Thymic Neuroendocrine Neoplasms: Biological Behaviour and Therapy

Jia, Ru; Šulentić, Petra; Xu, Jianming; Grossman, Ashley

doi:10.1159/000472255

Cited by 42 publications

(57 citation statements)

References 72 publications

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“…Thymic NETs are also an important cause of EAS due to thoracic tumors accounting for 5% to 10% of EATs, depending on the series (47,48,49,50,51). They are usually aggressive, poorly differentiated NEC, frequently accompanied by locoregional invasion and metastases (42,43,44,45,46).…”

Section: (A) Nets In the Cervical Spine Regionmentioning

confidence: 99%

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MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

Young

Haissaguerre

Viera-Pinto

et al. 2020

European Journal of Endocrinology

148

186

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Ectopic ACTH syndrome (EAS) is rare but is frequently a severe condition because of the intensity of the hypercortisolism that may be dissociated from the tumoral condition. EAS should often be considered as an endocrine emergency requiring an emergency response both in terms of diagnostic procedures and therapeutic interventions. Patient management is complex and necessitates dual skills, in the diagnosis and treatment of CS and in the specific management of neuroendocrine tumors (NET). Therefore, initial management should be performed ideally by experienced endocrinology teams in collaboration with specialized hormonal laboratory, modern imaging platforms and intensive care units. Diagnostic procedures vary according to the endocrine and tumoral contexts but should be reduced to a minimum in intense hypercortisolism. Preventive and curative treatments of cortisol-induced comorbidities, non-specific management of hypercortisolism and etiological treatments should be considered simultaneously. Therapeutic strategies vary according to (1.) the intensity of hypercortisolism, the general condition of the patient and associated comorbidities and (2.) the tumoral status, ranging from resectable ACTH secreting tumors to non-resectable metastatic endocrine tumors or occult tumors. The ideal treatment is complete excision of the ACTH-secreting tumor that can be performed rapidly or after preoperative preparation using cortisol-lowering drugs. When this is not possible, the therapeutic strategy should be discussed by a multidisciplinary experienced team in a personalized perspective and include variable combinations of pharmacological agents, bilateral adrenalectomy and non-specific tumoral interventions. Here we discuss the diagnosis and therapeutic strategies including the modern, currently available tools and emphasize on the operational effectiveness of care.

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Section: (A) Nets In the Cervical Spine Regionmentioning

confidence: 99%

“…Most of these are overt tumors that are readily visible on imaging studies. Pancreatic NETs are sometimes amenable to surgical resection, but they are often accompanied by node involvement and liver metastases (50).…”

Section: (C) Abdominal and Retroperitoneal Netsmentioning

confidence: 99%

MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

Young

Haissaguerre

Viera-Pinto

et al. 2020

European Journal of Endocrinology

148

186

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“…Non-functioning tumours usually present due to cough, recurrent infections or haemoptysis [ 78 ]. The location of bronchial NETs is the most important predictor of symptoms; central tumours frequently produce symptoms while those located more peripherally are more frequently picked up incidentally when imaging for other reasons [ 79 ].Thymic carcinoids are frequently functional and may secrete a wide range of hormones; they are responsible for a significant proportion of cases of Cushing’s syndrome due to ectopic ACTH secretion [ 80 ] [ 81 ]. Less frequently they present due to mass effect [ 81 ].…”

Section: Bronchial and Thymic Netsmentioning

confidence: 99%

“…Most bronchial/thymic NETs do not have an underlying genetic cause. Bronchial NETs are found in approximately 5% of patients with MEN1; whilst thymic carcinoids are found reported in 2–8% [ 41 , 80 , 82 ]. Conversely, approximately 25% of cases of bronchial/thymic NETs are associated with MEN1 [ 80 ].…”

Section: Bronchial and Thymic Netsmentioning

confidence: 99%

“…Bronchial NETs are found in approximately 5% of patients with MEN1; whilst thymic carcinoids are found reported in 2–8% [ 41 , 80 , 82 ]. Conversely, approximately 25% of cases of bronchial/thymic NETs are associated with MEN1 [ 80 ]. Bronchial/thymic NET has been reported as the presenting feature of MEN1 in one series; however all patients had concurrent primary hyperparathyroidism and several had other MEN1 associated tumours [ 82 ].…”

Section: Bronchial and Thymic Netsmentioning

confidence: 99%

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When should genetic testing be performed in patients with neuroendocrine tumours?

O'Shea

Druce

2017

Rev Endocr Metab Disord

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Neuroendocrine tumours (NETs) are a heterogenous group of tumours arising from neuroendocrine cells in several sites around the body. They include tumours of the gastroenteropancreatic system, phaeochromocytoma and paraganglioma and medullary thyroid cancer. In recent years, it has become increasingly apparent that a number of these tumours arise as a result of germline genetic mutations and are inherited in an autosomal dominant pattern. The number of genes implicated is increasing rapidly. Identifying which patients are likely to have a germline mutation enables clinicians to counsel patients adequately about their future disease risk, and allows for earlier detection of at-risk patients through family screening. The institution of screening and surveillance programmes may in turn lead to a major shift in presentation patterns for some of these tumours. In this review, we examine the features which may lead a clinician to suspect that a patient may have an inherited cause of a NET and we outline which underlying conditions should be suspected. We also discuss what type of screening may be appropriate in a variety of situations.

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The combination of etoposide and platinum for the treatment of thymic neuroendocrine neoplasms: A retrospective analysis

et al. 2023

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BackgroundTo provide real‐world outcomes for the combination of etoposide and platinum as a first‐line treatment for advanced thymic neuroendocrine neoplasms (TNENs).MethodsRetrospective analysis was performed on patients with advanced TNENs confirmed by pathology who received etoposide combined with platinum as a first‐line chemotherapy in our institution between 2010 and 2022.ResultsA total of 16 patients were included in this study. Twelve patients (75%) received etoposide combined with cisplatin, and four patients (25%) received etoposide combined with carboplatin. Efficacy was evaluated in all patients, with an objective response rate of 31.3%. One patient achieved a complete response, four achieved a partial response, and in eight patients the disease remained stable; the disease control rate was 81.3%. The median progression‐free survival (PFS) was 7.2 months with a 95% confidence interval (CI) of 2.1–12.3 months. The median overall survival (OS) was 50.4 months with a 95% CI of 32.1–68.8 months. No significant difference in efficacy was observed between the treatment groups with regards to PFS (p = 0.095) and OS (p = 0.061). Treatment‐related adverse events were observed in all 12 patients when evaluated for toxicity, manifesting as hematologic toxicity. Grade 3–4 bone marrow suppression occurred in six patients (50%). No treatment‐related deaths were recorded.ConclusionThis retrospective analysis, conducted in a real‐life setting, suggests that the combination of etoposide and platinum has a promising anti‐tumor activity in advanced TNENs, with a clinically significant overall response rate.

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Thymic Neuroendocrine Neoplasms: Biological Behaviour and Therapy

Cited by 42 publications

References 72 publications

MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

MANAGEMENT OF ENDOCRINE DISEASE: Cushing’s syndrome due to ectopic ACTH secretion: an expert operational opinion

When should genetic testing be performed in patients with neuroendocrine tumours?

The combination of etoposide and platinum for the treatment of thymic neuroendocrine neoplasms: A retrospective analysis

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