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Presented by William Westra, M.D. and prepared by Maryam Farinola M.D.
Case 5: 69 year-old man with a nodule on the gingival
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1. Question
Week 146: Case 5
69 year-old man with a nodule on the gingivalimages/071403case5fig1.jpg
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images/071403case5fig4.jpgCorrect
Answer: Granulocytic sarcoma
Histology: Histologic sections demonstrate a diffuse infiltrate of large, markedly atypical cells. The overlying surface epithelium is ulcerated and inflamed, but it is not dysplastic. The vast majority of the large and atypical cells are negative for B and T cell markers (CD20 and CD3), but are strongly positive for CLA and CD68. There is focal positivity for myeloperoxidase. Stains for CD34 and CD117 are negative.
Discussion: The histologic findings and the immunophenotype are consistent with an extramedullary myeloid tumor (granulocytic sarcoma) with myelomonocytic differentiation. Granulocytic sarcoma is a tumor mass of immature myeloid cells occurring in an extramedullary site. The oral cavity, especially the gums, is a frequently involved site. Oral graulocytic sarcoma presents as a localized soft tissue mass. Clinically, granulocytic sarcoma may occur in three settings: in a patient previously known to have AML; as a sign of blast transformation in a patient with CML or some other chronic myeloproliferative disorder; or in a patient who was previously well. In the present case, the patient was being treated for a myelodysplastic syndrome.
Incorrect
Answer: Granulocytic sarcoma
Histology: Histologic sections demonstrate a diffuse infiltrate of large, markedly atypical cells. The overlying surface epithelium is ulcerated and inflamed, but it is not dysplastic. The vast majority of the large and atypical cells are negative for B and T cell markers (CD20 and CD3), but are strongly positive for CLA and CD68. There is focal positivity for myeloperoxidase. Stains for CD34 and CD117 are negative.
Discussion: The histologic findings and the immunophenotype are consistent with an extramedullary myeloid tumor (granulocytic sarcoma) with myelomonocytic differentiation. Granulocytic sarcoma is a tumor mass of immature myeloid cells occurring in an extramedullary site. The oral cavity, especially the gums, is a frequently involved site. Oral graulocytic sarcoma presents as a localized soft tissue mass. Clinically, granulocytic sarcoma may occur in three settings: in a patient previously known to have AML; as a sign of blast transformation in a patient with CML or some other chronic myeloproliferative disorder; or in a patient who was previously well. In the present case, the patient was being treated for a myelodysplastic syndrome.
