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infantile digital fibromatosis

Wednesday 17 December 2003

inclusion body fibromatosis, infantile digital fibroma; Reye’s tumor, fibroma durum multiplex, infantile dermal fibromatosis, recurrent digital fibroma, and juvenile dermatofibroma

PO

Eight percent of infantile digital fibromatoses occur in the first year of life.

The condition may occur as a single nodule or in multiple lesions on the fingers or toes, and the thumb and great toe are always spared.

Characteristic “kissing” lesions may occur on adjacent surfaces of digits. These lesions may be associated with joint deformities and functional impairment.

Recurrence occurs relatively commonly after surgical excision; wide excision reduces the likelihood of recurrence. Lesions may resolve spontaneously, and so, a period of observation before excision is justified.

However, frequently, the clinical diagnosis is not appreciated, and the unwitting surgeon may underestimate the significance of a relatively innocuous appearing lesion and so perform inadequate excision.

Microscopy

The histology of infantile digital fibromatosis is characterized by fibroblastic proliferation within a collagenous matrix.

Mitotic figures are scant.

Eosinophilic, intracytoplasmic inclusion bodies are pathognomonic.

See also

-  fibroblastic tumors
- eosinophilic cytoplasmic inclusion bodies

Immunochemistry

Immunohistochemically, the inclusion bodies do not stain with antibodies to vimentin, keratin, or desmin and are thought to be actin contractile filaments.

- smooth muscle actin + (SMA+)

References

- Infantile digital fibroma/fibromatosis. A clinicopathologic and immunohistochemical study of 69 tumors from 57 patients with long-term follow-up. Laskin WB, Miettinen M, Fetsch JF. Am J Surg Pathol 2009; 33: 1-13.

- Fibrous tumors of infancy and childhood. Enzinger and Weiss’s Soft Tissue Tumors. Weiss SW, Goldblum JR. 5th ed. St Louis, MO: Elsevier/Mosby; 2008:p 257–302.

- Recurring digital fibrous tumors of childhood. Reye RDK. Arch Pathol. 1965;80:228–336.

- Fibroblastic-myofibroblastic tumors in children and adolescents: a clinicopathologic study of 108 examples in 103 patients. Coffin CM, Dehner LP. Pediatr Pathol 1991;11: 569-88.

- Les tumeurs mésenchymateuses du nouveau-né. Minard-Colin V, Orbach D, Martelli H, Bodemer C, Oberlin O. Arch Pediatr 2009;16:1039-48.

- A range of histologic findings in infantile digital fibromatosis. Grenier N, Liang C, Capaldi L, Ney A, Lapidus C, Schappell D, et al. Pediatr Dermatol 2008;25:72-5.

- Infantile digital fibromatosis. Immunohistochemical and immunoelectron microscopic studies. Choi KC, Hashimoto K, Setoyama M, Kagetsu N, Tronnier M, Sturman S. J Cutan Pathol 1990;17:225-232.

- Further documentation of spontaneous regression of infantile digital fibromatosis. Niamba P, Léauté-Labrèze C, Boralevi F, Lepreux S, Chamaillard M, Vergnes P, et al. Pediatr Dermatol 2007;24:280-4.

- Posttraumatic occurrence of infantile digital fibromatosis. A histologic and electron microscopic study. Miyamoto T, Mihara M, Hagari Y, Shimao S, Nakahara T, Kimura M. Arch Dermatol 1986; 122:915-8.