gastrointestinal ulceration, and kidney damage. For that reason, selective inhibition of COX-2 to treat neoplastic proliferation is preferable to nonselective inhibition. Selective COX-2 inhibitors, such as meloxicam, celecoxib (SC-58635), and rofecoxib (MK-0966), are NSAIDs that have been modified chemically to preferentially inhibit COX-2 but not COX-1. For instance, meloxicam inhibits the growth of cultured colon cancer cells (HCA-7 and Moser-S) that express COX-2 but has no effect on HCT-116 tumor cells that do not express COX-2. NS-398 induces apoptosis in COX-2 expressing LNCaP prostate cancer cells and, surprisingly, in colon cancer S/KS cells that does not express COX-2. This effect may due to induction of apoptosis through uncoupling of oxidative phosphorylation and down-regulation of Bcl-2, as has been demonstrated for some nonselective NSAIDs, for instance, flurbiprofen. COX-2 mRNA and COX-2 protein is constitutively expressed in the kidney, brain, spinal cord, and ductus deferens, and in the uterus during implantation. In addition, COX-2 is constitutively and dominantly expressed in the pancreatic islet cells. These findings might somewhat limit the use of presently available selective COX-2 inhibitors in cancer prevention but will probably not deter their successful application for the treatment of human cancers.
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Affiliation(s)
- E Fosslien
- Department of Pathology, College of Medicine, University of Illinois at Chicago, 60612, USA.
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33
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Ritter AM, Amaker BH, Graham RS, Broaddus WC, Ward JD. Central nervous system leiomyosarcoma in patients with acquired immunodeficiency syndrome. Report of two cases. J Neurosurg 2000; 92:688-92. [PMID: 10761660 DOI: 10.3171/jns.2000.92.4.0688] [Citation(s) in RCA: 41] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Abstract
Leiomyosarcomas (LMSs) of the central nervous system are extremely rare; however, they are becoming more prevalent in immunocompromised patients. The authors present the cases of two patients with acquired immunodeficiency syndrome: one with LMS of the thoracic vertebral body and the other with LMS originating from the region of the cavernous sinus. The epidemiological and histological characteristics of LMS and its association with latent Epstein-Barr virus are discussed, as well as the treatments for this neoplasm.
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Affiliation(s)
- A M Ritter
- Division of Neurosurgery, Medical College of Virginia, Virginia Commonwealth University, Richmond, USA
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34
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Hsu JL, Glaser SL. Epstein-barr virus-associated malignancies: epidemiologic patterns and etiologic implications. Crit Rev Oncol Hematol 2000; 34:27-53. [PMID: 10781747 DOI: 10.1016/s1040-8428(00)00046-9] [Citation(s) in RCA: 195] [Impact Index Per Article: 7.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/01/2023] Open
Abstract
Epstein-Barr virus (EBV), a ubiquitous B-lymphotrophic herpesvirus, has been found in the tumor cells of a heterogeneous group of malignancies (Burkitt's lymphoma, lymphomas associated with immunosuppression, other non-Hodgkin's lymphomas, Hodgkin's disease, nasopharyngeal carcinoma, gastric adenocarcinoma, lymphoepithelioma-like carcinomas, and immunodeficiency-related leiomyosarcoma). As the epidemiologic characteristics of these cancers have not been considered together, this review seeks to relate their incidence patterns and risk factors to EBV biology and virus-host interaction in an attempt to help elucidate factors involved in EBV-related carcinogenesis. We include a brief review of EBV virology and primary infection to provide a biologic context for considering the epidemiology, summarize the most salient epidemiologic features of each malignancy, synthesize epidemiologic data by risk factor to uncover commonalities and informative contrasts across the diseases, and propose hypotheses regarding etiologic mechanisms, based on the possible effect of the risk factors at various stages in the viral life cycle.
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Affiliation(s)
- J L Hsu
- Northern California Cancer Center, 32960 Alvarado-Niles Road, Suite 600, Union City, CA 94587, USA
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35
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Tsuji M, Takenaka R, Kashihara T, Hadama T, Terada N, Mori H. Primary hepatic leiomyosarcoma in a patient with hepatitis C virus-related liver cirrhosis. Pathol Int 2000; 50:41-7. [PMID: 10692176 DOI: 10.1046/j.1440-1827.2000.00999.x] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
We describe an autopsy case of primary hepatic leiomyosarcoma in a 68-year-old man with hepatitis C virus-related liver cirrhosis. The patient, who had a history of acute hepatitis 20 years previously, died of a ruptured hepatic tumor. At autopsy, a well-circumscribed 14 x 16 x 15 cm tumor replaced the medial site of the right hepatic lobe with multiple intrahepatic and distant metastases. Histologically the tumor, which had extensive central necrosis, consisted predominantly of well or moderately differentiated spindle-shaped cells, which were positive for smooth muscle actin and vimentin on immunohistochemical staining. In addition, clusters of markedly atypical cells and myxoid change of the matrix were discretely found in the focal and small areas of the tumor. These findings indicated that many sections were necessary for the histologically accurate estimation of primary hepatic smooth muscle tumor. The histological examination of a non-tumorous lesion showed liver cirrhosis. Hepatitis C virus was detected in the cytoplasm of cirrhotic hepatocytes by immunohistochemistry and reverse transcriptase-polymerase chain reaction, but not in the tumor cells. This suggested that the virus was not directly involved in the development of primary hepatic leiomyosarcoma.
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Affiliation(s)
- M Tsuji
- Division of Pathology, Department of Laboratory Medicine, Osaka Medical College Hospital, Takatsuki, Osaka, Japan.
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36
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Lam KY. Oesophageal mesenchymal tumours: clinicopathological features and absence of Epstein-Barr virus. J Clin Pathol 1999; 52:758-60. [PMID: 10674034 PMCID: PMC501571 DOI: 10.1136/jcp.52.10.758] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/18/2023]
Abstract
BACKGROUND Recent studies have suggested that the Epstein-Barr virus (EBV) is associated with smooth muscle tumours (leiomyoma and leiomyosarcoma) in patients with human immunodeficiency virus and in organ transplant recipients. Leiomyoma is the most common mensenchymal tumour found in the oesophagus. AIM To report a single institution experience on oesophageal mesenchymal tumours and to determine whether EBV is associated with these tumours. METHODS 40 sporadic oesophageal mesenchymal tumours were studied and their diagnosis confirmed on pathological review and immunohistochemical studies. Formalin fixed, paraffin was embedded tissues from these tumours were analysed for EBV using in situ hybridisation for two messenger RNA (mRNA) probes, EBER and BamH1 W. RESULTS The oesophageal mesenchymal tumours comprised 36 leiomyomas, two undifferentiated stromal tumours, and two gastrointestinal autonomic nerve tumours (GANTs). Median age of the patients with leiomyoma (26 men, 10 women) was 62 years (range 30 to 85) and 81% of them had an asymptomatic lesion. The median longitudinal size was 1.2 cm. Multiple leiomyomas were seen in 11% of the patients and calcification was noted in one tumour. Coexisting squamous cell carcinoma was found in one third of cases. The stromal tumours were small, asymptomatic, and located in the lower third of the oesophagus, while the GANTs were large, symptomatic, and found in the upper third of the oesophagus. EBV mRNAs were not detected in all these tumours. CONCLUSIONS The clinicopathological features of oesophageal leiomyoma, undifferentiated stromal tumour, and GANT were different. Some oesophageal leiomyomas were associated with oesophageal squamous cell carcinomas. EBV is not associated with sporadic oesophageal mesenchymal tumours.
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Affiliation(s)
- K Y Lam
- Department of Pathology, University of Hong Kong, Hong Kong.
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37
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Bluhm JM, Yi ES, Diaz G, Colby TV, Colt HG. Multicentric endobronchial smooth muscle tumors associated with the epstein-barr virus in an adult patient with the acquired immunodeficiency syndrome. Cancer 1997. [DOI: 10.1002/(sici)1097-0142(19971115)80:10<1910::aid-cncr6>3.0.co;2-r] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
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de Chadarévian JP, Wolk JH, Inniss S, Lischner HW, d'Amore F, Faerber EN, Isaacson GC. A newly recognized cause of wheezing: AIDS-related bronchial leiomyomas. Pediatr Pulmonol 1997; 24:106-10. [PMID: 9292901 DOI: 10.1002/(sici)1099-0496(199708)24:2<106::aid-ppul5>3.0.co;2-m] [Citation(s) in RCA: 19] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
We present two human immunodeficiency virus-infected children who developed wheezing and radiological evidence of pulmonary air trapping due to intra- and peribronchial leiomyomas. At autopsy, leiomyomas were also found in their spleens, which to our knowledge, has never been reported. The smooth muscle tumors were strongly positive for the Epstein-Barr virus, as demonstrated by in situ hybridization to Epstein-Barr virus-encoded ribonucleic acid, confirming the findings of recent investigators and linking these tumors to the Epstein-Barr virus.
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Affiliation(s)
- J P de Chadarévian
- Department of Pathology, St. Christopher's Hospital for Children, Philadelphia, Pennsylvania 19134-1095, USA
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Abstract
Specific pathologic processes, particularly oral, esophageal, and intestinal infections, are common in the alimentary tract of AIDS patients. Many of these diseases are adequately assessed only by biopsy with histologic examination. Most are rare or unreported in immunocompetent hosts and are easily missed by those not familiar with them. This article describes the gross or endoscopic and histologic appearances and the diagnostic criteria for enteric pathologic processes seen in HIV-infected individuals.
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Niedobitek G, Agathanggelou A, Herbst H, Whitehead L, Wright DH, Young LS. Epstein-Barr virus (EBV) infection in infectious mononucleosis: virus latency, replication and phenotype of EBV-infected cells. J Pathol 1997; 182:151-9. [PMID: 9274524 DOI: 10.1002/(sici)1096-9896(199706)182:2<151::aid-path824>3.0.co;2-3] [Citation(s) in RCA: 137] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
Primary Epstein-Barr virus (EBV) infection may manifest itself as a benign lymphoproliferative disorder, infections mononucleosis (IM). EBV infection has been characterized in lymphoreticular tissues from nine patients with IM using the abundantly expressed EBV-encoded nuclear RNAs (EBERs) as a marker of latent infection. Expression of the virus-encoded nuclear antigen (EBNA) 2 and of the latent membrane protein (LMP) 1 was seen in variable proportions of cells in all cases. Double labelling revealed heterogeneous expression patterns of these proteins. Thus, in addition to cells revealing phenotypes consistent with latencies I (EBNA2-/LMP1-) and III (EBNA2+/LMP1+), cells displaying a latency II pattern (EBNA2-/LMP1+) were observed. Cells expressing EBNA2 but not LMP1 were also detected; whilst this may represent a transitory phenomenon, the exact significance of this observation is at present uncertain. EBER-specific in situ hybridization in conjunction with immunohistochemistry revealed expression of the EBERs mainly in B-lymphocytes, many of which showed features of plasma cell differentiation. By contrast, convincing evidence of latent EBV infection was not found in T-cells, epithelial or endothelial cells. Double-labelling immunohistochemistry revealed expression of the replication-associated BZLF1 protein in small lymphoid cells, often showing plasmacytoid differentiation. There was no unambiguous expression of this protein in other cell types. These results suggest that B-cells are the primary target of EBV infection and that plasma cells may be a source of infectious virus found in the saliva of IM patients.
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Affiliation(s)
- G Niedobitek
- Department of Pathology, University of Birmingham, U.K
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Mierau GW, Greffe BS, Weeks DA. Primary leiomyosarcoma of brain in an adolescent with common variable immunodeficiency syndrome. Ultrastruct Pathol 1997; 21:301-5. [PMID: 9183831 DOI: 10.3109/01913129709021926] [Citation(s) in RCA: 45] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
A 14-year-old girl with common variable immunodeficiency syndrome was found to have a low-grade malignant neoplasm arising in the left temporal lobe of the brain. Ultrastructural and immunohistochemical studies established a diagnosis of leiomyosarcoma, despite the rarity of this tumor in children. In situ hybridization with the EBER probe revealed essentially all of the neoplastic cells to be infected with Epstein-Barr virus (EBV). Children with the acquired immunodeficiency syndrome (AIDS) are known to exhibit an increased incidence of smooth muscle tumors associated with EBV. Similar tumors have been reported in EBV-infected patients undergoing therapeutic immunosuppression. This appears to be the first reported case of childhood leiomyosarcoma where the cause of the underlying immunodeficiency was a genetic rather than acquired disorder. The authors conclude that electron microscopy, immunohistochemistry, and other ancillary techniques are essential in the evaluation of unusual tumors in immunocompromised children, whether the cause is hereditary or acquired.
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Affiliation(s)
- G W Mierau
- Department of Pathology, Children's Hospital, Denver, CO 80218, USA
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Morgello S, Kotsianti A, Gumprecht JP, Moore F. Epstein-Barr virus-associated dural leiomyosarcoma in a man infected with human immunodeficiency virus. Case report. J Neurosurg 1997; 86:883-7. [PMID: 9126907 DOI: 10.3171/jns.1997.86.5.0883] [Citation(s) in RCA: 35] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
A 35-year-old man infected with human immunodeficiency virus presented with cervical myelopathy of 2 months duration. Clinical and radiographic evaluation revealed a discrete, subdural mass at C-6. At surgery, the mass proved to have a dural attachment and thus clinically, radiographically, and grossly, it resembled meningioma. Histopathological analysis revealed a leiomyosarcoma that stained diffusely for muscle-specific actin. Electron microscopy revealed basal lamina surrounding the tumor cells and intracytoplasmic bundles of myofilaments. Epstein-Barr virus (EBV) was demonstrated within tumor cell nuclei by in situ hybridization for EBER1 messenger RNA and immunohistochemical staining for EBNA2 protein. Epstein-Barr virus latent membrane protein (LMP1) was not detected. This is the first documentation of an EBV-associated smooth-muscle tumor of the dura, and the first demonstration that tumors in this location contain EBV in an unusual form of latency not seen in lymphoid cell lines. With increasing numbers of individuals being afflicted with long-term immunosuppression, EBV-associated dural leiomyoma and leiomyosarcoma may be encountered more frequently in the future.
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Affiliation(s)
- S Morgello
- Department of Pathology, The Mount Sinai Medical Center, New York, New York 10029, USA.
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Sars PR, Molenaar WM, Koudstaal J, Hoekstra HJ. Simultaneous epstein barr virus and cytomegalovirus infection accompanied by leiomyomatous change in a well-differentiated liposarcoma in a patient with long-term corticosteroid treatment. Sarcoma 1997; 1:55-8. [PMID: 18521201 PMCID: PMC2373577 DOI: 10.1080/13577149778489] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/01/2022] Open
Abstract
Patient. A 59-year-old woman presented with a large tumour of the abdominal wall. She had been taking corticosteroids for severe chronic obstructive pulmonary disease for 15 years. On CT scan the tumour had the characteristics of lipomatous tissue with a dense core.Results. Histology showed a well-differentiated liposarcoma with a core of benign fibroleiomyomatous differentiation. Within the core, a third component was observed, characterized by more pleomorphism and the presence of an inflammatory infiltrate. In this component, immunoperoxidase stains and in situ hybridization demonstrated cytomegalovirus (CMV) and Epstein Barr virus (EBV) infection in large and small cells, respectively.Discussion. Long-term corticosteroid use for pulmonary disease may extend the list of immunosuppressed states associated with the development of leiomyomatous tumours with EBV infection, previously described in AIDS patients and liver transplant recipients. The role of CMV is uncertain.
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Affiliation(s)
- P R Sars
- Department of Surgical Oncology Groningen University Hospital The Netherlands
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Abstract
BACKGROUND In the classification of tumors of soft tissue, modern schemes describe tumors by the normal adult tissue type the tumor resembles. Thus, tumors are described as smooth muscle tumors if the cells are differentiating towards smooth muscle. We may infer that in fact the tumor arose from smooth muscle, but this is only an inference. Tumors showing differentiation towards smooth muscle include hamartomas, benign tumors, and malignant tumors. OBJECTIVE This review article describes the clinical presentation and course, histology, and treatment recommendations for benign and malignant smooth muscle tumors. METHODS An extensive literature review of tumors with differentiation towards smooth muscle. RESULTS Benign tumors exhibiting differentiation towards smooth muscle include smooth muscle hamartoma and leiomyoma. Myofibroma is a third tumor that some have argued is a smooth muscle tumor rather than a fibroblastic tumor. Characteristic fusiform shaped cells with a round central nucleus arranged in fasicles suggest smooth muscle differentiation. Special stains such as phosphotungenistic acid-hemotoxilin, analine blue, and Masson's trichrome are helpful in differentiating muscle from collagen. Immunohistochemical stains are also helpful in establishing a diagnosis. With solitary tumors, treatment for cosmesis or for painful leiomyomas is not a problem. Multiple leiomyomas, which may be painful or sensitive to cold or touch, are a therapeutic challenge, with reports of multiple medications being attempted in the literature. Leiomyosarcoma are malignant tumors of smooth muscle. They may be cutaneous and presumably arise from the arrector pilorum muscle, or subcutaneous, where they are believed to arise from vascular smooth muscle. Cutaneous leiomyosarcomas frequently reoccur following excision, but rarely metastasize. Subcutaneous leiomyosarcomas frequently reoccur following excision and have a high metastatic rate. Several recent reports have documented an increased frequency of visceral leiomyosarcomas in immunosuppressed patients. These tumors have been found to contain the Epstein Barr virus. Treatment of leiomyosarcoma is wide excision. CONCLUSIONS Smooth muscle tumors are rare neoplasms that may confront the dermatologic surgeon. While leiomyomas are benign, their frequent sensitivity or pain necessitates treatment. Leiomyosarcomas are malignancies with a high reoccurrence rate, and when deep, a high metastatic rate. The finding of an increased frequency of visceral leiomyomas and leiomyosarcomas in immunosuppressed patients may imply an increased frequency of cutaneous leiomyomas and leiomyosarcomas in this patient population.
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Affiliation(s)
- J M Spencer
- Department of Dermatology and Cutaneous Surgery, University of Miami, FL 33101, USA
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Jimenez-Heffernan JA, Hardisson D, Palacios J, Garcia-Viera M, Gamallo C, Nistal M. Adrenal gland leiomyoma in a child with acquired immunodeficiency syndrome. PEDIATRIC PATHOLOGY & LABORATORY MEDICINE : JOURNAL OF THE SOCIETY FOR PEDIATRIC PATHOLOGY, AFFILIATED WITH THE INTERNATIONAL PAEDIATRIC PATHOLOGY ASSOCIATION 1995; 15:923-9. [PMID: 8705202 DOI: 10.3109/15513819509027028] [Citation(s) in RCA: 24] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/01/2023]
Abstract
An adrenal gland leiomyoma was incidentally found at autopsy in a 2-year-old boy with acquired immunodeficiency syndrome (AIDS). Smooth muscle neoplasms have been described in pediatric AIDS and affect mainly the tracheobronchopulmonary, gastrointestinal, and hepatobiliary systems. An association between Epstein-Barr virus (EBV) and smooth muscle tumors in patients with AIDS has been described. This case is the first leiomyoma to be reported in the adrenal gland of a child with AIDS. EBV genome was demonstrated in the tumor by polymerase chain reaction.
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