Primary giant congenital infantile fibrosarcoma of the left forearm

“…2,3 Reports of giant infantile sarcomas are very rare and even rarer are the gross nodular presentations of such tumours. [4][5][6] Giant infantile fibrosarcomas have been reported to occur in the scalp but it is said to be commoner in the extremities and head and neck regions. 6,7 A biopsy is necessary to confirm the diagnosis.…”

Section: Introductionmentioning

confidence: 99%

Giant multinodular infantile fibrosarcoma: a case report

et al. 2018

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Infantile fibrosarcoma is a rare type of soft tissue sarcoma seen in children usually less than 2years of age. Few cases of giant infantile fibrosarcoma have been reported in literature. We report a rare case of a giant multi-nodular infantile fibrosarcoma in the left anterolateral chest wall in a 7-year-old boy. The tumour was said to have been recurrent twice for about 6 years. At presentation patient was evaluated and was commenced on 6 cycles of neoadjuvant chemotherapy with vincristine, adriamycin and cyclophosphamide (VAC) regimen to alternate with Ifosfamide/Etoposide (IE) regimen with very good response. Thereafter, had a wide local excision of the tumour and then had 4 more cycles of adjuvant chemotherapy. His 6months follow up showed no evidence of tumour recurrence. Infantile fibrosarcoma is said to be chemo-sensitive tumour with very good response, though surgical excision is the main treatment of choice and overall it is said to have a good prognosis.

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“…primary myxoid mesenchymal tumor of infancy, and some other rare tumors (1)(2)(3)(4)(5). Congenital infantile fibrosarcoma (CIF) is a rare pediatric soft-tissue sarcoma and is typically observed in children <1 year of age (6)(7)(8). Although it is locally aggressive, metastasis is rare.…”

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confidence: 99%

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

et al. 2019

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Background: Congenital infantile fibrosarcoma (CIF) is a rare malignant soft tissue tumor that predominantly occurs in children under 1 year of age. CIF is frequently misdiagnosed with other conditions like hemangioma of infancy, infantile fibromatosis, or kaposiform hemangioendothelioma. Disseminated intravascular coagulopathy (DIVC) is rarely reported to be associated with CIF. Case presentation: We describe an infant who presented with a large mass over the right arm. She was initially treated conservatively as hemangioma but was later confirmed by tissue histopathological examination to have CIF as the mass rapidly increased in size. She developed massive intra-tumoral bleed with DIVC whilst receiving neoadjuvant chemotherapy requiring multiple blood products transfusion. An urgent near-total resection of the tumor was performed in view of life threatening bleeding despite multiple blood transfusions. Post-operatively, she received further adjuvant chemotherapy. Subsequently, she remained in complete remission 32 months off-treatment and has full function of the affected limb. Conclusions: CIF is an important condition to be considered in infant who has large mass over the extremity. DIVC could be associated with large CIF and when it occurs can be life-threatening. Whenever feasible early surgery should be performed in very young patients with large CIF to prevent mortality from bleeding.

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Primary giant congenital infantile fibrosarcoma of the left forearm

Cited by 5 publications

References 13 publications

Elbow Cystic Lymphangioma in an 8-Month-Old Boy

Elbow Cystic Lymphangioma in an 8-Month-Old Boy

Giant multinodular infantile fibrosarcoma: a case report

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

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