Errihani Hassan, Berrada Narjisse, Raissouni Soundouss, Rais Fadoi, Mrabti Hind, Rais Ghizlane
Medical oncology department, National Institute of Oncology, Rabat, Morocco.
BMC Dermatol. 2011 Nov 11;11:15. doi: 10.1186/1471-5945-11-15.
Classic Kaposi's sarcoma (CKS) is a rare disease likely associated with human herpes virus 8 (HHV-8) infection, and occurs predominantly in Jewish, Mediterranean and middle eastern men. There is a dearth of data in Moroccan patients with CKS regarding epidemiology, clinical characteristics and outcomes. This report examines a cohort of patients with CKS evaluated at the national institute of oncology over 11-year period.
A retrospective analysis of patients referred to the national institute of oncology with classical Kaposi sarcoma, between January 1998 and February 2008, was performed. Reviewed information included demographics, clinical and pathological staging, death or last follow-up.
During the study period, 56 patients with a diagnosis of CKS have been referred to our hospital. There were 11 (19.7%) females and 45 (80.3%) males (male-to-female ratio: 4:1). Mean age at diagnosis was 61.7 ± 15 (range: 15-86 years). Nodules and/or plaques were the most frequent type of lesion. The most common location was the lower limbs, particularly the distal lower extremity (90%). In addition to skin involvement, visceral spread was evident in 9 cases. The most common visceral involvement sites were lymph nodes (44%), lung (22%), and gastrointestinal tract (22%). Associated lymphoedema was seen in 24 (42%) of the patients. There were 18 stage I patients (32.14%), 8: stage II (14.28%), 21 stage III (37.5%) and 9 stage IV (16.07%). A second primary malignancy was diagnosed in 6 cases (10.7%), none of the reticuloendothelial system. With a median follow-up of 45 months, 38 (67.8) patients are alive, of whom 25 (65.78%) patients with stable disease, five with progressive disease currently under systemic chemotherapy and 8 (21.05%) are alive and free of disease, over a mean interval of 5 years.
This is the largest reported series in our context. In Morocco, CKS exhibits some special characteristics including a disseminated skin disease at diagnosis especially in men, a more common visceral or lymph node involvement and a less frequent association with second malignancies.
经典型卡波西肉瘤(CKS)是一种罕见疾病,可能与人类疱疹病毒8(HHV - 8)感染有关,主要发生于犹太、地中海和中东男性。关于摩洛哥CKS患者的流行病学、临床特征及预后的数据匮乏。本报告研究了在国家肿瘤研究所接受评估的一组CKS患者,为期11年。
对1998年1月至2008年2月期间转诊至国家肿瘤研究所的经典型卡波西肉瘤患者进行回顾性分析。审查的信息包括人口统计学、临床和病理分期、死亡情况或末次随访。
在研究期间,56例诊断为CKS的患者转诊至我院。其中女性11例(19.7%),男性45例(80.3%)(男女比例为4:1)。诊断时的平均年龄为61.7±15岁(范围:15 - 86岁)。结节和/或斑块是最常见的病变类型。最常见的部位是下肢,尤其是下肢远端(90%)。除皮肤受累外,9例有明显的内脏转移。最常见的内脏受累部位是淋巴结(44%)、肺(22%)和胃肠道(22%)。24例(42%)患者伴有淋巴水肿。有18例I期患者(32.14%),8例II期(14.28%),21例III期(37.5%)和9例IV期(16.07%)。6例(10.7%)诊断出第二原发性恶性肿瘤,均非网状内皮系统肿瘤。中位随访45个月,38例(67.8%)患者存活,其中25例(65.78%)病情稳定,5例病情进展,目前正在接受全身化疗,8例(21.05%)存活且无疾病,平均间隔5年。
这是我们地区报道的最大系列病例。在摩洛哥,CKS表现出一些特殊特征,包括诊断时为播散性皮肤病,尤其是男性,内脏或淋巴结受累更常见,与第二原发性恶性肿瘤的关联较少。