A case of germinoma located in the fornix inducing transsynaptic atrophy of the Papez circuit

Germinoma is a rare CNS germ cell tumor preferentially affecting children and young adults. Intracranial germinomas arise typically in the neurohypophysis and pineal region and occasionally in the basal ganglia and thalamus. Germinomas in the basal ganglia and thalamus are characterized by the ipsil...

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Main Authors: Shintaro Kano, MD, PhD, Mitsuru Matsuki, MD, PhD, Rieko Furukawa, MD, PhD, Waka Nakata, MD, PhD, Hirofumi Oguma, MD, PhD, Akira Gomi, MD, PhD, Harushi Mori, MD, PhD
Format: Article
Language:English
Published: Elsevier 2023-01-01
Series:Radiology Case Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S193004332200944X
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author Shintaro Kano, MD, PhD
Mitsuru Matsuki, MD, PhD
Rieko Furukawa, MD, PhD
Waka Nakata, MD, PhD
Hirofumi Oguma, MD, PhD
Akira Gomi, MD, PhD
Harushi Mori, MD, PhD
author_facet Shintaro Kano, MD, PhD
Mitsuru Matsuki, MD, PhD
Rieko Furukawa, MD, PhD
Waka Nakata, MD, PhD
Hirofumi Oguma, MD, PhD
Akira Gomi, MD, PhD
Harushi Mori, MD, PhD
author_sort Shintaro Kano, MD, PhD
collection DOAJ
description Germinoma is a rare CNS germ cell tumor preferentially affecting children and young adults. Intracranial germinomas arise typically in the neurohypophysis and pineal region and occasionally in the basal ganglia and thalamus. Germinomas in the basal ganglia and thalamus are characterized by the ipsilateral cerebral and brainstem hemiatrophy with slowly progressive neurological deficits, which is due to tumor infiltration into the thalamocortical and corticospinal tract and induction of anterograde and retrograde Wallerian degeneration. We report an 11-year-old boy with a mass located in the fornix incidentally discovered on the first work-up of his minor head injury. Imaging findings revealed the ipsilateral atrophy of the mammillary body and the fornix. Stereotactic brain biopsy was performed and the final diagnosis was germinoma. The ipsilateral atrophy of the mammillary body and the fornix implied the transsynaptic degeneration via the Papez circuit. We discuss the unique nature of germinomas and underlying pathological mechanisms.
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spelling doaj.art-616f6275235c4ed58c9dda029ffffc622022-12-22T04:39:41ZengElsevierRadiology Case Reports1930-04332023-01-01181405409A case of germinoma located in the fornix inducing transsynaptic atrophy of the Papez circuitShintaro Kano, MD, PhD0Mitsuru Matsuki, MD, PhD1Rieko Furukawa, MD, PhD2Waka Nakata, MD, PhD3Hirofumi Oguma, MD, PhD4Akira Gomi, MD, PhD5Harushi Mori, MD, PhD6Department of Radiology, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke-shi, Tochigi-ken, 329-049, Japan; Corresponding author.Department of Radiology, Jichi Children's Medical Center Tochigi, Shimotsuke-shi, JapanDepartment of Radiology, Jichi Children's Medical Center Tochigi, Shimotsuke-shi, JapanDepartment of Radiology, Jichi Children's Medical Center Tochigi, Shimotsuke-shi, JapanDepartment of Neurosurgery, Jichi Medical University, Shimotsuke-shi, JapanDepartment of Neurosurgery, Jichi Medical University, Shimotsuke-shi, JapanDepartment of Radiology, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke-shi, Tochigi-ken, 329-049, JapanGerminoma is a rare CNS germ cell tumor preferentially affecting children and young adults. Intracranial germinomas arise typically in the neurohypophysis and pineal region and occasionally in the basal ganglia and thalamus. Germinomas in the basal ganglia and thalamus are characterized by the ipsilateral cerebral and brainstem hemiatrophy with slowly progressive neurological deficits, which is due to tumor infiltration into the thalamocortical and corticospinal tract and induction of anterograde and retrograde Wallerian degeneration. We report an 11-year-old boy with a mass located in the fornix incidentally discovered on the first work-up of his minor head injury. Imaging findings revealed the ipsilateral atrophy of the mammillary body and the fornix. Stereotactic brain biopsy was performed and the final diagnosis was germinoma. The ipsilateral atrophy of the mammillary body and the fornix implied the transsynaptic degeneration via the Papez circuit. We discuss the unique nature of germinomas and underlying pathological mechanisms.http://www.sciencedirect.com/science/article/pii/S193004332200944XGerminomaFornixMamillary bodyPapez circuitAtrophy
spellingShingle Shintaro Kano, MD, PhD
Mitsuru Matsuki, MD, PhD
Rieko Furukawa, MD, PhD
Waka Nakata, MD, PhD
Hirofumi Oguma, MD, PhD
Akira Gomi, MD, PhD
Harushi Mori, MD, PhD
A case of germinoma located in the fornix inducing transsynaptic atrophy of the Papez circuit
Radiology Case Reports
Germinoma
Fornix
Mamillary body
Papez circuit
Atrophy
title A case of germinoma located in the fornix inducing transsynaptic atrophy of the Papez circuit
title_full A case of germinoma located in the fornix inducing transsynaptic atrophy of the Papez circuit
title_fullStr A case of germinoma located in the fornix inducing transsynaptic atrophy of the Papez circuit
title_full_unstemmed A case of germinoma located in the fornix inducing transsynaptic atrophy of the Papez circuit
title_short A case of germinoma located in the fornix inducing transsynaptic atrophy of the Papez circuit
title_sort case of germinoma located in the fornix inducing transsynaptic atrophy of the papez circuit
topic Germinoma
Fornix
Mamillary body
Papez circuit
Atrophy
url http://www.sciencedirect.com/science/article/pii/S193004332200944X
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