今日の臨床サポート

縦隔腫瘍

著者: 花田豪郎 虎の門病院 呼吸器センター内科

監修: 高橋和久 順天堂大学大学院

著者校正/監修レビュー済:2021/01/07
患者向け説明資料

概要・推奨   

  1. 縦隔腫瘍の臨床において、縦隔の区分は、正確な存在診断を可能にする(推奨度1)
  1. 悪性リンパ腫など手術適応にならない疾患は過小評価されていることを考慮する(推奨度2)
  1. 正岡病期分類は腫瘍の浸潤と播種転移を主たる基準にした分類であり、国際的に最も汎用されている臨床病期分類である(推奨度1)
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となり
  1. 閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契約が必要となります。閲覧にはご契
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、林太祐、渡邉裕次、井ノ口岳洋、梅田将光による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、
著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適用の査定において保険適用及び保険適用外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適用の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
花田豪郎 : 特に申告事項無し[2021年]
監修:高橋和久 : 講演料(アストラゼネカ(株),MSD(株),中外製薬(株)),研究費・助成金など(小野薬品工業(株),中外製薬(株),ブリストル・マイヤーズスクイブ(株)),奨学(奨励)寄付など(中外製薬(株),大鵬薬品工業(株),杏林製薬(株),サノフィ(株),日本イーライリリー(株),日本ベーリンガーインゲルハイム(株),帝人ファーマ(株))[2021年]

改訂のポイント:
  1. 定期レビューを行い、胸腺の悪性上皮性腫瘍のTNM分類と病期、治療法、フォローアップについて加筆修正した。

病態・疫学・診察

疾患情報(疫学・病態)  
  1. 縦隔腫瘍とは、縦隔内臓器・組織から発生した腫瘍、嚢腫で、心疾患や食道腫瘍は除外する。
  1. 縦隔腫瘍のうち、胸腺関連腫瘍、先天性嚢胞、神経原性腫瘍、胚細胞性腫瘍、リンパ性腫瘍、縦隔内甲状腺腫などの頻度が高い。
  1. 縦隔はCT画像を基準に、縦隔上部、前縦隔、中縦隔、後縦隔に区分され(縦隔区分<図表>)、腫瘍占拠部位により起源組織・臓器が推定できる(縦隔腫瘍の好発部位<図表>)。
  1. 症状がないか、あっても軽度の咳嗽、胸痛の場合が多いが、周辺臓器の圧迫、浸潤による症状を呈することもある。
  1. 画像診断には、胸部造影CTが有用である。症例によっては、MRI、FDG-PETが役立つ。
  1. 腫瘍の鑑別が必要な場合や、術前治療の適応の場合、切除不能例の場合は、組織学的な確定診断が必要となり、経皮的針生検や胸腔鏡による組織採取が行われる。
  1. 治療は、原則として外科的切除が行われる。手術不能例や術後再発例では、化学療法や放射線療法が主体となる。
 
  1. 縦隔腫瘍は、胸腺腫、先天性嚢胞、神経原性腫瘍、リンパ性腫瘍、胸腺癌、胚細胞腫瘍、縦隔内甲状腺腫の順で頻度が高い(推奨度2、O(参考文献:[1]
  1. まとめ:2008年胸部外科学会の手術例の集計では、胸腺腫(36.8%)、先天性嚢胞(16.2%)、神経原生腫瘍(11.6%)、リンパ性腫瘍(6.4%)、胸腺癌(5.8%)、胚細胞腫瘍(5.6%)、縦隔内甲状腺腫(3.1%)の順で頻度が高い。
  1. 追記:悪性リンパ腫など手術適応にならない疾患は過小評価されていることを考慮する。
問診・診察のポイント  
問診のポイント:
  1. 縦隔腫瘍はさまざまな症状を呈することがあり、全身症状を含めた問診が必要となる。

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最新のエビデンスに基づいた二次文献データベース「今日の臨床サポート」。
常時アップデートされており、最新のエビデンスを各分野のエキスパートが豊富な図表や処方・検査例を交えて分かりやすく解説。日常臨床で遭遇するほぼ全ての症状・疾患から薬剤・検査情報まで瞬時に検索可能です。

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文献 

著者: Ryuzo Sakata, Yoshitaka Fujii, Hiroyuki Kuwano
雑誌名: Gen Thorac Cardiovasc Surg. 2010 Jul;58(7):356-83. doi: 10.1007/s11748-010-0604-0.
Abstract/Text
PMID 20628854  Gen Thorac Cardiovasc Surg. 2010 Jul;58(7):356-83. doi:・・・
著者: A Masaoka, Y Monden, K Nakahara, T Tanioka
雑誌名: Cancer. 1981 Dec 1;48(11):2485-92.
Abstract/Text Follow-up data were obtained for 96 cases of thymoma. The one-year survival rate was 84.3%, the three-year 77.1%, the five-year 74.1%, and the ten-year 57.1%. The five-year survival rate of total resection group was 88.9%; that of non-radically treated group was 44.4%. Clinical stages were defined: Stage I--macroscopically encapsulated and microscopically no capsular invasion; Stage II--1. macroscopic invasion into surrounding fatty tissue of mediastinal pleura, or 2. microscopic invasion into capsule; Stage III--macroscopic invasion into neighboring organ; Stage IVa--pleural or pericardial dissemination; Stage IVb--lymphogenous or hematogenous metastasis. Five-year survival rates of each clinical stage were 92.6% in Stage I, 85.7% in Stage II, 69.6% in Stage III, and 50% in Stage IV. Recurrence after total resection was found in six of 69 cases. Seven of 13 patients treated by subtotal resection survived more than five years with postoperative radiotherapy.

PMID 7296496  Cancer. 1981 Dec 1;48(11):2485-92.
著者: Anya M Litvak, Kaitlin Woo, Sara Hayes, James Huang, Andreas Rimner, Camelia S Sima, Andre L Moreira, Maria Tsukazan, Gregory J Riely
雑誌名: J Thorac Oncol. 2014 Dec;9(12):1810-5. doi: 10.1097/JTO.0000000000000363.
Abstract/Text BACKGROUND: Thymic carcinomas are rare cancers with limited data regarding outcomes, particularly for those patients with advanced disease.
METHODS: We identified patients with thymic carcinomas diagnosed between 1993 and 2012. Patient characteristics, recurrence-free survival (RFS), and overall survival (OS) were analyzed.
RESULTS: One hundred twenty-one patients with thymic carcinomas were identified. Higher Masaoka stage was associated with worse OS and RFS (5-year OS of 100%, 81%, 51%, 24%, and 17% for stage I, II, III, IVa, and IVb respectively, p < 0.001 and 5-year RFS of 80%, 28%, and 7% for stage I/II, III, and IV respectively, p < 0.001). Patients with stage IVb lymph node (LN) only disease had a better 5-year OS as compared with patients with distant metastasis (24% versus 7%, p = 0.025). Of the 61 patients with stage IVb disease, 22 of 29 patients (76%) with LN-only disease underwent curative intent resection versus 3 of 32 patients (9%) with distant metastasis. Twenty-two patients with LN involvement were treated with multimodality therapy. Three (14%) remain free of disease with long-term follow-up (range, 3.4+ years- to 6.8+ years).
CONCLUSIONS: We describe the clinical features of a large series of patients with thymic carcinoma in North America. The Masaoka staging system effectively prognosticated OS and RFS. Patients with stage IVb LN-only disease had significantly better OS as compared with patients with distant metastasis with a subset of patients sustaining long-term RFS with multimodality therapy. If validated, these data would support a revised staging system with subclassification of stage IVb disease into two groups.

PMID 25393794  J Thorac Oncol. 2014 Dec;9(12):1810-5. doi: 10.1097/JTO・・・
著者: Kohei Yokoi, Haruhisa Matsuguma, Rie Nakahara, Tetsuro Kondo, Yukari Kamiyama, Kiyoshi Mori, Naoto Miyazawa
雑誌名: J Thorac Oncol. 2007 Jan;2(1):73-8. doi: 10.1097/JTO.0b013e31802bafc8.
Abstract/Text BACKGROUND AND OBJECTIVES: Advanced invasive thymomas are not usually manageable by surgical resection and radiotherapy. We reviewed our experience with a multidisciplinary approach and evaluated chemotherapy in the treatment of invasive thymoma.
PATIENTS AND METHODS: Seventeen consecutive patients with invasive thymoma were treated with multimodality therapy consisting of chemotherapy, surgery, and/or radiotherapy. Four patients had stage III disease with superior vena cava invasion, nine had stage IVa disease, and four had stage IVb disease. The chemotherapy regimen consisted of cisplatin, doxorubicin, and methylprednisolone (CAMP). Chemotherapy was administered in a neoadjuvant setting to the 14 patients and in an adjuvant setting to the remaining three patients. Surgical resection was intended in all patients. After those treatments, chemotherapy and/or radiation therapy were performed.
RESULTS: All but one of the 14 patients with induction chemotherapy responded to the CAMP therapy, and the response rate was 92.9%. Seven of these patients underwent complete remission after surgical resection and chemoradiotherapy, and the others underwent partial remission. All three patients treated with surgical resection and then chemotherapy with or without radiotherapy also achieved complete remission. Tumor progression after multimodality therapy occurred in 10 patients. After retreatment, eight of these patients were alive at the time of analysis, with a median survival time after recurrence of 30 months. The 5- and 10-year overall survival rates for all patients were both 80.7%. The major side effect of CAMP therapy was acceptable neutropenia.
CONCLUSIONS: CAMP therapy was highly effective for invasive thymomas, and the multimodality therapy containing this chemotherapy brought about good disease control in the majority of patients. We believe that this multidisciplinary treatment with CAMP therapy, surgery, and radiotherapy is a justifiable initial treatment for patients with advanced invasive thymoma. Furthermore, appropriate treatments are essential for the long-term survival of patients with recurrences after multimodality therapy.

PMID 17410014  J Thorac Oncol. 2007 Jan;2(1):73-8. doi: 10.1097/JTO.0b・・・
著者: A Fornasiero, O Daniele, C Ghiotto, M Piazza, L Fiore-Donati, F Calabró, F Rea, M V Fiorentino
雑誌名: Cancer. 1991 Jul 1;68(1):30-3.
Abstract/Text From 1977 to 1990, 37 patients with Stage III or IV invasive thymoma (20 men and 17 women; median age, 40 years of age) were referred for chemotherapy to the Padova Medical Oncology Department. All patients initially received the same regimen (50 mg/m2 of cisplatin and 40 mg/m2 of doxorubicin intravenously (IV) on day 1, 0.6 mg/m2 of vincristine IV on day 3, and 700 mg/m2 of cyclophosphamide IV on day 4 [ADOC]), recycling at monthly intervals. No life-threatening side effects were noted. The overall clinical response rate (complete response plus partial response) was 91.8%, with 43% complete remissions. Median duration of response and survival were 12 months (range, 2 to 96+ months) and 15 months (range, 5 to 96+ months), respectively. Seven of the 16 complete remissions were pathologically confirmed at subsequent thoracotomy. Other chemotherapy combinations and radiation therapy have been applied as second-line treatment, achieving only minimal responses. In the opinion of the authors, such chemotherapy deserves evaluation for adjuvant and neo-adjuvant treatment of invasive (and/or inoperable) thymoma due to the high complete response rate and overall response rate.

PMID 2049749  Cancer. 1991 Jul 1;68(1):30-3.
著者: Kazuya Kondo, Yasumasa Monden
雑誌名: Ann Thorac Surg. 2003 Sep;76(3):878-84; discussion 884-5.
Abstract/Text BACKGROUND: Surgery remains the mainstay of treatment for thymic epithelial tumors, and radiation and chemotherapy also have been applied widely as adjuvant and palliative procedures.
METHODS: We compiled records of 1,320 patients with thymic epithelial tumors who were treated from 1990 to 1994 in 115 institutes certified as special institutes for general thoracic surgery by The Japanese Association for Chest Surgery.
RESULTS: Patients with stage I thymoma were treated with only surgery, and patients with stage II and III thymoma and thymic carcinoid underwent surgery and additional radiotherapy. Patients with stage IV thymoma and thymic carcinoma were treated with radiation or chemotherapy. The Masaoka clinical stage is an excellent predictor of the prognosis of thymoma and thymic carcinoma, but not thymic carcinoid. In stage III and IV thymoma, the 5-year survival rates of total resection, subtotal resection, and inoperable groups were 93%, 64%, and 36%, respectively. On the other hand, in thymic carcinoma, the 5-year survival rates of total resection, subtotal resection, and inoperable groups were 67%, 30%, and 24%, respectively. Prophylactic mediastinal radiotherapy could not prevent local recurrences effectively in patients with totally resected stage II and III thymoma. Adjuvant therapy including radiation or chemotherapy did not improve the prognosis in patients with totally resected III and VI thymoma and thymic carcinoma.
CONCLUSIONS: Total resection is the most important factor in the treatment of thymic epithelial tumors. There is value in debulking surgery in invasive thymoma, but not in thymic carcinoma. We doubt that adjuvant therapy is valuable for patients with totally resected invasive thymoma and thymic carcinoma.

PMID 12963221  Ann Thorac Surg. 2003 Sep;76(3):878-84; discussion 884-・・・
著者: James Huang, Nabil P Rizk, William D Travis, Venkatraman E Seshan, Manjit S Bains, Joseph Dycoco, Robert J Downey, Raja M Flores, Bernard J Park, Valerie W Rusch
雑誌名: J Thorac Cardiovasc Surg. 2007 Dec;134(6):1477-83; discussion 1483-4. doi: 10.1016/j.jtcvs.2007.07.049. Epub 2007 Oct 26.
Abstract/Text OBJECTIVE: Extended resections for advanced-stage thymomas are not commonly performed because of the potential morbidity in the face of unclear survival or palliative benefit. We reviewed our experience with multimodality treatment for Masaoka stage IVA thymomas for feasibility and outcomes.
METHODS: We conducted a retrospective review of a single-institution surgical database. Data included patient demographics, preoperative staging and treatment, perioperative events, pathologic findings, and postoperative outcomes.
RESULTS: During the period from 1996 to 2006, 18 patients who had Masaoka stage IVA thymoma underwent surgical resection. All patients received preoperative chemotherapy. Four patients with extensive pleural involvement underwent concomitant extrapleural pneumonectomy and postoperative hemithoracic radiation. Complete resection was achieved in 12 (67%) patients. There was no operative mortality. With a median follow-up of 32.2 months (range 1.4-129.9 months), 3-year, 5-year, and 10-year survivals were 91%, 78%, and 65%, respectively, and median survival has not yet been reached.
CONCLUSION: Multimodality therapy including extended surgical resection can be performed in select patients with stage IVA thymoma with low morbidity and mortality and can result in excellent long-term survival.

PMID 18023668  J Thorac Cardiovasc Surg. 2007 Dec;134(6):1477-83; disc・・・
著者: F Hirai, T Yamanaka, K Taguchi, H Daga, A Ono, K Tanaka, Y Kogure, J Shimizu, T Kimura, J Fukuoka, Y Iwamoto, H Sasaki, K Takeda, T Seto, Y Ichinose, K Nakagawa, Y Nakanishi, West Japan Oncology Group
雑誌名: Ann Oncol. 2015 Feb;26(2):363-8. doi: 10.1093/annonc/mdu541. Epub 2014 Nov 17.
Abstract/Text BACKGROUND: Thymic carcinoma (TC) is an exceptionally rare tumor, which has a very poor prognosis differing from thymoma. Till date, there has been no report of any results of clinical trials focusing on TC. The role of non-anthracycline-based chemotherapy has not been elucidated since the previous studies included a relatively small number of TC patients. This single-arm study evaluated carboplatin and paclitaxel (CbP) in chemotherapy-naive patients with advanced TC.
PATIENTS AND METHODS: The study treatment consisted of carboplatin (area under the curve 6) and paclitaxel (200 mg/m(2)) every 3 weeks for a maximum of six cycles. The primary end point was objective response rate (ORR) by independent review. The secondary end points included overall survival (OS), progression-free survival (PFS), and safety. Based on the SWOG 2-stage design, the planned sample size of 40 patients was determined to reject the ORR of 20% under the expectation of 40% with a power of 0.85 and a type I error of 0.05.
RESULTS: Forty patients from 21 centers were enrolled for this study from May 2008 to November 2010. Of the 39 patients evaluable for analysis, 36 were pathologically diagnosed by independent review, and 97% patients were eventually TC. There was 1/13 complete/partial responses with an ORR of 36% (95% confidence interval 21%-53%; P = 0.031). The median PFS was 7.5 (6.2-12.3) months, while OS did not reach the median value. Major adverse event was grade 3-4 neutropenia in 34 patients (87%). There was no treatment-related death.
CONCLUSIONS: In this largest trial with TC, CbP showed promising efficacy in advanced TC when compared with anthracycline-based chemotherapy, which is the current standard treatment of thymic neoplasm. Our results established that CbP, one of the standard treatments for non-small-cell lung cancer, might be an option as a chemotherapy regimen for TC.

© The Author 2014. Published by Oxford University Press on behalf of the European Society for Medical Oncology. All rights reserved. For permissions, please email: journals.permissions@oup.com.
PMID 25403584  Ann Oncol. 2015 Feb;26(2):363-8. doi: 10.1093/annonc/md・・・
著者: Akira Inoue, Shunichi Sugawara, Masao Harada, Kunihiko Kobayashi, Toshiyuki Kozuki, Shoichi Kuyama, Makoto Maemondo, Hajime Asahina, Akiko Hisamoto, Taku Nakagawa, Katsuyuki Hotta, Toshihiro Nukiwa
雑誌名: J Thorac Oncol. 2014 Dec;9(12):1805-9. doi: 10.1097/JTO.0000000000000362.
Abstract/Text BACKGROUND: There has been no standard chemotherapy for advanced or recurrent thymic malignancies including thymic carcinoma (TC) and invasive thymoma (IT), though platinum and anthracycline have been reported as effective agents for the treatment of these diseases. The objective of this study was to evaluate the efficacy and safety of the combination of amrubicin (AMR), a new anthracycline agent, and carboplatin (CBDCA) in patients with advanced thymic malignancies.
METHODS: Patients with histologically confirmed thymic malignancies received AMR (35 mg/m, days 1-3) and CBDCA (area under the curve 4.0, day 1) every 3 weeks. Patients who had received previous chemotherapy were treated with a reduced dose of AMR (30 mg/m). The primary end point was objective response rate (ORR), and secondary endpoints were progression-free survival, overall survival, and toxicity profile.
RESULTS: From December 2008 to October 2012, 51 patients (33 TC and 18 IT) were enrolled. The median number of treatment cycles was four in each group. The ORR and progression-free survival were 30% (95% confidence interval, 14-46) and 7.6 months in the TC group, and 17% (95% confidence interval, 0-34) and 7.6 months in the IT group, respectively. The ORR of TC patients without previous chemotherapy (n = 19) was 42%. Although grade 3 or 4 hematological toxicities were common including neutropenia (82%) and febrile neutropenia (22%), these were transient and manageable. Nonhematological toxicities were moderate and no treatment-related death was observed.
CONCLUSIONS: The combination of AMR with CBDCA was active for TC with acceptable toxicity, although it was not effective for IT. Further investigation of this regimen for advanced TC is warranted.

PMID 25393793  J Thorac Oncol. 2014 Dec;9(12):1805-9. doi: 10.1097/JTO・・・
著者: Takeshi Nagata, Shiro Makutani, Hideo Uchida, Kimihiko Kichikawa, Munehiro Maeda, Tetsuya Yoshioka, Hiroshi Anai, Hiroshi Sakaguchi, Hitoshi Yoshimura
雑誌名: Cardiovasc Intervent Radiol. 2007 Sep-Oct;30(5):959-67. doi: 10.1007/s00270-007-9088-4.
Abstract/Text PURPOSE: To retrospectively clarify the utility of metallic stent placement for the treatment of the malignant obstruction of the superior vena cava (SVC) in 71 patients with VC syndrome (SVCS) on the basis of long-term follow-up data.
MATERIALS AND METHODS: Seventy-one patients underwent stent placement and were followed until death. The applicability of the spiral Z-stent (S-Z-stent) mainly used the initial and follow-up results, stent placement for bilateral BCV obstruction and the value of concurrent anticancer therapy were studied.
RESULTS: The technical success rate was 100%, the initial clinical success rate was 87% (62/71), the primary clinical patency rate was 88% (57/65), and the secondary clinical patency rate was 95% (62/65). The obstruction rate of the stent was 12% (8/65), and an additional stent was useful for relief of recurrent SVCS. Survival of 57 patients in whom there was no recurrence of SVCS until death ranged from 1 week to 29 months (mean, 5.4 months and the S-Z-stent appeared to be suitable for the treatment of the malignant obstruction of SVC. Unilateral stent placement was effective for relief of SVCS with bilateral BCV obstruction. Patients who received concurrent anticancer therapy survived 2 months longer than those who did not.
CONCLUSION: Stent placement is an effective treatment for SVCS. Further, the utility of S-Z-stent for SVCS, an additional stent for recurrence, unilateral stent for patients with bilateral BCV obstruction, and anticancer therapy after stent placement were verified.

PMID 17546400  Cardiovasc Intervent Radiol. 2007 Sep-Oct;30(5):959-67.・・・

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