Debmalya Saha, Pawan K Singh, R. Sharma, S. Naqvi, Saket Aggarwal, M. Geelani
{"title":"胸腺源性巨大纵隔脂肪肉瘤1例:罕见的临床病例","authors":"Debmalya Saha, Pawan K Singh, R. Sharma, S. Naqvi, Saket Aggarwal, M. Geelani","doi":"10.4236/wjcs.2020.106010","DOIUrl":null,"url":null,"abstract":"Thymoliposarcoma is an exceedingly rare tumor of thymus with a very few \ncases reported till date. This case study presents a 45-year male with rare \ntype of thymoma. On the contrast-enhanced CT images, \nthere was a large mass lesion of predominantly fat attenuation in the \npre-vascular compartment of the mediastinum insinuating on both sides of the \nvisceral compartment of the mediastinum, and extending upto the bilateral \ncardio phrenic and anterior costophrenic angles, anterior to the right \nventricle with loss of fat plane with the pericardium, with few sub-centimetric \nlymph nodes in the right paratracheal and AP window and a calcified right hilar \nlymph node, suggestive of well-differentiated liposarcoma/thymoliposarcoma. Initial CT guided tru-cut tissue biopsy was inconclusive, \nand the repeat biopsy revealed as fibro-collagenous tissue with area of \nnecrosis, focal myxoid changes in the background with presence of cells which \nare spindle to oval in shape with mild nuclear pleomorphism and negative for \nS100, Cytokeratin, CD34, desmin. The entire tumor was resected en masse \nafter meticulous dissection without the support of cardiopulmonary bypass (CPB) \nwith an intact pericardium. Final histopathology report of the surgical biopsy \nspecimens is consistent with dedifferentiated thymoliposarcoma with focal \nganglionic cell differentiation. Postoperative follow-up \nCECT of thorax revealed no evidence of residual mass in the pre-vascular \ncompartment. The patient is disease-free and asymptomatic after 6-month and he is under routine \nfollow-up under Radiotherapy department since he received 30 Gy of postoperative radiotherapy \n(PORT).","PeriodicalId":23646,"journal":{"name":"World Journal of Cardiovascular Surgery","volume":"116 1","pages":"75-82"},"PeriodicalIF":0.0000,"publicationDate":"2020-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"A Case of Giant Mediastinal Liposarcoma of Thymic Origin: A Rare Clinical Entity\",\"authors\":\"Debmalya Saha, Pawan K Singh, R. Sharma, S. Naqvi, Saket Aggarwal, M. Geelani\",\"doi\":\"10.4236/wjcs.2020.106010\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Thymoliposarcoma is an exceedingly rare tumor of thymus with a very few \\ncases reported till date. This case study presents a 45-year male with rare \\ntype of thymoma. On the contrast-enhanced CT images, \\nthere was a large mass lesion of predominantly fat attenuation in the \\npre-vascular compartment of the mediastinum insinuating on both sides of the \\nvisceral compartment of the mediastinum, and extending upto the bilateral \\ncardio phrenic and anterior costophrenic angles, anterior to the right \\nventricle with loss of fat plane with the pericardium, with few sub-centimetric \\nlymph nodes in the right paratracheal and AP window and a calcified right hilar \\nlymph node, suggestive of well-differentiated liposarcoma/thymoliposarcoma. Initial CT guided tru-cut tissue biopsy was inconclusive, \\nand the repeat biopsy revealed as fibro-collagenous tissue with area of \\nnecrosis, focal myxoid changes in the background with presence of cells which \\nare spindle to oval in shape with mild nuclear pleomorphism and negative for \\nS100, Cytokeratin, CD34, desmin. The entire tumor was resected en masse \\nafter meticulous dissection without the support of cardiopulmonary bypass (CPB) \\nwith an intact pericardium. Final histopathology report of the surgical biopsy \\nspecimens is consistent with dedifferentiated thymoliposarcoma with focal \\nganglionic cell differentiation. Postoperative follow-up \\nCECT of thorax revealed no evidence of residual mass in the pre-vascular \\ncompartment. The patient is disease-free and asymptomatic after 6-month and he is under routine \\nfollow-up under Radiotherapy department since he received 30 Gy of postoperative radiotherapy \\n(PORT).\",\"PeriodicalId\":23646,\"journal\":{\"name\":\"World Journal of Cardiovascular Surgery\",\"volume\":\"116 1\",\"pages\":\"75-82\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2020-06-15\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"World Journal of Cardiovascular Surgery\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4236/wjcs.2020.106010\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"World Journal of Cardiovascular Surgery","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4236/wjcs.2020.106010","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
A Case of Giant Mediastinal Liposarcoma of Thymic Origin: A Rare Clinical Entity
Thymoliposarcoma is an exceedingly rare tumor of thymus with a very few
cases reported till date. This case study presents a 45-year male with rare
type of thymoma. On the contrast-enhanced CT images,
there was a large mass lesion of predominantly fat attenuation in the
pre-vascular compartment of the mediastinum insinuating on both sides of the
visceral compartment of the mediastinum, and extending upto the bilateral
cardio phrenic and anterior costophrenic angles, anterior to the right
ventricle with loss of fat plane with the pericardium, with few sub-centimetric
lymph nodes in the right paratracheal and AP window and a calcified right hilar
lymph node, suggestive of well-differentiated liposarcoma/thymoliposarcoma. Initial CT guided tru-cut tissue biopsy was inconclusive,
and the repeat biopsy revealed as fibro-collagenous tissue with area of
necrosis, focal myxoid changes in the background with presence of cells which
are spindle to oval in shape with mild nuclear pleomorphism and negative for
S100, Cytokeratin, CD34, desmin. The entire tumor was resected en masse
after meticulous dissection without the support of cardiopulmonary bypass (CPB)
with an intact pericardium. Final histopathology report of the surgical biopsy
specimens is consistent with dedifferentiated thymoliposarcoma with focal
ganglionic cell differentiation. Postoperative follow-up
CECT of thorax revealed no evidence of residual mass in the pre-vascular
compartment. The patient is disease-free and asymptomatic after 6-month and he is under routine
follow-up under Radiotherapy department since he received 30 Gy of postoperative radiotherapy
(PORT).