家族内発症した鼻腔髄外形質細胞腫の２例

Abstract

We report two cases of familial extramedullary plasmacytoma found primarily in the nasal cavity, which was considered to be extremely rare.Patient 1(mother) was a 63-year-old woman with an extramedullary plasmacytoma arising in the nasal cavity, which recurred twice in ten years. The first occurrence was observed in the left middle nasal meatus at 53 years of age, which was completely reactive to radiotherapy. In the next year, a local recurrence was found in the primary region, which was treated with surgical resection and chemotherapy. After ten years, we operated on the secondary extramedullary plasmacytoma that recurred in the right nasal septum.Patient 2 (daughter) was a 40-year-old woman with an extramedullary plasmacytoma arising in the left nasal septum, which was treated with surgical intervention. An immunohistochemical examination revealed IgA-λ-positive findings in both patients.This disease is considered to be a symptom of multiple myeloma. Genetic or environmental factors are considered to be responsible for the onset of multiple myeloma. Therefore, the immunohistochemical findings of these patients are very interesting. The resolution of the mechan-ism, such as genetic or environmental factors, is necessary in the future.