NCCN Guidelines Insights: Neuroendocrine and Adrenal Tumors, Version 2.2018

Shah, Manisha H.; Goldner, Whitney; Halfdanarson, Thorvardur R.; Bergsland, Emily K.; Berlin, Jordan; Halperin, Daniel M.; Chan, Jennifer A.; Kulke, Matthew H.; Benson, Al B.; Blaszkowsky, Lawrence S.; Eads, Jennifer R.; Engstrom, Paul F.; Fanta, Paul T.; Giordano, Thomas J.; He, Jin; Heslin, Martin J.; Kalemkerian, Gregory P.; Kandeel, Fouad; Khan, Sajid; Kidwai, Wajih Zaheer; Kunz, Pamela L.; Kuvshinoff, Boris W.; Lieu, Christopher H.; Pillarisetty, Venu G.; Saltz, Leonard; Sosa, Julie Ann; Strosberg, Jonathan R.; Sussman, Craig A.; Trikalinos, Nikolaos A.; Uboha, N.; Whisenant, Jonathan; Wong, Terence Z.; Yao, James C.; Burns, Jennifer L.; Ogba, Ndiya; Zuccarino-Catania, Griselda

doi:10.6004/jnccn.2018.0056

Cited by 333 publications

(319 citation statements)

References 41 publications

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“…As for clinical management of NTEs, the European Society for Medical Oncology (ESMO), the European Neuroendocrine Tumor Society (ENETS) and the National Comprehensive Cancer Network (NCCN) Guidelines recommend that surgical resection is the main therapy for localized disease, and also a choice for extensive disease. External radiotherapy, cytotoxic treatment and Interferon-Alpha can be used when disease progresses [30][31][32]. Besides, according to the ENETS and NCCN Guidelines [31,32], treatment with Octreotide or Lanreotide, peptide receptor radionuclide therapy (PRRT) or targeted therapy (Everolimus) are recommended for advanced disease.…”

Section: Discussionmentioning

confidence: 99%

p.L105Vfs mutation in a family with thymic neuroendocrine tumor combined with MEN1: a case report

Zheng¹,

Zhou²,

Tang³

et al. 2020

BMC Neurol

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Background: Multiple endocrine neoplasia type 1 (MEN1) is a rare autosomal dominant disorder arising from mutations of the MEN1 tumor suppressor gene on chromosome 11q13; MEN1 is characterized by the development of neuroendocrine tumors, including those of the parathyroid, gastrointestinal endocrine tissue and anterior pituitary. Additionally, thymic neuroendocrine tumors in MEN1 are also rarely reported. Case presentation: This case report observed a family that presented with MEN1 p.L105Vfs mutation, and two of the family members had been diagnosed with thymic neuroendocrine tumor combined with MEN1. To the best of our knowledge, this is the first time such a mutation in the MEN1 gene has been reported. The proband presented with thymic neuroendocrine tumor, parathyroid adenoma and rectum adenocarcinoma. The son of the proband presented with thymic neuroendocrine tumor, gastrinoma, hypophysoma and parathyroid adenoma. Genetic testing revealed the frameshift mutation p.L105Vfs, leading to the identification of one carrier in the pedigree (the patient's younger sister). The proband then underwent parathyroidectomy at the age of 26 years (in 1980) for a parathyroid adenoma. Subsequently, the patient underwent thymectomy, radiotherapy and chemotherapy. The patient is now 64 years old, still alive and still undergoing Lanreotide therapy. Conclusion: Thymic neuroendocrine MEN1 is rare, but it accounts for almost 20% of MEN1-associated mortality. Consequently, we should focus on regular clinical screening of the thymus in MEN1 patients.

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Section: Discussionmentioning

confidence: 99%

p.L105Vfs mutation in a family with thymic neuroendocrine tumor combined with MEN1: a case report

Zheng¹,

Zhou²,

Tang³

et al. 2020

BMC Neurol

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“…Endoscopic resection has been shown to be effective in removing rectal NETs, particularly those measuring <10 mm in size . However, the treatment choices still vary because sufficient and convictive data are lacking, rendering it difficult to ensure complete tumor resection and lower the rate of recurrence . The North American Neuroendocrine Tumors Society guidelines conclude that tumors <2 cm that are confined to the mucosa or submucosa are associated with very minimal risk of local and metastatic spread, and metastatic screening or follow‐up are not recommended after local resection .…”

Section: Discussionmentioning

confidence: 99%

“…In contrast, the National Comprehensive Cancer Network guidelines suggest that all patients should be screened with colonoscopy plus either abdominal/pelvic CT/MRI and endorectal ultrasound or endoscopic ultrasound . In addition, for lesions ≤2 cm, the National Comprehensive Cancer Network suggests trans‐anal excision if possible with no follow‐up for lesions <1 cm and follow‐up at 6 and 12 months for local recurrence with rectal MRI or endoscopic ultrasound for lesions between 1 and 2 cm . In practice, most lesions <2 cm are endoscopically resected without lymph node harvest.…”

Section: Discussionmentioning

confidence: 99%

“…The data were retrieved from the Surveillance, Epidemiology, and End Results (SEER) database based on the November 2018 submission of patients diagnosed with NETs located in the rectosigmoid junction and rectum between 2000 and 2016. We used the SEER*Stat 8.3.5 program to identify individuals in the SEER database as follows: ICD‐O‐3:8240 and 8249, and primary site codes: C19.9 rectum and C20.0 rectosigmoid junction . We excluded patients who met the following criteria: (a) patients whose survival data or follow up data were incomplete; (b) patients diagnosed with more than one primary tumor; and (c) patients whose death was due to nonneoplastic disease.…”

Section: Methodsmentioning

confidence: 99%

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Rectal NETs and rectosigmoid junction NETs may need to be treated differently

Cui

et al. 2019

Cancer Medicine

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Neuroendocrine tumors (NETs) are heterogeneous, and the incidence of NETs is rapidly increasing. We observed different survival in patients with rectal NETs and rectosigmoid junction NETs, which are treated similarly. We included patients with rectal and rectosigmoid junction NETs from the SEER database. The 5‐year survival was set as the end‐point. 6675 patients with rectal NETs and 329 patients with rectosigmoid junction NETs, were eligible for the analysis. Initially, the survival analyses suggested that the 5‐year survival significantly differed between the patients with rectal and rectosigmoid junction NETs (HR = 0.82, 95% CI 0.70‐0.95; P = .01). Tumor differentiation, an invasion deeper than T2, and lymph node and distant metastases were still important risk factors affecting survival for both location. While, the males showed better survival (HR = 0.69, 95% CI 0.55‐0.88; P < .01) and primary tumor surgery had no benefits (P = .56) for patients with rectosigmoid junction NETs. The factors that predict regional lymph node metastases varied by location. In rectal NETs, invasion deeper than T1 and a tumor larger than 1 cm could significantly increase the risk of regional lymph node metastases (all OR > 5, P < .01). In rectosigmoid junction NETs, the risk of regional lymph node metastases was considered significantly higher with invasion deeper than T1 (all OR > 5, P < .01) and a tumor larger than 2 cm (OR = 31.32, 95% CI 2.53‐387.57; P < .01). We advocate a clear and consistent definition of the rectosigmoid junction for future studies, and more studies are needed to determine the reason underlying differences between rectum and rectosigmoid junction.

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“…Although functional tumours represent the minority of PNET, most surgeons agree that these tumours should be resected in medically‐fit patients, regardless of size . In the setting of nonfunctional PNET, indications for resection are less clear.…”

Section: Management Of Small (<2 Cm) Tumoursmentioning

confidence: 99%

Contemporary issues in the surgical management of pancreatic neuroendocrine tumours

Sham

Gage

2019

Surgical Practice

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Pancreatic neuroendocrine tumours (PNET) are relatively rare entities, accounting for 1-2 per cent of pancreatic neoplasms. However, rates of diagnosis have recently increased, in part due to incidental discovery on the widespread use of cross-sectional imaging. While general consensus exists regarding the treatment of large and/or functional PNET, the management of smaller (<2 cm) asymptomatic lesions is less clear. Recent updates in staging guidelines have improved classification of high-and low-risk tumours, and additional genetic analysis (e.g. Death domain-associated protein 6 Gene, Alpha Thalassemia/Mental Retardation Syndrome X-Linked Gene) offers the possibility of enhanced risk stratification to further guide surgical management. In patients who do ultimately require pancreatectomy, the role of minimally-invasive surgery (laparoscopic or robotic) continues to expand.

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NCCN Guidelines Insights: Neuroendocrine and Adrenal Tumors, Version 2.2018

Cited by 333 publications

References 41 publications

p.L105Vfs mutation in a family with thymic neuroendocrine tumor combined with MEN1: a case report

p.L105Vfs mutation in a family with thymic neuroendocrine tumor combined with MEN1: a case report

Rectal NETs and rectosigmoid junction NETs may need to be treated differently

Contemporary issues in the surgical management of pancreatic neuroendocrine tumours

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