CLINICAL CASE: SUCCESSFUL SURGICAL TREATMENT OF A MALIGNANT PERIPHERAL NERVE SHEATH TUMOR (TRITON TUMOR)

Abstract

Introduction Malignant peripheral nerve sheath tumors belong to the category of rare neoplastic pathologies and form a distinct nosological group within the structure of soft tissue sarcomas, accounting for no more than 10 % of total cases.

Case description. We present a clinical case of diagnosis and treatment of a rare malignant tumor — a Triton tumor characterized by divergent differentiation (a combination of Schwann cell and rhabdomyoblastic components). The patient, a 38‑year‑old woman, presented with complaints of persistent dull pain localized in all abdominal regions and a body temperature rise up to 38 °C. According to imaging findings (multidetector computed tomography, MDCT), a space‑occupying pathological mass measuring 125×95×60 mm was detected in the anatomical area of the ligament of Treitz. Following preoperative diagnostics, radical surgical resection of the tumor was performed in compliance with oncological radicalism principles. The final diagnosis was established based on a comprehensive morphological examination: histological analysis revealed a polymorphic cell population, and immunohistochemical testing confirmed the expression of S100 markers (Schwann cell component) and Desmin/MyoD1 (rhabdomyoblastic component). The postoperative period proceeded without complications, and the patient was discharged in a satisfactory condition.

Conclusion. The malignant neoplasm of this etiology represents a rare neurogenic tumor, primarily developing from elements of nerve plexuses and peripheral nerve trunks