A 12-year-old girl with the diagaosi of multiple myeloma is described. She presented with a nasopharyngeal mass which was histologically found to be a plrsmacytoma. Serum immunoelectrophoresis revealed an IgA-kappa M-protein (4.9 g/dl). There were approximately 20% atypical plasma cells in a bone marrow biopsy specimen. The diagnosis was further supported by immunohistochemical demonstration of cytoplasmic monoclonal IgA-kappa in the tumor cells of both the nasopharyngeal and bone marrow biopsies.
The patient was treated with chemotherapy for 1 year, at which time she became refractory to treatment, based on serum IgA levels. Five months after cessation of therapy, she continues to exhibit a significant objective response, remaining clinically well with a stable, elevated serum IgA level.
Cancer 56:2 143-2 147, 1985.
ULTIPLE MYELOMA (plasma cell myeloma) is the M most common form of plasma cell neoplasm. The incidence of myeloma increases progressively with age; it is extremely rare in the pediatric age group. While a number of cases have been reported that lacked convincing evidence for the diagnosis,'" only two reports of welldocumented patients with myeloma have been described younger than age 30 years.'** This report describes a 12-year-old girl with the diagnosis of multiple myeloma, based on the presence of a nasopharyngeal plasmacytoma on tissue biopsy, bone marrow plasmacytosis of 2096, and IgA-kappa monoclonal immunoglobulin in the cytoplasm of the tumor cells and in the patient's serum (4.9 g/dl).
Case Report
A 12-year-old Chinese girl complained of inability to breathe through her nose and stuffiness of her ears for I month. There was no response to decongestants and antibiotics and she was referred to an otolaryngologist, who noted a mass in the posterior pharynx. She was otherwise completely asymptomatic, without weakness, fatigue, or bone pain. No other abnormalities were noted on exam.
Routine histologic sections of the biopsied mass revealed a diffuse, monotonous infiltration of immature plasma cells (Fig.