Guerriero Silvana, Sborgia Alessandra, Giancipoli Giovanni, Fiore Maria Grazia, Ross Roberta, Piscitelli Domenico
Department of Ophthalmology and ORL, University of Bari, Italy.
Orbit. 2011 Jun;30(3):169-71. doi: 10.3109/01676830.2011.574771.
To describe a rare case of conjunctival leiomyosarcoma initially diagnosed as a poorly differentiated squamous cell carcinoma.
Clinical, light microscopic, immunohistochemical, and ultrastructural findings are reported.
A 56-year-old Caucasian woman was referred with a history of a progressive, rapidly growing mass in her left eye. Biopsy of the mass and histology yielded a first diagnosis of a poorly differentiated conjunctival squamous cell carcinoma. Orbital exenteration was performed 2 weeks later. Macroscopically, the exenteration specimen showed a soft mass completely involving the conjunctiva and extending to the eyelids and orbital structures. Histological examination revealed a malignant tumour composed of atypical, predominantly epithelioid large cells. Immunohistochemical and ultrastructural studies combined with the light microscopic findings contributed to clarify the diagnosis of epithelioid leiomyosarcoma. The patient was started on chemotherapy and radiotherapy, but died a few months later from widespread metastases.
primary involvement of the orbit by a leiomyosarcoma is rare, but this eventuality should be considered in the differential diagnosis of rapidly growing orbital and conjunctival masses.
描述一例最初被诊断为低分化鳞状细胞癌的结膜平滑肌肉瘤罕见病例。
报告临床、光镜、免疫组化及超微结构检查结果。
一名56岁的白种女性因左眼有一进行性快速生长肿物的病史前来就诊。肿物活检及组织学检查最初诊断为低分化结膜鳞状细胞癌。两周后行眼眶内容物剜除术。肉眼观察，剜除标本显示一柔软肿物，完全累及结膜并延伸至眼睑及眼眶结构。组织学检查显示为一由非典型、主要为上皮样大细胞组成的恶性肿瘤。免疫组化及超微结构研究结合光镜检查结果有助于明确上皮样平滑肌肉瘤的诊断。患者开始接受化疗及放疗，但数月后因广泛转移死亡。
平滑肌肉瘤原发累及眼眶罕见，但在快速生长的眼眶及结膜肿物的鉴别诊断中应考虑到这种可能性。
