Sections

Pediatric Thymoma

Follow-up

Complications

Complications may include the following:

Structural problems, such as compression syndromes that involve the bronchi or lungs or superior vena cava syndrome (SVCS), can occur from local spread of benign thymoma, from thymic cysts, or from thymic carcinoma. Presenting symptoms may include chest pain, SVCS, dyspnea, dysphagia, and cough.
Areas of benign thymoma can become highly vascular or necrotic and lead to bleeding.
Clinical manifestations include paraneoplastic syndromes and immunodeficiency.

Prognosis

Adverse predictive factors include the following:^{[43, 44]}

Invasive or metastatic tumor
Tracheal or vascular compression
Age younger than 30 years
Epithelial or mixed histology
Tumor size of more than 8 cm^[45]

The presence of myasthenia gravis (MG) with thymomas is no longer considered a poor prognostic factor and is actually thought to be a favorable prognostic factor.^[46]

Based on WHO classification, the 5-year and 10-year survival rates are as follows:^{[47, 48]}

Type A - 100% and 95%, respectively
Type AB - 93% and 90%, respectively
Type B1 - 89% and 85%, respectively
Type B2 - 82% and 71%, respectively
Type B3 - 71% and 40%, respectively
Type C - 23% (5-year survival rate)

Recurrence of thymoma can occur after resection. A study surgical outcomes after recurrence of thymic epithelial tumors in 67 patients showed an overall survival rate at 10 years of 70% in those undergoing re-resection.^[32]

For patients with Masaoka stage IVA thymomas, a study of 18 patients reported survival rates at 3 years (91%), 5 years (78%), and 10 years (65%).^[49]These patients underwent multimodality therapy, including surgical resection, preoperative chemotherapy, and even postoperative radiation therapy (in select patients).

Stites DP. Diseases of the thymus. Wyngaarden J, Smith LH, eds. Cecil Textbook of Medicine. WB Saunders Co; 1988.
Rich RR. Clinical Immunology, Principles and Practice. 2nd ed. Mosby; 1996.
Roitt IM, Brostoff J, Male DK. Immunology. 5th ed. Mosby; 1998.
Temes R, Chavez T, Mapel D, et al. Primary mediastinal malignancies: findings in 219 patients. West J Med. 1999 Mar. 170(3):161-6. [QxMD MEDLINE Link].
Agarwala SS. Paraneoplastic syndromes. Med Clin North Am. 1996 Jan. 80(1):173-84. [QxMD MEDLINE Link].
Mygland A, Vincent A, Newsom-Davis J, et al. Autoantibodies in thymoma-associated myasthenia gravis with myositis or neuromyotonia. Arch Neurol. 2000 Apr. 57(4):527-31. [QxMD MEDLINE Link].
Fauci AS, Braunwald E, Isselbacher KJ. Harrison’s Principles of Internal Medicine. 14th ed. McGraw-Hill; 1998.
Hart IK. Acquired neuromyotonia: a new autoantibody-mediated neuronal potassium channelopathy. Am J Med Sci. 2000 Apr. 319(4):209-16. [QxMD MEDLINE Link].
Evoli A, Minicuci GM, Vitaliani R, et al. Paraneoplastic diseases associated with thymoma. J Neurol. 2007 Jun. 254(6):756-62. [QxMD MEDLINE Link].
Takamori M, Komai K, Iwasa K. Antibodies to calcium channel and synaptotagmin in Lambert-Eaton myasthenic syndrome. Am J Med Sci. 2000 Apr. 319(4):204-8. [QxMD MEDLINE Link].
Ammann AJ, Stiehm ER, Roberts RL. Antibody (B-cell) immunodeficiency disorders. Stites DP, Terr A, eds. Medical Immunology. Appleton & Lange; 1997.
Casadevall N, Dupuy E, Molho-Sabatier P, et al. Autoantibodies against erythropoietin in a patient with pure red-cell aplasia. N Engl J Med. 1996 Mar 7. 334(10):630-3. [QxMD MEDLINE Link].
Frank K, Austin M. Samter’s Immunologic Diseases. Little Brown & Co; 1995.
Gay CM, William WN Jr, Wang SA, Oo TH. Thymoma complicated by acquired amegakaryocytic thrombocytopenia and pure red cell aplasia. J Natl Compr Canc Netw. 2014 Nov. 12(11):1505-9. [QxMD MEDLINE Link].
Strollo DC, Rosado de Christenson ML, Jett JR. Primary mediastinal tumors. Part 1: tumors of the anterior mediastinum. Chest. 1997 Aug. 112(2):511-22. [QxMD MEDLINE Link].
Stiehm ER. Immunologic disorders in infants and children. Ochs HD, Winkelstein J, eds. Disorders of the B-Cell System. 4th ed. 1996. 320.
Watts RG, Kelly DR. Fatal varicella infection in a child associated with thymoma and immunodeficiency (Good's syndrome). Med Pediatr Oncol. 1990. 18(3):246-51. [QxMD MEDLINE Link].
Middleton E, Reed CE, Ellis EF, et al, eds. Allergy: Principles and Practice. 5th ed. Mosby; 1998.
Loehrer PJ Sr. Current approaches to the treatment of thymoma. Ann Med. 1999 Oct. 31 Suppl 2:73-9. [QxMD MEDLINE Link].
Weissferdt A, Kalhor N, Bishop JA, et al. Thymoma: a clinicopathological correlation of 1470 cases. Hum Pathol. 2018 Mar. 73:7-15. [QxMD MEDLINE Link].
Takanami I, Takeuchi K, Naruke M. Noninvasive large thymoma with a natural history of twenty-one years. J Thorac Cardiovasc Surg. 1999 Dec. 118(6):1134-5. [QxMD MEDLINE Link].
Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol. 2005 May. 94(5 Suppl 1):S1-63. [QxMD MEDLINE Link].
Kelleher P, Misbah SA. What is Good's syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol. 2003 Jan. 56(1):12-6. [QxMD MEDLINE Link].
Camera L, Brunetti A, Romano M, et al. Morphological imaging of thymic disorders. Ann Med. 1999 Oct. 31 Suppl 2:57-62. [QxMD MEDLINE Link].
Lastoria S, Palmieri G, Muto P, Lombardi G. Functional imaging of thymic disorders. Ann Med. 1999 Oct. 31 Suppl 2:63-9. [QxMD MEDLINE Link].
Panitch HS. Neurologic diseases. Stites DP, Terr AI, Parslow TG, eds. Medical Immunology. Appleton & Lange; 1997.
Muller-Hermelink HK, Marx A. Pathological aspects of malignant and benign thymic disorders. Ann Med. 1999 Oct. 31 Suppl 2:5-14. [QxMD MEDLINE Link].
Bott-Kothari T, Aron BS, Bejarano P. Malignant thymoma with metastases to the gastrointestinal tract and ovary: a case report and literature review. Am J Clin Oncol. 2000 Apr. 23(2):140-2. [QxMD MEDLINE Link].
Das A, Pandit S, Choudhury S, Das SK, Basuthakur S. Superior vena caval syndrome and ipsilateral pleural effusion: A rare presentation of anterior mediastinal thymoma. Lung India. 2014 Oct. 31(4):383-6. [QxMD MEDLINE Link].
Kim DJ, Yang WI, Choi SS. Prognostic and clinical relevance of the World Health Organization schema for the classification of thymic epithelial tumors: a clinicopathologic study of 108 patients and literature review. Chest. 2005 Mar. 127(3):755-61. [QxMD MEDLINE Link].
[Guideline] NCCN Clinical Practice Guidelines in Oncology. Thymomas and Thymic Carcinomas. V.2.2012. 11/9/2011. [Full Text].
Okumura M, Shiono H, Inoue M, et al. Outcome of surgical treatment for recurrent thymic epithelial tumors with reference to world health organization histologic classification system. J Surg Oncol. 2007 Jan 1. 95(1):40-4. [QxMD MEDLINE Link].
Werneck LC, Cunha FM, Scola RH. Myasthenia gravis: a retrospective study comparing thymectomy to conservative treatment. Acta Neurol Scand. 2000 Jan. 101(1):41-6. [QxMD MEDLINE Link].
Raschal S, Siegel JN, Huml J, Richmond GW. Hypogammaglobulinemia and anemia 18 years after thymoma resection. J Allergy Clin Immunol. 1997 Dec. 100(6 Pt 1):846-8. [QxMD MEDLINE Link].
Detterbeck FC, Zeeshan A. Thymoma: current diagnosis and treatment. Chin Med J (Engl). 2013. 126(11):2186-91. [QxMD MEDLINE Link].
Davenport E, Malthaner RA. The role of surgery in the management of thymoma: a systematic review. Ann Thorac Surg. 2008 Aug. 86(2):673-84. [QxMD MEDLINE Link].
Fornasiero A, Daniele O, Ghiotto C, et al. Chemotherapy of invasive thymoma. J Clin Oncol. 1990 Aug. 8(8):1419-23. [QxMD MEDLINE Link].
Macchiarini P, Chella A, Ducci F, et al. Neoadjuvant chemotherapy, surgery, and postoperative radiation therapy for invasive thymoma. Cancer. 1991 Aug 15. 68(4):706-13. [QxMD MEDLINE Link].
Loehrer PJ Sr, Chen M, Kim K, et al. Cisplatin, doxorubicin, and cyclophosphamide plus thoracic radiation therapy for limited-stage unresectable thymoma: an intergroup trial. J Clin Oncol. 1997 Sep. 15(9):3093-9. [QxMD MEDLINE Link].
Venuta F, Rendina EA, Pescarmona EO, et al. Multimodality treatment of thymoma: a prospective study. Ann Thorac Surg. 1997 Dec. 64(6):1585-91; discussion 1591-2. [QxMD MEDLINE Link].
Kunitoh H, Tamura T, Shibata T, Takeda K, Katakami N, Nakagawa K, 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. June 29, 2010. 103(1):6-11. [QxMD MEDLINE Link].
Palmieri G, Lastoria S, Montella L, et al. Role of somatostatin analogue-based therapy in unresponsive malignant thymomas. Ann Med. 1999 Oct. 31 Suppl 2:80-5. [QxMD MEDLINE Link].
Verley JM, Hollmann KH. Thymoma. A comparative study of clinical stages, histologic features, and survival in 200 cases. Cancer. 1985 Mar 1. 55(5):1074-86. [QxMD MEDLINE Link].
Pollack A, Komaki R, Cox JD. Thymoma: treatment and prognosis. Int J Radiat Oncol Biol Phys. 1992. 23(5):1037-43. [QxMD MEDLINE Link].
Wright CD, Wain JC, Wong DR. Predictors of recurrence in thymic tumors: importance of invasion, World Health Organization histology, and size. J Thorac Cardiovasc Surg. 2005 Nov. 130(5):1413-21. [QxMD MEDLINE Link].
Kondo K, Monden Y. Thymoma and myasthenia gravis: a clinical study of 1,089 patients from Japan. Ann Thorac Surg. 2005 Jan. 79(1):219-24. [QxMD MEDLINE Link].
Park MS, Chung KY, Kim KD. Prognosis of thymic epithelial tumors according to the new World Health Organization histologic classification. Ann Thorac Surg. 2004 Sep. 78(3):992-7; discussion 997-8. [QxMD MEDLINE Link].
Rena O, Papalia E, Maggi G. World Health Organization histologic classification: an independent prognostic factor in resected thymomas. Lung Cancer. 2005 Oct. 50(1):59-66. [QxMD MEDLINE Link].
Huang J, Rizk NP, Travis WD, et al. Feasibility of multimodality therapy including extended resections in stage IVA thymoma. J Thorac Cardiovasc Surg. 2007 Dec. 134(6):1477-83; discussion 1483-4. [QxMD MEDLINE Link].
Takeda S, Miyoshi S, Akashi A. Clinical spectrum of primary mediastinal tumors: a comparison of adult and pediatric populations at a single Japanese institution. J Surg Oncol. 2003 May. 83(1):24-30. [QxMD MEDLINE Link].
Curran WJ Jr, Kornstein MJ, Brooks JJ, Turrisi AT 3rd. Invasive thymoma: the role of mediastinal irradiation following complete or incomplete surgical resection. J Clin Oncol. 1988 Nov. 6(11):1722-7. [QxMD MEDLINE Link].
Goldman L, Cecil RL, Bennett JC, eds. Cecil Textbook of Internal Medicine. WB Saunders Co; 2000.
Graeber GM, Tamim W. Current status of the diagnosis and treatment of thymoma. Semin Thorac Cardiovasc Surg. 2000 Oct. 12(4):268-77. [QxMD MEDLINE Link].
Inoue M, Okumura M, Miyoshi S, et al. Impaired expression of MHC class II molecules in response to interferon- gamma (IFN-gamma) on human thymoma neoplastic epithelial cells. Clin Exp Immunol. 1999 Jul. 117(1):1-7. [QxMD MEDLINE Link].
Monden Y, Nakahara K, Iioka S, et al. Recurrence of thymoma: clinicopathological features, therapy, and prognosis. Ann Thorac Surg. 1985 Feb. 39(2):165-9. [QxMD MEDLINE Link].
Spigland N, Di Lorenzo M, Youssef S, et al. Malignant thymoma in children: a 20-year review. J Pediatr Surg. 1990 Nov. 25(11):1143-6. [QxMD MEDLINE Link].
Stephan JL, Galambrun C, Boucheron S, et al. Epstein-Barr virus--positive undifferentiated thymic carcinoma in a 12- year-old white girl. J Pediatr Hematol Oncol. 2000 Mar-Apr. 22(2):162-6. [QxMD MEDLINE Link].

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Table 1. Comparison of the Different Classifications of Thymic Epithelial Tumors ^[27]

Clinicopathologic Classification

WHO Type

Terminology of the Histogenetic Classification for the Histologic Subtypes of Thymic Epithelial Tumors

Benign thymoma

Medullary thymoma

Mixed thymoma

Malignant thymomas,

Category I

Predominantly cortical thymoma

Cortical thymoma

Well-differentiated thymic carcinoma

Malignant thymomas,

Category II

Epidermoid keratinizing (squamous cell) carcinoma

Epidermoid nonkeratinizing carcinoma

Lymphoepithelioma-like carcinoma

Sarcomatoid carcinoma (carcinosarcoma)

Clear cell carcinoma

Mucoepidermoid carcinoma

Undifferentiated carcinoma

Table. Macchiarini et al (1991) ^[38]

Cisplatin	75 mg/m² on day 1	3 courses repeated q3wk
Epirubicin	100 mg/m² on day 1
Etoposide	120 mg/m² on days 1, 3, and 5
Surgery and radiation in patients with complete or partial response to chemotherapy	4500 cGy if complete resection 6000 cGy if incomplete resection

Table. Loehrer et al (1997) ^[39]

Cisplatin	50 mg/m²	2-4 cycles q3wk
Doxorubicin	50 mg/m²
Cyclophosphamide	500 mg/m²
Followed by radiation	54 Gy to the primary tumor and lymph nodes

Table. Venuta et al (1997) ^[40]

Cisplatin	75-100 mg/m² on day 1	Repeated q3wk 3 times before surgery and 2 or 3 times after surgery
Epirubicin hydrochloride	100 mg/m² on day 1
Etoposide	120 mg/m² on days 1, 3, and 5
Postoperative radiation in patients with radical resection	30 Gy	Delivered in 3 wk with 5 fractions per wk
Postoperative radiation in patients with incomplete resection	50 Gy	Delivered in 5 wk with 5 fractions per wk

Table. Palmieri et al (1999) ^[42]

Octreotide

1.5 mg/d SC

In patients shown to have

somatostatin receptors

Lanreotide

30 mg/d SC q14d

Switch to this longer-acting

somatostatin analogue or depot form of octreotide if short-acting octreotide

is well tolerated

Prednisone

0.6 mg/kg/d PO

for 3 mo, then

decreasing to 0.2 mg/kg

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Pediatric Thymoma Follow-up

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