Biomaterial Database

[A malignant rhabdoid tumor appearing simultaneously in the kidney and the brain of an infant: case report]. 1997

A Morizane, and I Nakahara, and J A Takahashi, and M Ishikawa, and H Kikuchi

Department of Neurosurgery, Faculty of Medicine, Kyoto University.

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, 1.5 months after surgery, revealed a recurrence of the kidney tumor and metastasis to the chest wall and lung. The patient received 16.9 Gy 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 keratin 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.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D007680	Kidney Neoplasms	Tumors or cancers of the KIDNEY.	Cancer of Kidney,Kidney Cancer,Renal Cancer,Cancer of the Kidney,Neoplasms, Kidney,Renal Neoplasms,Cancer, Kidney,Cancer, Renal,Cancers, Kidney,Cancers, Renal,Kidney Cancers,Kidney Neoplasm,Neoplasm, Kidney,Neoplasm, Renal,Neoplasms, Renal,Renal Cancers,Renal Neoplasm
D008279	Magnetic Resonance Imaging	Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques.	Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D009378	Neoplasms, Multiple Primary	Two or more abnormal growths of tissue occurring simultaneously and presumed to be of separate origin. The neoplasms may be histologically the same or different, and may be found in the same or different sites.	Neoplasms, Synchronous,Neoplasms, Synchronous Multiple Primary,Multiple Primary Neoplasms,Multiple Primary Neoplasms, Synchronous,Synchronous Multiple Primary Neoplasms,Synchronous Neoplasms,Multiple Primary Neoplasm,Neoplasm, Multiple Primary,Neoplasm, Synchronous,Primary Neoplasm, Multiple,Primary Neoplasms, Multiple,Synchronous Neoplasm
D002528	Cerebellar Neoplasms	Primary or metastatic neoplasms of the CEREBELLUM. Tumors in this location frequently present with ATAXIA or signs of INTRACRANIAL HYPERTENSION due to obstruction of the fourth ventricle. Common primary cerebellar tumors include fibrillary ASTROCYTOMA and cerebellar HEMANGIOBLASTOMA. The cerebellum is a relatively common site for tumor metastases from the lung, breast, and other distant organs. (From Okazaki & Scheithauer, Atlas of Neuropathology, 1988, p86 and p141)	Benign Cerebellar Neoplasms,Cerebellar Cancer,Malignant Cerebellar Neoplasms,Cerebellar Neoplasms, Benign,Cerebellar Neoplasms, Malignant,Cerebellar Neoplasms, Primary,Cerebellar Tumors,Neoplasms, Cerebellar,Neoplasms, Cerebellar, Benign,Neoplasms, Cerebellar, Malignant,Neoplasms, Cerebellar, Primary,Primary Neoplasms, Cerebellum,Benign Cerebellar Neoplasm,Cancer, Cerebellar,Cerebellar Cancers,Cerebellar Neoplasm,Cerebellar Neoplasm, Benign,Cerebellar Neoplasm, Malignant,Cerebellar Neoplasm, Primary,Cerebellar Tumor,Cerebellum Primary Neoplasm,Cerebellum Primary Neoplasms,Malignant Cerebellar Neoplasm,Neoplasm, Benign Cerebellar,Neoplasm, Cerebellar,Neoplasm, Cerebellum Primary,Neoplasm, Malignant Cerebellar,Primary Cerebellar Neoplasm,Primary Cerebellar Neoplasms,Primary Neoplasm, Cerebellum,Tumor, Cerebellar
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014057	Tomography, X-Ray Computed	Tomography using x-ray transmission and a computer algorithm to reconstruct the image.	CAT Scan, X-Ray,CT Scan, X-Ray,Cine-CT,Computerized Tomography, X-Ray,Electron Beam Computed Tomography,Tomodensitometry,Tomography, Transmission Computed,X-Ray Tomography, Computed,CAT Scan, X Ray,CT X Ray,Computed Tomography, X-Ray,Computed X Ray Tomography,Computerized Tomography, X Ray,Electron Beam Tomography,Tomography, X Ray Computed,Tomography, X-Ray Computer Assisted,Tomography, X-Ray Computerized,Tomography, X-Ray Computerized Axial,Tomography, Xray Computed,X Ray Computerized Tomography,X Ray Tomography, Computed,X-Ray Computer Assisted Tomography,X-Ray Computerized Axial Tomography,Beam Tomography, Electron,CAT Scans, X-Ray,CT Scan, X Ray,CT Scans, X-Ray,CT X Rays,Cine CT,Computed Tomography, Transmission,Computed Tomography, X Ray,Computed Tomography, Xray,Computed X-Ray Tomography,Scan, X-Ray CAT,Scan, X-Ray CT,Scans, X-Ray CAT,Scans, X-Ray CT,Tomographies, Computed X-Ray,Tomography, Computed X-Ray,Tomography, Electron Beam,Tomography, X Ray Computer Assisted,Tomography, X Ray Computerized,Tomography, X Ray Computerized Axial,Transmission Computed Tomography,X Ray Computer Assisted Tomography,X Ray Computerized Axial Tomography,X Ray, CT,X Rays, CT,X-Ray CAT Scan,X-Ray CAT Scans,X-Ray CT Scan,X-Ray CT Scans,X-Ray Computed Tomography,X-Ray Computerized Tomography,Xray Computed Tomography
D018335	Rhabdoid Tumor	A rare but highly lethal childhood tumor found almost exclusively in infants. Histopathologically, it resembles RHABDOMYOSARCOMA but the tumor cells are not of myogenic origin. Although it arises primarily in the kidney, it may be found in other parts of the body. The rhabdoid cytomorphology is believed to be the expression of a very primitive malignant cell. (From Holland et al., Cancer Medicine, 3d ed, p2210)	Rhabdoid Tumors,Tumor, Rhabdoid,Tumors, Rhabdoid