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Case Reports
. 1997 Dec;81(12):1084-8.
doi: 10.1136/bjo.81.12.1084.

Orbital granulocytic sarcoma

F A Stockl  1 , A M Dolmetsch, M A Saornil, R L Font, M N Burnier Jr
Affiliations
Case Reports

Orbital granulocytic sarcoma

F A Stockl et al. Br J Ophthalmol. 1997 Dec.

Abstract

Aim: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

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Figures

Figure 1
Figure 1
(A) Nine year old boy with proptotic right globe. (B) Histopathological section of orbital tumour showing malignant proliferation invading fat (haematoxylin and eosin, ×26). (C) High magnification showing immunoreactive cells (arrow) using lysozyme antibody (anti-lysozyme, ×128). (D) Positive immunohistochemical stain (arrow) of the orbital tumour using MAC387 (MAC387 stain, ×104).
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