Christine Kögler, Bernhard W Ullrich, Klaus D Schaser, Alexander C Disch, Gunther O Hofmann, Felix Göhre, Christian Mawrin, Matthias Vogt
{"title":"胸椎罕见的原发性去分化脂肪肉瘤:病例报告和文献综述。","authors":"Christine Kögler, Bernhard W Ullrich, Klaus D Schaser, Alexander C Disch, Gunther O Hofmann, Felix Göhre, Christian Mawrin, Matthias Vogt","doi":"10.1016/j.bas.2024.103920","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Primary dedifferentiated liposarcomas of the spine mark a rare tumor entity.</p><p><strong>Research question and case description: </strong>We present a rare case of a primary dedifferentiated liposarcoma of the thoracic spine. A 36-year-old previously completely healthy woman presented with a sudden ascending paresthesia of both legs, persistently increasing over the course of two days before initial presentation.</p><p><strong>Case report: </strong>Computed tomography and magnetic resonance imaging revealed an expansively growing tumor mass extending from T5 to T6 and absolutely compressing the dural sac and spinal cord. The patient's neurological function completely recovered after emergency posterior decompression via laminectomy with intralesional tumor debulking. The tumor was histologically classified as primary grade 2 dedifferentiated liposarcoma (DDLPS) of the spine and after referral to a sarcoma center, the patient was treated with three courses of polychemotherapy (doxorubicin plus ifosfamide). Chemotherapy was followed by aggressive resection by en-bloc spondylectomy in cooperation with a spine tumor center. Subsequently, the patient also underwent radiation therapy.</p><p><strong>Results: </strong>The patient still undergoes structured tumor aftercare and is tumor- and metastasis-free 53 months after tumor resection.</p><p><strong>Discussion and conclusion: </strong>DDLPS rarely occur in the spine, with definitive resection of the tumor being the treatment of choice. Surgery should be accompanied by other (radio-) oncological treatment options in cases where only subtotal resection is possible. Also, referral of patients with primary sarcomas of the spine to specialized sarcoma centers is essential, so they can be provided with individual treatment options and structured interdisciplinary aftercare, that ensure the best possible outcome.</p>","PeriodicalId":72443,"journal":{"name":"Brain & spine","volume":"4 ","pages":"103920"},"PeriodicalIF":1.9000,"publicationDate":"2024-10-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11570891/pdf/","citationCount":"0","resultStr":"{\"title\":\"Rare primary dedifferentiated liposarcoma of the thoracic spine: A case report and literature review.\",\"authors\":\"Christine Kögler, Bernhard W Ullrich, Klaus D Schaser, Alexander C Disch, Gunther O Hofmann, Felix Göhre, Christian Mawrin, Matthias Vogt\",\"doi\":\"10.1016/j.bas.2024.103920\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>Primary dedifferentiated liposarcomas of the spine mark a rare tumor entity.</p><p><strong>Research question and case description: </strong>We present a rare case of a primary dedifferentiated liposarcoma of the thoracic spine. A 36-year-old previously completely healthy woman presented with a sudden ascending paresthesia of both legs, persistently increasing over the course of two days before initial presentation.</p><p><strong>Case report: </strong>Computed tomography and magnetic resonance imaging revealed an expansively growing tumor mass extending from T5 to T6 and absolutely compressing the dural sac and spinal cord. The patient's neurological function completely recovered after emergency posterior decompression via laminectomy with intralesional tumor debulking. The tumor was histologically classified as primary grade 2 dedifferentiated liposarcoma (DDLPS) of the spine and after referral to a sarcoma center, the patient was treated with three courses of polychemotherapy (doxorubicin plus ifosfamide). Chemotherapy was followed by aggressive resection by en-bloc spondylectomy in cooperation with a spine tumor center. Subsequently, the patient also underwent radiation therapy.</p><p><strong>Results: </strong>The patient still undergoes structured tumor aftercare and is tumor- and metastasis-free 53 months after tumor resection.</p><p><strong>Discussion and conclusion: </strong>DDLPS rarely occur in the spine, with definitive resection of the tumor being the treatment of choice. Surgery should be accompanied by other (radio-) oncological treatment options in cases where only subtotal resection is possible. Also, referral of patients with primary sarcomas of the spine to specialized sarcoma centers is essential, so they can be provided with individual treatment options and structured interdisciplinary aftercare, that ensure the best possible outcome.</p>\",\"PeriodicalId\":72443,\"journal\":{\"name\":\"Brain & spine\",\"volume\":\"4 \",\"pages\":\"103920\"},\"PeriodicalIF\":1.9000,\"publicationDate\":\"2024-10-23\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11570891/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Brain & spine\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1016/j.bas.2024.103920\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q3\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Brain & spine","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1016/j.bas.2024.103920","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/1/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
Rare primary dedifferentiated liposarcoma of the thoracic spine: A case report and literature review.
Introduction: Primary dedifferentiated liposarcomas of the spine mark a rare tumor entity.
Research question and case description: We present a rare case of a primary dedifferentiated liposarcoma of the thoracic spine. A 36-year-old previously completely healthy woman presented with a sudden ascending paresthesia of both legs, persistently increasing over the course of two days before initial presentation.
Case report: Computed tomography and magnetic resonance imaging revealed an expansively growing tumor mass extending from T5 to T6 and absolutely compressing the dural sac and spinal cord. The patient's neurological function completely recovered after emergency posterior decompression via laminectomy with intralesional tumor debulking. The tumor was histologically classified as primary grade 2 dedifferentiated liposarcoma (DDLPS) of the spine and after referral to a sarcoma center, the patient was treated with three courses of polychemotherapy (doxorubicin plus ifosfamide). Chemotherapy was followed by aggressive resection by en-bloc spondylectomy in cooperation with a spine tumor center. Subsequently, the patient also underwent radiation therapy.
Results: The patient still undergoes structured tumor aftercare and is tumor- and metastasis-free 53 months after tumor resection.
Discussion and conclusion: DDLPS rarely occur in the spine, with definitive resection of the tumor being the treatment of choice. Surgery should be accompanied by other (radio-) oncological treatment options in cases where only subtotal resection is possible. Also, referral of patients with primary sarcomas of the spine to specialized sarcoma centers is essential, so they can be provided with individual treatment options and structured interdisciplinary aftercare, that ensure the best possible outcome.