eMedicine Specialties > Thoracic Surgery > Tumors

Lymphomas, Endocrine, Mesenchymal, and Other Rare Tumors of the Mediastinum: Follow-up

Author: Mary C Mancini, MD, PhD, Professor and Chief, Cardiothoracic Surgery, Department of Surgery, Louisiana State University Health Sciences Center-Shreveport
Contributor Information and Disclosures

Updated: Jan 29, 2009

Outcome and Prognosis

Prognosis after resection of a mediastinal tumor varies widely depending on the type of lesion resected.

After resection of mediastinal cysts and benign tumors, prognosis is generally excellent. This group of tumors includes such neoplasms as thymolipomas; benign mesenchymal tumors such as fibromas, angiomas, and lymphangiomas; ectopic benign thyroid tissue, including intrathoracic extension of a cervical goiter; and parathyroid adenomas.

Prognosis after treatment of malignant mediastinal tumors depends on the type of lesion, its biological behavior, and the extent of the disease present.

Mediastinal lymphomas

Excellent survival rates are reported with appropriate chemotherapeutic treatment of Hodgkin disease. According to some reports, even patients with stage IV disease can have disease-free survival for more than 10 years in as many as 70% of cases.

Large cell lymphomas, including anaplastic varieties, are reported to have a more than 50% 3-year survival rate after chemotherapy, radiation therapy, or both.

MALTomas are rare indolent tumors for which long-term survival is good, even in the absence of chemotherapy.

Mantle cell tumors are rare but usually manifest with widespread disease. Median survival is approximately 4 years.

Malignant mesenchymal tumors

The prognosis for liposarcoma depends on several factors. Completely resected pseudoencapsulated tumors have a better prognosis compared to those that are nonencapsulated and invasive. Cell type and cell differentiation also play a role. Myxoid liposarcoma has a poorer prognosis. In one small series, approximately 30% of patients died of their disease after a mean period of less than 3 years.

Angiosarcomas of the mediastinum are rare but have a very poor prognosis, especially when they originate in the heart or great vessels.

Fibrosarcomas have a uniformly poor prognosis, and most patients die from their disease within a few years.

Primary leiomyosarcoma of the mediastinum has been described. In one reported series of 10 patients who underwent resection, 2 were alive and well at 4 and 6 years. Four of the others died of their disease or developed recurrence.

Rhabdomyosarcoma has the best long-term survival of all mesenchymal tumors because effective chemotherapeutic regimens have been identified. According to 1 report, 10-year actuarial survival rates range from 52-83% depending on the stage of disease at presentation and the existence of hematogenous metastases. Survival is much worse if the latter is present.

Mediastinal parathyroid carcinoma

Prognosis is generally good if complete resection can be accomplished.

Intrathoracic goiter with occult malignancy

Prognosis is generally good if complete resection of the gland can be accomplished.

Future and Controversies

A number of exciting advances have been made in areas of diagnostic imaging, biologic analysis, and therapy.

Emerging diagnostic modalities, such as PET scans, and other radionuclide studies may be able to assist in the diagnosis of specific neoplasms and in posttherapy surveillance for recurrent disease.29

Angiographic techniques using localized intra-arterial injection of hypertonic contrast and embolization techniques have been used in several centers to obliterate mediastinal parathyroid adenomas. At present, this technology is used in patients who are considered a poor risk for surgery; however, with increased experience and skill, these methods may become useful in the treatment of many such lesions.

Numerous biological markers have been identified for many tumors and will play a vital role in better identifying individual neoplasms so that treatment can be optimized.

Use of video-assisted thoracoscopy (VATS) technology has entered the armamentarium of the thoracic surgeon with respect to the treatment of a number of mediastinal diseases. This modality is already used commonly for biopsy of masses and lymph nodes. It has also been described for resection of various mediastinal cysts, mediastinal parathyroid adenomas, and localized benign tumors of the posterior mediastinum, such as ganglioneuromas. At several centers, thymectomy has been performed using this technology. The completeness of thymic resection remains to be seen.

 
Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Jane M Eggerstedt, MD, to the development and writing of this article.



More on Lymphomas, Endocrine, Mesenchymal, and Other Rare Tumors of the Mediastinum

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References
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References

  1. Wakeley CP, Mulvany JH. Intrathoracic goiter. Surg Gynecol Obstet. 1940;70:702-10.

  2. Meade RH. Surgery of the mediastinum. In: A History of Thoracic Surgery. Springfield, Ill: Charles C Thomas; 1961:257-71.

  3. Shields TW. Overview of primary mediastinal tumors and cysts. In: Shields TW, LoCicero J III, Ponn RB, eds. General Thoracic Surgery. Vol 2. 5th ed. Philadelphia, Pa: Williams & Wilkins; 2000:2105-9.

  4. Whooley BP, Urschel JD, Antkowiak JG, Takita H. Primary tumors of the mediastinum. J Surg Oncol. Feb 1999;70(2):95-9. [Medline].

  5. Strollo DC, Rosado de Christenson ML, Jett JR. Primary mediastinal tumors. Part I: tumors of the anterior mediastinum. Chest. Aug 1997;112(2):511-22. [Medline].

  6. Strollo DC, Rosado-de-Christenson ML, Jett JR. Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastinum. Chest. Nov 5 1997;112(5):1344-57. [Medline].

  7. Richards ML, Bondeson AG, Thompson NW. Mediastinal parathyroid adenomas and carcinomas. In: Shields TW, LoCicero J III, Ponn RB, eds. General Thoracic Surgery. Vol 2. 5th ed. Philadelphia, Pa: Williams & Wilkins; 2000:2383-90.

  8. Shields TW, Robinson PG. Mesenchymal tumors of the mediastinum. In: Shields TW, LoCicero J III, Ponn RB, eds. General Thoracic Surgery. Vol 2. 5th ed. Philadelphia, Pa: Williams & Wilkins; 2000:2357-81.

  9. Wakely PE Jr. Cytopathology-histopathology of the mediastinum II. Mesenchymal, neural, and neuroendocrine neoplasms. Ann Diagn Pathol. Feb 2005;9(1):24-32. [Medline].

  10. Gordon LI, Kies MS. Diagnosis and treatment of mediastinal lymphomas. In: Shields TW, LoCicero J III, Ponn RB, eds. General Thoracic Surgery. Vol 2. 5th ed. Philadelphia, Pa: Williams & Wilkins; 2000:2267-74.

  11. Suster S. Primary large-cell lymphomas of the mediastinum. Semin Diagn Pathol. Feb 1999;16(1):51-64. [Medline].

  12. Kolonic SO, Dzebro S, Kusec R, Planinc-Peraica A, Dominis M, Jaksic B. Primary mediastinal large B-cell lymphoma: a single-center study of clinicopathologic characteristics. Int J Hematol. May 2006;83(4):331-6. [Medline].

  13. Savage KJ. Primary mediastinal large B-cell lymphoma. Oncologist. May 2006;11(5):488-95. [Medline].

  14. Chnaris A, Barbetakis N, Efstathiou A, Fessatidis I. Primary mediastinal hemangiopericytoma. World J Surg Oncol. Apr 27 2006;4:23. [Medline].

  15. Sahni V, Sunandan S, Agarwal SK, Singh NP, Ganguli A. Rare thoracic mass lesion--myofibrobastoma. Indian J Cancer. Jul-Sep 2005;42(3):161-4. [Medline].

  16. Iyer RB, Whitman GJ, Sahin AA. Parathyroid adenoma of the mediastinum. AJR Am J Roentgenol. Jul 1999;173(1):94. [Medline].

  17. Priola AM, Priola SM, Cardinale L, Cataldi A, Fava C. The anterior mediastinum: diseases. Radiol Med. Apr 2006;111(3):312-42. [Medline].

  18. Lee HY, Goo JM, Lee HJ, Lee CH, Chun EJ, Im JG. The value of computed tomography for predicting empyema-associated malignancy. J Comput Assist Tomogr. May-Jun 2006;30(3):453-9. [Medline].

  19. Geibel A, Kasper W, Keck A, et al. Diagnosis, localization and evaluation of malignancy of heart and mediastinal tumors by conventional and transesophageal echocardiography. Acta Cardiol. 1996;51(5):395-408. [Medline].

  20. Giron J, Fajadet P, Sans N, et al. Diagnostic approach to mediastinal masses. Eur J Radiol. Mar 1998;27(1):21-42. [Medline].

  21. Bangerter M, Kotzerke J, Griesshammer M, et al. Positron emission tomography with 18-fluorodeoxyglucose in the staging and follow-up of lymphoma in the chest. Acta Oncol. 1999;38(6):799-804. [Medline].

  22. Demmy TL, Krasna MJ, Detterbeck FC, et al. Multicenter VATS experience with mediastinal tumors. Ann Thorac Surg. Jul 1998;66(1):187-92. [Medline].

  23. Kaga K, Nishiumi N, Iwasaki M, Inoue H. Thoracoscopic diagnosis and treatment of mediastinal masses. Usefulness of the Two Windows Method. J Cardiovasc Surg (Torino). Feb 1999;40(1):157-60. [Medline].

  24. Rieger R, Schrenk P, Woisetschlager R, Wayand W. Videothoracoscopy for the management of mediastinal mass lesions. Surg Endosc. Jul 1996;10(7):715-7. [Medline].

  25. Greif J, Staroselsky AN, Gernjac M, et al. Percutaneous core needle biopsy in the diagnosis of mediastinal tumors. Lung Cancer. Sep 1999;25(3):169-73. [Medline].

  26. Protopapas Z, Westcott JL. Transthoracic hilar and mediastinal biopsy. J Thorac Imaging. Oct 1997;12(4):250-8. [Medline].

  27. Serna DL, Aryan HE, Chang KJ, et al. An early comparison between endoscopic ultrasound-guided fine-needle aspiration and mediastinoscopy for diagnosis of mediastinal malignancy. Am Surg. Oct 1998;64(10):1014-8. [Medline].

  28. Lukes RJ, Butler JJ. The pathology and nomenclature of Hodgkin's disease. Cancer Res. Jun 1966;26(6):1063-83. [Medline].

  29. Mikhaeel NG. Use of FDG-PET to monitor response to chemotherapy and radiotherapy in patients with lymphomas. Eur J Nucl Med Mol Imaging. Jul 2006;33 Suppl 1:22-6. [Medline].

  30. Koltin D, Uziel Y, Schneidermann D, Kotzki S, Wolach B, Fainmesser P. A case of Jatropha multifida poisoning resembling organophosphate intoxication. Clin Toxicol (Phila). 2006;44(3):337-8. [Medline].

  31. Kornstein MJ. Biological markers and pathology of mediastinal lymphomas. In: Shields TW, LoCicero J III, Ponn RB, eds. General Thoracic Surgery. Vol 2. 5th ed. Philadelphia, Pa: Williams & Wilkins; 2000:2257-66.

  32. Laurent F, Latrabe V, Lecesne R, et al. Mediastinal masses: diagnostic approach. Eur Radiol. 1998;8(7):1148-59. [Medline].

  33. Luketich JD, Ginsberg RJ. The current management of patients with mediastinal tumors. Adv Surg. 1996;30:311-32. [Medline].

  34. Shields TW. Lesions masquerading as primary mediastinal tumors or cysts. In: Shields TW, LoCicero J III, Ponn RB, eds. General Thoracic Surgery. Vol 2. 5th ed. Philadelphia, Pa: Williams & Wilkins; 2000:2115-34.

  35. Stein H, Hummel M. Histopathology in the light of molecular profiling. Ann Oncol. May 2006;17 Suppl 4:iv5-7. [Medline].

  36. Virgo KS, Johnson FE, Naunheim KS. Follow-up of patients with thoracic malignancies. Surg Oncol Clin N Am. Apr 1999;8(2):355-69. [Medline].

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Further Reading

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Keywords

lymphomas, endocrine tumors, mesenchymal tumors, Hodgkin disease of the mediastinum, Hodgkin's disease of the mediastinum, nodular sclerosing lymphoma of the mediastinum, substernal goiter, mediastinal parathyroid adenoma, mediastinal parathyroid carcinoma, mediastinal thyroid adenoma, mediastinal thyroid carcinoma, lipoma, liposarcoma, hemangioma, angioma, angiosarcoma, rhabdomyoma, rhabdomyosarcoma, fibroma, fibrosarcoma, malignant fibrous histiocytoma, lymphangiomas, lymphangiosarcomas, leiomyoma, leiomyosarcoma, mediastinal Hodgkin lymphoma, mediastinal Hodgkin's lymphoma, mediastinal neoplasm, mediastinal lymphoma, neurogenic tumor, thymic tumors, germ cell tumor, foregut cyst, pericardial cyst, lymphangiomas, tumors of the thymus, thymic tumors, mesenchymal tumors, parathyroid tumors, mesenchymal mass, parathyroid mass

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Contributor Information and Disclosures

Author

Mary C Mancini, MD, PhD, Professor and Chief, Cardiothoracic Surgery, Department of Surgery, Louisiana State University Health Sciences Center-Shreveport
Mary C Mancini, MD, PhD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons, and Southern Surgical Association
Disclosure: Nothing to disclose.

Medical Editor

Richard Thurer, MD, B and Donald Carlin Professor of Thoracic Surgical Oncology, Miller School of Medicine, University of Miami
Richard Thurer, MD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Chest Physicians, American College of Surgeons, American Medical Association, American Thoracic Society, Florida Medical Association, Society of Surgical Oncology, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Daniel S Schwartz, MD, FACS, Assistant Clinical Professor of Cardiothoracic Surgery, Mount Sinai School of Medicine; Chief of Thoracic Surgery, Huntington Hospital
Daniel S Schwartz, MD, FACS is a member of the following medical societies: American College of Chest Physicians, American College of Surgeons, Society of Thoracic Surgeons, and Western Thoracic Surgical Association
Disclosure: Nothing to disclose.

CME Editor

Rajalaxmi McKenna, MD, FACP, Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

John Geibel, MD, DSc, MA, Vice Chairman, Professor, Department of Surgery, Section of Gastrointestinal Medicine and Department of Cellular and Molecular Physiology, Yale University School of Medicine; Director of Surgical Research, Department of Surgery, Yale-New Haven Hospital
John Geibel, MD, DSc, MA is a member of the following medical societies: American Gastroenterological Association, American Physiological Society, American Society of Nephrology, Association for Academic Surgery, International Society of Nephrology, New York Academy of Sciences, and Society for Surgery of the Alimentary Tract
Disclosure: AMGEN Royalty Other

 
 
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