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Year : 2011  |  Volume : 54  |  Issue : 3  |  Page : 660-661
Primary myeloid sarcoma with megakaryocytic differentiation in lymph nodes and skin

Department of Pathology, Cancer Institute (WIA), Chennai, India

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Date of Web Publication20-Sep-2011

How to cite this article:
Majhi U, Murhekar K, Sundersingh S. Primary myeloid sarcoma with megakaryocytic differentiation in lymph nodes and skin. Indian J Pathol Microbiol 2011;54:660-1

How to cite this URL:
Majhi U, Murhekar K, Sundersingh S. Primary myeloid sarcoma with megakaryocytic differentiation in lymph nodes and skin. Indian J Pathol Microbiol [serial online] 2011 [cited 2020 Dec 2];54:660-1. Available from: https://www.ijpmonline.org/text.asp?2011/54/3/660/85146


Myeloid sarcoma is a tumor mass of myeloblasts or immature myeloid cells occurring in extra-medullary sites. The sites for predilection include skin, lymphoid tissue, sub-periosteal bone, reproductive organs, gastrointestinal tract and central nervous system. [1],[2],[3] Several histiotypes of myeloid sarcoma are described. These include undifferentiated, differentiated, blastic, monoblastic, myelomonocytic, trilinear and megakaryocytic differentiation. Though rare, histopathologic evidence of megakaryoblastic differentiation in myeloid sarcoma is considered as a marker of poor prognosis. We report a rare case of myeloid sarcoma showing megakaryocytic differentiation in lymph nodes and skin without involvement of the bone marrow.

A 22-year-old lady presented with growth in the lower jaw for one month and swelling of the sub-mandibular area for two weeks and history of few episodes of epistaxis. On examination, the patient had hypertrophic nodular gingival mucosa, melanoplakia of left lower jaw with sub-mucosal fullness in the hard palate. She had matted left sub-mandibular lymph nodes and enlarged left level II lymph nodes. She had also multiple subcutaneous nodules.

Her white blood cell count was 3.1 10 3 per mm 3 without any abnormal cells in the peripheral smear and bone marrow. Platelet count was 3.16 10 3 per mm 3 . Cardiovascular and respiratory systems were within normal limits. Serological tests, clotting time, bleeding time and blood biochemistry were within normal limits. Left cervical lymph node biopsy and wedge biopsy of subcutaneous nodule showed diffuse infiltration by atypical small round cells along with scattered giant cells with multi-lobated nuclei and abundant eosinophilic cytoplasm. The giant cells were more prominent in lymph node sections. The atypical round cells had scanty cytoplasm with round to oval vesicular nuclei with fine chromatin and increased mitoses [Figure 1]. Immunohistochemical (IHC) studies revealed positive reaction for CD45, CD43, MPO, CD117, CD31, Factor VIII, Actin, CD30 (focally) and EMA [Figure 2] and [Figure 3]. The tumor cells were negative for CD15, CD20, CD79a, UCHL-1, CD68, CD34, CD15, ALK-1, CD99, Keratin, CD10, BCL 6, CD56 and TdT. The giant cells showed strong positive reaction for CD31 [Figure 2], factor VIII and Actin [Figure 3]. KI 67 score was 90%. Expression of CD45, MPO, CD43 and CD117 supported the diagnosis of myeloid sarcoma. Positivity for factor VIII, CD31, Actin and EMA suggested myeloid sarcoma with megakaryocytic differentiation.
Figure 1: (a) Lymph node partly replaced by sheets of large atypical cells (marked as 2), Scattered megakaryocytoid cells are seen (M), Residual lymphoid cells are also seen (marked as 1) (H and E, ×40), (b) Multi - lobulated megakaryocytoid cell (marked as 3) surrounded by atypical cells with blastic morphology, (H and E, ×100)

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Figure 2: (a) Atypical cells of subcutaneous nodule showing LCA positivity (IHC, ×100), (b) Atypical cells of subcutaneous nodule showing MPO positivity (IHC, ×100) (c) Atypical megakaryocytoid cells of lymph node showing strong CD31 positivity (IHC, ×100), (d) Atypical cells of subcutaneous nodule showing CD43 positivity (IHC, ×100)

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Figure 3: (a) Atypical cells of lymph node showing CD117 positivity, Large multi-lobulated megakaryocytoid cell in the center shows negative reaction (IHC, ×100), (b) Atypical cells of subcutaneous nodule showing CD99 positivity (IHC, ×100) (c) Atypical cells of subcutaneous nodule showing Actin positivity (IHC, ×100), (d) Atypical multi lobed megakaryocytes in the lymph node showing factor VIII positivity
(IHC, ×100)

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In the differential diagnosis of myeloid sarcoma, several important diseases such as non-Hodgkin's lymphomas of the lymphoblastic type, Burkitt lymphoma, large-cell lymphoma, small round cell tumors, anaplastic large cell lymphomas and Hodgkin's lymphoma (due to the presence of a good number of giant cells) need to be considered. It is therefore necessary to carry out immune-phenotyping in order to make a precise diagnosis and to exclude the possibility of other lymphoproliferative disorders. Treatment of myeloid sarcomas varies with the subtypes. [1],[2],[3],[4] Histopathologic evidence of megakaryoblastic differentiation itself is an independent poor prognostic factor. New therapeutic strategies are needed for patients with megakaryoblastic differentiation. Hence an accurate diagnosis is essential for prompt treatment especially when patient presents with an extramedullary presentation.

   References Top

1.Pileri SA, Ascani S, Cox MC, Campidelli C, Bacci F, Piccioli M, et al. Myeloid sarcoma: Clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. Leukemia 2007;21:340-50.  Back to cited text no. 1
2.Nakagawa S, Tagami H, Ichinohasama R, Aiba S. Rapidly growing cobblestone-like nodules as a manifestation of myeloid sarcoma. Acta Derm Venereol 2008;88:633-4.  Back to cited text no. 2
3.Daniëls L, Guerti K, Vermeulen K, De Raeve H, Van Assche E, Van de Velde AL, et al. Acute Myeloid Leukaemia of mixed Megakaryocytic and erythroid origin. A case report and review of literature. Acta Clin Belg 2007;62:308-14.  Back to cited text no. 3
4.Oki Y, Kantarjian HM, Zhou X, Cortes J, Faderl S, Verstovsek S, et al. Adult acute megakaryocytic leukemia: An analysis of 37 patients treated at M.D. Anderson Cancer Center. Blood 2006;107:880-4.  Back to cited text no. 4

Correspondence Address:
Urmila Majhi
Department of Pathology, Cancer Institute (WIA), 38, Sardar Patel Road, Chennai - 600 036
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.85146

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