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
Fibrous hamartoma of infancy: An Italian multi-institutional experience
DALL'IGNA P;
2006-01-01
Abstract
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 excellentI documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
