Tumor Teratóide Rabdóide Atípico Num Adulto

 

Cláudia Caeiro*, Isabel Augusto*, Teresa Jaraquemada*, Cristina Sarmento*, Margarida Damasceno*

*Serviço de Oncologia Médica, Hospital de São João, Porto

 

O tumor teratóide rabdóide atípico (TTRA) é uma neoplasia rara e agressiva, mais comum na infância e de mau prognóstico. Apenas 23 casos foram descritos em adultos. Permanece por estabelecer uma estratégia terapêutica, mas com ressecção macroscopicamente completa (RMC), radioterapia (RT) e quimioterapia baseada em platinos e agentes alquilantes parecem obter-se os melhores resultados. Descreve-se o caso de uma mulher de 39 anos que iniciou um quadro de cefaleias, vertigens, perturbações visuais e ataxia. A RMN mostrou hidrocefalia relacionada com uma massa na região pineal. O tratamento consistiu em RMC, RT e 3 ciclos do esquema ICE. A doente permanece sem evidência de doença 30 meses após o diagnóstico. A propósito deste caso, procedeu-se a uma revisão da literatura.

Palavras-chave: tumor teratóide rabdóide atípico; gene INI1; monossomia 22; quimioterapia.

 

 

Atypical Teratoid Rhabdoid Tumor in an Adult

Atypical teratoid rhabdoid tumor (AT/RT) is a rare and aggressive malignancy, which is more common in early childhood and carries a poor prognosis. Only 23 cases have been described in adults. A treatment strategy is yet to be established but gross total resection (GTR), early radiotherapy (RT) and platinum and alkylator-based chemotherapy seem to be associated with better outcome. We report on a 39 year-old female who presented with headache, vertigo, blurry vision and ataxia. The MRI revealed hydrocephalus related to a mass at the pineal region. The treatment consisted of GTR, RT and 3 cycles of an outpatient-based ICE regimen. The patient remains disease-free 30 months after diagnosis. Following this case, a review of the literature is undertaken.

Key-words: atypical teratoid rhabdoid tumor; INI1 gene; monosomy 22; chemotherapy.

 

 

Texto completo disponível apenas em PDF.

Full text only available in PDF format.

 

 

REFERÊNCIAS

1 -Hilden JM, Meerbaum S, Burger P, Finlay J, et al. Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry. J Clin Oncol 2004;22:2877-84.

2 -Tekautz TM, Fuller CE, Blaney S, Fouladi M, et al. Atypical teratoid/rhabdoid tumors (ATRT): improved survival in children 3 years of age and older with radiation therapy and high-dose alkylator-based chemotherapy. J Clin Oncol 2005;23:1491-99.

3 -Makuria AT, Rushing EJ, McGrail KM, Hartmann D, et al. Atypical teratoid rhabdoid tumor (AT/RT) in adults: review of four cases. J Neurooncol 2008;88:321-30.

4 -Meyers SP, Khademian ZP, BiegelJA, Chuang SH, Korones DN, Zimmerman RA. Primary intracranial atypical teratoid/ rhabdoid tumors of infancy and childhood: MRI features and patient outcomes. AJNR 2006;27:962-71.

5 -Judkins AR. Immunohistochemistry of INI1 expression: a new tool for old challenges in CNS and soft tissue pathology. Adv Anat Pathol 2007;14:335-39.

        [ Links ]

6 -Reddy AT. Atypical teratoid/rhabdoid tumors of the central nervous system. J Neurooncol 2005;75:309-13.

7 -Squire SE, Chan MD, Marcus KJ. Atypical teratoid/rhab-doid tumor: the controversy behind radiation therapy. J Neurooncol 2007;81:97-111.

8 -Erickson ML, Johnson R, Bannykh SI, Lotbiniere A, Kim JH. Malignant rhabdoid tumor in a pregnant adult female: literature review of centralnervous system rhabdoid tumors. J Neurooncol 2005;74:311-19.

9 -Hertzberg MS, Crombie C, Benson W, Taper J, Gottlieb D, Bradstock KF. Outpatient-based ifosfamide, carboplatine and etoposide (ICE) chemotherapy in transplant-eligible patients with non-Hodgkin’s lymphoma and Hodgkin’s disease. Ann Oncol 2003;14 suppl 1:i11-16.

10 - Zimmerman MA, Goumnerova LC, Proctor M, Scott RM, et al. Continuous remission of newly diagnosed and relapsed central nervous system atypical teratoid/rhabdoid tumor. J Neuroncol 2005;72:77-84.

 

 

Correspondência:

Drª. Cláudia Caeiro

Serviço de Oncologia Médica

Hospital de São João

Alameda Prof. Hernâni Monteiro

4200-319 Porto

e-mail: cbcaeiro@netcabo.pt