CD99 is expressed in chordoid glioma and suggests ependymal origin

Romero‐Rojas, Alfredo; Díaz-Pérez, Julio A.; Ariza-Serrano, Lina M.

doi:10.1007/s00428-011-1170-2

Cited by 12 publications

(7 citation statements)

References 15 publications

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“…It is recognized to occur in “primary neuroepithelial tumors of the kidney,” an entity we believe to be closely related to ATRT (24). It is also present in ependymoma, peripheral primitive neuroectodermal tumor, and in the rare chordoid glioma, all of which are of neuroepithelial origin (25–27). …”

Section: Resultsmentioning

confidence: 99%

Atypical Teratoid Rhabdoid Tumor: Two Case Reports and an Analysis of Adult Cases with Implications for Pathophysiology and Treatment

et al. 2017

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We present the first quantitative analysis of atypical teratoid rhabdoid tumors (ATRT) in adults, including two patients from our own institutions. These are of interest as one occurred during pregnancy and one is a long-term survivor. Our review of pathological findings of 50 reported cases of adult ATRT leads us to propose a solely ectodermal origin for the tumor and that epithelial–mesenchymal transition (EMT) is a defining feature. Thus, the term ATRT may be misleading. Our review of clinical findings shows that ATRT tends to originate in mid-line structures adjacent to the CSF, leading to a high rate of leptomeningeal dissemination. Thus, we hypothesize that residual undifferentiated ectoderm in the circumventricular organs, particularly the pituitary and pineal glands, is the most common origin for these tumors. We note that if growth is not arrested soon after diagnosis, or after the first relapse/progression, death is almost universal. While typically rapidly fatal (as in our first case), long-term remission is possible (as in our second). Significant predictors of prognosis were the extent of resection and the use of chemotherapy. Glial differentiation (GFAP staining) was strongly associated with leptomeningeal metastases (chi-squared p = 0.02) and both predicted markedly worse outcomes. Clinical trials including adults are rare. ATRT is primarily a disease of infancy and radiotherapy is generally avoided in those aged less than 3 years old. Treatment options in adults differ from infants in that cranio-spinal irradiation is a viable adjunct to systemic chemotherapy in the adult population. Given the grave prognosis, this combined approach appears reasonable. As effective chemotherapy is likely to cause myelosuppression, we recommend that stem-cell rescue be available locally.

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Section: Resultsmentioning

confidence: 99%

Atypical Teratoid Rhabdoid Tumor: Two Case Reports and an Analysis of Adult Cases with Implications for Pathophysiology and Treatment

et al. 2017

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“… Author (year) # of patients Average Age Extent of Resection (initial tumor) Reported # that underwent radiotherapy† Reported patient recurrences Reported deaths Al-Zubidi et al (2014)[41]137STR000Kobayashi et al (2013)[47]349Bx (n=2)STR (n=1)301Ni et al (2013)[18]440GTR (n=4)000Shiramizu et al (2013)[54]149GTR000Scheurkogel et al (2012)[55]130STR000Romero-Rojas et al (2012)[14]139GTR000Can et al (2012)[38]137

…”

Section: Resultsmentioning

confidence: 99%

“…For those patients who did have progression, specifically within the STR group, time to progression ranged from 4 months to 4 years. The limited follow up for select patients undergoing GTR[7, 13, 14, 21, 38, 39, 41, 50, 53] may have precluded detection of later recurrences.…”

Section: Discussionmentioning

confidence: 99%

Prognostic factors for recurrence and complications in the surgical management of primary chordoid gliomas: A systematic review of literature

Ampie

Choy

Lamano

et al. 2015

Clinical Neurology and Neurosurgery

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Introduction Chordoid Gliomas (CG) are rare neoplasms which frequently arise within the third ventricle. Surgery remains the mainstay treatment for CG. The present study comprehensively reviews all reported cases of CG within the literature in order to identify risk factors for surgical complications and tumor recurrence. Methods A comprehensive search on MEDLINE (OVID and Pubmed), Scopus, Embase, and Web of Science was conducted following PRISMA guidelines to identify all reported cases of CG. Results A total of 81 patients met the study criteria, comprised of 33 males and 48 females. Median age at diagnosis was 48 years with a range from 5 to 72 years, and mean tumor size was 3.1 cm. Biopsy, subtotal resection (STR), and gross total resection (GTR) was achieved in 8, 34, and 33 patients, respectively, with 6 cases not reporting extent of eresection (EOR). Thirteen patients underwent adjuvant radiotherapy. Postoperative complications were noted in 30 cases (37%), with new onset diabetes insipidus being the most common. Postoperative morbidity was not associated with age, tumor size, or extent of resection. A trans-lamina terminalis approach demonstrated a strong trend towards decreased overall rates of postoperative morbidity compared to other approaches (p=0.051). GTR was associated with improved progression-free survival (PFS; p=0.028), while adjuvant radiotherapy, age, tumor size and MIB-I were not predictive of patient outcomes. Conclusion GTR should be the primary goal for the management of CG, as it is associated with improved rates of tumor control without an increased rate of postoperative complications. Surgical approach was a stronger predictor of complication rates than extent of resection. Morbidity remains high, and future studies to further elaborate on factors predictive of postoperative complications are critical.

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“…Immunohistochemically, strong diffuse reactivity for GFAP distinguish chordoid glioma from its mimics. The other antibodies that reported to be positive in chordoid glioma are vimentin, S-100 protein, EMA, D2-40, CD34 and CD99 (6,7,9). Rare positivity to neurofilament protein, synaptophysin and p53 are also noted (6).…”

Section: Discussionmentioning

confidence: 93%

“…The ependymal nature of chordoid glioma has been suggested by findings on electron microscopy and positivity to EMA, D2-40, and CD99 (7,9). Ultrastructurally, the tumor exhibits features of ependymal differentiation which include intermediate filaments, intercellular lumina apical microvilli, hemidesmosomes, and basal lamina (2).…”

Section: Discussionmentioning

confidence: 97%

A 29‐Year‐Old Man with Progressive Short Term Memory Loss

2013

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A 29-year-old man presented with a 1 year history of memory impairment. He had become somnolent and lethargic for 8 months before seeking medical attention. He needed to sleep over 20 hours per day. The physical examination and neurological examination were unremarkable. RADIOLOGYBrain MRI revealed a 5.7x4.3x4.2 cm in the third ventricle with isosignal intensity on T1W, heterogeneous signal on T2W and FLAIR (not shown). The mass showed intense heterogeneous enhancement on T1W after gadolinium injection. Extension to the suprasellar cistern was demonstrated on sagittal T1W after gadolinium injection ( Figure 1a). There was also thickening and enhancement of the pituitary stalk, suggestive of involvement or origin of hypothalamus. The coronal T1W after gadolinium injection showed separation of the mass from lateral ventricle ( Figure 1b). The inferior wall of the lateral ventricle was displaced upward as well as the septum pellucidum. SURGICAL TREATMENT AND POST OPERATIVE OUTCOMEThe patient underwent biopsy and later bifrontal craniotomy for tumor removal. Intraoperatively, the tumor appeared white and had rubbery to hard consistency. It was located in the third ventricle adhering to lamina terminalis and both thalami. There was low tumor vascularization. The tumor was totally resected. Postoperatively, the patient developed transient diabetes insipidus. There was no evidence of tumor recurrence on follow up MRI after 9 months of surgery. MICROSCOPIC PATHOLOGYIntraoperative squash preparation showed hypercellular smear with epithelioid tumor nests, non-cohesive tumor cells, and 3D papillary-like fragments (Figure 2a). The epithelioid tumor cells contained moderate amount of eosinophilic cytoplasm. They were relatively uniform and possessed round to oval, central or eccentric nuclei, with open chromatin and small nucleoli (Figure 2b). The non-cohesive tumor cells had delicate cytoplasmic process and show similar nuclear features (Figure 2c). Permanent section revealed a well demarcated mass composed of clusters and cords of epithelioid cells in a myxoid stroma with scattered lymphoplasmacytic infiltration (Figure 2d, Figure 2e). Rosenthal fibers are observed in adjacent brain parenchyma. Mitotic figure, necrosis, and vascular proliferation were not present. Higher magnification showed uniform to mildly variate (Figure 2f) epithelioid tumor cells with moderate to ample amount of eosinophilic cytoplasm in a myxoid stroma. Findings on nuclear detail were similar to those present in squash cytology. Immunohistochemically, the tumor cells were diffusely positive to GFAP (Figure 3a), and vimentin (not shown). Focal positivity to CD34 was noted (Figure 3b). Staining for S-100 protein and EMA were negative (not shown). Ultrastructural study on specimen retrieved from paraffinembedded tissue revealed microvilli (Figure 3c) at the luminal surface, and intermediate junctions (not shown). The cytoplasm contained mitochondria, ribosome, lysosome, and dilated rER. Intermediate filaments (Figure 3d) are noted in the cytoplasm of m...

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CD99 is expressed in chordoid glioma and suggests ependymal origin

Cited by 12 publications

References 15 publications

Atypical Teratoid Rhabdoid Tumor: Two Case Reports and an Analysis of Adult Cases with Implications for Pathophysiology and Treatment

Atypical Teratoid Rhabdoid Tumor: Two Case Reports and an Analysis of Adult Cases with Implications for Pathophysiology and Treatment

Prognostic factors for recurrence and complications in the surgical management of primary chordoid gliomas: A systematic review of literature

A 29‐Year‐Old Man with Progressive Short Term Memory Loss

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