Case report of cutaneous histiocytic sarcoma: diagnostic and therapeutic dilemmas

Histiocytic sarcoma is a rare hematologic malignant neoplasia originating from histiocytic or dendritic cell clones. The lesions may be in nodal or extranodal sites, most commonly in the gastrointestinal tract. A small number of cases presents as unique cutaneous lesions. The definitive diagnosis is made by positivity for the immunohistochemical markers CD163, CD68, CD4 and lysozyme. The treatment is controversial, often with combined systemic chemotherapy. This is a case of cutaneous histiocytic sarcoma in an 82-year-old patient presenting two nodular lesions in the breast and right arm which were treated with simple excision and multidisciplinary follow-up, avoiding aggressive management and exhaustive investigations. Although most studies report aggressive evolution, the patient had good and stable clinical status during the twelve-month follow-up period.     

Nodular localized primary cutaneous amyloidosis: a long-term follow-up study

We present long-term follow-up data on patients with nodular localized primary cutaneous amyloidosis (NLPCA) seen at the St John's Institute of Dermatology between 1968 and 1999. This is the largest clinical follow-up study of this type of amyloid to date. Based on these cases we estimate the rate of progression of NLPCA to systemic amyloidosis to be only 7%, much lower than the 50% rate currently quoted in the literature.     

原发性皮肤间变性大细胞淋巴瘤4例

目的:探讨原发性皮肤间变性大细胞淋巴瘤(ALCL)临床及病理特征。方法:复习4例患者的临床资料、肿瘤组织的病理形态和免疫表型。结果:4例中男3例,女1例,平均年龄为55.5岁。患者临床上均表现为皮下结节。光镜下以75%以上CD30 间变性大细胞弥漫浸润真皮及皮下脂肪组织为特征,多数瘤细胞表达T细胞免疫表型。随访3例12~36个月肿瘤均无复发或转移。结论:原发性皮肤ALCL是少见的低度恶性T细胞性淋巴瘤,预后较好。依据其组织病理学特征及免疫组化CD30阳性标记,可与其他恶性肿瘤进行鉴别。     

Primary cutaneous plasmacytoma: a clinicopathological study of two cases with a long-term follow-up and review of the literature

BACKGROUND: Primary cutaneous plasmacytoma (PCP) is a rare type of cutaneous B-cell lymphoma arising primarily in the skin and derived from clonally expanded plasma cells with a various degrees of maturation and atypia. The disease is rare with only 30 cases reported so far. METHODS: Two cases of PCP with long-term follow-up of 17 and 15 years are presented. RESULTS AND CONCLUSIONS: Both patients were men with nodular lesions on the face. Histologically, the lesions were composed predominantly of variably maturated plasma cells with monotypic expression of immunoglobulin (Ig) lambda chains. Polymerase chain reaction for IgH genes did not reveal clonal rearrangement. Our cases are discussed in the context of previously reported cases of PCP with a long-term follow-up. We also include a review of all cases of PCP with known tumor progression earlier in the course of the disease (local relapse or visceral spread) to determine the clinical course of this primary cutaneous lymphoma.     

新生隐球菌不同变种所致小鼠原发性皮肤感染的比较

目的：探讨新生隐球菌不同变种在原发性小鼠新生隐球菌皮肤感染中的作用。方法：按照我们建立的原发性皮肤隐球菌感染模型的方法，将新生隐球菌新生变种标准野生株B3501与格特变种标准株ATCC32609分别皮内接种于免疫抑制与非抑制的BALB／c小鼠，皮损真菌培养与组织病理检查确证感染。观察2种隐球菌感染的病程，比较皮损形成与消退的平均时间。结果：2种变种的新生隐球菌皮下接种于BALB／c小鼠后，可以在免疫抑制与非抑制的BALB／c小鼠皮肤上产生丘疹、结节、溃疡、传染性软疣样皮损，皮损可以自愈，真菌培养与病理确证为隐球菌感染。2种菌株只在免疫正常小鼠的皮损形成时间上存在差异。结论：新生变种与格特变种的新生隐球菌均可以造成BALB／c小鼠相似的皮肤感染。推测2种变种对原发性皮肤感染的致病力可能无差异，新生变种发病较多可能与其分布有关。     

Primary cutaneous perivascular epithelioid cell tumor (PEComa): Five new cases and review of the literature

PEComas represent a family of uncommon mesenchymal tumors composed of “perivascular epithelioid cells” with a distinct immunophenotype that typically shows both myogenic and melanocytic differentiation. The PEComa family includes angiomyolipoma (AML), clear cell “sugar” tumor of the lung and extra pulmonary sites, lymphangioleiomyomatosis and clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres. Very rarely, PEComas may arise in the skin. Primary cutaneous PEComas typically display a dermal proliferation of epithelioid cells with pale, clear, or granular pink cytoplasm arranged in nests and trabecula with an intervening arborizing network of delicate capillaries. Primary cutaneous PEComas have a lower frequency of myogenic marker expression than their deep soft tissue and visceral counterparts. They also often express strong diffuse CD10, leading to potential confusion with metastatic renal cell carcinoma. Most cases behave indolently. We report 5 additional cases of this rare entity. All showed classic histologic features and expression of either HMB‐45 and/or Melan‐A/MART‐1. Four cases were tested for myogenic markers (2 were positive & 2 were negative). Three cases were tested for CD10 (all 3 were positive). All of our cases with clinical follow‐up behaved indolently. Table 1 provides a summary of findings for all 5 cases in our series.     

原发性皮肤边缘区B细胞淋巴瘤组织病理特征和免疫表型分析

目的：研究原发性皮肤边缘区B细胞淋巴瘤（primary cutaneous marginal zone lymphomas,PCMZLs）的临床及组织病理特点和免疫表型特征。方法：收集15例PCMZL,分析临床及组织病理特点,并用免疫组化检测CD21、BCL2、BCL10及B细胞标记CD20和CD79a等。结果：15例PCMZL中女9例、男6例,平均年龄61．2岁,中位年龄58岁。11例发生在头面部,其中1例为复发性,3例在背部,1例为全身多发。均表现为皮肤红斑性结节。苏木精-伊红染色为真皮内弥漫淋巴样细胞浸润,10例可见反应性淋巴滤泡或呈模糊的结节。免疫标记显示肿瘤细胞标记CD20（＋）,CD79a（＋）,BCL2（＋）,BCL10（＋）,5例CD43（＋）,CD5、CD10均（-）,CD21可显示淋巴滤泡形态。结论：PCMZL常见于头面部,老年女性多见,细胞形态学特点和BCL2和BCL10免疫组化染色对皮肤淋巴组织增牛病变有诊断和答别作用。     

Primary cutaneous ganglioneuroma: anatomico‐clinical study of 4 cases with focus on Merkel cells

Cutaneous ganglioneuromas (CGNs) are exceptional. We aim to describe the anatomico‐clinical profile of primary CGN and report 4 cases. Patients were 2 men and 2 women aged 53 to 76 years, who had flesh‐colored nodules on the back, associated with adjacent keratotic changes, that is, epidermal nevus (1 case) or seborrheic keratosis (3 cases). Histopathology showed ganglion cells within a proliferation of Schwann cells. The epidermis was acanthotic, associated with sebaceous induction in 2 cases, with follicular hyperplasia as in fibroepithelial tumors (1 case) or with tricholemmoma (2 cases). Cytokeratin‐20 immunostaining showed Merkel cells in the epidermis. A higher density of Merkel cells was observed in BerEP4+ follicular structures. Along with 16 published cases, our study indicates that a nodule associated with seborrheic keratosis on the back may represent a CGN, a complex mesenchymal and epidermal/follicular lesion of neuroectodermal lineage, associating neuronal proliferation and Merkel cell hyperplasia with follicular induction.