|
1
|
Porciani C, Colombatto P, Guadagni S, Ricco G, Morelli L, Caponi L, Campani D, Comandatore A, Sciume' GD, Migliorini P, Boraschi P, Brunetto M, Candio GD. Stauffer syndrome in a tumor of the pancreatic tail: A paracrine pathogenesis, rather than an inflammatory disimmune phenomenon – a case report. CURRENT PROBLEMS IN CANCER: CASE REPORTS 2024; 15:100320. [DOI: 10.1016/j.cpccr.2024.100320] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2024] Open
|
|
2
|
Ferrell LD, Kakar S, Terracciano LM, Wee A. Tumours and Tumour-Like Lesions. MACSWEEN'S PATHOLOGY OF THE LIVER 2024:842-946. [DOI: 10.1016/b978-0-7020-8228-3.00013-2] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
|
|
3
|
Muñoz Rossi FA, Gallo D, Guaiquil AM, Suarez R, Ricardo Ossio GP. Stauffer Syndrome: Another Strange Case of the Internist's Tumor. Cureus 2023; 15:e42307. [PMID: 37614277 PMCID: PMC10443892 DOI: 10.7759/cureus.42307] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 07/22/2023] [Indexed: 08/25/2023] Open
Abstract
Stauffer syndrome is a paraneoplastic disorder associated with renal cell carcinoma (RCC), which often can be the first manifestation of the tumor. It is characterized by nephrogenic hepatic dysfunction that reverses after removing the primary tumor. Stauffer syndrome has been associated with a worse prognosis in patients with RCC. We are presenting a case of a patient with an uncommon cholestatic jaundice variant. The patient also had hepatic profile alterations, but no metastasis existed. These findings meet the diagnostic criteria for Stauffer syndrome in the context of RCC as the first clinical manifestation in the diagnostic approach to malignancy-associated disease. A 74-year-old patient, with a history of obesity, hypertension, and type 2 diabetes mellitus, went for advice due to right upper abdominal pain, icterus accompanied by emetic episodes, and a cholestatic pattern in the hepatic profile. Autoimmune hepatitis was ruled out based on immunological testing. Imaging revealed evidence of a mass in the lower pole of the right kidney, suspicious of malignant neoplasia, and a distant paraneoplastic syndrome consistent with the cholestatic variant of Stauffer syndrome. This is an exciting case of Stauffer syndrome as the initial presentation of RCC associated with a cholestatic variant, providing relevant information about this uncommon condition in our setting.
Collapse
Affiliation(s)
| | - Diana Gallo
- Medical Affairs, Universidad Militar Nueva Granada, Bogota, COL
| | - Ana Maria Guaiquil
- Research in Health Sciences, Universitat Internacional de Catalunya, Catalunya, ESP
| | - Ralf Suarez
- Medical Affairs, Universidad Metropolitana de Barranquilla, Medellin, COL
| | | |
Collapse
|
|
4
|
Deacon AJ, Goetz NN, Weber N, Clouston A, Gonsalkorala E, Baskerville C, Leggett B. Relapsed nodular lymphocyte-predominant Hodgkin lymphoma presenting as severe paraneoplastic hepatitis: a case report. J Med Case Rep 2023; 17:269. [PMID: 37386640 DOI: 10.1186/s13256-023-04014-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/08/2022] [Accepted: 05/27/2023] [Indexed: 07/01/2023] Open
Abstract
BACKGROUND Hematological malignancies are an infrequent but important cause of liver dysfunction. There are several mechanisms by which this can occur, including direct malignant infiltration of the hepatic parenchyma and/or vasculature, vanishing bile duct syndrome, and paraneoplastic hepatitis. Paraneoplastic hepatitis is an extremely rare mechanism by which a hematological malignancy can cause liver dysfunction, and we present the first case, to our knowledge, of paraneoplastic hepatitis caused by nodular lymphocyte-predominant Hodgkin lymphoma in the literature. CASE PRESENTATION A 28-year-old Caucasian male presented with 3 weeks of fatigue, epigastric pain, and jaundice. His medical history was significant for early stage nodular lymphocyte-predominant Hodgkin lymphoma in the cervical region in remission for 5 years after primary treatment with involved-field radiotherapy. Liver biochemistry was normal at the time of treatment for lymphoma and there was no known liver disease before the current presentation. On physical examination, there was scleral icterus and ecchymoses, but no evidence of hepatic encephalopathy, other stigmata of chronic liver disease, or lymphadenopathy. A computed tomography scan of his neck, chest, abdomen, and pelvis showed heterogeneous enhancement of the liver, multiple enlarged upper abdominal lymph nodes, and an enlarged spleen with multiple rounded lesions. Portal and hepatic veins were patent. Initial workup for viral, autoimmune-, toxin-, and medication-related hepatitis was negative. A transjugular liver biopsy was performed with histology showing a predominantly T-cell mediated hepatitis with very extensive multiacinar hepatic necrosis, but no evidence of lymphoma within the liver. Retroperitoneal lymph node biopsy revealed nodular lymphocyte-predominant Hodgkin lymphoma. The patient's symptoms, bilirubin, and transaminases improved significantly after treatment with oral prednisolone and a staged introduction of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy. CONCLUSIONS Nodular lymphocyte-predominant Hodgkin lymphoma may cause paraneoplastic hepatitis. Physicians should be aware of the possibility of this life-threatening presentation and the importance of early liver biopsy and treatment before acute liver failure occurs. Interestingly, paraneoplastic hepatitis did not occur when nodular lymphocyte-predominant Hodgkin lymphoma was first diagnosed and confined to the cervical region, but was the presenting feature of the recurrence below the diaphragm.
Collapse
Affiliation(s)
- Anthony J Deacon
- Department of Gastroenterology and Hepatology, Royal Brisbane and Women's Hospital, Brisbane, Australia.
- Faculty of Medicine, University of Queensland, Brisbane, Australia.
| | - Naeman N Goetz
- Department of Gastroenterology and Hepatology, Royal Brisbane and Women's Hospital, Brisbane, Australia
- Faculty of Medicine, University of Queensland, Brisbane, Australia
| | - Nicholas Weber
- Department of Haematology, Royal Brisbane and Women's Hospital, Brisbane, QLD, Australia
| | - Andrew Clouston
- Pathology Queensland, Royal Brisbane and Women's Hospital, Brisbane, QLD, Australia
| | - Enoka Gonsalkorala
- Department of Gastroenterology and Hepatology, Royal Brisbane and Women's Hospital, Brisbane, Australia
| | - Catherine Baskerville
- Department of Internal Medicine and Aged Care, Royal Brisbane and Women's Hospital, Brisbane, QLD, Australia
| | - Barbara Leggett
- Department of Gastroenterology and Hepatology, Royal Brisbane and Women's Hospital, Brisbane, Australia
- Faculty of Medicine, University of Queensland, Brisbane, Australia
| |
Collapse
|
|
5
|
Peringeth G, Torka P, Wong J, Hernandez-Ilizaliturri FJ. Successful Treatment of Paraneoplastic Cholestasis in Relapsed/Refractory Hodgkin Lymphoma With Bridging Therapy and Checkpoint Blockade. CLINICAL LYMPHOMA MYELOMA & LEUKEMIA 2020; 20:e316-e319. [PMID: 32179034 DOI: 10.1016/j.clml.2020.01.013] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/24/2019] [Revised: 01/05/2020] [Accepted: 01/20/2020] [Indexed: 02/07/2023]
Affiliation(s)
- Gopisree Peringeth
- Department of Medicine, Roswell Park Comprehensive Cancer Center, Buffalo, NY
| | - Pallawi Torka
- Department of Medicine, Roswell Park Comprehensive Cancer Center, Buffalo, NY
| | - Jerry Wong
- Department of Pathology and Laboratory Medicine, Roswell Park Comprehensive Cancer Center, Buffalo, NY
| | | |
Collapse
|
|
6
|
Cutaneous Large B Cell Lymphoma Involving the Duodenum and the Bile Duct: a Case Report. J Gastrointest Cancer 2018; 50:681-683. [PMID: 29766412 DOI: 10.1007/s12029-018-0112-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/16/2022]
|
|
7
|
Fontes-Sousa M, Magalhães H, da Silva FC, Maurício MJ. Stauffer's syndrome: A comprehensive review and proposed updated diagnostic criteria. Urol Oncol 2018; 36:321-326. [PMID: 29657090 DOI: 10.1016/j.urolonc.2018.01.019] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/12/2017] [Revised: 12/07/2017] [Accepted: 01/28/2018] [Indexed: 12/20/2022]
Abstract
BACKGROUND Stauffer's syndrome corresponds to a set of clinical and analytical changes of paraneoplastic nature firstly recognized more than 50 years ago, in association to renal cell carcinoma. A definitive review including universal diagnostic criteria and updated knowledge since the original description is lacking. BASIC PROCEDURES The authors conducted a comprehensive bibliographical review and propose updated diagnostic criteria to standardize diagnosis for clinical practice purposes and avoid misclassification. MAIN FINDINGS Although having been described in association with renal cell carcinoma, the syndrome has been reported in correlation with other malignancies-either solid or hematological tumors. Additionally, a variant syndrome presenting with jaundice has also been characterized, but appears to have a similar clinical course to that of the classical Stauffer's syndrome. Although often described as rare, it may be more frequent than previously recognized. Stauffer's syndrome etiopathogenesis is still poorly understood, but immune mechanisms seem to play a role underscored by the malignancies to which the syndrome is associated, several of which having immunotherapy drugs approved for their treatment. PRINCIPAL CONCLUSIONS A set of diagnostic criteria should be used to simplify, broaden and standardized diagnosis, under the entity characterized by reversible paraneoplastic intrahepatic cholestasis. Clinicians should be aware of the syndrome, namely consider further investigation if a plausible cause for unexplained intrahepatic cholestasis in an otherwise healthy patient is not found. Even though no universal approach is available, investigation should be considered regarding metastatic disease after resection of a primary tumor which has revealed persistence or recurrence of symptoms.
Collapse
Affiliation(s)
- Mário Fontes-Sousa
- Department of Medical Oncology, Portuguese Oncology Institute of Porto, Porto, Portugal; Portuguese Genitourinary Group (GPGU), Lisbon, Portugal.
| | - Helena Magalhães
- Department of Medical Oncology, Portuguese Oncology Institute of Porto, Porto, Portugal; Portuguese Genitourinary Group (GPGU), Lisbon, Portugal
| | | | - Maria Joaquina Maurício
- Department of Medical Oncology, Portuguese Oncology Institute of Porto, Porto, Portugal; Portuguese Genitourinary Group (GPGU), Lisbon, Portugal
| |
Collapse
|
|
8
|
Metastatic Prostate Cancer Manifesting as Cholestatic Jaundice: A Case Report and Review of the Literature. Case Rep Oncol Med 2018; 2018:1809432. [PMID: 29780650 PMCID: PMC5892301 DOI: 10.1155/2018/1809432] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/21/2017] [Accepted: 02/26/2018] [Indexed: 11/18/2022] Open
Abstract
A paraneoplastic syndrome can often present as the first manifestation of an underlying malignancy. We report a patient who presented with cholestatic jaundice as a paraneoplastic syndrome from his newly diagnosed metastatic prostate cancer. He received initial treatment with androgen deprivation therapy followed by six cycles of docetaxel resulting in resolution of his cholestatic process, normalization of liver enzyme levels, and excellent biochemical and radiographic response. To the best of our knowledge, this is the first reported case of metastatic prostate cancer with cholestatic jaundice as a paraneoplastic phenomenon to be safely treated with androgen deprivation therapy and upfront docetaxel, reflecting the latest shift in the treatment of metastatic prostate cancer.
Collapse
|
|
9
|
Ferrell LD, Kakar S, Terracciano LM, Wee A. Tumours and Tumour-like Lesions of the Liver. MACSWEEN'S PATHOLOGY OF THE LIVER 2018:780-879. [DOI: 10.1016/b978-0-7020-6697-9.00013-3] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
|
|
10
|
Bakhit M, McCarty TR, Park S, Njei B, Cho M, Karagozian R, Liapakis A. Vanishing bile duct syndrome in Hodgkin’s lymphoma: A case report and literature review. World J Gastroenterol 2017; 23:366-372. [PMID: 28127210 PMCID: PMC5236516 DOI: 10.3748/wjg.v23.i2.366] [Citation(s) in RCA: 43] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/26/2016] [Revised: 07/04/2016] [Accepted: 08/05/2016] [Indexed: 02/06/2023] Open
Abstract
Vanishing bile duct syndrome (VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated with malignancy. It is an acquired condition characterized by progressive destruction and loss of the intra-hepatic bile ducts leading to cholestasis. Prognosis is variable and partially dependent upon the etiology of bile duct injury. Irreversible bile duct loss leads to significant ductopenia, biliary cirrhosis, liver failure, and death. If biliary epithelial regeneration occurs, clinical recovery may occur over a period of months to years. VBDS has been described in a number of cases of patients with Hodgkin’s lymphoma (HL) where it is thought to be a paraneoplastic phenomenon. This case describes a 25-year-old man found on liver biopsy to have VBDS. Given poor response to medical treatment, the patient underwent transplant evaluation at that time and was found to have classical stage IIB HL. Early recognition of this underlying cause or association of VBDS, including laboratory screening, and physical exam for lymphadenopathy are paramount to identifying potential underlying VBDS-associated malignancy. Here we review the literature of HL-associated VBDS and report a case of diagnosed HL with biopsy proven VBDS.
Collapse
|
|
11
|
Rota Scalabrini D, Caravelli D, Carnevale Schianca F, D'Ambrosio L, Tolomeo F, Boccone P, Manca A, De Rosa G, Nuzzo A, Aglietta M, Grignani G. Complete remission of paraneoplastic vanishing bile duct syndrome after the successful treatment of Hodgkin's lymphoma: a case report and review of the literature. BMC Res Notes 2014; 7:529. [PMID: 25125318 PMCID: PMC4143581 DOI: 10.1186/1756-0500-7-529] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2014] [Accepted: 08/04/2014] [Indexed: 12/16/2022] Open
Abstract
BACKGROUND Vanishing bile duct syndrome has been associated with different pathologic conditions (adverse drug reactions, autoimmune diseases, graft versus host disease, and cancer). Though its causes are unknown, an immune-related pathogenesis is the most likely one. Vanishing bile duct syndrome can evolve to hepatic failure and, eventually, to death. The treatment is uncertain, but it needs the resolution of the underlying pathologic condition. CASE PRESENTATION We describe the association of Hodgkin's lymphoma with a syndrome characterized by cholestasis, aminotransferase elevation and an histological picture of bile duct loss. All other causes of hepatic function impairment were excluded (in particular, drugs, viral and autoimmune related diseases) eventually leading to the diagnosis of vanishing bile duct syndrome. Despite the fact that the dysfunction is not caused by hepatic Hodgkin's lymphoma involvement, liver impairment can limit the optimal therapy of Hodgkin's lymphoma. A treatment consisting of ursodeoxycholic acid, prednisone, and full dose chemotherapy restored hepatic function and achieved complete and long-lasting remission of Hodgkin's lymphoma. CONCLUSION We reviewed all case reports showing that vanishing bile duct syndrome is a dismal paraneoplastic syndrome being fatal in a high proportion of patients if not adequately treated. Indeed, this syndrome requires both an early recognition and an appropriate aggressive treatment consisting of full dose upfront chemotherapy which is the only way to achieve a resolution of the vanishing bile duct syndrome. Delayed or reduced intensity treatments unfavorably correlate with survival.
Collapse
Affiliation(s)
| | | | | | | | | | | | | | | | | | | | - Giovanni Grignani
- Division of Oncology, Candiolo Cancer Institute, FPO, IRCCS, University of Torino Medical School, Candiolo, Italy.
| |
Collapse
|
|
12
|
Goodman ZD, Terracciano LM, Wee A. Tumours and tumour-like lesions of the liver. MACSWEEN'S PATHOLOGY OF THE LIVER 2012:761-851. [DOI: 10.1016/b978-0-7020-3398-8.00014-3] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
|
|
13
|
Abstract
The investigation of biliary dilatation forms a routine part of gastroenterology practice. In developed countries, biliary dilatation is usually the result of obstruction of bile flow by either stones or mitotic lesions of the pancreas or biliary tree, and standard radiologic and endoscopic techniques are used to identify and relieve the obstruction. In the absence of an obvious cause, however, the investigation and management of biliary dilatation can prove challenging, particularly while trying to minimize invasive studies. This review examines factors thought to influence bile duct size in the absence of obvious obstructing pathology and looks at some causes of biliary dilatation that are unusual and potentially difficult to diagnose.
Collapse
Affiliation(s)
- Alan Coss
- Pacific Gastroenterology Associates, #770-1190 Hornby Street, Vancouver, BC V6Z 2K5, Canada
| | | |
Collapse
|
|
14
|
BAL A, DUTTA U, DAS A, KOCCHAR R, SINGH K. Non-metastatic cholestatic paraneoplastic syndrome associated with soft tissue sarcoma. Asia Pac J Clin Oncol 2009. [DOI: 10.1111/j.1743-7563.2009.01197.x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
|
|
15
|
Ballonoff A, Kavanagh B, Nash R, Drabkin H, Trotter J, Costa L, Rabinovitch R. Hodgkin lymphoma-related vanishing bile duct syndrome and idiopathic cholestasis: statistical analysis of all published cases and literature review. Acta Oncol 2008; 47:962-70. [PMID: 17906981 DOI: 10.1080/02841860701644078] [Citation(s) in RCA: 29] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022]
Abstract
BACKGROUND Hodgkin lymphoma (HL)-related vanishing bile duct syndrome (VBDS) and idiopathic cholestasis (IC) are rare conditions that often lead to liver failure and death. The available literature consists primarily of case reports, resulting in little clarity as to the clinical course and ideal treatment for this disease. MATERIAL AND METHODS We performed a literature search from which we identified all published cases of HL-related VBDS or IC, and created a database of detailed presentation, treatment, and outcome information for all patients. Patient and disease factors were analyzed for an association with overall survival and liver failure-free survival. A case presentation introduces this analysis. RESULTS Thirty-seven cases of HL-related VBDS/IC were identified. Median follow-up was 7 months; 1-year OS and liver failure-free survival (LFFS) are 43% and 41%, respectively. Sixty-five percent of the patients died while 30% were alive with normal or near-normal stable liver function and no evidence of recurrent HL at last evaluation. Of the 20 patients without residual HL following therapy, 12 (60%) achieved liver failure-free survival. On univariate analysis, factors significantly associated with improved liver failure-free survival were stage I/II HL (p=0.02), a complete response of HL (p=0.0002), and delivery of radiotherapy (pB0.0001). Two patients received chemotherapy without radiation and survived with recovery of liver function. DISCUSSION HL-related VBDS/IC is potentially reversible and not uniformly fatal, with 30% of presenting patients demonstrating good lymphoma and liver outcomes after definitive therapy for HL. As a complete response of HL provides the only possibility of recovering liver function, patients with this disease should proceed to definitive treatment of HL as soon as feasible.
Collapse
Affiliation(s)
- Ari Ballonoff
- Department of Radiation Oncology, University of Colorado, Comprehensive Cancer Center, Aurora, CO 80045-0510, USA.
| | | | | | | | | | | | | |
Collapse
|
|
16
|
Barta SK, Yahalom J, Shia J, Hamlin PA. Idiopathic cholestasis as a paraneoplastic phenomenon in Hodgkin's lymphoma. ACTA ACUST UNITED AC 2007; 7:77-82. [PMID: 16879775 DOI: 10.3816/clm.2006.n.044] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
Paraneoplastic cholestasis is an uncommon presenting symptom in Hodgkin's lymphoma (HL). Two distinct entities causing this clinical picture have been described: idiopathic cholestasis and vanishing bile duct syndrome (VBDS). We report a patient with idiopathic cholestasis and early-stage HL with favorable risk factors whose liver biopsy results were consistent with intrahepatic cholestasis and no ductopenia. Other causes for cholestatic jaundice were ruled out. He was treated with subtotal lymphoid irradiation and subsequently experienced a steady improvement in his liver function tests to near normal over 21 months. This case illustrates a rare paraneoplastic phenomenon, and a review of the available literature is included. We also discuss the differences between HL-related idiopathic cholestasis and VBDS. The distinction between these 2 diseases has prognostic implications: idiopathic cholestasis is usually reversible in early-stage HL after treatment, whereas patients who develop VBDS commonly die from their disease. There is no established approach to the management of paraneoplastic cholestasis. We postulate that single-modality radiation therapy alone should be considered a valid treatment alternative in early-stage HL with idiopathic cholestasis.
Collapse
Affiliation(s)
- Stefan K Barta
- Department of Medicine, St. Luke's Roosevelt Hospital Center, New York, NY, USA
| | | | | | | |
Collapse
|
|
17
|
Giannakos G, Papanicolaou X, Trafalis D, Michaelidis I, Margaritis G, Christofilakis C. Stauffer's syndrome variant associated with renal cell carcinoma. Int J Urol 2005; 12:757-9. [PMID: 16174051 DOI: 10.1111/j.1442-2042.2005.01149.x] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/30/2023]
Abstract
Cholestasis, as a paraneoplastic syndrome, has been well described in patients with malignant lymphohyperplastic diseases and renal cell cancer. Non-metastatic nephrogenic hepatic dysfunction syndrome without jaundice has often been described in patients with Stauffer's syndrome. Paraneoplastic cholestatic jaundice is extremely uncommon. We report, a patient who presented with pruritus and cholestatic jaundice and was diagnosed with renal cell carcinoma (RCC) in the right kidney. Liver malfunction and cholestatic icterus was attributed to RCC. Jaundice and liver dysfunction gradually restored to normal after surgical resection of the tumor. Malignancies may cause cholestatic jaundice through well-recognized mechanisms. Paraneoplastic syndromes associated with malignancy, can induce a reversible form of cholestasis through an unclear pathogenetic mechanism.
Collapse
Affiliation(s)
- George Giannakos
- 1st Department of Internal Medicine, 401 General Army Hospital of Athens, Athens, Greece
| | | | | | | | | | | |
Collapse
|
|
18
|
Chung CH, Wang CH, Tzen CY, Liu CP. Intrahepatic cholestasis as a paraneoplastic syndrome associated with pheochromocytoma. J Endocrinol Invest 2005; 28:175-9. [PMID: 15887866 DOI: 10.1007/bf03345363] [Citation(s) in RCA: 16] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/23/2023]
Abstract
Pheochromocytoma is a rare tumor of chromaffin cells that secrete catecholamines and several cytokines. The clinical manifestations are protean and may include hypertension, weight loss, sweating, palpitation, headache, anxiety, tremor, nausea, vomiting, and hypercalcemia. The tumor can mimic many unrelated diseases, leading to significant delay and difficulty in diagnosis. We report a case of a 37-yr-old male admitted with jaundice, dark urine, fever, and signs of a systemic inflammatory response. Abdominal computed tomography revealed a heterogeneously enhancing tumor between the pancreatic tail and left kidney. There was no evidence of obstruction to bile flow, neoplastic involvement of the liver or bile ducts, or infectious etiology. The tumor was removed and found to be a pheochromocytoma. Immunohistochemical analysis revealed the presence of interleukin-1beta in the tumor cells. After surgery, the jaundice resolved without further treatment, leading us to the conclusion that it was a paraneoplastic phenomenon possibly related to interleukin-1beta production. We suggest that occult pheochromocytoma should be added to the differential diagnosis of unexplained intrahepatic cholestasis.
Collapse
Affiliation(s)
- C H Chung
- Division of Endocrinology and Metabolism, Department of Medicine, Mackay Memorial Hospital, Mackay Medicine, Nursing and Management College, Taipei, Taiwan
| | | | | | | |
Collapse
|
|
19
|
Di Sena V, Thuler FPBM, Macedo EP, Paulo GAD, Della Libera E, Ferrari AP. Obstructive jaundice secondary to bile duct involvement with Hodgkin's disease: a case report. SAO PAULO MED J 2005; 123:30-2. [PMID: 15821813 PMCID: PMC11052447 DOI: 10.1590/s1516-31802005000100007] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/21/2022] Open
Abstract
CONTEXT Obstructive jaundice due to lymphoma is very rare. It may be difficult to distinguish between this condition and a large number of causes of extrahepatic bile duct obstruction, even by endoscopic retrograde cholangiography. Its prognosis is poor. Combined chemotherapy and/or radiotherapy with bile duct drainage is a therapeutic option. CASE REPORT We describe a case of obstructive jaundice as the initial presentation of Hodgkin's disease. After chemotherapy and endoscopic bile duct stenting, it was noted that the enlarged lymph nodes, jaundice and bile duct dilation disappeared.
Collapse
Affiliation(s)
- Veruska Di Sena
- Universidade Federal de São Paulo, Escola Paulista de Medicina, São Paulo, Brazil
| | | | | | | | | | | |
Collapse
|
|
20
|
Abstract
Paraneoplastic syndromes are manifestations of malignancies that have produced effects that are distant from the primary tumor or metastases. Paraneoplastic syndromes are not caused by local effects of compression or infiltration into tissues, but are generally due to ectopic hormone production, autoimmune phenomena, or overproduction of cytokines. Paraneoplasia may be the presenting symptom of underlying malignancy and can affect almost any organ system, such as the neurologic syndromes associated with small-cell lung cancer or hypercalcemia associated with squamous cell carcinomas. Lymphoproliferative disorders are also associated with many paraneoplastic disorders; however, to date, most published information has been in the form of case reports and series of small numbers of patients. In this review, the most common paraneoplastic syndromes associated with non-Hodgkin's lymphoma and Hodgkin's disease will be discussed.
Collapse
Affiliation(s)
- Karl T Hagler
- Division of Hematology/Oncology, Department of Medicine, UF/Shands Cancer Center, University of Florida College of Medicine, Gainesville, 32610, USA
| | | |
Collapse
|
|
21
|
Karakolios A, Kasapis C, Kallinikidis T, Kalpidis P, Grigoriadis N. Cholestatic jaundice as a paraneoplastic manifestation of prostate adenocarcinoma. Clin Gastroenterol Hepatol 2003; 1:480-3. [PMID: 15017648 DOI: 10.1016/s1542-3565(03)00227-1] [Citation(s) in RCA: 39] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Malignancies may cause cholestatic jaundice through well-recognized mechanisms (e.g., bile duct obstruction or widespread hepatic infiltration). Paraneoplastic syndromes associated with malignancy, particularly with renal cell carcinoma (Stauffer's syndrome) and malignant lymphoproliferative diseases, can induce a reversible form of cholestasis through an unclear pathogenetic mechanism. Prostate cancer presenting initially with cholestatic jaundice without any obvious cause (i.e., obstruction or infiltration) has been reported in 2 cases in the medical literature. We report a patient who presented with pruritus and cholestatic jaundice. During the diagnostic work-up, prostate cancer was diagnosed. Conjugated bilirubin and alkaline phosphatase levels were increased markedly with modest increases of gamma-glutamyltranspeptidase and transaminase levels. The results of appropriate investigations performed during the patient's hospitalizations indicated no evidence of hepatic metastases or extrahepatic biliary obstruction. After treatment with flutamide and leuprolide, the patient's symptoms and the laboratory abnormalities reversed rapidly. We regard the cholestatic jaundice of this patient as part of a paraneoplastic syndrome; the cause of cholestasis remains an enigma. Patients with unexplained cholestasis should be investigated for malignancies, including prostate cancer.
Collapse
|
|
22
|
Opeskin K, Burke M, McCaffrey G, Nandurkar H. Hodgkin's disease associated cholestasis in a man with thymoma. Pathology 2003; 35:349-51. [PMID: 12959773 DOI: 10.1080/0031302031000152883] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
|
|
23
|
Liangpunsakul S, Kwo P, Koukoulis GK. Hodgkin's disease presenting as cholestatic hepatitis with prominent ductal injury. Eur J Gastroenterol Hepatol 2002; 14:323-7. [PMID: 11953700 DOI: 10.1097/00042737-200203000-00019] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/30/2022]
Abstract
In advanced stages of Hodgkin's disease, liver involvement is common. However, Hodgkin's disease mimicking cholestatic hepatitis at presentation is rare. We describe a patient with Hodgkin's disease who was initially considered to have acute cholestatic hepatitis. Liver biopsy demonstrated prominent bile duct injury associated with a florid inflammatory reaction. These changes may represent an early stage of ductal injury, subsequently leading to vanishing bile duct syndrome, a recently documented mechanism of cholestasis in Hodgkin's disease.
Collapse
Affiliation(s)
- Suthat Liangpunsakul
- Department of Medicine, Division of Gastroenterology and Hepatology, Indiana School of Medicine, Indianapolis, Indiana, USA.
| | | | | |
Collapse
|
|
24
|
Kömürcü S, Ozet A, Altundag MK, Arpaci F, Oztürk B, Celasun B, Tezcan Y. Vanishing bile duct syndrome occurring after high-dose chemotherapy and autologous peripheral stem cell transplantation in a patient with Hodgkin's disease. Ann Hematol 2002; 81:57-8. [PMID: 11807639 DOI: 10.1007/s00277-001-0406-7] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/14/2001] [Accepted: 10/14/2001] [Indexed: 12/30/2022]
|