Germinoma Involving the Retina: An Unusual Presentation of Recurrent Intracranial Mixed Germ Cell Tumor.

Germinoma Involving the Retina: An Unusual Presentation of Recurrent Intracranial Mixed Germ Cell Tumor.

World Neurosurg. 2019 Jan 07;:

Authors: Abu Arja MH, Stalling M, Governale LS, Pierson CR, Rusin JA, Palmer JD, Finlay JL, Olshefski R, Boué DR

Abstract
BACKGROUND: We report a patient with primary central nervous system mixed malignant germ cell tumor (GCT) who presented with recurrent malignant germinomatous infiltration of the retina.
CASE DESCRIPTION: A ten-year-old girl initially presented with a large suprasellar mixed malignant GCT with a near-complete response after initial induction chemotherapy and irradiation. Three and half years after initial therapy, she presented with progressively worsening vision in her left eye. Magnetic resonance imaging showed infiltrative changes within the left optic nerve but no discrete mass. Serum and cerebrospinal fluid (CSF) tumor markers were not elevated and CSF cytology was negative. Left optic nerve biopsy confirmed the presence of mature teratoma and pure germinoma components. She was treated with gross-total resection of the left eye and optic nerve and chemotherapy. Histopathologic evaluation of the optic nerve showed only mature teratoma elements but with pure germinoma cells infiltrating the inner layers of the retina.
CONCLUSIONS: Loco-regional extension of suprasellar GCT to the optic nerve is not uncommon; however, infiltration of the tumor into the retina is not reported in the literature. Early detection of optic pathway involvement and proper delineation of the irradiation field may prevent GCT infiltration of the retina with subsequent vision loss.

PMID: 30630044 [PubMed - as supplied by publisher]

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