A 43-year-old female presented with a history of epistaxis and left-sided nasal block of a one-month duration. Examination revealed a fleshy growth in the left nasal cavity and a left level II cervical lymph node enlargement. Computerized tomography (CT) scan of the local part revealed a large mass in the left nasal cavity, extending to the left maxillary antrum and ethmoidal sinus . The left medial wall of the maxillary antrum and inferior orbital plate was breached by the tumor.
Computerized tomography scan of the local part revealed a large mass in the left nasal cavity, extending to the left maxillary antrum and the ethmoidal sinus
A biopsy revealed that the tumor tissue was lined by respiratory epithelium. The cells were arranged in sheets and alveolar masses and were composed of round-to-oval cells of moderate size with a moderate amount of cytoplasm. The nuclei were round-to-oval and hyperchromatic. Mitoses varied from one to two/high power field. Moderate cellular and nuclear pleomorphism was seen. Few elongated cells were also seen among the rounded cells.
Immunohistochemistry showed a strong, positive reaction for muscle-specific actin, desmin, CD99, and the neuron-specific enolase. Areas showed positivity for synaptophysin, neurofilament, and S-100p, suggestive of neural differentiation. Some of them showed a faint positive reaction for the epithelial membrane antigen and pankeratin. Strap muscle cells and ganglion cells were not recognized. A diagnosis of ectomesenchymoma was made, showing mainly rhabdomyoblastoma- and neuroblastoma-like areas.
CT of the chest and bone marrow biopsy were normal. The bone scan showed uptake in the left nasal bone and the left maxillary antrum. The patient was staged as the International Rhabdomyosarcoma Group (IRSG) Stage III Group III.[2
] The patient has completed 50.5 Gy radiotherapy to the local part and has completed combination chemotherapy as per the IRS IV protocol. Primary surgery was not possible as the tumor was very extensive and it would be a mutilating surgery.