A 6-month-old female was admitted to the hospital with bad temper and decreased sucking power. CT scans revealed tumors in her right kidney and left cerebellum. The patient underwent right radical nephrectomy to excise the kidney tumor. The pathological diagnosis was malignant rhabdoid tumor (MRT). Seven days later, the patient underwent left suboccipital craniectomy for total excision of the cerebellar tumor. The cerebellar tumor existed extraaxially, and consisted of a solid mass lesion and a cystic lesion. Histological examination revealed that it was also a malignant rhabdoid tumor. A follow-up CT, L5 months after surgery, revealed a recurrence of the kidney tumor and metastasis to the chest wall and lung. The patient received 16.9Gy radiotherapy to the abdominal tumor and chemotherapy with etoposide, carboplatin, and ifosfamide. However, she died of respiratory insufficiency 4 months after surgery, though neither recurrence nor metastasis was found in the brain. Nor was there evidence of leptomeningeal dissemination. MRT is a highly malignant tumor that occurs most frequently in the kidney. However, it can also occur in other tissues, including the brain. This tumor occurs most commonly in children under 2 years of age. There is a 3:2 male predominance. The median length of overall survival of MRT in the brain is 6 months. MRT contains nests or sheets of rhabdoid cells. A typical rhabdoid cell has an eccentric round nucleus with a prominent nucleolus and a plump cell body. MRT is composed entirely or partly of rhabdoid cells. Many MRTs have other components, such as PNET areas, mesenchymal area, and epithelial areas. For this reason, they are sometimes called atypical teratoid/rhabdoid tumors. MRTs in the brain contain fewer rhabdoid cell areas than MRT in the kidney. This makes diagnosing MRT in the brain more difficult. A careful search of the entire specimen for variations in pattern and cell type, along with application of immunohistochemical methods is the most useful method of obtaining a diagnosis. In our case, the cerebellar tumor consisted of rhabdoid cell areas, mesenchymal areas, and PNET areas. The cerebellar tumor contained fewer rhabdoid cell areas than the kidney tumor. However, the rhabdoid cell areas in the cerebellar tumor were almost the same as those in the kidney tumor. Furthermore, immunohistochemical staining was positive for vimentin and keratinin in the rhabdoid cell areas. Therefore, we were able to make a diagnosis of MRT. It is possible that some of the previously reported cases diagnosed as CNS PNET were actually MRT in the brain, especially if the cases were associated with MRT in the kidney.
|Number of pages||5|
|Publication status||Published - 01-07-1997|
All Science Journal Classification (ASJC) codes
- Clinical Neurology