心膜に発生した線維形成性小円形細胞腫瘍の1例
線維形成性小円形細胞腫瘍（desmoplastic small round cell tumor：DSRCT）は若年男性の腹腔に好発する希少な高悪性度腫瘍である．今回DSRCTの心膜発生例を経験したので報告する．症例38歳，女性．心タンポナーデによるうっ血性心不全に対し，前医で心囊ドレナージを施行された．心囊液は血性で細胞診にて異型細胞が検出されたため，精査目的に当院紹介となった．CTでは心膜の肥厚と多量の心囊液を認めたが，心膜以外に原発巣を疑う病変は確認されなかった．組織採取と心囊液ドレナージを目的に，胸腔鏡下心囊開窓術を施行した．病理組織学的検討の結果，DSRCTの診断に至った．診断後5年...
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| Published in | 日本呼吸器外科学会雑誌 Vol. 36; no. 5; pp. 590 - 595 |
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| Main Authors | , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
特定非営利活動法人 日本呼吸器外科学会
15.07.2022
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0919-0945 1881-4158 |
| DOI | 10.2995/jacsurg.36.590 |
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| Abstract | 線維形成性小円形細胞腫瘍（desmoplastic small round cell tumor：DSRCT）は若年男性の腹腔に好発する希少な高悪性度腫瘍である．今回DSRCTの心膜発生例を経験したので報告する．症例38歳，女性．心タンポナーデによるうっ血性心不全に対し，前医で心囊ドレナージを施行された．心囊液は血性で細胞診にて異型細胞が検出されたため，精査目的に当院紹介となった．CTでは心膜の肥厚と多量の心囊液を認めたが，心膜以外に原発巣を疑う病変は確認されなかった．組織採取と心囊液ドレナージを目的に，胸腔鏡下心囊開窓術を施行した．病理組織学的検討の結果，DSRCTの診断に至った．診断後5年経過しているが，遠隔転移は認めていない．DSRCTは腹腔外臓器にも発生し得るが，本邦における心膜発生の報告は本症例が初めてである．DSRCTの中には本症例のように，非典型例も存在する． |
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| AbstractList | 線維形成性小円形細胞腫瘍（desmoplastic small round cell tumor：DSRCT）は若年男性の腹腔に好発する希少な高悪性度腫瘍である．今回DSRCTの心膜発生例を経験したので報告する．症例38歳，女性．心タンポナーデによるうっ血性心不全に対し，前医で心囊ドレナージを施行された．心囊液は血性で細胞診にて異型細胞が検出されたため，精査目的に当院紹介となった．CTでは心膜の肥厚と多量の心囊液を認めたが，心膜以外に原発巣を疑う病変は確認されなかった．組織採取と心囊液ドレナージを目的に，胸腔鏡下心囊開窓術を施行した．病理組織学的検討の結果，DSRCTの診断に至った．診断後5年経過しているが，遠隔転移は認めていない．DSRCTは腹腔外臓器にも発生し得るが，本邦における心膜発生の報告は本症例が初めてである．DSRCTの中には本症例のように，非典型例も存在する． |
| Author | 大貫, 雄一郎 中島, 博之 武藤, 護 笹沼, 玄信 小泉, 竜之介 松原, 寛知 |
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| References | 12. Honoré C, Delhorme JB, Nassif E, Faron M, Ferron G, Bompas E, et al. Can we cure patients with abdominal Desmoplastic Small Round Cell Tumor? Results of a retrospective multicentric study on 100 patients. Surg Oncol 2019; 29: 107-12. 2. Gerald WL, Rosai J. Case 2. Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol 1989; 9: 177-83. 8. Ladanyi M, Gerald W. Fusion of the EWS and WT1 genes in the desmoplastic small round cell tumor. Cancer Res 1994; 54: 2837-40. 4. Gerald WL, Ladanyi M, de Alava E, Cuatrecasas M, Kushner BH, LaQuaglia MP, et al. Clinical, pathologic, and molecular spectrum of tumors associated with t (11;22) (p13;q12): desmoplastic small round-cell tumor and its variants. J Clin Oncol 1998; 16: 3028-36. 7. Sridevi KH, Awasthy N, Singh V, Rana S, Sharma R. Infantile pericardial round cell tumor. Ann Pediatr Cardiol 2015; 8: 164-6. 5. Hamazaki M, Okita H, Hata J, Shimizu S, Kobayashi H, Aoki K, et al. Desmoplastic small cell tumor of soft tissue: molecular variant of EWS-WT1 chimeric fusion. Pathol Int 2006; 56: 543-8. 1. Honoré C, Amroun K, Vilcot L, Mir O, Domont J, Terrier P, et al. Abdominal desmoplastic small round cell tumor: multimodal treatment combining chemotherapy, surgery, and radiotherapy is the best option. Ann Surg Oncol 2015; 22: 1073-9. 13. López F, Costales M, Vivanco B, Fresno MF, Suárez C, Llorente JL. Sinonasal desmoplastic small round cell tumor. Auris Nasus Larynx 2013; 40: 573-6. 9. Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP. Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg 2005; 40: 251-5. 10. Saab R, Khoury JD, Krasin M, Davidoff AM, Navid F. Desmoplastic small round cell tumor in childhood: the St. Jude Children's Research Hospital experience. Pediatr Blood Cancer 2007; 49: 274-9. 3. Subbiah V, Lamhamedi-Cherradi SE, Cuglievan B, Menegaz BA, Camacho P, Huh W, et al. Multimodality Treatment of Desmoplastic Small Round Cell Tumor: Chemotherapy and Complete Cytoreductive Surgery Improve Patient Survival. Clin Cancer Res 2018; 24: 4865-73. 6. Fois AG, Pirina P, Arcadu A, Becciu F, Manca S, Marras V, et al. Desmoplastic small round cell tumors of the pleura: a review of the clinical literature. Multidiscip Respir Med 2017; 12: 22. 11. Wong HH, Hatcher HM, Benson C, Al-Muderis O, Horan G, Fisher C, et al. Desmoplastic small round cell tumour: characteristics and prognostic factors of 41 patients and review of the literature. Clin Sarcoma Res 2013; 3: 14. 15. Asadbeigi SN, Zhang L, Linos K. Subcutaneous desmoplastic small round-cell tumor: An unusual primary location expanding the differential of superficial round-cell tumors. J Cutan Pathol 2020; 47: 768-75. 14. Hatanaka KC, Takakuwa E, Hatanaka Y, Suzuki A, IIzuka S, Tsushima N, et al. Desmoplastic small round cell tumor of the parotid gland-report of a rare case and a review of the literature. Diagn Pathol 2019; 14: 43. |
| References_xml | – reference: 10. Saab R, Khoury JD, Krasin M, Davidoff AM, Navid F. Desmoplastic small round cell tumor in childhood: the St. Jude Children's Research Hospital experience. Pediatr Blood Cancer 2007; 49: 274-9. – reference: 1. Honoré C, Amroun K, Vilcot L, Mir O, Domont J, Terrier P, et al. Abdominal desmoplastic small round cell tumor: multimodal treatment combining chemotherapy, surgery, and radiotherapy is the best option. Ann Surg Oncol 2015; 22: 1073-9. – reference: 5. Hamazaki M, Okita H, Hata J, Shimizu S, Kobayashi H, Aoki K, et al. Desmoplastic small cell tumor of soft tissue: molecular variant of EWS-WT1 chimeric fusion. Pathol Int 2006; 56: 543-8. – reference: 3. Subbiah V, Lamhamedi-Cherradi SE, Cuglievan B, Menegaz BA, Camacho P, Huh W, et al. Multimodality Treatment of Desmoplastic Small Round Cell Tumor: Chemotherapy and Complete Cytoreductive Surgery Improve Patient Survival. Clin Cancer Res 2018; 24: 4865-73. – reference: 2. Gerald WL, Rosai J. Case 2. Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol 1989; 9: 177-83. – reference: 6. Fois AG, Pirina P, Arcadu A, Becciu F, Manca S, Marras V, et al. Desmoplastic small round cell tumors of the pleura: a review of the clinical literature. Multidiscip Respir Med 2017; 12: 22. – reference: 12. Honoré C, Delhorme JB, Nassif E, Faron M, Ferron G, Bompas E, et al. Can we cure patients with abdominal Desmoplastic Small Round Cell Tumor? Results of a retrospective multicentric study on 100 patients. Surg Oncol 2019; 29: 107-12. – reference: 7. Sridevi KH, Awasthy N, Singh V, Rana S, Sharma R. Infantile pericardial round cell tumor. Ann Pediatr Cardiol 2015; 8: 164-6. – reference: 14. Hatanaka KC, Takakuwa E, Hatanaka Y, Suzuki A, IIzuka S, Tsushima N, et al. Desmoplastic small round cell tumor of the parotid gland-report of a rare case and a review of the literature. Diagn Pathol 2019; 14: 43. – reference: 15. Asadbeigi SN, Zhang L, Linos K. Subcutaneous desmoplastic small round-cell tumor: An unusual primary location expanding the differential of superficial round-cell tumors. J Cutan Pathol 2020; 47: 768-75. – reference: 8. Ladanyi M, Gerald W. Fusion of the EWS and WT1 genes in the desmoplastic small round cell tumor. Cancer Res 1994; 54: 2837-40. – reference: 9. Lal DR, Su WT, Wolden SL, Loh KC, Modak S, La Quaglia MP. Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg 2005; 40: 251-5. – reference: 13. López F, Costales M, Vivanco B, Fresno MF, Suárez C, Llorente JL. Sinonasal desmoplastic small round cell tumor. Auris Nasus Larynx 2013; 40: 573-6. – reference: 11. Wong HH, Hatcher HM, Benson C, Al-Muderis O, Horan G, Fisher C, et al. Desmoplastic small round cell tumour: characteristics and prognostic factors of 41 patients and review of the literature. Clin Sarcoma Res 2013; 3: 14. – reference: 4. Gerald WL, Ladanyi M, de Alava E, Cuatrecasas M, Kushner BH, LaQuaglia MP, et al. Clinical, pathologic, and molecular spectrum of tumors associated with t (11;22) (p13;q12): desmoplastic small round-cell tumor and its variants. J Clin Oncol 1998; 16: 3028-36. |
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| Title | 心膜に発生した線維形成性小円形細胞腫瘍の1例 |
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