Work

A unique pediatric thoracic fibrosarcoma: Case report and successful therapeutic strategy

Year: 2025

Type: article

Abstract: Congenital Infantile Fibrosarcoma (CIFS) is an exceptionally rare pediatric malignancy, representing approximately 10 % of all soft tissue cancers in young children. Its clinical manifestations vary according to tumor location. When tumors occur in unusual anatomical sites, symptoms may be misleading and delay proper diagnosis and treatment. We report an 11-month-old male with a month-long persistent dry cough and high fever unresponsive to antibiotics. Chest CT revealed a pleural mass with mild effusion. Thoracotomy achieved complete excision (R0). Histopathology showed low-grade fibrosarcoma; immunohistochemistry was positive for Vimentin and SMA, focally CD34, and negative for S100, Desmin, and Myogenin, with low Ki-67 (<3 %). ETV6-NTRK3 fusion testing was unavailable. The patient received six cycles of adjuvant VAC chemotherapy and tolerated treatment well, with only mild transient neutropenia. Diagnosis depends on histopathological and immunohistochemical analysis, as CIFS mimics several other soft tissue sarcomas. Our review of medical literature found no prior cases of CIFS originating in the pleura, underlining the uniqueness of this case. Management followed a standard multidisciplinary approach consisting of complete surgical excision and adjuvant chemotherapy (VAC protocol). At 12-month follow-up, the patient remained recurrence-free. Persistent pneumonia-like symptoms unresponsive to standard treatment in infants should prompt consideration of underlying malignancy. Early recognition and prompt management are essential for improving outcomes in such rare presentations. • Congenital infantile fibrosarcoma (CIFS) is a rare tumor presenting shortly after birth and developing in early childhood. • Pleural localization of CIFS is exceptionally uncommon, with only few cases described in the literature. • Persistent pneumonia-like symptoms not responding to antibiotics should prompt consideration of malignant etiologies. • Surgical removal is the primary treatment, often combined with chemotherapy. • Local recurrence occurs in up to 40 % of cases.

Source: International Journal of Surgery Case Reports

DOI: 10.1016/j.ijscr.2025.111999

Authors: Khaled Alomar, Kamar Shaker, Nidal Alkhani

Institution: Damascus University

Language: English

Topic: Sarcoma Diagnosis and Treatment

Subfield: Pulmonary and Respiratory Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $2,090