肉瘤样型间变性大细胞淋巴瘤临床病理特征

目的探讨肉瘤样型间变性大细胞淋巴瘤(sALCL)临床病理特点、免疫表型及分子遗传学特征。方法对1例sALCL的临床、病理组织学、免疫表型及免疫球蛋白重链(IgH)和T细胞受体(TCR)基因克隆性重排情况进行观察并复习相关文献。结果眼观:送检淋巴结1枚,1.5cm×1.0cm×1.0cm,切面呈鱼肉状。镜检:淋巴结基本结构几乎完全被破坏,异型的梭形和上皮样细胞弥漫增生。免疫表型:瘤细胞呈CD30、ALK1、EMA、CD45RO、CD45、TIA1、granzymeB、perforin、CD68(部分)、SMA(梭形成分)阳性。基因重排:TCRβ1克隆性重排。结论sALCL属罕见恶性肿瘤,其形态不典型,易误诊为其他恶性肿瘤,免疫表型和遗传学异常有助于其诊断和鉴别诊断。     

ALK-negative anaplastic large cell lymphoma primarily involving the bronchus: a case report and literature review

Anaplastic large cell lymphoma (ALCL) is a mature T cell lymphoma with characteristic morphologic, immunophenotypic and cytogenetic features. Current WHO classification includes anaplastic lymphoma kinase (ALK)-positive and ALK-negative variants. ALCL rarely presents with obstructive symptoms of the main airway. In addition to reporting a HIV-associated bronchial ALK-negative ALCL in a 44 year-old female, our literature review identified eight cases of bronchial ALCL with several interesting clinicopathological features, including: 1) a female predominance (67%); 2) two thirds of patients younger than 18 years old; 3) uniformly presented with respiratory symptoms and progressed to respiratory failure; 4) the tumor involving the main airways; 5) often with localized disease at the initial presentation. This unusual presentation of ALCL may pose as a diagnostic pitfall and delay the treatment.     

Cytomorphologic features of primary anaplastic large cell lymphoma of the psoas muscle: A case report and literature review

Ki‐1 (CD30) positive anaplastic large cell lymphoma (ALCL) is an uncommon malignancy, which may present with nodal as well as extra‐nodal disease. Primary skeletal muscle Ki‐1 (CD30) positive ALCL is an even rarer event with few cases reported in the literature and only some with published cytomorphologic features. An 83‐year‐old woman underwent a fine needle aspiration (FNA) of a psoas muscle mass. Smears demonstrated a predominantly discohesive population of large pleomorphic cells. The nuclei were hypechromatic and lobulated, with often multinucleation. Nucleoli were prominent in a subset of cells. Cytoplasmic vacuolization was also present. No lymphoglandular bodies were identified. A cytodiagnosis of malignant cells favoring metastatic melanoma vs. poorly differentiated carcinoma was rendered. Morphologic and immunohistochemical features later revealed a primary psoas muscle Ki‐1 (CD30) positive ALCL with negative staining for anaplastic large cell lymphoma kinase (ALK). Cytologic features of ALCL mimic epithelial neoplasms, sarcomas, melanoma and other lymphomas. Although rare, ALCL should be a diagnostic consideration when discohesive pleomorphic malignant cells are encountered on FNA of a muscle neoplasm. Diagn. Cytopathol. 2010. © 2009 Wiley‐Liss, Inc.     

Primary cutaneous anaplastic large cell lymphoma occurring in an atopic dermatitis patient: a case report with review of the literature with emphasis on their association

Although the risk of malignant lymphoma in patients with atopic dermatitis (AD) remains controversial, an increased risk of malignant T-cell lymphoma in patients with AD has been reported. Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a relatively common distinct clinicopathological entity. However, occurrence of C-ALCL in patients with AD has been rarely reported. Herein, we describe the 5^th reported case of C-ALCL occurring in a patient with AD and review the clinicopathological features. A 30-year-old Japanese male with a long-standing history of AD presented with a gradually enlarged nodular lesion in the right abdominal wall, which had spontaneously regressed without therapy. Two years later, multiple nodular lesions appeared in his trunk, and swelling of multiple lymph nodes was also detected. Histopathological studies demonstrated diffuse proliferation of large-sized lymphocytes with large convoluted nuclei containing conspicuous nucleoli and relatively rich cytoplasm in the skin and lymph node. Immunohistochemically, these lymphocytes were positive for CD30, CD8, and MUM1, and negative for CD3, CD4, and ALK1. Accordingly, a diagnosis of primary C-ALCL was made. The patient died of disease after various courses of chemotherapy. Our clinicopathological review revealed that the prognosis of C-ALCL occurring in patients with AD is poor because two of 5 patients died of disease. Therefore, albeit extremely rare, AD patients with C-ALCL should be monitored closely, and additional clinicopathological studies are needed to clarify the pathogenesis of C-ALCL occurring in patients with AD.     

结肠原发性CD3O阳性间变性大细胞淋巴瘤1例报道及文献复习

目的 探讨结肠原发问变性大细胞淋巴瘤(ALCL)的组织学特点及免疫组化特征。方法 运用组织形态学、免疫组化方法研究1例发生在结肠的间变性大细胞淋巴瘤并结合文献进行讨论。结果 间变性大细胞淋巴瘤其形态学表现为多样性，免疫组化显示CD30 ，另外CD45、CD45RO、CD3和EMA也可 。结论 间变性大细胞淋巴瘤发生在结肠非常罕见，其形态学和免疫组化表型有一定的特殊性，并且预后良好，有必要与其它类型淋巴瘤、霍奇金淋巴瘤、恶性组织细胞增生症、恶性黑色素瘤、低分化腺癌等进行鉴别。     

Anaplastic large cell lymphoma involving the urinary bladder: A case report and review of the literature

T cell‐derived malignant lymphoma is rarely detected as a bladder neoplasm. A literature review for anaplastic large cell lymphoma (ALCL) involving urinary bladder reveals only seven previously reported cases. Here, we report a case of a 59‐year‐old HIV‐negative man with ALK‐positive ALCL. He presented an unusual clinical course with initial consideration of adult onset Still's Disease (AOSD) due to his negative results searching for malignancy and infectious diseases. He rapidly developed macrophage activation (hemophagocytic) syndrome and experienced an unusual rapid disease progression and died in 39 days after onset of symptoms. Compared to previously reported cases, the current case of ALK‐1‐positive ALCL is a rare case with an unusual presentation. From this case, we learned that ALCL is one malignancy that should be considered and screened in patients with suspected AOSD. Also, T‐cell lymphoma associated hemophagocytic syndrome should be considered in a patient with sustained corticosteroid‐resistant spike fever, high serum ferritin, and rapid exacerbation of the disease course. Diagn. Cytopathol. 2015;43:60–65. © 2014 Wiley Periodicals, Inc.     

Fine needle aspiration cytology of ALK 1(−), CD 30(+) anaplastic large cell lymphoma post renal transplantation: A case report and literature review

Post transplant lymphoproliferative disorders (PTLD) complicates the course of 0.3 to 3% of renal transplant patients receiving immunosuppression. 1 , 2 Epstein‐Barr Virus (EBV) related Non‐Hodgkin's Lymphomas of B‐cell type is more common than those of T‐cell origin. 1 CD30 positive Anaplastic Large Cell Lymphoma (ALCL) is a Non‐Hodgkin's lymphoma (B or T cell type) that accounts for a small percentage of PTLD's. ALCL of T‐cell type are a spectrum of disease ranging from primary cutaneous to systemic nodal ALCL. The systemic nodal ALCL is further subdivided into anaplastic lymphoma kinase‐1 (ALK‐1) positive or negative. 3 ALK‐1 protein is a gene fusion product of translocation (2;5) and carries prognostic implications. 4 - 6 We present an unusual manifestation of ALK‐1 negative CD30 positive ALCL in a post renal transplant patient in FNA cytology with all supportive adjuvant studies and differential diagnoses and review the cytology literature on this topic. Diagn. Cytopathol. 2010. © 2009 Wiley‐Liss, Inc.     

ALK‐negative anaplastic large cell lymphoma with urinary bladder involvement diagnosed in urine cytology: A case report and literature review

Anaplastic large cell lymphoma is an aggressive T‐cell neoplasm. It rarely involves the urinary bladder, with just twelve cases reported thus far and only one being ALK‐negative. Immunophenotyping (particularly for ALK) is mandatory, both for prognostic and therapeutic reasons. Herein, we report the case of a patient with an ALK‐negative anaplastic large cell lymphoma involving the bladder which was diagnosed and fully characterized by immunocytochemistry in urine cytology. The patient underwent a cystoscopy and the urine sample disclosed tumor diathesis background and aggregates of atypical cells, with evidence of multinucleation and mitotic figures. Immunocytochemistry revealed strong membrane/Golgi positivity for CD30 and negativity for ALK. The patient was submitted to transurethral resection for therapeutic purposes, which confirmed the diagnosis. To the best of our knowledge, this represents only the third case of anaplastic large cell lymphoma with bladder involvement diagnosed in urine cytology and the very first with diagnostic findings allowing for immunophenotyping of the disease in a bladder wash. The present report reinforces the role of urine cytology as a suitable method for establishing an earlier diagnosis and characterization of the disease, avoiding submitting patients to invasive procedures like transurethral resections. Diagn. Cytopathol. 2017;45:354–358. © 2016 Wiley Periodicals, Inc.     

Primary cerebral anaplastic large cell lymphoma containing abundant reactive histiocytes and eosinophils. A case report and literature review

Primary cerebral anaplastic large cell lymphoma (ALCL) is very rare. We report on our experience with such a case and review the literature. A 46-year-old Taiwanese woman presented with headache, weakness of her right extremity, and limited eye movement. A solid mass (5 cm x 4 cm) at the left occipital lobe was almost completely removed. The neoplastic cells, some of which had reniform or embryo-like nuclei, were large and were admixed with abundant eosinophils, histiocytes, and some small lymphocytes. These neoplastic cells expressed CD30, CD43, granzyme B and T-cell intracellular antigen-1, but not ALK1, CD3, CD20, CD45, CD79a, cytokeratin, and EMA. They were positive for Epstein-Barr virus-encoded mRNA by in situ hybridization. Polymerase chain reaction study of formalin-fixed tissue showed a clonal gene arrangement of the T-cell receptor-gamma chain. ALCL of T-cell lineage with cytotoxic phenotype was diagnosed. The patient received cranial irradiation and has remained with no evidence of disease for 25 months of follow-up.     

Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma presenting in nasal cavity: a case report and review of literature

Anaplastic lymphoma kinase (ALK)-positive diffuse large B-cell lymphoma (DLBCL) is a rare subtype of non-Hodgkin’s lymphoma (NHL) with distinct morphologic and immunohistochemical features. We reported a 57-year-old female with ALK-positive DLBCL in her left nasal cavity. Histologically, the tumor cells were characterized by plasmablastic morphology and tested positive for ALK in a cytoplasmic granular staining pattern. The neoplastic cells were positive for CD38, CD4, MUM1, CD138 and Vimentin. However, they failed to express CD56, CD30, as well as mature B cells markers, such as CD79a, CD20 and T cells markers such as CD2, CD3, CD5, CD7 and CD8. The patient achieved complete response after four cycles of CHOEP (cyclophosphamide, doxorubicin, vincristine, prednisone, and etoposide) treatment. Then she received radiotherapy of the originally involved area. This case represented a rare ALK-positive DLBCL in the nasal region.     

Fine-needle aspiration biopsy of anaplastic large cell lymphoma, small cell variant with prominent plasmacytoid features: case report

Anaplastic large cell lymphoma (ALCL), according to the new WHO classification, is a diagnosis limited to T/NK cell lymphomas. We present a case that demonstrates a new morphologic variant of ALCL with significant possible pitfalls for the cytopathologist. A fine-needle aspiration biopsy of a cervical lymph node showed a cellular aspiration comprised of medium-sized plasmacytoid cells in a discohesive and focally loosely cohesive pattern. The cytologic diagnosis confirmed the presence of malignancy and noted the prominent plasmacytoid features. An accompanying comment favored melanoma and included a broad differential. No cell block was available for immunohistochemical stains. Immunophenotyping of the subsequent excisional node biopsy showed an anaplastic lymphoma kinase (ALK)-positive ALCL. This case illustrates a new variant of ALCL. Although ALCL variants, such as small cell and lymphohistiocytic, are well recognized, the plasmacytoid features are an additional potential source for misdiagnosis. This case report shows that a cytopathologist should include ALK-positive ALCL in the differential diagnosis of plasmacytoid proliferations cell because of the clinical importance of the ALK-positive ALCL.     

软组织及骨的小细胞型间变性大细胞淋巴瘤1例报道

目的 :阐述罕见的小细胞型间变性大细胞淋巴瘤 (ALCL)的病理形态学特点。方法 :对 1例发生于双侧肩胛区、累及骨和周围软组织的小细胞型ALCL进行了光镜、免疫组织化学观察和PCR基因分析。结果 :病变以小到中体积的恶性淋巴细胞为主 ,局部可见大的间变性细胞 ,部分肿瘤细胞围绕小血管呈花环状排列 ,肿瘤间有较多的中性粒细胞和组织细胞。肿瘤细胞表达CD30和EMA ,并具有T细胞表型和TCR β基因重排。 结论 :此型ALCL具有特殊的组织学表现和侵袭性的生物学行为 ,应注意与炎症和嗜酸性肉芽肿等疾病进行鉴别。     

Clear cell variant of diffuse large B-cell lymphoma: a case report and review of the literature

Diffuse large B cell lymphoma (DLBCL) is a diffuse proliferation of large neoplastic B lymphoid cells with nuclear size equal to or exceeding that of normal macrophage nuclei. The DLBCL morphological variants are centroblastic, immunoblastic, T-cell- and histiocyte-rich, anaplastic, plasmablastic, anaplastic lymphoma kinase-positive, and primary mediastinal large B-cell lymphoma (PMBCL). These histopathologically-recognized morphological variants respond differently to treatment and have distinct prognoses. We report a case of a 43-year-old patient who presented pain in the lower abdomen that had begun four months prior. Ultrasound-guided biopsy revealed epithelial cell features and a partial alveolar growth pattern. We discovered large diffuse areas comprising large cells with slightly irregular nuclei and very clear cytoplasm. These features were similar to those of clear cell carcinoma in renal tissue, suggesting the possibility of an epithelial neoplasm. To test this possibility, immunohistochemistry for cluster designation markers was performed, but the diffuse areas were found to be positive only for CD45. Additional immunohistochemistry was performed, and the diffuse areas were found to be positive for CD20, CD79a, P53, and Mum-1. Based on these characteristics, a diagnosis of a clear cell variant of DLBCL was made, and the patient was treated with chemotherapy. Precise histological diagnosis is crucial for clinical management and ultimately for patient survival. There has been one additional report of a case of clear cell DLBCL, in outside the mediastinum. The features we identified can be used to define a new subtype of DLBCL. The expression of P53 and Mum-1 suggest a poor prognosis.     

Small cell variant of anaplastic large cell lymphoma diagnosed by a novel chromosomal abnormality t(2;5;3)(p23;q35;p21) of bone marrow cells

Because of the rarity and the morphological variations, small cell variant of anaplastic large cell lymphoma (ALCL) represents a diagnostic challenge. Herein is reported a case of leukemic type of small cell variant of ALCL, in which the diagnosis was established by a cytogenetic analysis. The patient was a 23-year-old woman who presented with fever and leukocytosis with circulatory atypical lymphoid cells. The initial differential diagnosis on bone marrow trephine biopsy sections included viral infection and peripheral T-cell lymphoma unspecified. But a cytogenetic study on bone marrow cells indicated a novel complex translocation, t(2;5;3)(p23;q35;p21), which led to confirmation of anaplastic lymphoma kinase (ALK)-positive pleomorphic small to medium-sized cells scattered in bone marrow cells, on immunohistochemistry. ALK was distributed in both nuclear and cytoplasmic regions of neoplastic cells. The patient achieved complete remission after four courses of combination chemotherapy, and received autologous peripheral stem cell transplantation (auto-PBSCT) after two additional courses of combination chemotherapy, but relapsed 2 months after auto-PBSCT in the bilateral lung. Allogeneic stem cell transplantation led to a second remission. This case demonstrates the diagnostic importance of cytogenetic study for malignant lymphoma involving bone marrow.     

Primary systemic anaplastic large cell lymphoma in a single Korean institution: clinical characteristics and treatment outcome

Despite advances in the characterization of anaplastic large cell lymphoma (ALCL), little data is available on Asian patients. We report here upon single Korean institution's experience regarding the clinical characteristics and outcomes of ALCL. We performed a retrospective study of 32 adults with ALCL. Most of the patients received anthracycline-based chemotherapy. Ann Arbor stage III-IV, B symptoms, high-intermediate/ high International Prognostic Index (IPI), and extranodal disease at diagnosis were present in 56%, 44%, 41%, and 63%, respectively. Compared with Western studies, the male/female ratio (4.3) was markedly higher and skin (9%) and bone involvement (9%) were less frequent. The staining results for anaplastic lymphoma kinase were positive in 6 (33%) of 18 cases available. The complete response (CR) rate was 62% (95% CI, 44-80%). With a median follow-up of 51.0 months, 5 yr overall survival was 40+/-11%. The 3 yr relapse-free survival for the 18 patients who achieved CR was 74+/-12%. Age, performance status, lactate dehydrogenase, extranodal disease sites number, and IPI were correlated with treatment response and survival. Our data suggest that Korean ALCL patients appear to have a higher male/female ratio, less frequent skin/bone involvement, and lower CR rate compared with those of Western studies.     

Peripheral T-cell lymphoma arising from an intraglandular lymph node in the parotid gland: a case report and literature review

T/NK-cell lymphoma of the salivary gland is rare. A 58-year-old man complained of a tumor mass in the left parotid gland region and he was diagnosed to have a left parotid tumor. The tumor was subsequently resected, revealing a diffuse growth pattern of medium to large sized atypical cells. The tumor was surrounded by fibrous connective tissue in the form of a capsule, and those were positive for CD3, CD4, CD5 and CD30, but negative for Bcl2, CD8, CD10, CD15, CD20, CD25, CD56, CD79a, CD246, EMA, granzyme B, TdT and TIA-1. There was no molecular evidence of Epstein-Barr virus (EBV) infection. It was diagnosed as peripheral T-cell lymphoma (PTCL) arising from an intraglandular lymph node in the parotid gland. In conclusion, Only 17 cases of primary T/NK-cell lymphoma of the salivary glands have been recorded until now, and the characteristics of these are not clear yet. Additional study is needed.     

淋巴结血管内T细胞淋巴瘤1例报道及文献复习

目的　探讨血管内淋巴瘤 (IVL)的临床病理特征。方法　对 1例腹股沟淋巴结IVL临床、病理组织学及免疫表型进行观察分析并复习文献。结果　男性 31岁 ,不明原因高热伴消瘦 5 0天 ,右腹股沟直径 1cm淋巴结 1枚 ,B超示肝脏轻度增大 ,血LDH明显升高伴ESR及转氨酶轻度升高 ,外周血WBC 3 3× 10 7/L ,骨髓像、多种病原及各肿瘤相关抗原检测均无异常。病理活检 :腹股沟淋巴结大部分破坏 ,代之以大量扩张的中小血管 ,腔内充满大量异型淋巴样细胞 ,局部伴管壁、管周浸润并累及结外脂肪组织。瘤细胞免疫表型CD4 5、CD4 5RO、CD3阳性 ,CK、CD6 8、CD79α、CD2 0均阴性 ,血管壁及内皮细胞CD31、CD34阳性。行CHOP化疗后症状缓解 ,现仍在随访中。结论　IVL是一罕见的非霍奇金淋巴瘤 ,好发于中枢神经系统及皮肤 ,其他部位少见 ,绝大数为B细胞型 ,T型罕见 ,以浅表淋巴结活检确诊者尚无报道。临床表现有一定提示性 ,确诊靠组织病理学检查 ,部分病例对化疗敏感 ,但多数病例预后差     

Nodal marginal zone B-cell lymphoma resembling plasmacytoma arising from a plasma cell variant of localized Castleman's disease: a case report

Nodal marginal zone B-cell lymphoma (NMZBL) occasionally represents prominent plasma cell differentiation. Recently, primary lymph node plasmacytoma has been suggested to represent an extremely plasmacytic differentiation of NMZBL. We here report a case of NMZBL showing histological features resembling plasmacytoma arising from a plasma cell variant of localized Castleman's disease (PCLCD). The patient was a 69-year-old Japanese female with a 20-year history of a right inguinal mass. Histologically, a prominent proliferation of plasma cells occupied the interfollicular area of the central portion of the lymph node, whereas centrocyte-like (CCL) cells were the main cellular component in the peripheral portion of the lymph node. Although most of the plasma cells were mature 'Marshalko-type', occasional atypical forms with enlarged nuclei were also present. The majority of the lymphoid follicles had atrophic or regressive germinal centers. A few lymphoid follicles were colonized by CCL cells. Immunohistochemistry study revealed that both plasma cells and some CCL cells had a monotypic intracytoplasmic lambda light chain. When monoclonal plasma cell infiltration is observed in PCLCD, the light chains are mostly restricted to the lambda chain. This case suggests that some plasma cell-containing tumors arising from PCLCD may represent a variant of NMZBL.