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Current Treatment Options in Oncology Jan 2018Retroperitoneal sarcomas are rare tumors of which liposarcoma is the most common histology. Surgical resection remains the mainstay of therapy, particularly for the... (Review)
Review
Retroperitoneal sarcomas are rare tumors of which liposarcoma is the most common histology. Surgical resection remains the mainstay of therapy, particularly for the well-differentiated subtype. They can grow to massive size before causing symptoms or detection. Well-differentiated liposarcoma, while having a negligible metastatic rate, is fraught with a high local recurrence rate, despite a complete surgical resection. Reasons for this are not completely known but may be related to a field defect of the retroperitoneal fat creating a niche for recurrence. These tumors are classically chemo- and radio-resistant. Surgical therapy of recurrences can be challenging, but remains the treatment of choice for well-differentiated liposarcoma. In an attempt to improve on survival and recurrence rates for retroperitoneal liposarcoma, an extended resection approach has been promoted by a few groups. This involves the en bloc resection of contiguous organs that are not macroscopically involved. While this has improved local recurrence rates, benefit for overall survival has not been demonstrated. Interestingly, the improvement in local recurrence rate appeared to be driven by histology and was most improved in the well-differentiated subtype compared to historical data. However, for well-differentiated liposarcomas that are multifocal, this approach may be less useful. The application of this approach still requires further study in terms of balancing increased morbidity of extended resection against the potential for multiple surgeries for recurrence.
Topics: Digestive System Surgical Procedures; Disease Progression; Humans; Liposarcoma; Neoplasm Recurrence, Local; Randomized Controlled Trials as Topic; Retroperitoneal Neoplasms; Tomography, X-Ray Computed
PubMed: 29335908
DOI: 10.1007/s11864-018-0520-6 -
Journal of Surgical Oncology Apr 2015Management of retroperitoneal sarcomas presents technical and oncological challenges. Imaging is crucial for diagnosis and to define local tumor extent. Complete gross... (Review)
Review
Management of retroperitoneal sarcomas presents technical and oncological challenges. Imaging is crucial for diagnosis and to define local tumor extent. Complete gross resection at initial presentation is the best chance for cure, but there is controversy as to how this can be best achieved. There is a long-term risk of local recurrence, which is best treated with repeat resection if feasible. The roles of radiation and chemotherapy remain undefined.
Topics: Humans; Neoplasm Staging; Pelvic Neoplasms; Retroperitoneal Neoplasms; Sarcoma
PubMed: 25482329
DOI: 10.1002/jso.23840 -
Surgical Oncology Aug 2022Retroperitoneal and pelvis sarcomas are uncommon tumors for which complete surgical resection is the mainstay of treatment. However, achieving complete gross resection... (Review)
Review
Retroperitoneal and pelvis sarcomas are uncommon tumors for which complete surgical resection is the mainstay of treatment. However, achieving complete gross resection with microscopically negative margins is challenging, and local recurrence rates can be high. Patients often succumb to uncontrolled local disease. Radiation therapy offers a potential means for sterilizing microscopic residual disease, although its use continues to be controversial. Chemotherapy alone or in combination with radiation continues to be investigated as an adjunct to surgery, along with immunotherapy and targeted therapies. In this review, we discuss the current management of retroperitoneal and pelvis sarcomas, focusing on studies of surgery and radiation therapy to maximize local control.
Topics: Humans; Margins of Excision; Neoplasm Recurrence, Local; Pelvic Neoplasms; Pelvis; Radiotherapy, Adjuvant; Retroperitoneal Neoplasms; Retrospective Studies; Sarcoma
PubMed: 35834940
DOI: 10.1016/j.suronc.2022.101814 -
The Journal of Surgical Research Dec 2019Sarcomas are malignant tumors of connective tissue that can vary widely in etiology. Parameters such as grade, extent of resection, and tumor integrity have been shown...
BACKGROUND
Sarcomas are malignant tumors of connective tissue that can vary widely in etiology. Parameters such as grade, extent of resection, and tumor integrity have been shown to affect prognosis. Our principal aim was to examine associations between the laterality of retroperitoneal sarcomas and tumor characteristics, treatment, and patient outcomes.
MATERIALS AND METHODS
We performed a retrospective study of patients treated at our tertiary referral center with a diagnosis of primary retroperitoneal sarcoma who underwent tumor resection. Categorical variables were compared using the chi-square test, whereas continuous variables were compared using one-way analysis of variance. Cox regression was used to estimate the risk of death.
RESULTS
Data from 106 patients were analyzed. A greater proportion of bilateral or midline tumors were leiomyosarcomas (P = 0.02), whereas right-sided tumors were more likely to be liposarcoma (P = 0.02). There was no significant relationship between laterality and tumor grade or stage. Two-thirds of patients had at least one contiguous organ resected (n = 68, 65.4%). Patients with nephrectomy during sarcoma resection were more likely to have right-sided disease (P = 0.02). Splenectomy and pancreatectomy were associated with left-sided disease (P < 0.01; P < 0.01), and pancreaticoduodenectomies with bilateral or midline disease (P < 0.001). Adjusting for age, sex, race, grade, stage, histology, and treatment, there was no increased risk of death or recurrence based on laterality.
CONCLUSIONS
Although laterality did not seem to have a measurable relationship with patient outcomes or survival, there was a significant association between laterality, tumor histology, and resection of contiguous organs. These preliminary findings warrant further investigation.
Topics: Aged; Female; Humans; Leiomyosarcoma; Liposarcoma; Male; Middle Aged; Neoplasm Grading; Neoplasm Recurrence, Local; Nephrectomy; Pancreatectomy; Pancreaticoduodenectomy; Prognosis; Retroperitoneal Neoplasms; Retroperitoneal Space; Retrospective Studies; Splenectomy; Treatment Outcome
PubMed: 31279261
DOI: 10.1016/j.jss.2019.05.016 -
Expert Review of Anticancer Therapy May 2015Retroperitoneal sarcomas are a group of diseases that behave differently from one another. Well-differentiated liposarcomas have an indolent biology but show a tendency... (Review)
Review
Retroperitoneal sarcomas are a group of diseases that behave differently from one another. Well-differentiated liposarcomas have an indolent biology but show a tendency to recur locally even years after primary resection. Dedifferentiated liposarcomas are characterized by a very high local recurrence risk, while the metastatic risk mainly depends on the histological characteristics of the dedifferentiated component. In leiomyosarcomas, hematogenous spread informs prognosis while local recurrences are far less common. Surgery is the cornerstone of treatment of all retroperitoneal sarcoma subtypes and its quality is the only treatment-related factor able to improve the oncological outcome. A frontline extended surgical approach is all the more critical in subtypes in which local control directly impacts prognosis.
Topics: Animals; Humans; Leiomyosarcoma; Liposarcoma; Neoplasm Metastasis; Neoplasm Recurrence, Local; Prognosis; Retroperitoneal Neoplasms; Risk; Sarcoma
PubMed: 25797538
DOI: 10.1586/14737140.2015.1028375 -
RoFo : Fortschritte Auf Dem Gebiete Der... Nov 2020
Topics: Abdominal Neoplasms; Contrast Media; Diagnosis, Differential; Humans; Liposarcoma; Liposarcoma, Myxoid; Magnetic Resonance Imaging; Muscle Neoplasms; Neoplasm Grading; Pelvic Neoplasms; Retroperitoneal Neoplasms; Soft Tissue Neoplasms; Tomography, X-Ray Computed
PubMed: 32106323
DOI: 10.1055/a-1108-1961 -
Current Oncology (Toronto, Ont.) Jan 2023Locoregional recurrence (LRR) is the predominant pattern of relapse and often the cause of death in patients with retroperitoneal sarcomas (RPS). As a result, reducing... (Review)
Review
Locoregional recurrence (LRR) is the predominant pattern of relapse and often the cause of death in patients with retroperitoneal sarcomas (RPS). As a result, reducing LRR is a critical objective for RPS patients. However, unlike soft tissue sarcomas (STS) of the superficial trunk and extremity where the benefits of radiation therapy (RT) are well-established, the role of RT in the retroperitoneum remains controversial. Historically, preoperative or postoperative RT, either alone or in combination with intraoperative radiation (IORT), was commonly justified for RPS based on extrapolation from the superficial trunk and extremity STS literature. However, long-awaited results were recently published from the European Organization for Research and Treatment of Cancer (EORTC) STRASS study of preoperative radiotherapy plus surgery versus surgery alone for patients with RPS; there was no statistical difference in the primary endpoint of abdominal recurrence-free survival. However, several subset analyses and study limitations complicate the interpretation of the results. This review explores and contextualizes the body of evidence regarding RT's role in managing RPS.
Topics: Humans; Radiotherapy, Adjuvant; Neoplasm Recurrence, Local; Sarcoma; Soft Tissue Neoplasms; Retroperitoneal Neoplasms
PubMed: 36661696
DOI: 10.3390/curroncol30010047 -
Asian Journal of Surgery Apr 2016Lipomatous ganglioneuroma (LG) is a rare variant of ganglioneuroma that is histologically characterized by a mature adipocytic component admixed with a conventional... (Review)
Review
Lipomatous ganglioneuroma (LG) is a rare variant of ganglioneuroma that is histologically characterized by a mature adipocytic component admixed with a conventional ganglioneuroma. We report the clinicopathological and immunohistochemical features of an LG in a 44-year-old Chinese male; additionally, we review the literature regarding this type of tumor. Magnetic resonance imaging revealed a left paravertebral soft-tissue mass at the T11-L3 levels. Grossly, the encapsulated neoplasm had a white to yellowish cut surface and rubbery consistency. Microscopic evaluation revealed an encapsulated lesion that consisted of areas of ganglioneuroma admixed with areas of mature fat. By immunohistochemistry, the ganglion cells were positive for chromogranin and synaptophysin, whereas the Schwann cells were positive for vimentin, S-100 protein, and glial fibrillary acidic protein (GFAP). This is the second known report of a retroperitoneal LG. The patient was well and without evidence of disease at 2 years' follow-up.
Topics: Adipocytes; Adult; Ganglioneuroma; Humans; Magnetic Resonance Imaging; Male; Retroperitoneal Neoplasms
PubMed: 23978430
DOI: 10.1016/j.asjsur.2013.07.011 -
Annals of Surgical Oncology May 2015
Topics: Humans; Retroperitoneal Neoplasms; Sarcoma
PubMed: 25316490
DOI: 10.1245/s10434-014-4135-2 -
Annals of the Royal College of Surgeons... Feb 2022Retroperitoneal liposarcomas are very infrequent retroperitoneal malignant tumours. Most patients complain of palpable abdominal mass and only half present with...
Retroperitoneal liposarcomas are very infrequent retroperitoneal malignant tumours. Most patients complain of palpable abdominal mass and only half present with abdominal pain. With haematogenous spread they can reach, mainly, lungs and liver, but only in 10% of cases. Here we report the presentation and surgical treatment of a giant massive retroperitoneal liposarcoma in a 53-year-old man.
Topics: Abdominal Pain; Humans; Liposarcoma; Male; Middle Aged; Retroperitoneal Neoplasms
PubMed: 34807770
DOI: 10.1308/rcsann.2021.0101