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- 2012年第13期—左侧臀部肿物
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该例来自某次读片会，相关权益归原单位所有。
病史：年龄性别均不详。左侧臀部皮下可扪及一约4*3cm肿块，质硬，无压痛，边界尚清。
大体：不整形组织一块，约5*5*2cm，切面暗红，质中
本例图片采用麦克奥迪MoticBA410显微镜+MoticPro285A摄像头采集制作。
点评专家：南京医科大学一附院 范钦和老师
专家介绍：http://teach.ipathology.cn/article/3548.html
获奖名单：待定
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本帖最后由 草原 于 2012-04-26 09:36:25 编辑
- 赚点散碎银子养家，乐呵呵的穿衣吃饭
×参考诊断
- PMID:
- 21915027
- [PubMed - indexed for MEDLINE]
诊断：乳头状淋巴管内血管内皮瘤/网状血管内皮瘤 (PAPILLARY INTRALYMPHATIC ANGIOENDOTHELIOMA AND RETIFORM HEMANGIOENDOTHELIOMAS）向上皮样血管肉瘤转化。
理由：1、皮下肿物
2、不规则扩张淋巴管样腔隙，内衬hobnail样肿瘤细胞，有乳头形成
3、腔内可见淋巴液样结构，周围有灶性淋巴细胞浸润
4，出现片状实性生长区域，浸润性生长，细胞异型性明显，可见病理性和分裂及肿瘤性坏死。
免疫组化：CD31，CD34，D2-40，Viii因子
鉴别诊断：恶黑，转移癌
参考文献：Am J Dermatopathol. 2011 Oct;33(7):e84-7.
Retiform hemangioendothelioma developed on the site of an earlier cystic lymphangioma in a six-year-old girl.
Source
Department of Pathology, Necker-Enfants Malades Hospital, APHP, Université Paris Descartes, Paris, France.
Abstract
Retiform hemangioendothelioma (RH) is a rare low-grade malignancy angiosarcoma, with a high rate of local recurrence and a low metastatic risk. A 6 year-old girl with a large cervical cystic lymphangioma diagnosed by ultrasound and Doppler ultrasound, which showed a large multiloculated anechoic cyst with no flow. The lymphangioma was treated with injections of Picibanil (OK-432). The tumor regressed, but after a year, she developed a poorly limited infiltrated plaque spreading out regularly over her chest, back, and shoulder. The biopsy showed a poorly limited dermal and subcutaneous vascular proliferation composed of elongated arborising vessels lined with ovoid endothelial cells in a hobnail pattern. In addition, the deep part of the lesion showed typical features of a papillary intralymphatic angioendothelioma pattern (PILA) or Dabska tumor. The endothelial cells strongly expressed podoplanin (D2-40). A diagnosis of RH with focal areas of PILA was reached. The girl died 8 months after surgery of hypovolemic shock in a context of diffuse lymphangiomatosis with pulmonary localization. To our knowledge, RH has hardly ever been described in children. This entity exhibits a continuum with the PILA, sharing not only morphological and immunohistochemical similarities but also its ability to develop in a context of a vascular anomaly, particularly a lymphangioma. The role of Picibanil in the development of this tumor can be discussed.
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恶性肿瘤，首先考虑为组织细胞肉瘤。
诊断依据：1.臀部皮下肿物，肿瘤呈多结节状；2.上皮样肿瘤细胞，实性型排列，有的呈腺样，甚至囊性结构，肿瘤细胞体积大，胞浆较丰富，大部分嗜酸性，少量胞浆透明、空泡变性，如脂肪母细胞样（15图），双核瘤细胞较多见，核大多偏位，如浆细胞样、横纹肌母细胞样，但未见明显包涵体，有核仁，核膜增厚不规则，核分裂象很惹眼，总体觉得属于恶性程度高的肿瘤，浸润周围脂肪等结缔组织，伴有灶性坏死，个人觉得单用中间性偶有转移性性质的血管瘤样纤维组织细胞瘤难以解释这个病变；3.13图中个别瘤细胞吞噬淋巴细胞；4.周围有淋巴组织，未免联想到淋巴造血组织肿瘤，如组织细胞肉瘤、树突状细胞肉瘤、髓系肉瘤等。
鉴别诊断：恶黑；近心型上皮样肉瘤；恶性间皮瘤；横纹肌肉瘤；髓外浆细胞瘤；髓系肉瘤；上皮样血管肉瘤；滑膜肉瘤；树突状细胞肉瘤；转移性肿瘤：如肾上腺皮质癌、支持细胞/间质细胞源性恶性肿瘤等。需要逐一排除！
