Giant fibrous hamartoma of infancy: A case report

Abstract

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.

Figure 1

A 13-month-old boy with fibrous hamartoma of infancy, which is 45.0 cm in length and 69.3 cm in circumference in the left lower extremity (anterior view).

Figure 2

The preoperative MRI, CTA, and DSA examination of the patient. (A) The Axial and coronal MRI image demonstrated a giant mass with ill-defined border. The musculature, fascia, overlying subcuticular fat and skin infiltration was obvious. (B and C) CTA and DSA demonstrated increased and abnormal morphology of vascular branches.

Figure 3

Histological findings. (A) Hematoxylin-eosin (H&E) staining indicated the classic histopathologic features of FHI (×200). (B and C) Immunohistochemical specimens for SMA and CD34, respectively (×200).