Three cases of diffuse large B-cell lymphoma presenting as primary splenic lymphoma

Primary splenic lymphoma (PSL) is often defined as generalized lymphoma with splenic involvement as the dominant feature. It is a rare disease that comprises approximately 1% of all malignant lymphomas. We investigated three cases of non-Hodgkin's splenic lymphoma that had different clinical features on presentation. The patients' survival times from diagnosis ranged from 59 to 143 months, without evidence of relapse after splenectomy and chemotherapy, with or without radiotherapy. This data suggest that PSL is potentially curable. Further studies are needed to evaluate the impact that different treatment modalities without splenectomy have on patient survival.     

Primary large-cell lymphoma of the thymus: a diffuse B-cell neoplasm presenting as primary mediastinal lymphoma

Primary mediastinal nonlymphoblastic non-Hodgkin's lymphoma (NLNHL) has distinct clinical, histologic (diffuse large-cell morphology, often with sclerosis and clear cytoplasm), and immunohistochemical features (predominantly B-cell lineage, usually immunoglobulin-negative), which suggest origin from a unique B-cell population. The thymus has a resident population of B cells with a unique immunophenotype, and can be involved by primary mediastinal NLNHL, in some cases selectively. Fifteen cases of NLNHL involving the thymus were studied by paraffin-section immunohistochemistry using antibodies to formalin-resistant epitopes of B cells (4KB5 [CD45RA] and L26 [CD20]) and T cells (L60 [CD43] and UCHL1 [CD45RO]). All were diffuse large-cell or immunoblastic lymphomas with sclerosis, and were also similar to primary mediastinal NLNHL in clinical features. Neoplastic cells stained with L26 in all but one case, which stained with 4KB5 and an antibody to a leukocyte-common antigen (PD7/26 [CD45RB]), and were uniformly nonreactive with L60 (with one exception) and UCHL1. Intermingled small lymphocytes were uniformly L26-negative and positive for T-cell markers, even in one case with atypia suggesting a lymphoma of mixed morphology. These findings demonstrate that primary thymic and primary mediastinal NLNHL are similar B-lineage neoplasms, and support previous suggestions that both may originate in thymic B cells.     

肺黏膜相关淋巴组织边缘区B细胞淋巴瘤及良性淋巴组织增生性疾病的临床病理分析

目的 研究肺原发性黏膜相关淋巴组织边缘区B细胞(MALT)淋巴瘤及良性淋巴组织增生性疾病的临床病理形态、免疫组织化学表型和B细胞重链基因重排,比较肺MALT淋巴瘤和良性淋巴组织增生性疾病的差异.方法 回顾性的分析原发性肺MALT淋巴瘤13例,7例肺良性淋巴组织增生性疾病资料.对标本行常规HE染色,EnVision免疫组织化学染色(抗体包括AE1/AE3、CD20、CD79α、CD3、CD5、CD10、CD21、bel-2、bcl-6、cyclinD-1)及免疫球蛋白重链IgH基因重排检测.结果 13例肺MALT淋巴瘤,细胞成分多样,分别由不同比例的小淋巴细胞样细胞、中心细胞样细胞、单核样B细胞组成,常伴有浆细胞分化.肿瘤细胞以弥漫性和滤泡边缘区排列为主,常见反应性淋巴滤泡和滤泡中心的植入.肿瘤细胞呈串珠状直接侵犯肺泡间隔和沿支气管血管束向周边及肺膜扩散.MALT淋巴瘤中,均未见坏死.9例可见肿瘤细胞侵犯血管壁,6例可见胸膜累及,2例肺门淋巴结侵犯.9例肺MALT淋巴瘤可见淋巴上皮样病变,免疫组织化学显示上皮细胞内的淋巴细胞CD20阳性,CD3阴性.7例肺良性淋巴组织增生性疾病,2例可见淋巴上皮样病变,免疫组织化学显示,其淋巴上皮样病变内的淋巴细胞,部分CD20阳性,部分CD3阳性.9例肺MALT淋巴瘤进行了免疫球蛋白重链IgH基因重排,8例阳性;7例良性淋巴组织增生性疾病均为阴性.结论 肺MALT淋巴瘤在细胞组成和排列上与其他部位结外MALT淋巴瘤相同,肿瘤细胞呈串珠状直接侵犯肺泡间隔和沿支气管血管束向周边及肺膜扩散.在肺内淋巴上皮样病变常见于MALT淋巴瘤,并有助于诊断,但并非其特异性病变,一些肺的反应性淋巴组织增生也可出现,用免疫组织化学有助于区别两种病变.免疫球蛋白重链IgH基因重排可以帮助鉴别肺MALT淋巴瘤和良性淋巴组织增生性疾病.     

Cytomorphology of a case of primary cutaneous B-cell lymphoma

Primary cutaneous lymphoma designates a heterogenous group of disorders arising from skin T and B cells with no evidence of extra cutaneous disease at the time of diagnosis and six months thereafter. We report the cytomorphological features of a case of primary cutaneous lymphoma, B cell type in a 60 year old female presenting with multiple large bosselated red coloured swellings all over the scalp. Clinical examination revealed no other swelling or lymphadenopathy. On cytology a diagnosis of B-cell cutaneous lymphoid hyperplasia (B-CLH) was given, however cutaneous lymphoma could not be ruled out. On biopsy and immuno-histochemistry a diagnosis of primary cutaneous lymphoma B cell type was made. Patient was started on specific chemotherapy of lymphoma to which she responded completely. Here we highlight the cytomorphologic, histopathological and immunohistochemical features of this rare lesion with a particular emphasis on the diagnostic dilemma encountered on cytology.     

T-cell-rich B-cell lymphoma presenting as liver disease

We describe a series of eight cases of T-cell-rich B-cell lymphoma diagnosed on liver biopsy and collected over a period of 15 years. Of seven cases that were referred from elsewhere, in only one was the correct diagnosis of B-cell lymphoma suggested. Common errors included misdiagnosis as inflammatory disease on histology, and misinterpretation as T-cell lymphoma on immunohistochemistry. However, the cases had a distinct morphological appearance and immunohistochemical profile. They showed a lymphohistiocytic or granulomatous infiltrate, usually centred on portal tracts and containing abundant small T-cells and scanty B-cell blasts. All patients had an atypical clinical presentation which favoured non-neoplastic liver disease. In seven cases liver involvement represented Stage IV disease and in one case disease was confined to the liver consistent with a primary hepatic lymphoma. Despite combination chemotherapy, the prognosis was poor with no patients surviving beyond 15 months from diagnosis. We believe T-cell-rich B-cell lymphoma to be an under-recognized subset of non-Hodgkin's lymphoma that may mimic primary liver disease.     

Intravascular large B-cell lymphoma presenting as cutaneous panniculitis

In this report the case of intravascular large B-cell lymphoma (angiotropic lymphoma, intravascular angioendotheliomatosis, malignant endotheliomatosis) with panniculitis features is present. The diagnosis was based on finding of typical CD20 positive tumorous cells within capillaries, veins, and small arteries in the biopsy of subcutaneous adipose tissue. An accurate and early diagnosis together with combined chemotherapy resulted in good response and remission of the disease.     

A case of primary unilateral adrenal Burkitt-like large cell lymphoma presenting as adrenal insufficiency

Primary adrenal lymphoma is extraordinarily rare, in comparison with secondary adrenal involvement by non-Hodgkin lymphoma. Although higher-resolution imaging techniques have enhanced detection of adrenal masses, biopsy or excision is often needed for definitive diagnosis. Percutaneous computed tomography-guided fine needle aspiration has great diagnostic value in the workup of adrenal masses, but is limited by sampling error and artifacts. Primary adrenal lymphoma most commonly manifests with diffuse large B-cell morphology. Burkitt-like large cell lymphoma morphology has been previously reported only once, to our knowledge. We report an 80-year-old man presenting with unilateral primary adrenal lymphoma showing Burkitt-like morphology and adrenal insufficiency. Fine needle aspiration yielded a dispersed population of monomorphic, medium to large cells suggestive of lymphoma. Although dispersed cell populations cytologically favor lymphoma, metastatic poorly differentiated carcinoma and adrenal cortical carcinoma can manifest similarly. Integrated histological, immunohistochemical, and flow cytometric immunophenotyping would provide an accurate and definitive diagnosis. We review the literature and discuss important issues with regard to diagnosis.     

Follicular dendritic cell sarcoma presenting as a submucosal tumor of the stomach

Follicular dendritic cell (FDC) sarcomas, especially those of extranodal origin, are extremely rare, and this entity could easily be missed without a high index of suspicion. We report a case of FDC sarcoma presenting as a submucosal tumor of the stomach in a 45-year-old man. The mass was a spindle and epithelioid mesenchymal tumor with many individually scattered and perivascular aggregates of lymphocytes. Immunohistochemical and ultrastructural studies confirmed the diagnosis. Although more than 50 cases of this tumor have been documented in the English literature, to our knowledge the presentation of FDC sarcoma as a submucosal tumor of the stomach has never been recorded. This case highlights the occurrence of FDC sarcoma as a submucosal tumor of the gastrointestinal tract. We believe that FDC sarcoma should be included in the differential diagnosis of spindle or epithelioid cell tumors of the gastrointestinal hollow viscus to prevent this still under-recognized tumor from being overlooked.     

Synchronous pulmonary adenocarcinoma and extranodal marginal zone/low-grade B-cell lymphoma of MALT type

A case of synchronous adenocarcinoma of lung and extranodal marginal zone/low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) is reported. Primary pulmonary non-Hodgkin's lymphoma is relatively rare, however, the majority of these lesions are low-grade B-cell lymphomas of MALT. After the stomach, the lung is the second most common location for such latter lesions. Lung adenocarcinoma in selected countries is fast becoming the leading form of non small-cell lung carcinoma. To our knowledge, this synchronous occurrence in the lung has not been previously reported. Such associations have been primarily limited to gastric lesions where an association with Helicobacter pylori infection has been identified. This case report highlights the importance of adjunctive diagnostic investigations such as molecular techniques in conclusive analysis of synchronous cases such as ours.     

Kuttner's tumor of salivary glands resembling marginal zone B-cell lymphoma of the MALT type: a histopathologic and immunohistochemical study of 7 cases

Kuttner's tumor is a benign inflammatory process of the submandibular gland that presents as a hard mass mimicking a malignant neoplasm clinically. The histologic feature varies according to stage of evolution and severity of inflammation. We report here 7 cases of Kuttner's tumor that morphologically resemble primary salivary gland marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type. Histologically, the lobular architecture was distorted and the septa showed sclerosis. There was a dense lymphoplasmacytoid infiltration with lymphoid follicle formation accompanied by loss of acini and ducts. In 4 cases, a few salivary gland ducts contained the lymphoid cells within the epithelium. However, a true lymphoepithelial lesion was observed in none of the 7 cases. Immunohistochemical study demonstrated a disrupted follicular dendritic cell network, which is a characteristic finding of follicular colonization of MALT-type lymphoma. In 6 cases, there were a few small foci of lymphocytes somewhat resembling centrocyte-like cells of MALT-type lymphoma. However, immunohistological study demonstrated the mixed nature of the cells resembling centrocyte-like cells. Moreover, the polytypic nature of B lymphocytes was demonstrated by immunohistochemistry and polymerase chain reaction.     

Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma presenting as an isolated nasopharyngeal mass: a case report and review of literature

Anaplastic lymphoma kinase (ALK)-positive diffuse large B-cell lymphoma is a rare and distinct variant of diffuse large B-cell lymphoma with characteristic morphologic, immunophenotypic, and cytogenetic features. We report a case of ALK-positive diffuse large B-cell lymphoma in a 44-year-old male with progressively worsening unilateral nasal congestion and obstruction secondary to a nasopharyngeal mass. Radiologically, the mass was showed to extend to orophanrynx from nasopharynx. Histologically, the tumor cells exhibited plasmablastic morphology with expression of Bob-1, CD4, CD10, CD45, CD56, CD138, EMA, MUM1, Oct-2, and kappa immunoglobulin light chain, but negative for CD20, CD30, CD79a, PAX-5, and lambda. More importantly, the neoplastic cells showed positive immunoreactivity for ALK with exclusive cytoplasmic granular staining pattern. This case represented the second reported ALK-positive diffuse large B-cell lymphoma in the nasopharyngeal region.     

Primary B-cell mucosa-associated lymphoid tissue lymphoma presenting as a solitary colorectal polyp

We describe two patients with low-grade and one patient with mixed low- and high-grade B-cell lymphoma of mucosa associated lymphoid tissue (MALT) type arising in the large intestine. In each patient the lesion occurred as a single polyp. Two patients presented with rectal bleeding and in one the lesion was discovered incidentally. The bone marrow was uninvolved in all three cases but in the patient with mixed low- and high-grade lymphoma involvement of mesenteric lymph nodes and liver was found. CT scan revealed no lymphadenopathy or splenomegaly in any of the patients. Two patients remain well 9 and 24 months respectively after polypectomy whereas the patient with mixed low- and high-grade lymphoma died 7 days after hemicolectomy due to cardiac failure. These previously undescribed solitary polypoid MALT lymphomas can closely resemble both benign lymphoid polyposis of the colon and lymphomatous polyposis (mantle cell lymphoma). Because of their different behaviour accurate diagnosis of polypoid MALT lymphoma is important.     

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.     

A reassessment of primary thyroid lymphoma: high-grade MALT-type lymphoma as a distinct subtype of diffuse large B-cell lymphoma

AIMS: Primary lymphoma of the thyroid gland (PTL) is a relatively rare disease. During an 18-year period, 53 cases of primary non-Hodgkin's lymphoma involving this extranodal site were seen at our institutions. The aims of this study were to evaluate the spectrum of PTLs using current lymphoma classification concepts and immunocytochemical markers, determine whether features of MALT-type lymphoma were evident in PTL, and if there was any clinical significance of such a finding. METHODS AND RESULTS: The cases were retrospectively studied clinically, histologically and immunohistochemically. The tumours were classified according to the Revised European-American Lymphoma Classification of lymphoid malignancies (REAL classification). Thirty-eight patients were females, 15 were males and mean age at diagnosis was 66.3 years (range 38-90). Three cases were low-grade marginal zone lymphomas (low-grade MALT-type lymphomas). There were 45 diffuse large B-cell lymphomas (DLBCL) of which there were 27 DLBCL-NOS and 18 high-grade MALT-type lymphomas. Within the diffuse large B-cell lymphoma (DLBCL) category, cases were subdivided into those without (DLBCL-NOS) and those with features of 'high-grade' MALT-type lymphoma based on presence of a low-grade component or large cell lymphoepithelial lesions (HG MALT-type lymphoma). In addition there were three follicle centre lymphomas, one anaplastic large cell lymphoma and one peripheral T-cell lymphoma. Twenty cases were stage IE, 18 stage IIE, and four stage IV. All patients with low-grade MALT-type lymphoma are alive without disease. The 5-year survivals for DLBCL-NOS and HG MALT-type lymphoma were 75% and 25%, respectively. Univariate analysis (log rank) among the DLBCLs showed stage (P < 0.001) and subtype (P = 0.005) were associated with survival. Stage was associated with type of DLBCL, 65% of DLBCL-NOS being stage IE compared to 20% of HG MALT-type lymphomas. CONCLUSIONS: We conclude that primary thyroid lymphomas occur most commonly in elderly women and are frequently present in clinical stage IE and IIE. Low-grade MALT-type lymphomas are relatively uncommon but appear to have a favourable prognosis. DLBCL is the most common lymphoma and features of MALT can be seen in over one-third of cases. As a group, HG MALT-type lymphomas had a worse outcome than DLBCL-NOS, primarily due to higher clinical stage at diagnosis. These two subtypes of DLBCL appear to be distinct clinical and histological entities.     

原发皮肤弥漫性大B细胞淋巴瘤(腿型)一例

患者男,60岁.因右小腿皮肤结节2个月于2006年3月21日入院.患者于2005年11月发现右小腿皮肤有斑片状色素沉着,2006年1月右小腿出现红色斑疹及结节,进行性增大,高出皮面.     

Intravascular large B-cell lymphoma presenting as cholecystitis and pancytopenia: case report with literature review

Intravascular large B-cell lymphoma (IVLBCL) is a rare variant of extranodal diffuse large Bcell lymphoma with only a few more than 300 cases reported. It is characterized as lymphoma cells confined to the lumina of small vessels, so patients usually do not present with masses or lymphadenopathy. Clinical presentations of these patients are non-specific and the pathologic changes may be subtle, which often leads to delayed diagnoses and, in many instances, a postmortem diagnosis. IVLBCL can essentially involve the vessels of any organ, but it is quite rare for the gallbladder to serve as the initial presenting site; there are only four such cases reported in the English literature. Furthermore, IVLBCL of the gallbladder with peripheral blood involvement is even less common. We report a recent case of IVLBCL presenting as acute cholecystitis and pancytopenia. The patient underwent a simple cholecystectomy. Examination of the gallbladder showed clusters of large lymphoma cells within lumina of small vessels in the gallbladder wall. These cells were positive for CD5/CD20 and negative for CD3, CD10, and TdT. Based on these findings, a diagnosis of IVLBCL was made. Coincidently, circulating lymphoma cells were identified in the peripheral blood and confirmed by flow cytometric analysis (positive for CD19/CD20/CD5, without light chain expression). The patient was started on chemotherapy but subsequently died of chemotherapy related multi-organ failure 10 days after the initial diagnosis.     

Report of a primary lymphoma of the conjunctiva. A lymphoma of MALT origin?

A case of primary conjunctival lymphoma is reported herein. A 47-year-old woman presented with an indolent salmon-coloured tumour of the lower epibulbar conjunctiva. Histologically, we found a lymphoid infiltrate in the epithelium and subepithelium consisting of two components. One component was characterized by centrocytic-like cells and the other by lymphocytic, plasmacytoid and plasma cells. An immunohistological study demonstrated a light chain restricted immunoglobulin expression of the tumour cells as well as some residual polytypic follicles exhibiting reticular dendritic cells. Tumour cell proliferation was associated with a T cell reactive population. A comprehensive investigation (in particular an orbital scan) did not demonstrate any other localization of this lymphoma. Such a lesion raised the question of a possible MALT origin, by analog with MALT-lymphomas of the gastrointestinal tract described by Isaacson and colleagues.     

原发皮肤弥漫性大B细胞淋巴瘤(腿型)一例

患者男,60岁.因右小腿皮肤结节2个月于2006年3月21日入院.患者于2005年11月发现右小腿皮肤有斑片状色素沉着,2006年1月右小腿出现红色斑疹及结节,进行性增大,高出皮面.     

原发皮肤弥漫性大B细胞淋巴瘤(腿型)一例

患者男,60岁.因右小腿皮肤结节2个月于2006年3月21日入院.患者于2005年11月发现右小腿皮肤有斑片状色素沉着,2006年1月右小腿出现红色斑疹及结节,进行性增大,高出皮面.     

Recurrent diffuse large B-cell lymphoma presenting initially as hemophagocytic syndrome

Hemophagocytic syndrome (HPS) is an extremely rare and life-threatening abnormality, and the cases secondary to B cell lymphoma are rare. We report a case of relapsed diffuse large B-cell lymphoma initially presenting with hemophagocytic syndrome. The patient developed multiple erythematous macules and progressive thrombocytopenia during the treatment, and died two weeks after admission. The HPS presented as an initial manifestation of the relapsed diffuse B-cell lymphoma and the maculea that appeared during the treatment might be a strong predictor of unfavorable outcome.