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Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2015; 34(03): 241-244
DOI: 10.1055/s-0035-1560034
Case Report | Relato de Caso
Thieme Publicações Ltda Rio de Janeiro, Brazil

Atypical Teratoid Rhabdoid Tumor in a Child: Case Report

Tumor teratóide rabdóide atípico em uma criança: relato de caso
Lucas Crociati Meguins
1   Departamento de Ciências Neurológicas. Hospital de Base da Faculdade de Medicina de São José do Rio Preto (FAMERP), São José do Rio Preto, São Paulo, Brazil
,
Ronaldo Brasileiro de Miranda Batista Fernandes
1   Departamento de Ciências Neurológicas. Hospital de Base da Faculdade de Medicina de São José do Rio Preto (FAMERP), São José do Rio Preto, São Paulo, Brazil
,
Adriana Giubilei Pimenta
1   Departamento de Ciências Neurológicas. Hospital de Base da Faculdade de Medicina de São José do Rio Preto (FAMERP), São José do Rio Preto, São Paulo, Brazil
,
Ricardo Lourenço Caramanti
1   Departamento de Ciências Neurológicas. Hospital de Base da Faculdade de Medicina de São José do Rio Preto (FAMERP), São José do Rio Preto, São Paulo, Brazil
,
Eliane Milharcix Zanovelo
2   Departamento de Patologia, Hospital de Base da Faculdade de Medicina de São José do Rio Preto (FAMERP), São José do Rio Preto, São Paulo, Brazil
,
Carmen Lúcia Penteado Lancellotti
2   Departamento de Patologia, Hospital de Base da Faculdade de Medicina de São José do Rio Preto (FAMERP), São José do Rio Preto, São Paulo, Brazil
,
Mário José Góes
1   Departamento de Ciências Neurológicas. Hospital de Base da Faculdade de Medicina de São José do Rio Preto (FAMERP), São José do Rio Preto, São Paulo, Brazil
› Author Affiliations
Further Information

Publication History

01 May 2015

12 June 2015

Publication Date:
24 September 2015 (online)

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Abstract

Atypical teratoid/rhabdoid tumor (ATRT) is a rare and aggressive type of embryonal tumor of the central nervous system (CNS) occurring in childhood. The present study aims to describe the case of a 16-year-old girl who presented with an occipital mass lesion that was diagnosed as ATRT. We present a brief review of the current knowledge of the treatment of this rare neoplasm. A previously healthy 16-year-old girl was referred after two episodes of partial complex seizure 2 weeks before admission. MRI showed a right parieto-occipital lesion with homogeneous contrast-enhancing and significant surrounding brain edema. The patient underwent uneventful surgical resection of the lesion and was discharged home on the fifth postoperative day. Pathologic examination revealed it to be ATRT. The patient was referred to chemotherapy and radiotherapy. After 6 months of follow-up, the patient remains free of seizure and disease progression. ATRT is a rare and aggressive disease. Therefore, early diagnosis and treatment may improve the patient's prognosis and quality of life.

Resumo

O tumor teratoide rabdoide atípico (TTRA) é um tipo de neoplasia rara, com comportamento maligno, que atinge o sistema nervoso central (SNC) de crianças. O objetivo do presente estudo é relatar o caso de uma paciente de 16 anos de idade admitida no nosso serviço com uma lesão occipital que revelou tratar-se de TTRA em estudo anatomopatológico. Uma paciente previamente hígida foi admitida no nosso serviço referindo ocorrência de dois episódios de crises convulsivas nas últimas 2 semanas. A ressonância magnética de crânio mostrou a presença de lesão occipital direita com captação homogênea do meio de contraste. A paciente foi submetida a cirurgia de ressecção da lesão expansiva sem intercorrências. A análise histopatológica e imuno-histoquímica revelou tratar-se de TTRA. A paciente foi encaminhada para a realização de quimioterapia e radioterapia complementares ao tratamento cirúrgico. Após 6 meses de seguimento ambulatorial, a paciente encontra-se assintomática, sem recorrência das crises convulsivas e/ou progressão radiológica da doença. TTRA é uma doença rara e agressiva. Com isso, diagnóstico e tratamento antecipados podem aprimorar prognóstico e qualidade de vida dos pacientes.

Keywords

atypical teratoid/rhabdoid tumor - childhood - embryonal tumor

Palavras-chave

tumor teratoide rabdoide atípico (TTRA) - criança - tumor embrionário

Consent Statement

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editor of this journal.


 

  • References

  • 1 Güner G, Önder S, Söylemezoğlu F. Cytomorphological features of atypical teratoid/rhabdoid tumor: an account of 12 years' experience. Diagn Cytopathol 2014; 42 (10) 856-862
    CrossrefPubMedSearch in Google Scholar
  • 2 Souki C, Abdel-Rhaman M, Qasem A, Al-Hussaini M. Atypical teratoid rhabdoid tumor in adulthood. Clin Neuropathol 2014; 33 (3) 245-250
    CrossrefPubMedSearch in Google Scholar
  • 3 Al-Hussaini M, Dissi N, Al-Jumaily U, Swaidan M. Atypical teratoid rhabdoid tumor in childhood, 15 cases of a single institute experience. Turk Patoloji Derg 2014; 30 (1) 43-54
    PubMedSearch in Google Scholar
  • 4 Ostrom QT, Gittleman H, Farah P , et al. CBTRUS statistical report: primary brain and central nervous system tumors diagnosed in the United States in 2006–2010. Neuro-oncol 2013; 15 (Suppl. 02) ii1-ii56
    PubMedSearch in Google Scholar
  • 5 Hilden JM, Meerbaum S, Burger P , et al. Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry. J Clin Oncol 2004; 22 (14) 2877-2884
    CrossrefPubMedSearch in Google Scholar
  • 6 Spiegler BJ, Kennedy K, Maze R , et al. Comparison of long-term neurocognitive outcomes in young children with acute lymphoblastic leukemia treated with cranial radiation or high-dose or very high-dose intravenous methotrexate. J Clin Oncol 2006; 24 (24) 3858-3864
    CrossrefPubMedSearch in Google Scholar
  • 7 Ostrom QT, Chen Y, de Blank PM , et al. The descriptive epidemiology of atypical teratoid/rhabdoid tumors in the United States, 2001-2010. Neuro-oncol 2014; 16 (10) 1392-1399
    PubMedSearch in Google Scholar
  • 8 Rorke LB, Packer RJ, Biegel JA. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood: definition of an entity. J Neurosurg 1996; 85 (1) 56-65
    CrossrefPubMedSearch in Google Scholar
  • 9 Kleihues P, Cavenee W. Tumours of the Nervous System: World Health Organization Classification of Tumours. Lyon, France: IARC Press; 2000
    Search in Google Scholar
  • 10 Wick MR, Ritter JH, Dehner LP. Malignant rhabdoid tumors: a clinicopathologic review and conceptual discussion. Semin Diagn Pathol 1995; 12 (3) 233-248
    PubMedSearch in Google Scholar
  • 11 Lee HY, Yoon CS, Sevenet N, Rajalingam V, Delattre O, Walford NQ. Rhabdoid tumor of the kidney is a component of the rhabdoid predisposition syndrome. Pediatr Dev Pathol 2002; 5 (4) 395-399
    CrossrefPubMedSearch in Google Scholar
  • 12 Caeiro C, Augusto I, Jaraquemada T, Sarmento C, Damasceno M. Tumor teratóide rabdóide atípico num adulto. Caso clínico e revisão da literatura. Arquivos de Medicina 2008; 22 (6) 165-167
    PubMedSearch in Google Scholar
  • 13 Bhattacharjee M, Hicks J, Langford L , et al. Central nervous system atypical teratoid/rhabdoid tumors of infancy and childhood. Ultrastruct Pathol 1997; 21 (4) 369-378
    CrossrefPubMedSearch in Google Scholar
  • 14 Burger PC, Yu IT, Tihan T , et al. Atypical teratoid/rhabdoid tumor of the central nervous system: a highly malignant tumor of infancy and childhood frequently mistaken for medulloblastoma: a Pediatric Oncology Group study. Am J Surg Pathol 1998; 22 (9) 1083-1092
    CrossrefPubMedSearch in Google Scholar
  • 15 Cheng YC, Lirng JF, Chang FC , et al. Neuroradiological findings in atypical teratoid/rhabdoid tumor of the central nervous system. Acta Radiol 2005; 46 (1) 89-96
    CrossrefPubMedSearch in Google Scholar
  • 16 Bambakidis NC, Robinson S, Cohen M, Cohen AR. Atypical teratoid/rhabdoid tumors of the central nervous system: clinical, radiographic and pathologic features. Pediatr Neurosurg 2002; 37 (2) 64-70
    CrossrefPubMedSearch in Google Scholar
  • 17 Yoon CS, Chuang S, Jay V. Primary malignant rhabdoid tumor of the brain: CT and MR findings. Yonsei Med J 2000; 41 (1) 8-16
    CrossrefPubMedSearch in Google Scholar
  • 18 Hanna SL, Langston JW, Parham DM, Douglass EC. Primary malignant rhabdoid tumor of the brain: clinical, imaging, and pathologic findings. AJNR Am J Neuroradiol 1993; 14 (1) 107-115
    PubMedSearch in Google Scholar
  • 19 Evans A, Ganatra R, Morris SJ. Imaging features of primary malignant rhabdoid tumour of the brain. Pediatr Radiol 2001; 31 (9) 631-633
    CrossrefPubMedSearch in Google Scholar
  • 20 Howlett DC, King AP, Jarosz JM , et al. Imaging and pathological features of primary malignant rhabdoid tumours of the brain and spine. Neuroradiology 1997; 39 (10) 719-723
    CrossrefPubMedSearch in Google Scholar
  • 21 Tez S, Köktener A, Güler G, Ozişik P. Atypical teratoid/rhabdoid tumors: imaging findings of two cases and review of the literature. Turk Neurosurg 2008; 18 (1) 30-34
    PubMedSearch in Google Scholar