Morphological spectrum of inclusion body fibromatosis: A rare case report

A rare case of inclusion body fibromatosis

“…Inclusion body fibromatosis is a benign, sometimes multicentric, myofibroblastic tumor with characteristic eosinophilic intracytoplasmic inclusions and the potential for local recurrence. 1 This tumor accounts for 0.2% of registered soft tissue tumors and 2% of pediatric fibroblastic tumors. 1 The classic subtype involves the dorsal or dorsolateral aspect of the distal or medial portion of the second, third, and fourth digits followed by the fifth digit, usually sparing the first digit, hand, and foot.…”

“…1 This tumor accounts for 0.2% of registered soft tissue tumors and 2% of pediatric fibroblastic tumors. 1 The classic subtype involves the dorsal or dorsolateral aspect of the distal or medial portion of the second, third, and fourth digits followed by the fifth digit, usually sparing the first digit, hand, and foot. 1,2 It presents as an asymptomatic dome-shaped or polypoid skin nodule, typically no larger than 2 cm.…”

“…1 The classic subtype involves the dorsal or dorsolateral aspect of the distal or medial portion of the second, third, and fourth digits followed by the fifth digit, usually sparing the first digit, hand, and foot. 1,2 It presents as an asymptomatic dome-shaped or polypoid skin nodule, typically no larger than 2 cm. 3 Tumors are firm or rubbery, ill-defined protuberant or polypoid dermal nodules.…”

“…The cut surface is fibrous and almost white to gray. [1][2][3] Microscopic images show spindle-shaped cells with slightly eosinophilic cytoplasm and an elongated cytologically unremarkable nucleus. Cells proliferate in whorls, interspersed with short fascicles or storiform fields in a variably collagenous dermis.…”

“…Inclusion body fibromatosis, more commonly known as infantile digital fibromatosis, most commonly presents as a small digital nodule in young children of both sexes. 1 In up to a third of patients, the lesion is congenital, and about 15% have multiple lesions that may affect both hands or feet, either synchronously or asynchronously. Strangely, the thumb and big toe always seem to be spared.…”