|Year : 2016 | Volume
| Issue : 1 | Page : 82-84
Maxillo-orbital granulocytic sarcoma in acute myeloid leukemia
Chandana Chakraborti1, Krittika Pal Choudhury2
1 Department of Ophthalmology, Calcutta National Medical College and Hospital, Kolkata, West Bengal, India
2 Department of Ophthalmology, Burdwan Medical College, Burdwan, West Bengal, India
|Date of Web Publication||22-Dec-2015|
A/1/1, Pearl Apartment, 50B, Kailas Bose Street, Kolkata - 700 006, West Bengal
Source of Support: None, Conflict of Interest: None
Granulocytic sarcoma or chloroma, a manifestation of acute myeloid leukemia (AML) is a rare cause of childhood proptosis. A 14-year-old boy presented with progressively increasing unilateral proptosis and swelling of lower eyelid and face on the right side. Contrast enhanced computed tomographic images revealed enhancing infiltrates occupying the right orbit, maxillary antrum, and infratemporal fossa. Incisional biopsy from the orbital swelling and the bone marrow aspirate showing leukemic blast cells confirmed the diagnosis of AML. The peripheral smear was normal initially, but high total leukocytic count with immature blast cells was evident after 1-month of presentation. Chemotherapy brought about the remission of the disease. However, the delay in diagnosis because of negative peripheral blood smear examination and inconclusive fine-needle aspiration biopsy led to the loss of vision in right eye. Diagnosis of such case can be made by a combination of good clinical examination and relevant investigations. This case of maxillo-orbital granulocytic sarcoma is reported because of its rarity and to emphasize the clinical and cyto-histological features and problems concerning differential diagnosis.
Keywords: Granulocytic sarcoma, maxillo-orbital infiltration, proptosis
|How to cite this article:|
Chakraborti C, Choudhury KP. Maxillo-orbital granulocytic sarcoma in acute myeloid leukemia. Med J DY Patil Univ 2016;9:82-4
| Introduction|| |
Granulocytic sarcoma (chloroma or extramedullary myeloblastoma) is a rare solid tumor composed of primitive precursors of the granulocytic series of white blood cell that include myeloblasts, promyelocytes, and myelocytes.  Due to their characteristic green color, King in 1853 named these tumors as chloromas (Greek "chloros" meaning green).  Rappaport suggested the name GS considering the association of these tumors to leukemia.  GS lesions are multiple and solid, recurring at different sites, including several extracranial locations, in nearly 50% of the patients. Involvement of the head and neck region (skull, orbit, and paranasal sinuses) is uncommon. The lesions may arise primarily within the orbit, or they may extend into the orbit from neighboring structures.  We report this case because of its rarity.
| Case Report|| |
A 14-year-old male patient presented with a history of swelling of the lower lid, gradually developing proptosis and redness of the right eye (RE) for 1 month. There was no history of fever, weight loss or poor appetite. On examination, best corrected visual acuity was 20/30 in RE and 20/20 in left eye (LE). Conjunctival congestion and chemosis were present in the right lower fornix. A firm to hard nontender, noncompressible mass, 1.5-5 cm in size, was palpable through the right lower lid [Figure 1]. There was a diffuse swelling over the face and infratemporal region (right side) with normal overlying skin. Extraocular muscle movement was restricted in downgaze. Funduscopy revealed disc edema, macular edema, and folds. LE examination was within normal limit. Right sided submandibular and preauricular lymphadenopathy was detected. Thorough physical examination revealed no palpable abdominal organomegaly. His neurological examination was normal. Routine blood investigations were within normal limit. Peripheral blood smear failed to detect any abnormal cell. The contrast enhanced computed tomographic (CECT) images showed an enhancing homogeneous infiltrate occupying the lateral orbital wall pushing the globe and the optic nerve outward and manifesting as proptosis. The infiltrate extended toward the infratemporal fossa beneath the temporalis muscle [Figure 2]a]. Erosion of the posterolateral wall of the maxillary antrum with the invasion of the pterygopalatine fossa and right nasal cavity was also seen [Figure 2]b]. Computed tomography guided fine-needle aspiration cytology showed mostly smudge cells and nuclear streak suggestive of non-Hodgkin lymphoma (NHL). Nasal endoscopy by ENT surgeon revealed medicalisation of the right maxilla. Incisional biopsy of the intraorbital mass showed extramedullary myeloid cell tumor (granulocytic sarcoma) on histopathological examination. Immunohistochemistry showed expression of MPO, CD117, and CD34 and was negative for desmin (FAB-acute myeloid leukemia [AML]-M2) [Figure 3]. Bone marrow biopsy revealed areas of normal hematopoiesis associated with focal areas of blast cell proliferation with vesicular nuclei, distinct nucleoli, irregular nuclear membrane with moderate amount of granular cytoplasm, and some areas of myelofibrosis [Figure 4]. The patient was treated by pediatric oncologist by intensive chemotherapy including cytosine arabinoside (100 mg/m 2 ) and doxorubicin (30 mg/m 2 ). After two cycles, the swelling subsided but severe proptosis led to exposure of the cornea, resulting in an ulcer and eventual loss of vision in the RE. However, the patient then returned home and was lost to further follow-up with us.
|Figure 1: A 14-year-old boy with right eye proptosis and upward displacement of the globe. A firm mass was palpable in the inferior part of the right orbit|
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|Figure 2: (a) Axial contrast enhanced computed tomography showing intraorbital soft tissue mass with a fine-needle aspiration cytology needle in situ and temporal fossa infiltration in the right side. (b) Axial contrast enhanced computed tomography showing erosion of posterolateral wall of maxillary antrum with invasion of enhancing homogeneous infiltrates into the pterygomaxillary fossa and right nasal cavity|
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|Figure 3: Histopathology of orbital mass showing an extramedullary myeloid cell proliferation infiltrating the orbital fat tissues (H and E, ×400)|
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|Figure 4: Bone marrow biopsy shows proliferation of blast cells having vesicular chromatin, distinct nucleoli, and moderate amount of granular cytoplasm (H and E, ×40)|
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| Discussion|| |
Granulocytic sarcoma was first described by Burns in 1811. AML accounts for nearly 15% of all leukemia in children. GS or an extramedullary leukemic deposit is an unusual manifestation of AML, accounting for about 3% of cases of AML.  Children are more often affected than adults, and about 60% of patients are younger than 15 years old.  Chloromas develop mostly concomitantly with the FAB subtype M5a, M5b, M4, and M2 of the AML.  GS may develop before, during, or after the occurrence of systemic leukemia. The onset of orbital GS in relation to systemic AML can be variable. The orbit can be involved even before the bone marrow and peripheral blood show features of the malignancy.  When a patient has a history of leukemia and then develops orbital GS, the diagnosis is usually not difficult. However, when the orbital tumor precedes the development of systemic leukemia the diagnosis can be difficult for both clinician and pathologist, which happened in our case.
The clinical features of orbital GS can vary considerably. The presence of unilateral and bilateral proptosis, as well as simultaneous presence of both bilateral proptosis and bitemporal swellings has been reported with AML. ,, The proptosis in these cases is mainly due to leukemic infiltrates, retrobulbar hemorrhage, orbital muscle infiltration or venous blockage.  In a study of 86 Indian patients with AML, 8 (9.3%) were found to have orbital deposits in one or both eyes.  Focal well-defined, homogeneous masses, may arise intraconally or extraconally, and mold to the bone and contiguous structures, including the sclera and the orbital bones.  GS can also present as ptosis, lacrimal gland involvement, conjunctival mass, iridic, and diffuse uveal involvement.  In our case, CECT images showing bone destruction point toward orbital invasion originating from lesions within the maxillary sinus.
The primary differential diagnoses of a retrobulbar orbital mass in a child include rhabdomyosarcoma, metastatic neuroblastoma, African Burkitt's lymphoma, and idiopathic inflammatory pseudotumor. The histological differential diagnosis may be difficult due to the poorly differentiated myeloblasts or in the absence of the greenish color. The tumors that can be confused with chloroma are histiocytic lymphoma, poorly differentiated lymphoblastic lymphoma, large cell NHL, Ewing sarcoma, some acute lymphocytic leukemia, as well as primitive neuroepithelial tumors.  In our case, fine-needle aspiration biopsy misdiagnosed it as NHL. Bone marrow aspiration and biopsy confirmed the diagnosis of AML.
The prognosis for patients with GS depends on the course of the underlying systemic malignancy. , However, the presence of orbital GS does not significantly alter the survival in patients with AML.  Chemotherapy is the main stay of treatment. Chloromas are radiosensitive and local radiotherapy can be associated with chemotherapy. The earlier detection of extramedullary GS could better define the real prognosis of these rare neoplasms. 
GS, especially maxillo-orbital GS, with involvement of infratemporal and pterygopalatine fossa, as seen in our case, is a rare cause of childhood proptosis and its detection was especially challenging as there were no signs of systemic leukemia. Hence, a categorized differential diagnosis is important to order relevant investigations to facilitate early treatment.
Declaration of Patient Consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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