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.
We report the case of a 70-year-old North African man who, two months prior to medical examination, presented with mild laryngeal respiratory distress that worsened until he ultimately required a tracheotomy. A direct laryngoscopy had been performed, revealing a subglottic, submucosal lump. A biopsy revealed a mantle cell type B NHL with the following antigen constellation: cluster of differentiation (CD)5+, CD20+, CD23-, cyclin D1+. Importantly, a physical examination and computed tomography (CT) scans showed a lack of lymph node involvement, establishing the larynx as the primary site of the neoplastic lesion.\nThe patient’s history and physical examination found no comorbidities. The patient did not present any B symptoms and had a performance status 3 as measured using the World Health Organization performance scale. During a follow-up four weeks after his initial admission, a physical examination found laterocervical lymph nodes. An investigation into possible tumoral extension by cervical, thoracoabdominal and pelvic CT scans showed a subglottic tumor (Figures and ) and cervical lymph nodes.\nA bone marrow biopsy did not show any anomalies. A blood workup showed he had normal red blood cell sedimentation rate and lactate dehydrogenase levels.\nThe patient, who has been staged IIEAa (Ann Arbor Classification), received one course of rituximab-cyclophosphamide, doxorubicin, vincristine and prednisone but unfortunately died of unknown causes. No autopsy was performed in compliance with the family’s wish.