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Papillary ependymoma

MedGen UID:
90810
•Concept ID:
C0334578
•
Neoplastic Process
Synonyms: Ependymoma, Papillary; Ependymomas, Papillary; Papillary Ependymoma; Papillary Ependymomas
SNOMED CT: Papillary ependymoma (128839002)
 
Monarch Initiative: MONDO:0003475

Definition

A rare variant of ependymoma characterized by well formed papillae. Tumor cell processes abutting capillaries are usually GFAP-positive. Differential diagnoses include choroid plexus papilloma, papillary meningioma and metastatic papillary carcinoma. (Adapted from WHO) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPapillary ependymoma

Professional guidelines

PubMed

Clinicopathologic study of endolymphatic sac tumor (ELST) and differential diagnosis of papillary tumors located at the cerebellopontine angle.
Du J, Wang J, Cui Y, Zhang C, Li G, Fang J, Yue S, Xu L
Neuropathology 2015 Oct;35(5):410-20. Epub 2015 May 6 doi: 10.1111/neup.12200. PMID: 25944396

Recent clinical studies

Etiology

Molecular characterization of histopathological ependymoma variants.
Neumann JE, Spohn M, Obrecht D, Mynarek M, Thomas C, Hasselblatt M, Dorostkar MM, Wefers AK, Frank S, Monoranu CM, Koch A, Witt H, Kool M, Pajtler KW, Rutkowski S, Glatzel M, Schüller U
Acta Neuropathol 2020 Feb;139(2):305-318. Epub 2019 Nov 2 doi: 10.1007/s00401-019-02090-0. PMID: 31679042
A mixed choroid plexus papilloma and ependymoma.
Lee Y, Kim SI, Kim SK, Kim IO, Park SH
Brain Tumor Pathol 2016 Apr;33(2):147-50. Epub 2015 Dec 15 doi: 10.1007/s10014-015-0242-4. PMID: 26670168
Papillary ependymoma with unique superficial cortical location: immunohistochemical and ultrastructural studies. A case report.
Grajkowska W, Matyja E, Pronicki M, Daszkiewicz P, Roszkowski M, Perek D, Drogosiewicz M
Folia Neuropathol 2009;47(4):354-61. PMID: 20054788
Clinicopathological features from long-term observation of a papillary tumor of the pineal region (PTPR): a case report.
Yano H, Ohe N, Nakayama N, Shinoda J, Iwama T
Brain Tumor Pathol 2009;26(2):83-8. Epub 2009 Oct 27 doi: 10.1007/s10014-009-0246-z. PMID: 19856220
Histological spectrum of ependymomas and correlation of p53 and Ki-67 expression with ependymoma grade and subtype.
Suri VS, Tatke M, Singh D, Sharma A
Indian J Cancer 2004 Apr-Jun;41(2):66-71. PMID: 15318011

Diagnosis

Clinical Image of a Spinal Ependymoma Discovered 8 Years after Initial Misdiagnosis as an Idiopathic Syringomyelia.
Ng S, Aghakhani N, Bauchet L
World Neurosurg 2021 Jan;145:338-339. Epub 2020 Oct 3 doi: 10.1016/j.wneu.2020.09.162. PMID: 33022432
Cytodiagnosis of Sacral Chordoma.
Saumya S, Awasth NP, Husain N, Anand N
JNMA J Nepal Med Assoc 2014 Oct-Dec;52(196):1014-6. PMID: 26982902
Clinicopathological features from long-term observation of a papillary tumor of the pineal region (PTPR): a case report.
Yano H, Ohe N, Nakayama N, Shinoda J, Iwama T
Brain Tumor Pathol 2009;26(2):83-8. Epub 2009 Oct 27 doi: 10.1007/s10014-009-0246-z. PMID: 19856220
Metastasis of a histologically benign choroid plexus papilloma: case report and review of the literature.
Jinhu Y, Jianping D, Jun M, Hui S, Yepeng F
J Neurooncol 2007 May;83(1):47-52. Epub 2007 Mar 27 doi: 10.1007/s11060-006-9300-4. PMID: 17387433
Cytologic features of ependymomas in smear preparations.
Ng HK
Acta Cytol 1994 May-Jun;38(3):331-4. PMID: 8191821

Therapy

Metastasis of a histologically benign choroid plexus papilloma: case report and review of the literature.
Jinhu Y, Jianping D, Jun M, Hui S, Yepeng F
J Neurooncol 2007 May;83(1):47-52. Epub 2007 Mar 27 doi: 10.1007/s11060-006-9300-4. PMID: 17387433

Prognosis

Posterior fossa ependymoma with preoperative cerebrospinal metastases: a case report with literature review.
Tang H, Wu T, Zhao F, Zhang J, Yang Z, Li P, Wang X, Wang B, Wang Z, Liu P
Br J Neurosurg 2023 Oct;37(5):1277-1280. Epub 2020 Dec 10 doi: 10.1080/02688697.2020.1853044. PMID: 33300386
Molecular characterization of histopathological ependymoma variants.
Neumann JE, Spohn M, Obrecht D, Mynarek M, Thomas C, Hasselblatt M, Dorostkar MM, Wefers AK, Frank S, Monoranu CM, Koch A, Witt H, Kool M, Pajtler KW, Rutkowski S, Glatzel M, Schüller U
Acta Neuropathol 2020 Feb;139(2):305-318. Epub 2019 Nov 2 doi: 10.1007/s00401-019-02090-0. PMID: 31679042
A mixed choroid plexus papilloma and ependymoma.
Lee Y, Kim SI, Kim SK, Kim IO, Park SH
Brain Tumor Pathol 2016 Apr;33(2):147-50. Epub 2015 Dec 15 doi: 10.1007/s10014-015-0242-4. PMID: 26670168
Clinicopathological features from long-term observation of a papillary tumor of the pineal region (PTPR): a case report.
Yano H, Ohe N, Nakayama N, Shinoda J, Iwama T
Brain Tumor Pathol 2009;26(2):83-8. Epub 2009 Oct 27 doi: 10.1007/s10014-009-0246-z. PMID: 19856220
Histological spectrum of ependymomas and correlation of p53 and Ki-67 expression with ependymoma grade and subtype.
Suri VS, Tatke M, Singh D, Sharma A
Indian J Cancer 2004 Apr-Jun;41(2):66-71. PMID: 15318011

Clinical prediction guides

Posterior fossa ependymoma with preoperative cerebrospinal metastases: a case report with literature review.
Tang H, Wu T, Zhao F, Zhang J, Yang Z, Li P, Wang X, Wang B, Wang Z, Liu P
Br J Neurosurg 2023 Oct;37(5):1277-1280. Epub 2020 Dec 10 doi: 10.1080/02688697.2020.1853044. PMID: 33300386
A mixed choroid plexus papilloma and ependymoma.
Lee Y, Kim SI, Kim SK, Kim IO, Park SH
Brain Tumor Pathol 2016 Apr;33(2):147-50. Epub 2015 Dec 15 doi: 10.1007/s10014-015-0242-4. PMID: 26670168
Histopathological patterns of papillary tumour of the pineal region.
Matyja E, Grajkowska W, Nauman P, Bonicki W
Folia Neuropathol 2011;49(3):181-90. PMID: 22101951
Clinicopathological features from long-term observation of a papillary tumor of the pineal region (PTPR): a case report.
Yano H, Ohe N, Nakayama N, Shinoda J, Iwama T
Brain Tumor Pathol 2009;26(2):83-8. Epub 2009 Oct 27 doi: 10.1007/s10014-009-0246-z. PMID: 19856220
Choroid plexus papilloma. Report of a case with cytologic differential diagnosis.
Buchino JJ, Mason KG
Acta Cytol 1992 Jan-Feb;36(1):95-7. PMID: 1312286

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