J Korean Ophthalmol Soc.  1988 Feb;29(1):225-229.

A Case of Granulocytic Sarcoma of the Bilateral Orbits

• Kim SJ
• Shim CB
• Kim KS
• Jeon DS

Abstract

Granulocytic sarcoma is characterized by the formation of clinically apparent invasive and destructive neoplasm composed of immature cells of the granulocytic (myeloid) series. It may arise either during the course of myelogenous leukemia or may precede the leukemic process for weeks to months. Because of its classical greenish appearance productd by a pigmented enzyme called myeloperoxidase, this tumor has long been called chloroma. But the currently preferred terms "granulocytic sarcoma" and "myeloid sarcoma" are more appropriate, as they reflect the cytologic composition of this tumor. Granulocytic sarcoma develops preferentially in bones or periosteum, especially those of the skull, and proptosis attributable to orbital involvement has long been recognized as a classical clinical feature. Children are affected more often than adults, and boys more frequently than girls. In the absence of systemic signs of leukemia, the histopathologic diagnosis may be quite difficult and may be confused with that of lymphoma, rhabdomyosarcoma, metastatic neuroblastoma and histiocytic or inflammatory tumors. Vigorous therapy directed toward the orbital lesion of granulocytic sarcoma may prevent or retard the development of systemic leukemia, a disease that frequently has a rapidly fatal outcome. The authors experienced a case of granulocytic sarcoma of bilateral orbits with exophthalmos and ptosis(O.S.) in a 7-year-old girl, which precede the acute myelogenous leukemia by 3 weeks.

MeSH Terms

Adult
Child
Diagnosis
Exophthalmos
Fatal Outcome
Female
Humans
Leukemia
Leukemia, Myeloid
Leukemia, Myeloid, Acute
Lymphoma
Neuroblastoma
Orbit*
Periosteum
Peroxidase
Rhabdomyosarcoma
Sarcoma, Myeloid*
Skull
Peroxidase