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[Clival chordoma in an infant; case report and review of the literature].
No Shinkei Geka. 1992 Jul; 20(7):809-13.NS

Abstract

Skull base chordoma in infants is a very rare entity in spite of its congenital origin; only 11 clinical cases can be found in the literature so far. Here we report such a case and review the literature. The case is that of a 3.5-year old boy suffering from left abducent nerve palsy for 5 months. CT scan revealed an isodense mass lesion with bone destruction involving the clivus and left petrous apex, and was homogeneously enhanced on post-contrast study. MRI disclosed the clival tumor as a long T1 and long T2 mass. Angiogram showed no tumor stain. The tumor was preoperatively diagnosed as clival chordoma, and was partially removed via left transpetrosal-transtentorial approach. The tumor was found to extend into the subdural space through Dorrello's canal and compress the abducent nerve. Histological examination with H & E, PAS, mucicarmine, and reticulin stainings led us to a diagnosis of "typical chordoma". Electron microscopy demonstrated the mitochondria-endoplasmic reticulum complexes (MERC), glycogen granules, and vacuoles in the tumor cells. Postoperative irradiation (total dose 55 Gy) was performed. At present, 30 months after the operation, no evidence of tumor regrowth nor hypothalamus-pituitary deficiency is recognizable and the patient is free from left abducent nerve palsy. It is concluded that skull base chordoma in infants should be postoperatively irradiated in an appropriate manner.

Authors+Show Affiliations

Inagaki H
Department of Neurosurgery, Tottori University School of Medicine, Yonago, Japan.
Anno Y
No affiliation info available
Hori T
No affiliation info available
Ohama E
No affiliation info available

MeSH

Child, PreschoolChordomaCranial Fossa, PosteriorHumansMagnetic Resonance ImagingMaleRadiotherapy DosageSkull Neoplasms

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

jpn

PubMed ID

1630574

Citation

Inagaki, H, et al. "[Clival Chordoma in an Infant; Case Report and Review of the Literature]." No Shinkei Geka. Neurological Surgery, vol. 20, no. 7, 1992, pp. 809-13.
Inagaki H, Anno Y, Hori T, et al. [Clival chordoma in an infant; case report and review of the literature]. No Shinkei Geka. 1992;20(7):809-13.
Inagaki, H., Anno, Y., Hori, T., & Ohama, E. (1992). [Clival chordoma in an infant; case report and review of the literature]. No Shinkei Geka. Neurological Surgery, 20(7), 809-13.
Inagaki H, et al. [Clival Chordoma in an Infant; Case Report and Review of the Literature]. No Shinkei Geka. 1992;20(7):809-13. PubMed PMID: 1630574.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Clival chordoma in an infant; case report and review of the literature]. AU - Inagaki,H, AU - Anno,Y, AU - Hori,T, AU - Ohama,E, PY - 1992/7/1/pubmed PY - 1992/7/1/medline PY - 1992/7/1/entrez SP - 809 EP - 13 JF - No shinkei geka. Neurological surgery JO - No Shinkei Geka VL - 20 IS - 7 N2 - Skull base chordoma in infants is a very rare entity in spite of its congenital origin; only 11 clinical cases can be found in the literature so far. Here we report such a case and review the literature. The case is that of a 3.5-year old boy suffering from left abducent nerve palsy for 5 months. CT scan revealed an isodense mass lesion with bone destruction involving the clivus and left petrous apex, and was homogeneously enhanced on post-contrast study. MRI disclosed the clival tumor as a long T1 and long T2 mass. Angiogram showed no tumor stain. The tumor was preoperatively diagnosed as clival chordoma, and was partially removed via left transpetrosal-transtentorial approach. The tumor was found to extend into the subdural space through Dorrello's canal and compress the abducent nerve. Histological examination with H & E, PAS, mucicarmine, and reticulin stainings led us to a diagnosis of "typical chordoma". Electron microscopy demonstrated the mitochondria-endoplasmic reticulum complexes (MERC), glycogen granules, and vacuoles in the tumor cells. Postoperative irradiation (total dose 55 Gy) was performed. At present, 30 months after the operation, no evidence of tumor regrowth nor hypothalamus-pituitary deficiency is recognizable and the patient is free from left abducent nerve palsy. It is concluded that skull base chordoma in infants should be postoperatively irradiated in an appropriate manner. SN - 0301-2603 UR - https://www.unboundmedicine.com/medline/citation/1630574/[Clival_chordoma_in_an_infant DB - PRIME DP - Unbound Medicine ER -