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Year : 2017  |  Volume : 32  |  Issue : 1  |  Page : 59-60  

Ocular granulocytic sarcoma as an initial clinical presentation of acute myeloid leukemia identified on flurodeoxyglucose positron emission tomography/computed tomography


Department of Nuclear Medicine and Molecular Imaging, Tata Memorial Hospital, Parel, Mumbai, Maharashtra, India

Date of Web Publication17-Jan-2017

Correspondence Address:
Venkatesh Rangarajan
Department of Nuclear Medicine and Molecular Imaging, Tata Memorial Hospital, E. Borges Road, Parel, Mumbai Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-3919.198485

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   Abstract 

Granulocytic sarcoma (GS) or chloroma, rare extramedullary manifestation of acute myeloid leukemia and not infrequently, can be presenting clinical feature. Multiple studies have demonstrated the clinical utility of fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) in early detection and follow-up assessment of GS after chemotherapy. Commonly involved areas include bones, lymph nodes, breasts, and skin and not uncommonly, the disease can be multifocal. We present a rare case of ocular GS, where FDG-PET/CT in addition to the identifying the ocular mass, revealed multiple clinically occult extramedullary lesions.

Keywords: acute myeloid leukemia, granulocytic sarcoma, ocular, flurodeoxyglucose positron emission tomography/computed tomography, FDG


How to cite this article:
Chandra P, Purandare N, Shah S, Agrawal A, Rangarajan V. Ocular granulocytic sarcoma as an initial clinical presentation of acute myeloid leukemia identified on flurodeoxyglucose positron emission tomography/computed tomography. Indian J Nucl Med 2017;32:59-60

How to cite this URL:
Chandra P, Purandare N, Shah S, Agrawal A, Rangarajan V. Ocular granulocytic sarcoma as an initial clinical presentation of acute myeloid leukemia identified on flurodeoxyglucose positron emission tomography/computed tomography. Indian J Nucl Med [serial online] 2017 [cited 2019 Dec 5];32:59-60. Available from: http://www.ijnm.in/text.asp?2017/32/1/59/198485

An 11-year-old child presented with a progressively increasing swelling in the left eye with gradual visual loss for 3 months. 2-fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) was done for suspected clinical diagnosis of lymphoma. The maximum intensity projection image revealed FDG uptake in region of the left orbit, left paraspinal region in the lumbar, and multiple sites in pelvis [[Figure 1]a, black arrows] with low-grade diffuse FDG uptake in the marrow of vertebrae, pelvis, and bilateral proximal femur and humeri [[Figure 1]a, bold black arrows] - suggesting marrow involvement by disease. Transaxial PET/CT image of the left orbit revealed FDG avid large soft tissue mass (7.2 cm ×5.2 cm – maximum standardized uptake values [SUVmax] - 6.14) infiltrating the extraocular muscles, optic nerve, choroid with extraorbital causing outward, downward, and lateral displacement of the eyeball [[Figure 1]b, white arrow head]. The additional FDG-PET/CT revealed clinically occult lesion in the paraspinal region at L1–L2 junction [[Figure 1]c, long white arrow], soft tissue mass in the left S1 canal [[Figure 2]a and [Figure 2]b, short black arrow], and mass in the right paraspinal region posterosuperior to sacroiliac joint [[Figure 2]c and [Figure 2]d, short white arrow]. Concurrent laboratory evaluation revealed pancytopenia and high serum lactate dehydrogenase. Bone marrow biopsy was suggestive of acute leukemia. Fluorescent situ hybridization test was suggestive of acute myeloid leukemia (AML), M2 subtype. Cytogenetic studies revealed translocation, t(8,14), in 94% of cells.
Figure 1: Maximum intensity projection image (a) reveals fluorodeoxyglucose uptake in the region of left orbit, left paraspinal region in the lumbar, and multiple sites in the pelvis (black arrows) with diffuse uptake in the marrow of vertebrae, pelvis, and bilateral proximal femur and humeri (bold black arrows). (b) Transaxial positron emission tomography/computed tomography image of the left orbit shows a large soft tissue mass infiltrating the extraocular muscles, optic nerve, choroid (white arrowhead). (c) Transaxial fused positron emission tomography/computed tomography shows a fluorodeoxyglucose avid lesion in the paraspinal region at L1–L2 junction (long white arrow)

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Figure 2: Trans-axial computed tomography (a) and fused positron emission tomography/computed tomography (b) images shows uptake in enhancing mass in the left S1 canal (short black arrow). Coronal computed tomography (c) and fused positron emission tomography/computed tomography (d) images mass in the right paraspinal region posterosuperior to sacroiliac joint (short white arrow)

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Granulocytic sarcomas (GSs) are a rare extramedullary presentation of AML and occur in about 2–8% of cases. It can precede the diagnosis of AML in 25% of cases, occurs concomitantly in 15–35% or occurs after diagnosis/at relapse in up to 50% of patients.[1] The studies have shown that presence of GS in patients with AML is associated with poor overall survival.[2],[3]

Common sites of presentation include bones, lymph nodes, skin, breast, and rarely in the gastrointestinal or genitourinary tract or in the head neck regions.[4] Ocular GS (OGS), rare in adults, is relatively common in pediatric patients with AML. Symptoms may be nonspecific and include proptosis, pain, and visual deficits. M2, M4, and M5 are the common subtype of AML associated with OGS. The most common cytogenetic abnormality associated with OGS is t(8,21).[5] There is no consensus on the optimal treatment of OGS. Few reports have shown beneficial outcomes with the addition of local external beam radiotherapy to standard chemotherapy with cytosine arabinoside.[5],[6]

PET/CT has been previously evaluated as a noninvasive diagnostic tool for the identification of extramedullary disease in patients with AML. Although FDG uptake in GS is usually moderate, it may be variable and is relatively less as compared to patients with hematological malignancies such as Hodgkin's lymphoma or non-Hodgkin's lymphoma. In a study done by Stölzel et al. in ten patients with histologically proven GS, there was interindividual/intraindividual variation in the absolute SUVmax (range 2.5–9.3), and 1 tumor showed no significant FDG uptake (false negative). In the same study, FDG-PET/CT also identified additional extramedullary/multifocal disease in about 60% of patients.[7] Multifocal disease is usually associated with adverse prognosis.[8] The studies have also demonstrated the potential clinical utility of PET/CT in monitoring response to treatment and in early identification of relapse of extramedullary disease during induction or at the end of chemotherapy.[7],[9],[10]

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Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Avni B, Koren-Michowitz M. Myeloid sarcoma, Current approach and therapeutic options. Ther Adv Hematol 2011;2:309-16.  Back to cited text no. 1
    
2.
Lan TY, Lin DT, Tien HF, Yang RS, Chen CY, Wu K. Prognostic factors of treatment outcomes in patients with granulocytic sarcoma. Acta Haematol 2009;122:238-46.  Back to cited text no. 2
    
3.
Schaich M, Schlenk RF, Al-Ali HK, Döhner H, Ganser A, Heil G, et al. Prognosis of acute myeloid leukemia patients up to 60 years of age exhibiting trisomy 8 within a non-complex karyotype: Individual patient data-based meta-analysis of the German acute myeloid leukemia intergroup. Haematologica 2007;92:763-70.  Back to cited text no. 3
    
4.
Lee EY, Anthony MP, Leung AY, Loong F, Khong PL. Utility of FDG PET/CT in the assessment of myeloid sarcoma. AJR Am J Roentgenol 2012;198:1175-9.  Back to cited text no. 4
    
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Ohanian M, Borthakur G, Quintas-Cardama A, Mathisen M, Cortés JE, Estrov Z, et al. Ocular granulocytic sarcoma: A case report and literature review of ocular extramedullary acute myeloid leukemia. Clin Lymphoma Myeloma Leuk 2013;13:93-6.  Back to cited text no. 5
    
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Fleckenstein K, Geinitz H, Grosu A, Goetze K, Werner M, Molls M. Irradiation for conjunctival granulocytic sarcoma. Strahlenther Onkol 2003;179:187-90.  Back to cited text no. 6
    
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Stölzel F, Röllig C, Radke J, Mohr B, Platzbecker U, Bornhäuser M. 18F-FDG-PET/CT for detection of extramedullary acute myeloid leukemia. Haematologica 2011;96:1552-6.  Back to cited text no. 7
    
8.
Cunningham I. Extramedullary sites of leukemia relapse after transplant. Leuk Lymphoma 2006;47:1754-67.  Back to cited text no. 8
    
9.
Aschoff P, Häntschel M, Oksüz M, Werner MK, Lichy M, Vogel W, et al. Integrated FDG-PET/CT for detection, therapy monitoring and follow-up of granulocytic sarcoma. Initial results. Nuklearmedizin 2009;48:185-91.  Back to cited text no. 9
    
10.
Karlin L, Itti E, Pautas C, Rachid M, Bories D, Cordonnier C, et al. PET-imaging as a useful tool for early detection of the relapse site in the management of primary myeloid sarcoma. Haematologica 2006;91:12-Suppl:ECR54  Back to cited text no. 10
    


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