Orbit, 2015; 34(1): 30–32 ! Informa Healthcare USA, Inc. ISSN: 0167-6830 print / 1744-5108 online DOI: 10.3109/01676830.2014.938169
C ASE REPORT
Eyelid Myeloid Sarcoma: Ominous Presentation of Acute Myelogenous Leukemia Paul O. Phelps1, Marcus M. Marcet2, Augustine R. Hong1, and Jeffrey W. Nichols1
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1
Department of Ophthalmology, John H. Stroger, Jr., Hospital of Cook County, Chicago, IL, USA and 2 Department of Ophthalmology, University of Hong Kong, Cyberport, Hong Kong
ABSTRACT A 19 year-old African American man presented to our clinic for a second opinion about a right upper eyelid mass which had been recalcitrant to treatment for nonspecific orbital inflammation by an outside facility. Examination for systemic causes of the patients eyelid lesion led to a diagnosis of acute myelogenous leukemia (AML) FAB subtype M1. A subsequent biopsy of the eyelid tumor confirmed the diagnosis of a myeloid sarcoma. The patient succumbed to complications from his leukemia within 13 months of presentation. This case report is the first, to our knowledge, of an eyelid myeloid sarcoma as the presenting sign of AML and demonstrates the poor prognosis of this lesion. Keywords: Acute myelogenous leukemia, eyelid, granulocytic sarcoma, myeloid sarcoma
INTRODUCTION
to presentation at our facility, he had been diagnosed elsewhere with idiopathic orbital inflammation and treated with 40 mg of prednisone daily for the preceding seven months. One month before seeking a second opinion in our clinic, he self-stopped the prednisone due to concerns about the long-term effects of steroids and noted a worsening in size of the mass (Figure 1). At the time of presentation, he had headaches, night sweats, photophobia, and painless blurred vision in his right eye. His review of symptoms was otherwise normal. A diagnostic workup revealed a critically low hemoglobin of 4.6 g/dl. The patient was referred to the emergency department for further workup and urgent hematology/oncology consultation. Subsequent to the discovery of profound anemia, acute myelogenous leukemia (AML) M1 was diagnosed by bone-marrow biopsy. After induction chemotherapy, the patient’s eyelid lesion greatly reduced in size and an eyelid A biopsy was deferred (Figure 2). Six months after initial presentation (5 months after induction chemotherapy with Cytarabine and Idarubicin), the patient returned to
Myeloid sarcoma (also known as chloroma or granulocytic sarcoma) is a rare tumor that can occur in isolation or concurrently with a myelodysplastic syndrome, myeloproliferative disease, or acute myeloid leukemia (AML). Associated with different types of myeloid leukemia, myeloid sarcoma is a tumor composed of myeloid blasts that occur in an extramedullary site. Orbital and ocular adenexal involvement is uncommon, but can be the presenting sign of systemic disease.1
METHODS This is a retrospective case report.
RESULTS A 19-year-old African-American man presented with a one-year history of a right upper eyelid mass. Prior
Received 17 February 2014; Revised 16 March 2014; Accepted 19 June 2014; Published online 22 July 2014 Correspondence: Paul O. Phelps, Department of Ophthalmology, John H. Stroger Jr., Hospital of Cook County, 420 S Clinton St., Apt 306, Chicago, 60607 IL, USA, Tel: 608-406-0471, E-mail: [email protected]
30
Eyelid Myeloid Sarcoma 31
Orbit Downloaded from informahealthcare.com by University of Ulster at Jordanstown on 03/31/15 For personal use only.
our clinic with significant enlargement of his right upper eyelid mass (Figure 3). Biopsy of the eyelid confirmed myeloid sarcoma (Figure 4). The patient then received two more consolidation cycles of chemotherapy and orbital radiotherapy, which successfully decreased the size of the lesion. Thirteen months after presentation to our clinic, he presented to the emergency department with severe dehydration and shortness of breath. He became asystolic during intubation and could not be resuscitated.
CONCLUSIONS This is a case report of a patient who was diagnosed with AML one year after developing a symptomatic right upper eyelid mass. Several case reports and small case series identify orbital involvement in patients diagnosed with myeloid sarcoma.1–4 One case series identified pediatric patients with bilateral orbital masses to have an increased risk of myeloid sarcoma, and suggested that those patients should be promptly evaluated for AML.2 Patients with AML
FIGURE 1. (A) Clinical photograph demonstrated a right sided mechanical blepharoptosis caused by a lesion leading to frontalis muscle; (B) Right upper eyelid eversion demonstrates eyelid infiltration by tumor.
FIGURE 2. (A) Clinical photograph demonstrates improvement of right mechanical blepharoptosis with mild residual ptosis; (B) Right upper eyelid eversion demonstrates an improvement in eyelid infiltration by tumor.
FIGURE 3. (A) Clinical photograph 5 months after completion of chemotherapy demonstrates an enlargement of right upper eyelid mass; (B) CT Orbit, demonstrates a large lesion isolated to eyelid.
!
2015 Informa Healthcare USA, Inc.
32 P. O. Phelps et al.
Orbit Downloaded from informahealthcare.com by University of Ulster at Jordanstown on 03/31/15 For personal use only.
FIGURE 4. (A) H&E staining (40) demonstrates immature myeloid cells with scanty cytoplasm; (B) CD117 (c-kit) expression is high, which confirmed the diagnosis of AML.
typically do not demonstrate ocular findings, but when they do, findings are usually limited to the retina and optic nerve.5 One case of eyelid myeloid sarcoma in all four eyelids did not precede systemic involvement.6 Another case of myeloid sarcoma isolated to the eyelid was associated with leukemia in an infant, which was not differentiated at the time of death.7 Prior case series have noted that myeloid sarcoma is more prevalent in children from the Mediterranean basin.3,8 To our knowledge, myeloid sarcoma isolated to the eyelid as the presenting sign of AML has not been previously reported. This case demonstrates such a presentation, and the poor prognosis of this rare but important lesion. Additionally, the patient in our case had delayed diagnosis with a fatal outcome, whereas a recent case report identified a good outcome in a patient for whom a correct diagnosis was made at initial presentation and treatment was initiated rapidly.1
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
REFERENCES 1. Montoro J, Tormo M. Images in clinical medicine. Myeloid sarcoma. N Engl J Med 2012;369(24):2332. 2. Shields JA, Stopyra GA, Marr BP, et al. Bilateral orbital myeloid sarcoma as initial sign of acute myeloid leukemia: case report and review of the literature. Arch Ophthalmol 2003;121:138–142. 3. Zimmerman LE, Font RL. Ophthalmic manifestations of granulocytic sarcoma. Am J Ophthalmol 1975;80: 975–990. 4. Chaudhry IA, Alaraj AM, Alkatan HM. Unilateral eyelid swelling, proptosis and diplopia as initial manifestation of acute myeloid leukemia. Saudi J Ophthalmol 2012; 26(2):241–244. 5. Karesh JW, Goldman EJ, Reck K, et al. A prospective ophthalmic evaluation of patient with acute myeloid leukemia: correlation of ocular and hematologic findings. J Clin Oncol 1989;10:1528–1532. 6. Thomas SA, Durairaj VD. Isolated myeloid sarcoma presenting in all four eyelids. Ophthal Plas Reconstur Surg 2007;23:336–337. 7. Ford JG, Yeatts RP, Hartz JW, Chauvenet A. Granulocytic sarcoma of the eyelid as a presenting sign of leukemia. J Pediatr Ophthalmol Strabismus 1993;30:386–387. 8. Cavdar AO, Arcasoy A, Babascan E, et al. Ocular granulocytic sarcoma (chloroma) with acute myelomonocytic leukemia in Turkish children. Cancer 1978;41(4): 1606–1609.
Orbit
C ASE REPORT
Eyelid Myeloid Sarcoma: Ominous Presentation of Acute Myelogenous Leukemia Paul O. Phelps1, Marcus M. Marcet2, Augustine R. Hong1, and Jeffrey W. Nichols1
Orbit Downloaded from informahealthcare.com by University of Ulster at Jordanstown on 03/31/15 For personal use only.
1
Department of Ophthalmology, John H. Stroger, Jr., Hospital of Cook County, Chicago, IL, USA and 2 Department of Ophthalmology, University of Hong Kong, Cyberport, Hong Kong
ABSTRACT A 19 year-old African American man presented to our clinic for a second opinion about a right upper eyelid mass which had been recalcitrant to treatment for nonspecific orbital inflammation by an outside facility. Examination for systemic causes of the patients eyelid lesion led to a diagnosis of acute myelogenous leukemia (AML) FAB subtype M1. A subsequent biopsy of the eyelid tumor confirmed the diagnosis of a myeloid sarcoma. The patient succumbed to complications from his leukemia within 13 months of presentation. This case report is the first, to our knowledge, of an eyelid myeloid sarcoma as the presenting sign of AML and demonstrates the poor prognosis of this lesion. Keywords: Acute myelogenous leukemia, eyelid, granulocytic sarcoma, myeloid sarcoma
INTRODUCTION
to presentation at our facility, he had been diagnosed elsewhere with idiopathic orbital inflammation and treated with 40 mg of prednisone daily for the preceding seven months. One month before seeking a second opinion in our clinic, he self-stopped the prednisone due to concerns about the long-term effects of steroids and noted a worsening in size of the mass (Figure 1). At the time of presentation, he had headaches, night sweats, photophobia, and painless blurred vision in his right eye. His review of symptoms was otherwise normal. A diagnostic workup revealed a critically low hemoglobin of 4.6 g/dl. The patient was referred to the emergency department for further workup and urgent hematology/oncology consultation. Subsequent to the discovery of profound anemia, acute myelogenous leukemia (AML) M1 was diagnosed by bone-marrow biopsy. After induction chemotherapy, the patient’s eyelid lesion greatly reduced in size and an eyelid A biopsy was deferred (Figure 2). Six months after initial presentation (5 months after induction chemotherapy with Cytarabine and Idarubicin), the patient returned to
Myeloid sarcoma (also known as chloroma or granulocytic sarcoma) is a rare tumor that can occur in isolation or concurrently with a myelodysplastic syndrome, myeloproliferative disease, or acute myeloid leukemia (AML). Associated with different types of myeloid leukemia, myeloid sarcoma is a tumor composed of myeloid blasts that occur in an extramedullary site. Orbital and ocular adenexal involvement is uncommon, but can be the presenting sign of systemic disease.1
METHODS This is a retrospective case report.
RESULTS A 19-year-old African-American man presented with a one-year history of a right upper eyelid mass. Prior
Received 17 February 2014; Revised 16 March 2014; Accepted 19 June 2014; Published online 22 July 2014 Correspondence: Paul O. Phelps, Department of Ophthalmology, John H. Stroger Jr., Hospital of Cook County, 420 S Clinton St., Apt 306, Chicago, 60607 IL, USA, Tel: 608-406-0471, E-mail: [email protected]
30
Eyelid Myeloid Sarcoma 31
Orbit Downloaded from informahealthcare.com by University of Ulster at Jordanstown on 03/31/15 For personal use only.
our clinic with significant enlargement of his right upper eyelid mass (Figure 3). Biopsy of the eyelid confirmed myeloid sarcoma (Figure 4). The patient then received two more consolidation cycles of chemotherapy and orbital radiotherapy, which successfully decreased the size of the lesion. Thirteen months after presentation to our clinic, he presented to the emergency department with severe dehydration and shortness of breath. He became asystolic during intubation and could not be resuscitated.
CONCLUSIONS This is a case report of a patient who was diagnosed with AML one year after developing a symptomatic right upper eyelid mass. Several case reports and small case series identify orbital involvement in patients diagnosed with myeloid sarcoma.1–4 One case series identified pediatric patients with bilateral orbital masses to have an increased risk of myeloid sarcoma, and suggested that those patients should be promptly evaluated for AML.2 Patients with AML
FIGURE 1. (A) Clinical photograph demonstrated a right sided mechanical blepharoptosis caused by a lesion leading to frontalis muscle; (B) Right upper eyelid eversion demonstrates eyelid infiltration by tumor.
FIGURE 2. (A) Clinical photograph demonstrates improvement of right mechanical blepharoptosis with mild residual ptosis; (B) Right upper eyelid eversion demonstrates an improvement in eyelid infiltration by tumor.
FIGURE 3. (A) Clinical photograph 5 months after completion of chemotherapy demonstrates an enlargement of right upper eyelid mass; (B) CT Orbit, demonstrates a large lesion isolated to eyelid.
!
2015 Informa Healthcare USA, Inc.
32 P. O. Phelps et al.
Orbit Downloaded from informahealthcare.com by University of Ulster at Jordanstown on 03/31/15 For personal use only.
FIGURE 4. (A) H&E staining (40) demonstrates immature myeloid cells with scanty cytoplasm; (B) CD117 (c-kit) expression is high, which confirmed the diagnosis of AML.
typically do not demonstrate ocular findings, but when they do, findings are usually limited to the retina and optic nerve.5 One case of eyelid myeloid sarcoma in all four eyelids did not precede systemic involvement.6 Another case of myeloid sarcoma isolated to the eyelid was associated with leukemia in an infant, which was not differentiated at the time of death.7 Prior case series have noted that myeloid sarcoma is more prevalent in children from the Mediterranean basin.3,8 To our knowledge, myeloid sarcoma isolated to the eyelid as the presenting sign of AML has not been previously reported. This case demonstrates such a presentation, and the poor prognosis of this rare but important lesion. Additionally, the patient in our case had delayed diagnosis with a fatal outcome, whereas a recent case report identified a good outcome in a patient for whom a correct diagnosis was made at initial presentation and treatment was initiated rapidly.1
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
REFERENCES 1. Montoro J, Tormo M. Images in clinical medicine. Myeloid sarcoma. N Engl J Med 2012;369(24):2332. 2. Shields JA, Stopyra GA, Marr BP, et al. Bilateral orbital myeloid sarcoma as initial sign of acute myeloid leukemia: case report and review of the literature. Arch Ophthalmol 2003;121:138–142. 3. Zimmerman LE, Font RL. Ophthalmic manifestations of granulocytic sarcoma. Am J Ophthalmol 1975;80: 975–990. 4. Chaudhry IA, Alaraj AM, Alkatan HM. Unilateral eyelid swelling, proptosis and diplopia as initial manifestation of acute myeloid leukemia. Saudi J Ophthalmol 2012; 26(2):241–244. 5. Karesh JW, Goldman EJ, Reck K, et al. A prospective ophthalmic evaluation of patient with acute myeloid leukemia: correlation of ocular and hematologic findings. J Clin Oncol 1989;10:1528–1532. 6. Thomas SA, Durairaj VD. Isolated myeloid sarcoma presenting in all four eyelids. Ophthal Plas Reconstur Surg 2007;23:336–337. 7. Ford JG, Yeatts RP, Hartz JW, Chauvenet A. Granulocytic sarcoma of the eyelid as a presenting sign of leukemia. J Pediatr Ophthalmol Strabismus 1993;30:386–387. 8. Cavdar AO, Arcasoy A, Babascan E, et al. Ocular granulocytic sarcoma (chloroma) with acute myelomonocytic leukemia in Turkish children. Cancer 1978;41(4): 1606–1609.
Orbit
