Isolated granulocytic sarcoma of the orbit

Isolated granulocytic sarcoma of the orbit

A 10-year-old boy presented with progressive proptosis

of the left globe for the last 8 months. He had no consti-

tutional symptoms. On examination, he had a large mass

involving the left orbit. Rest of the physical examination

was unremarkable. Computed tomography (CT) and

magnetic resonance imaging of the orbit revealed a large

enhancing mass located in the left orbit, causing propto-

sis with invasion into the left ethmoid and maxillary

sinuses with some destruction of the intervening bones.

Axial [(18)F] fluorodeoxyglucose positron emission tomo-

graphy (FDG PET) CT (Fig. 1A) revealed that the mass

was hypermetabolic.

A complete haemogram and bone marrow biopsy was

normal. Fine needle aspiration cytology and biopsy

(Fig. 2) of the mass revealed an infiltrating neoplasm

composed of small round cells that were positive for leu-

cocyte common antigen and myeloperoxidase. Thus, a

diagnosis of isolated orbital granulocytic sarcoma was

made. Repeat FDG PET CT (Fig. 1B) after 1 month of

induction chemotherapy (daunomycin and cytosine ara-

binoside) and first consolidation therapy (high-dose cyto-

sine arabinoside 18 g/m2) revealed marked decrease in

the size of the mass as well as FDG avidity. The patient

was given two additional courses of consolidation with

high-dose cytosine arabinoside followed by local radio-

therapy (24 Gy over 12 fractions). Patient is in remission

at 3-month follow-up after completion of therapy.

Our case is unique in providing exquisite pre- and

post-chemotherapy FDG PET CT images.

Jyoti Kumar1, Ashu Seith1, Sameer Bakhshi2,Rakesh Kumar3, Atin Kumar1, Seema Sen4

Departments of 1Radiology, 2Medical Oncology,3Nuclear Medicine and 4Pathology, All India

Institute of Medical Sciences, New Delhi, India

Correspondence Dr Ashu Seith, MD, Associate

Professor, Department of Radiology, All India Institute

of Medical Sciences, New Delhi 110 029, India.

Tel: 91 11 26593326; Fax: 91 11 26588663;

e-mail: ashubhalla1@yahoo.com

A B

Figure 1 (A) Axial FDG PET CT images of the orbit reveal a large hypermetabolic mass in the left orbit extending into the ipsilateral maxillary

sinus with bone destruction. (B) Repeat FDG PET CT images of the orbit after 1 month of induction chemotherapy revealed dramatic decrease in

the size of the mass as well as FDG avidity.

Figure 2 Biopsy shows large round to oval cells with irregular nuclei

and indistinct cytoplasmic outlines (H&E ·400).

doi:10.1111/j.1600-0609.2007.00830.x European Journal of Haematology ISSN 0902-4441

CLINICAL PICTURE

456ª 2007 The Authors

Journal compilation 78 (456) ª 2007 Blackwell Munksgaard