A 58-year-old Chinese female who was a retired doctor presented in October 2010 with a history of progressive hoarseness for about 10 months. The patient did not suffer from fever, night sweat, or weight loss. Multiple laryngoscopy examinations in other hospitals revealed severe hypertrophy of left ventricular band. As Figure showed, the left ventricular band and laryngeal ventricle bulged with smooth surface, covering most of the left vocal cord. Her past history was remarkable for rheumatoid arthritis and Sjogren syndrome for many years. Her family and psychosocial history was unremarkable. Partial resection of tumor by laser in another hospital was performed and hoarseness relieved significantly. The postoperative pathology demonstrated neoplastic cells invaded adjacent epithelial cells, forming lymphoepithelial lesion (Figure ). The immunohistochemical results showed CD3 and CD20 (focally+), κ(diffusely +), λ(−), Ki-67(5–10%). Therefore, the patient was diagnosed as non-Hodgkin MALT marginal zone B-cell lymphoma.\nOn physical examination, no enlarged superficial lymph nodes were palpated. Laboratory investigations showed a normal complete blood cell count and a generally normal serum biochemical profile. Lactate dehydrogenase was normal. β-2 Microglobulin was 2.47 mg/L (0.7–1.8 mg/L). Erythrocyte sedimentation rate was 41 mm/h (10–20 mm/h). The subsequent thoracic computed tomography (CT) and ultrasound for bilateral neck, armpit, and groin did not reveal any enlarged lymph node. Gastroscopy showed chronic nonatrophic gastritis. The bone marrow aspiration did not find neoplastic involvement. Thus, an MALT lymphoma of larynx, Ann Arbor stage IEA, International Prognostic Index score 0, was diagnosed.\nOne month after surgery, the patient was admitted to our department. Laryngoscopy demonstrated that there was a red mucosa uplift in the left ventricular band, and bilateral vocal cords were normal. The throat CT scan also revealed the front part of left ventricular band was more hypertrophy than its right counterpart (Figure ). All these findings were consistent with residual tumor. Then irradiation of 7-field intensity modulated radiotherapy technique with a dose of 30.6 Gy/17f to the tumor and 25.5 Gy/17f to the related lymphatic drainage area was carried out. The side effects were mild. The repeated laryngoscopy when she finished radiotherapy showed the tumor disappeared. Radiotherapy alone achieved a complete remission (CR) response. Until now, her condition remains stable and the DFS has reached to 4 years.
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.