Mediastinale Tumoren

Der Begriff Mediastinaltumor beschreibt eine überaus heterogene Entität von über 90 derartig beschriebenen Läsionen. Die Gemeinsamkeit liegt in der anatomischen Zuordnung und im raumfordernden Charakter.

Abstract

If mediastinal tumours cause symptoms these are related to their anatomical localization or a paraneoplastic syndrome. The differential diagnosis is based on the clinical situation with finding the lesion, and, furthermore, taking into account the age and sex of the patient, and the mediastinal compartment where the lesion is located. Cross-sectional radiographic diagnostic is essential for defining the therapeutic strategy. The anterior mediastinum is dominated by thymic tumours, mediastinal lymphomas, germ cell tumours and ectopic mediastinal poiters. The middle mediastinal compartment is the most frequent place of mediastinal cystic tumours, whereas the posterior mediastinum is the domain of neurogenic tumours. For selected cases a tissue biopsy is required. Surgery is the mainstay for most mediastinal tumours. Median sternotomy is the most frequent conventional surgical technique while minimally invasive surgery with thoracoscopic and above all robot assisted operation techniques are increasingly frequent. Combined chemotherapy and modern radiotherapy are essential components of the comprehensive treatment for mediastinal tumours.

Kernaussagen

• Wenn mediastinale Tumoren Symptome verursachen, stehen diese meist im Zusammenhang mit ihrer Lokalisation oder einer Paraneoplasie.

• Die Differenzialdiagnose beruht auf der klinischen Situation bei Feststellung des Tumors, dem Alter und dem Geschlecht des Patienten/der Patientin und der Zuordnung zum jeweiligen mediastinalen Kompartment.

• Die Schnittbilddiagnostik ist wesentlicher Bestandteil der Therapieplanung.

• Im vorderen Mediastinum dominieren Thymustumoren, mediastinale Lymphome, Keimzelltumoren und mediastinale ektope Schilddrüsen. Im mittleren Mediastinum sind vielfach zystische Raumforderungen vorhanden, während das hintere Mediastinum die Domäne der neurogenen mediastinalen Tumoren darstellt.

• In ausgewählten Fällen ist eine Biopsie zur Therapieplanung erforderlich.

• Die Chirurgie spielt eine wesentliche Rolle in der Behandlung mediastinaler Tumoren.

• Bei den konventionellen Verfahren dominiert die mediane Sternotomie.

• Die minimalinvasive Chirurgie ist mit thorakoskopischen und insbesondere roboterassistierten Operationstechniken zunehmend etabliert.

• Die Kombinationschemotherapie und die moderne Radiotherapie sind essenzielle Bestandteile der komplexen Behandlung mediastinaler Tumoren.

Publication History

Article published online:
14 April 2022

• Literatur

• 1 Shields TW. Mediastinal Surgery. Malvern: Lea & Febiger; 1991: 400
• 2 Ahmad U, Yao X, Detterbeck F. et al. Thymic carcinoma outcomes and prognosis: results of an international analysis. J Thorac Cardiovasc Surg 2015; 149: 95-100, 101.e1–2
• 3 Johnson DW, Hoppe RT, Cox RS. et al. Hodgkin’s disease limited to intrathoracic sites. Cancer 1983; 52: 8-13
• 4 Levitt LJ, Aisenberg AC, Harris NL. et al. Primary non-Hodgkin’s lymphoma of the mediastinum. Cancer 1982; 50: 2486-2492
• 5 Davis jr. RD, Oldham jr. HN, Sabiston jr. DC. Primary cysts and neoplasms of the mediastinum: recent changes in clinical presentation, methods of diagnosis, management, and results. Ann Thorac Surg 1987; 44: 229-237
• 6 Roden AC, Fang W, Shen Y. et al. Distribution of mediastinal lesions across multi-institutional, international, radiology databases. J Thorac Oncol 2020; 15: 568-579
• 7 Bergh NP, Gatzinsky P, Larsson S. et al. Tumors of the thymus and thymic region: III. Clinicopathological studies onteratomas and tumors of Germ Cell type. Ann Thorac Surg 1978; 25: 107-111
• 8 Cox JD. Primary malignant germinal tumors of the mediastinum. A study of 24 cases. Cancer 1975; 36: 1162-1168
• 9 Mikuz G. Keimzell- und Gonadenstromatumoren des Hodens. WHO-Klassifikation 2016. Pathologe 2017; 38: 209-220
• 10 Moran CA, Suster S. Primary germ cell tumors of the mediastinum: I. Analysis of 322 cases with special emphasis on teratomatous lesions and a proposal for histopathologic classification and clinical staging. Cancer 1997; 80: 681-690
• 11 Dieckmann KP, Radtke A, Geczi L. et al. Serum levels of MicroRNA-371a-3p (M371 Test) as a new biomarker of testicular germ cell tumors: results of a prospective multicentric study. J Clin Oncol 2019; 37: 1412-1423
• 12 Le Roux BT. Mediastinal teratomata. Thorax 1960; 15: 333-338
• 13 Carter BW, Okumura M, Detterbeck FC. et al. Approaching the patient with an anterior mediastinal mass: a guide for radiologists. J Thorac Oncol 2014; 9(9 Suppl. 2): S110-S118
• 14 Bremmer F, Strobel P. Mediastinale Keimzelltumoren. Pathologe 2016; 37: 441-448
• 15 Kawahara K, Miyawaki M, Anami K. et al. A patient with mediastinal mature teratoma presenting withparaneoplastic limbic encephalitis. J Thorac Oncol 2012; 7: 258-259
• 16 Stover DG, Eisenberg R, Johnson DH. Anti-N-methyl-D-aspartate receptor encephalitis in a young woman with a mature mediastinal teratoma. J Thorac Oncol 2010; 5: 1872-1873
• 17 Berry CL, Keeling J, Hilton C. Teratomata in infancy and childhood: a review of 91 cases. J Pathol 1969; 98: 241-252
• 18 Oberman HA, Libcke JH. Malignant Germinal Neoplasms of the Mediastinum. Cancer 1964; 17: 498-507
• 19 Comiter CV, Kibel AS, Richie JP. et al. Prognostic features of teratomas with malignant transformation: a clinicopathological study of 21 cases. J Urol 1998; 159: 859-863
• 20 Puc HS, Heelan R, Mazumdar M. et al. Management of residual mass in advanced seminoma: results and recommendations from the Memorial Sloan-Kettering Cancer Center. J Clin Oncol 1996; 14: 454-460
• 21 Bokemeyer C, Droz JP, Horwich A. et al. Extragonadal seminoma: an international multicenter analysis of prognostic factors and long term treatment outcome. Cancer 2001; 91: 1394-1401
• 22 Bokemeyer C, Nichols CR, Droz JP. et al. Extragonadal germ cell tumors of the mediastinum and retroperitoneum: results from an international analysis. J Clin Oncol 2002; 20: 1864-1873
• 23 Oosting SF, de Haas EC, Links TP. et al. Prevalence of paraneoplastic hyperthyroidism in patients with metastatic non-seminomatous germ-cell tumors. Ann Oncol 2010; 21: 104-108
• 24 Vuky J, Bains M, Bacik J. et al. Role of postchemotherapy adjunctive surgery in the management of patients with nonseminoma arising from the mediastinum. J Clin Oncol 2001; 19: 682-688
• 25 Berrocal T, Madrid C, Novo S. et al. Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics 2004; 24: e17
• 26 Arbona JL, Fazzi JG, Mayoral J. Congenital esophageal cysts: case report and review of literature. Am J Gastroenterol 1984; 79: 177-182
• 27 Ildstad ST, Tollerud DJ, Weiss RG. et al. Duplications of the alimentary tract. Clinical characteristics, preferred treatment, and associated malformations. Ann Surg 1988; 208: 184-189
• 28 Jung HS, Kim DK, Lee GD. et al. Video-assisted thoracic surgery for bronchogenic cysts: is this the surgical approach of choice?. Interact Cardiovasc Thorac Surg 2014; 19: 824-829
• 29 Travis WD, Brambilla E, Nicholson AG. et al. The 2015 World Health Organization classification of lung tumors: impact of genetic, clinical and radiologic advances since the 2004 classification. J Thorac Oncol 2015; 10: 1243-1260
• 30 Marx A, Weis CA, Strobel P. Thymomas. Pathologe 2016; 37: 412-424
• 31 Detterbeck FC, Nicholson AG, Kondo K. et al. The Masaoka-Koga stage classification for thymic malignancies: clarification and definition of terms. J Thorac Oncol 2011; 6(7 Suppl. 3): S1710-S1716
• 32 Ruckert JC, Feng L, Swierzy M. et al. Was ist richtig beim Thymom?. Chirurgische Allgemeine 2018; 3: 1-13
• 33 Zhao J, Bhatnagar V, Ding L. et al. A systematic review of paraneoplastic syndromes associated with thymoma: Treatment modalities, recurrence, and outcomes in resected cases. J Thorac Cardiovasc Surg 2020; 160: 306-314.e14
• 34 Papatestas AE, Genkins G, Kornfeld P. et al. Effects of thymectomy in myasthenia gravis. Ann Surg 1987; 206: 79-88
• 35 Weissferdt A, Kalhor N, Bishop JA. et al. Thymoma: a clinicopathological correlation of 1470 cases. Hum Pathol 2018; 73: 7-15
• 36 Nicholson AG, Detterbeck FC, Marino M. et al. The IASLC/ITMIG Thymic Epithelial Tumors Staging Project: proposals for the T component for the forthcoming (8th) edition of the TNM classification of malignant tumors. J Thorac Oncol 2014; 9(9 Suppl. 2): S73-S80
• 37 Hwang Y, Park IK, Park S. et al. Lymph node dissection in thymic malignancies: implication of the ITMIG lymph node map, TNM stage classification, and recommendations. J Thorac Oncol 2016; 11: 108-114
• 38 Marom EM, Milito MA, Moran CA. et al. Computed tomography findings predicting invasiveness of thymoma. J Thorac Oncol 2011; 6: 1274-1281
• 39 Marom EM, Rosado-de-Christenson ML, Bruzzi JF. et al. Standard report terms for chest computed tomography reports of anterior mediastinal masses suspicious for thymoma. J Thorac Oncol 2011; 6(7 Suppl. 3): S1717-S1723
• 40 Marom EM. Advances in thymoma imaging. J Thorac Imaging 2013; 28: 69-80
• 41 Priola AM, Gned D, Veltri A. et al. Chemical shift and diffusion-weighted magnetic resonance imaging of the anterior mediastinum in oncology: Current clinical applications in qualitative and quantitative assessment. Crit Rev Oncol Hematol 2016; 98: 335-357
• 42 Blalock A, Mason MF, Morgan HJ. et al. Myasthenia gravis and tumors of the thymic region: report of a case in which the tumor was removed. Ann Surg 1939; 110: 544-561
• 43 Rea F, Marulli G, Bortolotti L. et al. Experience with the “da Vinci” robotic system for thymectomy in patients with myasthenia gravis: report of 33 cases. Ann Thorac Surg 2006; 81: 455-459
• 44 Balduyck B, Hendriks JM, Lauwers P. et al. Quality of life after anterior mediastinal mass resection: a prospective study comparing open with robotic-assisted thoracoscopic resection. Eur J Cardiothorac Surg 2011; 39: 543-548
• 45 Wright CD. Management of thymomas. Crit Rev Oncol Hematol 2008; 65: 109-120
• 46 Solis-Pazmino P, Baiu I, Lincango-Naranjo E. et al. Impact of the surgical approach to thymectomy upon complete stable remission rates in myasthenia gravis: a meta-analysis. Neurology 2021; 97: e357-e368
• 47 Marulli G, Rea F, Melfi F. et al. Robot-aided thoracoscopic thymectomy for early-stage thymoma: a multicenter European study. J Thorac Cardiovasc Surg 2012; 144: 1125-1130
• 48 Marulli G, Comacchio GM, Stocca F. et al. Robotic-assisted thymectomy: current perspectives. Robot Surg 2016; 3: 53-63
• 49 Kang CH, Hwang Y, Lee HJ. et al. Robotic thymectomy in anterior mediastinal mass: propensity score matching study with transsternal thymectomy. Ann Thorac Surg 2016; 102: 895-901
• 50 Yang CJ, Hurd J, Shah SA. et al. A national analysis of open versus minimally invasive thymectomy for stage I to III thymoma. J Thorac Cardiovasc Surg 2020; 160: 555-567.e15
• 51 Nation Comprehensive Cancer Network. Clinical practice guidelines in oncology. Thymomas and thymic carcinomas. V1.2021. Accessed December 22, 2021 at: https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1469
• 52 Kim ES, Putnam JB, Komaki R. et al. Phase II study of a multidisciplinary approach with induction chemotherapy, followed by surgical resection, radiation therapy, and consolidation chemotherapy for unresectable malignant thymomas: final report. Lung Cancer 2004; 44: 369-379
• 53 Kunitoh H, Tamura T, Shibata T. et al. A phase II trial of dose-dense chemotherapy, followed by surgical resection and/or thoracic radiotherapy, in locally advanced thymoma: report of a Japan Clinical Oncology Group trial (JCOG 9606). Br J Cancer 2010; 103: 6-11
• 54 Korst RJ, Bezjak A, Blackmon S. et al. Neoadjuvant chemoradiotherapy for locally advanced thymic tumors: a phase II, multi-institutional clinical trial. J Thorac Cardiovasc Surg 2014; 147: 36-44, 46.e1
• 55 Moser B, Fadel E, Fabre D. et al. Surgical therapy of thymic tumours with pleural involvement: an ESTS Thymic Working Group Project. Eur J Cardiothorac Surg 2017; 52: 346-355
• 56 Ried M, Potzger T, Braune N. et al. Cytoreductive surgery and hyperthermic intrathoracic chemotherapy perfusion for malignant pleural tumours: perioperative management and clinical experience. Eur J Cardiothorac Surg 2013; 43: 801-807
• 57 Tang EK, Chang JM, Chang CC. et al. Prognostic factor of completely resected and pathologic t3 n0 m0 thymic epithelial tumor. Ann Thorac Surg 2021; 111: 1164-1173
• 58 Song SH, Suh JW, Yu WS. et al. The role of postoperative radiotherapy in stage II and III thymoma: a Korean multicenter database study. J Thorac Dis 2020; 12: 6680-6689
• 59 Lim YJ, Kim E, Kim HJ. et al. Survival impact of adjuvant radiation therapy in masaoka stage II to IV thymomas: A systematic review and meta-analysis. Int J Radiat Oncol Biol Phys 2016; 94: 1129-1136
• 60 Falkson CB. Postoperative radiation for completely resected stage II/III thymoma: What do we know in 2021?. J Thorac Oncol 2021; 16: 515
• 61 Basse C, Botticella A, Molina TJ. et al. RADIORYTHMIC: Phase III, opened, randomized study of postoperative radiotherapy versus surveillance in stage IIb/III of Masaoka Koga thymoma after complete surgical resection. Clin Lung Cancer 2021; 22: 469-472
• 62 Ruffini E, Filosso PL, Oliaro A. The role of surgery in recurrent thymic tumors. Thorac Surg Clin 2009; 19: 121-131
• 63 Regnard JF, Zinzindohoue F, Magdeleinat P. et al. Results of re-resection for recurrent thymomas. Ann Thorac Surg 1997; 64: 1593-1598
• 64 Kirschner PA. Reoperation for thymoma: report of 23 cases. Ann Thorac Surg 1990; 49: 550-554
• 65 Fan XW, Yang Y, Wang HB. et al. Intensity modulated radiation therapy plus etoposide/cisplatin for patients with limited advanced unresectable thymic epithelial tumors: A prospective phase 2 study. Int J Radiat Oncol Biol Phys 2020; 107: 98-105
• 66 Radovich M, Pickering CR, Felau I. et al. The integrated genomic landscape of thymic epithelial tumors. Cancer Cell 2018; 33: 244-258.e10
• 67 Mack E. Management of patients with substernal goiters. Surg Clin North Am 1995; 75: 377-394
• 68 Buckley JA, Stark P. Intrathoracic mediastinal thyroid goiter: imaging manifestations. AJR Am J Roentgenol 1999; 173: 471-475
• 69 White ML, Doherty GM, Gauger PG. Evidence-based surgical management of substernal goiter. World J Surg 2008; 32: 1285-1300
• 70 Sakorafas GH, Vlachos A, Tolumis G. et al. Ectopic intrathoracic thyroid: case report. Mt Sinai J Med 2004; 71: 131-133
• 71 de Perrot M, Fadel E, Mercier O. et al. Surgical management of mediastinal goiters: when is a sternotomy required?. Thorac Cardiovasc Surg 2007; 55: 39-43
• 72 Noppen M, Poppe K, D’Haese J. et al. Interventional bronchoscopy for treatment of tracheal obstruction secondary to benign or malignant thyroid disease. Chest 2004; 125: 723-730
• 73 Casella C, Pata G, Cappelli C. et al. Preoperative predictors of sternotomy need in mediastinal goiter management. Head Neck 2010; 32: 1131-1135
• 74 Vadasz P, Kotsis L. Surgical aspects of 175 mediastinal goiters. Eur J Cardiothorac Surg 1998; 14: 393-397
• 75 Monchik JM, Materazzi G. The necessity for a thoracic approach in thyroid surgery. Arch Surg 2000; 135: 467-471
• 76 Grondin SC, Buenaventura P, Luketich JD. Thoracoscopic resection of an ectopic intrathoracic goiter. Ann Thorac Surg 2001; 71: 1697-1698
• 77 Al-Mufarrej F, Margolis M, Tempesta B. et al. Novel thoracoscopic approach to posterior mediastinal goiters: report of two cases. J Cardiothorac Surg 2008; 3: 55
• 78 Akerström G, Rudberg C, Grimelius L. et al. Causes of failed primary exploration and technical aspects of re-operation in primary hyperparathyroidism. World J Surg 1992; 16: 562-568
• 79 Russell CF, Edis AJ, Scholz DA. et al. Mediastinal parathyroid tumors: experience with 38 tumors requiring mediastinotomy for removal. Ann Surg 1981; 193: 805-809
• 80 Gasparri G, Camandona M, Abbona GC. et al. Secondary and tertiary hyperparathyroidism: causes of recurrent disease after 446 parathyroidectomies. Ann Surg 2001; 233: 65-69
• 81 Akbaba G, Berker D, Isik S. et al. A comparative study of pre-operative imaging methods in patients with primary hyperparathyroidism: ultrasonography, 99mTc sestamibi, single photon emission computed tomography, and magnetic resonance imaging. J Endocrinol Invest 2012; 35: 359-364
• 82 Alesina PF, Moka D, Mahlstedt J. et al. Thoracoscopic removal of mediastinal hyperfunctioning parathyroid glands: personal experience and review of the literature. World J Surg 2008; 32: 224-231
• 83 Ismail M, Maza S, Swierzy M. et al. Resection of ectopic mediastinal parathyroid glands with the da Vinci robotic system. Br J Surg 2010; 97: 337-343
• 84 Wei B, Inabnet W, Lee JA. et al. Optimizing the minimally invasive approach to mediastinal parathyroid adenomas. Ann Thorac Surg 2011; 92: 1012-1017
• 85 Bicakcioglu P, Demirag F, Yazicioglu A. et al. Intrathoracic neurogenic tumors. Thorac Cardiovasc Surg 2014; 62: 147-152
• 86 Marchevsky AM, Kaneko M. Surgical Pathology of the Mediastinum. 2. New York: Raven Press; 1992: 335
• 87 Adachi Y, Mita H, Sasaki Y. et al. Malignant paraganglioma of the posterior mediastinum: a case report with genetic analysis. Mol Clin Oncol 2019; 10: 10-16
• 88 Nordback P, Halkic N, Boumghar M. Intrathoracic tumours in von Recklinghausen’s neurofibromatosis. Schweiz Med Wochenschr 2000; 130: 1105-1111
• 89 Radner H, Blümcke I, Reifenberger G. et al. The new WHO classification of tumors of the nervous system 2000. Pathology and genetics. Pathologe 2002; 23: 260-283
• 90 Tsilimparis N, Gregor JI, Swierzy M. et al. Intrapericardial paraganglioma in a 78-year-old female patient. Am Surg 2010; 76: 450-451
• 91 Besterman E, Bromley LL, Peart WS. An intrapericardial phaeochromocytoma. Br Heart J 1974; 36: 318-320
• 92 Benjamin SP, McCormack LJ, Effler DB. et al. Primary tumors of the mediastinum. Chest 1972; 62: 297-303
• 93 Dienemann H. Erkrankungen des Mediastinums. In: Heberer G, Schildberg FW, Sunder-Plassmann L, Vogt-Moykopf I. Lunge und Mediastinum. Anatomie Diagnostik Indikationen Technik Ergebnisse. Berlin, Heidelberg: Springer; 1991: 678
• 94 Endo S, Murayama F, Otani S. et al. Alternative surgical approaches for apical neurinomas: a thoracoscopic approach. Ann Thorac Surg 2005; 80: 295-298
• 95 Yamaguchi M, Yoshino I, Fukuyama S. et al. Surgical treatment of neurogenic tumors of the chest. Ann Thorac Cardiovasc Surg 2004; 10: 148-151
• 96 Pacchiarotti G, Wang MY, Kolcun JPG. et al. Robotic paravertebral schwannoma resection at extreme locations of the thoracic cavity. Neurosurg Focus 2017; 42: E17
• 97 Kajiwara N, Kakihana M, Usuda J. et al. Extended indications for robotic surgery for posterior mediastinal tumors. Asian Cardiovasc Thorac Ann 2012; 20: 308-313
• 98 Finley D, Sherman JH, Avila E. et al. Thorascopic resection of an apical paraspinal schwannoma using the da Vinci surgical system. J Neurol Surg A Cent Eur Neurosurg 2014; 75: 58-63
• 99 Shadmehr MB, Gaissert HA, Wain JC. et al. The surgical approach to “dumbbell tumors” of the mediastinum. Ann Thorac Surg 2003; 76: 1650-1654
• 100 Brodeur GM, Pritchard J, Berthold F. et al. Revisions of the international criteria for neuroblastoma diagnosis, staging, and response to treatment. J Clin Oncol 1993; 11: 1466-1477
• 101 Morris JA, Shcochat SJ, Smith EI. et al. Biological variables in thoracic neuroblastoma: a pediatric oncology group study. J Pediatr Surg 1995; 30: 296-302
• 102 Rendina EA, Venuta F, Andreeti C. et al. Rare tumors of the mediastinum. In: Kuzdzal J, Asamura H, Detterbeck F, Goldstraw P, Lerut T, Thomas P, Treasure T. ESTS Textbook of thoracic Surgery. Krakow, Poland: Medycyna Praktyczna; 2014: 319-327
• 103 Rosai J. Histological typing of tumours of the thymus. Berlin: Springer-Verlag; 1999
• 104 Marx A, Chan JK, Coindre JM. et al. The 2015 World Health Organization Classification of Tumors of the Thymus: Continuity and Changes. J Thorac Oncol 2015; 10: 1383-1395