Limaiem F, Chelly I, Bellil S, Mekni A, Nidhameddine K, Haouet S, Bellil K, Zitouna M
Department of Pathology, Hospital La Rabta, 1007 Bab Saâdoun, Tunis, Tunisia.
Pathologica. 2007 Dec;99(6):415-9.
Primary cutaneous leiomyosarcoma is an uncommon malignant neoplasm with a predilection for the lower extremities. A retrospective study of 4 cases was undertaken to analyse the clinicopathological characteristics and immunohistochemical profile of these neoplasms with emphasis on prognosis. Two male and 2 female patients aged between 49 and 80 years presented with painless tumours involving the lower lip, the chin, the scrotum and the shoulder. Histological examination of the initial biopsy specimen established a diagnosis of cutaneous leiomyosarcoma. All cases co-expressed smooth muscle actin and vimentin regardless of primary tumour site. Wide surgical excision of the tumour was performed in only 3 cases, and the remaining patient refused further treatment. Of the patients undergoing surgical intervention, local recurrence occurred in one case. No metastases were observed. Long-term follow-up of patients with cutaneous leiomyosarcoma is mandatory to detect local recurrence and distant metastases that can occur even years after the initial excision.
原发性皮肤平滑肌肉瘤是一种罕见的恶性肿瘤,好发于下肢。对4例患者进行了回顾性研究,以分析这些肿瘤的临床病理特征和免疫组化特征,并着重探讨预后情况。2例男性和2例女性患者,年龄在49至80岁之间,表现为无痛性肿瘤,累及下唇、下巴、阴囊和肩部。对初始活检标本进行组织学检查确诊为皮肤平滑肌肉瘤。所有病例均共同表达平滑肌肌动蛋白和波形蛋白,无论原发肿瘤部位如何。仅3例患者进行了肿瘤的广泛手术切除,其余患者拒绝进一步治疗。接受手术干预的患者中,1例发生局部复发。未观察到转移情况。对皮肤平滑肌肉瘤患者进行长期随访是必要的,以便发现甚至在初始切除数年之后仍可能发生的局部复发和远处转移。