眼眶原发性MALT淋巴瘤临床病理观察

目的 探讨眼眶原发性黏膜相关淋巴组织结外边缘区B细胞淋巴瘤(MALT淋巴瘤)临床病理特点及影响预后因素。方法 复习7例眼眶原发性MALT淋巴瘤临床病理资料及病理切片,并用免疫组化S—P法重新标记瘤细胞。结果 5例位于泪腺及结膜,2例位于眼肌,7例均为IE期。瘤细胞以边缘区B细胞为主,混有少量浆细胞及母细胞化的淋巴细胞。位于泪腺及结膜者可见淋巴上皮病变及生发中心植入现象。瘤细胞表达CD45、CD20、IgM、bcl-2及单一的轻链,CD3、CD5、CD10、cyclin D1、CK均阴性。7例均作手术治疗;1例辅以放疗,2例结合化疗。7例随访4～139个月,均存活。结论 眼眶原发性MALT淋巴瘤好发于泪腺及结膜,预后与临床分期有关,与治疗方法关系不大。     

Malignant lymphoma of mucosa-associated lymphoid tissue

Lymphomas of the gastrointestinal tract, salivary glands, lung and thyroid are grouped together as tumours arising in mucosa-associated lymphoid tissue. The great majority of them are of B-cell origin but distinctive T-cell lymphomas are also recognized in the gastrointestinal tract. These lymphomas tend to remain localized for prolonged periods but, whereas the B-cell group respond favourably to local therapy, the T-cell group are associated with severe morbidity and their overall prognosis is extremely poor. Accepted histological classifications of non-Hodgkin's lymphomas are difficult to apply to these tumours. In this paper their morphological features are reviewed; recent findings based on immunohistochemistry and DNA analysis are presented; and the biological behaviour of these tumours is discussed insofar as they offer insight into mucosal immunological mechanisms.     

Low-grade mucosa-associated lymphoid tissue lymphoma involving the kidney: report of 3 cases and review of the literature

Low-grade B-cell lymphoma of mucosa-associated lymphoid tissue involving the kidney is rare. We report a series of 3 cases. The first case occurred in an 83-year-old woman who presented with back pain. The second case was a 53-year-old man with a history of sarcoidosis who was found, in the course of evaluation of sarcoidosis, to have a right renal mass. The third case occurred in a 72-year-old man who had a history of periorbital mucosa-associated lymphoid tissue lymphoma and had been treated with surgery and radiation 1 year prior to this presentation. Histologically, all 3 patients showed infiltrate of uniform small-to-medium-sized lymphocytes with irregular nuclear contours and abundant cytoplasm resembling centrocytes or monocytoid lymphoid cells. The first patient received chemotherapy without complications. The second patient underwent a partial nephrectomy and was asymptomatic at the subsequent follow-up. The third patient developed a pulmonary embolism following nephrectomy, and further follow-up is not available.     

Primary hepatic mucosa-associated lymphoid tissue lymphoma: case report and literature review

Background: The prevalence of primary hepatic mucosa-associated lymphoid tissue (MALT) lymphomas is extremely low. Here, we describe a case of this disease misdiagnosed as hepatocellular carcinoma (HCC) and review relevant literature to prevent future misdiagnoses. Case presentation: a 58-year-old woman complained about abdominal pain for more than four months. About two months prior, she came to our hospital with elevated levels of HBV DNA and positive HBsAg and HBcAb. After two months of entecavir treatment, HBV DNA decreased to a normal level. She returned to the hospital with worsened abdominal pain for over a month. Magnetic resonance imaging and systemic positron emission tomography-computed tomography identified two nodes in the liver, and she was diagnosed with HCC. The patient then underwent a laparoscopic hepatectomy. Microscopic examination showed a diffuse infiltrate of small-to-medium-sized lymphocytes and lymphoepithelial lesions. Immunohistochemical staining showed that most of the lymphoid cells were strongly positive for CD20, CD79a, BCL2, IgM and weakly positive for IgD, while negative for CD3, CD10, BCL6, MUM1, CD43, CD5, cyclin D1, CD23, CD30, and PD1. The Ki-67 index of lymphoid cells was 5%. Further pathologic analysis confirmed the diagnosis of primary hepatic MALT lymphoma. The patient received antiviral treatment and recovered well with no sign of relapse for 17 months. Conclusions: Primary hepatic MALT lymphoma is an uncommon disease that is difficult to diagnose and has no widely accepted treatment. Surgical resection is a good choice for both diagnosis and local therapy, and strict follow-up of the patient is essential.     

Localized lymphoid hyperplasia of the rectum representing progressive transformation of the germinal center. A report of two cases

Histologically, benign lymphoid hyperplasia (BLH) of the rectum is usually characterized by large lymphoid follicles with active germinal centers, and a narrow surrounding mantle zone and marginal zone. We present two cases of BLH of the rectum demonstrating progressive transformation of the germinal center (PTGC). The patients were 50- and 71-year-old Japanese women. Colonoscopy demonstrated small sessile polyps in both cases. The resected specimen contained numerous lymphoid follicles with active germinal centers and a portion of the lymphoid follicles exhibited PTGC. The area showing PTGC contained a few large lymphoid cells resembling lymphocytic and histiocytic Reed-Sternberg cells of nodular lymphocyte-predominant Hodgkin lymphoma. These PTGC contained small- to-medium clusters of epithelioid cells in both cases. In situ hybridization studies demonstrated scattered Epstein-Barr virus (EBV)-encoded small RNA-positive medium and large lymphoid cells and crypt epithelium in both lesions. EBV may be involved in the etiology of a subset of BLH of the rectum. However, reactivity of lymphoid cells for EBV has been reported in lymphoid tissues in a high percentage of "normal" individuals. The etiology of BLH of the rectum remains unclear.     

Primary low-grade B-cell lymphoma of the conjunctiva: a mucosa-associated lymphoid tissue type lymphoma

We describe the clinical presentation, morphology, immunophenotypic features and molecular biology of seven cases of conjunctival lymphoid infiltration. In five cases there was either immunophenotypic or molecular evidence of B-cell lymphoma. Each of these cases showed the morphological, immunophenotypic and molecular feature of low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type. The findings suggest that most conjunctival lymphomas are of this type and explains their uniformly favourable prognosis.     

Primary hepatic lymphoma of mucosa-associated lymphoid tissue type: a case report with cytogenetic study

Primary hepatic lymphoma of mucosa-associated lymphoid tissue type is extremely rare. Only 38 cases have been reported to date. A case of a 59-year-old man with Helicobacter pylori-resistant gastric ulcers and Buerger disease who was followed up since 1999 is reported. A 2-cm hepatic nodule was incidentally found during partial gastrectomy and corresponded to mucosa-associated lymphoid tissue-type lymphoma without underlying liver disease. Molecular studies showed a clonal immunoglobulin heavy-chain gene rearrangement. Investigations for the mucosa-associated lymphoid tissue lymphoma-associated translocations t(11;18) and t(14;18), as well as the t(3;14)(q27;q32), were negative, whereas trisomy 3 and trisomy 18 were detected.     

Primary low-grade gastric mucosa-associated lymphoid tissue (MALT) lymphoma with polypoid appearance. Polypoid gastric MALT lymphoma: A clinicopathologic study of eight cases

In the current study, we report eight cases with primary low-grade gastric mucosa-associated lymphoid tissue (MALT) lymphoma endoscopically characterized by polypoid lesions in order to highlight their clinicopathologic significance. Four patients were male, their ages ranging from 40 to 78 years old. The resected specimens revealed a histology of low-grade MALT lymphoma characterized by dense lymphocytic infiltration predominantly in the submucosa and a relatively monotonous proliferation of centrocyte-like cells with reactive follicles and infrequent lymphoepithelial lesions. The tumor cells were of CD5-, CD10-, CD20+, BCL2+ and cycline D1- phenotype, and showed a monoclonal rearrangement of immunoglobulin heavy chain genes in the five of six cases examined. Interestingly, Helicobacter pylori (H. pylori) was detected in three (37.5%) of the eight patients, which was significantly lower than previous reports. Two of the H. pylori-positive cases initially underwent H. pylori eradication, but showed no change in their lymphomas after the cure of H. pylori infection. The clinicopathologic findings of the present cases appeared to closely resemble those of colorectal MALT lymphoma with a polypoid appearance and few association of H. pylori infection in their pathogenesis. These gastric polypoid cases may merit separate consideration because of the therapeutic problems they pose.     

胆囊粘膜相关型淋巴瘤4例临床病理观察及文献复习

目的：探讨胆囊粘膜相关型淋巴瘤的诊断、鉴别诊断及其发病机制。方法：手术切除标本常规石蜡切片和免疫组化ＡＢＣ法标记。结果：４例胆囊ＭＡＬＴ－ＭＬ均 反应性滤泡。免疫表型：４例ＬＣＡ、Ｌ２６均呈阳性ＩｇＭ／ｋ３例,ＩｇＧ／λ１例,ｂｃｌ－２阳性３例。结论：胆囊ＭＡＬＴ－ＭＬ大多为低度恶性,以手术治疗为主。临床应积极控制胆囊慢性炎性病变,防止产生获得性ＭＡＬＴ,进一步发展为ＭＡＬＴ－ＭＬ。     

Primary malignant lymphoma of the thyroid-a tumour of mucosa-associated lymphoid tissue: review of seventy-six cases

Seventy-six cases of primary thyroid lymphoma have been reviewed employing the Kiel classification. These lymphomas are almost entirely of follicle centre cell origin, occur predominantly in elderly females, and are frequently associated with lymphocytic thyroiditis or Hashimoto's disease. The overall prognosis is variable, with long-term survival in a substantial number of cases following thyroidectomy and radiotherapy. Results of histological examination including immunoperoxidase studies are described. It is proposed that thyroid lymphoma represents a neoplasm of mucosa-associated lymphoid tissue (MALT), and the implications of this are discussed.     

CD5-positive mucosa-associated lymphoid tissue (MALT) lymphoma: a clinicopathologic study of 14 cases

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a B-cell neoplasm that is typically CD5 negative. We describe the clinicopathologic, immunophenotypic, and cytogenetic features of 14 cases of CD5+ MALT lymphoma. There were 9 men and 5 women (median age, 68 years; range, 34-87 years). MALT lymphoma was initially diagnosed in salivary glands (n = 4), nasopharynx (n = 2), and 1 case each in conjunctiva, thyroid, stomach, colon, skin, lung, kidney, and retroperitoneum. Two patients had localized disease; 9 had disseminated disease with generalized lymphadenopathy (n = 8), multifocal lymphoma (n = 6), or bone marrow involvement (n = 5). No staging information was available for the remaining patients. None presented with B symptoms, splenomegaly, cytopenias, lymphocytosis, monoclonal gammopathy, or elevated serum lactate dehyrogenase. Serum β2-microglobulin was elevated in 6. Morphologically, the neoplasms had features typical of MALT lymphoma being composed of small- to medium-sized cells with round to slightly irregular nuclear contours and moderate amount of cytoplasm. Lymphoepithelial lesions were noted in 4 cases. CD5 was positive in all cases by immunohistochemistry (n = 12) and/or flow cytometry (n = 11). All cases assessed were negative for cyclin D1 (13/13) and CD10 (11/11). Conventional cytogenetics in 7 cases showed trisomy 3 in 3 and diploid in 4. With a median follow-up of 71 months (range, 2-131 months), overall survival at 5 years was 100%, although 5 patients required chemotherapy. Our results show that CD5 expression is rare in MALT lymphoma, and is often associated with nongastric disease and an increased tendency to present with disseminated disease. Overall survival is excellent with appropriate therapy.     

Primary hepatic B-cell lymphoma of mucosa-associated lymphoid tissue.

Mucosa-associated lymphoid tissue (MALT) lymphomas are low-grade B-cell lymphomas that occur in a variety of extranodal sites but rarely as a primary hepatic lymphoma. We describe the histological findings, immunophenotype, and immunohistochemistry of one such lymphoma found incidentally in a 69-year-old woman. The lymphoid infiltrate invaded the liver in a serpiginous configuration with entrapment of nodules of normal liver. Reactive follicles were surrounded by intermediate-sized lymphoid cells with slightly irregular nuclei and pale cytoplasm. Only a few scattered lymphoepithelial lesions were identified since most of the bile ducts were destroyed. The immunophenotype determined by flow cytometry identified the lymphoid cells as being CD19, CD20 positive and exhibiting lambda light chain restriction. CD5, CD10, and CD23 were negative. Immunohistochemistry showed the neoplastic cells to be positive for CD20 (L-26) and bcl-2. The reactive follicles were negative for bcl-2. CD3 showed only a few scattered T cells. Cyclin D1 did not stain the neoplastic cells. Cytokeratin (AE1/AE3) highlighted the lymphoepithelial lesions and residual bile ducts. MALT lymphomas need to be recognized and distinguished from other B-cell lymphomas, particularly mantle cell lymphomas, because of the difference in behavior and treatment.     

Ocular adnexal marginal zone lymphoma of mucosa-associated lymphoid tissue

Ocular adnexal lymphomas are a group of heterogeneous neoplasms representing approximately 1–2% of non-Hodgkin lymphomas and 8% of extranodal lymphomas. The incidence of primary ocular adnexal lymphoid tumors has raised over the last decades, and this could be probably attributed to the more sophisticated diagnostic techniques. Due to the wide spectrum of clinical manifestations, ocular tissue biopsy is important in order to set a precise diagnosis based on histological, immunophenotypical and, in some cases, molecular findings. The most common subtype, which may account for up to 80% of primary ocular adnexal lymphomas, is extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissue. This lymphoma is usually asymptomatic in the early phase of the disease causing a delay in the final diagnosis and prompt therapy. The pathogenesis of a proportion of these tumors has been linked to chronic inflammatory stimulation from specific infectious factors (e.g., Chlamydia psittaci) or to autoimmunity. The further improvement in diagnostic methods and the further understanding of the pathogenesis of ocular adnexal EMZL may contribute to the establishment of a more successful multidisciplinary therapeutic planning.     

A case of primary mucosa-associated lymphoid tissue lymphoma of the vagina

We report the first case of primary mucosa-associated lymphoid tissue (MALT) lymphoma of the vagina, the diagnosis of which is supported by genetic and immunophenotypic studies. A 65-year-old, para 2 woman presented to our hospital in July 1997 with a history of prolonged vaginal discharge. Although cytologic examination suggested possible malignancy, a biopsy of the vaginal wall was diagnosed as chronic inflammation. In June 2000, she underwent gynecologic examination because of anuria. Excisional biopsy revealed subepithelial infiltration of atypical lymphoid cells that stained for CD20, CD79a, and BCL-2; stained weakly for IgM; and did not stain for CD3, CD5, CD7, CD10, CD56, CD23, and IgD, suggesting marginal zone B-cell lineage. Monoclonality was detected by Southern blot analysis, and this patient was finally diagnosed as having primary MALT lymphoma of the vagina. She received 3 cycles of chemotherapy (THP-COP) and concurrent radiation to the whole pelvis. The patient is alive and well 40 months after treatment. Because the vagina is one of the mucosa-associated tissues, MALT lymphoma, though rare, must be included in the differential diagnosis of the vaginal neoplasms.     

涎腺粘膜相关淋巴瘤临床与病理研究

目的 探讨涎腺粘膜相关淋巴瘤的病理诊断特征及发病机制,方法 分析临床资料,利用ＨＥ染色,白细胞共同抗原,ＣＤ２０,ＣＤ４５ＲＯ,上皮膜抗原的ＳＰ法免疫组化及电镜观察了２７例涎粘膜相关淋巴瘤,结果 男性２４例,女性３例,平均年龄５３．４５岁,其中腮腺１２例,颌下腺１５例,涎腺粘膜相关淋巴瘤是由弥漫性中心细胞（ＣＣＬ）细胞组成,并有“淋巴上皮病变”。ＣＣＬ细胞呈ＣＤ２０阳性,ＣＤ４５ＲＯ阴性,电镜下瘤     

Spontaneous regression of intraoral mucosa-associated lymphoid tissue lymphoma: molecular study of a case

Mucosa-associated lymphoid tissue (MALT) lymphoma presentation in the oral cavity is very rare. Reported herein is a case of intraoral MALT lymphoma of the minor salivary gland in a 70-year-old woman with Sjogren's syndrome. Unexpectedly, a spontaneous clinically and histologically confirmed regression occurred 1 month after the tumor biopsy for diagnosis. Considering that salivary MALT lymphoma is associated with Sjogren's syndrome and that the chronic inflammation caused by Sjogren's syndrome persisted, it is hypothesized that the tumor clone might be present in the regressed lesion. Minimal residual tumor clone identical with the primary lesion was detected using the polymerase chain reaction (PCR) clonality assay for immunoglobulin heavy chain gene (IgH) rearrangement. No recurrence was clinically evident 38 months after the diagnosis. Spontaneous regression of MALT lymphoma should be examined at the molecular level in addition to clinical and histological evaluations. When minimal residual disease is detected, close follow up is necessary for early detection of the tumor relapse.     

干燥综合征与粘膜相关型淋巴瘤临床病理分析

目的：探讨干燥综合征（ＳＳ）病理分析对诊断ＳＳ的意义、Ｍｉｋｕｌｉｃｚ病与粘膜相关淋巴组织（ＭＡＬＴ）型淋巴瘤的关系。方法：回顾性分析１５９例经病理证实的ＳＳ,对疑为ＭＡＬＴ型淋巴瘤的６例用免疫组化ＡＢＣ法标记,使用抗体ＬＣＡＬ－１、Ｌ２６及免疫球蛋白ＩｇＧ、ＩｇＡ、ＩｇＭ、ＩｇＤ、ＩｇＥ和к、λ。结果：６例可疑病例中有４例发生ＭＡＬＴ型淋巴组化ＬＣＡ、Ｌ２６、ｂｃｌ－２、ＩｇＭ、к和ＩｇＭ、λ呈