3 year old male brought to the pediatric department with history of left eye swelling

 

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Doctor's Information

Name : Bulabai karpagam
Family : Bulabai desai
Affiliation :Senior Assistant Professor,Department Of Radiology
Academic Degree : M.D,FRCR
Email : This email address is being protected from spambots. You need JavaScript enabled to view it.
Resident : M.S.Hemhnath

 

Case Section

Head & Neck Imaging

 

Patient's Information

Gender : Male
Age : 3

 

Clinical Summary

3 year old male child brought to the pediatric department with history of left eye swelling for one week duration. There was history of mild fever preceding the eye swelling, for which child was treated in other institution. On clinical examination there was proptosis of left eye with restricted movement of the eye. The vision examination could not be assessed due to the periorbital edema .

 

Imaging Findings

Figure 1 -- On axial Ct study of the orbit reveals complete opacification of the ethmoidal sinus and the l medial aspect of the retro orbital and medial rectus muscle appears bulky. Figure 2- Axial T1 image shows isointense lesion involving left medial extra conal compartment with bulky medial rectus muscle. Figure 3 –.on Flair axial image there is hyperintense lesion involving the nasal periorbital , medial extra and intra conal aspects encasing the muscle. Optic nerve is not involved. . Figure 4 – On T2 series lesion is isointense with normal signal of the sclera and the eyeball contents.. Figure 5- T1 post contrast series shows homogenous good enhancement of the mass at medial and superior aspect of the orbit including the preseptal plane

 

Differential Diagnosis

Most childhood orbital tumors are unilateral. Most benign conditions, like dermoid cyst, capillary hemangioma, lymphangioma usually affect only a single orbit. Rhabdomyosarcoma is the most common .Malignant orbital tumor of childhood, is invariably unilateral. The primary differential diagnoses of a retrobulbar orbital mass in a child with bilateral involvement are idiopathic nongranulomatous orbital inflammation, metastatic neuroblastoma, and Granulocytic [ myeloid] sarcoma . Pediatric idiopathic nongranulomatous orbital inflammation is initially unilateral in 10% of cases , Focal soft-tissue masses as such developing in the course of myelogenous leukemia may consist of infection, hemorrhage, secondary neoplasms, or granulocytic sarcoma. Early diagnosis is crucial, because each complication requires specific therapy. Many of these lesions can be differentiated on the basis of clinical examination or on the results of imaging findings.

 

Final Diagnosis

Orbital Granulocytic Sarcoma

 

Discussion (Related Text)

Extramedullary proliferation of cells of myeloid origin is termed as Granulocytic sarcoma. This tumor was first described by Allen Burns in 1811. The terminology presently in use is granulocytic sarcoma as by WHO nomenclature. Granulocytic sarcoma is an unusual manifestation of acute myelogenous leukemia in children and presents a diagnostic dilemma when it precedes the development of systemic disease. Granulocytic sarcoma is an extramedullary proliferation of myeloblasts and immature myeloid cells. It is well known that AML can be seen initially with orbital involvement, before the diagnosis of the underlying leukemia. Soft tissue accumulations of leukemic cells were previously referred to as granulocytic sarcoma or chloroma, the broader term myeloid sarcoma is also preferred. Granulocytic sarcomas have been observed in patients with acute myelogenous leukemia, chronic myelogenous leukemia, and other myeloproliferative disorders such as myelofibrosis. They occur in less than 10 % of patients with acute myelogenous leukemia and less frequently in patients with chronic myelogenous leukemia. Study shows same prevalence rate in both sexes. Children are more often affected than adults: commonly between age of 3 to 5years. Granulocytic sarcoma can occur concurrently as the presenting sign of leukemia as seen in our case or precedes hematological leukemia nearly in 35% , for months to years. And can present as remission of disease within 2 years. The disease is relatively uncommon in the western hemisphere, but is more prevalent in the Middle East, Asia, and Africa. Most of the larger reported series have come from Turkey and India. Unilateral or bilateral proptosis is the commonest sign of presentation with orbital involvement . In our case, the patient presented with unilateral proptosis with restricted left eye movements. Myeloid Sarcoma often occur in multiples and affect any part of the body , preferentially involve orbits and PNS in extra medullary proliferation . The lesions may arise primarily within the orbit, or they may extend into the orbit from neighboring structures , mainly from the paranasal sinuses. Lesion may cause bony destruction and sclera involvement. Subperiosteal bone involvement in vertebrae, sternum, orbit and cranium is observed with orbital involvement in about 3%. We report a case of 3 years old male child presented with left orbital swelling for 1week gradually increasing , associated with fever prior to onset of orbital lesion. This case is unique in providing exquisite CT and MRI features correlating with subsequent complete blood cell count , revealed leukaemic cells and diagnosis of acute myeloid leukaemia with orbital granulocytic sarcoma was made. In our CT,MRI study there is opacification of the sinuses including the ethmoidal air cells,both maxillary sinuses and the nasal cavity with erosion of the lamina papyracea on left and unilateral propto

 

References

1. A. Guermazi C. Feger P. Rousselot ,M. Merad , N. Benchaib , P. Bourrier X Mariette AJR February 2002, Volume 178, Number 2 2. Jerry A. Shields, MD; Gary A. Stopyra, MD; Brian P. Marr, MD; Carol L. Shields, MD; Arch Ophthalmol 2003;121 :138. doi:10.1001/archopht.121.1.138. 3. Stein-Wexler R, Wootton-Gorges SL,Pediatr Radiol. 2003 Feb;33(2):136-9, 2002 Nov 12. 4.Batia AvniMD,maya –Michwitz MD, Therapeutic Advances in Hematology,2011;2(5):309-316. 5. Atul B. Shinagare,,atherine M.Krajewski,JasonL. Hornick, , Vikram Kurra, AJR 2012 Dec ,volume 199. Competing interests: The authors received no financial or other type of support to carry out this study. There is no conflict of interests.This article has not been published in any other journal

 

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