A 68-year-old Japanese male was indicated a chest radiographic abnormality in the right upper lung field by routine health check-up. One month later, a right chest pain occurred, and he was diagnosed with right upper lobe pneumonia at the previous hospital. Despite antibiotic treatment, chest radiographic abnormality got worse and he was referred to our hospital for more thorough examinations. The patient was a current smoker with a 24-pack-year history of smoking. He had no medical history. His vital signs were within the normal limits. A general physical examination revealed an enlarged lymph node in the right axillary region. His lymph node was firm, unmovable and non-tender and measured 3.0 x 3.0 cm. A chest X-ray showed consolidation in the right upper lung field (Fig. 1). Contrast-enhanced whole-body computed tomography (CT) disclosed a right axillary lymphadenopathy and a right hilar mass with obstruction of the main bronchus (Fig. 2). 18-F-fluorodeoxyglucose (FDG) positron emission tomography (PET) revealed an abnormal FDG accumulation in the subcutaneous nodules on the mandibular and left chest wall, a part of the trachea, lymph nodes (submandibular, right axillary and mediastinal), right hilar mass, retroperitoneal mass and mass in the right femoral muscle with high metabolic activity (SUVmax: 5.75-21.1) (Fig. 3).
Bronchoscopy showed a white, moss-like appearance of the trachea just below the glottis and right B3 entrance stenosis with a large amount of purulent discharge (Fig. 4). A transbronchial biopsy (TBB) was performed on both lesions, and atypical medium-to large-sized cells were found arranged in nests with marked lymphocyte infiltration. An immunohistochemical study revealed focal positive staining for CD3, CD8, TIA-1, and C:C chemokine receptor type 4 (CCR4), and negative staining for CD4, CD20, and EBV (Fig. 5A and B). In addition, a biopsy of the subcutaneous nodules on the left chest wall also showed the same findings (Fig. 5C and D). A bone marrow biopsy showed hypoplastic marrow, and invasion of lymphoma was not suspected. From these results, the patient was diagnosed with stage IVA PTCL-NOS, and chemotherapy was started. The chemotherapy regimen included cyclophosphamide, adriamycin, vincristine, and prednisolone (CHOP therapy). Grade 4 neutropenia and Grade 1 thrombocytopenia were observed during the treatment. After five cycles of chemotherapy, contrast-enhanced whole-body CT showed a reduction in the right hilar mass and all of the lymph nodes (right axillary, mental, submandibular, cervical, subcarinal, splenic hilar and retroperitoneal region) (Fig. 6), with a partial response according to the Response Evaluation Criteria in Solid Tumors (version 1.1). After six courses of CHOP, contrast whole-body CT revealed an enlargement of the tumor mass in the right hilum, and salvage chemotherapy (CHASE therapy: cyclophosphamide, cytarabine, etoposide, and dexamethasone) was started. The patient ultimately died because of septic shock after undergoing three courses of CHASE therapy.
CT images showed the marked reduction in the right hilar mass and multiple lymphadenopathies after five cycles of chemotherapy.