Vulvar sarcoma outcomes by histologic subtype: a Surveillance, Epidemiology, and End Results (SEER) database review

ObjectiveVulvar cancers account for 5% of all gynecologic malignancies; only 1%–3% of those vulvar cancers are primary vulvar sarcomas. Given the rarity of vulvar sarcomas, outcome data specific to histopathologic subtypes are sparse. The aim of this study was to identify clinical and pathologic factors of primary vulvar sarcomas that are associated with survival and may inform treatment decisions.MethodsThe Surveillance, Epidemiology, and End Results (SEER) database was searched for women diagnosed with vulvar sarcoma between 1973 and 2018. We identified 315 patients and reviewed their demographic, clinicopathologic, surgical, and survival information. Statistical analyses included χ2 and t-tests, Kaplan–Meier survival, and Cox regression analyses.ResultsThe most common histopathologies of vulvar sarcomas were dermatofibrosarcomas (85/315, 27%) and leiomyosarcomas (72/315, 22.9%). Rhabdomyosarcomas (18/315, 5.7%), liposarcomas (16/315, 5.1%), and malignant fibrous histiocytomas (16/315, 5.1%) were less frequent. The majority of patients underwent surgery (292/315, 92.7%), which included lymph node dissections in 21.6% (63/292). Survival and lymph node involvement varied significantly with histologic subtype. The 5-year disease-specific survival for dermatofibrosarcomas, liposarcomas, and fibrosarcomas was 100% and only 60.3% and 62.5% for malignant fibrous histiocytomas and rhabdomyosarcomas, respectively. None of the patients with (dermato)fibrosarcomas, liposarcomas, or leiomyosarcomas had positive lymph nodes, in contrast to rhabdomyosarcomas and malignant fibrous histiocytomas with 77.8% and 40% positive lymph nodes, respectively. The 5-year disease-specific survival for women with positive lymph nodes was 0%.ConclusionsVulvar sarcomas are heterogeneous with survival highly dependent on the histopathologic subtype. While surgical excision is the mainstay of treatment for all vulvar sarcomas, staging lymphadenectomy should be deferred for (dermato)fibrosarcomas, liposarcomas, and leiomyosarcomas as there were no cases of lymph nodes metastases.

Primary giant cell malignant fibrous histiocytoma-associated with renal calculus

Malignant fibrous histiocytomas (MFH) are the most commonly seen soft tissue sarcomas in adults. It is rarely seen in some visceral organs. Kidneys are the parenchymal organs in which MFHs are most frequently seen. More than 50 cases of primary renal MFH have been reported. Among these cases, only 1 was reported as primary giant cell subtype in association with urolithiasis. This case report is the second such case with the these characteristics.

Diagnostic approach to malignant fibrous histiocytomas of soft tissue in dogs: a case report

Malignant fibrous histiocytomas (MFHs), newly named as ‘undifferentiated pleomorphic sarcomas’ in 2002 by the World Health Organization, generally show an ambiguous origin. They have been described to fibroblastic or histiocytic in origin, while storiform-pleomorphic variants share a similar morphologic pattern with other sarcomas. For this reason additional diagnostic methods including immunology, ultrastructure analysis and molecular approaches are necessary for a more accurate diagnosis. We report three cases of MFH in individual dogs which presented histological characteristics of pleomorphic sarcomatoid tumours. Microscopic investigation, immunohistochemistry (vimentin, CD68, desmin, α-smooth muscle actin, and S-100) and electron microscopy were performed for diagnosis. One case was diagnosed as storiform-pleomorphic type and two were diagnosed as giant cell type MFHs. The present study demonstrates concepts of MFHs based on newly described categories and suggests useful diagnostic approaches for uncertain sarcomatoid soft tissue tumours in canines.

MASL1: a neglected ROCO protein

The human ROCO proteins are a family of four proteins characterized by a conserved supradomain: a Ras-like GTPase domain. This domain consists of ROC (Ras of complex proteins) occurring in tandem with a COR (C-terminal of ROC) domain. Together, these proteins are linked to various pathologies including cancer and PD (Parkinson's disease). Despite an increasing research focus on these proteins, their functions in general, and their specific roles in disease, are still unknown. In the case of MASL1 (malignant fibrous histiocytoma amplified sequences with leucine-rich tandem repeats 1), a predicted oncoprotein in MFHs (malignant fibrous histiocytomas), there is a particular lack of information available in the literature. The aim of the present review is therefore to summarize the existing information on MASL1 and also to compile data that could be linked to MASL1 and thus help our understanding of this neglected ROCO protein.