granulocytic sarcoma or extramedullary myeloid tumor ( emmt ) is an uncommon neoplasm of immature hematopoetic cells .
these tumors are reported in 3.1 - 9.1% of patients with myeloblastic leukemia and occur concomitantly with , after , or rarely before the onset of leukemia .
we report a case with multiple tumors involving the lymph nodes , breast and orbit .
the diagnosis was established on fine needle aspiration cytology ( fnac ) of the masses which subsequently led to the diagnosis of the underlying leukemia .
a 15 year - old girl presented with bilateral , cervical lymphadenopathy , bilateral breast masses , and gradually developing proptosis of both eyes , in that order .
nodes were firm to hard with restricted mobility and the breast masses were 4.5 cm ( left ) and 5.0 cm ( right ) along their maximum dimension .
fine needle aspiration cytology ( fnac ) was done from the lymph nodes and breast masses .
both wet - fixed and air - dried smears were prepared for papanicolaou and may - grnwald giemsa ( mgg ) staining respectively .
smears from lymph nodes and the breast showed similar features with a polymorphous population of large atypical cells scattered in a background of mostly mature and a few reactive lymphoid cells [ figures 1 and 2 ] .
cytoplasm was pale blue and a fair number of cells showed auer rods and a few fine granules ; the background showed lymphoglandular bodies .
cell morphology from both sites was suggestive of myeloid blasts , so a diagnosis of granulocytic sarcoma / extramedullary myeloid tumor was made .
smears from lymph node show infiltration by numerous myelomonoblasts ( mgg , 400 ) aspirate from breast with leukemic cells ( mgg , 400 ) the peripheral smear showed normocytic , normochromic anemia with leukocytosis .
total leucocyte count ( tlc ) was 48000 cells / mm with the differential count showing 70% blasts , 2% promyelocytes , 8% myelocytes , 8% metamyelocytes , 2% band forms , and 10% lymphocytes .
platelet count was adequate and the blasts were as described above with mostly indented nuclei and auer rods [ figure 3 ] .
out of these , a considerable proportion showed monocytoid features ; the blasts were diffusely positive for sudan black b ( sbb ) and negative for periodic acid schiff ( pas ) .
non specific enolase ( nse ) was noncontributory , based on which , a diagnosis of acute myeloid leukemia ( aml)-m4 was given . peripheral smear shows myeloblasts with auer rods and cells in mitosis ( giemsa , 1000 ) the patient was started on standard chemotherapy to which she responded well .
granulocytic sarcoma or extramedullary myeloid tumor may be seen in association with chronic myeloid leukemia ( cml ) , myelodysplastic syndrome , ( mds ) , myeloproliferative syndrome ( mps ) , polycythemia vera , and essential thrombocytosis .
emmt may be seen in known patients of aml or may occur ( rarely ) as the first manifestation of the disease .
in other cases , it may be the first sign of systemic relapse of a case of treated aml .
emmts may occur at any site in the body , common ones being the skin ( leukemia cutis ) , lymph nodes , and mediastinum .
other sites such as epidural , uterus , ovaries , testis , and orbits have also been reported .
a few case reports have described it in the breast[13 ] where it is often misdiagnosed as lymphoma .
granulocytic sarcomas of the orbit have mostly been described as isolated masses which may precede systemic manifestations .
the soft tissue counterparts may present months before the involvement of peripheral blood and bone marrow . they produce symptoms according to their anatomic location but they may be asymptomatic as well .
problems in diagnosis arise when they are suspected in patients without a background of myeloprolifrative disease .
they may often be misdiagnosed as non - hodgkin 's lymphomas , rhabdomyosarcomas , amelanotic melanomas , or undifferentiated carcinomas .
light microscopy reveals collections of cells that can be usually recognised as being myeloid . undifferentiated or minimally differentiated blasts generally pose diagnostic problems and these are cases that are misdiagnosed as lymphoma .
the cells in our case were positive for sbb and myeloperoxidase ; pas was negative .
immunostaining with monoclonal antibodies against myeloperoxidase ; anti - cd43 , and anti - lysozyme are the most sensitive antibodies ; cd68 and cd20 are useful in differentiating it from lymphoma .
it is regarded as a poor prognostic indicator by some , whereas others have not considered it as an independent prognostic factor .
if they are diagnosed in patients previously treated for aml , they signify relapse whereas in a patient of chronic myeloproliferative disease , they may be indicative of an impending blast crisis .
most tumors , whether detected prior to or during the therapy of leukemia , respond well to standard chemotherapeutic agents .
 granulocytic sarcomas or extramedullary myeloid tumors represent the soft tissue counterpart of acute myeloid leukemia . the term is used for any solid collection of leukemic cells . 
 there have been reports of these tumors occurring before the involvement of blood or bone marrow . 
 our patient had simultaneous involvement of three sites , which was diagnosed on cytology . 
 further confirmation was done on peripheral blood and bone marrow evaluation . 
Introduction
Case Report
Discussion