Chapalain M, Goldman-Lévy G, Kramkimel N, Carlotti A, Franck N, Lheure C, Audard V, Avril M-F, Marcelin A-G, Damotte D, Terris B, Aractingi S, Dupin N
Service de dermatologie, hôpital Cochin, pavillon Tarnier, université Paris Descartes, AP-HP, 89, rue d'Assas, 75006 Paris, France.
Service d'anatomopathologie, hôpital Cochin, AP-HP, université Paris Descartes, 27, rue du Faubourg Saint-Jacques, 75014 Paris, France.
Ann Dermatol Venereol. 2018 Jan;145(1):21-28. doi: 10.1016/j.annder.2017.09.593. Epub 2017 Dec 28.
Anaplastic Kaposi's sarcoma (KS) is a rare form of KS characterized clinically by the development of a tumour mass with unusual local aggressiveness and histologically by a specific architecture and cytological morphology. A very small number of limited series in endemic countries have established characteristics common to these anaplastic forms of KS. We present five patients with an anaplastic form in a context of KS ongoing for several years in a non-endemic country.
We collected 5 cases of anaplastic KS followed in our department over a period of 20years. We describe the main developmental, clinical, virological and histological features.
The cases involved 4 men and 1 woman whose mean age at diagnosis of anaplastic KD was 70years, with an average time of 25years between initial diagnosis of KD and anaplastic transformation. Our patients were all treated with chemotherapy and/or radiotherapy (RT) prior to diagnosis of anaplastic transformation. All patients had a tumour mass of the lower limbs developing in classically indolent KS with associated chronic lymphoedema. Progression was very aggressive locally with deep invasion of the soft tissues as well as osteoarticular involvement, without visceral dissemination. At present, three patients are dead, one patient is showing partial response, and one patient is in locoregional progression. Diagnosis of the disease was based on histopathological findings. The tumour cells were undifferentiated, pseudo-cohesive, and chiefly organized in sheets. The mitotic count was high (27 mitoses per 10 fields at high magnification). Necrosis was constant.
To our knowledge, this is the first series describing anaplastic Kaposi's sarcoma in a non-endemic country. The severity of the prognosis, despite the absence of visceral dissemination, is related to the local aggressiveness of anaplastic KS and to its resistance to radiotherapy and chemotherapy, with amputation being required in certain cases.
间变性卡波西肉瘤（KS）是KS的一种罕见形式，临床特征为出现具有异常局部侵袭性的肿瘤块，组织学特征为特定的结构和细胞学形态。在地方性流行国家，仅有极少数有限的系列研究确定了这些间变性KS形式的共同特征。我们报告了在一个非地方性流行国家中，5例患有持续数年的KS背景下的间变性形式的患者。
我们收集了20年间在我科随访的5例间变性KS病例。我们描述了其主要的发病、临床、病毒学和组织学特征。
病例包括4名男性和1名女性，诊断间变性KS时的平均年龄为70岁，从最初诊断KS到间变性转化的平均时间为25年。我们的患者在诊断间变性转化之前均接受了化疗和/或放疗（RT）。所有患者的下肢均出现肿瘤块，发生于典型的惰性KS并伴有慢性淋巴水肿。局部进展非常迅速，软组织深层浸润以及骨关节受累，但无内脏播散。目前，3例患者死亡，1例患者显示部分缓解，1例患者处于局部区域进展期。疾病诊断基于组织病理学发现。肿瘤细胞未分化，假黏附性，主要呈片状排列。有丝分裂计数高（高倍镜下每10个视野有27个有丝分裂）。坏死常见。
据我们所知，这是首个描述非地方性流行国家中间变性卡波西肉瘤的系列研究。尽管没有内脏播散，但预后严重程度与间变性KS的局部侵袭性及其对放疗和化疗的耐药性有关，某些情况下需要截肢。
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