A rare case of Non Renal/ Non CNS malignant rhabdoid tumor originating from the omentum
Malignant Rhabdoid Tumor (MRT) is a rare and aggressive malignancy in pediatric patients. These tumors typically originate in the kidney and central nervous system (CNS). There are approximately 15 cases of non-renal/non-CNS MRT diagnosed each year in the United States, originating from the liver or...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Elsevier
2018-09-01
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| Series: | Journal of Pediatric Surgery Case Reports |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S221357661830109X |
| _version_ | 1818261404557246464 |
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| author | Kayle Stevenson William Sessions Vijay Linga Parth Bhatt Smita Bhaskaran Jason Nirgiotis Janet Meller |
| author_facet | Kayle Stevenson William Sessions Vijay Linga Parth Bhatt Smita Bhaskaran Jason Nirgiotis Janet Meller |
| author_sort | Kayle Stevenson |
| collection | DOAJ |
| description | Malignant Rhabdoid Tumor (MRT) is a rare and aggressive malignancy in pediatric patients. These tumors typically originate in the kidney and central nervous system (CNS). There are approximately 15 cases of non-renal/non-CNS MRT diagnosed each year in the United States, originating from the liver or other soft tissues. The development of MRT is associated with a deletion or inactivating mutation of the SMARCB1 tumor suppressor gene. The average age at diagnosis is 15 months. These tumors carry a poor prognosis due to advanced stage presentation and poor response to therapy. We present a case of a 9 year old male with symptoms suggestive of complicated appendicitis. Surgical exploration revealed the entire omentum studded with tumors and peritoneal carcinomatosis. Biopsy of the omentum and appendix confirmed the diagnosis of non-renal/non-CNS Malignant Rhabdoid Tumor. The omentum is often a site of metastasis for MRT, but is rarely a primary tumor site, as was suspected in this case. |
| first_indexed | 2024-12-12T18:46:42Z |
| format | Article |
| id | doaj.art-e646de6a2ee5400ba765a6f0d14daf15 |
| institution | Directory Open Access Journal |
| issn | 2213-5766 |
| language | English |
| last_indexed | 2024-12-12T18:46:42Z |
| publishDate | 2018-09-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Journal of Pediatric Surgery Case Reports |
| spelling | doaj.art-e646de6a2ee5400ba765a6f0d14daf152022-12-22T00:15:30ZengElsevierJournal of Pediatric Surgery Case Reports2213-57662018-09-01364043A rare case of Non Renal/ Non CNS malignant rhabdoid tumor originating from the omentumKayle Stevenson0William Sessions1Vijay Linga2Parth Bhatt3Smita Bhaskaran4Jason Nirgiotis5Janet Meller6Department of Pediatrics, Texas Tech University Health Sciences Center, 1400 S. Coulter St. Amarillo, Texas 79106, USADepartment of Pediatrics, Texas Tech University Health Sciences Center, 1400 S. Coulter St. Amarillo, Texas 79106, USADepartment of Pediatrics, Texas Tech University Health Sciences Center, 1400 S. Coulter St. Amarillo, Texas 79106, USADepartment of Pediatrics, Texas Tech University Health Sciences Center, 1400 S. Coulter St. Amarillo, Texas 79106, USADepartment of Pediatrics, Texas Tech University Health Sciences Center, 1400 S. Coulter St. Amarillo, Texas 79106, USADepartment of Pediatrics, Texas Tech University Health Sciences Center, 1400 S. Coulter St. Amarillo, Texas 79106, USACorresponding author. School of Medicine, Texas Tech University Health Sciences Center, 1400 S. Coulter St. Amarillo, TX 79106, USA.; Department of Pediatrics, Texas Tech University Health Sciences Center, 1400 S. Coulter St. Amarillo, Texas 79106, USAMalignant Rhabdoid Tumor (MRT) is a rare and aggressive malignancy in pediatric patients. These tumors typically originate in the kidney and central nervous system (CNS). There are approximately 15 cases of non-renal/non-CNS MRT diagnosed each year in the United States, originating from the liver or other soft tissues. The development of MRT is associated with a deletion or inactivating mutation of the SMARCB1 tumor suppressor gene. The average age at diagnosis is 15 months. These tumors carry a poor prognosis due to advanced stage presentation and poor response to therapy. We present a case of a 9 year old male with symptoms suggestive of complicated appendicitis. Surgical exploration revealed the entire omentum studded with tumors and peritoneal carcinomatosis. Biopsy of the omentum and appendix confirmed the diagnosis of non-renal/non-CNS Malignant Rhabdoid Tumor. The omentum is often a site of metastasis for MRT, but is rarely a primary tumor site, as was suspected in this case.http://www.sciencedirect.com/science/article/pii/S221357661830109X |
| spellingShingle | Kayle Stevenson William Sessions Vijay Linga Parth Bhatt Smita Bhaskaran Jason Nirgiotis Janet Meller A rare case of Non Renal/ Non CNS malignant rhabdoid tumor originating from the omentum Journal of Pediatric Surgery Case Reports |
| title | A rare case of Non Renal/ Non CNS malignant rhabdoid tumor originating from the omentum |
| title_full | A rare case of Non Renal/ Non CNS malignant rhabdoid tumor originating from the omentum |
| title_fullStr | A rare case of Non Renal/ Non CNS malignant rhabdoid tumor originating from the omentum |
| title_full_unstemmed | A rare case of Non Renal/ Non CNS malignant rhabdoid tumor originating from the omentum |
| title_short | A rare case of Non Renal/ Non CNS malignant rhabdoid tumor originating from the omentum |
| title_sort | rare case of non renal non cns malignant rhabdoid tumor originating from the omentum |
| url | http://www.sciencedirect.com/science/article/pii/S221357661830109X |
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