Indian Journal of Medical Sciences Medknow Publications on behalf of Indian Journal of Medical Sciences Trust ISSN: 0019-5359 EISSN: 1998-3654 Vol. 61, Num. 3, 2007, pp. 152-155

Indian Journal of Medical Sciences, Vol. 61, No. 3, March, 2007, pp. 152-155

Case Report

Granulocytic sarcoma of breast: An aleukemic presentation

D'costa GraceF, Hastak MeenalS, Patil YoganandV

Dept. of Pathology, Grant Medical College and Sir J.J. Gr. of Hospitals, Byculla, Mumbai, Maharashtra
Correspondence Address:Dept. of Pathology, Grant Medical College and Sir J.J. Gr. of Hospitals, Byculla, Mumbai - 400 008, drpatilyoganand@rediffmail.com

Code Number: ms07024

Abstract

Granulocytic sarcoma is a rare extramedullary tumor composed of immature myeloid cells. It is usually associated with leukemia or other myeloproliferative disorders but can also occur without overt hematologic diseases. The breast is an uncommon site of presentation and requires a high index of suspicion for diagnosis. We report such a case in a 45-year-old female, who presented with nontender left breast lump of 6 months' duration. A peripheral smear and bone marrow examination at that time was normal. A lumpectomy was done. An H and E diagnosis of lobular carcinoma vs. non-Hodgkin's lymphoma was entertained. Immunostains, however, revealed myeloperoxidase, naphthol AS-D chloroacetate esterase and CD43 positivity, indicating a diagnosis of granulocytic sarcoma. It appears that early initiation of systemic AML-type chemotherapy is beneficial and may delay or avert the development of AML in bone marrow and blood. Eight months later, the patient presented with an orbital mass; bone marrow and peripheral smear involvement by AML.

Keywords: Breast, granulocytic sarcoma

Introduction

Case Report

A 45-year-old female presented with a left-sided nontender breast lump of 6 months' duration. A peripheral smear at that stage was normal. Mammography showed a single noncalcified, irregular mass with poorly defined feathery margin. A lumpectomy was done, following an inconclusive fine needle aspiration cytology report, which was scanty and nonrepresentative in nature.

Gross examination

Gross examination revealed a well-demarcated but unencapsulated tumor measuring 6 x 5 x 2.5 cm, covered by fibroadipose tissues. Cut section was firm, grayish white and solid [Figure - 1].

Histopathology

Histopathology revealed a diffuse discohesive proliferation of small round-to-oval cells (myeloblasts), with high N/C ratio, hyperchromatic vesicular nuclei, with raised mitotic activity. The rest of the neoplastic cells in all stages of maturation included promyelocytes, myelocytes, metamyelocytes and stab forms. Tubule formation was not seen. An Indian file and targetoid pattern were seen at places [Figure - 2],[Figure - 3]. The stroma was densely desmoplastic. A diagnosis of lobular carcinoma v/s. non-Hodgkin lymphoma (NHL) was made based on the HandE slides.

Immunohistochemistry

Immunohistochemistry study revealed myeloperoxidase, naphthol AS-D chloroacetate esterase and CD43 positivity. LCA, CK and EMA were negative, establishing a diagnosis of granulocytic sarcoma and ruling out diagnoses of lymphoma and carcinoma.

Peripheral smear and bone marrow examination were performed after diagnosis of GS on lumpectomy and IHC, but they revealed normal findings. Eight months later, the patient presented with an orbital mass, bone marrow and peripheral smear involvement by acute myeloid leukemia. Peripheral smear showed 50% myeloblasts, and bone marrow aspirate showed 80% myeloblasts. The blasts were myeloperoxidase-positive, showing auer rodes; and naphthol AS-D chloroacetate esterase positive, showing myeloid differentiation. There was no hepatosplenomegaly, lymphadenopathy, and the other breast was normal.

Discussion

This primary extramedullary lesion may indeed represent a diagnostic and therapeutic dilemma for both the hematopathologist and oncologist[2] and requires a high index of suspicion for diagnosis. It may follow, accompany or signal AML or CML.[2],[3] Granulocytic sarcoma may also occur in patients with myelodysplastic syndrome, where it is a sign of imminent disease progression.[4] In our case, it antedated the presentation of AML by 8 months, as described in literature.[5],[6]

The primary sites of occurrence are usually the lymph nodes, skin, CNS and small intestine,[7],[8] but the breast is an uncommon site of presentation.[7],[8] The age at presentation ranges from 31-73 years, with an average of 52 years.[3] Our patient was 45 years of age. The patients are usually female, as was our case, though it has been reported in males too, who present with gynecomastia.[9] Histologically well-differentiated, poorly differentiated and blastic forms have been described in literature.[3] Our case was a well-differentiated form [Figure - 2],[Figure - 3].

The differential diagnosis should include all tumors with a diffuse proliferation of small tumor cells, i.e., lymphoma, NHL, carcinomas and melanomas.[8] Among the carcinomas, lobular carcinoma and undifferentiated carcinoma enter the differential diagnosis.[10] Non-neoplastic conditions like inflammation and extramedullary hemopoiesis should also be considered.[10] In our case also, the initial diagnosis was lobular carcinoma v/s. NHL. It was only after immunohistochemistry was done, which revealed CD ₄₃ positivity, LCA, CK and EMA negativity, that a diagnosis of granulocytic sarcoma was made, for which the patient was treated as acute myelogenous leukemia with systemic chemotherapy. Eight months later, the patient presented with an orbital mass, bone marrow and peripheral smear involvement by acute myeloid leukemia. Our case presented with isolated mass in the breast, without history of acute myeloid leukemia at last follow-up. Immunohistochemical studies were extremely helpful for recognizing isolated myeloid sarcoma. Such isolated presentation is extremely rare, and to diagnose such cases early is beneficial and may delay or avert the development of AML in bone marrow and blood.

References

Copyright 2007 - Indian Journal of Medical Sciences

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