Humans ; Thymoma/pathology* ; Thymus Neoplasms/pathology*

BACKGROUND: Thymic carcinomas (TCs) and thymic neuroendocrine neoplasms (TNENs) are two aggressive subtypes of thymic malignancy. Traditional therapy for advanced TCs and TNENs has limited outcome. New genomic profiling of TCs and TNENs might provide insights that contribute to the development of new treatment approaches. METHODS: We used gene panel sequencing technologies to investigate the genetic aberrations of 32 TC patients and 15 TNEN patients who underwent surgery at Shanghai Chest Hospital between 2015 and 2017. Patient samples were sequenced using a 324-gene platform with licensed technologies. In this study, we focused on clinically relevant genomic alterations (CRGAs), which are previously proven to be pathogenic alterations, to identify the pathology-specific mutational patterns, prognostic signatures of TCs and TNENs. RESULTS: The mutational profiles between TCs and TNENs were diverse. The genetic alterations that ranked highest in TCs were in CDKN2A, TP53, ASXL1, CDKN2B, PIK3C2G, PTCH1, and ROS1 , while those in TNENs were in MEN1, MLL2, APC, RB1 , and TSC2 . Prognostic analysis showed that mutations of ROS1, CDKN2A, CDKN2B, BRAF, and BAP1 were significantly associated with worse outcomes in TC patients, and that mutation of ERBB2 indicated shortened disease-free survival (DFS) and overall survival (OS) in TNEN patients. Further investigation found that the prognosis-related genes were focused on signal pathways of cell cycle control, chromatin remodeling/DNA methylation, phosphoinositide 3-kinases (PI3K)/protein kinase B (AKT)/mammalian target of rapamycin (mTOR), and receptor tyrosine kinase (RTK)/RAS/mitogen-activated protein kinase (MAPK) signaling. CONCLUSION We profiled the mutational features of 47 Chinese patients with thymic malignancy of diverse pathologic phenotypes to uncover the integrated genomic landscape of these rare tumors, and identified the pathology-specific mutational patterns, prognostic signatures, and potential therapeutic targets for TCs and TNENs.
Humans ; Thymoma ; Protein-Tyrosine Kinases/genetics* ; Proto-Oncogene Proteins/genetics* ; China ; Thymus Neoplasms/pathology* ; Prognosis ; Neuroendocrine Tumors/pathology* ; Mutation/genetics*

Surgery is the most important therapy for thymic malignances. The last decade has seen increasing adoption of minimally invasive surgery (MIS) for thymectomy. MIS for early stage thymoma patients has been shown to yield similar oncological results while being helpful in minimize surgical trauma, improving postoperative recovery, and reduce incisional pain. Meanwhile, With the advance in surgical techniques, the patients with locally advanced thymic tumors, preoperative induction therapies or recurrent diseases, may also benefit from MIS in selected cases.
Humans ; Minimally Invasive Surgical Procedures ; methods ; trends ; Retrospective Studies ; Thymoma ; surgery ; Thymus Neoplasms ; mortality ; pathology ; surgery

This paper introduced surgical treatment of malignancy-related superior vena cava syndrome. Typical cases were presented with diagnostic radiology results. Authors focused on the main approach to the malignancy-related superior vena cava syndrome of surgery. In order to make it simple for junior doctors to learn and practice, all 4 operation methods were described in details. The writer hopes it would be helpful for all the young thoracic surgeons.
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Humans ; Neoplasm Metastasis ; Superior Vena Cava Syndrome ; diagnostic imaging ; etiology ; surgery ; Thymoma ; complications ; diagnostic imaging ; surgery ; Thymus Neoplasms ; complications ; diagnostic imaging ; surgery ; Vena Cava, Superior ; diagnostic imaging ; pathology ; surgery

Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.
Agammaglobulinemia ; Humans ; Middle Aged ; Myasthenia Gravis ; Paraneoplastic Syndromes ; Pathology ; Red-Cell Aplasia, Pure* ; Thymectomy ; Thymoma

Cell Differentiation ; Choristoma ; pathology ; Humans ; Soft Tissue Neoplasms ; pathology ; Thymoma ; pathology ; Thymus Neoplasms ; pathology

Humans ; Male ; Middle Aged ; Positron-Emission Tomography ; Situs Inversus ; complications ; pathology ; Thymoma ; diagnosis ; pathology

Humans ; Pathology, Molecular ; Prognosis ; Thymoma ; diagnosis ; pathology ; Thymus Neoplasms ; diagnosis ; pathology

OBJECTIVETo evaluate the different pathological and clinical characteristics of thymomas with and without myasthenia gravis (MG) and to determine whether the presence of MG influences the prognosis in thymoma patients.

METHODSThe clinical data from 228 consecutive patients (median sternotomy were used in 153, video-assisted thoracoscopic themectomy were used in 75) operated on from January 1992 to December 2007 was analyzed retrospectively. These thymoma patients had been subdivided into two groups: thymoma with MG (n = 125) and thymoma without MG (n = 103). All thymic epithelial tumors were classified according to the WHO histologic classification and the Masaoka clinical staging system. The result was evaluated according to the Myasthenia Gravis Foundation of America's criterion. The clinical features of the 2 test was compared between the two groups by χ² test, and the survival were compared between the two groups by Cox analysis.

RESULTSThere were no peri-operative deaths. 19 cases were inoperable (6 in the group with MG, 13 without MG (χ² = 4.52, P = 0.035)). The proportions of type A and thymic carcinoma were 0 in the group with MG, 10.5% (11/103) and 11.6% (12/103) respectively in the group without MG. According to the Masaoka's clinical staging, in the group MG, 24.8% (31/125) patients were stage III and IV; in the group without MG, 33.0% (34/103) patients were stage III and IV. There was a significant difference between hyperplastic paraneoplastic thymus coexisting in 28.8% (36/125) patients with MG and only 5.8% (6/103) in patients without MG (χ² = 20.91, P = 0.000) Microthymoma was identified in the paraneoplastic thymus of 3 patients with MG. There were 198 patients followed up, the rate was 86.8% (198/228). There was no recurrence in patients with type A and a few patients with type AB, B1, B2, B3 thymoma and thymic carcinoma recurred. The actuarial 5- and 10-year survival rates were 89.3% and 81.2% for patients with MG respectively, and 90.0% and 78.9% for patients without MG respectively. Within 5 years postoperatively, 6 of 9 patients with MG died of myasthenia crisis, while 6 out of 7 deaths in patients without MG were attributable to inoperable tumors (stage IV) and thymic carcinoma.

CONCLUSIONSThe existence of myasthenia gravis has little influence on the prognosis of thymomas, but it is good for early diagnosis and treatment. Extended thymectomy should be performed to all patients with thymoma, no matter they have myasthenia gravis or not. The main cause of death is myasthenia crisis for thymoma patients with MG and stage IV and (or) thymic carcinoma for patients without MG.

Humans ; Myasthenia Gravis ; complications ; pathology ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Neoplasms, Glandular and Epithelial ; complications ; pathology ; surgery ; Postoperative Period ; Prognosis ; Retrospective Studies ; Sternotomy ; Survival Rate ; Thoracic Surgery, Video-Assisted ; Thymectomy ; Thymoma ; complications ; pathology ; surgery ; Thymus Neoplasms ; complications ; pathology ; surgery

Carcinoma ; classification ; pathology ; Consensus ; Humans ; Thymoma ; classification ; pathology ; Thymus Neoplasms ; classification ; pathology ; World Health Organization