A case of anaplastic large cell lymphoma of skeletal muscle

Anaplastic large cell lymphoma (ALCL) is a high grade non‐Hodgkin lymphoma (NHL) that is comprised of the malignant proliferation of large lymphoid cells, which express CD30. Primary ALCL of the skeletal muscle is extremely uncommon. A 51‐year‐old Japanese female presented at our hospital with a 2‐month history of severe pain and swelling of the right leg. A gallium‐67 SPECT/CT scan showed a large mass involving the skeletal muscles from the gluteus to femoris. A biopsy of the mass demonstrated diffuse infiltration of medium and large neoplastic cells with round or lobulated hyperchromatic pleomorphic nuclei. A subset of Reed‐Sternberg‐like cells was also identified. Immunohistochemically, the neoplastic cells were strongly positive for CD4 and CD30, but negative for CD3, CD8, anaplastic lymphoma kinase (ALK), CD20, CD79α, CD21 and CD23. Based on the histological examination, this patient was diagnosed to have ALK‐negative ALCL of the skeletal muscle. Further studies are needed to clarify the biological behavior of primary skeletal muscle ALCL.     

结外NK/T细胞淋巴瘤发生的分子机制研究进展

结外NK/T细胞淋巴瘤(extranodal NK/T cell lymphoma,ENKTCL) 是我国继弥漫大B淋巴细胞淋巴瘤之后第二常见的非霍奇金淋巴瘤(non-hadgkin lymphoma,NHL).结外NK/T细胞淋巴瘤恶性程度高、临床进展快、预后差,对其发病机制的研究尤为重要.本文综述了近年来结外NK/T细胞淋巴瘤发生的分子机制研究进展,主要涉及该淋巴瘤的常见基因突变、NF-κB信号通路及JAK-STAT信号通路的主要激活机制.     

MALT lymphoma in children: a report from the NHL-BFM Study Group

Marginal zone lymphomas of MALT type comprise a considerable group of indolent B-cell non-Hodgkin lymphoma (NHL) in adult patients. In childhood, however, these tumors are extremely rare, as nearly all pediatric patients have aggressive NHL. Among 2,703 children and adolescents registered into the prospective multicenter NHL-BFM treatment studies since 1986, only 4 patients (0.1%) displayed features of MALT lymphoma. These tumors were localized in the stomach, breast, lower lid, and conjunctiva, respectively and they were associated with H. pylori infection in two patients. All children are alive but long-term follow-up will be mandatory to assess the behavior of MALT lymphoma in this age group.     

Prognostic factors for primary gastrointestinal lymphoma

The gastrointestinal tract is a common primary extranodal site for non-Hodgkin's lymphoma. There is however no uniform consensus on its pathological classification, clinical staging system and management. This paper reports the experience in the management of 425 Chinese patients with primary gastrointestinal lymphoma in Hong Kong from January 1975 to June 1993. There were 230 (54 per cent) males and 195 (46 per cent) females. Their median age was 53 years. The primary sites were: the esophagus in three (1 per cent), stomach in 238 (56 per cent), small intestine in 131 (31 per cent) and large intestine in 53 (12 per cent). According to the Working Formulation, there were 20 (4.7 per cent) small lymphocytic, 10 (2.4 per cent) follicular small cleaved cell, 15 (3.5 per cent) follicular mixed, five (1.2 per cent) follicular large cell, 40 (9.4 per cent) diffuse small cleaved cell, 50 (12 per cent) diffuse mixed, 181 (43 per cent) diffuse large cell, 30 (7.1 per cent) immunoblastic, five (1.2 per cent) lymphoblastic, 10 (2.4 per cent) diffuse small non-cleaved cell and 50 (14 per cent) unclassifiable lymphoma. Immunophenotyping was performed in 199 (47 per cent) patients: 90 per cent B-cell, 7 per cent T-cell and 3 per cent uncertain. According to a Manchester system, 81 (19 per cent) patients had stage I disease, 44 (10 per cent) stage II, 85 (20 per cent) stage III and 215 (51 per cent) stage IV. B symptoms were present in 275 (65 per cent) patients and bulky disease in 104 (25 per cent). Surgery followed by chemotherapy was the mainstaly of treatment. Of the 408 patients treated, 63 per cent had a complete remission with relapse rate of 42 per cent. For those with complete remission, 47 per cent were free from disease at 5 years. The overall median survival of all patients was 45 per cent at 5 years. Multivariate analysis revealed that significant independent prognostic factors predicting better survival were young age of <60 years, low grade histology, stage I and II disease and absence of bulky tumour. For gastric lymphoma, aggressive surgery did not significantly improve their outcome. Chemotherapy appears to play an important role in the management of gastrointestinal lymphoma. Better classification of the primary gastrointestinal lymphoma and more refined stratification of the patients according to the prognostic variables my allow individualization of treatment. Prospective randomized studies are essential to define the relative roles of surgery, chemotherapy and radiotherapy.     

Autopsy case of lymphoplasmacytic lymphoma with a large submucosal tumor in the stomach

An autopsy case of lymphoplasmacytic lymphoma with a large submucosal tumor in the stomach is presented. The patient was a 77-year-old woman with gastric lymphoma associated with Waldenstrom's macroglobulinemia of IgM-lambda type. Diagnosis was initially mucosa-associated lymphoid tissue (MALT) lymphoma of the stomach, because gastric biopsy specimens showed epitheliotropic proliferation (lymphoepithelial lesion) of the lymphoma cells. Postmortem examination revealed a large gastric lymphoma with metastatic foci in the esophagus, larynx, trachea, lungs, spleen and lymph nodes. The bone marrow was also involved. Lymphoma cells consisted of small lymphocytoid cells occasionally admixed with blast-like large cells and a large number of plasmacytoid or plasma cells. Centrocyte-like cells were not found. Lymphoepithelial lesions were not conspicuous in autopsy specimens. Immunohistochemically, lymphoma cells reacted with CD20, CD45, CD79a, anti-IgM, anti-lambda protein and anti-BCL-2, but not with CD5, CD10, CD23 or CD38. Based on these findings, the revised diagnosis of the present case was lymphoplasmacytic lymphoma, and it highlighted the differential diagnostic problem from marginal zone B-cell lymphoma of MALT type.     

结外鼻型NK/T细胞淋巴瘤的研究进展

结外鼻型NK／T细胞淋巴瘤是鼻腔淋巴瘤中较为常见、恶性程度高且预后较差的病种。因此．了解其发生发展、临床表现、病理诊断、治疗及预后是非常重要的。     

Clinical Significance of Intranodal and Extranodal Growth in Lymph Node Metastases of Non-Small Cell Lung Cancer

In non-small cell lung cancer with mediastinal lymph node metastasis, intranodal growth is regarded as prognostically more favourable than extranodal growth. We evaluated the clinical implications. Mediastinal lymph node metastases removed at mediastinoscopy and/or surgery were classified as intranodal, extranodal or indefinite. “Minimal N2 disease” denoted a solitary, intranodal metastasis, “extranodal” at least one extranodal lymph node metastasis, and “indefinite” more than one intranodal or at least one indefinite metastasis. Although in patients with resected N2 disease, c. 21% of the nodal metastases were “indefinite”, survival was significantly better in minimal N2 disease than in the combined groups with extranodal and indefinite lymph node metastases. Of the metastatic nodes removed at mediastinoscopy, 75% were unsuitable for definite classification as only intranodal or extranodal. Only 1 of 49 patients had purely intranodal N2 disease. Thus, it was seldom feasible to classify mediastinoscopic lymph node involvement as intranodal or extranodal, and this classification is unhelpful as regards decisions on thoracotomy. However, when nodal involvement in resected N2 disease was limited to a single node with intranodal growth, the prognosis was better than in patients with extranodal disease or more than one intranodal metastasis or indefinite nodes.     

CD56‐negative extranodal nasal type NK/T‐cell lymphoma

Extranodal natural killer (NK)/T‐cell lymphoma, nasal type, is a rare lymphoma that occurs predominantly in Asian adults. In this report, we describe the clinical and pathologic features of an unusual aggressive lymphoid neoplasm in a child and review the literature on NK/T‐cell lymphoma in children. The patient was a 4‐year‐old Native American male with facial swelling, lymphadenopathy, and fevers. Biopsy demonstrated neoplastic lymphoid cells that expressed CD3, CD8, TIA‐1, and EBV‐encoded RNA without CD56. The patient failed multiagent chemotherapy and died of therapy‐related complications. This case represents an extranodal NK/T‐cell lymphoma, nasal type, with an unusual lack of CD56. Pediatr Blood Cancer 2010;55:186–189. © 2010 Wiley‐Liss, Inc.