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1. Bodner-Adler B, Bodner K, Zeisler H: Primitive neuroectodermal tumor (PNET) of the brain diagnosed during pregnancy. Anticancer Res; 2006 May-Jun;26(3B):2499-501
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  • [Title] Primitive neuroectodermal tumor (PNET) of the brain diagnosed during pregnancy.
  • BACKGROUND: Brain tumors in pregnancy are extremely rare events.
  • A case of a patient with primitive neuroectodermal tumor (PNET) of the brain diagnosed during the second half of pregnancy is reported.
  • CASE REPORT: The first case of PNET of the brain diagnosed in a 26-year-old woman, gravida 1 para 0, in her 20th week of pregnancy is presented.
  • Pathological evaluation revealed histology and immuno-histochemistry consistent with the diagnosis of PNET.
  • Adjuvant radiation therapy to the neurocranium was administered.
  • Additionally, chemotherapy with Temodal 5 mg (Temozolomid) was initiated at 30 weeks of gestation.
  • After delivery, external beam radiation to the craniospinal axis completed the therapy.
  • The patient is currently being followed-up at the Department of Oncology University of Vienna Medical School, without any evidence of disease, 3 months after diagnosis.
  • CONCLUSION: This case is the first reported description of a PNET in pregnancy.
  • The treatment options seem to be reduced in pregnant women and mainly depend on the patient's condition as well as the gestational age at presentation.
  • In a multidisciplinary approach, an optimal therapy schedule should be assessed depending on these two parameters.
  • [MeSH-major] Brain Neoplasms / pathology. Brain Neoplasms / therapy. Neuroectodermal Tumors, Primitive / pathology. Neuroectodermal Tumors, Primitive / therapy. Pregnancy Complications, Neoplastic / pathology. Pregnancy Complications, Neoplastic / therapy

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  • (PMID = 16821639.001).
  • [ISSN] 0250-7005
  • [Journal-full-title] Anticancer research
  • [ISO-abbreviation] Anticancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Greece
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2. Yip CM, Hsu SS, Chang NJ, Wang JS, Liao WC, Chen JY, Liu SH, Chen CH: Primary vaginal extraosseous Ewing sarcoma/primitive neuroectodermal tumor with cranial metastasis. J Chin Med Assoc; 2009 Jun;72(6):332-5
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  • [Title] Primary vaginal extraosseous Ewing sarcoma/primitive neuroectodermal tumor with cranial metastasis.
  • Extraosseous Ewing sarcoma is now regarded as a member of the Ewing sarcoma/primitive neuroectodermal tumor (PNET) family.
  • It typically involves the soft tissues of the chest wall, pelvis, paravertebral region, abdominal wall, retroperitoneal region and extremities of children, adolescents and young adults, but it seldom occurs in the female genital tract.
  • We report an extremely rare case of retrospective diagnosis of vaginal extraosseous Ewing sarcoma/PNET which metastasized to the right frontoparietal scalp, skull, and dura.
  • Surgical resection, followed by adjuvant radiotherapy and chemotherapy resulted in a favourable clinical outcome.
  • Both the vaginal and head tumors had similar light microscopic features supporting the diagnosis.
  • [MeSH-major] Brain Neoplasms / secondary. Neuroectodermal Tumors, Primitive / pathology. Sarcoma, Ewing / pathology. Vaginal Neoplasms / pathology

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  • (PMID = 19541570.001).
  • [ISSN] 1726-4901
  • [Journal-full-title] Journal of the Chinese Medical Association : JCMA
  • [ISO-abbreviation] J Chin Med Assoc
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China (Republic : 1949- )
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules
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3. Adibi A, Barikbin R, Koleini N, Farghadani M, Mougouei K, Farshidfar F: Solitary epidural brain metastasis of a peripheral neuroepithelioma (a primitive neuroectodermal tumor): a case report. J Radiol Case Rep; 2008;2(1):16-9

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  • [Title] Solitary epidural brain metastasis of a peripheral neuroepithelioma (a primitive neuroectodermal tumor): a case report.
  • The patient was a known case of cervical soft tissue primitive neuroectodermal tumor (PNET) who has undergone surgery and radiotherapy 4 years ago.
  • After diagnosis the patient has undergone chemotherapy and radiotherapy.
  • He remained symptom free, and also follow up CT scans of the brain, chest, and abdomen were normal after two years post surgery.
  • This is the first reported case of epidural metastasis of a head and neck (peripheral) PNET.

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  • (PMID = 22470583.001).
  • [ISSN] 1943-0922
  • [Journal-full-title] Journal of radiology case reports
  • [ISO-abbreviation] J Radiol Case Rep
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC3303233
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4. Chung YN, Wang KC, Shin SH, Kim N, Chi JG, Min KS, Cho BK: Primary intracranial atypical teratoid/rhabdoid tumor in a child: a case report. J Korean Med Sci; 2002 Oct;17(5):723-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Primary intracranial atypical teratoid/rhabdoid tumor in a child: a case report.
  • These tumors show similar histological and radiological features to primitive neuroectodermal tumormedulloblastoma (PNET-MB) but have different biological behaviors.
  • Preoperative radiological diagnosis was PNET-MB, but pathological diagnosis is ATT/ RhT.
  • He was treated by surgical resection, chemotherapy, and radiotherapy.
  • Despite aggressive therapy, he died 19 months after diagnosis.
  • Clinical, radiological, and histopathological features of primary intracranial ATT/RhT are discussed with a special emphasis on the differential diagnosis from PNET-MB.
  • [MeSH-major] Brain Neoplasms / diagnosis. Rhabdoid Tumor / diagnosis. Teratoma / diagnosis
  • [MeSH-minor] Diagnosis, Differential. Humans. Infant. Magnetic Resonance Imaging. Male. Medulloblastoma / diagnosis. Neuroectodermal Tumors, Primitive / diagnosis. Tomography, X-Ray Computed

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  • (PMID = 12378033.001).
  • [ISSN] 1011-8934
  • [Journal-full-title] Journal of Korean medical science
  • [ISO-abbreviation] J. Korean Med. Sci.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC3054946
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5. Kim DG, Lee DY, Paek SH, Chi JG, Choe G, Jung HW: Supratentorial primitive neuroectodermal tumors in adults. J Neurooncol; 2002 Oct;60(1):43-52
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  • [Title] Supratentorial primitive neuroectodermal tumors in adults.
  • A retrospective clinical analysis was made of 12 patients with supratentorial primitive neuroectodermal tumor (PNET) who ranged in age from 20 to 62 years (median 24) and were managed at Seoul National University Hospital between January 1987 and December 1997.
  • Calcification was seen in five out of six patients who underwent computed tomography scan.
  • Ten patients received postoperative whole neuraxis radiation therapy and five patients received additional chemotherapy.
  • Mean survival after diagnosis was 86 months.
  • In conclusion, supratentorial PNET must be included, even in adults, in the differential diagnoses if a tumor has characteristic radiological features.
  • The adult supratentorial PNET seemed similar to that of children in the clinical features and the prognosis.
  • [MeSH-major] Brain Neoplasms / diagnosis. Neoplasm Recurrence, Local / diagnosis. Neuroectodermal Tumors, Primitive / diagnosis
  • [MeSH-minor] Adult. Calcinosis. Combined Modality Therapy. Craniotomy. Diagnosis, Differential. Female. Glioblastoma / diagnosis. Humans. Ki-67 Antigen / metabolism. Magnetic Resonance Imaging. Male. Middle Aged. Oligodendroglioma / diagnosis. Prognosis. Retrospective Studies

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  • (PMID = 12416545.001).
  • [ISSN] 0167-594X
  • [Journal-full-title] Journal of neuro-oncology
  • [ISO-abbreviation] J. Neurooncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Ki-67 Antigen
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6. Chintagumpala M, Hassall T, Palmer S, Ashley D, Wallace D, Kasow K, Merchant TE, Krasin MJ, Dauser R, Boop F, Krance R, Woo S, Cheuk R, Lau C, Gilbertson R, Gajjar A: A pilot study of risk-adapted radiotherapy and chemotherapy in patients with supratentorial PNET. Neuro Oncol; 2009 Feb;11(1):33-40
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A pilot study of risk-adapted radiotherapy and chemotherapy in patients with supratentorial PNET.
  • We undertook this study to estimate the event-free survival (EFS) of patients with newly diagnosed supratentorial primitive neuroectodermal tumor (SPNET) treated with risk-adapted craniospinal irradiation (CSI) with additional radiation to the primary tumor site and subsequent high-dose chemotherapy supported by stem cell rescue.
  • High-risk (HR) disease was differentiated from average-risk (AR) disease by the presence of residual tumor (M(0) and tumor size > 1.5 cm(2)) or disseminated disease in the neuraxis (M(1)-M(3)).
  • Patients received risk-adapted CSI: those with AR disease received 23.4 Gy; those with HR disease, 36-39.6 Gy.
  • The tumor bed received a total of 55.8 Gy.
  • The median age at diagnosis was 7.9 years; eight patients were female.
  • Seven patients had pineal PNET.
  • High-dose cyclophosphamide-based chemotherapy with stem cell support after risk-adapted CSI results in excellent EFS estimates for patients with newly diagnosed AR SPNET.
  • Further, this chemotherapy allows for a reduction in the dose of CSI used to treat AR SPNET without compromising EFS.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / therapy. Cranial Irradiation. Hematopoietic Stem Cell Transplantation. Neuroectodermal Tumors, Primitive / therapy. Supratentorial Neoplasms / therapy
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Cisplatin / administration & dosage. Combined Modality Therapy. Cyclophosphamide / administration & dosage. Disease-Free Survival. Dose-Response Relationship, Radiation. Female. Gamma Rays. Humans. Male. Pilot Projects. Risk Factors. Survival Rate. Treatment Outcome. Vincristine / administration & dosage


7. Jingyu C, Jinning S, Hui M, Hua F: Intraspinal primitive neuroectodermal tumors: Report of four cases and review of the literature. Neurol India; 2009 Sep-Oct;57(5):661-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Intraspinal primitive neuroectodermal tumors: Report of four cases and review of the literature.
  • Intraspinal primitive neuroectodermal tumors (PNETs) are extremely rare and are aggressive with poor outcomes.
  • Till date only 41 cases of intraspinal PNET have been reported.
  • Total excision of the tumor was done in three patients and partial excision in one patient.
  • Three patients were treated with craniospinal radiotherapy and chemotherapy.
  • [MeSH-major] Neuroectodermal Tumors, Primitive / diagnosis. Spinal Neoplasms / diagnosis
  • [MeSH-minor] Brain / pathology. Female. Humans. Magnetic Resonance Imaging / methods. Male. Middle Aged. Phosphopyruvate Hydratase / metabolism. Spinal Cord / metabolism. Spinal Cord / pathology. Young Adult

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  • (PMID = 19934573.001).
  • [ISSN] 0028-3886
  • [Journal-full-title] Neurology India
  • [ISO-abbreviation] Neurol India
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] India
  • [Chemical-registry-number] EC 4.2.1.11 / Phosphopyruvate Hydratase
  • [Number-of-references] 37
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8. Allen J, Donahue B, Mehta M, Miller DC, Rorke LB, Jakacki R, Robertson P, Sposto R, Holmes E, Vezina G, Muraszko K, Puccetti D, Prados M, Chan KW: A phase II study of preradiotherapy chemotherapy followed by hyperfractionated radiotherapy for newly diagnosed high-risk medulloblastoma/primitive neuroectodermal tumor: a report from the Children's Oncology Group (CCG 9931). Int J Radiat Oncol Biol Phys; 2009 Jul 15;74(4):1006-11
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] A phase II study of preradiotherapy chemotherapy followed by hyperfractionated radiotherapy for newly diagnosed high-risk medulloblastoma/primitive neuroectodermal tumor: a report from the Children's Oncology Group (CCG 9931).
  • PURPOSE: To verify feasibility and monitor progression-free survival and overall survival in children with high-risk medulloblastoma and noncerebellar primitive neuroectodermal tumors (PNETs) treated in a Phase II study with preradiotherapy chemotherapy (CHT) followed by high-dose, hyperfractionated craniospinal radiotherapy (CSRT).
  • METHODS AND MATERIALS: Eligibility criteria included age >3 years at diagnosis, medulloblastoma with either high M stage and/or >1.5 cm(2) postoperative residual disease, and all patients with noncerebellar PNET.
  • Treatment was initiated with five alternating monthly cycles of CHT (A [cisplatin, cyclophosphamide, etoposide, and vincristine], B [carboplatin and etoposide], A, B, and A) followed by hyperfractionated CSRT (40 Gy) with a boost to the primary tumor (72 Gy) given in twice-daily 1-Gy fractions.
  • RESULTS: The valid study group consisted of 124 patients whose median age at diagnosis was 7.8 years.
  • Eighty-four patients (68%) completed the entire protocol according to study guidelines (within 9 months), and the median time to complete CSRT was 1.6 months.
  • No significant differences were detected in subset analysis related to response to CHT, site of primary tumor, postoperative residual disease, or M stage.

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  • (PMID = 19356859.001).
  • [ISSN] 1879-355X
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA003888-44; United States / NCI NIH HHS / CA / U10 CA003888; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / U10 CA003888-44
  • [Publication-type] Clinical Trial, Phase II; Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 143011-72-7 / Granulocyte Colony-Stimulating Factor; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; BG3F62OND5 / Carboplatin; Q20Q21Q62J / Cisplatin
  • [Other-IDs] NLM/ NIHMS128135; NLM/ PMC2739055
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9. Horinaka N, Ito Y, Miyajima M, Hishii M, Suzuki K, Saito M, Arai H: Intracranial peripheral primitive neuroectodermal tumor manifesting as abducens nerve palsy. Childs Nerv Syst; 2005 Apr;21(4):327-30
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  • [Title] Intracranial peripheral primitive neuroectodermal tumor manifesting as abducens nerve palsy.
  • INTRODUCTION: Peripheral primitive neuroectodermal tumor (PNET) occurring as an extra-axial lesion within the intracranial space and extending to the subarachnoid space is extremely rare.
  • CASE REPORT: An 18-month-old girl presented with an intracranial peripheral PNET manifesting as abducens nerve palsy.
  • The tumor was partially removed via the subtemporal approach.
  • Histological examination showed a high-grade, undifferentiated neoplasm of small cell type with positive immunostaining for MIC2.
  • The histological diagnosis was peripheral PNET.
  • OUTCOME: Craniospinal radiotherapy reduced the tumor size, but adjuvant chemotherapy designed for Ewing's sarcomas and PNETs was not effective.
  • She died 1 month after the last chemotherapy, despite whole craniospinal irradiation (total dose 53.2 Gy) and chemotherapy.
  • [MeSH-major] Abducens Nerve Diseases / etiology. Brain Neoplasms / complications. Neuroectodermal Tumors, Primitive / complications

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  • (PMID = 15657787.001).
  • [ISSN] 0256-7040
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Protozoan Proteins; 120300-02-9 / thrombospondin-related adhesive protein, protozoan
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10. Paulino AC, Cha DT, Barker JL Jr, Lo S, Manera RB: Patterns of failure in relation to radiotherapy fields in supratentorial primitive neuroectodermal tumor. Int J Radiat Oncol Biol Phys; 2004 Mar 15;58(4):1171-6
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  • [Title] Patterns of failure in relation to radiotherapy fields in supratentorial primitive neuroectodermal tumor.
  • PURPOSE: Supratentorial primitive neuroectodermal tumor (PNET) accounts for 2-3% of all pediatric brain tumors.
  • We retrospectively reviewed all supratentorial PNET cases treated with radiotherapy (RT) at our institutions.
  • Five patients (20%) had neuraxis dissemination (M+ disease) at initial diagnosis.
  • The RT treatment volumes were craniospinal (CS) in 17 (68%), whole brain (WB) followed by a boost in 2 (8%), and primary site (PS) alone in 6 (24%).
  • The median dose to the primary site was 54 Gy (range, 31-55.8 Gy).
  • The median dose to patients receiving WB and spinal fields was 36 Gy (range, 23.4-39.6 Gy).
  • Sixteen patients (64%) received chemotherapy; the most common type was the "8 in 1" chemotherapy regimen in 9 children.
  • RESULTS: The 5-year and 10-year progression-free survival rate was 36% and 27%, respectively, and the median time to progression was 22 months.
  • The 5-year and 10-year progression-free survival rate for those with M0 disease was 40.0% and 30.0%, respectively; for those with M+ disease, the corresponding figures were 20.0% and 0%.
  • Four (80%) of 5 M+ children and 4 (33%) of 12 M0 children who underwent CSRT developed recurrence in the neuraxis (p = 0.1, Fisher's exact test).
  • CONCLUSION: The craniospinal axis is the standard volume that needs to be treated in supratentorial PNET.
  • [MeSH-major] Brain Neoplasms / radiotherapy. Neuroectodermal Tumors / radiotherapy
  • [MeSH-minor] Adolescent. Adult. Analysis of Variance. Child. Child, Preschool. Female. Humans. Infant. Male. Radiotherapy Dosage. Retrospective Studies. Supratentorial Neoplasms / radiotherapy. Survival Rate. Treatment Failure


11. Asano K, Kikuchi J, Munakata A, Ohkuma H, Kubo O: An infant case of intracranial peripheral-type primitive neuroectodermal tumor with long-term survival. Brain Tumor Pathol; 2007;24(2):69-74
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  • [Title] An infant case of intracranial peripheral-type primitive neuroectodermal tumor with long-term survival.
  • Supratentorial primitive neuroectodermal tumors (S-PNET) that develop in children have recently been classified into two types: central-type PNET (C-PNET), which has been reported over the years, and peripheral-type PNET (P-PNET), which develops intracranially and was referred to as Ewing's sarcoma in the past.
  • P-PNET is fundamentally a malignant tumor, but the patient reported here represents a case of long-term survival from onset without recurrence.
  • At the age of 21 months, a male infant developed a cranial bone deformity and symptoms of high intracranial pressure.
  • A CT scan revealed a cystic tumor attaching to the falx, and cyst drainage operation was immediately performed.
  • The intracranial tumor was then resected.
  • The tumor was an intradural extramedullary tumor, and it was totally excised with the falx attachment.
  • The tumor was initially diagnosed as a neuroblastoma, and postoperative treatment consisted of administration of radiotherapy and chemotherapy using cyclophosphamide and vincristine.
  • Recent repeated performance of histopathological analysis resulted in a diagnosis of P-PNET.
  • In recent years, studies in molecular biology have demonstrated that P-PNET involves the EWS-FLI1 chimeric gene, and immunohistochemical staining has shown P-PNET to be MIC2 positive.
  • P-PNET also differs from C-PNET with regard to prognosis, and for this reason it is believed that P-PNET and C-PNET should be considered separate entities.
  • That is, in spite of the fact that P-PNET is a malignant tumor, patient survival can be comparatively long.
  • Because P-PNET originates intracranially, it is fundamentally an intradural extramedullary tumor.
  • For this reason, treatment should consist of surgical excision that is as complete as possible, followed by appropriate radiotherapy and chemotherapy.
  • [MeSH-major] Diagnostic Errors. Neuroblastoma / pathology. Neuroectodermal Tumors, Primitive, Peripheral / pathology. Supratentorial Neoplasms / pathology
  • [MeSH-minor] Adult. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Humans. Immunohistochemistry. Infant. Magnetic Resonance Imaging. Male. Neurosurgical Procedures. Radiotherapy. Tomography, X-Ray Computed

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  • (PMID = 18095134.001).
  • [ISSN] 1433-7398
  • [Journal-full-title] Brain tumor pathology
  • [ISO-abbreviation] Brain Tumor Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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12. Gunluoglu MZ, Kara HV, Demir A, Dincer SI: Results of multimodal treatment of two patients with thoracic primitive neuroectodermal tumor. Is surgery really helpful for survival? Thorac Cardiovasc Surg; 2007 Oct;55(7):460-1
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  • [Title] Results of multimodal treatment of two patients with thoracic primitive neuroectodermal tumor. Is surgery really helpful for survival?
  • Primitive neuroectodermal tumors (PNET) belong to the group of small round cell tumors and are rarely seen.
  • They are rapidly progressive tumors, despite usually being treated by a multimodal therapy which includes surgery and chemoradiotherapy.
  • We present two patients with PNET of the thorax treated in our clinic.
  • The first patient had a huge tumor in the right hemithorax, which shifted the mediastinum to the contralateral hemithorax.
  • Diagnosis was established by transthoracic fine-needle aspiration biopsy and the tumor was treated by surgical resection.
  • The second patient had a small tumor on the right costovertebral angle which protruded towards the skin and was diagnosed by incisional biopsy.
  • The tumor responded very well to preoperative chemotherapy and complete resection was achieved surgically.
  • This patient had bone metastasis, local recurrence and pleural pulmonary metastasis after 6, 18 and 28 months, respectively, and died 30 months after the operation.We discuss the limited effect of surgery on the treatment of thoracic PNET on the basis of the results of these patients in whom we performed surgery.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Bone Neoplasms / therapy. Brain Neoplasms / therapy. Neoplasm Recurrence, Local / therapy. Neuroectodermal Tumors, Primitive, Peripheral / therapy. Pleural Neoplasms / therapy. Thoracic Neoplasms / therapy. Thoracotomy
  • [MeSH-minor] Adult. Chemotherapy, Adjuvant. Combined Modality Therapy. Fatal Outcome. Female. Humans. Male. Neoadjuvant Therapy. Radiotherapy, Adjuvant. Tomography, X-Ray Computed

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  • (PMID = 17902071.001).
  • [ISSN] 0171-6425
  • [Journal-full-title] The Thoracic and cardiovascular surgeon
  • [ISO-abbreviation] Thorac Cardiovasc Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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13. Ferri Niguez B, Martínez-Lage JF, Almagro MJ, Fuster JL, Serrano C, Torroba MA, Sola J: Embryonal tumor with abundant neuropil and true rosettes (ETANTR): a new distinctive variety of pediatric PNET: a case-based update. Childs Nerv Syst; 2010 Aug;26(8):1003-8
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  • [Title] Embryonal tumor with abundant neuropil and true rosettes (ETANTR): a new distinctive variety of pediatric PNET: a case-based update.
  • BACKGROUND: Embryonal central nervous system (CNS) tumors are currently classified into three types: medulloblastoma, atypical rhabdoid/teratoid tumors, and primitive neuroectodermal tumor (PNET).
  • A distinctive subtype of PNET called "embryonal tumor with abundant neuropil and true rosettes" (ETANTR) was reported in 2000.
  • DISCUSSION: ETANTR is a recently described variety of PNET that combines microscopic features of neuroblastoma and ependymoblastoma, demonstrating areas of fine fibrillary neuropil intermingled with cellular zones and ependymoblastic rosettes.
  • ETANTR is an eminently pediatric tumor that has been reported exclusively in children younger than 4 years.
  • A 23-month-old girl was submitted to surgery for a frontoparietal tumor.
  • In both instances, the histopathological diagnosis confirmed ETANTR.
  • Both children were treated with chemotherapy and one with radiotherapy.
  • CONCLUSIONS: By reporting these two new instances of ETANTR, we want to contribute to the knowledge of this highly malignant CNS embryonal neoplasm that occurs only in young children, given its present lethal prognosis, the scarcity of reported cases, and the lack of treatment guidelines.
  • [MeSH-major] Brain Neoplasms / pathology. Neuroectodermal Tumors, Primitive / pathology. Neuropil / pathology
  • [MeSH-minor] Antineoplastic Agents / therapeutic use. Combined Modality Therapy. Fatal Outcome. Female. Humans. Infant. Neurosurgical Procedures. Radiotherapy

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  • [Cites] Am J Surg Pathol. 2009 Feb;33(2):211-7 [18987548.001]
  • [Cites] Acta Neuropathol. 2009 Apr;117(4):457-64 [19057917.001]
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  • (PMID = 20499240.001).
  • [ISSN] 1433-0350
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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14. Kim AV, Shim KV, Kim DS, Khachaturian VA, Choĭ DY: [The leptomeningeal form of primary neuroectodermal tumor]. Zh Vopr Neirokhir Im N N Burdenko; 2007 Jan-Mar;(1):45-8; discussion 48

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  • [Title] [The leptomeningeal form of primary neuroectodermal tumor].
  • Brain neoplasms as primary leptomeningeal forms are rare, particularly if the case in point is primary neuroectodermal tumors (PNET).
  • Brain computed tomography and magnetic resonance imaging (MRI) revealed internal hydrocephalus and slightly uniform meningeal thickening without the signs of space-occupying lesions.
  • Ten months after ventriculoperitoneostomy, the patient suddenly developed tetraparesis and respiratory disorders.
  • MRI showed a pronounced diffuse non-uniform leptomeningeal dissemination of a tumor.
  • Open biopsy of the lesion in the projection of the right fissure detected PNET.
  • The patient underwent a course of chemotherapy (CCNU, vincristine) and radiotherapy (total focal dose 45 Gy).
  • [MeSH-major] Meningeal Neoplasms / diagnosis. Meningeal Neoplasms / surgery. Neuroectodermal Tumors, Primitive / diagnosis. Neuroectodermal Tumors, Primitive / surgery
  • [MeSH-minor] Adult. Combined Modality Therapy. Humans. Magnetic Resonance Imaging. Male. Ventriculostomy

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  • (PMID = 17526253.001).
  • [ISSN] 0042-8817
  • [Journal-full-title] Zhurnal voprosy neĭrokhirurgii imeni N. N. Burdenko
  • [ISO-abbreviation] Zh Vopr Neirokhir Im N N Burdenko
  • [Language] rus
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
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15. Utsunomiya A, Uenohara H, Suzuki S, Shimosaka S, Numagami Y, Nishimura S, Nishino A, Suzuki H, Sakurai Y: [A case of peripheral-type primitive neuroectodermal tumor arising in the dura mater at the frontal base]. No To Shinkei; 2004 Mar;56(3):237-41
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  • [Title] [A case of peripheral-type primitive neuroectodermal tumor arising in the dura mater at the frontal base].
  • Magnetic resonance image (MRI) revealed a large tumor in the frontal base with tumoral hemorrhage.
  • Angiography showed the tumor was fed by anterior meningeal arteries.
  • At surgery, the tumor was arising in the dura mater at the frontal base, and was removed totally.
  • Histological examination showed the tumor to be composed of small cells with uniform round nuclei and minimal cytoplasm.
  • The tumor was histologically confirmed to be peripheral-type primitive neuroectodermal tumor(pPNET).
  • Following surgery, he underwent whole brain, whole spine and local radiation therapy(30 Gy in total respectively) and received two 5-day cycles of chemotherapy, consisting of intravenous administration of cisplatin 20 mg/m2/day, etoposide 60 mg/m2/day and IFOS 900 mg/m2/day.
  • After these therapies, follow-up radiological examination showed there was no recurrence of the tumor for 24 months.
  • Ewing sarcoma and pPNET(ES/pPNET) is the designation given to a family of small round cell tumor arising in bone or soft tissues.
  • Intracranial PNETs are devided into central nervous system PNET(cPNET) and pPNET.
  • It is necessary that intracranial PNETs are divided into two types of PNETs because of different prognosis between these tumors.
  • MIC-2 is a specific marker for pPNET/ES family and is useful in the differential diagnosis of these two types of tumors.
  • [MeSH-major] Dura Mater. Meningeal Neoplasms / diagnosis. Neuroectodermal Tumors, Primitive, Peripheral / diagnosis
  • [MeSH-minor] Antigens, CD / analysis. Biomarkers, Tumor / analysis. Cell Adhesion Molecules / analysis. Chemotherapy, Adjuvant. Child. Diagnosis, Differential. Humans. Magnetic Resonance Angiography. Male. Radiotherapy, Adjuvant. Treatment Outcome

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  • (PMID = 15112448.001).
  • [ISSN] 0006-8969
  • [Journal-full-title] Nō to shinkei = Brain and nerve
  • [ISO-abbreviation] No To Shinkei
  • [Language] jpn
  • [Publication-type] Case Reports; English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Biomarkers, Tumor; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules
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16. Flannery T, Kano H, Martin JJ, Niranjan A, Flickinger JC, Lunsford LD, Kondziolka D: Boost radiosurgery as a strategy after failure of initial management of pediatric primitive neuroectodermal tumors. J Neurosurg Pediatr; 2009 Mar;3(3):205-10
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  • [Title] Boost radiosurgery as a strategy after failure of initial management of pediatric primitive neuroectodermal tumors.
  • OBJECT: The aim in this study was to determine the outcomes of boost stereotactic radiosurgery, specifically Gamma Knife surgery (GKS), for recurrent primitive neuroectodermal tumors (PNETs) in children in whom initial multimodality management had failed.
  • Nine procedures were directed at the primary site, whereas 6 were directed at distant brain sites.
  • All patients received multimodality therapy, including resection, fractionated radiotherapy, and chemotherapy.
  • The mean interval from diagnosis to first stereotactic radiosurgery was 25.8 months (range 11-35 months).
  • The mean tumor volume was 3.9 cm(3) (range 1.1-13.1 cm(3)), and an average marginal dose of 14.5 Gy (range 9-20 Gy) was administered.
  • CONCLUSIONS: The GKS modality was a well-tolerated and useful boost strategy in pediatric patients with PNET and a poor prognosis in whom initial multimodality management had failed.
  • [MeSH-major] Brain Neoplasms / surgery. Neuroectodermal Tumors, Primitive / surgery. Radiosurgery
  • [MeSH-minor] Child. Child, Preschool. Combined Modality Therapy. Disease Progression. Female. Humans. Infant. Male. Reoperation. Retrospective Studies. Salvage Therapy. Treatment Outcome. Tumor Burden

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  • [CommentIn] J Neurosurg Pediatr. 2009 Mar;3(3):203; discussion 204 [19267519.001]
  • (PMID = 19338466.001).
  • [ISSN] 1933-0707
  • [Journal-full-title] Journal of neurosurgery. Pediatrics
  • [ISO-abbreviation] J Neurosurg Pediatr
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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17. Jennings MT, Cmelak A, Johnson MD, Moots PL, Pais R, Shyr Y: Differential responsiveness among "high risk" pediatric brain tumors in a pilot study of dose-intensive induction chemotherapy. Pediatr Blood Cancer; 2004 Jul;43(1):46-54
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  • [Title] Differential responsiveness among "high risk" pediatric brain tumors in a pilot study of dose-intensive induction chemotherapy.
  • BACKGROUND: These factors have been predictive for progressive disease on therapy (PDOT) among pediatric brain tumors: >1.5 cm(2) unresectable tumor, glioblastoma, supratentorial primitive neuroectodermal tumor, and metastatic medulloblastoma (MBL).
  • Maintenance chemotherapy consisted of eight cycles of carboplatin, etoposide, and vincristine.
  • RESULTS: Twenty newly diagnosed patients [nine primitive neuroectodermal tumors/MBL, one choroid plexus carcinoma, eight malignant gliomas, and two anaplastic ependymomas] were treated.
  • Ten patients, who required neuraxis irradiation, constituted the "PNET" group.
  • Induction chemotherapy produced partial and minor responses (MRs) among 5/10.
  • Their estimated median progression free survival was 6.9 months (P = 0.035 relative to the PNET) with a median survival of 10.7 months (P = 0.04).
  • CONCLUSIONS: This induction regimen produced a cytoreductive response in 6/10 and achieved a significant improvement in progression free survival among 7/10 in the PNET group.
  • Expression of the biologic factors was similar between both groups and did not correlate with diagnosis or response.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Brain Neoplasms / drug therapy. Glioma / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Cisplatin / administration & dosage. Cyclophosphamide / administration & dosage. Disease-Free Survival. Dose Fractionation. Etoposide / administration & dosage. Female. Humans. Male. Neoadjuvant Therapy. Pilot Projects. Statistics, Nonparametric. Survival Rate. Vincristine / administration & dosage

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  • [Copyright] Copyright 2004 Wiley-Liss, Inc.
  • (PMID = 15170889.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 8N3DW7272P / Cyclophosphamide; Q20Q21Q62J / Cisplatin
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18. Postovsky S, Ben Arush MW, Elhasid R, Davidson S, Leshanski L, Vlodavsky E, Guilburd JN, Amikam D: A novel case of a CAT to AAT transversion in codon 179 of the p53 gene in a supratentorial primitive neuroectodermal tumor harbored by a young girl. Case report and review of the literature. Oncology; 2003;65(1):46-51
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  • [Title] A novel case of a CAT to AAT transversion in codon 179 of the p53 gene in a supratentorial primitive neuroectodermal tumor harbored by a young girl. Case report and review of the literature.
  • BACKGROUND: The most common cytogenetic abnormality encountered in primitive neuroectodermal tumors (PNET)/medulloblastoma is loss of heterozygosity in the region of the short arm of chromosome 17.
  • There is some evidence that supratentorial PNET has different cytogenetic markers than infratentorial PNET/medulloblastoma.
  • We describe a young girl diagnosed with supratentorial PNET (SPNET).
  • Analysis of the tumor suppressor gene p53 in the tumorous tissue revealed a rare transversion mutational event of CAT to AAT in position 179 of exon 5.
  • MRI of the brain showed a large space- occupying lesion in the right frontal lobe.
  • Histological examination of the macroscopic resection of the tumor revealed PNET of the brain.
  • Therapy was started according to the Children's Cancer group protocol (CCG-99702) designated for treatment of high-risk central nervous system embryonal tumors.
  • She received an initial course of chemotherapy, consisting of cyclophosphamide and vincristine for mobilizing and harvesting peripheral blood stem cells (PBSCs).
  • Then she was given craniospinal irradiation (3,600 cGy) with a boost to the tumor bed (1,980 cGy) and three consecutive courses of high-dose chemotherapy with carboplatin, vincristine and thiotepa/cyclophosphamide, with PBSCs support after each course.
  • RESULTS: The patient is in complete remission 17 months after diagnosis, based on the results of physical examination and imaging studies.
  • There are only 12 known mutations of the reported type, and the finding of such a rare mutational event in a low-incidence p53 mutation tumor, such as SPNET, might add additional insight into the p53-SPNET relationship in tumorigenesis.
  • Although not widely accepted, it is possible that different mutations of the p53 gene in patients with brain tumors may imply a different ultimate prognosis.
  • In our case, we cannot exclude the fact that transversion of CAT to AAT in position 179 of exon 5 may explain prolonged survival of a patient with good response to therapy.
  • [MeSH-major] Genes, p53 / genetics. Loss of Heterozygosity. Neuroectodermal Tumors, Primitive / diagnosis. Neuroectodermal Tumors, Primitive / genetics. Supratentorial Neoplasms / diagnosis. Supratentorial Neoplasms / genetics
  • [MeSH-minor] Child. Combined Modality Therapy. Diagnosis, Differential. Female. Headache / etiology. Humans. Magnetic Resonance Imaging. Nausea / etiology. Polymerase Chain Reaction. Vision Disorders / etiology


19. Kanamori M, Kumabe T, Saito R, Yamashita Y, Sonoda Y, Tominaga T: [The safety of combination chemotherapy with ifosfamide, cisplatin, and etoposide (ICE): single-institution retrospective review of 108 cases]. No Shinkei Geka; 2010 Nov;38(11):997-1005
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  • [Title] [The safety of combination chemotherapy with ifosfamide, cisplatin, and etoposide (ICE): single-institution retrospective review of 108 cases].
  • PURPOSE: The adverse effects of combination chemotherapy of ifosfamide, cisplatin, and etoposide (ICE) were evaluated in the treatment of various intracranial brain tumors.
  • The histological diagnosis was newly diagnosed or recurrent germ cell tumor in 45 cases, medulloblastoma in 19, primitive neuroectodermal tumor (PNET) in 7, anaplastic ependymoma in 6, recurrent glioblastoma in 13, and others in 18 cases.
  • Patients received 1-8 cycles of ICE chemotherapy with or without radiation therapy.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / adverse effects. Brain Neoplasms / drug therapy
  • [MeSH-minor] Adolescent. Adult. Antineoplastic Agents / administration & dosage. Antineoplastic Agents / adverse effects. Antineoplastic Agents, Alkylating / administration & dosage. Antineoplastic Agents, Alkylating / adverse effects. Antineoplastic Agents, Phytogenic / administration & dosage. Antineoplastic Agents, Phytogenic / adverse effects. Child. Child, Preschool. Cisplatin / administration & dosage. Cisplatin / adverse effects. Ependymoma / drug therapy. Etoposide / administration & dosage. Etoposide / adverse effects. Female. Glioblastoma / drug therapy. Hematologic Diseases / chemically induced. Humans. Ifosfamide / administration & dosage. Ifosfamide / adverse effects. Infant. Male. Medulloblastoma / drug therapy. Middle Aged. Neoplasms, Germ Cell and Embryonal / drug therapy. Neuroectodermal Tumors, Primitive / drug therapy. Retrospective Studies

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  • (PMID = 21081811.001).
  • [ISSN] 0301-2603
  • [Journal-full-title] No shinkei geka. Neurological surgery
  • [ISO-abbreviation] No Shinkei Geka
  • [Language] jpn
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Antineoplastic Agents, Alkylating; 0 / Antineoplastic Agents, Phytogenic; 6PLQ3CP4P3 / Etoposide; Q20Q21Q62J / Cisplatin; UM20QQM95Y / Ifosfamide
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20. Gutenberg A, Schulten HJ, Gunawan B, Ludwig HC, Brück W, Larsen J, Rohde V: CNS tumor 22 years after spinal neuroblastoma IV: diagnostic dilemma between recurrence and secondary malignancy. Pediatr Neurosurg; 2009;45(1):61-8
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  • [Title] CNS tumor 22 years after spinal neuroblastoma IV: diagnostic dilemma between recurrence and secondary malignancy.
  • We present the very unusual case of a young woman suffering from a brain tumor 22 years after a stage IV spinal neuroblastoma as an infant, demonstrating the difficulties of differentiating late neuroblastoma relapse from secondary supratentorial primitive neuroectodermal tumor (sPNET).
  • Lacking specific immunohistochemical features, the first cerebral tumor at the age of 21 was regarded as sPNET, and we pursued a therapeutic approach consisting of neurosurgical resection as well as irradiation and high-dose alkylator-based chemotherapy according to the HIT2000 protocol.
  • Moreover, the lack of PNET-specific translocations (EWS/FLI1 gene fusion) in both brain tumors as well as the development of hepatic metastases was more compatible with the diagnosis of a very late relapse 22 years after initial stage IV spinal neuroblastoma.
  • [MeSH-major] Brain Neoplasms / pathology. Neoplasm Recurrence, Local / pathology. Neoplasms, Second Primary / pathology. Neuroblastoma / pathology. Spinal Neoplasms / pathology
  • [MeSH-minor] Adult. DNA, Neoplasm / genetics. Diagnosis, Differential. Female. Genetic Markers. Humans. Immunohistochemistry. Infant. Magnetic Resonance Imaging. Neoplasm Staging. Time Factors

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  • (PMID = 19258732.001).
  • [ISSN] 1423-0305
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / Genetic Markers
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21. Le BH, Sandusky M: 81 year-old male with confusion and weakness. Brain Pathol; 2010 Jul;20(4):867-70
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  • Glioblastoma, the most common primary brain tumor, is a highly infiltrative, malignant astrocytic neoplasm that demonstrates a wide spectrum of morphologic heterogeneity.
  • Cases with a primitive neuroectodermal tumor (PNET)-like component are rare, but are being increasingly recognized and studied.
  • The primitive component typically shows immunohistochemical features that are indicative of potential for divergent differentiation along glial and neuronal pathways; when present, the entire neuraxis may be at risk for involvement, portending a particularly poor prognosis.
  • Recently, data from the largest case series studying malignant gliomas with a PNET-like component suggest that the primitive component likely arises from the malignant glial component.
  • This report presents an example of glioblastoma with a prominent primitive neuroectodermal-like component in an 81 year-old male who, during the course of concurrent chemotherapy and radiation therapy, died five weeks following initial diagnosis.
  • [MeSH-major] Brain Neoplasms / pathology. Confusion / pathology. Glioblastoma / pathology. Muscle Weakness / pathology. Neuroectodermal Tumors, Primitive / pathology. Temporal Lobe / pathology

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  • (PMID = 20626749.001).
  • [ISSN] 1750-3639
  • [Journal-full-title] Brain pathology (Zurich, Switzerland)
  • [ISO-abbreviation] Brain Pathol.
  • [Language] eng
  • [Publication-type] Letter
  • [Publication-country] Switzerland
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22. Nakamura H, Makino K, Kochi M, Nakazato Y, Kuratsu J: Successful treatment of neoadjuvant therapy for papillary tumor of the pineal region. Brain Tumor Pathol; 2009;26(2):73-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Successful treatment of neoadjuvant therapy for papillary tumor of the pineal region.
  • The fi nal diagnosis was papillary tumor of the pineal region (PTPR).
  • Hydrocephalus was addressed by immediate ventricle drainage; subsequently, we attempted tumor removal.
  • As the intraoperative diagnosis of the hemorrhagic tumor was primitive neuroectodermal tumor (PNET), we did not proceed to total removal.
  • After the delivery of radiotherapy (50.4 Gy) and one course of Nimustine hydrochloride (ACNU) chemotherapy, the residual tumor was completely resected.
  • The diagnosis at that time (1994) was papillary pineocytoma.
  • This type of tumor was later proposed to represent a new distinct tumor subtype, papillary tumor of the pineal region (PTPR).
  • Our data indicate that our patient's tumor should be included in this category.
  • [MeSH-major] Brain Neoplasms / therapy. Carcinoma, Papillary / diagnosis. Carcinoma, Papillary / therapy. Pinealoma / therapy
  • [MeSH-minor] Child. Humans. Hydrocephalus / therapy. Image Processing, Computer-Assisted. Magnetic Resonance Imaging. Male. Neoadjuvant Therapy. Neurosurgical Procedures. Nimustine / therapeutic use. Pineal Gland / pathology. Pineal Gland / surgery. Treatment Outcome

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  • (PMID = 19856218.001).
  • [ISSN] 1861-387X
  • [Journal-full-title] Brain tumor pathology
  • [ISO-abbreviation] Brain Tumor Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
  • [Chemical-registry-number] 0S726V972K / Nimustine
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23. Herrlinger U, Steinbrecher A, Rieger J, Hau P, Kortmann RD, Meyermann R, Schabet M, Bamberg M, Dichgans J, Bogdahn U, Weller M: Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse. J Neurol; 2005 Mar;252(3):291-9
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  • [Title] Adult medulloblastoma: prognostic factors and response to therapy at diagnosis and at relapse.
  • Adult medulloblastoma is a rare tumor with few retrospective studies published so far.
  • The role of adjuvant chemotherapy or chemotherapy at relapse is unclear.
  • This study reports therapy and outcome in all adult (>or=16 years old) medulloblastoma (n=34) and supratentorial primitive neuroectodermal tumor (PNET) patients (n=2) treated in 2 neuro-oncological centers between 1976 and 2002.
  • After resection, 16 patients were treated with craniospinal radiotherapy alone, 20 patients also received adjuvant chemotherapy (8 vincristine, CCNU, cisplatin; 7 methotrexate alone or methotrexate/vincristine-based polychemotherapy; 5 other protocols).
  • Adjuvant chemotherapy was associated with a non-significant trend to prolonged survival (relative risk (RR) 1.89; p=0.068).
  • The median progression-free survival (PFS) after primary therapy was 83 months.
  • At relapse, 10 of 12 evaluable patients achieved a complete response upon second-line therapy.
  • The median survival times from first (n=17) and second relapse (n=9) were 21 months (0-67+ months; 5/17 without second relapse) and 20 months (1-29 months).
  • Cox regression analysis revealed the infiltration of the floor of the 4(th) ventricle at diagnosis as the only therapy-independent prognostic factor (RR 0.48; p=0.03).
  • In conclusion, adjuvant chemotherapy may prolong survival in adult medulloblastoma patients.
  • Moreover, second-line therapy may be beneficial for these patients.
  • As in pediatric medulloblastoma patients, primary infiltration of the floor of the 4(th) ventricle indicates a poor prognosis.
  • [MeSH-major] Cerebellar Neoplasms / therapy. Medulloblastoma / therapy
  • [MeSH-minor] Adolescent. Adult. Aged. Analysis of Variance. Combined Modality Therapy. Demography. Disease Progression. Disease-Free Survival. Dose-Response Relationship, Radiation. Drug Therapy / methods. Female. Humans. Male. Middle Aged. Radiotherapy, High-Energy / methods. Recurrence. Regression Analysis. Retrospective Studies. Risk Factors. Time Factors. Treatment Outcome

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  • (PMID = 16189725.001).
  • [ISSN] 0340-5354
  • [Journal-full-title] Journal of neurology
  • [ISO-abbreviation] J. Neurol.
  • [Language] eng
  • [Publication-type] Clinical Trial; Comparative Study; Journal Article
  • [Publication-country] Germany
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24. Inenaga C, Toyoshima Y, Mori H, Nishiyama K, Tanaka R, Takahashi H: A fourth ventricle atypical teratoid/rhabdoid tumor in an infant. Brain Tumor Pathol; 2003;20(2):47-52
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  • [Title] A fourth ventricle atypical teratoid/rhabdoid tumor in an infant.
  • Atypical teratoid/rhabdoid tumor (AT/RT), a recently established central nervous system tumor entity, occurs in children and is more malignant than medulloblastoma/primitive neuroectodermal tumors (PNET).
  • We report here a case of AT/RT in a male infant who was 9 months old at the time of diagnosis.
  • Magnetic resonance imaging revealed that the tumor occupied the fourth ventricle, and at surgery it was found to adhere to the floor of the fourth ventricle.
  • After subtotal removal of the tumor mass, chemotherapy and radiotherapy were performed, but the patient died about 8 months after the diagnosis following rapid regrowth of the residual tumor.
  • Light-microscopically, the tumor was composed mainly of nests of rhabdoid cells with fields of PNET.
  • [MeSH-major] Brain Neoplasms / pathology. Fourth Ventricle / pathology. Rhabdoid Tumor / pathology

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  • (PMID = 14756440.001).
  • [ISSN] 1433-7398
  • [Journal-full-title] Brain tumor pathology
  • [ISO-abbreviation] Brain Tumor Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Japan
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25. Massimino M, Gandola L, Cefalo G, Lasio G, Riva D, Fossati-Bellani F, Gianni MC, Luksch R, Tesoro-Tess JD, Lombardi F: Management of medulloblastoma and ependymoma in infants: a single-institution long-term retrospective report. Childs Nerv Syst; 2000 Jan;16(1):15-20
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  • To reduce the sequelae from CNS irradiation (RT), 16 children younger than 3 years with medulloblastoma-PNET (13 cases) and ependymoma (3 cases) were treated between 1987-1993 according to different postsurgical chemotherapy (CT) programs.
  • Eleven patients were rendered disease-free by surgery, while 5 had residual tumor.
  • Adjuvant therapy depended on patients' age, postsurgical status and parents' consent to radiotherapy (RT).
  • Seven patients relapsed a median of 13 months after diagnosis, and all 7 of them died of their disease.
  • One child in whom the entire CNS was irradiated developed glioblastoma multiforme 120 months after the first diagnosis of medulloblastoma.
  • [MeSH-major] Brain Neoplasms / surgery. Cerebellar Neoplasms / surgery. Ependymoma / surgery. Medulloblastoma / surgery. Neuroectodermal Tumors, Primitive / surgery
  • [MeSH-minor] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Chemotherapy, Adjuvant. Child, Preschool. Combined Modality Therapy. Cranial Irradiation. Female. Follow-Up Studies. Humans. Infant. Male. Radiotherapy, Adjuvant. Retrospective Studies. Survival Rate

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  • (PMID = 10672424.001).
  • [ISSN] 0256-7040
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] GERMANY
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26. Yazigi-Rivard L, Masserot C, Lachenaud J, Diebold-Pressac I, Aprahamian A, Avran D, Doz F: [Childhood medulloblastoma]. Arch Pediatr; 2008 Dec;15(12):1794-804
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  • Medulloblastoma is one of the most common malignant childhood brain tumors.
  • It is a primitive neuroectodermal tumor (PNET) and predominantly arises in the cerebellum and 4th ventricle.
  • Brain and spinal MRI can establish the diagnosis of posterior fossa tumor and define the extent of the disease.
  • Histologic examination of the tumor confirms the diagnosis of medulloblastoma.
  • Patients are classified into 2 risk groups: standard-risk medulloblastoma, defined by nonmetastatic disease treated by total or subtotal tumor resection; and high-risk patients who have disseminated disease and/or residual disease.
  • Tumor molecular genetic findings allow the use of emerging prognostic factors and may ultimately contribute to the development of targeted therapy.
  • Current treatment in the oldest children combines surgical resection followed by radiotherapy and chemotherapy.
  • Treatment in younger patients is as much as possible restricted to surgery and chemotherapy.
  • However, long-term sequelae after treatment for medulloblastoma remain frequent and the detection and treatment of those sequelae is an essential part of the follow-up of the patients.
  • [MeSH-minor] Age Factors. Child. Child, Preschool. Combined Modality Therapy. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Meta-Analysis as Topic. Neoplasm Metastasis. Neoplasms, Second Primary / etiology. Prognosis. Prospective Studies. Radiotherapy / adverse effects. Radiotherapy Dosage. Randomized Controlled Trials as Topic. Risk Factors. Treatment Outcome

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  • (PMID = 18995998.001).
  • [ISSN] 0929-693X
  • [Journal-full-title] Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie
  • [ISO-abbreviation] Arch Pediatr
  • [Language] fre
  • [Publication-type] Comparative Study; English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 52
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27. Dang T, Vassilyadi M, Michaud J, Jimenez C, Ventureyra EC: Atypical teratoid/rhabdoid tumors. Childs Nerv Syst; 2003 Apr;19(4):244-8
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  • This tumor is typically misdiagnosed as a primitive neuroectodermal tumor (PNET)/medulloblastoma.
  • The unique immunohistochemistry profile of an ATRT helps distinguish it from a PNET/medulloblastoma.
  • This is of clinical importance because the prognosis of a patient with an ATRT is worse than that of a PNET/medulloblastoma despite aggressive surgical treatment with or without adjuvant chemotherapy and radiation therapy.
  • [MeSH-major] Brain Neoplasms. Rhabdoid Tumor. Teratoma
  • [MeSH-minor] Child, Preschool. Diagnosis, Differential. Female. Humans. Immunohistochemistry. Infant. Magnetic Resonance Imaging. Male. Medulloblastoma / diagnosis. Prognosis. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 12682757.001).
  • [ISSN] 0256-7040
  • [Journal-full-title] Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
  • [ISO-abbreviation] Childs Nerv Syst
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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