Primary Intranodal Epithelioid Rhabdomyosarcoma

Abstract

We report an unusual, rare case of primary epithelioid rhabdomyosarcoma of a lymph node in the parotid basin. A 72-year-old man with a history of squamous cell carcinoma of the forehead and cheek had a 5-cm mobile nontender mass of the parotid tail and right level II region. A positron emission tomography/computed tomography scan confirmed a hypermetabolic soft tissue mass in the right parotid gland. Histologic sections showed an intraparotid lymph node almost completely effaced by a centrally necrotic malignant epithelioid neoplasm consisting of uniform-appearing dyshesive cells exhibiting rhabdoid morphologic features with abundant eosinophilic fibrillary cytoplasm, eccentric nuclei, and prominent nucleoli. Bizarre cells were not seen. In immunohistochemical studies, neoplastic cells expressed desmin and myogenin. Electron microscopy showed a mixture of thick and thin filaments, primitive Z-band formation, and well-formed sarcomeres. Fluorescence in situ hybridization studies for FOXO1, PAX3, and/or PAX7 rearrangements were negative. An extensive clinical and radiologic workup showed no evidence of primary tumor elsewhere. Complete resection of the tumor was performed, and adjuvant chemotherapy was given; patient was disease free 12 months after surgery.