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Intrasellar Chordoma Mimicking Pituitary Macroadenoma with Hyperprolactinemia and Hypopituitarism: Clinical Images with a Surgical Video

Fujita, Yuichi Uozumi, Yoichi Sasayama, Takashi 神戸大学

2023.02

概要

A purely intrasellar chordoma is rare among skull base chordomas and is recognized as originating from ectopic embryological notochord located in the sella turcica. In view of its rarity and nonspecific symptoms, clinicians may misdiagnose intrasellar chordoma as pituitary adenoma based on preoperative radiographic images. In this report, we present an intrasellar chordoma that clinically mimicked pituitary macroadenoma with hyperprolactinemia and hypopituitarism and was successfully resected by endoscopic endonasal transsphenoidal surgery. This case demonstrated radiographic features that chordoma should be suspected in sellar lesions. The enlarged sellar with thinned remodeled bone without clival destruction was firstly reminiscent of pituitary adenoma, whereas the very high signal on T2-weighted images and heterogeneous enhancement characteristically suggested chordoma. This rare diagnosis must be considered in the preoperative evaluation of sellar lesions because it can affect how the neurosurgeon prepares for surgery and the surgical goals.

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Figure legends

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Figure. 1 Preoperative computed tomography images. Sagittal brain window image (A)

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shows an isodense sellar lesion. Sagittal (B) and axial (C) bone window images show

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ballooning of the sella turcica and thinning of the dorsum sellae without intratumoral

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calcification or clival invasion.

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Figure. 2 Preoperative sagittal (A–C), coronal (E, F), and axial (G, H) magnetic resonance

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images with position of coronal and axial slice (H). A solid sellar tumor with the right

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cavernous sinus invasion shows iso-intensity on T1-weighted image (A), very high-intensity

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on T2-weighted images (C, E, G), and well-enhanced margin with heterogeneous internal

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enhancement on contrast-enhanced T1-weighted images (B, D, F).

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Figure. 3 Postoperative sagittal (A), coronal (B), and axial (C) contrast-enhanced T1-

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weighted images confirm total resection of the tumor with anatomic preservation of the

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normal pituitary gland tissue (white arrow in B).

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Figure. 4 Photomicrographs show tissue corresponding to histopathological diagnosis of

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chordoma (A, B).

Fig. 1

Axial

CT

Sagittal

Fig. 2

CE-T1WI

T2WI

Sagittal

Coronal

Axial

Axial

CE-T1WI

Coronal

Sagittal

T1WI

Fig. 3

Coronal

Axial

Postoperative CE-T1WI

Sagittal

Fig. 4

Brachyury

Histopathology

H-E

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