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Rapidly Progressive Malignant Rhabdoid Tumor of the Omentum in an Adult. A Case Report and Review of the Literature

Caroline E. Moore, Pasha Bentley, Jula Veerapong, Diego A. Vicente, Teresa Cox, Heather Tracy


Introduction: Malignant Rhabdoid Tumors (MRT) are exceedingly rare neoplasms that characteristically occur in infants and children and sparsely develop in adults. Primary MRTs of the omentum are particularly rare.

Presentation of Case: A previously healthy 24-year-old male who presented with progressive abdominal distention and nausea. Further investigations revealed ascites with multifocal omental-based tumors. Biopsy with immunohistochemistry demonstrated dually positive cells for cytokeratin and vimentin and loss of Integrase Interactor 1 (INI1), and Next generation sequencing showed a copy number loss of SMARCB1 which established the diagnosis of malignant rhabdoid tumor. The patient’s clinical course was characterized by rapid local and metastatic progression with subsequent clinical deterioration, and he expired within three weeks of his initial presentation.

Conclusion: Herein we describe the clinical course, difficulty with diagnosis, and paucity of treatment options for a rare and very aggressive malignant tumor. The ideal treatment regimen for MRTs has yet to be elucidated and additional studies are required to discern therapies that offer benefit.


SMARCB1/INI; malignant rhabdoid tumor; omentum; oncology

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Beckwith JB, Palmer NF. Histopathology and prognosis of Wilms tumors: results from the First National Wilms' Tumor Study. Cancer. 1978, 41(5):1937-48

D'Amico F, Bertacco A, Cesari M, et al. Extraordinary disease-free survival in a rare malignant extrarenal rhabdoid tumor: a case report and review of the literature. J Med Case Rep. 2018, 12(1):39

Brennan B, Stiller C, Bourdeaut F. Extracranial rhabdoid tumours: what we have learned so far and future directions. Lancet Oncol. 2013, 14(8):e329-e336

Pathak P, Nautiyal M, Sachan PK, Shirazi N. Omental rhabdomyosarcoma (primary rhabdoid tumor of greater omentum): a rare case report. Surg Case Rep. 2015, 1(1):73.

Hong Qi Peng, Albert E. Stanek, Saul Teichberg, Barry Shepard, and Ellen Kahn.Malignant Rhabdoid Tumor of the Kidney in an Adult: A Case Report and Review of the Literature. Archives of Pathology & Laboratory Medicine: September 2003, Vol. 127, No. 9, pp. e371-e373

Geller JI, Roth JJ, Biegel JA. Biology and Treatment of Rhabdoid Tumor. Crit Rev Oncog. 2015, 20(3-4):199-216

Nam SH, Park JA, Kim YM. Primary malignant rhabdoid tumor of greater omentum in 10-year-old girl. Ann Surg Treat Res. 2014, 86(1):50-3.

Pancione M, Remo A, Sabatino L, et al. Right-sided rhabdoid colorectal tumors might be related to the serrated pathway. Diagn Pathol.2013, 8:31

Oda Y, Tsuneyoshi M. Extrarenal rhabdoid tumors of soft tissue: clinicopathological and molecular genetic review and distinction from other soft-tissue sarcomas with rhabdoid features. Pathol Int. 2006, 56:287-95

Horazdovsky R, Manivel JC, Cheng EY. Surgery and actinomycin improve survival in malignant rhabdoid tumor. Sarcoma. 2013, 2013:315170

Nagano H, Izumi T, Kawahara E, Oyama T, Goi T. SMARCB1- and vimentin-positive esophageal carcinoma with undifferentiated components, rhabdoid features, and a good prognosis: a case report. Surg Case Rep. 2019, 5(1):8

Hollmann TJ, Hornick JL. INI1-deficient tumors: Diagnostic features andmolecular genetics. Am J Surg Pathol. 2011, 35:e47-63.

Podduturi V, Campa-Thompson MM, Zhou XJ, Guileyardo JM. Malignant rhabdoid tumor of the kidney arising in an adult patient. Proc (BaylUniv Med Cent). 2014, 27(3):239-41

Horazdovsky R, Manivel JC, Cheng EY. Successful salvage and long-term survival after recurrent malignant rhabdoid tumor. Sarcoma. 2007, 2007:53549

Mazzocchi M, Chiummariello S, Bistoni G, Marchetti F, Alfano C. Extrarenal malignant rhabdoid tumour of the heel—a case report. Anticancer Research. 2005, 25(6 C):4573-4576


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