Myelodysplasia Presenting as Granulocytic Sarcoma of Mediastinum Causing Superior Vena Cava Syndrome

Abstract

Granulocytic sarcomas (GS) are extramedullary tumor masses of immature myeloid cells, most frequently associated with hematological disorders including acute myeloid leukemia (AML), chronic myelogenous leukemia (CML), and myelodysplastic syndrome (MDS). Recent interest has centered upon the possible biologic properties that enable theses myeloid cells to adhere to tissues and establish a tumor mass. GS presenting as a mediastinal mass is relatively infrequent, and more uncommon is presentation with the superior vena cava syndrome. We present one such case and review some of the available literature.

Intrathoracic Myeloid Sarcoma

“…A literature search revealed 27 other reported cases of intrathoracic myelocytic sarcomas, with 6 cases causing superior vena cava syndrome 4,5,[11][12][13][14][15][16][17][18][19][20][21][22][23][24][25] (Table 1). Of the 28 reported cases, 13 patients (46%) responded to treatment, 11 (39%) died, 1 patient signed out against medical advice before receiving treatment, and in the other 3, the clinical course was not reported.…”

Thoracic t(9;22)-Positive Granulocytic Sarcoma as Initial Presentation of Chronic Myeloid Leukemia

“…1,2 “Chloroma,” derived from the Greek word chloros , meaning green, reflected the characteristic green appearance resulting from the presence of the enzyme myeloperoxidase in the cells. 3,4 In 1893, the association between the tumor and acute leukemia was made by Dock. 5 The tumor was finally called granulocytic sarcoma by Rappaport in 1966 because not all the cells are green, depending on the concentration of myeloperoxidase in the cells.…”

Malignancy in Common Variable Immune Deficiency: Report of Two Rare Cases of Gastrointestinal Malignancy and a Review of the Literature

“…The combination of anorectal myeloid tumor and myelodysplasia has been reported before in various locations [14,15,17,18]. In one series of 32 patients with granulocytic sarcoma, 2 demonstrated myelodysplasia, while the majority had some evidence of an underlying hematological malignancy [18].…”

“…The diagnosis may be missed if there is a low index of suspicion. These tumors have been shown to occur in the skin, bone/spine, chest/mediastinum, nervous system, gastrointestinal tract and in the gynecological and genitourinary systems [9][10][11][12][13][14][15][16]. Bony tissue of the sternum, skull and ribs as well as soft tissue around the orbit, sinuses and dura appear to be the most common sites for tumor localization.…”