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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.

Poroid hidradenoma (PH) is a rare benign neoplasm of the skin appendages. There are currently no guidelines for its management, and little information available regarding the natural history, treatment options, or outcomes. Systematic literature review identified 19 cases of isolated PH. Mean age at presentation was 54 years. Male to female ratio was 3:1, and the majority of cases were in the head and neck region. One-third of lesions were painful or tender. Sizes varied from 0.11 × 0.9 × 0.2 cm (digit) to 6 cm (presternal region). Twelve cases were treated by surgical excision, while the remainder were biopsy specimens. Follow-up was reported in five cases, with a median follow-up of 1 year. No cases of local invasion or spread have been reported, although there was one case of possible recurrence identified 8 years after surgical excision. The presented case is the first to identify a PH on the hand, represents the smallest lesion to date, and was successfully treated by excision. We suggest that PHs be treated by excision and followed up for 6 to 12 months. Given one case of potential (but unconfirmed) recurrence, and no report of malignant transformation, we suggest that narrow margins may be appropriate.

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