Emergency surgical treatment of an ulcerative and hemorrhagic congenital/infantile fibrosarcoma of the lower leg

Abstract

Fibrosarcomas are rare malignant soft-tissue tumors occurring mostly in infants younger than 1 year of age. Fibrosarcomas can ulcerate and cause various complications, which could threaten a fetus in utero or a child in the early neonatal period. We report a unique case of congenital infantile fibrosarcoma of the lower leg, its treatment and pathology. The large expansive and destructive lesion was not appreciated on routine prenatal ultrasound exams at 20 and 33 weeks gestation. The newborn required immediate emergency surgical intervention after delivery to prevent death by hemorrhagic shock. Initial debulking of the tumor was performed and hemostasis was attained on the day of birth. The child was resuscitated and definitive treatment of the leg was deferred until a pathologic diagnosis was obtained. Given the extent of the fibrosarcoma, the lower leg was not salvageable and the patient received a through-the-knee amputation in the neonatal period. The patient is free of disease at 2 years of age.

Superficial and Deep Infiltrating Congenital Juvenile Xanthogranuloma Involving Multiple Skeletal Muscles and Associated with Ulceration and Generalized Postinvolution Atrophy

“…A major clinical and radiologic differential diagnosis for our patient was IFS, a rare pediatric soft tissue tumor involving the extremities. As in the present case, IFSs can ulcerate and present with a poorly circumscribed and highly vascular tumor on ultrasound and MRI . Because IFS is characterized histologically by spindle cells with a herringbone pattern in a myxoid background, it can be easily distinguished from JXG upon biopsy .…”

Early Surgery Is Feasible for a Very Large Congenital Infantile Fibrosarcoma Associated With Life Threatening Coagulopathy: A Case Report and Literature Review

“…Bleeding or consumptive coagulopathy is a rare event in CIF and can be life-threatening. Most literature reported the occurrence of massive bleeding or coagulopathy in patients diagnosed within the first 2 months of life, which led to mortality in some patients (11,13). Many of the patients were initially treated as hemangioma of infancy.…”

Congenital infantile fibrosarcoma mimicking sacrococcygeal teratoma in a Ghanaian infant: A case report and review of the literature

“…The tumour is a fibroblastic soft-tissue sarcoma with a low-to-intermediate malignant potential. [5,[7][8][9][10][11][12][13][14][15] An incidence of five per million infants has been reported, with males more commonly affected. [1,5,16] CIFS can occur in any part of the body and has been found in unusual parts of the body; [17,18] however, the extremities, namely the head, neck and trunk, in that order, are the most frequently affected locations.…”