Myeloid Sarcoma: A Rare Case of an Orbital Mass Mimicking Orbital Pseudotumor Requiring Neurosurgical Intervention

Abstract

Objective. A rare case of myeloid sarcoma (MS), previously referred to as granulocytic sarcoma or chloroma, is presented. Representing a unique form of acute myeloid leukemia (AML), MS may rarely occur in adults. Even rarer, MS may occur as the initial presentation of AML. Methods. We report a singular and illustrative case of an orbital pseudotumor mimicking mass in a 65-year-old male as the initial presentation of AML. Results. Neurosurgical intervention was required to establish the definitive diagnosis via right modified orbitofrontozygomatic craniotomy as well as to decompress the optic canal, superior orbital fissure, and orbit. Conclusion. Postoperatively, he reported decreased pain and improvement of his vision. Further examination revealed decreased proptosis and improved extraocular mobility. Pathological findings demonstrated MS. We review the literature and discuss the neurosurgical relevance of MS as the initial presentation of AML.

Myeloid sarcoma, a rarity of the head and neck region - A case report

“…Myeloid sarcoma (MS) was previously referred to as granulocytic sarcoma and also by the name chloroma meaning 'green tumor' [3] .King in 1853 coined the term chloroma due to its frequent green color [4].This macroscopic green appearance is caused by the production of myeloperoxidase in many of these tumors [3]. MS is a tumoral mass of either myeloblasts or immature myeloid cells in extramedullary sites.…”

“…Diagnosing myeloid sarcoma of the head and neck region poses a big challenge, because of the low frequency of occurrence and the potential for almost any lineage of tumour to occur in this region. MS can be mistaken on histology for non-Hodgkin's lymphoma, small round cell tumors such as neuroblastoma or undifferentiated carcinoma [4]. As such, a high degree of suspicion is needed when dealing with a probable case of myeloid sarcoma [5].…”

“…MS can be the initial presentation of acute myeloid leukemia or it can arise de-novo; on its own [3]. MS usually occurs in the pediatric population, and rarely occurs in adults [4]. Besides simple blood parameters, MS of head and neck region requires the aid of immunohistochemical staining as a definite diagnostic tool [5].…”

Multifocal myeloid sarcomas: a rare presentation of AML

“…Orbital granulocytic sarcoma occurs more commonly in paediatric rather than adult AML. Rapid visual loss from orbitopathy leading to compression of the optic nerve is an oncological emergency 3. Decompressive surgery and radiation have both been used successfully.…”

Myeloid Sarcoma of the Orbit Preceding Acute Myeloid Leukaemia: A Diagnostic Challenge

“…MS can occur in diverse clinical presentations including; (Christopher, William, & Bonnie, Seong-Jin, Farahvar, Chen, & Wang, 2014;Dock, 1983). a) Initial presentation in a healthy individual in whom a typical AML with leukaemic phase evolves after an interval of weeks, months or years b) The first manifestation of relapse in a patient previously treated for leukaemia c) Patients in the active phase of AML MS occurs in all age groups with some reports showing a median age of 56 years and a wide range of 1 month to 89 years (Falini et al, 2007;Piled et al, 2007;Neiman, Barcos, Berard, Bonner, Mann, Rydell, & Bennett, 1981;Goyal et al, 2017).…”