Korean J Intern Med.  2007 Jun;22(2):133-137. 10.3904/kjim.2007.22.2.133.

Extraskeletal Ewing's Sarcoma of the Head and Neck Presenting as Blindness

Affiliations
  • 1Department of Internal Medicine, Korea Institute of Radiological & Medical Sciences, Seoul, Korea. ryooby@kcch.re.kr
  • 2Department of Neurosurgery, Korea Institute of Radiological & Medical Sciences, Seoul, Korea.
  • 3Department of Pathology, Korea Institute of Radiological & Medical Sciences, Seoul, Korea.

Abstract

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.

Keyword

Sarcoma; Head and neck neoplasms; Blindness

MeSH Terms

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
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