View source for Thymoma

{{Infobox medical condition (new)
| name            = Thymoma
| synonyms        = 
| image           = Encapsulated thymoma.jpg
| caption         = An encapsulated thymoma (mixed lymphocytic and epithelial type)
| pronounce       = 
| field           = [[Oncology]], [[cardiothoracic surgery]]
| symptoms        = 
| complications   = 
| onset           = [[Adulthood]]
| duration        = 
| types           = 
| causes          = 
| risks           =
| diagnosis       = 
| differential    = 
| prevention      = 
| treatment       = surgical removal, [[chemotherapy]] (in malignant cases).
| medication      = 
| prognosis       = 
| frequency       = 
| deaths          = 
}}

A '''thymoma''' is a [[tumor]] originating from the [[epithelial cell]]s of the [[thymus]] that is considered a rare neoplasm.<ref>WHO classification of tumors editorial Board. Thoracic Tumors. WHO Classification of Tumors. Lyon: IARC; 2021.</ref> Thymomas are frequently associated with neuromuscular disorders such as  [[myasthenia gravis]];<ref name=Thomas1999>{{cite journal | vauthors = Thomas CR, Wright CD, Loehrer PJ |author3-link=Patrick J. Loehrer | title = Thymoma: state of the art | journal = Journal of Clinical Oncology | volume = 17 | issue = 7 | pages = 2280–9 | date = July 1999 | pmid = 10561285 | doi = 10.1200/JCO.1999.17.7.2280 |s2cid=12621667 }}</ref> thymoma is found in 20% of patients with myasthenia gravis.<ref name="isbn1-4160-2973-7">{{cite book |author1=Mitchell, Richard Sheppard |author2=Kumar, Vinay |author3=Robbins, Stanley L. |author4=Abbas, Abul K. |author5=Fausto, Nelson |title=Robbins basic pathology |publisher=Saunders/Elsevier |year=2007 |isbn=978-1-4160-2973-1 }}{{pn|date=May 2018}}</ref> Once diagnosed, thymomas may be removed surgically. In the rare case of a malignant tumor, [[radiation therapy]] may be used.

==Signs and symptoms==
A third of all people with a thymoma have symptoms caused by compression of the surrounding organs by an expansive mass. These problems may take the form of [[superior vena cava syndrome]], [[dysphagia]] (difficulty swallowing), [[cough]], or [[chest pain]].<ref name=Thomas1999/>

One-third of patients have their tumors discovered because they have an associated [[autoimmune disorder]]. As mentioned earlier, 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. Additional associated autoimmune conditions include [[thymoma-associated multiorgan autoimmunity]], [[pure red cell aplasia]] and [[Good syndrome]] (thymoma with [[combined immunodeficiency]] and [[hypogammaglobulinemia]]). Other reported disease associations are with [[acute pericarditis]], [[agranulocytosis]], [[alopecia areata]], [[ulcerative colitis]], [[Cushing's disease]], [[hemolytic anemia]], [[limbic encephalopathy]], [[myocarditis]], [[nephrotic syndrome]], [[panhypopituitarism]], [[pernicious anemia]], [[polymyositis]], [[rheumatoid arthritis]], [[sarcoidosis]], [[scleroderma]], sensorimotor radiculopathy, ''[[stiff person syndrome]]'', [[systemic lupus erythematosus]] and [[thyroiditis]].<ref name=Thomas1999/><ref>{{cite journal | vauthors = Bernard C, Frih H, Pasquet F, Kerever S, Jamilloux Y, Tronc F, Guibert B, Isaac S, Devouassoux M, Chalabreysse L, Broussolle C, Petiot P, Girard N, Sève P | title = Thymoma associated with autoimmune diseases: 85 cases and literature review | journal = Autoimmunity Reviews | volume = 15 | issue = 1 | pages = 82–92 | date = January 2016 | pmid = 26408958 | doi = 10.1016/j.autrev.2015.09.005 }}</ref>

One-third to one-half of all persons with thymoma have no symptoms at all, and the mass is identified on a [[chest X-ray]] or [[CT scan|CT/CAT scan]] performed for an unrelated problem.<ref name=Thomas1999/>

==Pathology==
Thymoma originates from the [[epithelium|epithelial]] cell population in the thymus, and several microscopic subtypes are now recognized.<ref name=Thomas1999/> There are three principal histological types of thymoma, depending on the appearance of the cells by microscopy:

* Type A if the epithelial cells have an oval or fusiform shape (less lymphocyte count);
* Type B if they have an epithelioid{{clarify|date=November 2015}} shape (Type B has three subtypes: B1 (lymphocyte-rich), B2 (cortical) and B3 (epithelial).);<ref>{{cite journal | vauthors = Dadmanesh F, Sekihara T, Rosai J | title = Histologic typing of thymoma according to the new World Health Organization classification | journal = Chest Surgery Clinics of North America | volume = 11 | issue = 2 | pages = 407–20 | date = May 2001 | doi = 10.1016/S1052-3359(25)00577-0 | pmid = 11413764 }}</ref>
* Type AB if the tumor contains a combination of both cell types.

Thymic cortical epithelial cells have abundant cytoplasm, vesicular nucleus with finely divided chromatin and small nucleoli and cytoplasmic filaments contact adjacent cells.
Thymic medullary epithelial cells in contrast are spindle shaped with oval dense nucleus and scant cytoplasm
thymoma if recapitulates cortical cell features more, is thought to be less benign.

==Diagnosis==
[[Image:Anterior mediastinal mass thymoma diagram.jpg|thumb|[[CT scan]] of the chest revealing a large [[necrosis|necrotic]] mass in the left [[anterior]] [[mediastinum]] (indicated by the red line). [[Histology]] later established the diagnosis of a thymoma.]]
[[Image:Tumor Thymoma1.JPG|thumb|Another axial slice of a [[CT scan]] of the chest showing a small thymoma anterior to the heart (marked with the red line).]]

When a thymoma is suspected, a [[Computed axial tomography|CT/CAT scan]] is generally performed to estimate the size and extent of the tumor, and the lesion is sampled with a CT-guided needle [[biopsy]]. Increased vascular enhancement on CT scans can be indicative of malignancy, as can be pleural deposits.<ref name=Thomas1999/>  Limited{{clarify|date=November 2015}} biopsies are associated with a very small risk of [[pneumomediastinum]] or [[mediastinitis]] and an even-lower risk of damaging the [[heart]] or large blood vessels. Sometimes thymoma metastasize for instance to the abdomen.<ref>{{cite journal | vauthors = van Geffen WH, Sietsma J, Roelofs PM, Hiltermann TJ | title = A malignant retroperitoneal mass – a rare presentation of recurrent thymoma | journal = BMJ Case Reports | volume = 2011 | pages = bcr0920114737 | date = December 2011 | pmid = 22674945 | pmc = 3229325 | doi = 10.1136/bcr.09.2011.4737 }}</ref>

The diagnosis is made via histologic examination by a pathologist, after obtaining a tissue sample of the mass.  Final tumor classification and staging is accomplished pathologically after formal{{clarify|date=November 2015}} surgical removal of the thymic tumor.

Selected laboratory tests can be used to look for associated problems or possible tumor spread. These include: [[full blood count]], [[protein electrophoresis]], [[antibodies]] to the [[acetylcholine receptor]] (indicative of myasthenia), [[electrolyte]]s, [[liver enzyme]]s and [[renal function]].<ref name=Thomas1999/>
===Staging===
The Masaoka Staging System is used widely and is based on the anatomic extent of disease at the time of surgery:<ref name="pmid7296496">{{cite journal | vauthors = Masaoka A, Monden Y, Nakahara K, Tanioka T | title = Follow-up study of thymomas with special reference to their clinical stages | journal = Cancer | volume = 48 | issue = 11 | pages = 2485–92 | date = December 1981 | pmid = 7296496 | doi = 10.1002/1097-0142(19811201)48:11<2485::AID-CNCR2820481123>3.0.CO;2-R | doi-access = free }}</ref>

* I:   Completely encapsulated
* IIA: Microscopic invasion through the capsule into surrounding fatty tissue
* IIB: Macroscopic invasion into capsule
* III: Macroscopic invasion into adjacent organs
* IVA: Pleural or pericardial implants
* IVB: Lymphogenous or hematogenous metastasis to distant (extrathoracic) sites

==Treatment==
Surgery is the mainstay of treatment for thymoma. If the tumor is apparently invasive and large, preoperative (neoadjuvant) chemotherapy and/or radiotherapy may be used to decrease the size and improve resectability, before surgery is attempted. When the tumor is an early stage (Masaoka I through IIB), no further therapy is necessary. Removal of the thymus in adults does not appear to induce [[immune deficiency]]. In children, however, postoperative immunity may be abnormal and vaccinations for several infectious agents are recommended. Invasive thymomas may require additional treatment with radiotherapy and chemotherapy ([[cyclophosphamide]], [[doxorubicin]] and [[cisplatin]]).<ref name=Thomas1999/>{{Citation needed|date=November 2007}}.<ref>{{Cite web|last=NCCN Thymoma|first=Guidelines|date=2016|title=NCCN Thymoma Guidelines|url=https://thymicuk.org/wp-content/uploads/2019/10/NCCN-Thymoma-and-Thymic-cancer-guidelines.pdf|website=NCCN Guidelines}}</ref> Recurrences of thymoma are described in 10-30% of cases up to 10 years after surgical resection, and in the majority of cases also pleural recurrences can be removed. Recently, surgical removal of pleural recurrences can be followed by hyperthermic intrathoracic perfusion chemotherapy or [[intrathoracic hyperthermic perfused chemotherapy (ITH)]].<ref>{{cite journal |last1=Ambrogi |first1=Marcello |title=Pleural recurrence of thymoma: surgical resection followed by hyperthermic intrathoracic perfusion chemotherapy |journal=Eur J Cardiothorac Surg |date=2016 |volume=49 |issue=1 |pages=321–6 |doi=10.1093/ejcts/ezv039 |pmid=25666471 }}</ref>

==Prognosis==
Prognosis is much worse for stage III or IV thymomas as compared with stage I and II tumors.  Invasive thymomas uncommonly can also [[metastasis|metastasize]], generally to [[pleura]], [[bone]]s, [[liver]] or [[Human brain|brain]] in approximately 7% of cases.<ref name=Thomas1999/>  A study found that slightly over 40% of observed patients with stage III and IV tumors survived for at least 10 years after diagnosis. The median age of these patients at the time of thymoma diagnosis was 57 years. <ref>{{cite journal | vauthors = Wilkins KB, Sheikh E, Green R, Patel M, George S, Takano M, Diener-West M, Welsh J, Howard S, Askin F, Bulkley GB | title = Clinical and pathologic predictors of survival in patients with thymoma | journal = Annals of Surgery | volume = 230 | issue = 4 | pages = 562–72; discussion 572–4 | date = October 1999 | pmid = 10522726 | pmc = 1420905 | doi = 10.1097/00000658-199910000-00012 }}</ref>

Patients who have undergone thymectomy for thymoma should be warned of possible severe side effects after yellow fever vaccination. This is probably caused by inadequate T-cell response to live attenuated yellow fever vaccine. Deaths have been reported.{{citation needed|date=April 2010}}

==Epidemiology==
The incidence of thymomas is around 0.13-0.26 per 100,000 people per year.<ref name=WHO2021>{{cite book |author1=WHO Classification of Tumours Editorial Board |title=Thoracic Tumours |date=2021 |publisher=World Health Organization |location=Lyon (France) |isbn=978-92-832-4506-3 |pages=320–325 |edition=5th |volume=5 |url=https://publications.iarc.fr/Book-And-Report-Series/Who-Classification-Of-Tumours/Thoracic-Tumours-2021 |language=en |chapter=5. Tumors of the thymus }}</ref> Males are affected slightly less frequently than females.<ref name=WHO2021/> The typical age at diagnosis is in the 40s and 50s, though the age may range from six years to 83 years.<ref name=WHO2021/>

== Gallery ==
<gallery>
File:Encapsulated cystic thymoma.jpg|An encapsulated cystic thymoma.
File:Locally invasive circumscribed thymoma.jpg|A locally invasive circumscribed thymoma (mixed lymphocytic and epithelial, mixed polygonal and spindle).
File:Thymoma B1 (2).JPG|Histopathological image of thymoma type B1. Anterior mediastinal mass surgically resected. Hematoxylin & eosin stain.
File:Thymoma B1 (3) CK CAM5-2.JPG|Histopathological image of thymoma type B1. Anterior mediastinal mass surgically resected. Cytokeratin CAM5.2 immunostain.
File:Thymoma type B1 (1).JPG|Histopathological image representing a noninvasive thymoma type B1, surgically resected. Hematoxylin & eosin.
File:Thymoma - cytology high mag.jpg|Thymoma. [[FNA specimen]]. [[Field stain]].
</gallery>

== See also ==
*[[Mediastinal tumor]]

== References ==
{{Reflist}}

== External links ==
{{Medical resources
| DiseasesDB      = 13067
| ICD10           = {{ICD10|C|37||c|37}}, {{ICD10|D|15|0|d|15|0}}
| ICD9            = {{ICD9|164.0}}, {{ICD9|212.6}}
| ICDO            = 8580
| OMIM            = 
| MedlinePlus     = 001086
| eMedicineSubj   = med
| eMedicineTopic  = 2752
| eMedicine_mult  = {{eMedicine2|med|3448}} {{eMedicine2|ped|2246}}
| MeshID          = D013945
| SNOMED CT       = 128856005
| Orphanet        = 99867
}}

{{Epithelial neoplasms}}
{{Lymphatic organ and vessel disease}}
{{Authority control}}

[[Category:Rare cancers]]
[[Category:Lymphatic organ neoplasia]]