Large low‐grade cystic pancreatic neuroendocrine neoplasm

Author(s):  
Hui Zhang ◽  
Jianguo Hong ◽  
Tao Li
2020 ◽  
Vol 52 (11) ◽  
pp. 784-787
Author(s):  
Sandrine Petignot ◽  
Adrian F. Daly ◽  
Emilie Castermans ◽  
Esther Korpershoek ◽  
Irène Scagnol ◽  
...  

AbstractMost pancreatic neuroendocrine neoplasms (pNEN) occur sporadically but they can also occur as part of multiple endocrine neoplasia type 1 (MEN1). MAX was originally described as an inherited pheochromocytoma-paraganglioma risk gene, but also has recently been implicated in pituitary tumorigenesis. Here we describe the first case of a pNEN associated with an inherited MAX gene deletion in a family with endocrine tumors. The patient was a male carrier of an intragenic exon 3 deletion inherited from his father who had recurrent pheochromocytomas and a macroprolactinoma. The patient underwent screening and hormonal studies but no pheochromocytoma-paraganglioma, pituitary or renal tumors were identified. However, abdominal magnetic resonance imaging (MRI) identified a 1 cm lesion in body of the pancreas. The lesion was hyperintense on T2-weighted signal, and there was hyperfixation of the tumor on 68Ga-DOTANOC PET-CT images. No biochemical evidence of pancreatic hormone excess was identified. Following a guided biopsy, a pathological diagnosis of a low grade pNEN was made and immunohistochemistry showed loss of MAX nuclear staining. Genetic analysis of the tumor tissue indicated copy number neutral loss of heterozygosity consistent with uniparental disomy. This is the first reported case of a MAX deletion associated pNEN and strengthens the argument that MAX may represent an inheritable multiple endocrine neoplasia risk gene. Further analysis of germline and somatic MAX mutations/deletions in large cohorts of unexplained NEN cases could help clarify the potential role of MAX in NEN etiology.


2020 ◽  
Vol 2020 ◽  
pp. 1-8 ◽  
Author(s):  
Wentao Zhou ◽  
Yuan Fang ◽  
Xu Han ◽  
Tiantao Kuang ◽  
Xuefeng Xu ◽  
...  

Backgrounds. Pancreatic neuroendocrine neoplasm (pNEN) is a highly heterogeneous entity, presenting widely varied biological behavior as well as long-term prognosis. Reliable biomarkers are urgently needed to make risk stratifications for pNEN patients, which could be beneficial to the development of individualized therapeutic strategy in the clinical practice. Here, we aimed to evaluate the predictive and prognostic roles of serum alkaline phosphatase-to-albumin ratio (APAR) in well-differentiated pNEN patients. Methods. We retrospectively analyzed the pathologically confirmed grade 1/2 pNEN patients, who were originally treated in our hospital from February 2008 to April 2018. Univariate and multivariate analyses were performed to assess the value of APAR in detecting synchronous metastases and predicting relapses following curative resections. Results. A total of 170 eligible cases were included into analysis. Logistic univariate analysis indicated APAR (P=0.002) was significantly associated with synchronous distant metastasis among well-differentiated pNEN patients, which was further demonstrated to be an independent risk factor by multivariate analysis (odds ratio 8.127, 95% confidence interval (CI) 2.105–31.372, P=0.002). For the prognostic value, APAR (P=0.007) was statistically associated with recurrence-free survival (RFS) in nonmetastatic resected pNEN patients, but it was not an independent predictor. Further subgroup analysis showed that APAR was independently related to RFS in patients with no nerve (hazard ratio (HR) 7.685, 95% CI 1.433–41.209, P=0.017) or vascular invasion (HR 4.789, 95% CI 1.241–18.473, P=0.023), respectively. Conclusion. APAR may work as a convenient pretreatment marker to detect synchronous distant metastasis for well-differentiated pNEN patients and predict recurrences for curatively resected cases without nerve or vascular invasion. However, these findings should be further verified in prospectively well-designed studies.


2020 ◽  
Vol 59 (24) ◽  
pp. 3245-3246
Author(s):  
Akinobu Koiwai ◽  
Morihisa Hirota ◽  
Kennichi Satoh

2021 ◽  
Author(s):  
Amelia Lanier ◽  
Chenchan Huang, MD ◽  
Sooah Kim, MD

2013 ◽  
Vol 28 (5) ◽  
pp. 750 ◽  
Author(s):  
Woo Hyun Paik ◽  
Ji Kon Ryu ◽  
Byeong Jun Song ◽  
Jaihwan Kim ◽  
Joo Kyung Park ◽  
...  

2019 ◽  
Vol 10 ◽  
Author(s):  
Giulia Bresciani ◽  
Leo J. Hofland ◽  
Fadime Dogan ◽  
Georgios Giamas ◽  
Teresa Gagliano ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document