Isolated Orbital Endodermal Sinus Tumor

Abstract

Endodermal sinus tumor is a malignant germ-cell tumor that usually involves the gonadal tissue. Primary extragonadal sites occur rarely, and include the orbit. Treatment options include surgical removal and chemotherapy.

Primary orbital endodermal sinus tumor (yolk sac tumor)

“…[3][4][5] In contrast to visceral ESTs, which often prove fatal within a short time, patients with orbital ESTs may survive longer as the result of earlier recognition and prompt treatment. 3,5 Serum ␣-fetoprotein, a useful marker in the diagnosis and follow-up of gonadal ESTs, also has been found to be increased in patients with orbital ESTs. 5,9,10 In our patient, serum ␣-fetoprotein levels decrease to within normal range 6 weeks after the institution of chemotherapy.…”

“…4,6,8,9 Most patients were managed with incisional biopsy, surgical debulking, or exenteration followed by chemotherapy. [3][4][5][6][7][8][9][10] Although long-term follow-up data are available in only a few cases, those patients who had tumors limited to the orbit and who were managed by extensive surgery and chemotherapy seemed to have a better prognosis. [3][4][5] In contrast to visceral ESTs, which often prove fatal within a short time, patients with orbital ESTs may survive longer as the result of earlier recognition and prompt treatment.…”

“…[3][4][5][6][7][8][9][10] Although long-term follow-up data are available in only a few cases, those patients who had tumors limited to the orbit and who were managed by extensive surgery and chemotherapy seemed to have a better prognosis. [3][4][5] In contrast to visceral ESTs, which often prove fatal within a short time, patients with orbital ESTs may survive longer as the result of earlier recognition and prompt treatment. 3,5 Serum ␣-fetoprotein, a useful marker in the diagnosis and follow-up of gonadal ESTs, also has been found to be increased in patients with orbital ESTs.…”

“…3 The majority of these extragonadal lesions shows an axial distribution pattern, supporting the view that ectopic germ cells resulting from migration abnormalities and misplacement during embryogenesis may give rise to EST. 4,5 The tumor is composed of primitive cells that are committed to reproduce the extraembryonic structures of the early embryonic stages. Schiller-Duval bodies ( Figure 3B) are pathognomonic for EST.…”

“…4 Since the first description of EST in the orbit by Katz et al 6 in 1982, 12 orbital cases have been reported in the English-language literature. [3][4][5][6][7][8][9][10] The patients presented between 3 months and 4 years of age, with unilateral proptosis being the most common initial sign. 2-10 Eight of these patients had concomitant intracranial, nasopharyngeal, maxillary sinus, or pterygopalatine fossa extensions.…”