Therapeutic options in thymomas and thymic carcinomas

Muto, Y.; Okuma, Yusuke

doi:10.1080/14737140.2022.2052278

Cited by 9 publications

(7 citation statements)

References 97 publications

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“…The treatment is based on surgery as soon as possible to reduce the incidence of metastasis [123,125,126], while radiotherapy and/or chemotherapy are indicated in advanced/metastatic tumors [135]. A phase 2 study is evaluating whether the TKI lenvatinib plus pembrolizumab benefits patients with type B3 thymoma or thymic carcinoma [170].…”

Section: Thymus Neuroendocrine Tumorsmentioning

confidence: 99%

Neuroendocrine neoplasms in the context of inherited tumor syndromes: a reappraisal focused on targeted therapies

Ruggeri

Benevento²,

Cicco³

et al. 2022

J Endocrinol Invest

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Purpose Neuroendocrine neoplasms can occur as part of inherited disorders, usually in the form of well-differentiated, slow-growing tumors (NET). The main predisposing syndromes include: multiple endocrine neoplasias type 1 (MEN1), associated with a large spectrum of gastroenteropancreatic and thoracic NETs, and type 4 (MEN4), associated with a wide tumour spectrum similar to that of MEN1; von Hippel-Lindau syndrome (VHL), tuberous sclerosis (TSC), and neurofibromatosis 1 (NF-1), associated with pancreatic NETs. In the present review, we propose a reappraisal of the genetic basis and clinical features of gastroenteropancreatic and thoracic NETs in the setting of inherited syndromes with a special focus on molecularly targeted therapies for these lesions. Methods Literature search was systematically performed through online databases, including MEDLINE (via PubMed), and Scopus using multiple keywords' combinations up to June 2022. Results Somatostatin analogues (SSAs) remain the mainstay of systemic treatment for NETs, and radiolabelled SSAs can be used for peptide-receptor radionuclide therapy for somatostatin receptor (SSTR)-positive NETs. Apart of these SSTR-targeted therapies, other targeted agents have been approved for NETs: the mTOR inhibitor everolimus for lung, gastroenteropatic and unknown origin NET, and sunitinib, an antiangiogenic tyrosine kinase inhibitor, for pancreatic NET. Novel targeted therapies with other antiangiogenic agents and immunotherapies have been also under evaluation. Conclusions Major advances in the understanding of genetic and epigenetic mechanisms of NET development in the context of inherited endocrine disorders have led to the recognition of molecular targetable alterations, providing a rationale for the implementation of treatments and development of novel targeted therapies.

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Section: Thymus Neuroendocrine Tumorsmentioning

confidence: 99%

Neuroendocrine neoplasms in the context of inherited tumor syndromes: a reappraisal focused on targeted therapies

Ruggeri

Benevento²,

Cicco³

et al. 2022

J Endocrinol Invest

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“…Based on available data, the most popular and active regimens are cisplatin-anthracycline (CAP or ADOC) or cisplatin-etoposide combinations, which showed response rates above 50% in most of the series, whatever in front-line therapy or in case of relapsing tumours (8). However, these therapeutic regimens have not reached a satisfactory result (9)(10)(11). The study conducted by Ma et al found that different front-line chemotherapy regimens of cisplatin, doxorubicin, and cyclophosphamide (CAP); cisplatin and etoposide (EP); or cisplatin and paclitaxel (TP) may provide similar long-term PFS and OS in patients with advanced thymoma (12).…”

Section: Commentmentioning

confidence: 99%

Section: Commentmentioning

confidence: 99%

Neoadjuvant radiotherapy plus single-incision thoracoscopic surgery in the treatment of type B3 thymomas

Huang

2023

Front. Oncol.

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BackgroundAlthough minimally invasive surgery is the standard treatment for thymomas, minimally invasive thymectomy is difficult for patients with type B3 thymomas, especially for giant or aggressive lesions. These tumors are frequently treated with radical radiation therapy or surgery plus adjuvant radiotherapy. Few studies, however, have tested the efficacy of neoadjuvant radiotherapy prior to thoracoscopic surgery.MethodsPatients with type B3 thymomas >5 cm or with infiltrates into vital organs on CT-guided puncture biopsy who underwent neoadjuvant radiotherapy followed by single-incision minimally invasive thymectomy from March 2016 to July 2020 were retrospectively evaluated. Reduction ratios, TNM stage changes according to WHO stage criteria, resectability, long-term survival, and the response in terms of RECIST v1.1 criteria achieved by preoperative RT were analyzed.ResultsThe 11 patients who underwent neoadjuvant radiotherapy plus minimally invasive thymectomy included five men and six women, of mean age 49.5 years. Four patients had myasthenia gravis. Neoadjuvant radiotherapy consisted of 50 Gy in 25 fractions, with all patients showing varying degrees of lesion reduction after radiotherapy. Surgery was performed about 1 month after neoadjuvant radiotherapy, with none of these patients having severe radiation pneumonitis. All patients underwent radical resection of the tumor and adjacent tissue, with none experiencing tumor seeding or rupture during surgery. The median postoperative hospital stay was 3 days (range: 2–6 days) and the frequency of additional regular analgesics (including those for wound pain and neuralgia) was 2.5 times per person. On follow-up, one patient experienced pleural metastasis and one experienced pulmonary metastasis, with the other nine patients showing no evidence of tumor recurrence.ConclusionNeoadjuvant radiotherapy followed by minimally invasive surgery was a safe and efficacious procedure for the treatment of type B3 thymomas, with less postoperative pain and faster recovery. This strategy, of tumor shrinkage prior to surgery, may make possible the easier removal of type B3 thymomas by single-incision thoracoscopy.

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“…1 TC usually appears as a space-occupying lesion in the anterior mediastinum on imaging, and the lesion is usually large at the time of diagnosis, so patients often have corresponding symptoms due to tumor compression, such as chest pain and dyspnea. 2 In addition, TC is highly aggressive and most patients with TC have local or distant metastases when diagnosed. 3 At present, systemic therapy is the primary treatment for patients with unresectable TC.…”

Section: Introductionmentioning

confidence: 99%

“…Thymic carcinoma (TC) is a rare malignant tumor with limited data regarding its poor outcome, particularly for those patients with unresectable disease 1 . TC usually appears as a space‐occupying lesion in the anterior mediastinum on imaging, and the lesion is usually large at the time of diagnosis, so patients often have corresponding symptoms due to tumor compression, such as chest pain and dyspnea 2 . In addition, TC is highly aggressive and most patients with TC have local or distant metastases when diagnosed 3 .…”

Section: Introductionmentioning

confidence: 99%

Chemotherapy versus chemotherapy plus immune checkpoint inhibitors for the first‐line treatment of unresectable thymic carcinoma: A multicenter retrospective study

Zhang,

Liu,

Chen

et al. 2024

Intl Journal of Cancer

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Thymic carcinoma (TC) is a rare malignant tumor with a poor prognosis, and there is currently limited data on the use of immunotherapy in patients with unresectable TC. In this study, data of patients with unresectable TC diagnosed from January 2017 were retrospectively collected from multiple centers. Treatment response, progression‐free survival (PFS), overall survival (OS), survival‐independent prognostic factor, and adverse events (AEs) were further analyzed. As a result, a total of 93 patients with unresectable TC were enrolled, of which 54 received first‐line chemotherapy, and 39 received chemotherapy plus immune checkpoint inhibitors (ICIs). The objective response rate was 50% (27/54) in the chemotherapy group and 76.9% (30/39) in the chemotherapy plus ICIs group. The chemotherapy plus ICIs group achieved significant median PFS benefit (8.8 vs. 34.9 months, p < .001) and median OS benefit (41.8 months vs. not reached, p = .025). Multivariate analysis showed that ICIs and local therapy were independent prognostic factors for PFS. In addition, 17 patients developed immune‐related AEs (IRAEs), of which 15 (38.5%) had Grade 1 or 2 IRAEs and 2 (5.1%) had Grade 3 IRAEs in the chemotherapy plus ICIs group. In conclusion, the efficacy of chemotherapy plus ICIs is superior to chemotherapy, and the adverse effects are manageable in patients with unresectable TC.

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Therapeutic options in thymomas and thymic carcinomas

Cited by 9 publications

References 97 publications

Neuroendocrine neoplasms in the context of inherited tumor syndromes: a reappraisal focused on targeted therapies

Neuroendocrine neoplasms in the context of inherited tumor syndromes: a reappraisal focused on targeted therapies

Neoadjuvant radiotherapy plus single-incision thoracoscopic surgery in the treatment of type B3 thymomas

Chemotherapy versus chemotherapy plus immune checkpoint inhibitors for the first‐line treatment of unresectable thymic carcinoma: A multicenter retrospective study

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