Cutaneous Leiomyosarcoma of the Face

Eric E. Santos, D. Sarma
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Abstract

We are reporting a case of cutaneous leiomyosarcoma occurring on the face of a 98-year-old woman with a brief review of the literature. SOURCE OF SUPPORT None REPORT OF A CASE A 98-year-old woman presented with a painful, nodular skin lesion localized to her left cheek. The skin surface was raised and dark brown to black. She reported no other associated symptoms and denied having other similar skin lesions. Her past medical history was otherwise unremarkable. On microscopic examination of the shave biopsy, the skin lesion showed a diffuse nodular proliferation of spindle cells with moderate cellular pleomorphism and an increased mitotic rate of up to 10 mitoses per high power field. The overlying epidermis was unremarkable (Figures 1 & 2). Figure 1 Figure 1: Cutaneous leiomyosarcoma, low magnification: Dermal proliferation of interlacing fascicles of spindle cells. Figure 2 Figure 2: Cutaneous leiomyosarcoma, high magnification. Multiple immunohistochemical stains were performed that showed positive tumor cell staining for vimentin and smooth muscle actin (Figure 3), and negative staining for S-100, CD34, and MelanA. The lesion was interpreted as a leiomyosarcoma. A complete excision was recommended. Cutaneous Leiomyosarcoma of the Face 2 of 3 Figure 3 Figure 3: Cutaneous leiomyosarcoma, Focal positive staining with SMA (smooth muscle actin). COMMENT Primary cutaneous leiomyosarcomas are rare neoplasms, either located in the dermis or subcutaneous tissue. More than 100 cases of dermal leiomyosarcomas have been reported [1], most occurring in patients usually around the sixth decade of life. Our patient probably represents one of the oldest patients with this lesion. Most of the lesions are located in the extremities. We could only uncover one report of dermal leiomyosarcoma occurring in the face [2]. Although the lesion was localized to the dermis in this case, skin leiomyosarcomas can also appear within the subcutis. Surgical excision is the treatment of choice. High rates of local recurrence for both cutaneous and subcutaneous lesions ranging from 50% to 70% have been previously reported. Histological factors that have been reported to predict recurrence rate include size of lesion, increased mitotic count, and tumor extension to the subcutis. CORRESPONDENCE TO Deba P. Sarma, MD Department of Pathology Creighton University Medical Center Omaha, NE 68131 Tel: 402-449-4951 E-mail: debasarma@creighton.edu References 1. Weedon, D. Skin Pathology. 2nd ed. Edinburgh: Churchill Livingston, 2002. 971. 2. Orellana-Diaz, O, and E Hernandez-Perez. "Leiomyoma Cuis and Leiomyosarcoma: a 10-Year Study and a Short Review." Journal of Dermatology and Surgical Oncology 9 (1983): 283-287. Cutaneous Leiomyosarcoma of the Face 3 of 3 Author Information Eric E. Santos, M.D. Senior Resident, Department of Pathology, Creighton University Medical School Deba P. Sarma, M.D. Professor, Department of Pathology, Creighton University Medical School
面部皮肤平滑肌肉瘤
我们报告一例皮肤平滑肌肉瘤发生在脸上的一个98岁的妇女简要回顾文献。支持来源无病例报告一名98岁女性表现为疼痛,结节性皮肤病变定位于她的左脸颊。皮肤表面凸起,呈深褐色至黑色。她没有报告其他相关症状,并否认有其他类似的皮肤病变。除此之外,她的既往病史没有什么特别之处。在皮肤活检的显微镜检查中,皮肤病变显示梭形细胞弥漫性结节增生,细胞多形性中等,有丝分裂率增加,每高倍视场可达10次。图1:皮肤平滑肌肉瘤,低倍镜:纺锤细胞交错束状真皮增生。图2:皮肤平滑肌肉瘤,高倍镜。多次免疫组化染色显示肿瘤细胞vimentin和平滑肌肌动蛋白阳性(图3),S-100、CD34和MelanA阴性。病变被解释为平滑肌肉瘤。建议完全切除。图3:皮肤平滑肌肉瘤,局灶性SMA(平滑肌肌动蛋白)阳性染色。原发性皮肤平滑肌肉瘤是一种罕见的肿瘤,位于真皮或皮下组织。皮肤平滑肌肉瘤已经报道了100多例[1],大多数患者通常发生在60岁左右。我们的病人可能是最老的有这种病变的病人之一。大多数病变位于四肢。我们只能发现一例发生在面部的真皮平滑肌肉瘤[2]。虽然本例病变局限于真皮层,但皮肤平滑肌肉瘤也可出现在皮下。手术切除是治疗的首选。皮肤和皮下病变的局部复发率高,从50%到70%,以前有报道。据报道,预测复发率的组织学因素包括病变大小、有丝分裂计数增加和肿瘤向皮下扩散。通信Deba P. Sarma,医学博士病理学科克雷顿大学医学中心奥马哈,ne68131电话:402-449-4951电子邮件:debasarma@creighton.edu《皮肤病理学》第2版。爱丁堡:丘吉尔利文斯顿出版社,2002年。971. 2. Orellana-Diaz, O和E Hernandez-Perez。平滑肌瘤和平滑肌肉瘤:10年研究和简短回顾。皮肤外科肿瘤学杂志(1983):283-287。面部皮肤平滑肌肉瘤3 / 3作者信息Eric E. Santos,医学博士,克莱顿大学医学院病理系高级住院医师Deba P. Sarma,医学博士,克莱顿大学医学院病理系教授
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