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Cancer Control : Journal of the Moffitt... Jul 2016Retroperitoneal sarcoma is rare. Using initial specimens on biopsy, a definitive diagnosis of histological subtypes is ideal but not always achievable. (Review)
Review
BACKGROUND
Retroperitoneal sarcoma is rare. Using initial specimens on biopsy, a definitive diagnosis of histological subtypes is ideal but not always achievable.
METHODS
A retrospective institutional review was performed for all cases of adult retroperitoneal sarcoma from 1996 to 2015. A review of the literature was also performed related to the distribution of retroperitoneal sarcoma subtypes. A meta-analysis was performed.
RESULTS
Liposarcoma is the most common subtype (45%), followed by leiomyosarcoma (21%), not otherwise specified (8%), and undifferentiated pleomorphic sarcoma (6%) by literature review. Data from Moffitt Cancer Center demonstrate the same general distribution for subtypes of retroperitoneal sarcoma. A pathology-based algorithm for the diagnosis of retroperitoneal sarcoma is illustrated, and common pitfalls in the pathology of retroperitoneal sarcoma are discussed.
CONCLUSIONS
An informative diagnosis of retroperitoneal sarcoma via specimens on biopsy is achievable and meaningful to guide effective therapy. A practical and multidisciplinary algorithm focused on the histopathology is helpful for the management of retroperitoneal sarcoma.
Topics: Female; Humans; Male; Retroperitoneal Neoplasms; Retrospective Studies; Sarcoma
PubMed: 27556665
DOI: 10.1177/107327481602300308 -
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 -
RoFo : Fortschritte Auf Dem Gebiete Der... May 2023Peritoneal and retroperitoneal tumors consist of a heterogenous group of benign and malignant lesions of different origin. Due to often complex multidisciplinary...
Peritoneal and retroperitoneal tumors consist of a heterogenous group of benign and malignant lesions of different origin. Due to often complex multidisciplinary treatment concepts in patients with peritoneal surface malignancies radiological imaging plays a pivotal role regarding the therapeutic options. Moreover, tumor entity, abdominal tumor distribution and common as well as rare differential diagnoses have to be taken into account. Using different radiological modalities non-invasive pretherapeutic diagnostics might be significantly improved. KEY POINTS:: · Diagnostic CT is a valuable part of the initial diagnostic approach to peritoneal surface malignancies.. · Sensitivity might be increased by the additional use of dwMRI and PET/CT considering tumor entity and individual diagnostic issues.. · The Peritoneal Cancer Index (PCI) should be determined independent of radiologic modality.. CITATION FORMAT: · Glockzin G, Helmberger T. Radiologic staging of peritoneal and retroperitoneal disease. Fortschr Röntgenstr 2023; 195: 377 - 384.
Topics: Humans; Peritoneal Neoplasms; Positron Emission Tomography Computed Tomography; Retroperitoneal Neoplasms; Radiography; Abdominal Neoplasms; Neoplasm Staging
PubMed: 36863365
DOI: 10.1055/a-1999-7057 -
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 -
Cancer Control : Journal of the Moffitt... Jul 2011Retroperitoneal sarcomas are rare neoplasms that often present with multivisceral involvement. Treatment for these tumors requires careful decision making requiring a... (Review)
Review
BACKGROUND
Retroperitoneal sarcomas are rare neoplasms that often present with multivisceral involvement. Treatment for these tumors requires careful decision making requiring a combination of surgery, chemotherapy, and radiation therapy.
METHODS
We reviewed the scientific literature pertaining to the diagnosis and management of retroperitoneal sarcomas. We also identify recent developments in treatment and discuss future trends in the care of patients with this disease.
RESULTS
Retroperitoneal tumors often present as large, locally advanced lesions. Evaluation of these tumors requires careful consideration of a multimodality approach. Retrospective data and historical prospective series have demonstrated the survival benefit of radical resection for these tumors with en bloc resection of involved structures. Compartmental resections in the retroperitoneum along with debulking of high-grade disease and regional therapy are controversial approaches with significant morbidity that can lead to long-term survival. The application of neoadjuvant and adjuvant therapies in select tumor histologies may improve local control and survival.
CONCLUSIONS
The management of retroperitoneal sarcomas requires a multidisciplinary approach and is best accomplished at high-volume centers specializing in the care of patients with these complex malignancies. Current data suggest that radical resection remains the only chance for cure and that chemotherapy and radiation therapy may confer a survival benefit.
Topics: Combined Modality Therapy; Disease-Free Survival; Humans; Neoadjuvant Therapy; Neoplasm Metastasis; Neoplasm Recurrence, Local; Patient Care; Prognosis; Retroperitoneal Neoplasms; Sarcoma
PubMed: 21666580
DOI: 10.1177/107327481101800305 -
European Journal of Vascular and... Nov 2020Radical excision of retroperitoneal or intra-abdominal soft tissue sarcomas may necessitate vessel resection and reconstruction. The aim of this study was to assess... (Review)
Review
Multidisciplinary Oncovascular Surgery is Safe and Effective in the Treatment of Intra-abdominal and Retroperitoneal Sarcomas: A Retrospective Single Centre Cohort Study and a Comprehensive Literature Review.
OBJECTIVE
Radical excision of retroperitoneal or intra-abdominal soft tissue sarcomas may necessitate vessel resection and reconstruction. The aim of this study was to assess surgical results of retroperitoneal or intra-abdominal sarcomas involving major blood vessels.
METHODS
This was a retrospective single centre cohort study and a comprehensive review of literature. Patients with retroperitoneal or intra-abdominal sarcomas treated by the oncovascular team in Helsinki University Hospital from 2010 to 2018 were reviewed for vascular and oncological outcomes. A comprehensive literature review of vascular reconstructions in patients with retroperitoneal sarcoma was performed.
RESULTS
Vascular reconstruction was performed in 17 patients, 11 of whom required arterial reconstructions. Sixteen of the operations were sarcoma resections; the post-operative diagnosis for one patient was thrombosis instead of the presumed recurrent leiomyosarcoma. Early graft thrombosis occurred in two venous and one arterial reconstruction. Late thrombosis was detected in three (18%). The median follow up was 27 (range 0-82) months. Of the patients with sarcoma resections 5 (31%) died of sarcoma and further 4 (25%) developed local recurrence or new distant metastases. The comprehensive review of literature identified 37 articles with 110 patients, 89 of whom had inferior vena cava reconstruction only. Eight arterial reconstructions were described. Late graft thrombosis occurred in 14%. The follow up was 0-181 months, during which 57% remained disease free and 7% died of sarcoma.
CONCLUSION
Vascular reconstructions enable radical resection of retroperitoneal and intra-abdominal sarcomas in patients with advanced disease. The complex operations are associated with an acceptable rate of serious peri-operative complications and symptomatic thrombosis of the repaired vessel is rare. However, further studies are needed to assess the performance of the vascular reconstructions in the long term.
Topics: Adult; Aged; Arteries; Blood Vessel Prosthesis Implantation; Female; Follow-Up Studies; Graft Occlusion, Vascular; Humans; Male; Middle Aged; Neoplasm Staging; Postoperative Complications; Retroperitoneal Neoplasms; Retroperitoneal Space; Retrospective Studies; Sarcoma; Thrombosis; Treatment Outcome; Vascular Patency; Vena Cava, Inferior
PubMed: 32741678
DOI: 10.1016/j.ejvs.2020.05.029 -
CA: a Cancer Journal For Clinicians 2004Sarcomas are a heterogeneous group of rare tumors that arise predominantly from the embryonic mesoderm. They present most commonly as an asymptomatic mass originating in... (Review)
Review
Sarcomas are a heterogeneous group of rare tumors that arise predominantly from the embryonic mesoderm. They present most commonly as an asymptomatic mass originating in an extremity but can occur anywhere in the body, particularly the trunk, retroperitoneum, or the head and neck. Pretreatment radiologic imaging is critical for defining the local extent of a tumor, staging the disease, guiding biopsies, and aiding in diagnosis. Core-needle biopsy is the preferred biopsy technique for diagnosing soft tissue sarcomas. The American Joint Committee on Cancer (AJCC) staging system for soft tissue sarcomas is based on histologic grade, the tumor size and depth, and the presence of distant or nodal metastases. Despite improvements in local control rates with wide local resections and radiation therapy, metastasis and death remain a significant problem in 50% of patients who present with high-risk soft tissue sarcomas. The most common site of metastasis is the lungs, and metastasis generally occurs within two to three years after the completion of therapy. Progress in the molecular characteristics of these tumors should in the near future translate into molecularly based therapies that can be incorporated into standard treatment strategies.
Topics: Gastrointestinal Neoplasms; Humans; Incidence; Neoplasm Recurrence, Local; Neoplasm Staging; Prognosis; Retroperitoneal Neoplasms; Sarcoma
PubMed: 15061599
DOI: 10.3322/canjclin.54.2.94 -
Primary retroperitoneal soft tissue sarcoma: Imaging appearances, pitfalls and diagnostic algorithm.European Journal of Surgical Oncology :... Jul 2017Although retroperitoneal sarcomas are rare tumours, they can be encountered by a wide variety of clinicians as they can be incidental findings on imaging or present with... (Review)
Review
Although retroperitoneal sarcomas are rare tumours, they can be encountered by a wide variety of clinicians as they can be incidental findings on imaging or present with non specific symptoms and signs. Surgical resection can offer hope of cure and patient outcomes are improved when patients are managed in high-volume specialist centers. Failure to recognize retroperitoneal sarcomas on imaging can lead to inappropriate management in inexperienced centers. Therefore it is critical that a diagnosis of retroperitoneal sarcoma should be considered in the differential diagnosis of a retroperitoneal mass with prompt referral to a soft tissue sarcoma unit. In particular, the most common retroperitoneal sarcoma subtypes, liposarcoma and leiomyosarcoma, have characteristic imaging appearances which are discussed. This review therefore aims to set the context and guide clinicians through a diagnostic pathway for retroperitoneal masses in adults which arise extrinsic to the solid abdominal viscera.
Topics: Algorithms; Biopsy, Large-Core Needle; Contrast Media; Diagnosis, Differential; Humans; Image-Guided Biopsy; Magnetic Resonance Imaging; Retroperitoneal Neoplasms; Sarcoma; Tomography, X-Ray Computed
PubMed: 28057392
DOI: 10.1016/j.ejso.2016.10.032 -
European Journal of Surgical Oncology :... Jun 2023Retroperitoneal sarcomas (RPS) refer to a heterogeneous group of malignancies of mesenchymal origin developing from retroperitoneal tissues and vessels. The most... (Review)
Review
Retroperitoneal sarcomas (RPS) refer to a heterogeneous group of malignancies of mesenchymal origin developing from retroperitoneal tissues and vessels. The most frequent RPS are well differentiated/dedifferentiated liposarcomas and leiomyosarcomas, but other rare histological subtypes can be observed. Over the last decade, significant advances have been made in the pathological and molecular characterization of sarcomas. These advances have led to major changes in their diagnostic management as well as in the development of new therapeutic strategies based on tumor biology and microenvironment. This review describes the current knowledge and recent findings in the pathology and molecular biology of the most frequent RPS subtypes.
Topics: Humans; Sarcoma; Liposarcoma; Leiomyosarcoma; Retroperitoneal Neoplasms; Soft Tissue Neoplasms; Molecular Biology; Tumor Microenvironment
PubMed: 35151525
DOI: 10.1016/j.ejso.2022.02.005