A 60-year-old Caucasian female presented with a 2-month history of progressive dysphagia, hoarseness, and globus sensation, accompanied by a 10-pound weight loss over this period. At time of presentation, she also reported exertional dyspnea. She denied nausea, vomiting, fever, hemoptysis, or hematemesis. Her only other medical comorbidity was hypertension, which was well controlled. She denied smoking, drinking, or related family history of malignancy.\nA CT neck with contrast revealed a large sessile, isointense mass on the laryngeal surface of epiglottis measuring 4.7 cm in its greatest dimension, with no submucosal extension or cervical lymphadenopathy (see ).\nThe patient was subsequently referred to the otolaryngology service for further evaluation. On examination she had a muffled voice and minimal inspiratory stridor without respiratory distress. On palpation, she had normal external laryngeal landmarks and no palpable neck masses. Using a tongue depressor, a large mass could be seen in the oropharynx behind the upper edge of epiglottis. Flexible fiberoptic examination confirmed an exophytic mass in oropharynx that was sessile to the laryngeal surface of the epiglottis and occupied most of the oropharynx. Just beyond the mass, the hypopharynx and laryngeal inlet were normal in appearance (see ).\nA transoral biopsy of the mass was conducted under local anesthesia, since it was easily visualized through her mouth and the mass was stable without glottis obstruction. The biopsy revealed an unusual B-cell lymphoma subtype (see ), based on immunostaining and in situ hybridization ().\nCT of chest, abdomen, and pelvis was negative for additional lymphadenopathy. Bone marrow aspiration and biopsy showed no evidence of bone marrow involvement. These findings were consistent with a diagnosis of diffuse large B-cell (high grade; also she has intermingled low grade areas) non-Hodgkin lymphoma of the larynx, stage IE, with an R-IPI score of 1 (age > 60, ECOG 0–2, normal LDH of 209 U/L, 0-1 extranodal sites, and stage I/II disease). The patient was subsequently treated with definitive chemotherapy and radiation.\nShe was given three cycles of R-CEOP (rituximab, cyclophosphamide, etoposide, vincristine, and prednisone) followed by involved field radiotherapy to the neck with 40 Gy radiation. The patient was seen in clinic 8 weeks later with complete resolution of dysphonia and dysphagia. Repeat fiberoptic examination demonstrated complete resolution of the epiglottic mass without evidence of recurrent or residual tumor. She was seen again four months later with normal voice and remained disease free on examination (). Her PET scan 3 months after treatment was negative.
We report a case of a 43-year-old woman with no particular pathological history. She presented with progressive dyspnea which has evolved towards an inspiratory bradypnea that worsened until she ultimately required an emergency tracheotomy. Laryngoscopic examination showed an obstructive mass in the right infraglottic area. A punch biopsy of larynx tumor and excision of left cervical adenopathy were performed. The anatomopathological and immunohistochemical examination revealed a blastoid variant of mantle cell lymphoma CD20+, CD5+, cycline-D1+, LCA+, BCL6+/−, MUM1+/−, CD3−, CD30−, ALK−, and Ki67 at 90%. The patient reported general signs such as weight loss and nocturnal sweats in the last six months. The ECOG (Eastern Cooperative Oncology Group) performance status was 2, and the clinical examination revealed a tumoral syndrome made of bilateral cervical lymphadenopathy. Blood test showed leukocytosis at 11300/mm3 with hyperlymphocytosis at 6200/mm3 predominantly of small mature lymphocytes and a high LDH level at 779 U/l (the upper normal level limit of the laboratory is 245U/l). The rest of the biological assessment was normal. The 18-FDG positron emission tomography (PET) scan showed an over and under diaphragmatic lymph node involvement and bilateral pathological pulmonary nodules (). A pathological laryngeal hypermetabolism with cricoid cartilage involvement (SUVmax = 15) was noticed (), and bone marrow biopsy was negative. The patient has been staged IVB (Ann Arbor Classification), and the Mantle Cell Lymphoma International Prognostic Index (MIPI) score was 6 (high-risk group). She was treated by immunochemotherapy with 4 cycles of R-DHAOx (rituximab, high-dose aracytine, oxaliplatin, and dexamethasone), followed by autologous hematopoietic stem cell transplantation (ASCT). The therapeutic evaluation by PET scan shows a complete remission (), and it was decided to continue with maintenance therapy with rituximab every 2 months, but it was refused by the patient. At the last visit, 18 months of ASCT, the patient was always disease free.