A 65-year-old Caucasian gentleman, with a history of ischaemic heart disease, presented to ENT with a two-month history of hoarseness. Physical examination was unremarkable, with no lymphadenopathy or organomegaly. Investigations showed a white blood count of 5.7 × 109/L, haemoglobin of 148 g/L, platelets of 213 × 109/L, and a normal serum lactate dehydrogenase level of 192 U/L.\nIndirect laryngoscopy showed vocal polyps, which were excised. Histology showed a monotonous population of lymphoid cells within the lamina propria of mucosal polyps, with slightly irregular nuclear contours and inconspicuous to clear cytoplasm. Immunohistochemistry was positive for CD45, CD20, and bcl2 and negative for CD5, CD23, bcl6, CD138, CD43, CD10, and cyclin D1 (), consistent with a diagnosis of extranodal marginal zone (low-grade) non-Hodgkin lymphoma.\nStaging with computed tomography and bone marrow aspiration and biopsy showed no evidence of disease involvement outside of the larynx.\nThe patient was managed with involved field radiotherapy to the larynx with clinical resolution of symptoms and no radiological evidence of residual disease. He remains alive and well 5 years later.
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.