Dedifferentiated Liposarcoma of the Retroperitoneum With Hyperleukocytosis as Paraneoplastic Syndrome: A Rare Presentation
Background: Paraneoplastic syndrome is a clinical syndrome with systemic effects, triggered by altered immune response to a neoplasm. Paraneoplastic syndrome was described mostly in association with epithelial malignancies, while in a soft tissue sarcoma it is unusual and hyperleucocytosis as a para...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Medknow Publications
2019-01-01
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| Series: | MAMC Journal of Medical Sciences |
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| Online Access: | http://www.mamcjms.in/article.asp?issn=2394-7438;year=2019;volume=5;issue=3;spage=139;epage=141;aulast=Mehta |
| _version_ | 1818247362068348928 |
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| author | Sangita S Mehta Rajeshwari K Muthusamy Firoz Rajan Shobhanaa P. S. |
| author_facet | Sangita S Mehta Rajeshwari K Muthusamy Firoz Rajan Shobhanaa P. S. |
| author_sort | Sangita S Mehta |
| collection | DOAJ |
| description | Background: Paraneoplastic syndrome is a clinical syndrome with systemic effects, triggered by altered immune response to a neoplasm. Paraneoplastic syndrome was described mostly in association with epithelial malignancies, while in a soft tissue sarcoma it is unusual and hyperleucocytosis as a paraneoplastic phenomenon is very rare. Case presentation: We report a middle aged male patient with bilateral retroperitoneal masses with histomorphology of myxoid liposarcoma with dedifferentiation. Hyper-leucocytosis in the peripheral blood and pronounced myeloid hyperplasia in marrow was found with no obvious focus of infection. There was gradual reduction in white cell count in the postoperative period and reached normal values on Day 8. Hence a diagnosis of dedifferentiated liposarcoma of the retroperitoneum with hyperleucocytosis as a paraneoplastic manifestation was made. Conclusion: Paraneoplastic manifestation could probably be due to production of G-CSF by tumor cells as the leucocytosis disappeared after tumor resection. Further, leucocytosis is not reported in association with well differentiated liposarcoma, and if happens to be so, it might be a clue to search for the dedifferentiated component. |
| first_indexed | 2024-12-12T15:03:30Z |
| format | Article |
| id | doaj.art-5b7d3498d09e4092b8c922fbf55ad895 |
| institution | Directory Open Access Journal |
| issn | 2394-7438 |
| language | English |
| last_indexed | 2024-12-12T15:03:30Z |
| publishDate | 2019-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | MAMC Journal of Medical Sciences |
| spelling | doaj.art-5b7d3498d09e4092b8c922fbf55ad8952022-12-22T00:20:46ZengWolters Kluwer Medknow PublicationsMAMC Journal of Medical Sciences2394-74382019-01-015313914110.4103/mamcjms.mamcjms_8_19Dedifferentiated Liposarcoma of the Retroperitoneum With Hyperleukocytosis as Paraneoplastic Syndrome: A Rare PresentationSangita S MehtaRajeshwari K MuthusamyFiroz RajanShobhanaa P. S.Background: Paraneoplastic syndrome is a clinical syndrome with systemic effects, triggered by altered immune response to a neoplasm. Paraneoplastic syndrome was described mostly in association with epithelial malignancies, while in a soft tissue sarcoma it is unusual and hyperleucocytosis as a paraneoplastic phenomenon is very rare. Case presentation: We report a middle aged male patient with bilateral retroperitoneal masses with histomorphology of myxoid liposarcoma with dedifferentiation. Hyper-leucocytosis in the peripheral blood and pronounced myeloid hyperplasia in marrow was found with no obvious focus of infection. There was gradual reduction in white cell count in the postoperative period and reached normal values on Day 8. Hence a diagnosis of dedifferentiated liposarcoma of the retroperitoneum with hyperleucocytosis as a paraneoplastic manifestation was made. Conclusion: Paraneoplastic manifestation could probably be due to production of G-CSF by tumor cells as the leucocytosis disappeared after tumor resection. Further, leucocytosis is not reported in association with well differentiated liposarcoma, and if happens to be so, it might be a clue to search for the dedifferentiated component.http://www.mamcjms.in/article.asp?issn=2394-7438;year=2019;volume=5;issue=3;spage=139;epage=141;aulast=Mehtadedifferentiatedhyperleukocytosisliposarcomaparaneoplasticretroperitoneum |
| spellingShingle | Sangita S Mehta Rajeshwari K Muthusamy Firoz Rajan Shobhanaa P. S. Dedifferentiated Liposarcoma of the Retroperitoneum With Hyperleukocytosis as Paraneoplastic Syndrome: A Rare Presentation MAMC Journal of Medical Sciences dedifferentiated hyperleukocytosis liposarcoma paraneoplastic retroperitoneum |
| title | Dedifferentiated Liposarcoma of the Retroperitoneum With Hyperleukocytosis as Paraneoplastic Syndrome: A Rare Presentation |
| title_full | Dedifferentiated Liposarcoma of the Retroperitoneum With Hyperleukocytosis as Paraneoplastic Syndrome: A Rare Presentation |
| title_fullStr | Dedifferentiated Liposarcoma of the Retroperitoneum With Hyperleukocytosis as Paraneoplastic Syndrome: A Rare Presentation |
| title_full_unstemmed | Dedifferentiated Liposarcoma of the Retroperitoneum With Hyperleukocytosis as Paraneoplastic Syndrome: A Rare Presentation |
| title_short | Dedifferentiated Liposarcoma of the Retroperitoneum With Hyperleukocytosis as Paraneoplastic Syndrome: A Rare Presentation |
| title_sort | dedifferentiated liposarcoma of the retroperitoneum with hyperleukocytosis as paraneoplastic syndrome a rare presentation |
| topic | dedifferentiated hyperleukocytosis liposarcoma paraneoplastic retroperitoneum |
| url | http://www.mamcjms.in/article.asp?issn=2394-7438;year=2019;volume=5;issue=3;spage=139;epage=141;aulast=Mehta |
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