Myeloid sarcomas of the head and neck in pediatric patients with myeloid leukemia.
Myeloid sarcoma is a rare extramedullary tumor composed of malignant myeloid cells that occur in the presence of myeloid leukemia. We report a case series of pediatric head and neck myeloid sarcomas representative of the epidemiology, symptomatology, laboratorial correlations, prognoses, and treatment of extramedullary leukemia. Presented are 3 cases involving patients ranging from 17 months to 11 years of age. Two patients were successfully treated with chemotherapy, and in the third patient, a large lytic lesion was treated palliatively with proton beam therapy. Knowledge and recognition of myeloid sarcomas is important as they can be locally invasive, and they may also be used as a diagnostic tool or a prognostic indicator for leukemia.
Myeloid sarcomas are rare tumors composed of malignant immature myeloid precursor cells. They most commonly arise in patients previously diagnosed with a myeloproliferative disorder, but they can be the presenting sign of cancer or even precede the onset of hematogenous disease by months to years. (1,2) The incidence of these tumors is quite low; they occur in 3 to 8% of patients diagnosed with acute myeloid leukemia (AML) and have a predilection for the pediatric population but with no gender predominance. (3)
In this article we report a case series of head and neck myeloid sarcomas representative of the epidemiology, symptomatology, laboratorial correlations, prognoses, and treatment of extramedullary leukemia.
Knowledge and recognition of myeloid sarcomas is important as they can be locally invasive, and they may also be used as a diagnostic tool or a prognostic indicator for leukemia.
Patient 1. A 17-month-old girl presented with leg pain and difficulty walking. A peripheral blood smear showed blasts, and a bone marrow biopsy and flow cytometry confirmed AML with monocytic differentiation. Fluorescence in situ hybridization (FISH) revealed no abnormalities. Chromosome analysis revealed a complex abnormal clonal population including multiple translocations and loss of genetic material.
The patient was treated with chemotherapy and a bone marrow transplant, but after 6 months she developed right-sided periorbital edema and proptosis. Computed tomography (CT) demonstrated a 3-cm lytic, soft-tissue mass obstructing the right nasal cavity. Biopsy of the mass was consistent with myeloid sarcoma, and bone marrow biopsy revealed relapsed AML.
The patient was then treated palliatively. Although her myeloid sarcoma responded to proton beam radiotherapy, the patient died from her disease approximately 8 months after her initial presentation.
Patient 2. An 11-year-old girl presented with nausea, weakness, and fatigue. Her peripheral blood smear and bone marrow biopsy demonstrated blasts containing Auer rods, diagnostic of AML (figure 1). During treatment, the patient underwent CT of her head and magnetic resonance imaging (MRI) of her brain, revealing a destructive, 2.3-cm mass in the right maxillary sinus extending into the orbit and retromaxillary fat (figure 2). The biopsy of the mass confirmed myeloid sarcoma (figure 1). The tumor resolved with chemotherapy, but the patient subsequently had a relapse of the AML. She was treated with a bone marrow transplant and chemotherapy but ultimately died from the disease.
[FIGURE 1 OMITTED]
Patient 3. A 4-year-old boy presented with easy bruising, gingival bleeding, and thrombocytopenia. A bone marrowbiopsy was worrisome for myelodysplastic syndrome, but a definitive diagnosis was not initially reached. The patient subsequently developed a slowly enlarging, right-upper-lip lesion and facial swelling. A biopsy showed the mass to be a myeloid sarcoma, and a second bone marrow biopsy confirmed the diagnosis of AML. The patient underwent chemotherapy and a bone marrow transplant, and he remains free of disease.
Myeloid sarcomas, initially named chloromas because of their green color, were first described by Allen Burns in 1811 and later associated with AML by George Dock in 1895. (4,5) They are rare tumors, with an incidence of 3 to 4.7% among myeloproliferative disorders, predominantly in AML. (3,6) There is no gender predilection, but the incidence is higher in the pediatric population. (3) In the head and neck region, the tumors most commonly occur in the soft palate, nasopharynx, orbit, scalp, and face. (1,4)
[FIGURE 2 OMITTED]
Myeloid sarcomas are thought to arise from the migration of neoplastic cells from the bone marrow through Haversian canals. Once unimpeded by bone, the cells may spread hematogenously to more distant locations. (4) Symptoms are caused by local mass effect and may include pain, swelling, vision changes, epistaxis, nasal obstruction, and otitis media secondary to eustachian tube obstruction.
The tumors are radiographically nonspecific; their signal intensity is similar to that of bone marrow on MRI, and they appear as a well-defined area ofincreased attenuation with a peripheral zone of enhancement on CT. Although nondiagnostic, imaging is useful in evaluating the extent of disease involvement. (7)
Myeloid sarcomas characteristically have a greenish hue due to the presence of myeloperoxidase in the immature granulocytic cells. Histologically, the tumors are composed of infiltrating mononuclear cells and granulocytic cells at various stages of maturation, and often include eosinophilic myelocytes. (6) Immunohistochemical staining varies based on the degree of cellular differentiation within the tumor; it is particularly helpful in avoiding misdiagnoses in patients in whom myeloid sarcoma is the presenting symptom of systemic hematologic disease. (8) As many as 53.6% of patients carry a chromosomal abnormality, most commonly monosomy 7 or trisomy 8. (9)
Cytogenetic studies of myeloid sarcomas have shown they are more common in patients with a t(8;21) abnormality, (5) monocytic leukemic cell lineage, (10) cellular expression of CD56,1 lq23 mutation, (11) or FAB classification as M2, M4, M5a, or M5b. (6) In the cases presented here, staining with myeloperoxidase, CD68, and lysozyme was particularly helpful in making the diagnosis of myeloid sarcoma. Patient 2 had a karyotype with t(8;21), and patient 1 had complex and progressive chromosomal abnormalities, including the loss of chromosome 20, t(8; 16) abnormality, and the deletion and addition of material to chromosome 6.
Myeloid sarcomas are generally quite responsive to chemotherapy and therefore do not require surgical resection unless it is needed for debulking or palliative measures. The tumors are also radiosensitive, and directed radiation therapy may be used if faster involution is required. (4)
From a prognostic standpoint, myeloid sarcomas may signal disease relapse or progression in patients known to have leukemia, and they may portend the rapid onset of the disease in those not yet diagnosed. (1,12) Myeloid sarcomas are associated with low rates of complete remission, but their effect on overall survival has not been conclusively demonstrated. (11,13)
Knowledge of myeloid sarcoma is vital because its recognition may lead to earlier treatment and improved prognosis. Immunohistochemistry and cytogenetic studies play a critical role in differentiating myeloid sarcoma from histologically similar tumors that are treated much differently. In this article we seek to increase understanding of the disease to facilitate more rapid diagnosis and initiation of treatment, with a view to improved outcomes in patient care and survival.
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David W. Cuthbertson, MD; Jyotinder Nain Punia, MD; Vicki L. Owczarzak, MD
From ENT Associates of Lubbock, Lubbock, Texas (Dr. Cuthbertson); the Department of Pathology and Immunology, Baylor College of Medicine and Texas Childrens Hospital, Houston (Dr. Punia); and Otolaryngology Associates, Fairfax, Va. (Dr. Owczarzak). The cases described in this article occurred at Texas Childrens Hospital.
Corresponding author: David W. Cuthbertson, 3802 22nd St., Ste. 200, Lubbock, TX 79410. Email: firstname.lastname@example.org
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|Title Annotation:||ORIGINAL ARTICLE|
|Author:||Cuthbertson, David W.; Punia, Jyotinder Nain; Owczarzak, Vicki L.|
|Publication:||Ear, Nose and Throat Journal|
|Date:||Sep 1, 2016|
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