Indian Journal of Pathology and Microbiology

LETTER TO EDITOR
Year
: 2011  |  Volume : 54  |  Issue : 3  |  Page : 660--661

Primary myeloid sarcoma with megakaryocytic differentiation in lymph nodes and skin


Urmila Majhi, Kanchan Murhekar, Shirley Sundersingh 
 Department of Pathology, Cancer Institute (WIA), Chennai, India

Correspondence Address:
Urmila Majhi
Department of Pathology, Cancer Institute (WIA), 38, Sardar Patel Road, Chennai - 600 036
India




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;54:660-661


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 2019 Sep 18 ];54:660-661
Available from: http://www.ijpmonline.org/text.asp?2011/54/3/660/85146


Full Text

Sir,

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}{Figure 2}{Figure 3}

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

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2Nakagawa 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.
3Daniëls 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.
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