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Practical Issues for Retroperitoneal Sarcoma Vicky Pham, MS, Evita Henderson-Jackson, MD, Matthew P

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Practical Issues for Retroperitoneal Sarcoma Vicky Pham, MS, Evita Henderson-Jackson, MD, Matthew P Pathology Report Practical Issues for Retroperitoneal Sarcoma Vicky Pham, MS, Evita Henderson-Jackson, MD, Matthew P. Doepker, MD, Jamie T. Caracciolo, MD, Ricardo J. Gonzalez, MD, Mihaela Druta, MD, Yi Ding, MD, and Marilyn M. Bui, MD, PhD 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. Introduction tic, and predictive information based on a relatively Soft-tissue sarcomas are mesenchymal neoplasms small amount of tissue obtained on biopsy. that account for up to 1% of all newly diagnosed ma- In this study, we compare our experience, espe- lignancies at a rate of 3.6 per 100,000 per year.1-3 Com- cially the distribution of histological subtypes, with pared with bone or visceral sarcomas, they make up the literature to develop a better understanding of 58% of all sarcomas.3 Although the extremities rep- common and rare tumor types, pertinent ancil- resent the most common location of soft-tissue sarco- lary testing, diagnostic pitfalls, and refine a practi- ma, retroperitoneal sarcoma accounts for 9% to 15% cal, multidisciplinary, and algorithmic approach to of all adult soft-tissue sarcomas.3,4 For surgical pathol- achieve an informative diagnosis to help guide the ogists, retroperitoneal tumors will be encountered in therapeutic plan. daily practice regardless of the practice setting. Ret- roperitoneal sarcoma presents diagnostic challenges Methods due to its rarity, variety of tumor types, a general level After obtaining Institutional Review Board approval, of unfamiliarity among surgical pathologists, and lack the database Transmed (H. Lee Moffitt Cancer Center of generally accepted guidelines in its diagnostic ap- & Research Institute, Tampa, Florida) was retrospec- proach. In the era of personalized medicine, pathol- tively reviewed for all histological subtypes of retro- ogists play a critical and central role in patient care. peritoneal sarcomas from 1996 to 2015. Transmed in- Demand has increased to obtain diagnostic, prognos- tegrates data from all patients seen at Moffitt Cancer Center since 1996 (approximately 395,000 patients, From the University of South Florida Morsani College of Medicine regardless of diagnosis) as well as other patients not (VP), the Departments of Anatomic Pathology (EH-J, MMB), Sar- treated at Moffitt Cancer Center but who consented to coma (EH-J, JTC, RJG, MD, MMB), Surgical Oncology (MPD), and the Total Cancer Care protocol at one of 17 consor- Diagnostic Imaging (JTC), H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida, and the Department of Pathology (YD), tium sites (approximately 36,000 patients). This result JiShuiTan Hospital, Beijing, China. was cross-checked by sarcoma pathologists as well as Address correspondence to Marilyn M. Bui, MD, PhD, Department the deidentified result of another retroperitoneal sar- of Anatomic Pathology, Moffitt Cancer Center, 12902 Magnolia coma retrospective institutional review. Drive, Tampa, FL 33612. E-mail: [email protected] Submitted August 17, 2015; accepted December 29, 2015. A systematic review of the English literature This study received financial assistance from Information Shared was conducted for works published between 2000 Services Department/Collaborative Data Services Core at the H. Lee and 2015. This yielded 85 search results. Another Moffitt Cancer Center & Research Institute supported under NIH literature search was conducted yielding an ad- grant P30-CA76292. This research was also supported by an award from the Scholarly Concentrations Program at USF Health Morsani ditional 444 results. After excluding case reports College of Medicine. and studies with locations of retroperitoneal sarco- July 2016, Vol. 23, No. 3 Cancer Control 249 ma mixed with other locations, our search yielded 54 studies from which we extracted information Table 1. — Distribution of Histological Subtypes of Retroperitoneal Soft-Tissue Sarcoma about the distribution of histological subtypes with- in each study patient population. Subtype Distribution Distribution From the From The meta-analysis was conducted using Stats- Literature, Moffitt, % Direct (Cheshire, United Kingdom). % Liposarcoma (all subtypes) 45.1 54.6 Results Well differentiated 23.9 15.3 Literature Review A literature search revealed the following list of most Dedifferentiated 20.9 15.6 to least common histological subtypes of tumors. Per- Myxoid/round cell 0.9 6.8 centages shown are out of all retroperitoneal sarcoma Pleomorphic 0.4 1 subtypes (Table 1). Our review and data analysis of Leiomyosarcoma 21.3 26.1 retroperitoneal sarcoma studies concluded that the Other 8.2 0 most common subtype of retroperitoneal sarcoma Malignant fibrous histiocytoma/ 6.4 1 is liposarcoma, which constitutes 45.1% of all retro- undifferentiated pleomorphic peritoneal sarcoma (Fig 1). The next most common sarcoma subtypes are leiomyosarcoma (21.3%), other (8.2%), Sarcoma not otherwise specified 1.6 4.9 and malignant fibrous histiocytoma/undifferentiated Malignant peripheral nerve sheath 1.3 1 pleomorphic sarcoma (6.4%; see Table 1). Well-differ- tumor Well differentiated entiated liposarcoma and dedifferentiated liposarco- Solitary fibrous tumor/ 0.8 0 Dedifferentiated hemangiopericytoma ma constituted 45.8% and 44.8% of all retroperitoneal Myxoid/round cell liposarcomas, respectively (Fig 2). The less common Fibrosarcoma (nondesmoid) 0.5 1 Pleomorphic subtypes were myxoid/round cell liposarcoma and Rhabdomyosarcoma 0.4 1 pleomorphic liposarcoma. Schwannoma 0.3 0 Angiosarcoma 0.1 0 Institutional Results Anaplastic sarcoma < 0.1 0 A review of Transmed, which represents single-insti- tution data, is summarized in Table 1. Our data for Adenosarcoma < 0.1 0 retroperitoneal sarcoma subtypes are consistent with Chondrosarcoma < 0.1 0 our literature search. Liposarcomas of all subtypes at Desmoplastic small-round cell tumor < 0.1 0 Moffitt Cancer Center make up 54.6% (168 cases) of all Epithelioid sarcoma < 0.1 0 retroperitoneal sarcoma (307 cases), followed by leio- Extraskeletal osteosarcoma < 0.1 1 myosarcomas (80 cases), sarcoma not otherwise spec- Fibromyxosarcoma < 0.1 1 ified (15 cases), malignant peripheral nerve tumor Giant cell sarcoma < 0.1 3 (3 cases), and other less common subtypes (1–2 cases; see Table 1). The trend for most to least common tu- Mesenchymoma < 0.1 0 mor types appears to be similar (Fig 3). In Fig 4, data Primitive neuroectodermal tumor/ < 0.1 1 extraskeletal Ewing sarcoma from Moffitt Cancer Center on the histological sub- types of retroperitoneal liposarcomas are compared Small cell/embryonal/synovial/ < 0.1 0 undifferentiated sarcoma with the data found during our literature search. Spindle cell sarcoma < 0.1 1 The median age of the patients in all 54 studies was 58 years. The percentages of those who were men Undifferentiated round-cell < 0.1 1 sarcoma and women were 53.2% and 46.8%, respectively. Gap Between Practice and Literature tochemical hallmarks and characteristics that can be Most of the literature we reviewed describes sarcoma diagnosed with a small amount of tissue acquired on in categories based on histological type and morpho- biopsy; however, commonly, sarcoma does not have logical pattern.5 Morphological features are evaluated such a signature and morphologically overlaps with to determine the differential diagnosis, ranking pos- other tumors. Ancillary tests can be performed to sible diagnoses from most likely to least likely in con- narrow the diagnosis, with the understanding that it junction with clinical and radiological clues, and then may not be possible to achieve a definitive diagno- applying ancillary testing to narrow down the diagno- sis. However, it is important to realize that, when a sis. Thus, it is important to understand the common definitive diagnosis is not achievable, an alternative and rare types of retroperitoneal sarcoma. option is to provide useful, diagnostic information to Some sarcomas have molecular and immunohis- guide the clinical team in the next appropriate step 250 Cancer Control July 2016, Vol. 23, No. 3 Liposarcoma 45.1 Leiomyosarcoma 21.3 Other 8.2 Malignant fibrous histiocytoma/ 6.4 undifferentiated pleomorphic sarcoma Sarcoma not otherwise specified 1.6 Malignant peripheral nerve sheath tumor 1.3 Histological Subtype Solitary fibrous tumor/hemangiopericytoma 0.8 Rhabdomyosarcoma 0.4 0 5 10 15 20 25 30 35 40 45 50 Percentage Fig 1. — Distribution of histological subtypes of retroperitoneal sarcoma. Well
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