A 64-year-old man presented to the primary care physician with a 1-year history of nasal congestion. He was referred to our hospital because of abnormal physical findings in the nasal cavity. Other than hypertension, the patient had no medical or surgical history. Rhinoscopic examination showed an ulcerative lesion in the inferior concha, and physical examination indicated multiple lymphadenopathies in both axillae and subcutaneous nodules in the left back. Except for thrombocytosis (472×109/L), laboratory tests did not show abnormal findings. After obtaining written informed consent, an excisional biopsy of the inferior concha was performed. The lesion showed diffuse infiltration of spindle cells, large pleomorphic cells, foamy histiocytes, lymphocytes, and plasma cells (). The distinction between inflammatory conditions, such as rhinoscleroma, and neoplastic lesions, such as Rosai-Dorfman disease, IDCS, Langerhans cell histiocytosis (LCH), and follicular dendritic cell sarcoma (FDCS), was difficult; therefore, various immunohistochemical studies were performed. On the basis of the immunohistochemical results, i.e., a strong positive reaction for CD68, lysozyme, LCA, and S-100 protein but a negative reaction for CD34, CD1a, smooth muscle actin, and CD21 (), IDCS was diagnosed.
Fig. 1 Histological features of the nasal cavity include diffuse infiltration of spindle cells, large pleomorphic cells, foamy histiocytes, and various inflammatory cells (A). Immunohistochemical staining shows a strong positive reaction for S-100 protein (B) (more ...)
Computed tomography (CT) scans of the chest, abdomen, and pelvis showed multiple enhancing nodules in the subcutaneous layer of the back, with lymphadenopathies in both axillae (). A head and neck CT scan showed a soft tissue attenuating lesion in the right anterior ethmoid and nasal cavities and multiple lymphadenopathies on both sides of the neck level II (). Bone marrow involvement was not observed. The lesion in the nasal cavity was partially removed by conchotomy. Further to the previously reported successful treatment of IDCS with ABVD chemotherapy [8
], the same ABVD doses (25 mg/m2
adriamycin, 10 mg/m2
bleomycin, 6 mg/m2
vinblastine, and 375 mg/m2
dacarbazine) were infused on days 1 and 15 every 4 weeks. During chemotherapy, no significant complications were observed. After 8 cycles, CT scans of the chest, abdomen, pelvis, and neck showed complete resolution in the lymph nodes of both axillae and the subcutaneous nodules (). Lesions in the nasal cavity and cervical lymphadenopathies also showed complete resolution (). CT and PET scans showed no evidence of relapse after 1 year.
Computed tomography (CT) scans show multiple lymphadenopathies in both axillae (A) and lymphadenopathies on both sides of the neck (B).
CT scans after 8 cycles of ABVD (adriamycin, bleomycin, vinblastine, and dacarbazine) shows complete resolution of axillary lymphadenopathies (A) and cervical lymphadenopathies (B).