Peripheral T Cell Lymphoma Not Otherwise Specified (PTCL-NOS) Presenting as Endobronchial Lesion

Abstract

Introduction: This case report describes a rare presentation of peripheral T cell lymphoma not otherwise specified (PTCL-NOS) and emphasizes the importance of including lymphoma in the differential diagnosis of endobronchial lesion. CASE PRESENTATION: A 68-year-old man was referred to our hospital for the evaluation and treatment of pneumonia. One month before admission, he had undergone a physical check-up with his doctor, and chest X-ray revealed consolidation in the right upper lung field. Three weeks after the physical check-up, he presented with productive cough, and admitted to the previous doctor. He was diagnosed as pneumonia and started the treatment with antibiotics. His symptoms became less severe after the treatment, however, the consolidation in the right upper lung field got worse. His past medical history was bowel obstruction and chronic sinusitis. He had a 24 pack years smoking history, and no alcoholic history. Vital signs were as follows: heart rate, 60 bpm. respiratory rate, 20 breaths per minute. blood pressure, 110/68 mmHg. temperature, 36.7degreeC. and oxygen saturation, 96% on room air. Physical examination revealed left-sided facial pressure with yellow nasal discharge. Breathing sound was decreased over the right upper lung field and no adventitious sound was heard. Laboratory test values were as follows: white blood cells, 5,410/mm3; hemoglobin, 13.1g/dl; platelets, 400,000/mm3; serum C-reactive protein (CRP), 20.84 mg/dl; AST, 75U/l; ALT, 83U/l; ALP, 671U/l; another biochemistry and serology data were normal. Sputum acid-fast bacillus (AFB) smears and T-SPOT. TB blood tests were negative. The chest X-ray revealed consolidation on the right middle lung field. The chest CT scan revealed consolidation with central low attenuation area in the right upper lobe and the right axillary lymphadenopathy. The patient underwent fiberoptic bronchoscopy which revealed the presence of an endobronchial lesion with near-total occlusion of the anterior segment in the right upper lobe, and also mucosal ulceration of the subglottic trachea. Biopsy from both of the lesions revealed PTCL-NOS. An anti-HTLV-1 antibody testing was negative. Positron-emission tomographic-CT revealed marked increase in 18F-FDG uptake in the right hilar mass, mass in the bilateral femoral muscles, multiple subcutaneous nodules, and multiple lymph nodes (submandibular, cervical, axillary, mediastinal, hilar, inguinal, and para-aortic nodes). Additional biopsy of subcutaneous nodule in the left anterior chest wall also revealed PTCL-NOS. The patient received the standard dose of CHOP (cyclophosphamide, adriamycin, vincristine, and prednisone) every 3 weeks. After 4 cycles of chemotherapy, followup whole-body CT scan showed marked reduction of those lesions. DISCUSSION: It is rare for an endobronchial lesion to be the primary presentation of lymphoma, and an autopsy study reported that the prevalence of airway lesions in non-Hodgkin lymphoma (NHL) was 0.05%. PTCL constituted 4-25% of all NHLs, and PTCL-NOS was the most common subtype accounting for 25-35% of PTCLs. Patients with PTCL-NOS are usually adults with generalized disease. The 5-year overall survival and median survival period of PTCL-NOS patients was 31% and 20-24 months, respectively. Among PTCL patients, the most common primary site of presentation was lymph nodes followed by cutaneous, upper aerodigestive sites, spleen, and gastrointestinal tract. Our patient also had multiple lymphadenopathy, cutaneous and muscular lesions, however, to our knowledge, this may be one of the first instances where PTCL-NOS has been detected in the airway. Conclusion(s): This patient's presentation was unique in that endobronchial lesions of PTCL-NOS caused atelectasis as the primary presentation. This case highlights the importance of including lymphoma in the differential diagnosis of endobronchial lesion.