Department of Pathology, Medstar Georgetown University Hospital, Washington, USA.
Department of Medical Genetics, Integrated Oncology, New York, USA.
Balkan Med J. 2018 Mar 15;35(2):203-207. doi: 10.4274/balkanmedj.2017.0796. Epub 2017 Oct 26.
Clear cell sarcoma is a rare malignant soft tissue neoplasm that typically involves tendons and aponeurosis. Clear cell sarcoma in the dermis is an extremely rare occurrence, and it is difficult to differentiate between this neoplasm and dermal malignant melanoma because they have similar morphologic and immunohistochemical features. Although rare, clear cell sarcoma of the skin typically occurs in the extremities. To our knowledge, there are no reported cases of primary clear cell sarcoma of the skin occurring in the neck. Here, we report an unusual case of clear cell sarcoma arising in the skin of the neck.
A 43-year-old female presented with a right neck lesion. Histologic sections of the lesion showed a nodular proliferation of spindle cells with pale cytoplasm with epithelioid features involving the entire dermis with no epidermal component. The tumour cells were positive for melanocytic markers, including S100 and Human Melanoma Black 45, which led to an initial diagnosis of malignant melanoma. Fluorescence hybridization showed a rearrangement of the gene on chromosome 22q12, which led to a diagnosis of primary clear cell sarcoma in the skin.
Because the treatments for clear cell sarcoma and conventional melanoma are different, fluorescence hybridization for should be performed in any dermal lesions with melanocytic features that do not have an component.
透明细胞肉瘤是一种罕见的恶性软组织肿瘤，通常涉及肌腱和腱膜。真皮透明细胞肉瘤极为罕见，由于其具有相似的形态学和免疫组织化学特征，因此很难将该肿瘤与真皮恶性黑色素瘤区分开来。尽管罕见，但皮肤透明细胞肉瘤通常发生在四肢。据我们所知，尚无颈部原发性透明细胞肉瘤的报道。在此，我们报告一例发生在颈部皮肤的不寻常透明细胞肉瘤病例。
一名 43 岁女性出现右侧颈部病变。病变的组织学切片显示，整个真皮层呈结节状增生的梭形细胞，细胞质呈淡色，具有上皮样特征，无表皮成分。肿瘤细胞表达黑色素细胞标志物，包括 S100 和人黑色素瘤黑 45，这导致最初诊断为恶性黑色素瘤。荧光原位杂交显示 22q12 染色体上基因的重排，从而诊断为皮肤原发性透明细胞肉瘤。
由于透明细胞肉瘤和常规黑色素瘤的治疗方法不同，因此对于任何无表皮成分但具有黑色素细胞特征的真皮病变，都应进行 基因的荧光原位杂交检测。
