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.
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.
Subject(s)
Humans , Female , Adolescent , Teratoma/diagnosis , Teratoma/therapy , Brain Neoplasms/physiopathology , Rhabdoid Tumor/diagnosis , Rhabdoid Tumor/therapyABSTRACT
Primary rhabdoid tumor of lung is a rare histological and clinical entity. Lung tumors with rhabdoid features have been included as variants of large-cell carcinoma in the 1999 World Health Organization (WHO) classification of lung tumors. A large-cell carcinoma with a rhabdoid phenotype (LCCRP) is unusual, with only 38 cases reported till date. We report the clinical details of one such case that was treated with pneumonectomy and adjuvant chemotherapy. We also present a review of the literature. To identify relevant articles, we searched PubMed, Ovid, and IngentaConnect databases using the key words 'rhabdoid,' 'lung cancer,' and 'primary rhabdoid tumor of lung.'
Subject(s)
Adult , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Combined Modality Therapy , Female , Humans , Immunohistochemistry , Liver Neoplasms/pathology , Liver Neoplasms/secondary , Liver Neoplasms/therapy , Lung Neoplasms/metabolism , Lung Neoplasms/pathology , Lung Neoplasms/therapy , Lymphatic Metastasis/pathology , Pneumonectomy , Rhabdoid Tumor/metabolism , Rhabdoid Tumor/secondary , Rhabdoid Tumor/therapyABSTRACT
Rhabdoid tumors of the brain are rare with an invariable dismal prognosis despite treatment. This is the case of a 3 year old boy who presented lethargy, somnolence, nausea, vomiting, and headaches one week prior to hospitalization. A posterior fossa tumor with hydrocephalus was noted on a head computed tomography (CT) scan. A ventriculoperitoneal shunt was placed with subsequent gross total tumor resection. Pathology findings were those of a rhabdoid tumor. The histopathology, immunohistochemistry and ultrastructure of this unusual pediatric cerebral neoplasia is discussed.
Subject(s)
Humans , Male , Child, Preschool , Brain Neoplasms/pathology , Rhabdoid Tumor/pathology , Immunohistochemistry , Brain Neoplasms , Brain Neoplasms/therapy , Neurosurgical Procedures/methods , Tomography, X-Ray Computed , Treatment Outcome , Rhabdoid Tumor , Rhabdoid Tumor/therapyABSTRACT
A Malignant rhabdoid tumor (MRT) arising in the right temporoparietal lobe of a 9-year-old boy is described along with the results of an immunohistochemical study. The patient initially sought medical attention for a ptosis and right sided headache. The child underwent a subtotal resection of the tumor, followed by radiotherapy and systemic chemotherapy, but died three years after surgery. A MRT, a primary neoplasm of the central nervous system (CNS), is an entity of unknown histogenesis with a dismal prognosis, which only occurs in early childhood. Histologically similar tumors with more varied morphological features have been designated as an atypical teratoid/rhabdoid tumor. However, a classical MRT is extremely rare in the CNS and our case represents a classical CNS MRT.