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Thymoma type A

MedGen UID:
266099
•Concept ID:
C1266091
•
Neoplastic Process
Synonyms: Medullary Thymoma; medullary thymoma; Primary thymic epithelial neoplasm type A; primary thymic epithelial neoplasm type A; Primary thymic epithelial tumor type A; primary thymic epithelial tumor type A; primary thymic epithelial tumour type A; Spindle Cell Thymoma; spindle cell thymoma; Thymoma Type A; thymoma type A; thymoma, medullary
SNOMED CT: Thymoma type A (1144928005)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0002588
Orphanet: ORPHA263310

Definition

A thymic epithelial neoplasm characterized by the presence of spindle and/or oval neoplastic epithelial cells. Lymphocytic infiltration is minimal or absent. It may be associated with myasthenia gravis or pure red cell aplasia. The majority of cases occur in the anterior mediastinum as Masaoka stage I tumors. Approximately 20% of the cases occur as stage II or stage III tumors. Type A thymoma generally behaves as a benign tumor and the overall survival is reported to be 100% at 5 and 10 years. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVThymoma type A
Follow this link to review classifications for Thymoma type A in Orphanet.

Professional guidelines

PubMed

Clinical Features, Treatment Modalities, and Outcomes of Elderly Thymoma Patients: A Propensity-Matched Study Based on the SEER Database.
Wang Z, Gong C, Zhang Y, Wang M, Chao C, Qian Y, Di D, Wang B
Oncol Res Treat 2023;46(12):520-529. Epub 2023 Nov 14 doi: 10.1159/000535020. PMID: 37963448
Significance of YAP1-MAML2 rearrangement and GTF2I mutation in the diagnosis and differential diagnosis of metaplastic thymoma.
Wang M, Xu H, Han Q, Wang L
Ann Med 2023;55(2):2237040. doi: 10.1080/07853890.2023.2237040. PMID: 37489594Free PMC Article
ITMIG consensus statement on the use of the WHO histological classification of thymoma and thymic carcinoma: refined definitions, histological criteria, and reporting.
Marx A, Ströbel P, Badve SS, Chalabreysse L, Chan JK, Chen G, de Leval L, Detterbeck F, Girard N, Huang J, Kurrer MO, Lauriola L, Marino M, Matsuno Y, Molina TJ, Mukai K, Nicholson AG, Nonaka D, Rieker R, Rosai J, Ruffini E, Travis WD
J Thorac Oncol 2014 May;9(5):596-611. doi: 10.1097/JTO.0000000000000154. PMID: 24722150

Recent clinical studies

Etiology

RADIORYTHMIC: Phase III, Opened, Randomized Study of Postoperative Radiotherapy Versus Surveillance in Stage IIb/III of Masaoka Koga Thymoma after Complete Surgical Resection.
Basse C, Botticella A, Molina TJ, Falcoz PE, Oulkhouir Y, Kerjouan M, Pichon E, Westeel V, Thiberville L, Quantin X, Clément-Duchêne C, Khalifa J, Tinier FL, Ginoux M, Thillays F, Mordant P, Besse B, Thomas PA, Péchoux CL, Girard N
Clin Lung Cancer 2021 Sep;22(5):469-472. Epub 2021 Feb 4 doi: 10.1016/j.cllc.2021.01.020. PMID: 33736940
Influence of Smoking and Histologic Subtype on Developing Extrathymic Malignancy in Thymoma Patients.
Yanagiya M, Matsumoto J, Kawahara T, Yamaguchi H, Nagayama K, Anraku M, Sato M, Nakajima J
Ann Thorac Surg 2019 May;107(5):1532-1539. Epub 2018 Dec 18 doi: 10.1016/j.athoracsur.2018.11.038. PMID: 30576633
Histogram analysis of apparent diffusion coefficient maps for assessing thymic epithelial tumours: correlation with world health organization classification and clinical staging.
Kong LY, Zhang W, Zhou Y, Xu H, Shi HB, Feng Q, Xu XQ, Yu TF
Br J Radiol 2018 Apr;91(1084):20170580. Epub 2018 Jan 10 doi: 10.1259/bjr.20170580. PMID: 29260882Free PMC Article
Diffusion-weighted MR imaging vs. multi-detector row CT: Direct comparison of capability for assessment of management needs for anterior mediastinal solitary tumors.
Seki S, Koyama H, Ohno Y, Nishio M, Takenaka D, Maniwa Y, Itoh T, Nishimura Y, Sugimura K
Eur J Radiol 2014 May;83(5):835-42. Epub 2014 Jan 16 doi: 10.1016/j.ejrad.2014.01.005. PMID: 24636535
Pulmonary metastasis from encapsulated cervical ectopic type a thymoma.
Kinoshita T, Yoshida J, Ishii G, Aokage K, Hishida T, Nagai K
Ann Thorac Surg 2012 Dec;94(6):e141-2. doi: 10.1016/j.athoracsur.2012.05.092. PMID: 23176955

Diagnosis

Ectopic thymoma in the middle mediastinum: A case report and literature review.
Tuan PA, Minh Duc N
Clin Ter 2021 Mar 15;172(2):94-98. doi: 10.7417/CT.2021.2291. PMID: 33763676
A mutation analysis of the EGFR pathway genes, RAS, EGFR, PIK3CA, AKT1 and BRAF, and TP53 gene in thymic carcinoma and thymoma type A/B3.
Sakane T, Murase T, Okuda K, Saida K, Masaki A, Yamada T, Saito Y, Nakanishi R, Inagaki H
Histopathology 2019 Nov;75(5):755-766. Epub 2019 Oct 3 doi: 10.1111/his.13936. PMID: 31179560
Good's Syndrome-Association of the Late Onset Combined Immunodeficiency with Thymoma: Review of Literature and Case Report.
Tavakol M, Mahdaviani SA, Ghaemi MR, Vaezi M, Dorudinia A, Jamaati H, Velayati AA
Iran J Allergy Asthma Immunol 2018 Feb;17(1):85-93. PMID: 29512373
Diffusion-weighted MR imaging vs. multi-detector row CT: Direct comparison of capability for assessment of management needs for anterior mediastinal solitary tumors.
Seki S, Koyama H, Ohno Y, Nishio M, Takenaka D, Maniwa Y, Itoh T, Nishimura Y, Sugimura K
Eur J Radiol 2014 May;83(5):835-42. Epub 2014 Jan 16 doi: 10.1016/j.ejrad.2014.01.005. PMID: 24636535
Pulmonary metastasis from encapsulated cervical ectopic type a thymoma.
Kinoshita T, Yoshida J, Ishii G, Aokage K, Hishida T, Nagai K
Ann Thorac Surg 2012 Dec;94(6):e141-2. doi: 10.1016/j.athoracsur.2012.05.092. PMID: 23176955

Therapy

RADIORYTHMIC: Phase III, Opened, Randomized Study of Postoperative Radiotherapy Versus Surveillance in Stage IIb/III of Masaoka Koga Thymoma after Complete Surgical Resection.
Basse C, Botticella A, Molina TJ, Falcoz PE, Oulkhouir Y, Kerjouan M, Pichon E, Westeel V, Thiberville L, Quantin X, Clément-Duchêne C, Khalifa J, Tinier FL, Ginoux M, Thillays F, Mordant P, Besse B, Thomas PA, Péchoux CL, Girard N
Clin Lung Cancer 2021 Sep;22(5):469-472. Epub 2021 Feb 4 doi: 10.1016/j.cllc.2021.01.020. PMID: 33736940
Influence of Smoking and Histologic Subtype on Developing Extrathymic Malignancy in Thymoma Patients.
Yanagiya M, Matsumoto J, Kawahara T, Yamaguchi H, Nagayama K, Anraku M, Sato M, Nakajima J
Ann Thorac Surg 2019 May;107(5):1532-1539. Epub 2018 Dec 18 doi: 10.1016/j.athoracsur.2018.11.038. PMID: 30576633
Good's Syndrome-Association of the Late Onset Combined Immunodeficiency with Thymoma: Review of Literature and Case Report.
Tavakol M, Mahdaviani SA, Ghaemi MR, Vaezi M, Dorudinia A, Jamaati H, Velayati AA
Iran J Allergy Asthma Immunol 2018 Feb;17(1):85-93. PMID: 29512373

Prognosis

RADIORYTHMIC: Phase III, Opened, Randomized Study of Postoperative Radiotherapy Versus Surveillance in Stage IIb/III of Masaoka Koga Thymoma after Complete Surgical Resection.
Basse C, Botticella A, Molina TJ, Falcoz PE, Oulkhouir Y, Kerjouan M, Pichon E, Westeel V, Thiberville L, Quantin X, Clément-Duchêne C, Khalifa J, Tinier FL, Ginoux M, Thillays F, Mordant P, Besse B, Thomas PA, Péchoux CL, Girard N
Clin Lung Cancer 2021 Sep;22(5):469-472. Epub 2021 Feb 4 doi: 10.1016/j.cllc.2021.01.020. PMID: 33736940
A mutation analysis of the EGFR pathway genes, RAS, EGFR, PIK3CA, AKT1 and BRAF, and TP53 gene in thymic carcinoma and thymoma type A/B3.
Sakane T, Murase T, Okuda K, Saida K, Masaki A, Yamada T, Saito Y, Nakanishi R, Inagaki H
Histopathology 2019 Nov;75(5):755-766. Epub 2019 Oct 3 doi: 10.1111/his.13936. PMID: 31179560
Influence of Smoking and Histologic Subtype on Developing Extrathymic Malignancy in Thymoma Patients.
Yanagiya M, Matsumoto J, Kawahara T, Yamaguchi H, Nagayama K, Anraku M, Sato M, Nakajima J
Ann Thorac Surg 2019 May;107(5):1532-1539. Epub 2018 Dec 18 doi: 10.1016/j.athoracsur.2018.11.038. PMID: 30576633
Thymoma and thymic carcinoma associated with multilocular thymic cyst: a clinicopathologic analysis of 18 cases.
Shen X, Jin Y, Shen L, Sun Y, Chen H, Li Y
Diagn Pathol 2018 Jun 26;13(1):41. doi: 10.1186/s13000-018-0719-7. PMID: 29945642Free PMC Article
Pulmonary metastasis from encapsulated cervical ectopic type a thymoma.
Kinoshita T, Yoshida J, Ishii G, Aokage K, Hishida T, Nagai K
Ann Thorac Surg 2012 Dec;94(6):e141-2. doi: 10.1016/j.athoracsur.2012.05.092. PMID: 23176955

Clinical prediction guides

Ectopic thymoma in the middle mediastinum: A case report and literature review.
Tuan PA, Minh Duc N
Clin Ter 2021 Mar 15;172(2):94-98. doi: 10.7417/CT.2021.2291. PMID: 33763676
RADIORYTHMIC: Phase III, Opened, Randomized Study of Postoperative Radiotherapy Versus Surveillance in Stage IIb/III of Masaoka Koga Thymoma after Complete Surgical Resection.
Basse C, Botticella A, Molina TJ, Falcoz PE, Oulkhouir Y, Kerjouan M, Pichon E, Westeel V, Thiberville L, Quantin X, Clément-Duchêne C, Khalifa J, Tinier FL, Ginoux M, Thillays F, Mordant P, Besse B, Thomas PA, Péchoux CL, Girard N
Clin Lung Cancer 2021 Sep;22(5):469-472. Epub 2021 Feb 4 doi: 10.1016/j.cllc.2021.01.020. PMID: 33736940
Influence of Smoking and Histologic Subtype on Developing Extrathymic Malignancy in Thymoma Patients.
Yanagiya M, Matsumoto J, Kawahara T, Yamaguchi H, Nagayama K, Anraku M, Sato M, Nakajima J
Ann Thorac Surg 2019 May;107(5):1532-1539. Epub 2018 Dec 18 doi: 10.1016/j.athoracsur.2018.11.038. PMID: 30576633
Thymoma and thymic carcinoma associated with multilocular thymic cyst: a clinicopathologic analysis of 18 cases.
Shen X, Jin Y, Shen L, Sun Y, Chen H, Li Y
Diagn Pathol 2018 Jun 26;13(1):41. doi: 10.1186/s13000-018-0719-7. PMID: 29945642Free PMC Article
Diffusion-weighted MR imaging vs. multi-detector row CT: Direct comparison of capability for assessment of management needs for anterior mediastinal solitary tumors.
Seki S, Koyama H, Ohno Y, Nishio M, Takenaka D, Maniwa Y, Itoh T, Nishimura Y, Sugimura K
Eur J Radiol 2014 May;83(5):835-42. Epub 2014 Jan 16 doi: 10.1016/j.ejrad.2014.01.005. PMID: 24636535

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