Hamartoma fibroso infantil: lesão volumosa com envolvimento de plexo braquil

Abstract

OBJETIVO: Apresentar um caso de hamartoma fibroso da infância em lactente ressecado tardiamente e se apresentando como lesão extensa, com envolvimento de elementos vasculares e neurais do plexo braquial. MÉTODOS: Estudo de caso clínico e revisão de literatura pertinente. RESULTADOS: Criança do sexo masculino, com massa tumoral axilar direita, irregular surgida aos 2 meses de idade e relacionada à administração da vacina BCG, sendo tratada com agentes anti-tuberculosos, sem resposta. Mediante biópsia a lesão foi diagnosticada como hamartoma fibroso infantil, e, após sofrer período de crescimento rápido, foi submetida à exérese cirúrgica completa. CONCLUSÃO: O hamartoma fibroso juvenil é um tumor benigno raro, tipicamente se apresentando no primeiro ano de vida em meninos, com localização mais comum no oco axilar. O diagnóstico diferencial se faz com tumores de partes moles em geral e, em casos de apresentação na axila direita, com adenopatias axilares causadas por reação à BCG. O tratamento é exerese completa da lesão e o prognóstico é favorável.

Fibrous hamartoma of infancy associated with bony remodeling and muscle atrophy

“…The differential diagnosis for indurated subcutaneous plaques in the newborn period encompasses benign and malignant entities, including but not limited to cystic hygromas, hemangiomas, neurofibromas, fibrolipomas, juvenile fibromatosis, myofibromas, congenital desmoid tumors, fibrosarcomas, and rhabdomyosarcomas …”

“…In most cases, the definitive treatment of FHI is complete surgical resection, which carries a 10% recurrence rate . There has been a prior recommendation to excise with a surgical margin of 1.0 cm that should include the adjacent unaffected tissue plane .…”

“…The differential diagnosis for indurated subcutaneous plaques in the newborn period encompasses benign and malignant entities, including but not limited to cystic hygromas, hemangiomas, neurofibromas, fibrolipomas, juvenile fibromatosis, myofibromas, congenital desmoid tumors, fibrosarcomas, and rhabdomyosarcomas. [6][7][8] The histologic diagnosis is very important in differentiating among pathologic entities. FHI diagnosis criteria outlines the presence of three components in various proportions including (a) tive, and the adipocytes are S100 protein positive.…”

“…Yearly standing bilateral leg radiographs were recommended to evaluate her leg lengths and alignment. Pediatric neurology was consulted to evaluate the patient's muscle development and nerve function, as some FHI have been shown to involve neurovascular structures . No recommendation was made to limit activities, and at all follow‐up appointments reviewed over the first year of life, she continued to display good overall growth and function.…”

Clinical and radiological characterization of fibrous hamartoma of infancy

“…Recurrence after complete resection is uncommon, having been reported in only approximately 10% of cases (5) .…”