Stockl F A, Dolmetsch A M, Saornil M A, Font R L, Burnier M N
Department of Ophthalmology, McGill University, Montreal, Quebec, Canada.
Br J Ophthalmol. 1997 Dec;81(12):1084-8. doi: 10.1136/bjo.81.12.1084.
Orbital granulocytic sarcoma is a localised tumour composed of cells of myeloid origin. Histological diagnosis can be difficult in patients with poorly differentiated orbital tumours and no evidence of systemic leukaemia. The naphthol AS-D chloracetate esterase (Leder stain) and immunohistochemical stains for lysozyme and MAC387 were used to determine the staining characteristics of these tumours. A case series of seven patients with orbital granulocytic sarcoma is presented.
Seven patients with orbital granulocytic sarcoma were studied. Haematoxylin and eosin, Leder, and lysozyme stained sections were available in seven cases. Unstained formalin fixed paraffin embedded sections of seven cases were available for immunohistochemical evaluation using the avidin-biotin-complex technique for MAC387.
The mean age of presentation of the orbital tumour was 8.8 years. Four patients presented with an orbital tumour before any systemic manifestations of leukaemia. In two cases the diagnosis of the orbital tumour and systemic leukaemia was made simultaneously. There was one case of established systemic myeloid leukaemia in remission with the subsequent development of orbital granulocytic sarcoma. Six of seven cases (86%) were positive for the Leder stain. Five of seven cases (71%) showed positive immunoreactivity with lysozyme. The immunohistochemical stain for MAC387 was positive in all seven cases (100%) including one case that was negative for both lysozyme and Leder stains.
Orbital granulocytic sarcoma is a tumour that affects children and can present with rapidly progressive proptosis. This tumour may develop before, during, or after the occurrence of systemic leukaemia. The combination of Leder and lysozyme stains is useful in the diagnosis of orbital granulocytic sarcoma. MAC387 may be a more reliable marker for orbital granulocytic sarcoma.
眼眶粒细胞肉瘤是一种由髓系起源细胞组成的局限性肿瘤。对于低分化眼眶肿瘤且无系统性白血病证据的患者，组织学诊断可能具有挑战性。采用萘酚AS-D氯乙酸酯酶（Leder染色）以及溶菌酶和MAC387的免疫组织化学染色来确定这些肿瘤的染色特征。本文报告了7例眼眶粒细胞肉瘤患者的病例系列。
对7例眼眶粒细胞肉瘤患者进行研究。7例均有苏木精-伊红染色、Leder染色及溶菌酶染色切片。7例未染色的福尔马林固定石蜡包埋切片可用于采用抗生物素蛋白-生物素复合物技术检测MAC387的免疫组织化学评估。
眼眶肿瘤出现时的平均年龄为8.8岁。4例患者在白血病出现任何全身表现之前就出现了眼眶肿瘤。2例患者眼眶肿瘤和系统性白血病的诊断同时做出。有1例系统性髓系白血病处于缓解期，随后发生了眼眶粒细胞肉瘤。7例中有6例（86%）Leder染色呈阳性。7例中有5例（71%）溶菌酶免疫反应呈阳性。MAC387的免疫组织化学染色在所有7例（100%）中均呈阳性，包括1例溶菌酶和Leder染色均为阴性的病例。
眼眶粒细胞肉瘤是一种影响儿童的肿瘤，可表现为迅速进展的眼球突出。这种肿瘤可能在系统性白血病发生之前、期间或之后出现。Leder染色和溶菌酶染色联合应用有助于眼眶粒细胞肉瘤的诊断。MAC387可能是眼眶粒细胞肉瘤更可靠的标志物。
