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Thymomas associated with autoimmune disorders usually are benign. Malignant thymomas can [[metastasis|metastasize]], generally to [[pleura]], [[kidney]], [[bone]], [[liver]] or [[brain]].<ref name="pmid10561285">{{cite journal |author=Thomas CR, Wright CD, Loehrer PJ |title=Thymoma: state of the art |journal=[[Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology]] |volume=17 |issue=7 |pages=2280–9 |year=1999 |month=July |pmid=10561285 |doi= |url=http://www.jco.org/cgi/pmidlookup?view=long&pmid=10561285 |accessdate=2012-01-18}}</ref>
Thymomas associated with autoimmune disorders usually are benign. Malignant thymomas can [[metastasis|metastasize]], generally to [[pleura]], [[kidney]], [[bone]], [[liver]] or [[brain]].<ref name="pmid10561285">{{cite journal |author=Thomas CR, Wright CD, Loehrer PJ |title=Thymoma: state of the art |journal=[[Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology]] |volume=17 |issue=7 |pages=2280–9 |year=1999 |month=July |pmid=10561285 |doi= |url=http://www.jco.org/cgi/pmidlookup?view=long&pmid=10561285 |accessdate=2012-01-18}}</ref>
==Historical Perspective==
The thymic epithelial tumors staging was initially proposed by Bergh and his colleagues in 1978,<ref name="Bergh-1978">{{Cite journal  | last1 = Bergh | first1 = NP. | last2 = Gatzinsky | first2 = P. | last3 = Larsson | first3 = S. | last4 = Lundin | first4 = P. | last5 = Ridell | first5 = B. | title = Tumors of the thymus and thymic region: I. Clinicopathological studies on thymomas. | journal = Ann Thorac Surg | volume = 25 | issue = 2 | pages = 91-8 | month = Feb | year = 1978 | doi =  | PMID = 626543 }}</ref> modified by Wilkins and Castleman in 1979,<ref name="Wilkins-1979">{{Cite journal  | last1 = Wilkins | first1 = EW. | last2 = Castleman | first2 = B. | title = Thymoma: a continuing survey at the Massachusetts General Hospital. | journal = Ann Thorac Surg | volume = 28 | issue = 3 | pages = 252-6 | month = Sep | year = 1979 | doi =  | PMID = 485626 }}</ref> and advanced by Masaoka et al. in 1981.<ref name="Masaoka-1981">{{Cite journal  | last1 = Masaoka | first1 = A. | last2 = Monden | first2 = Y. | last3 = Nakahara | first3 = K. | last4 = Tanioka | first4 = T. | title = Follow-up study of thymomas with special reference to their clinical stages. | journal = Cancer | volume = 48 | issue = 11 | pages = 2485-92 | month = Dec | year = 1981 | doi =  | PMID = 7296496 }}</ref><ref name="Kondo-2005">{{Cite journal  | last1 = Kondo | first1 = K. | title = Invited commentary. | journal = Ann Thorac Surg | volume = 80 | issue = 6 | pages = 2000-1 | month = Dec | year = 2005 | doi = 10.1016/j.athoracsur.2005.08.053 | PMID = 16305832 }}</ref>
==Classification==
In 1999, a WHO Working group suggested a non-committal terminology (Masaoka classification), preserving the distinct categories of the histogenetic classification, but using letters and numbers to designate tumour entities. Recently, it has been very well accepted as it provides an easy comparison of clinical, pathological and immunological studies.<ref>{{Cite web  | last =  | first =  | title = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | url = http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf | publisher =  | date =  | accessdate = }}</ref>
==Pathology==
On gross pathology, well circumscribed mass, that is locally invasive is a characteristic finding of thymoma. On gross pathology, well circumscribed mass, that is locally invasive is a characteristic
==Causes==
There are no established causes for thymoma.
==Differential Diagnosis==
Thymoma must be differentiated from other thymic diseases such as [[thymic carcinoma]], thymic [[cyst]], thymic [[hyperplasia]] and [[germ cell tumors]].
==Epidemiology and Demographic==
The incidence of thymoma is approximately 0.13 per 100,000 individuals. Thymic neoplasms are the most common tumors located in the anterior mediastinum (20%). Incidence increases in middle age, and peaks in the seventh decade of life. Men and women are equally affected.
==Risk Factors==
There are no established risk factors for thymoma.<ref name="Engels-2010">{{Cite journal  | last1 = Engels | first1 = EA. | title = Epidemiology of thymoma and associated malignancies. | journal = J Thorac Oncol | volume = 5 | issue = 10 Suppl 4 | pages = S260-5 | month = Oct | year = 2010 | doi = 10.1097/JTO.0b013e3181f1f62d | PMID = 20859116 }}</ref>
==Natural History, Complication and Prognosis==
If left untreated thymoma may progress to invade the [[mediastinum]] and the surrounding structure. Depending on the stage of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good. Common complications of the thymoma include the pressure effect of the mass itself, autoimmune diseases, and rarely, malignancy.
==Signs and symptoms==
Symptoms of thymoma include [[muscle weakness]], [[cough]], [[wheezing]], and [[dysphagia]]. In addition to the symptoms of associated immune syndromes such as [[anemia]], [[arthralgia]], and [[skin rash]].


====Pathophysiology====
In 1999, a WHO Working group suggested a non-committal terminology (Masaoka classification), preserving the distinct categories of the histogenetic classification, but using letters and numbers to designate tumour entities. Recently, it has been very well accepted as it provides an easy comparison of clinical, pathological and immunological studies.
====Epidemiology and Demographics====
Thymic neoplasms are the most common tumors located in the anterior mediastinum (20%). Thymoma is a rare malignancy of unknown etiology that accounts for 0.2–1.5% of all malignancies. It's incidence in the U.S. population is 0.13 per 100,000 person-years.
====Risk Factors====
Most available evidence regarding risk factors for thymoma derives from descriptive epidemiologic studies of thymoma and associated malignancies. Unfortunately, no available data concerning the role of occupation, environmental exposures, or diet and nutrition.
====Complications and Prognosis====
The main prognostic factors for recurrence and survival are the stage at diagnosis and whether a complete resection has been achieved. Other prognostic factors with less impact on prognosis are the histological subtype, tumour size at diagnosis, age, gender and presence or absence of myasthenia gravis.


The natural history of thymoma certainly illustrates the tendency for local mediastinal recurrence and pleural ‘‘droplet’’ recurrence presumably caused by mediastinal pleural invasion after resection. Local recurrences have been noted in the surgical incision used to completely remove a thymoma.
====Diagnosis====
One-third of patients have their tumors discovered because they have an associated autoimmune disorder. The most common of those conditions is myasthenia gravis (MG); 10–15% of patients with MG have a thymoma and, conversely, 30–45% of patients with thymomas have MG.
Historically, a classifiaction system used both histological features and clinical behavior. Masaoka staging is the clinically used staging system, although it may not be predictive for thymic carcinoma. GETT staging is the surgical staging system, and may have better correlation with outcome.
Computed Tomography is the radiologic modality of choice to diagnose and evaluate thymoma.
====Treatment====
Surgery is the only curative treatment. Surgical biopsy should be avoided if a thymoma is highly suspecte based on clinical and radiological evaluation.The goal of surgery is complete resection of the tumor by total thymectomy and complete resection of the contiguous and noncontiguous disease. Chemo and radio therapy are used as adjuvant or neoadjuvant therapies. Sometimes they are used prior to surgery to make the tumor resectable. Signs and symptoms of myasthenia gravis should be evaluated before surgery and medically controlled.
<gallery>
Image:T008.png|MEDIASTINUM: ENCAPSULATED CYSTIC [[THYMOMA]]. Post-formalin-fixed cut surface reveals an ivory-colored tumor with multiple cystic spaces varying from pin-head sized to a few centimeters in diameter. Small hemorrhages are also noted.
</gallery>


==References==
==References==

Revision as of 18:07, 23 September 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amr Marawan, M.D. [2]

Overview

Thymoma is a beingin thymic neoplasm located in the anterior mediastinum, behind the sternum and in front of the great vessels that involutes during puberty, it takes part in lymphocytes maturation throughout adulthood. Thymic malignancies count for less than 0.13/100,000/year.

They can be divided into two major groups: thymoma and thymic carcinoma. Prognosis and proper management are essentially determined by pathologic classification. Surgery is the only curative treatment, but they can be sensitive to chemotherapy and radiotherapy especially in unresectable disease.

Thymoma is the most common tumor of the anterior mediastinum, consisting of any type of thymic epithelial cell as well as lymphocytes that are usually abundant and probably not neoplastic. Thymoma usually is benign, and frequently encapsulated uncommon tumor, best known for its association with the neuromuscular disorder myasthenia gravis. Thymoma is found in 15% of patients with myasthenia gravis. Once diagnosed, thymomas may be removed surgically. When occasionally malignant, then it is invasive: metastasis is extremely rare. In the rare case of a malignant tumor, chemotherapy may be used.

Malignant lymphomas that involve the thymus, e.g., lymphosarcoma, Hodgkin's disease (termed "granulomatous thymoma" in the past), should not be regarded as thymoma.[1]

Thymomas associated with autoimmune disorders usually are benign. Malignant thymomas can metastasize, generally to pleura, kidney, bone, liver or brain.[2]

Historical Perspective

The thymic epithelial tumors staging was initially proposed by Bergh and his colleagues in 1978,[3] modified by Wilkins and Castleman in 1979,[4] and advanced by Masaoka et al. in 1981.[5][6]

Classification

In 1999, a WHO Working group suggested a non-committal terminology (Masaoka classification), preserving the distinct categories of the histogenetic classification, but using letters and numbers to designate tumour entities. Recently, it has been very well accepted as it provides an easy comparison of clinical, pathological and immunological studies.[7]

Pathology

On gross pathology, well circumscribed mass, that is locally invasive is a characteristic finding of thymoma. On gross pathology, well circumscribed mass, that is locally invasive is a characteristic

Causes

There are no established causes for thymoma.

Differential Diagnosis

Thymoma must be differentiated from other thymic diseases such as thymic carcinoma, thymic cyst, thymic hyperplasia and germ cell tumors.

Epidemiology and Demographic

The incidence of thymoma is approximately 0.13 per 100,000 individuals. Thymic neoplasms are the most common tumors located in the anterior mediastinum (20%). Incidence increases in middle age, and peaks in the seventh decade of life. Men and women are equally affected.

Risk Factors

There are no established risk factors for thymoma.[8]

Natural History, Complication and Prognosis

If left untreated thymoma may progress to invade the mediastinum and the surrounding structure. Depending on the stage of the tumor at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good. Common complications of the thymoma include the pressure effect of the mass itself, autoimmune diseases, and rarely, malignancy.

Signs and symptoms

Symptoms of thymoma include muscle weakness, cough, wheezing, and dysphagia. In addition to the symptoms of associated immune syndromes such as anemia, arthralgia, and skin rash.


References

  1. Thomas CR, Wright CD, Loehrer PJ (1999). "Thymoma: state of the art". J. Clin. Oncol. 17 (7): 2280–9. PMID 10561285.
  2. Thomas CR, Wright CD, Loehrer PJ (1999). "Thymoma: state of the art". Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology. 17 (7): 2280–9. PMID 10561285. Retrieved 2012-01-18. Unknown parameter |month= ignored (help)
  3. Bergh, NP.; Gatzinsky, P.; Larsson, S.; Lundin, P.; Ridell, B. (1978). "Tumors of the thymus and thymic region: I. Clinicopathological studies on thymomas". Ann Thorac Surg. 25 (2): 91–8. PMID 626543. Unknown parameter |month= ignored (help)
  4. Wilkins, EW.; Castleman, B. (1979). "Thymoma: a continuing survey at the Massachusetts General Hospital". Ann Thorac Surg. 28 (3): 252–6. PMID 485626. Unknown parameter |month= ignored (help)
  5. Masaoka, A.; Monden, Y.; Nakahara, K.; Tanioka, T. (1981). "Follow-up study of thymomas with special reference to their clinical stages". Cancer. 48 (11): 2485–92. PMID 7296496. Unknown parameter |month= ignored (help)
  6. Kondo, K. (2005). "Invited commentary". Ann Thorac Surg. 80 (6): 2000–1. doi:10.1016/j.athoracsur.2005.08.053. PMID 16305832. Unknown parameter |month= ignored (help)
  7. "http://www.iarc.fr/en/publications/pdfs-online/pat-gen/bb10/BB10.pdf" (PDF). External link in |title= (help)
  8. Engels, EA. (2010). "Epidemiology of thymoma and associated malignancies". J Thorac Oncol. 5 (10 Suppl 4): S260–5. doi:10.1097/JTO.0b013e3181f1f62d. PMID 20859116. Unknown parameter |month= ignored (help)


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