Radiosensitivity of malignant roundcell rhabdomyosarcoma in the nasal fossa of a child.

Abstract

Rhabdomyosarcomas of the nasal cavity in childhood are rare. Of the 167 rhabdomyosarcomas in children reported by Stout (4), only 3 were primary in the nasal cavity, the nasopharynx being the more common site. Fifteen cases of embryonal rhabdomyosarcoma arising in cervical, facial, and orbital musculature were reported from the New York Memorial Hospital by Stobbe and Dargeon (3), in patients ranging in age from sixteen months to sixteen years. In 1948 Cappell (1) described 3 such tumors in children, arising beneath the mucous membrane in the uvula and palate. Treatment consisted of local radium application and surgery but was of no avail. All patients had local recurrences and ultimately died with distant metastases in lymph nodes and lungs. Dargeon (2) has stated that rhabdomyosarcoma is radiosensitive but recurs after radiotherapy. Because of the rarity of this type of neoplasm and the paucity of knowledge of its radioresponsiveness, we are reporting a case in a young girl, arising in the nasal fossa, with histologically verified metastases in the cervical lymph nodes. This tumor, although unusually radiosensitive both at the primary and secondary sites, ultimately recurred and the patient died with pulmonary metastases. Case Report An 8-year-old Caucasian girl was admitted to the University of California Medical Center (San Francisco) on Jan. 6, 1960, with a history of recurrent episodes of right nasal bleeding since June 1959. In August 1959, there had been discovered a polypoid pink-gray mass on the right nasal fossa floor, extending along the nasal septum posteriorly to the entrance of, but not into, the nasopharynx. The tumor was removed surgically at that time and was histologically reported as “chronic granulation tissue.” Two months later a recurrence at the same site was removed (October 1959) by the same surgeon. The child was then referred to the University of California Medical Center for further treatment. On admission to this hospital, the right nostril was completely obstructed by a fleshy, red, polypoid tumor which extended through the right choana into the vault of the nasopharyngeal cavity. The left nasal fossa was not unusual. Otoscopic examination showed a fluid level on the right associated with diminished hearing on that side. Several enlarged, firm, tender lymph nodes were palpable in the right midcervical area under the sternocleidomastoid muscle. The left cervical lymph nodes were also slightly enlarged. The remainder of the physical examination was not significant. The child's birth, development, and past history were normal. The results of laboratory studies were normal. Roentgenograms showed a density in the right nasal fossa and nasopharynx (Fig. 1) and opacification of both maxillary antra. No bone destruction was seen. There was no abnormality apparent on the chest film.