Dermatology Department, Hospital de Santa Maria, Centro Hospitalar e Universitário de Lisboa Norte, Lisbon, Portugal.
Faculty of Medicine, Universidade de Lisboa, Lisbon, Portugal.
An Bras Dermatol. 2021 May-Jun;96(3):278-283. doi: 10.1016/j.abd.2020.10.003. Epub 2021 Mar 21.
Cutaneous leiomyosarcoma is a rare malignant neoplasm with muscular origin, representing 2%-3% of all cutaneous soft tissue sarcomas.
The aim of this study was to characterize clinicopathological features of patients diagnosed with cutaneous leiomyosarcoma in our center over the last 20-years.
A retrospective study of patients with a histopathological diagnosis of leiomyosarcoma between 1999 and 2018 was conducted.
Eleven patients were diagnosed with cutaneous leiomyosarcoma during this period. Most cases occurred in men (n = 7). Age at presentation ranged from 47 to 92 years (mean 64.9 years). Head and neck were the most frequently involved locations (n = 5). Ten leiomyosarcoma were dermal, with one cutaneous metastasis. Immunohistochemical staining was available for 7 patients, demonstrating positivity for smooth muscle actin in all of them. All neoplasms were treated surgically. Mean survival was 32.2-months.
This was a retrospective study based on medical and pathological records.
Histopathology is essential for the diagnosis of leiomyosarcoma, usually revealing a dermal or subcutaneous lesion composed of intertwined fascicles of smooth muscle fibers. Immunohistochemistry is then used to adequately differentiate leiomyosarcoma from other spindle cell tumors. When dealing with cutaneous leiomyosarcoma, it is advisable to carefully evaluate the depth of subcutaneous extension, since even minimal subcutaneous involvement may be associated with a poorer prognosis.
皮肤平滑肌肉瘤是一种罕见的起源于肌肉的恶性肿瘤,占所有皮肤软组织肉瘤的 2%-3%。
本研究旨在描述过去 20 年来在我们中心诊断为皮肤平滑肌肉瘤患者的临床病理特征。
对 1999 年至 2018 年间病理诊断为平滑肌肉瘤的患者进行回顾性研究。
在此期间,共诊断出 11 例皮肤平滑肌肉瘤患者。大多数病例发生于男性(n=7)。发病年龄为 47 岁至 92 岁(平均 64.9 岁)。头颈部是最常受累的部位(n=5)。10 例平滑肌肉瘤为真皮型,其中 1 例为皮肤转移。对 7 例患者进行了免疫组织化学染色,所有患者的平滑肌肌动蛋白均呈阳性。所有肿瘤均行手术治疗。平均生存时间为 32.2 个月。
这是一项基于医疗和病理记录的回顾性研究。
组织病理学对于平滑肌肉瘤的诊断至关重要,通常表现为真皮或皮下有相互交织的平滑肌纤维束。免疫组织化学随后用于充分区分平滑肌肉瘤和其他梭形细胞肿瘤。在处理皮肤平滑肌肉瘤时,建议仔细评估皮下延伸的深度,因为即使是最小的皮下受累也可能与预后较差相关。