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__NOTOC__ | __NOTOC__ | ||
{{Thymoma}} | {{Thymoma}} | ||
{{CMG}}; {{AE}} {{AM}} {{AAM}} | I{{CMG}}; {{AE}} {{AM}} {{AAM}} | ||
==Overview== | ==Overview== | ||
* Thymoma is a [[benign]] 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. | |||
Thymoma is a [[benign]] 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. The incidence of thymoma is approximately 0.13 per 100,000 individuals. | * The incidence of thymoma is approximately 0.13 per 100,000 individuals. | ||
* Thymic neoplasm can be divided into two major groups: [[thymoma]] and [[thymic carcinoma]]thymoma | |||
Thymic neoplasm can be divided into two major groups: [[thymoma]] and [[thymic carcinoma]]thymoma | * Thymoma is the most common tumor of the anterior [[mediastinum]], consisting of any type of thymic [[epithelial cell]] as well as [[lymphocyte]]s that are usually abundant and probably not [[neoplastic]]. | ||
* Thymoma usually is [[benign]], and frequently encapsulated uncommon tumor, best known for its association with the autoimmune disorder such as [[myasthenia gravis]]. Thymoma is found in 15% of patients with [[myasthenia gravis]]. | |||
Thymoma is the most common tumor of the anterior [[mediastinum]], consisting of any type of thymic [[epithelial cell]] as well as [[lymphocyte]]s that are usually abundant and probably not [[neoplastic]]. Thymoma usually is [[benign]], and frequently encapsulated uncommon tumor, best known for its association with the autoimmune disorder such as [[myasthenia gravis]]. Thymoma is found in 15% of patients with [[myasthenia gravis]]. Once diagnosed, thymomas may be removed surgically. 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]]. [[Metastasis]] is extremely rare. In the rare case of a [[malignant tumor]], [[chemotherapy]] may be used. | * Once diagnosed, thymomas may be removed surgically. 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. | |||
Malignant [[lymphomas]] that involve the [[thymus]], e.g., [[lymphosarcoma]], [[Hodgkin's disease]] (termed "granulomatous thymoma" in the past), should not be regarded as thymoma. | * Common complications of the thymoma include the pressure effect of the mass itself, [[autoimmune diseases]], and rarely, [[malignancy]]. [[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. | |||
Thymomas associated with autoimmune disorders usually are benign. Malignant thymomas can [[metastasis|metastasize]], generally to [[pleura]], [[kidney]], [[bone]], [[liver]], or [[brain]]. | * Thymomas associated with autoimmune disorders usually are benign. Malignant thymomas can [[metastasis|metastasize]], generally to [[pleura]], [[kidney]], [[bone]], [[liver]], or [[brain]]. | ||
==Historical Perspective== | ==Historical Perspective== | ||
The thymic epithelial tumors staging was initially proposed by Bergh and his colleagues in 1978, | * The thymic epithelial tumors staging was initially proposed by Bergh and his colleagues in 1978, modified by Wilkins and Castleman in 1979, and advanced by Masaoka et al. in 1981. | ||
==Classification== | ==Classification== | ||
In 1999, a World Health Organization (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. | * In 1999, a World Health Organization (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. | |||
==Pathology== | ==Pathology== | ||
On [[gross pathology]], well circumscribed [[mass]], that is locally invasive is a characteristic finding of thymoma. On microscopic [[histopathological]] analysis, round cells, with ample vacuolated [[cytoplasm]]s, and fat droplets are characteristic findings of thymoma. | * On [[gross pathology]], well circumscribed [[mass]], that is locally invasive is a characteristic finding of thymoma. | ||
* On microscopic [[histopathological]] analysis, round cells, with ample vacuolated [[cytoplasm]]s, and fat droplets are characteristic findings of thymoma. | |||
==Causes== | ==Causes== | ||
There are no established causes for thymoma. | * There are no established causes for thymoma. | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
Thymoma must be differentiated from other thymic diseases such as [[thymic carcinoma]] | Thymoma must be differentiated from other thymic diseases such as | ||
* [[thymic carcinoma]] | |||
* Thymic [[cyst]] | |||
* Thymic [[hyperplasia]] | |||
* [[germ cell tumors]]. | |||
==Epidemiology and Demographic== | ==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. | * 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== | ==Risk Factors== | ||
There are no established risk factors for thymoma. | * There are no established risk factors for thymoma. | ||
==Natural History, Complication and Prognosis== | ==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. | * 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. | ||
* 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]]. | |||
==Diagnosis== | ==Diagnosis== | ||
===History and symptoms=== | ===History and symptoms=== | ||
Symptoms of thymoma include [[muscle weakness]] | Symptoms of thymoma include | ||
* [[muscle weakness]] | |||
* [[cough]] | |||
* [[wheezing]] | |||
* [[dysphagia]]. | |||
In addition to the symptoms of associated immune syndromes such as, | |||
* [[anemia]] | |||
* [[arthralgia]] | |||
* [[skin rash]]. | |||
===Physical examination=== | ===Physical examination=== | ||
Patients with thymoma usually appear asymptomatic. Physical examination of patients with thymoma is may be remarkable for | Patients with thymoma usually appear asymptomatic. Physical examination of patients with thymoma is may be remarkable for, | ||
* Neck lump, | |||
* Facial [[swelling]] | |||
* [[wheezing]]. | |||
===Staging=== | ===Staging=== | ||
Staging of thymic epithelial tumors was initially proposed by Bergh and his colleagues in 1978, | * Staging of thymic epithelial tumors was initially proposed by Bergh and his colleagues in 1978, modified by Wilkins and Castleman in 1979, and advanced by Masaoka et al. in 1981. | ||
* Modified Masaoka staging grouped with TNM classification is the most widely adopted system for thymic epithelial tumors currently in use. | |||
===Laboratory Findings=== | ===Laboratory Findings=== | ||
Laboratory findings associated with thymoma may include [[antibodies]] to the [[acetylcholine receptor]], | Laboratory findings associated with thymoma may include, | ||
* A[[antibodies|ntibodies]] to the [[acetylcholine receptor]], | |||
* Abnormal [[electrolyte]]s, [[renal]], and [[liver function]] tests. | |||
===X-Ray=== | ===X-Ray=== | ||
On chest x-ray, thymoma is characterized by oval to rounded, well demarcated, asymmetric, homogeneous mass of soft tissue density on one side of the midline. | * On chest x-ray, thymoma is characterized by oval to rounded, well demarcated, asymmetric, homogeneous mass of soft tissue density on one side of the midline. | ||
===CT Scan=== | ===CT Scan=== | ||
[[Computed Tomography]] scan may be diagnostic of thymoma. The tumor is generally located inside the thymus, and can be calcified. Increased vascular enhancement can be indicative of malignancy, as can be pleural deposits. | * [[Computed Tomography]] scan may be diagnostic of thymoma. The tumor is generally located inside the thymus, and can be calcified. | ||
* Increased vascular enhancement can be indicative of malignancy, as can be pleural deposits. | |||
===MRI=== | ===MRI=== | ||
On [[thoracic]] MRI, thymoma is characterized by increased heterogenous signal on T2WI. | * On [[thoracic]] MRI, thymoma is characterized by increased heterogenous signal on T2WI. | ||
===Ultrasound=== | ===Ultrasound=== | ||
Ultrasound is used to guide [[fine needle aspiration]] or core [[needle biopsy]] in patients with thymoma. | * Ultrasound is used to guide [[fine needle aspiration]] or core [[needle biopsy]] in patients with thymoma. | ||
===Other Imaging Studies=== | ===Other Imaging Studies=== | ||
[[PET scan]] may be used in the diagnosis of thymoma. | * [[PET scan]] may be used in the diagnosis of thymoma. | ||
===Other Diagnostic Studies=== | ===Other Diagnostic Studies=== | ||
Other diagnostic studies for Thymoma include CT scan guided core needle [[biopsy]] | Other diagnostic studies for Thymoma include | ||
* CT scan guided core needle [[biopsy]] | |||
* CT scan guided [[fine needle aspiration]] | |||
* [[mediastinoscopy]] | |||
* Videothoracoscopy. | |||
==Treatment== | ==Treatment== | ||
===Medical Therapy=== | ===Medical Therapy=== | ||
[[Chemotherapy]] and [[radiotherapy]] are used as [[adjuvant therapy|adjuvant]] or [[neoadjuvant therapy|neoadjuvant therapies]]. Neoadjuvant therpy may be administered prior to surgery to make the tumor resectable. | * [[Chemotherapy]] and [[radiotherapy]] are used as [[adjuvant therapy|adjuvant]] or [[neoadjuvant therapy|neoadjuvant therapies]]. | ||
* Neoadjuvant therpy may be administered prior to surgery to make the tumor resectable. | |||
===Surgery=== | ===Surgery=== | ||
Surgery is the mainstay of treatment of thymoma. | * Surgery is the mainstay of treatment of thymoma. | ||
===Primary Prevention=== | ===Primary Prevention=== | ||
There are no primary preventive measures available for thymoma. | * There are no primary preventive measures available for thymoma. | ||
===Secondary Prevention=== | ===Secondary Prevention=== | ||
Complete surgical resection may help to prevent the recurrence of thymoma. | * Complete surgical resection may help to prevent the recurrence of thymoma. | ||
==References== | ==References== | ||
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Latest revision as of 23:11, 31 May 2019
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IEditor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amr Marawan, M.D. [2] Ahmad Al Maradni, M.D. [3]
Overview
- Thymoma is a benign 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.
- The incidence of thymoma is approximately 0.13 per 100,000 individuals.
- Thymic neoplasm can be divided into two major groups: thymoma and thymic carcinomathymoma
- 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 autoimmune disorder such as myasthenia gravis. Thymoma is found in 15% of patients with myasthenia gravis.
- Once diagnosed, thymomas may be removed surgically. 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. 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.
- Thymomas associated with autoimmune disorders usually are benign. Malignant thymomas can metastasize, generally to pleura, kidney, bone, liver, or brain.
Historical Perspective
- The thymic epithelial tumors staging was initially proposed by Bergh and his colleagues in 1978, modified by Wilkins and Castleman in 1979, and advanced by Masaoka et al. in 1981.
Classification
- In 1999, a World Health Organization (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.
Pathology
- On gross pathology, well circumscribed mass, that is locally invasive is a characteristic finding of thymoma.
- On microscopic histopathological analysis, round cells, with ample vacuolated cytoplasms, and fat droplets are characteristic findings of thymoma.
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
- 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.
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.
- 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.
Diagnosis
History and symptoms
Symptoms of thymoma include
In addition to the symptoms of associated immune syndromes such as,
Physical examination
Patients with thymoma usually appear asymptomatic. Physical examination of patients with thymoma is may be remarkable for,
Staging
- Staging of thymic epithelial tumors was initially proposed by Bergh and his colleagues in 1978, modified by Wilkins and Castleman in 1979, and advanced by Masaoka et al. in 1981.
- Modified Masaoka staging grouped with TNM classification is the most widely adopted system for thymic epithelial tumors currently in use.
Laboratory Findings
Laboratory findings associated with thymoma may include,
- Antibodies to the acetylcholine receptor,
- Abnormal electrolytes, renal, and liver function tests.
X-Ray
- On chest x-ray, thymoma is characterized by oval to rounded, well demarcated, asymmetric, homogeneous mass of soft tissue density on one side of the midline.
CT Scan
- Computed Tomography scan may be diagnostic of thymoma. The tumor is generally located inside the thymus, and can be calcified.
- Increased vascular enhancement can be indicative of malignancy, as can be pleural deposits.
MRI
- On thoracic MRI, thymoma is characterized by increased heterogenous signal on T2WI.
Ultrasound
- Ultrasound is used to guide fine needle aspiration or core needle biopsy in patients with thymoma.
Other Imaging Studies
- PET scan may be used in the diagnosis of thymoma.
Other Diagnostic Studies
Other diagnostic studies for Thymoma include
- CT scan guided core needle biopsy
- CT scan guided fine needle aspiration
- mediastinoscopy
- Videothoracoscopy.
Treatment
Medical Therapy
- Chemotherapy and radiotherapy are used as adjuvant or neoadjuvant therapies.
- Neoadjuvant therpy may be administered prior to surgery to make the tumor resectable.
Surgery
- Surgery is the mainstay of treatment of thymoma.
Primary Prevention
- There are no primary preventive measures available for thymoma.
Secondary Prevention
- Complete surgical resection may help to prevent the recurrence of thymoma.