CASE REPORT Myeloid Sarcoma in a Child with Acute Myeloblastic Leukaemia Jamal Ahmad1, Lubna Zafar1, Gul Hussain2 and Sajida Kausar3 ABSTRACT We report a rare occurrence of myeloid sarcoma in a 7 years old child with acute myeloblastic leukaemia (AML - FAB type M2). He presented with fever, generalized weakness, bilateral proptosis and left parotid swelling. CT scan revealed a mass in paranasal sinuses extending into brain and retro-orbital region. Diagnosis of AML M2 was made on bone marrow aspiration and special stains. Induction therapy for AML was given according to standard protocol. The extramedullary lesion as well as the acute leukaemia went into complete remission. Key words: Myeloid sarcoma. Chloroma. Acute myeloblastic leukaemia. Paranasal sinus. INTRODUCTION palpable. Fundoscopy showed established Myeloid (granulocytic) sarcoma or chloroma is an extra papilloedema. Ear examination showed left conductive medullary tumour composed of immature malignant deafness due to collapse of left external auditory canal white blood cells or myeloblasts with or without (pressure effect of tumour). CT scan of brain and neck maturation.1 It affects older adults more frequently. showed locally infiltrative mass in nasopharyngeal Simple infiltration by myeloid blasts in any part of the region with intraorbital and intracranial extension body to form tumour mass is not labelled as myeloid (Figure 2). A differential diagnoses of rhabdomyo- sarcoma unless the tissue architecture of the part is lost. sarcoma, lymphoma and meningioma were suggested This tumour may precede or occur concurrently with by the radiologist. Fine needle aspiration cytology of acute or chronic myeloid leukaemia. It can also occur parotid region swelling showed benign epithelial lesion. with other myeloproliferative disorders (MPD) or CSF examination showed no abnormality. myelodysplastic syndrome (MDS). Sometimes, it is the initial manifestation of relapse in a previously treated case of acute myeloblastic leukaemia or herald the onset of blast transformation in a case of CML.1 Myeloid sarcoma is a rare condition, especially its occurrence in a child in association with AML.2,3 This is a case report of this rare condition. CASE REPORT A 7 years old male child presented in Fauji Foundation Hospital, Rawalpindi, in January 2008, with proptosis and swelling in left parotid region for 40 days. He had 3 weeks history of generalized weakness and 2 weeks history of high grade fever. Figure 1: Left parotid swelling and Figure 2: Mass in nasopharyngeal proptosis. region with intracranial and retro- Physical examination revealed a weak looking child with orbital extension. large left parotid region swelling about 4 x 5 cm in size (Figure I). It was hard in consistency and not fixed to Blood CP revealed TLC 14.8 x 109/1, HB 7.8 g/dl, underlying structures. Overlying skin was normal looking platelets 32 x 109/l. Peripheral blood smear showed and not fixed to this growth. There was also bilateral 65% blasts, some showing Auer rods. Liver and renal proptosis especially marked in the left eye. Pallor was function were in normal limits. positive. Lymph nodes, liver and spleen were not Bone marrow aspiration revealed hypercellular marrow with 85% blast cells some showing Auer rods. The Department of Haematology1/Paediatrics2/Oncology3, Fauji blasts were Sudan black B positive. A diagnosis of acute Foundation Hospital, Rawalpindi. myeloblastic leukaemia FAB type M2 was made. Correspondence: Dr. Jamal Ahmad, Flat No. 14, Block No. 02, Induction chemotherapy (ADE regimen: injectable Alshifa Eye Trust Hospital, Rawalpindi. cytosar, daunorubicin and etoposide) was started in first E-mail: [email protected] week of February 2008. He went into complete clinical Received January 20, 2010; accepted March 26, 2011. and haematological remission. The bone marrow Journal of the College of Physicians and Surgeons Pakistan 2011, Vol. 21 (6): 369-370 369 Jamal Ahmad, Lubna Zafar, Gul Hussain and Sajida Kausar aspiration report in March 2008 showed < 5% blast the most commonly expressed marker followed by cells. Parotid swelling and proptosis disappeared. He MPO, CD117, CD 99, CD 34, AND TdT.1 was then lost to follow-up. In this patient we had a concurrent diagnosis of AML-M2 with myeloid sarcoma, so induction chemotherapy was DISCUSSION started immediately which resulted in prompt relief of Myeloid (granulocytic) sarcoma has been called chloroma, symptoms. There is no standard protocol of chemo- derived from greek word chloros meaning green due to therapy for de-novo myeloid sarcoma and standard cut surface appearance caused by the myeloperoxidase chemotherapy against AML is only moderately effective enzyme.4 in suitable cases.4 The clinical behaviour and response These tumours can virtually affect any organ, but to treatment is not influenced by factors such as age, subperiosteal bone structures like skull, paranasal gender, anatomical site, pattern of presentation, histo- sinuses, ribs and vertebrae are more common sites. logical, cytogenetic and immunophenotypic features.1,9 Skin and lymph nodes may also be involved. This However, some studies suggest that systemic chemo- patient had a mass in paranasal sinuses which extended therapy against AML can help reduce rate of leukaemic intracranially and retro-orbitally and manifested with transformation of de-novo myeloid sarcoma.5 Only bone proptosis due to pressure effect. A similar case of marrow transplant results in prolonged survival.9 myeloid sarcoma associated with CML, presenting with To conclude, myeloid sarcomas are rare tumours which proptosis and blindness has been reported.2 usually present in association with myeloid leukaemias. Paraparesis due to extradural myeloid sarcoma in When presenting as primary tumours, they may be association with AML was reported in another patient.3 misdiagnosed and only a high degree of suspicion and thorough workup lead to correct diagnosis and prompt Myeloid sarcoma can present de-novo as a primary tumour or concurrent with AML (as in this patient) or with treatment thus preventing serious complications. a myeloproliferative disorders.1 Incidence of myeloid sarcoma in AML is 3-5%.5 REFERENCES The latest classification of AML by WHO considers 1. Pilleri SA, Orazi A, Fallini B. Myeloid sarcoma. In: Swerdlow HS, Compo E, Jaffe ES, Pileri SA, Stein H, Thiele L, editors. myeloid sarcoma as a separate entity and the et al, de-novo WHO classification of tumours of haemopoietic and lymphoid detection of myeloid sarcoma is taken as de-novo tissue. 4th ed. Lyon: IARC Press; 2008. p. 140-1. equivalent of diagnosis of AML.1 myeloid De-novo 2. Enosolease ME, Awodu AO. Proptosis and blindness: an sarcoma eventually develops AML (median time of 7-12 unusual primary presentation of chronic granulocytic leukemia. months) and these transformed cases show very poor Pak J Med Sci 2006; 22:326-8. prognosis.5 Patients of AML in remission, who develop 3. Siddiqui AA, Rehman NU. Extradural Granulocytic Sarcoma myeloid sarcoma, almost always have bone marrow causing acute paraparesis. J Coll Physicians Surg Pak 2004; 14:45-7. relapse (within a median time of 7 months) and have a poor prognosis.4 4. Cui Y, Zhou JL, Wu JH, Zhang JZ. Synchronous granulocytic sarcoma of the breast and spine: a case report and review of the Myeloid sarcoma is characterized according to the literature. Chinese Med J 2008; 121:1854-6. predominant cell type, most common being granulocytic 5. 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Comparison in treatment of non- leukaemic granulocytic sarcoma: report of two cases and a diagnostic dilemma.6 In one study 47% of myeloid review of 72 cases in the literature. 2002; 94:1739-46. sarcoma cases were initially misdiagnosed as malignant Cancer lymphoma.7 A patient from Pakistan initially diagnosed 8. Safoorah K, Naseem AS, Ahmed VI. Granulocytic sarcoma in as T-cell non-Hodgkin’s lymphoma was later reviewed the absence of acute myeloid leukemia: a case report. Indian J Pathol Microbiol 2007; 50:88-90. and found to have de-novo myeloid sarcoma.8 9. Pileri SA, Ascani S, Cox MC, Campidelli C, Bacci F, Piccioli M, Definite diagnosis of myeloid sarcoma is helped by et al. Myeloid sarcoma: clinico-pathological, phenotypic and morphology, cytochemistry, and immunophenotype of cytogenetic analysis of 92 adult patients. Leukaemia 2007; tumour cells. On immunohistochemistry, CD 68KP1 is 21:340-50. Epub 2006 Dec 14. lllllOlllll 370 Journal of the College of Physicians and Surgeons Pakistan 2011, Vol. 21 (6): 369-370.