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CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2022; 41(02): e187-e191
DOI: 10.1055/s-0042-1742709
Case Report

Cervical Primitive Neuroectodermal Tumor in an Adult: Case Report and Literature Review[*]

Tumor neuroectodérmico primitivo cervical em adulto: Relato de caso e revisão da literatura
Guilherme dos Santos de Alencar 
1   Department of Neurosurgery, Curso de Medicina, Universidade Federal do Paraná (UFPR), Curitiba, PR, Brazil
,
Maria Eduarda Turczyn De Lucca 
2   Universidade Positivo (UP), Curitiba, PR, Brazil
,
Felipe Antonio Torres Mazzo
3   Medicine Program, Pontifícia Universidade Católica do Paraná (PUCPR), Curitiba, PR, Brazil
,
Milena Massumi Konozoe 
4   Department of Pathology, Hospital Erasto Gaertner, Curitiba, PR, Brazil
,
Eduardo Talib Bacchi Jaouhari 
5   Department of Neurosurgery, Hospital Erasto Gaertner, Curitiba, PR, Brazil
,
Rodrigo Leite de Morais
5   Department of Neurosurgery, Hospital Erasto Gaertner, Curitiba, PR, Brazil
,
Carlos Eliseu Barcelos
5   Department of Neurosurgery, Hospital Erasto Gaertner, Curitiba, PR, Brazil
,
Rosângela Stadnick Lauth de Almeida Torres 
6   Department of Pharmaceutical Sciences, Faculdade de Farmácia, Universidade Positivo (UP), Curitiba, PR, Brazil
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Abstract

Introduction Primitive neuroectodermal tumors are rare neoplasms of the central nervous system that occur in children, with few reports in adults. These tumors are found most often in the cerebral hemispheres, with spinal cord disorders being rare.

Case Report A 71-year-old man with motor and sensory deficits in the upper limbs, cervical pain, and urinary incontinence presented to the Neurosurgery Service. The physical examination revealed grade-III motor strength on the right side, grade IV- on the left upper limb, and grade IV+ on the left lower limb. A magnetic resonance imaging scan showed an expansive intramedullary lesion with a C3-C4 epicenter. Spinal decompression, lesional biopsy, and adjuvant radiotherapy were performed. The anatomopathological report showed a primitive neuroectodermal tumor. After a new treatment with adjuvant radiotherapy (20 × 1.8 Gy in the skull and neuroaxis and 5 × 1.8 Gy in tumor boost), the patient progressed without recurrence.

Conclusion Since the characteristics of the tumor are similar to those of medulloblastoma, it is necessary to expand the studies on these lesions, to better understand their pathophysiology and list better diagnostic and therapeutic methods, in addition to those already available.

Resumo

Introdução Os tumores neuroectodérmicos primitivos são neoplasias raras do sistema nervoso central que ocorrem em crianças, com escassos relatos em adultos. Esses tumores são encontrados mais frequentemente nos hemisférios cerebrais, sendo raros os acometimentos medulares.

Relato de Caso Um homem de 71 anos com déficits motor e sensitivo em membros superiores, algia cervical e incontinência urinária apresentou-se ao Serviço de Neurocirurgia. O exame físico revelou força de grau III à direta, de grau IV- no membro superior esquerdo, e de grau IV+ no membro inferior esquerdo. Um exame de ressonância magnética denotou lesão expansiva intramedular com epicentro em C3-C4. Foram realizadas descompressão medular, biópsia lesional e radioterapia adjuvante. O laudo anatomopatológico evidenciou tumor neuroectodérmico primitivo. Após novo tratamento com radioterapia adjuvante (20 × 1,8 Gy no crânio e neuroeixo e 5 × 1,8 Gy em boost tumoral), o paciente seguiu sem recidiva.

Conclusões Uma vez que as características do tumor se assemelham às do meduloblastoma, torna-se necessário ampliar os estudos acerca dessas lesões, a fim de compreender melhor sua fisiopatologia e elencar melhores métodos diagnósticos e terapêuticos, além dos já disponíveis.

Keywords

primitive neuroectodermal tumors - adult - medulloblastoma

Palavras-chave

tumor neuroectodérmico primitivo - adulto - meduloblastoma

Ethics Statement

The present study complied with all institutional guidelines for studies on human beings. Informed consent was obtained from the person responsible for the patient.


* Study conducted at Hospital Erasto Gaertner, Curitiba, Paraná - PR, Brazil.




Publikationsverlauf

Eingereicht: 11. Mai 2021

Angenommen: 20. Dezember 2021

Artikel online veröffentlicht:
08. März 2022

© 2022. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • References

  • 1 Jain A, Sharma MC, Suri V. et al. Spectrum of pediatric brain tumors in India: a multi-institutional study. Neurol India 2011; 59 (02) 208-211 DOI: 10.4103/0028-3886.79142.
    CrossrefPubMedGoogle Scholar
  • 2 Adamski J, Ramaswamy V, Huang A, Bouffet E. Advances in managing medulloblastoma and intracranial primitive neuro-ectodermal tumors. F1000Prime Rep 2014; 6: 56-67 DOI: 10.12703/p6-56.
    CrossrefPubMedGoogle Scholar
  • 3 Zheng YC, Jung SM, Lee ST. et al. Adult supratentorial extra-pineal primitive neuro-ectodermal tumors. J Clin Neurosci 2014; 21 (05) 803-809 DOI: 10.1016/j.jocn.2013.07.025.
    CrossrefPubMedGoogle Scholar
  • 4 Biswas S, Burke A, Cherian S. et al. Non-pineal supratentorial primitive neuro-ectodermal tumors (sPNET) in teenagers and young adults: Time to reconsider cisplatin based chemotherapy after cranio-spinal irradiation?. Pediatr Blood Cancer 2009; 52 (07) 796-803 DOI: 10.1002/pbc.21899.
    CrossrefPubMedGoogle Scholar
  • 5 Terheggen F, Troost D, Majoie CB, Leenstra S, Richel DJ. Local recurrence and distant metastasis of supratentorial primitive neuro-ectodermal tumor in an adult patient successfully treated with intensive induction chemotherapy and maintenance temozolomide. J Neurooncol 2007; 82 (01) 113-116 DOI: 10.1007/s11060-006-9249-3.
    CrossrefPubMedGoogle Scholar
  • 6 Zhang Q, Su X, Liang S. [A clinicopathological analysis of primitive neuroectodermal tumors of the CNS]. Zhonghua Bing Li Xue Za Zhi 1998; 27 (04) 282-285
    PubMedGoogle Scholar
  • 7 Vassal G, Terrier-Lacombe MJ, Lellouch-Tubiana A. et al. Tumorigenicity of cerebellar primitive neuro-ectodermal tumors in athymic mice correlates with poor prognosis in children. Int J Cancer 1996; 69 (02) 146-151 DOI: 10.1002/(sici)1097-0215(19960422)69:2%3C146:aid-ijc14%3E3.0.co;2-7.
    PubMedGoogle Scholar
  • 8 Halliday GC, Junckerstorff RC, Bentel JM. et al. The case for DNA methylation based molecular profiling to improve diagnostic accuracy for central nervous system embryonal tumors (not otherwise specified) in adults. J Clin Neurosci 2018; 47: 163-167 DOI: 10.1016/j.jocn.2017.09.013.
    CrossrefPubMedGoogle Scholar
  • 9 Maaz AUR, Yousif T, Saleh A. et al. Presenting symptoms and time to diagnosis for Pediatric Central Nervous System Tumors in Qatar: a report from Pediatric Neuro-Oncology Service in Qatar. Childs Nerv Syst 2021; 37 (02) 465-474 DOI: 10.1007/s00381-020-04815-z.
    CrossrefPubMedGoogle Scholar
  • 10 Rombi B, Vennarini S, Vinante L, Ravanelli D, Amichetti M. Proton radiotherapy for pediatric tumors: review of first clinical results. Ital J Pediatr 2014; 40: 74-82 DOI: 10.1186/s13052-014-0074-6.
    CrossrefPubMedGoogle Scholar
  • 11 Miralbell R, Lomax A, Russo M. Potential role of proton therapy in the treatment of pediatric medulloblastoma/primitive neuro-ectodermal tumors: spinal theca irradiation. Int J Radiat Oncol Biol Phys 1997; 38 (04) 805-811 DOI: 10.1016/s0360-3016(97)00005-9.
    CrossrefPubMedGoogle Scholar
  • 12 Louis D, Ohgaki H, Wiestler O, Cavenee W. WHO classification of tumours of the central nervous system. Lyon: IARC Press; 2007
    Google Scholar