PMC10164933_01
Preoperative sagittal (A), axial (B) T2, and axial (C) T2-fluid-attenuated inversion recovery (FLAIR) sequences showed a huge mass located in the median and paramedian portion of the hypothalamus-chiasmatic region (Dmax 50 cm), with irregular margins infiltrating the uncus-amygdaloid complex in the hypothalamus region and the floor of the third ventricle; the lesion presented central colliquative necrotic component and its signal was relatively homogenous with slightly hypointense in T1 and hyperintense on T2-weighted images, with significant heterogenous post-Gad enhancement and peripherical vasogenic edema. (D) After dural opening, the lesion appeared diffusely infiltrating the infundibulum and third ventricle, and it presented as a grayish-yellow tissue and was highly vascularized. (E) Further resection of the tumor was not performed because the tumor adhered tenaciously to surrounding structures. (F) Histological examination revealed a highly cellular neoplasm having a fibrillary background, composed of pleomorphic, medium-sized cells. Necrosis and microvascular proliferation were also seen (hematoxylin-eosin, original magnification 10x). (G) On immunohistochemistry, tumor cells were GFAP positive (immunoperoxidase staining, original magnification 10x). IDH1 immunostaining was negative (not shown in the figure). ON, optic nerve; Ch, chiasm; T, tumor; Pg, pituitary gland.
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F1
The patient died 1 year after the surgery due to the progression of the disease (Figure 1).
Preoperative sagittal (A) and coronal (B), post-gadolinium MRI scan showing a suprasellar mass (Dmax 3.9 cm) with the third ventricle involvement and heterogenous contrast enhancement compressing the optic chiasm. (C) After dural opening, (D) a fibro-elastic and infiltrating lesion was partially removed in order to obtain optic nerve decompression.
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F2
Intraoperative histological examination showed the presence of atypical cells with a plasmacytoid appearance (Figure 2).
PMC10164933_02
Preoperative sagittal (A) and coronal (B), post-gadolinium MRI scan showing a suprasellar mass (Dmax 3.9 cm) with the third ventricle involvement and heterogenous contrast enhancement compressing the optic chiasm. (C) After dural opening, (D) a fibro-elastic and infiltrating lesion was partially removed in order to obtain optic nerve decompression.
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F2
Intraoperative histological examination showed the presence of atypical cells with a plasmacytoid appearance (Figure 2).
(A) Hematoxylin-eosin slides showed a hypercellular tumor composed of large, epithelioid, often nucleolated cells, with abundant cytoplasm, arranged in a "vertebral-like" fashion (hematoxylin-eosin, original magnification 40x). (B) Tumor cells were positive for pancytokeratin (AE1/AE3; immunoperoxidase staining, original magnification 10x). (C) Neuroendocrine markers were consistently positive, as for synaptophysin shown in the figure (immunoperoxidase staining, original magnification 10x). (D) The cellular proliferation index Ki67 was nearly 70-80% (immunoperoxidase staining, original magnification 10x).
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F3
Histology and immunohistochemical staining ultimately confirmed the diagnosis of a primary neuroendocrine tumor (Figure 3).
PMC10164933_03
Preoperative sagittal (A) axial (B) post-gadolinium, and sagittal (C) T2 MRI scan demonstrated a suprasellar mass with heterogenous low signal in T1 images and an intermediate high signal in T2 images, with cystic and calcific components of the lesion. Axial (D) susceptibility-weighted imaging (SWI) showed signal dropout (hypointensity) in the sellar region. The lesion measured ~3 x 2,4 x 3, and 3 cm on anteroposterior, cephalocaudal, and transverse dimensions. (E) After dura opening, the evacuation of the intralesional hemorrhagic component localized inside the optic chiasm and infundibulum of the third ventricle was performed, which appeared dislocated below; (F) at the end of the procedure, a yellowish granulomatous formation was removed in fragments of dubious vascularization but suspected of a possible, already site of previous bleeding and adhering to the ventricular walls. ON, optic nerve; T, tumor. *Calcification.
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F4
Considering its high vascularity and the difficulty of dissection from the adjacent structures, a subtotal resection of the lesion aiming at optic nerve decompression was achieved (Figure 4).