scholarly journals Natural History of Renal Neuroendocrine Neoplasms: A NET by Any Other Name?

2021 ◽  
Vol 11 ◽  
Author(s):  
Andrew H. Nguyen ◽  
Michael P. O’Leary ◽  
James P. De Andrade ◽  
Philip H. G. Ituarte ◽  
Jonathan Kessler ◽  
...  

Renal neuroendocrine neoplasms are rare, with descriptions of cases limited to individual reports and small series. The natural history of this group of neuroendocrine neoplasms is poorly understood. In this study, we queried the Surveillance, Epidemiology and End Results (SEER) database over a four-decade period where we identified 166 cases of primary renal neuroendocrine neoplasms. We observed a 5-year overall survival of 50%. On multivariate analysis, survival was influenced by stage, histology, and if surgery was performed. We observed that patients managed by operative management had a greater frequency of localized or regional stage disease as well as a greater frequency of neuroendocrine tumor, grade 1 histology; whereas those managed non-operatively tended to have distant disease and histologies of neuroendocrine carcinoma, NOS and small cell neuroendocrine carcinoma. This is the largest description of patients with renal neuroendocrine neoplasms. Increased survival was observed in patients with earlier stage and favorable histologies.

2021 ◽  
Author(s):  
Jasna Metovic ◽  
Marco Barella ◽  
Fabrizio Bianchi ◽  
Paul Hofman ◽  
Veronique Hofman ◽  
...  

AbstractNeuroendocrine neoplasms (NENs) of the lung encompass neuroendocrine tumors (NETs) composed of typical (TC) and atypical (AC) carcinoids and full-fledged carcinomas (NECs) inclusive of large cell neuroendocrine carcinoma (LCNEC) and small cell carcinoma (SCLC). NETs and NECs are thought to represent distinct and separate lesions with neither molecular overlap nor common developmental continuum. Two perspectives were addressed regarding the morphologic and molecular classification of lung NENs: (i) a supervised approach by browsing the traditional classification, the relevant gene alterations, and their clinical implications; and (ii) an unsupervised approach, by reappraising neoplasms according to risk factors and natural history of disease to construct an interpretation model relied on biological data. We herein emphasize lights and shadows of the current classification of lung NENs and provide an alternative outlook on these tumors focused on what we currently know about the biological determinants and the natural history of disease.


2021 ◽  
Vol 2021 ◽  
pp. 1-9
Author(s):  
Kanako Bokuda ◽  
Satoshi Morimoto ◽  
Yasufumi Seki ◽  
Noriyoshi Takano ◽  
Atsuhiro Ichihara

Objective. Persistence of hyperparathyroidism (HPT) after renal transplantation leads to undesirable outcomes such as increase in cardiovascular events, graft dysfunction, and increased mortality. Options for therapy include medical management with calcimimetic or operative management. The present study was undertaken to evaluate the natural history of HPT after renal transplantation and to determine risk factors for persistent HPT in the era of calcimimetic. Design. The study is a retrospective review of data from 74 consecutive patients who underwent renal transplantation at our institution from April 2011 to November 2019. Methods. The natural history of HPT after renal transplantation and associations between intact parathyroid hormone (PTH) level after transplantation and clinical variables such as age, sex, duration of pretransplant dialysis, and use of calcimimetic before transplantation were evaluated. Results. Intact PTH decreased after renal transplantation in most of the patients without receiving parathyroidectomy. Known risk factors of persistent HPT did not associate with intact PTH level after renal transplantation in patients who had been receiving calcimimetic before transplantation. Conclusion. In conclusion, we have found that HPT after renal transplantation could be managed successfully by medical treatments. When predicting the prognosis of HPT after transplantation, pretransplant use of calcimimetic should be taken into consideration.


1995 ◽  
Vol 83 (5) ◽  
pp. 820-824 ◽  
Author(s):  
Douglas Kondziolka ◽  
L. Dade Lunsford ◽  
John R. W. Kestle

✓ To determine the natural history of brain cavernous malformations, the authors entered patients referred to their center into a prospective registry between 1987 and 1993. All patients underwent magnetic resonance imaging, which showed the typical appearance of this lesion, and conservative management was recommended in all. Patients or their referring physicians were contacted for follow-up data. The purpose of the study was to define the rate of symptomatic hemorrhage and to determine the outcome in those patients who had suffered seizures. Follow-up data were available for 122 patients with a mean age at entry of 37 years (range 4–82 years). The malformation was located in the brainstem in 43 cases (35%), the basal ganglia/thalamus in 20 (17%), and a hemispheric area in 59 (48%). Fifty percent of patients had never had a symptomatic hemorrhage, 41% had one bleed, 7% had two, and 2% had three. Seizures were reported in 23% of patients and headaches in 15%. Lesions were solitary in 80% of patients and multiple in 20%. The retrospective annual hemorrhage rate (61 bleeds/4550.6 patient-years of life) was 1.3%. The mean prospective follow-up period was 34 months. There were nine bleeds during this time, six with new neurological deficits. In patients without a prior bleed, the prospective annual rate of hemorrhage was 0.6%. In contrast, patients with prior hemorrhage had an annual bleed rate of 4.5% (p = 0.028). Patient sex (p = 0.97) or the presence of seizures (p = 0.11), headaches (p = 0.06), or solitary versus multiple lesions (p = 0.15) were not significant predictors of later hemorrhage. There was no difference in the rate of bleeds between brain locations. Four patients with seizures became seizurefree and four patients without seizures later developed seizures; only one patient developed intractable seizures. Fourteen patients (11%) underwent surgery (two after hemorrhage, five with seizures, and seven with progressive deficits), and five had radiosurgery. No patient died in the follow-up period. This study indicates that conservative versus operative management strategies may need to be redefined, especially in patients who present with hemorrhage and who appear to have a significantly increased risk of subsequent rehemorrhage.


2009 ◽  
Vol 27 (15_suppl) ◽  
pp. 10555-10555
Author(s):  
A. P. Dei Tos ◽  
S. Rossi ◽  
L. Messerini ◽  
I. Bearzi ◽  
G. Mazzoleni ◽  
...  

10555 Background: GIST represent a rare form of malignancy and one of best paradigms of molecularly targeted therapy. While the natural history of GIST following treatment with tyrosine kinase inhibitors is relatively well known, most information on imatinib naïve GIST are drawn form either small series or larger studies with possible referral bias. Methods: 1,021 GIST diagnosed between 1980 and 2000 were retrieved from the archives of 35 Departments of Pathology throughout Italy. Pathologic and clinical data were collected. All cases were centrally reviewed and stratified according to NIH 2002 and NCCN 2008 risk classifications. The prognostic meaning of a large set of morphologic and clinical parameters were evaluated by uni- and multi-variate analysis. Informed consent was obtained from all living patients. Results: 92 cases were excluded as non-GIST. Mean and median age were 63 and 65 years. Females were 52%. Disease status at diagnosis was localized in 767 and metastatic in 111 cases. 516 cases were gastric, 229 ileal/jejunal, 26 duodenal, 22 colonic, 28 rectal, and 25 peritoneal/retroperitoneal. NIH 2002 risk classification was available in 917 cases: 121 (13%) very low, 247 (27%) low, 201 (22%) moderate and 348 (38%) high risk. NCCN 2008 risk classification was available in 892 patients. Metastatic sites included liver (5%), peritoneum (10%), lymph-node (1%), skin and soft tissue (0.5%), and lungs (0.2%). Median follow-up was 75 months (range 1–323). Median survival was 88 months. 62% patients died (ED in 46%). Mitotic activity, tumor size and anatomic site correlated with overall survival at multivariate analysis. Striking differences were observed for groups with < 5 mitoses/50HPF, 5 and 10, 10 and 30, and >30. Non gastric GIST exhibit a significant poorer outcome. R2 surgery represents a negative prognostic factor. Conclusions: Mitotic rate has a deep prognostic impact if split in more than two intervals. Setting only two categories may be a limitation of existing prognostic classifications, although the mitotic count is affected by reproducibility issues. Survival curves according to the two main prognostic classifications will be shown. [Table: see text]


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