Myxoinflammatory Fibroblastic Sarcoma of the Scalp

Abstract

Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare low-grade malignant soft tissue neoplasm, which usually presents on the distal extremities of adults and sometimes children. It has a characteristic appearance, of a lobulated or multinodular neoplasm of moderately atypical epithelioid or spindled cells, vacuolated cells, and enlarged or bizarre cells with macronuclei, with prominent mixed inflammatory infiltrate, and variably myxoid stroma. However, the proportions of each component vary, making diagnosis difficult, particularly when tumors arise at unusual nonextremity sites. We describe a case of MIFS occurring as a primary neoplasm on the scalp of an 80-year-old male, which recurred locally after 2 years and developed extensive bilateral cervical lymph node metastases. MIFS is exceptionally rare in the head and neck, and it has been described only once on the scalp. This case showed classical histologic features, and additionally a high mitotic index and atypical mitoses, which may be suggestive of a poorer prognosis. This case highlights the need to consider MIFS in the differential diagnoses of pleomorphic tumors of the head and neck, and it adds to the small number of MIFS showing highly aggressive behavior.

Recurrent BRAF Gene Rearrangements in Myxoinflammatory Fibroblastic Sarcomas, but Not Hemosiderotic Fibrolipomatous Tumors

“…MIFS with high mitotic count have been rarely reported, and their clinical significance remains unclear. 16,17 …”

Myxoinflammatory fibroblastic sarcoma of the liver: Case report and literature review

“…It often manifests as a painless mass at the distal end of the limb, primarily on the hands and feet, accounting for almost two-thirds of all cases. [4] However, it can occur in multiple body parts, including the iris, [5] parotid gland, [6] maxilla, [7] back, [8] eyeball, [9] nasal dorsum, [10] scalp, [11] and groin. [12] As a result, the World Health Organization (WHO) renamed it myxoinflammatory fibroblastic sarcoma (MIFS) in 2002.…”

Acral myxoinflammatory fibroblastic sarcoma with hybrid features of hemosiderotic fibrolipomatous tumor occurring 10 years after renal transplantation

“…MIFS typically behaves as a low-grade sarcoma with a high local recurrence rate, but very low metastatic rate 1 , 2 with only small numbers of regional nodal and distant metastases reported. 3 – 8 Histologically, MIFS is characteristically a multinodular lesion composed of three cell types: variably atypical spindle to epithelioid cells, vacuolated fibroblasts, and large pleomorphic virocyte-like or Reed–Sternberg-like cells 1 – 3 within prominent myxoid stroma containing a mixed inflammatory cell population and variable hemosiderin deposition and fibrosis. The etiology of MIFS remains unknown, although some are associated with characteristic genetic abnormalities.…”