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 Fibrous Hamartoma of Infancy

Visit:  Soft Tissue Pathology

Fibrous hamartoma of infancy.J Coll Physicians Surg Pak. 2006 May;16(5):381-2.

Two cases of male infants with masses since birth are presented. The first infant was four months of age, had mass on the medial surface right upper arm while the other infant, who was one year of age, had a mass above left eye. Both masses were present at birth and were slowly growing since then. The masses were excised after clinical and radiological assessments. Histopathological examinations of the masses were reported to be the fibrous hamartoma of infancy (FHI) which is a very rare entity.

Fibrous hamartoma of infancy manifesting as multiple nodules with hypertrichosis.J Dermatol. 2006 Jun;33(6):427-9.

Fibrous hamartoma of infancy is a rare benign tumor, which usually occurs within the first year of life. Most cases present as a solitary, painless, skin-colored subcutaneous nodule; multiple lesions are rarely reported. There have also been a few cases which have showed overlying skin changes, including hypertrichosis and alterations in pigmentation. We report a rare case of fibrous hamartoma of infancy in a 6-month-old female who presented as multiple nodules with overlying hypertrichosis on the left buttock. To the best of our knowledge, there have been only two reported cases in the dermatological published work that showed these unusual findings simultaneously.

Fibrous hamartoma of infancy: an Italian multi-institutional experience. J Am Acad Dermatol. 2006 May;54(5):800-3.

BACKGROUND: Fibrous hamartoma (FH) of infancy is a benign mesenchymal tumor, occurring as a superficial mass. Complete excision is curative. OBJECTIVE AND METHODS: The clinical features and treatment results of 18 children with FH are described. RESULTS: Local excision was the most common procedure. Surgery was radical in 10 patients, with microscopic residual disease in 6; all of them are alive with no evidence of disease 2 to 49 months after diagnosis. One patient, treated with a local reexcision for macroscopic residual disease (and chemotherapy for a synchronous desmoid fibromatosis) is well 83 months after diagnosis; the last patient, with a lesion of the labia majora, only underwent biopsy and is doing well, awaiting plastic surgery. LIMITATIONS: The results did not reach statistical significance due to difficulties in collecting cases. CONCLUSIONS: FH should be treated by complete excision; in our experience a nonradical excision was also able to achieve the cure. An aggressive approach should be avoided, as the overall prognosis is excellent.

 

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