Suprasellar chordoid neoplasm with expression of thyroid transcription factor 1: evidence that chordoid glioma of the third ventricle and pituicytoma may form part of a spectrum of lineage-related tumors of the basal forebrain

Hewer, Ekkehard; Beck, Jürgen; Kellner-Weldon, Frauke; Vajtai, István

doi:10.1016/j.humpath.2015.03.005

Cited by 21 publications

(14 citation statements)

References 13 publications

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“…). Furthermore, TTF‐1 expression in chordoid glioma has been reported by Michotte et al Moreover, based on the consistent expression of marker protein pS6 in pituicytoma and chordoid glioma, which is activated by TTF‐1 and the mechanistic target of rapamycin (mTOR) pathway, Hewer et al have hypothesized that chordoid glioma and pituicytoma might belong to the same tumor lineage, which originates from the base of the telencephalon . However, we believe that further molecular biology research is needed to evaluate this issue, as these two entities may exhibit differences in their histology and immunophenotype.…”

Section: Discussionmentioning

confidence: 70%

The clinicopathological features of pituicytoma and the differential diagnosis of sellar glioma

Wang

et al. 2016

Neuropathology

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Pituicytoma is rare and difficult to diagnose. This study explored the clinicopathological features, immunophenotype and differential diagnosis of pituicytoma. We compared 11 cases of pituicytoma and 26 cases of sellar glioma (16 pilocytic astrocytomas, four diffuse astrocytomas, three pilomyxoid astrocytomas, and three third ventricle chordoid gliomas). The 11 pituicytoma cases involved six men and five women (age: 33-65 years). Three of the 11 patients experienced recurrence due to a residual tumor, and one patient underwent three surgeries during a 6-month period. Imaging findings revealed tumors were in the intrasellar region (four cases), suprasellar region (four cases) and intra-suprasellar regions (three cases). The tumor diameters were 1.3-3.8 cm, and the preoperative diagnoses were pituitary adenoma, craniopharyngioma and meningioma. The tumors were solid and contained spindle or slightly chubby cells that were densely arranged with visible cleft-like or expanded sinusoid structures. The cells had vague boundaries, circular nuclei, fine chromatin, and a small nucleolus. Immunohistochemical staining of the pituicytomas revealed positive expression of thyroid transcription factor-1 (TTF-1) and S-100 protein (S-100), positive focal expression of glial GFAP;(five of 11 cases), and negative oligodendrocyte transcription factor 2 (Olig2), CD34 and neurofilament expression. The Ki67 index was 6% in one case and 1-2% in the other cases. Unlike pituicytoma, most sellar glioma cases exhibited GFAP and Olig2 expression, and negative TTF-1 expression. Third ventricle chordoid gliomas expressed TTF-1, GFAP and CD34, and were negative for Olig2. Our results indicate that pituicytoma typically involves dense arrangements of spindle or slightly chubby cells. The morphology is occasionally atypical, with ependymoma-like or meningioma-like structures, and occasionally exhibits pilomyxoid degeneration. Abundant sinusoids are characteristic of hemorrhagic tumors. The dense spindle cell arrangement is a relatively specific morphology, and staining for GFAP, TTF-1, Olig2 and CD4 may help differentiate pituicytoma from sellar glioma.

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Section: Discussionmentioning

confidence: 70%

The clinicopathological features of pituicytoma and the differential diagnosis of sellar glioma

Wang

et al. 2016

Neuropathology

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“…Evidence supporting the concept that these three morphologic variants represent the same tumor category is derived from common ultrastructural features and immunolabeling with TTF1, a transcription factor involved in the morphogenesis and differentiation of thyroid, lung and ventral forebrain(10). Based on shared TTF1-expression, it also has been postulated that chordoid glioma of the third ventricle and pituicytoma may form part of a spectrum of lineage-related tumors of the basal forebrain(11).Given the rarity of these tumors, it is not surprising that the molecular characterization remains a work in progress. Recently DNA methylation profiles have shown great potential for the classification of various types of brain tumors.…”

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confidence: 97%

Synchronous pituitary adenoma and pituicytoma

et al. 2016

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“…Interestingly, two other brain tumors harbor a specific signature affecting the PRKCA gene: the SLC44A1-PRKCA gene fusion in papillary glioneuronal tumors and the PRKCA D294G in pituicytomas, another tumor from the diencephalic region of the brain 22 . ChG and pituicytomas share several similarities such as TTF1 expression and mTor pathway activation 23 . However, we found no overlap between the two entities since (i) PRKCA D463H was absent in the pituicytomas we analyzed (Supplementary Data 3 ) and PRKCA D294G was absent in ChG (Table 1 ), and (ii) PRKCA D294G does not affect the active site but rather impairs the membrane attachment at the intercellular junctions 22 , 24 .…”

Section: Discussionmentioning

confidence: 99%

A recurrent point mutation in PRKCA is a hallmark of chordoid gliomas

et al. 2018

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Chordoid glioma (ChG) is a characteristic, slow growing, and well-circumscribed diencephalic tumor, whose mutational landscape is unknown. Here we report the analysis of 16 ChG by whole-exome and RNA-sequencing. We found that 15 ChG harbor the same PRKCAD463H mutation. PRKCA encodes the Protein kinase C (PKC) isozyme alpha (PKCα) and is mutated in a wide range of human cancers. However the hot spot PRKCAD463H mutation was not described in other tumors. PRKCAD463H is strongly associated with the activation of protein translation initiation (EIF2) pathway. PKCαD463H mRNA levels are more abundant than wild-type PKCα transcripts, while PKCαD463H is less stable than the PCKαWT protein. Compared to PCKαWT, the PKCαD463H protein is depleted from the cell membrane. The PKCαD463H mutant enhances proliferation of astrocytes and tanycytes, the cells of origin of ChG. In conclusion, our study identifies the hallmark mutation for chordoid gliomas and provides mechanistic insights on ChG oncogenesis.

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Suprasellar chordoid neoplasm with expression of thyroid transcription factor 1: evidence that chordoid glioma of the third ventricle and pituicytoma may form part of a spectrum of lineage-related tumors of the basal forebrain

Cited by 21 publications

References 13 publications

The clinicopathological features of pituicytoma and the differential diagnosis of sellar glioma

The clinicopathological features of pituicytoma and the differential diagnosis of sellar glioma

Synchronous pituitary adenoma and pituicytoma

A recurrent point mutation in PRKCA is a hallmark of chordoid gliomas

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