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Santangelo A, Fernicola A, Santangelo D, Peluso G, Calogero A, Crocetto F, Jamshidi A, Pelosio L, Scotti A, Tammaro V, Tranquillo V, Tammaro D, De Cocinis C, Della Gaggia F, Capezio E, Carlomagno N, Santangelo M. Dark Topics on Giant Retroperitoneal Liposarcoma: A Systematic Review of 157 Cases. Cancers (Basel) 2025; 17:740. [PMID: 40075588 PMCID: PMC11899555 DOI: 10.3390/cancers17050740] [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: 01/23/2025] [Revised: 02/17/2025] [Accepted: 02/19/2025] [Indexed: 03/14/2025] Open
Abstract
BACKGROUND/OBJECTIVES Giant Retroperitoneal Liposarcomas (giant RPLs) represent a rare malignant disease of adulthood that does not yet have a univocal definition in the scientific literature. The symptoms may be late, depending on the position and the size reached. The weight may exceed 20 kg, and the diameter 25 cm. The main treatment is the surgical approach. This systematic review aims to collect data from the present literature and to answer some questions on the nature of this pathology. METHODS We performed a search on the PubMed, Cochrane, and Scopus databases using specific search strings. Non-English written articles and abstracts were excluded. RESULTS Dimensional, histological, and pathological data of giant RPLs were extracted and recorded in an electronic database, and charts were used to synthesize the results. We selected 126 manuscripts, all case reports and case series, and obtained data for 157 giant RPLs. The major axis varied from 15 to 80 cm, and the weight ranged between 2.5 and 98 kg. Sex distribution was homogenous. Age was reported 146 times, and almost 1/3 of the study population was under 50 years old. The most frequent hystotype reported was well-differentiated liposarcomas, while the rarest was pleomorphic liposarcomas. In 139 cases, the symptoms were reported and generally included a mass effect on surrounding organs. The exclusive surgical operation was the most frequent treatment option, and it included both the resection of the tumor and other organs involved. Chemo- and radiotherapy were also performed, in a few cases. In 36 reports, distant metastasis was suspected, but only 6 were effectively positive. CONCLUSIONS Giant RPLs are a clinical entity that shares some common features with normal-size liposarcomas but are different in dimensions, age distribution, histologic prevalence, rate of incidental diagnosis, organ sparing, and R0 resection. More studies are needed to completely characterize these tumors.
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Affiliation(s)
- Alfonso Santangelo
- Unit of Urology, Division of Oncology, Gianfranco Soldera Prostate Cancer Lab, IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, 20132 Milan, Italy;
| | - Agostino Fernicola
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
| | - Domenico Santangelo
- Department of Radiology, Vita-Salute San Raffaele University, IRCCS San Raffaele, 20132 Milan, Italy;
- Surgical and Biomorphological Sciences PhD, Federico II University, 80138 Naples, Italy
| | - Gaia Peluso
- Department of General Surgery, Fatebenefratelli Hospital of Naples, 80123 Naples, Italy;
| | - Armando Calogero
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
| | - Felice Crocetto
- Department of Neurosciences, Sciences of Reproduction and Odontostomatology, University of Naples Federico II, 80131 Naples, Italy;
| | - Akbar Jamshidi
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
| | - Luigi Pelosio
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
| | - Alessandro Scotti
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
| | - Vincenzo Tammaro
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
| | - Valentina Tranquillo
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
| | - Dario Tammaro
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
| | - Carmen De Cocinis
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
| | - Francesca Della Gaggia
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
| | - Emanuela Capezio
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
| | - Nicola Carlomagno
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
| | - Michele Santangelo
- Department of Advanced Biomedical Sciences, Unit of Emergency Surgery, Federico II University, 80131 Naples, Italy; (A.C.); (A.J.); (L.P.); (A.S.); (V.T.); (N.C.); (M.S.)
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Sun JN, Yang R, Jiang XL, Zhang F, Zhao HW. Giant retroperitoneal liposarcoma with multiple organ involvement: a case report and literature review. BMC Nephrol 2024; 25:281. [PMID: 39210338 PMCID: PMC11363381 DOI: 10.1186/s12882-024-03701-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/12/2023] [Accepted: 08/07/2024] [Indexed: 09/04/2024] Open
Abstract
BACKGROUND Retroperitoneal liposarcoma (RPLPS) is a relatively rare disease. Liposarcomas vary in size, but sizeable RPLPS larger than 30 cm in diameter are very rare, and their diagnosis and treatment present significant challenges. CASE PRESENTATION We report a 58-year-old male patient who was admitted to the hospital with an increased abdominal circumference and was later diagnosed with a giant RPLPS. The liposarcoma was found to adhere to the right kidney and the entire ureter, invading the ascending colon. The patient underwent complete combined surgical resection. The tumor was removed intact, measured 55.0 cm × 30.0 cm × 18.0 cm, and weighed 19.8 kg. Histopathologic analysis revealed well-differentiated liposarcoma (WDLPS). The patient was successfully discharged from the hospital and followed up for 6 months with no signs of recurrence. CONCLUSION RPLPS is a rare tumor with atypical clinical presentation. Surgery remains the most effective method of treatment for retroperitoneal sarcomas, with complete removal if there is local invasion. Preoperative examination, including three-dimensional (3D) reconstruction, is essential for surgical success. The role of adjuvant radiotherapy or chemotherapy remains controversial. However, clinicians should not rule them out as viable options.
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Affiliation(s)
- Jia-Ning Sun
- School of Clinical Medicine, Shandong Second Medical University, Weifang, China
| | - Rui Yang
- School of Clinical Medicine, Shandong Second Medical University, Weifang, China
| | - Xiao-Lu Jiang
- Affiliated Yantai Yuhuangding Hospital of Qingdao University, No. 20 East Yuhuangding Road, Yantai, 264000, Shandong, China
| | - Feng Zhang
- Second Clinical Medical College, Binzhou Medical University, Yantai, China
| | - Hong-Wei Zhao
- Affiliated Yantai Yuhuangding Hospital of Qingdao University, No. 20 East Yuhuangding Road, Yantai, 264000, Shandong, China.
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Vlăsceanu VI, Soroceanu RP, Timofte DV, Iordache AG, Strobescu CC, Timofeiov S. Retroperitoneal liposarcoma: unveiling diagnostic delays and multimodal treatment dilemmas. Arch Clin Cases 2023; 10:187-190. [PMID: 38098695 PMCID: PMC10719986 DOI: 10.22551/2023.41.1004.10272] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2023] Open
Abstract
Liposarcomas are a prevalent subtype of soft tissue sarcomas, constituting less than 1% of all malignancies. Originating in the adipose tissue, they can manifest in various locations and are categorized by the World Health Organization into several subtypes: well-differentiated liposarcomas, dedifferentiated liposarcomas, myxoid liposarcomas, pleomorphic liposarcomas and mixed-type liposarcomas. These tumors typically affect middle-aged and older individuals, and their incidence has been progressively increasing over the years. As liposarcomas advance they tend to encase blood vessels and major organs, particularly in the retroperitoneal area. Often asymptomatic initially, symptoms arise as the tumor reaches a considerable size, exerting pressure on adjacent tissues and organs. This report features a 54-year-old patient incidentally diagnosed with a substantial retroperitoneal tumor extending to the antero-lateral abdominal wall and inner thigh via the right inguinal ligament. The patient, with a previous three-year history of a right inguinal mass, sought acute care for a perianal abscess The histological examination revealed morphological aspects consistent with a low-grade myxoid liposarcoma. While surgery remains the primary treatment for retroperitoneal liposarcomas, controversies exist regarding the role of radiotherapy and chemotherapy in improving survival rates. This case highlights the challenges in managing retroperitoneal tumors and underscores the importance of a personalized, multidisciplinary approach to optimize patient outcomes.
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Affiliation(s)
- Vlad Ionuţ Vlăsceanu
- 3 Surgical Unit, “Sf. Spiridon” County Clinical Emergency Hospital, 700111 Iaşi, Romania
| | - Radu Petru Soroceanu
- 3 Surgical Unit, “Sf. Spiridon” County Clinical Emergency Hospital, 700111 Iaşi, Romania
- Department of Surgery, “Grigore T. Popa” University of Medicine and Pharmacy, 700115 Iaşi, Romania
| | - Daniel Vasile Timofte
- 3 Surgical Unit, “Sf. Spiridon” County Clinical Emergency Hospital, 700111 Iaşi, Romania
- Department of Surgery, “Grigore T. Popa” University of Medicine and Pharmacy, 700115 Iaşi, Romania
| | - Andi Gabriel Iordache
- 3 Surgical Unit, “Sf. Spiridon” County Clinical Emergency Hospital, 700111 Iaşi, Romania
| | - Cristina Ciobanu Strobescu
- 3 Surgical Unit, “Sf. Spiridon” County Clinical Emergency Hospital, 700111 Iaşi, Romania
- Vascular Surgical Unit, “Sf. Spiridon” County Clinical Emergency Hospital, 700111 Iaşi, Romania
| | - Sergiu Timofeiov
- 3 Surgical Unit, “Sf. Spiridon” County Clinical Emergency Hospital, 700111 Iaşi, Romania
- Department of Surgery, “Grigore T. Popa” University of Medicine and Pharmacy, 700115 Iaşi, Romania
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Kassi ABF, Yenon KS, Kassi FMH, Adjeme AJ, Diarra KM, Bombet-Kouame C, Kouassi M. Giant dedifferentiated liposarcoma of the gastrocolic ligament: A case report. World J Gastrointest Surg 2023; 15:2376-2381. [PMID: 37969706 PMCID: PMC10642459 DOI: 10.4240/wjgs.v15.i10.2376] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/01/2023] [Revised: 08/17/2023] [Accepted: 08/23/2023] [Indexed: 10/27/2023] Open
Abstract
BACKGROUND Dedifferentiated liposarcoma (DDLS) has a worse prognosis and occurs most commonly in the retroperitoneal region and rarely in the intraperitoneal region. Histological diagnosis was revolutionized by the combined contributions of histo-immuno-chemistry and molecular biology. Aside from surgery, there is no consensus on the optimal treatment for this chemoresistant cancer. CASE SUMMARY A thirty-year-old black female presented with a large painful abdominal mass occupying nearly the entire abdomen and progressive weight loss was admitted for surgery. Abdominal computed tomography showed a large heterogeneous mass of the mesentery that was sized 18 cm × 16 cm in size and had heterogeneous contrast enhancement. During laparotomy, en bloc excision of the large and multilobulated gastrocolic ligament mass was performed. The initial postoperative histopathological diagnosis was undifferentiated sarcoma. Finally, the results of immunohistochemistry and molecular biology allowed us to confirm the diagnosis of DDLS. The tumour followed an aggressive evolution with diffuse metastasis, causing the death of the patient less than 5 mo after the operation. CONCLUSION Dedifferentiated liposarcomas are rare tumours that typically originate in the retroperitoneum but may arise in unexpected locations.
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Affiliation(s)
- Assamoi Brou Fulgence Kassi
- Surgery and Surgical Specialities, Medical Sciences Training and Research Unit, Abidjan Felix Houphouet-Boigny University, Abidjan 01 BP V 34, Cote d'Ivoire
- Surgery and Surgical Specialities, Digestive Surgery Unit, University Hospital of Cocody, Abidjan 01 BP V13, Cote d'Ivoire
| | - Kacou Sebastien Yenon
- Surgery and Surgical Specialities, Medical Sciences Training and Research Unit, Abidjan Felix Houphouet-Boigny University, Abidjan 01 BP V 34, Cote d'Ivoire
- Surgery and Surgical Specialities, Digestive Surgery Unit, University Hospital of Cocody, Abidjan 01 BP V13, Cote d'Ivoire
| | - Fian Marc Herve Kassi
- Surgery and Surgical Specialities, Digestive Surgery Unit, University Hospital of Cocody, Abidjan 01 BP V13, Cote d'Ivoire
| | - Adja Jacob Adjeme
- Surgery and Surgical Specialities, Digestive Surgery Unit, University Hospital of Cocody, Abidjan 01 BP V13, Cote d'Ivoire
| | - Khader Morel Diarra
- Surgery and Surgical Specialities, Digestive Surgery Unit, University Hospital of Cocody, Abidjan 01 BP V13, Cote d'Ivoire
| | - Cynthia Bombet-Kouame
- Surgery and Surgical Specialities, Digestive Surgery Unit, University Hospital of Cocody, Abidjan 01 BP V13, Cote d'Ivoire
| | - Marcellin Kouassi
- Surgery and Surgical Specialities, Digestive Surgery Unit, University Hospital of Cocody, Abidjan 01 BP V13, Cote d'Ivoire
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Qiu M, Lin DR, Xiong ZK. Survival of a patient with five recurrences of retroperitoneal liposarcoma over a period of 13 years: A case report and review. Oncol Lett 2023; 26:367. [PMID: 37559572 PMCID: PMC10407717 DOI: 10.3892/ol.2023.13953] [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: 03/08/2023] [Accepted: 06/28/2023] [Indexed: 08/11/2023] Open
Abstract
Retroperitoneal liposarcoma (RPLS) is a rare tumor that occurs in the retroperitoneal region and accounts for ~0.2% of all malignant tumor incidence. The present study describes the treatment of a surviving patient with RPLS that recurred five times in 13 years. This case has a long survival time and numerous relapses, which is a rare occurrence. The 40-year-old female patient was admitted to Shaoxing Second Hospital (Shaoxing, China) with an abdominal mass. Abdominal computed tomography (CT) scan revealed multiple liposarcoma recurrences in the abdominal, pelvic and retroperitoneal areas. As the diagnosis of RPLS was the fifth recurrence, cytoreductive surgery was performed and post-operative pathology suggested mixed liposarcoma. The patient underwent six cycles of doxorubicin and ifosfamide regimen chemotherapy and targeted therapy with anlotinib following surgery; no evidence of disease progression was found on abdominal CT scan during follow-up. The present study also reviewed the literature in terms of the clinical diagnosis, treatment, pathological characteristics and similar cases of liposarcoma with the aim of improving diagnosis and treatment.
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Affiliation(s)
- Miao Qiu
- Department of Tumor Radiotherapy, Shaoxing Second Hospital, Shaoxing, Zhejiang 312000, P.R. China
| | - De-Rong Lin
- Department of Tumor Radiotherapy, Shaoxing Second Hospital, Shaoxing, Zhejiang 312000, P.R. China
| | - Zhong-Kui Xiong
- Department of Tumor Radiotherapy, Shaoxing Second Hospital, Shaoxing, Zhejiang 312000, P.R. China
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Liao T, Du W, Li X, He S, Guan G, Zhu H, Wu J. Recurrent metastatic retroperitoneal dedifferentiated liposarcoma: a case report and literature review. BMC Urol 2023; 23:63. [PMID: 37095466 PMCID: PMC10123999 DOI: 10.1186/s12894-023-01252-3] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/22/2022] [Accepted: 04/13/2023] [Indexed: 04/26/2023] Open
Abstract
BACKGROUND Retroperitoneal liposarcoma (RPLPS), a rare tumor, is often treated using surgical procedures as the first choice for treatment. However, there is no consensus on the scope of surgical resection. In addition, the treatment outcomes of conventional radiotherapy and chemotherapy have not been satisfactory, particularly for specific types of LPS, such as dedifferentiated LPS. In this case study, we present a brief review of other cases of RPLPS, highlighting the selection of surgical scope for RPLPS and related adjuvant treatment for advanced RPLPS. CASE PRESENTATION A case study is reported regarding an extremely rare recurrent and metastatic retroperitoneal dedifferentiated LPS. The primary RPLPS tumor, with a diameter of 20 cm and a weight of 2.5 kg, occupied the whole left abdomen and adhered to the left kidney. A surgical tumor resection combined with a left nephrectomy is performed. During the 6th -month postoperative follow-up examination, we observed the local recurrence of the tumor in the operation area, in addition to multiple metastatic tumors in both lungs. Further, the prescribed 3-month targeted treatment with anlotinib significantly reduced the size of the metastatic pulmonary tumors. However, the recurrent retroperitoneal tumors showed no significant change in size. Eventually, we observed no substantial evidence of tumor progression, with the patient's condition under control. CONCLUSION The case demonstrated that the postoperative recurrence of widespread RPLPS required R0 resection to cure the disease, considering targeted therapy for advanced RPLPS control.
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Affiliation(s)
- Tuming Liao
- Department of Urology Surgery, Guangdong Provincial Hospital of Integrated Traditional Chinese and Western Medicine (Nanhai District Hospital of Traditional Chinese Medicine of Foshan City), No. 16, Guicheng South Fifth Road, Nanhai District, Foshan, 528200, Guangdong, People's Republic of China
| | - Wei Du
- Department of Urology Surgery, Guangdong Provincial Hospital of Integrated Traditional Chinese and Western Medicine (Nanhai District Hospital of Traditional Chinese Medicine of Foshan City), No. 16, Guicheng South Fifth Road, Nanhai District, Foshan, 528200, Guangdong, People's Republic of China
| | - Xiongcai Li
- Department of Urology Surgery, Guangdong Provincial Hospital of Integrated Traditional Chinese and Western Medicine (Nanhai District Hospital of Traditional Chinese Medicine of Foshan City), No. 16, Guicheng South Fifth Road, Nanhai District, Foshan, 528200, Guangdong, People's Republic of China.
| | - Shen He
- Department of Urology Surgery, Guangdong Provincial Hospital of Integrated Traditional Chinese and Western Medicine (Nanhai District Hospital of Traditional Chinese Medicine of Foshan City), No. 16, Guicheng South Fifth Road, Nanhai District, Foshan, 528200, Guangdong, People's Republic of China
| | - Gangqiang Guan
- Department of Urology Surgery, Guangdong Provincial Hospital of Integrated Traditional Chinese and Western Medicine (Nanhai District Hospital of Traditional Chinese Medicine of Foshan City), No. 16, Guicheng South Fifth Road, Nanhai District, Foshan, 528200, Guangdong, People's Republic of China
| | - Herong Zhu
- Department of Urology Surgery, Guangdong Provincial Hospital of Integrated Traditional Chinese and Western Medicine (Nanhai District Hospital of Traditional Chinese Medicine of Foshan City), No. 16, Guicheng South Fifth Road, Nanhai District, Foshan, 528200, Guangdong, People's Republic of China
| | - Jiaqiao Wu
- Department of Urology Surgery, Guangdong Provincial Hospital of Integrated Traditional Chinese and Western Medicine (Nanhai District Hospital of Traditional Chinese Medicine of Foshan City), No. 16, Guicheng South Fifth Road, Nanhai District, Foshan, 528200, Guangdong, People's Republic of China
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Hadjimichael AC, Bekos A, Tsukamoto S, Nitta Y, Righi A, Errani C, Mavrogenis AF. Pleomorphic Liposarcoma Revisited. Orthopedics 2023; 46:e72-e80. [PMID: 35876778 DOI: 10.3928/01477447-20220719-05] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
Pleomorphic liposarcoma (PLPS) is the rarest and more aggressive subtype of liposarcomas, accounting for 10% of all liposarcomas. The diagnosis should be considered after the detection of multivacuolated pleomorphic lipoblasts in biopsy specimens. Wide-margin resection is the treatment of choice. Complementary treatment options, such as radiation therapy and chemotherapy, are debatable in terms of their contribution to curing patients with PLPS. This article reviews the clinical, histopathological, and molecular characteristics of PLPS and discusses the latest trends in the management, therapeutic strategies, and novel investigations of the subject. [Orthopedics. 2023;46(2):e72-e80.].
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Cheng SH, Huang YS, Lee HH, Yen HH, Jhong YP, Chao TY. Case report and literature review: Conversion surgery for initially unresectable huge retroperitoneal liposarcoma after preoperative radiotherapy. Front Oncol 2023; 12:1096411. [PMID: 36686723 PMCID: PMC9852908 DOI: 10.3389/fonc.2022.1096411] [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] [Received: 11/12/2022] [Accepted: 12/05/2022] [Indexed: 01/07/2023] Open
Abstract
Background Retroperitoneal liposarcoma (RPLS) is a rare malignancy that is notorious for recurrence. Surgical resection with clean margin is the current treatment of choice. However, owing to the large retroperitoneal space, RPLSs often grow to significant sizes before being diagnosed. Neoadjuvant and adjuvant therapies have potentials to improve long term treatment outcome. Case presentation A 55-year-old Han Chinese male presented to the general surgery department with a one-year history of abdominal fullness and a one-week history of palpable right inguinal mass. At first, he was diagnosed with incarcerated inguinal hernia. However, abdominal computer tomography (CT) and biopsy confirmed his final diagnosis to be retroperitoneal well-differentiated liposarcoma, cT2bN0M0, stage IIb. The tumor, which measured 44.5cm in maximum diameter, was too large for primary surgical resection. Neoadjuvant radiotherapy with 70 Gy in 35 fractions was delivered to the tumor, which shrunk the target volume from 6300 cc to 4800 cc, as observed in the middle of the radiotherapy course. The right testicular mass also received 70Gy/35Fx. Conversion surgery was performed after radiotherapy. Unfortunately, due to residual tumor, adjuvant chemotherapy consisting of AIM (ifosfamide, Mesna, and doxorubicin) and MAID (Mesna, doxorubincin, ifosfamide, and dacarbazine) regimens were administered sequentially. Afterward, debulking surgery was conducted, plus another 18 cycles of ifosfamide monotherapy when residual tumor was still seen on CT. Since the completion of ifosfamide chemotherapy, the patient has been cancer free with no evidence of tumor recurrence for more than 26 months. Conclusion Despite conflicting evidence in the literature, our case supports the use of high dose neoadjuvant radiotherapy and adjuvant chemotherapy in treating large, unresectable RPLSs. It also highlights the importance of using individualized, multidisciplinary approach in achieving cure for large, unresectable rare tumors.
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Affiliation(s)
- Sarah Hsin Cheng
- School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Yen-Shuo Huang
- Department of Pathology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Hsin-Hua Lee
- School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan,Department of Radiation Oncology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan,Ph.D. Program in Environmental and Occupational Medicine, Kaohsiung Medical University and National Health Research Institutes, Kaohsiung, Taiwan,Department of Radiation Oncology, Faculty of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan,Center for Cancer Research, Kaohsiung Medical University, Kaohsiung, Taiwan,*Correspondence: Hsin-Hua Lee,
| | - Heng-Hsuan Yen
- Department of Radiation Oncology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Ying-Pei Jhong
- Department of Radiation Oncology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
| | - Tzu-Yuan Chao
- Department of Radiation Oncology, Kaohsiung Municipal Siaogang Hospital, Kaohsiung, Taiwan
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Tani A, Tarumi Y, Kakibuchi A, Aoyama K, Kokabu T, Kataoka H, Yoriki K, Nagamine M, Mori T. Giant retroperitoneal dedifferentiated liposarcoma mimicking ovarian cancer: A case report. Gynecol Oncol Rep 2022; 44:101088. [PMID: 36299399 PMCID: PMC9589010 DOI: 10.1016/j.gore.2022.101088] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/31/2022] [Revised: 10/11/2022] [Accepted: 10/17/2022] [Indexed: 11/06/2022] Open
Abstract
Clinical features of the dedifferentiated subtype of LPS that has a diameter of >30 cm remains unknown. Surgical resection entails a high risk due to the size and anatomical location and difficulty in preoperative diagnosis. CT is the most useful imaging method for evaluating tumor location, size, origin, and relationship to adjacent organs. Retroperitoneal LPS should be considered in the case of a giant intra-abdominal tumor with a fatty component. Surgical resection is the mainstay of treatment for retroperitoneal LPS. Retroperitoneal liposarcoma is a rare tumor, and its dedifferentiated subtype and a larger diameter are associated with a poor prognosis. However, there are few reports of retroperitoneal liposarcomas, both with a dedifferentiated subtype and a diameter of >30 cm. We report a case of a giant retroperitoneal liposarcoma with a dedifferentiated subtype. A 78-year-old woman presented to our hospital with abdominal distension and loss of appetite. Computed tomography and magnetic resonance imaging findings revealed a 35-cm-diameter solid tumor in the peritoneal cavity. CA125 (64.8 U/mL) and HE4 (229.0 pmol/L) were elevated preoperatively raising suspicion for ovarian malignancy. However, intraoperative findings revealed that the tumor originated in the retroperitoneal cavity. Reductive surgery for the tumor and partial resection of the sigmoid colon and left ureter were performed, and pathological examination confirmed a retroperitoneal dedifferentiated liposarcoma. Although her symptoms improved postoperatively, she died 11 months after surgery due to disease progression.
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Affiliation(s)
- Akihiro Tani
- Department of Obstetrics and Gynecology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan
| | - Yosuke Tarumi
- Department of Obstetrics and Gynecology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan,Corresponding author.
| | - Akiyo Kakibuchi
- Department of Obstetrics and Gynecology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan
| | - Kohei Aoyama
- Department of Obstetrics and Gynecology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan
| | - Tetsuya Kokabu
- Department of Obstetrics and Gynecology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan
| | - Hisashi Kataoka
- Department of Obstetrics and Gynecology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan
| | - Kaori Yoriki
- Department of Obstetrics and Gynecology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan
| | - Michiko Nagamine
- Department of Surgical Pathology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan
| | - Taisuke Mori
- Department of Obstetrics and Gynecology, Kyoto Prefectural University of Medicine, Graduate School of Medical Science, 465 Kajii-cho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan
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Patel S, Alhatem A, Patel JS, Zhang R, Chokshi RJ. Long-Term Recurrence of Retroperitoneal Dedifferentiated Liposarcoma After a Complete Surgical Resection: A Report of a Rare Phenomenon. Cureus 2021; 13:e17003. [PMID: 34405078 PMCID: PMC8352796 DOI: 10.7759/cureus.17003] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 08/08/2021] [Indexed: 11/21/2022] Open
Abstract
The objective of this report is to present a rare case of a recurrence after 20 years of retroperitoneal dedifferentiated liposarcoma after surgical resection and to discuss the lessons learned from this rare phenomenon for patients management and understanding the behavior of these aggressive tumors. A 75-year-old woman presented with recurrent retroperitoneal dedifferentiated liposarcoma who had undergone a surgical resection 20 years earlier and had no evidence of disease on frequent follow-ups during that period. The histopathologic examination revealed different morphologic characteristics between the initial and recurrent presentations. The fluorescence in situ hybridization showed amplification of the mouse double minute 2 homolog (MDM2), a regulator of p53 gene on chromosome 12q15, and positive cyclin-dependent kinase 4 (CDK4) immunostain. Liposarcoma long-term recurrence is a challenging surgical disease to provide the best survival outcome. Incomplete resection could explain the recurrence in anatomic locations where the lesions are intermixed with the neighboring adipose tissue. However, dedifferentiated liposarcoma can rarely recur after 20 years. The molecular transformation and the survival analysis of these tumors predict certain behaviors. The refraction for radiation therapy in our case and the mixed morphology provide some insight into the biology and the clinical management for these aggressive tumors.
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Affiliation(s)
- Saarang Patel
- Division of Surgical Oncology, Rutgers New Jersey Medical School, Newark, USA
| | - Albert Alhatem
- Department of Pathology, Immunology, and Laboratory Medicine, Rutgers New Jersey Medical School, Newark, USA.,Department of Dermatology, Saint Louis University School of Medicine, St. Louis, USA
| | - Jimmy S Patel
- Division of Surgical Oncology, Rutgers New Jersey Medical School, Newark, USA.,Department of Radiation Oncology, Emory University School of Medicine, Atlanta, USA
| | - Ruonan Zhang
- Department of Pathology, Immunology, and Laboratory Medicine, Rutgers New Jersey Medical School, Newark, USA
| | - Ravi J Chokshi
- Division of Surgical Oncology, Rutgers New Jersey Medical School, Newark, USA
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