An 82-year-old Caucasian lady known to suffer from ischaemic heart disease, hypertension, and mild renal impairment was admitted to hospital in November 2010 complaining of a 3-month history of increasing facial pain and difficulty breathing. She had previously undergone surgery for a complicated diverticular abscess two years prior to this presentation and this had resulted in a defunctioning colostomy. Physical examination showed no lymphadenopathy or organomegaly. Indirect laryngoscopy showed a mass at the base of the tongue, involving the epiglottis and causing airway obstruction. The patient underwent an emergency tracheostomy to secure the airway. Investigations showed a white blood count of 11.3 × 109/L, haemoglobin of 104 g/L, platelets of 296 × 109/L, and a normal serum lactate dehydrogenase level of 209 U/L. Staging with computed tomography only confirmed disease in the laryngeal area () and did not show any thoracic, abdominal, or pelvic lymphadenopathy. Bone marrow aspiration and biopsy showed no evidence of bone marrow involvement.\nBiopsy of the laryngeal mass showed that the mucosa and submucosa were infiltrated by large atypical lymphoid cells with irregular nuclear outlines and multiple prominent nucleoli. The cells were positive for CD45 and CD20 and negative for CD3 and bcl2 on immunohistochemistry (). These findings were consistent with a diagnosis of diffuse large B-cell (high-grade) 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).\nThe patient was administered three cycles of R-CEOP (rituximab, cyclophosphamide, etoposide, vincristine, and prednisone) followed by involved field radiotherapy. Anthracyclines were avoided in view of a decreased left ventricular ejection fraction of 40% on echocardiography performed at diagnosis.\nThe first cycle of chemotherapy was complicated by an acute coronary syndrome, from which the patient recovered with supportive care. The tracheostomy was removed following the second cycle of cytotoxics and the patient 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.