胎児性横紋筋腫型腎芽腫
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English Journal
- Immunohistochemical expression of glypican-3 in pediatric tumors: an analysis of 414 cases.
- Chan ES, Pawel BR, Corao DA, Venneti S, Russo P, Santi M, Sullivan LM.Author information Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.AbstractGlypican-3 (GPC3) is a proteoglycan thought to play an important role during development. Germline GPC3 mutations are seen in the rare Simpson-Golabi-Behmel syndrome (SGBS), which predisposes patients to Wilms tumor, hepatoblastoma, and neuroblastoma. While numerous adult tumors have been evaluated by immunohistochemistry for GPC3, no comprehensive assessment has been done in pediatric tumors. We therefore investigated GPC3 expression in 143 pediatric central nervous system (CNS) tumors and 271 non-CNS tumors. Among non-CNS tumors, GPC3 expression was seen in 9/9 (100%) hepatoblastomas, 4/6 (67%) malignant rhabdoid tumors, 5/13 (38%) Wilms tumors, 11/37 (30%) alveolar rhabdomyosarcomas, and 8/45 (18%) embryonal rhabdomyosarcomas. All 136 neuroblastomas, 14 Ewing sarcoma/primitive neuroectodermal tumors, and 11 synovial sarcomas were immunonegative for GPC3. Among CNS tumors, GPC3 had restricted expression, with positivity in 6/6 (100%) atypical teratoid rhabdoid tumors and 1/4 (25%) craniopharyngiomas. The remaining 136 CNS tumors-23 medulloblastomas, 21 pilocytic astrocytomas, 13 gangliogliomas, 12 ependymomas, 12 glioblastomas, 11 choroid plexus neoplasms, 10 diffuse astrocytomas (grade II/III), 10 meningiomas, 8 dysembryoplastic neuroepithelial tumors, 8 oligodendrogliomas, 3 craniopharyngiomas, 3 germinomas, and 2 neurocytomas-were entirely negative for GPC3. These results showed GPC3 positivity in a number of non-CNS tumors, with no consistent discrimination between tumors that were or were not associated with SGBS. Within the CNS, GPC3 positivity was limited to a small subset of CNS neoplasms and may thus serve as a useful positive diagnostic biomarker (P < 0.0001) in addition to negative INI1/BAF47/SMARCB1 staining to differentiate atypical teratoid rhabdoid tumors from other high-grade pediatric brain tumors.
- Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society.Pediatr Dev Pathol.2013 Jul-Aug;16(4):272-7. doi: 10.2350/12-06-1216-OA.1. Epub 2013 Mar 26.
- Glypican-3 (GPC3) is a proteoglycan thought to play an important role during development. Germline GPC3 mutations are seen in the rare Simpson-Golabi-Behmel syndrome (SGBS), which predisposes patients to Wilms tumor, hepatoblastoma, and neuroblastoma. While numerous adult tumors have been evaluated
- PMID 23530909
- Teratoid Wilms' tumor in a child: A report of a rare case.
- Sinha A, Phukan JP, Bandyopadhyay G, Mukherjee S.Author information Department of Pathology, Bankura Sammilani Medical College, Bankura, India.AbstractTeratoid Wilms' tumor is an unusual variant of nephroblastoma in which heterologous tissue predominates. We report a case of teratoid Wilms' tumor in a 2-year-old male. Right sided abdominal mass was the presenting complaint. Ultrasonography of the abdomen showed a mass in the right kidney. Histopathological examination revealed blastemal, epithelial, and mesenchymal components along with areas presenting heterologous elements. More than 75% predominance of squamous differentiation with the keratin pearl formation was observed. The patient underwent nephrectomy and was followed post-operatively for 1 year and was normal.
- International journal of applied & basic medical research.Int J Appl Basic Med Res.2013 Jan;3(1):72-4. doi: 10.4103/2229-516X.112248.
- Teratoid Wilms' tumor is an unusual variant of nephroblastoma in which heterologous tissue predominates. We report a case of teratoid Wilms' tumor in a 2-year-old male. Right sided abdominal mass was the presenting complaint. Ultrasonography of the abdomen showed a mass in the right kidney. Histopat
- PMID 23776845
- Familial synchronous bilateral teratoid Wilms tumor with elevated alpha-fetoprotein level.
- Okur A, Pinarli FG, Karadeniz C, Poyraz A, Fidan K, Basaklar C, Oguz A.Author information Department of Pediatric Oncology, Gazi University Medical Faculty, Ankara, Turkey. arzu-okur@hotmail.comAbstractFamilial Wilms tumor is a rare entity that accounts for only 1-2% of all Wilms tumor cases, with an earlier age of onset and an increased frequency of bilateral tumors. Teratoid Wilms tumor is a variant of nephroblastoma with a predominance of heterologous tissues comprising more than 50% of the tumor volume. Wilms tumor does not usually secrete any specific tumor marker and all teratoid Wilms tumor cases previously reported were sporadic non-secreting neoplasms. Here we describe an infant with familial synchronous bilateral teratoid Wilms tumor whose serum alpha-fetoprotein level was elevated. To our knowledge, this extremely rare type of case is reported for the first time in the literature.
- Tumori.Tumori.2012 Nov;98(6):179e-82e. doi: 10.1700/1217.13518.
- Familial Wilms tumor is a rare entity that accounts for only 1-2% of all Wilms tumor cases, with an earlier age of onset and an increased frequency of bilateral tumors. Teratoid Wilms tumor is a variant of nephroblastoma with a predominance of heterologous tissues comprising more than 50% of the tum
- PMID 23389381
Japanese Journal
- Stroma-predominant Wilms tumor with teratoid features : report of a rare case and review of the literature
- GUPTA Ruchika,SHARMA Alok,ARORA Raman,DINDA Amit Kumar
- Pediatric surgery international 25(3), 293-295, 2009-03-01
- NAID 10029304787
- Adult teratoid Wilms' tumor with prominent neuroepithelial differentiation
- SEO Jinwon,SUH Yeon-Lim,CHOI Han Yong
- Pathology international 59(1), 44-48, 2009-01-01
- NAID 10027378972
Related Links
- INTRODUCTION Variend et al. termed teratoid Wilms’ tumor in a nephroblastoma with significant heterologous components.[] Fernandes et al. defined teratoid Wilms’ tumor in which heterologous components like adipose ...
- 1. J Pediatr Urol. 2007 Aug;3(4):282-6. doi: 10.1016/j.jpurol.2006.11.004. Epub 2007 Jan 22. Teratoid Wilms' tumor, an important variant of nephroblastoma. Myers JB(1), Dall'Era J, Odom LF, McGavran L, Lovell MA, Furness P 3rd. ...
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- 英
- fetal rhabdomyomatous nephroblastoma
- 同
- 類奇形腎芽腫 teratoid nephroblastoma、類奇形ウィルムス腫瘍 teratoid Wilms tumor