Murugan Paari, Basu Debdatta, Kumar Surendra, Jagadish Sadasivan
Dept. of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India.
Cytojournal. 2007 Jul 15;4:15. doi: 10.1186/1742-6413-4-15.
Clear cell sarcoma of soft parts is most commonly found associated with the tendons and aponeuroses of distal extremities in young adults with a peak incidence in the third decade. Location in the abdominal wall and in a child is very rare.
A nine-year-old female child presented with a swelling in the anterior abdominal wall in the suprapubic region. Fine needle aspiration revealed predominantly discrete cells with loose clustering at places. The cytoplasm was abundant, finely granular, and eosinophilic with some cells exhibiting clear vacuolated zones. No pigment was seen. The nuclei were rounded and eccentrically placed with a striking single eosinophilic macro nucleolus present in all the cells. Taking into consideration, the history, age of the patient, location of the tumor and absence of melanin pigment, a diagnosis of soft tissue sarcoma was made, the differential including Clear cell sarcoma. This was subsequently confirmed on histopathological examination and immunohistochemistry
The atypical presentation of the case made the cytological diagnosis rather challenging. Clear cell sarcoma should be considered when cytology of a soft-tissue tumor shows uncharacteristically high cellularity and relatively uniform cells with macronucleoli.
软组织透明细胞肉瘤最常见于年轻成年人四肢远端的肌腱和腱膜,发病高峰在第三个十年。位于腹壁且发生于儿童的情况非常罕见。
一名9岁女童,耻骨上区前腹壁出现肿胀。细针穿刺显示主要为离散细胞,部分地方有松散聚集。细胞质丰富,呈细颗粒状,嗜酸性,部分细胞有透明空泡区。未见色素。细胞核圆形,偏心位,所有细胞均有显著的单个嗜酸性大核仁。考虑到患者的病史、年龄、肿瘤位置以及无黑色素沉着,诊断为软组织肉瘤,鉴别诊断包括透明细胞肉瘤。随后经组织病理学检查和免疫组化得以证实。
该病例的非典型表现使细胞学诊断颇具挑战性。当软组织肿瘤的细胞学表现为细胞数量异常增多且细胞相对均匀、有大核仁时,应考虑透明细胞肉瘤。