Primary cutaneous anaplastic large cell lymphoma

Primary cutaneous anaplastic large cell lymphoma (PC‐ALCL) is a CD30+ lymphoproliferative disorder (LPD) of the skin with a relatively good prognosis in the absence of high‐stage disease. CD30+ LPDs comprise approximately 25%‐30% of primary cutaneous lymphomas and as a group represent the second most common clonal T‐cell neoplasm of the skin behind mycosis fungoides. Diagnosis of PC‐ALCL relies strongly on clinicopathologic correlation given the potential morphologic, clinical and molecular overlap with the other cutaneous CD30+ LPD, lymphomatoid papulosis, and more aggressive hematolymphoid neoplasms.     

Myxoid variant of anaplastic large cell lymphoma involving the skin: a case report

We report the case of a 62-year-old white male who presented with a 2.6-cm ulcerating mass on the skin of the left buttock and ipsilateral inguinal lymphadenopathy. Microscopic sections of the skin lesion showed a nodular and plaque-like growth pattern of a mixed cellular infiltrate throughout the dermis and subcutaneous tissue with prominent myxoid change. There was a dominant population of medium-sized mitotically active atypical cells that expressed CD30, CD4 and EMA. These atypical cells were mixed with eosinophils, neutrophils, mature lymphocytes and histiocytes. Tissue from the inguinal lymphadenopathy showed similar pathologic features, although no residual lymph node tissue was present. A diagnosis of secondary anaplastic large cell lymphoma, myxoid variant, with skin and lymph node/perinodal soft tissue involvement was rendered at the time of complete excision of the buttock mass. The patient received five cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy with complete resolution of lymphadenopathy and no residual cutaneous disease. He was disease-free by PET/CT scan and physical examination at 16 months after chemotherapy. We present this case to highlight the histopathologic and immunophenotypic features of this entity with a discussion of the differential diagnosis and a review of the literature.     

原发性皮肤CD30+间变性大细胞淋巴瘤临床及组织病理分析

目的：探讨原发性皮肤CD30 间变性大细胞淋巴瘤(ALCL)的临床及组织病理学特征。方法：对3例患者的皮损行组织病理学及免疫组化检查,观察原发性皮肤CD30 ALCL组织病理学特征及CD45、CD3、CD20、CD30、EMA、HMB45等抗体染色结果。结果：3例患者临床上均表现为皮下结节。组织病理学特征为肿瘤细胞异形性明显,呈多形性,胞质丰富,核大,核仁明显。CD30标记阳性,不表达T细胞标记CD45RO及CD3。结论：原发性皮肤CD30 ALCL是具有独特形态特点及临床特征的肿瘤,根据组织学特征及免疫组化CD30阳性,可与其他恶性肿瘤进行鉴别。     

原发性皮肤间变性大细胞淋巴瘤

报告1例原发性皮肤间变性大细胞淋巴瘤.患者女,24岁.左手中指红斑33 d,根据皮损组织病理及免疫组化标记结果诊断为原发性皮肤间变性大细胞淋巴瘤.     

原发性皮肤间变性大细胞淋巴瘤1例

报告1例原发性皮肤间变性大细胞淋巴瘤。患者女,49岁。右小腿结节1年,溃烂5个月。皮损组织病理检查:真皮内有密集的淋巴样细胞浸润,瘤细胞大、核呈肾形或不规则形、核分裂像多见,免疫组化示瘤细胞约70?30阳性、约20?45Ro阳性,而CD3、CD20、MPO、TIA-1、ALK-1均为阴性。诊断为原发性皮肤间变性大细胞淋巴瘤。     

ALK‐positive (2p23 rearranged) anaplastic large cell lymphoma with localization to the skin in a pediatric patient

Anaplastic large cell lymphoma (ALCL) either as primary cutaneous or nodal disease is rare in children and difficult to distinguish, which is important both prognostically and for treatment purposes. We present a case of anaplastic lymphoma kinase (ALK)+ skin‐limited ALCL that highlights this challenge and draws attention to pitfalls in assessing ALK status. The patient was an 11‐year old girl with a twice recurrent nodule on her right shoulder. Each biopsy revealed a deep infiltrate of atypical lymphocytes that expressed CD3, CD4, CD43, CD45RO and CD30. The initial biopsy was epithelial membrane antigen (EMA)+ with vague cytoplasmic ALK‐1 positivity by immunohistochemistry, while the second biopsy was EMA+ and nuclear ALK‐1+. Fluorescence in situ hybridization analysis for an ALK (2p23) rearrangement of the first specimen was negative, while an ALK gene rearrangement was present in the second specimen. Therefore, this case was treated as nodal ALCL, despite negative bone marrow and radiographic imaging studies. The patient was treated with combination chemotherapy and remains disease‐free. Demonstration of nuclear ALK‐positivity, ALK (2p23) gene rearrangement is suggestive of systemic ALCL. Without evidence of systemic disease, this case highlights challenges of skin‐limited ALCL, whose clinical behavior as either cutaneous ALCL systemic ALCL may not be immediately apparent.     

原发性皮肤CD30+间变性大细胞淋巴瘤1例

报告1例原发性皮肤CD30+间变性大细胞淋巴瘤.患者男,72岁.右足拇趾红肿,外侧有一2.0 cm×2.5 cm红色溃疡面,伴血性液体渗出,近端有一1.5 cm×2.0 cm暗红色结节,表面结痂.皮损组织病理及免疫组化标记确诊为原发性皮肤CD30+间变性大细胞淋巴瘤.     

CD56 expression in a case of primary cutaneous CD30+ anaplastic large cell lymphoma

We describe clinicopathological features of an unusual case of CD30+/CD56+ T-cell lymphoma in a 58-year-old Korean man who presented with disseminated nodules, papules and hyperpigmented patches. Coexpression of CD30 and CD56 in T-cell lymphoma is very rare. Our patient did not respond to an intensive chemotherapy regimen, in contrast to the previously reported cases of primary cutaneous CD30+ anaplastic large cell lymphoma. Coexpression of CD56 might therefore identify a subset of CD30+ lymphomas with more aggressive features.     

CD30+ lymphoproliferative disorder: primary cutaneous anaplastic large cell lymphoma followed by lymphomatoid papulosis

CD30+ large anaplastic lymphoid cells are seen in anaplastic large cell lymphoma (ALCL), and also in lymphomatoid papulosis (LyP) and other lymphoproliferative disorders. It can be difficult precisely to categorize these disorders with CD30+ cells. We report a case of primary cutaneous CD30+ ALCL with systemic metastases in whom the clinical disease subsequently evolved into LyP. The patient was initially administered cisplatin and etoposide and made a good response. Eighteen months later, recurrent, self-healing cutaneous small nodules appeared around the original tumour site without any systemic involvement. Histopathological examination of the recurrent lesions revealed infiltration with a mixture of cells that included neutrophils, eosinophils and CD30+ large anaplastic cells cytologically identical with those in the primary lesion. The anaplastic cells in both the primary and recurrent lesions were positive for monoclonal antibodies CD30, CD25 and a monoclonal antibody directed against the chimeric protein p80(NPM-ALK). These observations suggest the possibility that the ALCL and the subsequent LyP represent different clinical manifestations of proliferation of the same clone.     

Clinical spectrum of primary cutaneous CD30‐positive anaplastic large cell lymphoma: an analysis of the Mannheim Cutaneous Lymphoma Registry

Background: Primary cutaneous CD30⁺ anaplastic large cell lymphomas (C‐ALCL) have indolent clinical behavior with an estimated 5‐year survival rate of 95%. The clinical features and disease courses of C‐ALCL identified in the lymphoma registry of Mannheim University hospital are described in the following. Patients and methods: All C‐ALCL patients identified in the database were analyzed in regard to clinical picture, histology, immunohistochemistry, molecular biology, staging, therapy, follow‐up, and outcome. Results: 14 C‐ALCL patients were identified. The mean age was 69 years and 57% were men. Solitary skin lesions in one anatomical region were seen in 12 patients upon initial diagnosis. Two patients presented with multiple lesions at different anatomical sites. In 2 patients there was specific lymph node involvement. In one C‐ALCL patient, follow‐up over 17 months revealed extracutaneous infiltration. Half of the patients relapsed and 36% had multiple episodes. The majority of our patients were treated with surgical excision followed by electron beam radiotherapy. The 5‐year survival rate was 93% in C‐ALCL. Conclusions: The clinical presentation of C‐ALCL varies. Staging procedures and a close clinical pathological correlation at initial diagnosis are essential. Due to a high rate of relapses and the possibility of developing extranodal manifestations over the course of the disease, close follow‐up is recommended.     

Disseminated CD8‐positive,CD30‐positive cutaneous lymphoproliferative eruption with overlapping features of mycosis fungoides and primary cutaneous anaplastic large cell lymphoma following remote solitary lesional presentation

CD8‐positive, CD30‐positive cutaneous lymphoproliferative disorders constitute a rare subset of T‐cell lymphoproliferative conditions, including variants of primary cutaneous anaplastic large cell lymphoma (ALCL), mycosis fungoides, lymphomatoid papulosis type D, cutaneous gamma‐delta T‐cell lymphoma and cutaneous peripheral T‐cell lymphoma. These entities share overlapping clinical, histopathologic and immunophenotypic features, presenting both a clinical and pathological diagnostic challenge. Presented here is a 73‐year‐old man with a disseminated, indolent CD30+, CD8+ cutaneous lymphoproliferative disorder with overlapping clinical and histopathological features of both mycosis fungoides and primary cutaneous ALCL, as well as features of lymphomatoid papulosis. To our knowledge, this is the first case of a generalized CD8+, CD30+ eruption with features of both mycosis fungoides and primary cutaneous ALCL arising following an episode of solitary primary cutaneous CD8‐positive ALCL.     

Primary cutaneous anaplastic large cell lymphoma with subsequent leg involvement

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is regarded as an indolent type of cutaneous T-cell lymphoma. However, a few recent publications revealed that C-ALCL patients with initial leg involvement had significantly worse survival than those without initial leg involvement. Herein, we report a case of C-ALCL with subsequent leg involvement, which led to death after chemoradiation therapy. A 75 years old Japanese man presented with multiple erythematous nodules in his left arm and the side of his left chest. Histopathological and immunohistochemical studies led to the diagnosis of primary C-ALCL. At the initial diagnosis, no leg lesion was found. One year after the initial diagnosis, C-ALCL appeared in his right lower thigh and left hip. Radiation therapy, low-dose etoposide and CHOP therapy were performed; however, the patient died of malignant lymphoma 4 years after the initial diagnosis. We speculated that the occurrence of subsequent leg involvement may also be indicative of a worse prognosis, as in the case with initial leg involvement in C-ALCL. Therefore, we propose that C-ALCL patients with initial or subsequent leg involvement should be classified as a distinct clinicopathological variant of C-ALCL (“leg-type” involvement) and that they may require intense therapy.     

原发皮肤CD30阳性间变大细胞淋巴瘤七例临床病理分析

目的 探讨原发皮肤CD30阳性间变大细胞淋巴瘤（PC-ALCL）的临床与组织病理学特征。 方法 回顾性分析7例PC-ALCL患者的临床及病理资料情况。 结果 7例患者中,男6例,女1例,平均发病年龄52岁。皮损为红色结节、肿块和（或）斑块,3例多发,4例单发,6例伴溃疡形成。所有患者均未见系统受累。组织病理改变：肿瘤细胞在真皮内弥漫性分布,细胞体积大,胞质丰富,胞核呈异形改变,可见核分裂象。肿瘤细胞CD30和细胞毒蛋白阳性,CD20、CD56、间变性淋巴瘤激酶（ALK）和EB病毒编码小RNA原位杂交均阴性。结论 PC-ALCL是一种少见的原发于皮肤的低度恶性T细胞淋巴瘤,结合临床表现、皮损组织病理及免疫组化检查可确诊,罕见系统受累及转移。     

Pediatric case of anaplastic lymphoma kinase‐positive anaplastic large cell lymphoma forming a solitary skin tumor on the forearm

A 5‐year‐old girl noticed a rapidly growing reddish nodule on her right forearm. Although oral antibiotics had been administrated for 2 weeks, the tumor enlarged. Skin biopsy revealed excessive infiltration of atypical neoplastic cells expressing CD4, CD30 and anaplastic lymphoma kinase (ALK). These histological and immunohistochemical findings were consistent with anaplastic large cell lymphoma (ALCL). Computed tomography showed multiple lymphadenopathy, but lymph node biopsy and bone marrow examination did not show any evidence of systemic dissemination. However, due to the positive results for ALK and multiple lymphadenopathy, we diagnosed ALK‐positive ALCL forming a solitary skin tumor on the forearm. The patient received chemotherapy and presented marked improvement. This paper discusses the difficulty of diagnosing pediatric ALK‐positive ALCL limited to the skin and reviews the medical published work.     

Primary cutaneous anaplastic large cell lymphoma with intralymphatic involvement associated with chronic lymphedema

Chronic lymphedema predisposes to develop malignant cutaneous tumors, including angiosarcoma, Kaposi's sarcoma and B‐cell lymphoma. T‐cell malignancy has rarely been associated with chronic lymph stasis. Here, we report a case of primary cutaneous anaplastic large cell lymphoma (pcALCL) with lymphatic spread associated with chronic lymphedema. The patient is a 56‐year‐old man who received orchiectomy and right inguinal lymphadenectomy for malignant seminoma 10 years ago, which led to prominent lymphedema of the right leg. He developed extensive skin nodules on the lymphedematous area for 3 months. Histopathology findings confirmed a diagnosis of pcALCL, which is a subtype of cutaneous T‐cell lymphoma characterized by the presence of CD30+ T cells. Intralymphatic infiltration of malignant cells is prominent. The pathogenesis of intralymphatic cutaneous anaplastic large cell lymphoma is largely unknown. Our case suggests that chronic lymphedema resulted in persistent CD4+ T‐cell inflammation, which then may contribute to the development of pcALCL.     

ALK-1蛋白阳性的原发性皮肤CD30+间变性大细胞淋巴瘤1例

报告1例ALK-1蛋白阳性的原发性皮肤CD30+间变性大细胞淋巴瘤.患儿女,6岁.因左下眼睑包块5个月就诊.入院检查见左下眼睑一2.5 cm×3.0 cm×0.5 cm红色包块,组织病理学检查示真皮内大片大细胞浸润,细胞核呈间变性;免疫组化染色示UCHL-1(+)、CD30(+)、ALK-1(+),EBER原位杂交为阴性.ALK蛋白表达模式为细胞质与细胞核.回顾相关文献,该例原发性皮肤CD30+间变性大细胞淋巴瘤检测到ALK蛋白阳性表达系国内首例报告.     

Primary cutaneous anaplastic large cell lymphoma successfully treated with local thermotherapy using pocket hand warmers

Apart from for cutaneous deep fungal or mycobacterial infections, thermotherapy has been used for various malignant tumors. We report a case of primary cutaneous anaplastic large cell lymphoma, which responded quite well to topical thermotherapy using chemical pocket hand warmers. The treatment resulted in an immediate tumor regression without recurrence. This method is simple and might be a useful tool against solitary cutaneous lymphoma, especially of elderly patients with poor performance status or with various systemic complications.