Extraskeletal Ewing's sarcoma is rarely found in the head and neck regions. We report an unusual case of extraskeletal Ewing's Sarcoma of the parapharynx region in a 49-year-old man who presented with blindness. MRI examination showed marked enhancement of tumor thrombosis involving the superior sagittal sinus, straight sinus, transverse sinus, sigmoid sinus, and internal jugular vein. The final diagnosis was extraskeletal Ewing's sarcoma after biopsy of the internal jugular vein thrombosis by histopathological evaluation and immunohistochemical assay. In addition, the patient was diagnosed as having adenocarcinoma of the rectum by biopsy of the rectal mass. The patient was treated with systemic chemotherapy and showed improved response with durable remission. The patient's visual acuity, however, did not improve.
Antineoplastic Agents/therapeutic use ; *Blindness ; Cyclophosphamide/therapeutic use ; Doxorubicin/therapeutic use ; Head and Neck Neoplasms/diagnosis/drug therapy/*pathology ; Humans ; Male ; Middle Aged ; Sarcoma, Ewing's/diagnosis/drug therapy/*pathology ; Vincristine/therapeutic use

OBJECTIVETo study the clinicopathologic features of granulocytic sarcoma.

METHODSThe clinical and pathologic findings of 38 cases of granulocytic sarcoma were retrospectively analyzed. Immunohistochemical study was performed and the literature was reviewed.

RESULTSThe age of patients ranged from 2 to 77 years (mean = 43.3 years). The male-to-female ratio was 1.5:1. Major clinical presentations included superficial lymph node enlargement and painful soft tissue mass. Follow-up data were available in 18 patients; and 14 of them died of tumor-related diseases. The average duration of survival of the patients was 16.9 months. Histologically, the tumor cells were relatively uniform in appearance and small to medium in size. The cytoplasm was scanty and pale in color. The nuclei were round or focally irregular, with fine chromatin and inconspicuous nucleoli. Mitosis figures were readily identified. Scattered immature eosinophilic myelocytes were seen. Immunohistochemical study showed that the tumor cells in all cases expressed MPO and CD43. Most cases were also positive for CD68, lysozyme, CD99 and TdT. The staining for CD3, CD20, CD79a, pan-cytokeratin and PLAP were negative.

CONCLUSIONSGranulocytic sarcoma is a known histologic mimicker of non-Hodgkin lymphoma, Ewing sarcoma/PNET and embryonal rhabdomyosarcoma. Detailed morphologic examination, when coupled with immunohistochemical study, is useful in arriving at a correct diagnosis.

Adolescent ; Adult ; Aged ; Burkitt Lymphoma ; metabolism ; pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Leukosialin ; metabolism ; Lymph Nodes ; pathology ; Male ; Middle Aged ; Muscle Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Ovarian Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Peroxidase ; metabolism ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; metabolism ; pathology ; Retrospective Studies ; Sarcoma, Ewing ; metabolism ; pathology ; Sarcoma, Myeloid ; drug therapy ; metabolism ; pathology ; surgery ; Skin Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Survival Rate ; Young Adult