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Giant fibrous hamartoma of infancy: A case report

RATIONALE: Fibrous hamartoma of infancy (FHI) is a rare benign soft-tissue tumor in children with a triphasic organoid histologic appearance. PATIENT CONCERNS: We here report a case with the largest FHI published so far. A 13-month-old boy with a rapidly growing tumor 45 cm in length and 69.3 cm in...

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Detalles Bibliográficos
Autores principales: Wang, Sun, Ma, Qichao, Ying, Hao, Jiao, Qin, Yang, Dan, Zhang, Bin, Zhao, Lihua
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7440069/
https://www.ncbi.nlm.nih.gov/pubmed/32176087
http://dx.doi.org/10.1097/MD.0000000000019489
Descripción
Sumario:RATIONALE: Fibrous hamartoma of infancy (FHI) is a rare benign soft-tissue tumor in children with a triphasic organoid histologic appearance. PATIENT CONCERNS: We here report a case with the largest FHI published so far. A 13-month-old boy with a rapidly growing tumor 45 cm in length and 69.3 cm in circumference of the left lower extremity was identified immediately at birth. DIAGNOSES: The diagnosis was uncertain before the operation, although biopsy was conducted. The postoperation histological examination showed arranged spindle cells, adipose tissue, and nests of immature small cells embedded in a myxoid background, which is the characteristic triphasic histology of FHI. INTERVENTIONS: Under general anesthesia, hip and lower extremity amputation was performed. OUTCOMES: The patient was followed-up for 6 years and no signs of recurrence were found, suggesting a favorable prognosis, although a part of the residual tumor was present after the surgery. LESSONS: FHI exhibits pathological and clinical characteristics. However, differential diagnosis of FHI from other soft-tissue tumors before operation remains a challenge. Thus, sometimes, aggressive therapy for the treatment of FHI might be a good choice to get a satisfactory.