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LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 6  |  Page : 693-695

Intracranial granulocytic sarcomaina in a non-leukemia patient


1 Department of Neurosurgery, The Affiliated Hospital of Qingdao University, Qingdao, Shandong, China
2 Department of Pathology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong, China

Date of Submission08-Oct-2014
Date of Decision21-Oct-2014
Date of Acceptance06-Dec-2014
Date of Web Publication16-Jan-2015

Correspondence Address:
Ming-Chao Fan
Department of Neurosurgery, The Affiliated Hospital of Qingdao University, Qingdao, Shandong
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.149415

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How to cite this article:
Wang JF, Sun P, Lin DL, Fan MC. Intracranial granulocytic sarcomaina in a non-leukemia patient. Neurol India 2014;62:693-5

How to cite this URL:
Wang JF, Sun P, Lin DL, Fan MC. Intracranial granulocytic sarcomaina in a non-leukemia patient. Neurol India [serial online] 2014 [cited 2019 Sep 22];62:693-5. Available from: http://www.neurologyindia.com/text.asp?2014/62/6/693/149415


Sir,

Granulocytic sarcoma (GS), also known as chloroma, is a solid tumor composed of immature myeloid cells at extramedullary sites. [1] The most common sites of GS includes visceral organs, soft tissue, lymph nodes, bone, and skin. [2] The majority of these cases occurred in association with myelogenous leukemia and myeloproliferative disorders. [3],[4],[5],[6] Review of English literature revealed only eight reported cases of GS's involvement with the brain without any evidence of systemic myeloid disease [Table 1]. Here we present the ninth unique case.

A 38-year-old male patient without significant past medical history presented with left lower limb weakness of 2 month's duration and progressive severe headache of 1 week. Computed tomography (CT) scanning showed anirregular high-density occupying lesion in the front oparietal region [Figure 1]a. Magnetic resonance imaging (MRI) routine scanning showed the irregular lesion appeared as isointense on both T1- and T2-weighted images with significant perilesional edema [Figure 1]b and c. Contrast MRI revealed inhomogeneous enhancement and irregular long-T1 lamellar signal [Figure 1]d. Bone marrow biopsyand biochemical tests were normal before and after operation. Total excision of the tumor was performed via right frontoparietal approach. During operation, there was destruction of the skull due to the lesion and hypoderm of the scalp. The lesion was grayish with rich blood supply. Histopathology and immuno his to chemistry confirmed diagnosis of GS [Figure 2]. No complication occurred after the operation. He received systemic chemotherapy in regional hospital after discharge. Clinical and laboratory findings were normal during a 17-month follow-up.
Figure 1: Brain computed tomography (CT) and magnetic resonance imaging (MRI) of the patient with intracranial GS. (a) CT scan of the brain revealed an irregular high-density occupying lesion in the frontoparietal region. (b) T1-weighted MRI showed the irregular mass lesion appeared as iso-T1 signal. (c) T2-weighted MRI showed the tumor appeared as iso-T2signal. (d) Contrast-enhanced MRI revealed the tumor was visible inhomogeneous enhancement, an irregular long-T1 lamellar signal was found in the lesion. It was approximately 4.2x4.5x5.2 cm in size

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Figure 2: Histopathological examination of granulocytic sarcoma (GS) in the presented case. Histopathological examination (hematoxylin and eosin (H and E) staining) showed that the tumor is composed of uniform population of malignant round cells with a high nuclear-cytoplasmic ratio, irregular nuclei, and prominent nucleoli (a). Immunohistochemically, most tumor cells were positive for myeloperoxidase (MPO) (b), negative for CD3 (c) and CD20 (d). Original magnification: x200

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Table 1: GS involvements the cerebrum or cerebellum without any evidence of systemic myeloid disease

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Clinically, these tumors occur in all age groups without any sex predilection. About 2-8% of patients who had acute myeloid leukemia (AML) or chronic myeloid leukemia would accompany with GS. [7],[8] The diagnosis of GS is difficult and more than 40% of GS were initially misdiagnosed. [9] The reasons include the rarity of this tumor and most clinicians defect sufficient cognition of it. GS should be considered as a differential diagnosis for any intracranial malignant neoplasm, regardless of evidence of leukemia. Commonly, intracranial GS appear asiso- or hyperdense on brain CT scan. They are usually typically iso-or hyper-intense on T1- and T2-weighted MRI images, and homogeneous enhancement following contrast administration. In order to prevent misdiagnosis, circumstantial histological test is necessary. Basic histopathologic analyses with proper immunohistochemistry staining provide essential information for diagnosis. Immunohistochemically, myeloperoxidase (MPO), CD3, and CD20 are the most useful antibody for GS. MPO is positive for GS, but CD3 (T-cell marker) and CD20 (B-cell marker) are negative. Bone marrow sampling is imperative to assess the absence of AML if a final diagnosis of GS is made.

There is no standard treatment protocol that has been established. The prevailing opinion is that GS with or without bone marrow involvement should be treated as AML because of the high risk of metachronous AML occurrence. Systemic chemotherapy is significantly useful for these patients to have a good long-term survival. Surgical therapy is the only reserved presenting clinical symptom due to mass effect. The long-term prognosis of non-leukemia GS is still despondent.

 
  References Top

1.
Llena JF, Kawamoto K, Hirano A, Feiring E. Granulocytic sarcoma of the central nervous system: Initial presentation of leukemia. Acta Neuropathol 1978;42:145-7.  Back to cited text no. 1
    
2.
Nickels J, Koivuniemi A, Heiskanen O. Granulocytic sarcoma (chloroma) of the cerebellum and meninges a case report. Acta Neurochir (Wien) 1979;46:297-301.  Back to cited text no. 2
    
3.
Yoon DH, Cho KJ, Suh YL, Kim CW, Chi JG, Han DH, et al. Intracranial granulocytic sarcoma (chloroma) in a nonleukemic patient. J Korean Med Sci 1987;2:173-8.  Back to cited text no. 3
    
4.
Levy R, Shvero J, Sandbank J. Granulocytic sarcoma (chloroma) of the temporal bone. Int J Pediatr Otorhinolarygol 1989;18:163-9.  Back to cited text no. 4
    
5.
Vohra AH, Khawaja MA, Raja IA. Chloroma of the cranium with dural involvement. J Clin Neurosci 1997;4:268-70.  Back to cited text no. 5
    
6.
Chelly I, Mekni A, Kchir N, Karim BH, Khadija B, Selma B, et al. Intracerebellar granulocytic sarcoma. A case report. Pathologica 2005;97:335-7.  Back to cited text no. 6
    
7.
Widhalm G, Dietrich W, Müllauer L, Streubel B, Rabitsch W, Kotter MR, et al. Myeloid sarcoma with multiple lesions of the central nervous system in a patient without leukemia. J Neurosurg 2006;105:916-9.  Back to cited text no. 7
    
8.
Grier DD, Al-Quran SZ, Gray B, Li Y, Braylan R. Intracranial myeloid sarcoma. Br J Haematol 2008;142:681.  Back to cited text no. 8
    
9.
Balleari E, Panarello S, Capello E, Grosso M, Passalia C, Pitto P, et al. Granulocytic sarcoma: An unusual cause of spinal cord compression. Int J Clin Oncol 2007;12:234-7.  Back to cited text no. 9
    


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