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Clinicopathologic Reports, Case Reports, and Small Case Series
December 2001

Extramedullary Myeloid Cell Tumor in an Elderly Man

Arch Ophthalmol. 2001;119(12):1861-1864. doi:

Extramedullary myeloid cell tumor (granulocytic sarcoma, chloroma) is a rare cause of proptosis that can masquerade as lymphoma. Proper distinction between the two allows appropriate radiation dosing. We demonstrate in a 72-year-old man we saw with epiphora, proptosis, binocular diplopia, and pain in the right eye that extramedullary myeloid cell tumor may be treated with radiation alone using as little as 450-rad (4.5 Gy) applied in small fractions. This approach spares the elderly patient from the general myelosuppression of chemotherapy.

Report of a Case

An otherwise healthy 72-year-old man developed epiphora, proptosis, binocular diplopia, and pain in his right eye over an 8-month period. Ocular and medical histories were noncontributory. Visual acuity was 20/50 OD and 20/40 OS. External examination revealed right-sided hypoglobus, exophthalmos, and a ruddy, red bulbar conjunctiva associated with a large orbital mass that was firm to the touch and the mass did not perform retropulsion, instead when pushed it was firm and moved back (Figure 1). Hertel exophthalmometry measurements were 23 mm OD and 13 mm OS with a base of 125 mm. Confrontational fields were full. Ocular motility was restricted in all fields of gaze in the right eye. Intraocular pressure was 21 mm Hg OD compared with 15 mm Hg OS. Slitlamp biomicroscopy revealed the conjunctiva of the right eye was severely congested and expanded by a solid, salmon-colored mass (Figure 2).

Figure 1.
When first seen for evaluation,
the patient had right-sided hypoglobus, exophthalmos, and a ruddy, red bulbar
conjunctiva.

When first seen for evaluation, the patient had right-sided hypoglobus, exophthalmos, and a ruddy, red bulbar conjunctiva.

Figure 2.
When first seen for evaluation,
the conjunctiva of the patient's right eye was severely congested and expanded
by a solid, salmon-colored mass.

When first seen for evaluation, the conjunctiva of the patient's right eye was severely congested and expanded by a solid, salmon-colored mass.

Orbital computed tomography (Figure 3) revealed a homogeneously enhancing mass in the region of the right upper eyelid. The mass molded to the lateral aspect of the globe, involved the tendinous insertion of the lateral rectus muscle, and extended to the intraconal space.

Figure 3.
Orbital computed tomography with
contrast. A homogeneously enhancing mass is demonstrated in the lid, conjunctiva,
and orbit, molding to the lateral aspect of the globe.

Orbital computed tomography with contrast. A homogeneously enhancing mass is demonstrated in the lid, conjunctiva, and orbit, molding to the lateral aspect of the globe.

A transconjunctival biopsy of the lesion was performed. Intraoperative touch preparations demonstrated a malignant hematolymphoid neoplasm. Portions of the tissue were placed in Zenker fixative and 10% buffered formalin for routine light microscopy. Additional specimens were submitted fresh for flow cytometry and cytogenetics. Histopathological examination findings demonstrated a solid sheet of monomorphous, intermediate to large cells filling the conjunctival stroma. Individual cells showed a high nuclear-cytoplasmic ratio, with only a scant rim of eosinophilic cytoplasm. Nuclei were hyperchromatic, irregularly contoured, and contained central nucleoli and dispersed chromatin (Figure 4). On immunohistochemical staining, the neoplastic cells were reactive to CD45 (leukocyte common antigen), CD43, and lysozyme (Figure 5) but did not react with CD3 (pan T-cell) and CD20 (pan B-cell) markers. Flow cytometry confirmed the myeloid nature of the neoplasm, as demonstrated by expression of CD33 tumor cells and a subset of CD34 tumor cells (Figure 6A). There was an absence of CD5 and CD20 cells (Figure 6B). The pathological diagnosis was extramedullary myeloid cell tumor.

Figure 4.
Tumor cells are discohesive with
higher nuclear-cytoplasmic ratios. Round to irregular nuclear contours with
prominent central nucleoli.

Tumor cells are discohesive with higher nuclear-cytoplasmic ratios. Round to irregular nuclear contours with prominent central nucleoli.

Figure 5.
A, CD45 stain. Diffuse, strong
membrane positivity. Diaminobenzadine reaction product (original magnification
× 100). B, Lysozyme stain. Positive staining in a majority of the tumor
cells. Diaminobenzadine reaction product (original magnification × 100).

A, CD45 stain. Diffuse, strong membrane positivity. Diaminobenzadine reaction product (original magnification × 100). B, Lysozyme stain. Positive staining in a majority of the tumor cells. Diaminobenzadine reaction product (original magnification × 100).

Figure 6.
A, Flow cytometry demonstrates
a large majority of cells to be CD33 positive (upper half) and a small subset
to be CD34 positive (upper right quadrant). B, Flow cytometry demonstrates
tumor cells fail to reveal CD5 or CD20 reactivity.

A, Flow cytometry demonstrates a large majority of cells to be CD33 positive (upper half) and a small subset to be CD34 positive (upper right quadrant). B, Flow cytometry demonstrates tumor cells fail to reveal CD5 or CD20 reactivity.

Systemic evaluation showed no lymphadenopathy and findings from the patient's peripheral blood smear were normal. No other extramyelogenous deposits were found on metastatic workup. Therapy with 30 rad (0.3 Gy) of orbital irradiation in 15 fractions was accomplished over a 3-week period. All signs and symptoms were eliminated by 1 month (Figure 7). After 13 and again at 21 months, he reported no symptoms by telephone but declined to return for evaluation.

Figure 7.
One month status after radiation
therapy. Initial signs seen at first evaluation are absent.

One month status after radiation therapy. Initial signs seen at first evaluation are absent.

Comment

Extramedullary myeloid cell tumor (granulocytic sarcoma, chloroma) is a rare tumor composed of immature granulocytes.1,2 The disease may manifest in several different clinical settings. Most commonly it occurs in childhood,1 and most often in combination with preexisting acute myelogenous leukemia. It also occurs as a harbinger of acute myelogenous leukemia in nonleukemic patients. Less often, it has been reported in conjunction with myelodysplastic disorders or chronic myelogenous leukemia.3 The majority of nonleukemic patients with extramedullary myeloid cell tumor will develop leukemia within a matter of months,4 but a delay of 16 years following initial evaluation has been observed.3 The tumor is exceedingly rare in the elderly.5

Extramedullary sites of involvement are usually in extraocular tissues. Most commonly involved are bone, lymph nodes, periosteum, and skin.4 When ophthalmic manifestations occur, they are usually orbital.6 Signs and symptoms frequently include pain, proptosis, chemosis, and epiphora. Computed tomography frequently reveals a homogeneous, well-defined tumor that molds to surrounding bone.7

Histopathological diagnosis of extramedullary myeloid cell tumor may be difficult to ascertain on routinely processed tissue sections, and it is often confused with malignant lymphoma. The distinction is important because successful treatment of lymphoma of the orbit and ocular adnexa requires 2400 to 4000 rad (24-40 Gy) of total irradiation.810 Immunophenotyping tumor cells either by flow cytometry or immunohistochemistry is efficacious in differentiating these 2 entities. Extramedullary myeloid cell tumors are positive for CD45 and CD43 and negative for other B-cell and T-cell markers. In addition, they usually show some degree of reactivity with antibodies to lysozyme or myeloperoxidase.11 By flow cytometry, tumor cells are CD33 positive and variably CD34 positive.

Prognosis and treatment of extramedullary myeloid cell tumor is dependent on the presence or lack of an associated leukemia or dysplasia,6 but in the absence of an associated systemic malignancy, the prognosis is indeterminate. In our case there was no identified associated malignancy. Treatment in such cases is not standardized. Frequently, chemotherapy and irradiation are used together and result in a rapid response.2,5 Irradiation alone, as in this case, may be efficacious in the treatment of a systemically nonleukemic patient with isolated orbital extramedullary myeloid cell tumor, and it will spare the elderly patient from the general myelosuppression that often accompanies chemotherapy.

This work was supported in part by an unrestricted grant from Research to Prevent Blindness, Inc, New York, NY.

Corresponding author and reprints: Christopher T. Westfall, MD, Harvey and Bernice Jones Eye Institute, Department of Ophthalmology, University of Arkansas for Medical Sciences, 4301 W Markham, Slot 523, Little Rock, AR 72205-7199.

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