Date of Award

3-1-1980

Document Type

Open Access Thesis

Degree Name

Medical Doctor (MD)

First Advisor

Dr. Edwin Cadman

Second Advisor

Dr. James Waldron

Third Advisor

Dr. Thomas Duffy

Abstract

[From the Introduction] Extramedullary solid tumors composed of partially differentiated hematopoietic precursors have been variously described in the literature, with some 500 case reports since 1824 employing such terms as chloroma, chloroleukemia, and myeloblastoma. Early case reports took particular note of the green color of many of the lesions, hence the term chloroma, and the dramatic orbital involvement in children, sometimes producing frog-like facies. Frequently however, these lesions lack distinctive coloration so that Rappaport has suggested the more general term, granulocytic sarcoma. Presently, this term is used to characterize invasive and destructive tumor masses composed of immature cells of the myelomonocytic series. Although for many years the lesions were associated with leukemia of various cell types, presently they are understood to be an uncommon variant of acute myelogenous leukemia, sometimes presenting before systemic involvement is detectable. In these cases, a retrospective review of the pathologic interpretation(s) reveals a wide spectrum of diagnoses, the most common misdiagnosis being histiocytic lymphoma. Thus, although extra medullary tumors of myeloid origin are thought to be fairly rare, they present a difficult diagnostic challenge, particularly in those cases in which definitive diagnosis maybe of some therapeutic value. An elevated index of suspicion would therefore be of value in certain clinical settings, for example, the evaluation of proptosis in African children, where granulocytic sarcoma ranks second to Burkitts lymphoma in incidence of orbital neoplasms. Perhaps a full hematologic evaluation should also be performed in persons with "lymphomatous" skin nodules of questionable nature, after consultation with an experienced hematopathologist. Indeed, a component of the presumed rarity of granulocytic sarcoma may correspond to a low degree of surveillance, a situation which would merit correction, particularly as the possibilities for "prophylaxis" or early treatment of acute myelocytic leukemia become less debilitating and the outcome more favorable. The present study considers such diagnostic problems associated with granulocytic sarcoma in the context of eight cases presenting to Yale-New Haven Hospital and its affiliated hospitals during the years 1976-1979.

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