Acral myxoinflammatory fibroblastic sarcoma: case series and immunohistochemical analysis.
ABSTRACT Acral myxoinflammatory fibroblastic sarcoma (AMFS) is a rare, low-grade neoplasm most often occurring on the extremities of adults. It consists of mixed inflammatory infiltrates with nodules of epithelioid, spindled and bizarre-appearing cells within a fibrosclerotic-to-myxoid stroma. AMFS frequently recurs, but only rarely metastasizes.
A retrospective analysis of all cases of AMFS seen in the past 4 years from the Stanford University Laboratory of Surgical Pathology and collaborating institutions was performed. We sought to better characterize the clinicopathologic characteristics of this rare tumor. Immunohistochemical stains, including CD34, epithelial membrane antigen (EMA), epidermal growth factor receptor (EGFR), CD117, CD163, Ki67 and p53, were also performed.
Eighteen cases were analyzed, and clinical information was available on 13 of them. The mean age at diagnosis was 48 years old, 10/13 (77%) occurred on the distal extremities and diameter of the lesions ranged from 1.0 to 10.0 cm. Treatment included wide local or radical excision and local recurrences were not reported. Many of the lesions were multinodular. Histologic characteristics included the presence of fibrosclerotic and myxoid stroma, sheets of spindled to round epithelioid cells, Reed-Sternberg or virocyte-like cells, lipoblast-like cells and rare mitotic figures. In most cases, CD34, EGFR and CD163 were diffusely positive. EMA and CD117 were weakly positive in some cases. Ki67 labeled < 10% of cells, and staining with P53 was variable.
Because AMFS may be mistaken for lymphoma, infection or tumors with higher metastatic potential, correct diagnosis is important to avoid unnecessary procedures and allow for proper clinical management. EGFR positivity suggests possible therapeutic use in aggressive cases.
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ABSTRACT: Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare low-grade, malignant soft tissue tumor that is usually observed in the extremities of adult patients. Magnetic resonance imaging (MRI) findings for this tumor type have rarely been reported. We report a case involving the distal left femur of a middle-aged man and tumoral invasion of the bone, which, to our knowledge, has been previously described only once. He was treated by distal femoral tumor resection and reconstruction with a modular prosthesis. Histopathologic diagnosis confirmed MIFS. We reviewed literature of the diagnostic imaging and bone invasion findings associated with this tumor type.Ai zheng = Aizheng = Chinese journal of cancer 07/2014;
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ABSTRACT: Myxoinflammatory fibroblastic sarcoma (MIFS) is a malignant mesenchymal neoplasm most frequently arising in the distal extremities of adults, which usually behaves in a low-grade manner but is capable of metastasizing to local and distant sites, rarely leading to death. It is a rare tumor whose unusual morphology can lead to erroneous histologic diagnosis, either as a nonneoplastic (infectious or inflammatory) process or as a variety of neoplastic diseases. While its exact origin is uncertain, ultrastructural studies have shown at least some of the constituent cells to be modified fibroblasts. Distinct and reproducible genetic abnormalities identified in MIFS are translocation t(1;10)(p22:q24), with rearrangements of the TGFBR3 and MGEA5 genes associated with increased levels of FGF8, and formation of marker/ring chromosome 3, with amplification of the VGLL3 locus. Because these genetic abnormalities are shared by both MIFS and hemosiderotic fibrohistiocytic lipomatous tumor, it is thought that these 2 morphologically distinct neoplasms may comprise a spectrum of disease defined by these genetics. We review the literature on MIFS and discuss morphology (including that of MIFS/hemosiderotic fibrohistiocytic lipomatous tumor hybrid lesions), immunohistochemistry, the differential diagnosis, and recent molecular genetic developments.Archives of pathology & laboratory medicine 10/2014; 138(10):1406-1411. · 2.88 Impact Factor
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ABSTRACT: Acral myxoinflammatory fibroplastic sarcoma is an extremely rare soft-tissue sarcoma. It typically presents as an inflammatory mass in the distal extremities of adult patients. The authors present a review of the available literature as well as a discussion on the surgical management of a patient with acral myxoinflammatory fibroplastic sarcoma who originally requested conservative management but ultimately required a two-digit ray amputation after local recurrence.The Canadian journal of plastic surgery, Journal canadien de chirurgie plastique 01/2013; 21(2):92-4. · 0.27 Impact Factor