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Extrarenal rhabdoid tumor

MedGen UID:
224918
•Concept ID:
C1304517
•
Neoplastic Process
Synonyms: Extrarenal Rhabdoid Tumor; extrarenal rhabdoid tumor; Malignant Extrarenal Rhabdoid Neoplasm; malignant extrarenal rhabdoid neoplasm; Rhabdoid Tumor of Soft Tissue; rhabdoid tumor of soft tissue; rhabdoid tumour of soft tissue
SNOMED CT: Extrarenal rhabdoid tumor (404089007)
 
Monarch Initiative: MONDO:0044916

Definition

A rhabdoid tumor which arises in the soft tissues. It occurs in infants and children and may be associated with loss of chromosome 22. It is characterized by the presence of cells with a large eccentric nucleus, prominent nucleolus, and abundant cytoplasm. [from NCI]

Professional guidelines

PubMed

Biology and Treatment of Rhabdoid Tumor.
Geller JI, Roth JJ, Biegel JA
Crit Rev Oncog 2015;20(3-4):199-216. doi: 10.1615/critrevoncog.2015013566. PMID: 26349416Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Molecularly confirmed primary malignant rhabdoid tumor of the urinary bladder: implications of accurate diagnosis.
Savage N, Linn D, McDonough C, Donohoe JM, Franco A, Reuter V, Biddinger PW, Eaton KW, Biegel JA, Sharma S
Ann Diagn Pathol 2012 Dec;16(6):504-7. Epub 2011 Jul 19 doi: 10.1016/j.anndiagpath.2011.04.008. PMID: 21775180Free PMC Article
Rare nerve lesions of non-nerve sheath origin: a 17-year retrospective series.
Strom T, Kleinschmidt-Demasters BK, Donson A, Foreman NK, Lillehei KO
Arch Pathol Lab Med 2009 Sep;133(9):1391-402. doi: 10.5858/133.9.1391. PMID: 19722745
An extrarenal rhabdoid tumor of the cervical spine with bony involvement.
Robbens C, Vanwyck R, Wilms G, Sciot R, Debiec-Rychter M
Skeletal Radiol 2007 Apr;36(4):341-5. Epub 2006 Apr 7 doi: 10.1007/s00256-006-0104-6. PMID: 16602014
Maxillary undifferentiated carcinoma with rhabdoid features.
Villarreal PM, Junquera LM, Herreros M, Ferreras J, Rodríguez O
Med Oral 2001 Jan-Feb;6(1):31-5. PMID: 11488128
The clinicopathologic spectrum of putative extrarenal rhabdoid tumors. An analysis of 42 cases studied with immunohistochemistry or electron microscopy.
Parham DM, Weeks DA, Beckwith JB
Am J Surg Pathol 1994 Oct;18(10):1010-29. doi: 10.1097/00000478-199410000-00005. PMID: 8092393

Diagnosis

Diplopia Related to a Pediatric Cervical Rhabdoid Tumor in 18 F-FDG PET.
Jacquet-Francillon N, Habouzit V, Besson L, Bonnefoy PB, Prevot N
Clin Nucl Med 2022 Nov 1;47(11):e689-e691. Epub 2022 Jul 26 doi: 10.1097/RLU.0000000000004327. PMID: 35867984
Diagnostically Challenging Epithelioid Soft Tissue Tumors.
James AW, Dry SM
Surg Pathol Clin 2015 Sep;8(3):309-29. doi: 10.1016/j.path.2015.05.002. PMID: 26297059
Rare nerve lesions of non-nerve sheath origin: a 17-year retrospective series.
Strom T, Kleinschmidt-Demasters BK, Donson A, Foreman NK, Lillehei KO
Arch Pathol Lab Med 2009 Sep;133(9):1391-402. doi: 10.5858/133.9.1391. PMID: 19722745
Novel oncogene amplifications in tumors from a family with Li-Fraumeni syndrome.
Rieber J, Remke M, Hartmann C, Korshunov A, Burkhardt B, Sturm D, Mechtersheimer G, Wittmann A, Greil J, Blattmann C, Witt O, Behnisch W, Halatsch ME, Orakcioglu B, von Deimling A, Lichter P, Kulozik A, Pfister S
Genes Chromosomes Cancer 2009 Jul;48(7):558-68. doi: 10.1002/gcc.20665. PMID: 19378321
Role of electron microscopy in the evaluation of soft tissue neoplasms, with emphasis on spindle cell and pleomorphic tumors.
Erlandson RA, Woodruff JM
Hum Pathol 1998 Dec;29(12):1372-81. doi: 10.1016/s0046-8177(98)90005-3. PMID: 9865822

Therapy

Malignant extrarenal rhabdoid tumour (MERT) with liver metastases as a rare cause of an esophageal tumor in a 57 years old patient.
Kaechele V, Vogelpohl J, Boeck W, Riecke A, Eisele R, Barth T
Z Gastroenterol 2015 Jul;53(7):660-3. Epub 2015 Jul 13 doi: 10.1055/s-0034-1399694. PMID: 26167696
An extrarenal rhabdoid tumor of the cervical spine with bony involvement.
Robbens C, Vanwyck R, Wilms G, Sciot R, Debiec-Rychter M
Skeletal Radiol 2007 Apr;36(4):341-5. Epub 2006 Apr 7 doi: 10.1007/s00256-006-0104-6. PMID: 16602014
Soft-tissue extrarenal rhabdoid tumor with a unique long-term survival.
Fabre A, Eyden B, Ali HH
Ultrastruct Pathol 2004 Jan-Feb;28(1):49-52. PMID: 14967599
Malignant rhabdoid tumor of the orbit.
Gündüz K, Shields JA, Eagle RC Jr, Shields CL, De Potter P, Klombers L
Arch Ophthalmol 1998 Feb;116(2):243-6. doi: 10.1001/archopht.116.2.243. PMID: 9488282
Adult extrarenal rhabdoid tumor of the lacrimal gland.
Niffenegger JH, Jakobiec FA, Shore JW, Albert DM
Ophthalmology 1992 Apr;99(4):567-74. doi: 10.1016/s0161-6420(92)31948-7. PMID: 1374874

Prognosis

Malignant Extrarenal Rhabdoid tumor derived from the greater omentum: A case report and literature review.
Shao X, Liu M, Wang X, Han L, Luo S
Cancer Rep (Hoboken) 2024 May;7(5):e2086. doi: 10.1002/cnr2.2086. PMID: 38767517Free PMC Article
Cytomorphological and immunohistochemical features of renal and extrarenal rhabdoid tumors.
D P, Sadasivan B, Patil Okaly GV, MukundaPai M, Alashetty S, B L K
Diagn Cytopathol 2021 Jun;49(6):711-717. Epub 2021 Feb 27 doi: 10.1002/dc.24727. PMID: 33638610
Ewing Sarcoma and Atypical Teratoid Rhabdoid Tumor: A FISH and Immunohistochemical Comparison.
Pacheco MC, Dolan M, Bendel A
Pediatr Dev Pathol 2017 Sep-Oct;20(5):381-386. Epub 2017 Apr 6 doi: 10.1177/1093526617698599. PMID: 28382842
Diagnostically Challenging Epithelioid Soft Tissue Tumors.
James AW, Dry SM
Surg Pathol Clin 2015 Sep;8(3):309-29. doi: 10.1016/j.path.2015.05.002. PMID: 26297059
Cytomorphology and immunohistochemistry of extrarenal rhabdoid tumor: a case report with review of literature.
Jain M, Harbhajanka A, Choudhary SR
Indian J Pathol Microbiol 2011 Oct-Dec;54(4):819-21. doi: 10.4103/0377-4929.91534. PMID: 22234121

Clinical prediction guides

Good response to chemotherapy spares irradiation for extrarenal rhabdoid tumor conferring better activities of daily living.
Asada N, Kato I, Daifu T, Umeda K, Hiramatsu H, Okamoto T, Toguchida J, Yamawaki S, Yoshikawa K, Adachi S, Heike T, Watanabe K
J Pediatr Hematol Oncol 2015 Jan;37(1):e57-9. doi: 10.1097/MPH.0000000000000150. PMID: 24663072
Molecularly confirmed primary malignant rhabdoid tumor of the urinary bladder: implications of accurate diagnosis.
Savage N, Linn D, McDonough C, Donohoe JM, Franco A, Reuter V, Biddinger PW, Eaton KW, Biegel JA, Sharma S
Ann Diagn Pathol 2012 Dec;16(6):504-7. Epub 2011 Jul 19 doi: 10.1016/j.anndiagpath.2011.04.008. PMID: 21775180Free PMC Article
Aggressive uterine sarcoma with rhabdoid features: diagnosis by peritoneal fluid cytology and absence of INI1 gene mutation.
Knapik J, Yachnis AT, Ripley D, Biegel JA, Rathor S, Hardt NS, Talerman A, Wilkinson EJ
Hum Pathol 2001 Aug;32(8):884-6. doi: 10.1053/hupa.2001.26476. PMID: 11521235

Supplemental Content

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    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

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    Reviews

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