Evaluation of a Nep-Score Threshold and the Derived Nep-D Score in Predicting Survival of Patients With Typical and Atypical Bronchial Carcinoids

Abstract Background: Typical and atypical bronchial carcinoids (TBC and ABC) display a wide range of clinical presentations and may behave very differently. Survival prognostic markers are necessary to better define therapeutic strategies. AIM: verify that the NEP-Score, recently proposed as prognostic score, can be applied in a homogeneous TBC and ABC cohort and identify a derivate prognostic marker taking into account clinical and pathological characteristics at diagnosis. Methods: Age, site of primary tumor, primary tumor surgery, symptoms, Ki67, timing of metastases of 64 patients including TBC and ABC were evaluated to calculate the NEP-Score at the end of follow-up (NEP-T). We then assessed a derivative score considering the NEP-Score at diagnosis (NEP-D): this score does not consider the appearance of new metastases during follow-up. We then considered the patients that were alive or dead at the end of follow-up (EOF). A NEP-Score threshold to predict survival was investigated. Results: live patients at EOF displayed a mean NEP-T and mean NEP-D significantly lower as compared to those that were dead. A NEP-T threshold >138 significantly predicts survival. ABC relapsed more frequently as compared to TBC. Male gender as well as previous malignancy were negative prognostic factors for survival. Conclusions: We found that NEP-Score is applicable to a series of bronchial neuroendocrine neoplasms. In addition, we propose NEP-D as a simple, quick and cheap prognostic score that can help clinicians in decision making. Moreover, the use of a NEP-D threshold can predict NEN aggressiveness and may be used to define the best personalized therapeutic strategy. Furthermore we found additional prognostic factors that together with the NEP-Score could improve prognosis evaluation at diagnosis by using easily accessible information.

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NEP-Score Thresholds Predict Survival of Patients With Bronchial Carcinoids

Frontiers in Endocrinology ◽

10.3389/fendo.2020.621557 ◽

2021 ◽

Vol 11 ◽

Author(s):

Irene Gagliardi ◽

Mario Tarquini ◽

Maria Rosaria Ambrosio ◽

Elisa Giannetta ◽

Patricia Borges de Souza ◽

...

Keyword(s):

Decision Making ◽

Prognostic Factors ◽

Therapeutic Strategy ◽

Prognostic Markers ◽

Prognostic Score ◽

Therapeutic Strategies ◽

Male Gender ◽

Bronchial Carcinoids ◽

Score Threshold

Survival prognostic markers are extremely needed to better define therapeutic strategies in patients with bronchial carcinoids (BC). We aim to verify the applicability of the NEP-Score in a homogeneous BC cohort and identify a derivative prognostic marker, the NEP-Score at diagnosis (NEP-D) that does not consider new metastases during follow-up. Sixty-four patients (38 females, and 26 males, mean age at diagnosis 58.9 ± 1.7 years) with BC were retrospectively evaluated. NEP-Score was calculated at the end of follow-up (NEP-T). A derivative score, the NEP-Score at diagnosis (NEP-D) that does not consider new metastases during follow-up, was then assessed. Patients were subdivided according to their living status at the end of follow-up. A NEP-Score threshold was investigated to predict survival. Mean NEP-T and mean NEP-D were significantly lower in live patients at end of follow-up. A NEP-T cut-off >138 significantly predicts survival. Atypical BC relapsed more frequently than Typical BC. Male gender and previous malignancy were negative prognostic factors for survival. We confirmed NEP-Score applicability in BC and NEP-D utility, being the latter a simple, quick, and cheap prognostic score that can help clinicians in decision making. The identified NEP-D threshold can predict NEN aggressiveness and may be used to define the best personalized therapeutic strategy. In this context, a validation study is needed.

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Update Regarding Imaging of Neuroendocrine Neoplasms

RöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren ◽

10.1055/a-1001-2412 ◽

2019 ◽

Vol 192 (02) ◽

pp. 171-182

Author(s):

Jens Holger Figiel ◽

Simon G. Viniol ◽

Jannis Görlach ◽

Anja Rinke ◽

Damiano Librizzi ◽

...

Keyword(s):

Neuroendocrine Neoplasm ◽

Neuroendocrine Neoplasms ◽

Primary Tumors ◽

Advantages And Disadvantages ◽

Key Points ◽

Wide Range ◽

Small Primary ◽

Noninvasive Therapy ◽

Spectrum Of Indications

Background Neuroendocrine neoplasms (NEN) are a heterogeneous group of tumors characterized by the expression of typical proteins. A wide range of morphological and functional imaging methods is required in order to adequately assess the course of the disease and to optimally treat the patient. The spectrum of indications ranges from the detection of small primary tumors to the documentation of the metastasis pattern and the assessment of the suitability for certain invasive or noninvasive therapy methods. The exact recording and quantification of findings is indispensable. Methods This article is based on a comprehensive literature search on the different aspects of neuroendocrine neoplasm imaging. Results This article is intended to provide an overview of the available imaging procedures with their respective advantages and disadvantages for diagnostics and their value for the follow-up of neuroendocrine neoplasms. Recommendations for examination protocols, typical image findings, and an outlook regarding future developments are presented. Key Points: Citation Format

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Survival after locoregional recurrence in patients after breast cancer surgery.

Journal of Clinical Oncology ◽

10.1200/jco.2011.29.27_suppl.141 ◽

2011 ◽

Vol 29 (27_suppl) ◽

pp. 141-141

Author(s):

T. Osako ◽

R. Nishimura ◽

Y. Okumura ◽

R. Tashima ◽

Y. Toyozumi ◽

...

Keyword(s):

Breast Cancer ◽

Multivariate Analysis ◽

Prognostic Factors ◽

Primary Tumor ◽

Locoregional Recurrence ◽

Distant Metastases ◽

Breast Conserving Therapy ◽

Breast Cancer Surgery ◽

Systemic Metastasis

141 Background: The purpose of this study was to investigate factors for survival after locoregional recurrence in patients who underwent mastectomy or breast-conserving therapy (BCT) for primary breast cancer in our hospital. Methods: Out of 3,332 patients operated on from 1989 to 2008, 50 patients had chest wall recurrences after mastectomy (CWR), 40 patients had regional nodal recurrences (RNR), and 24 patients had ipsilateral breast tumor recurrences (IBTR) from 1997 to 2008. To investigate the prognostic factors for survival after locoregional recurrence, we conducted uni- and multivariate analyses of these cases. Results: The median follow-up time was 49.2 months. The 5-year survivals after recurrence of the patients with CWR, RNR and IBRT were 52%, 28%, and 68%, respectively. And the 10-year survivals were 15%, 0%, and 62%, respectively. Furthermore, the 5-year distant metastasis-free survivals were 24%, 13%, and 59%, respectively. In a multivariate analysis of the patients with CWR, type of recurrent nodules (diffuse/single, RR 21.0, p= 0.001), pT (T3 or 4 /T1, RR 11.4, p=0.01), pN (N3/N0, RR 15.5, p= 0.03), Ki67 of primary tumor (>50%/<20%, RR6.7, p=0.02) and ER of the primary tumor (+ / -, RR 2.6, p = 0.02) were independent prognostic factors. In a multivariate analysis of RNR, the method of first line salvage therapy (local /local + systemic, RR 16.1, p = 0.01) was only an independent prognostic factor. In the cases of IBTR, there were no independent prognostic factors for survival after recurrence. Conclusions: Although CWR developed distant metastases within 5 years, the survival depended upon the several biological factors. RNR developed distant metastases within a few years and provided poor prognosis. These suggested that RNR would be the first appearance of systemic metastasis not local disease. In contrast, IBTR provided better prognosis and a salvage treatment cured about 60% of the patients.

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A 15-year pheochromocytoma and paraganglioma experience in a single centre: a Singapore perspective

Singapore Medical Journal ◽

10.11622/smedj.2021135 ◽

2021 ◽

Author(s):

Y Lee ◽

LYR Tan ◽

SAKK Abdul Shakoor

Keyword(s):

Positive Family History ◽

Distant Metastases ◽

Disease Recurrence ◽

Haemodynamic Instability ◽

Preoperative Preparation ◽

Presenting Symptoms ◽

Clinical Presentations ◽

Wide Range

Introduction: Pheochromocytomas (PCC) and paragangliomas (PGL) are rare endocrine tumours. The objective of this study was to describe our experience with these two entities in a Singapore population. Methods: We identified patients with positive histopathological confirmations of PCC and PGL who were treated at a tertiary Singapore hospital between January 2000 and December 2015. The results were analysed for clinical presentations, treatment and long-term outcomes. Results: A total of 27 cases (20 PCC, 7 PGL) were identified over a 15-year period. One case of PGL developed bilateral disease on follow-up. There were 17 male and 10 female patients with a median age of 57 (range 24–77) years. A positive family history was uncommon and present in only 3.7% of patients. Uniquely, the top three presenting symptoms were abdominal discomfort, palpitations and diaphoresis. Despite adequate preoperative preparation, intraoperative haemodynamic instability occurred in 70.4% and early postoperative hypotension occurred in 11.1% of patients. After surgery, hypertension was resolved in 41.2% (7/17) and diabetes mellitus in 60% (3/5). Disease recurrence was reported in 22.2% and distant metastases in 14.8%. At the end of the follow-up period (median 35 [range 3–148] months), 70.4% were still alive. Conclusion: PCC and PGL can present with a wide range of symptoms. Intraoperative haemodynamic instability was frequent despite good preoperative preparation. Disease recurrences and metastasis occurred in up to one-fifth of the patients. Genetic screening should be offered to patients with PCC and PGL.

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Gingival Bleeding of a High-Flow Mandibular Arteriovenous Malformation in a Child with 8-Year Follow-Up

Case Reports in Pediatrics ◽

10.1155/2015/745718 ◽

2015 ◽

Vol 2015 ◽

pp. 1-5 ◽

Cited By ~ 2

Author(s):

Elvira Ferrés-Amat ◽

Jordi Prats-Armengol ◽

Isabel Maura-Solivellas ◽

Eduard Ferrés-Amat ◽

Javier Mareque-Bueno ◽

...

Keyword(s):

Imaging Study ◽

Neck Region ◽

Arterial Embolization ◽

High Flow ◽

Gingival Bleeding ◽

Clinical Presentations ◽

Wide Range ◽

Life Threatening ◽

Flow Through

Intraosseous arteriovenous malformations (AVMs) in the head and neck region are uncommon. There are several types and they can have a wide range of clinical presentations. Depending on the blood flow through the AVM, the treatment may be challenging for the attending team and may lead to life-threatening hemorrhages. A clinical case report is presented. A 9-year-old girl, seen for gingival bleeding during oral hygiene, was found to have a high-flow AVM located within and around the mandible. Two-stage treatment consisted of intra-arterial embolization followed by intraoral injection of a sclerosing agent 8 weeks later. At the 8-year follow-up, imaging study showed no evidence of recurrent lesion inside or outside the bone. The final outcome is a correct occlusion with a symmetric facial result. This case shows that conservative treatment may be the first treatment option mostly in children. Arteriography and transcortical injection were enough to control the AVM.

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Clinical Presentation and Outcomes of Coil Embolization of Remnant or Recurred Intracranial Aneurysm After Clipping

Neurosurgery ◽

10.1227/01.neu.0000367998.33743.d6 ◽

2010 ◽

Vol 66 (6) ◽

pp. 1128-1133 ◽

Cited By ~ 18

Author(s):

Byung Moon Kim ◽

Dong Joon Kim ◽

Dong Ik Kim ◽

Sung Il Park ◽

Sang Hyun Suh ◽

...

Keyword(s):

Prognostic Factors ◽

Coil Embolization ◽

Clinical Presentation ◽

Imaging Modality ◽

Mass Effect ◽

Poor Outcome ◽

Ct Angiogram ◽

Clinical Presentations ◽

Complete Obliteration

Abstract OBJECTIVE To evaluate clinical presentation, safety, techniques, clinical and angiographic outcomes, and prognostic factors of coiling for remnant/recurred aneurysm after clipping. METHODS Twenty-four consecutive patients (11 men and 13 women; mean age, 52 years) with 24 recurred/remnant aneurysms after clipping underwent coil embolization between September 2000 and December 2008. Clinical presentations of remnant/recurred aneurysms, safety, techniques, clinical and angiographic outcomes, and prognostic factors of coil embolization were retrospectively evaluated. RESULTS Twenty-two aneurysms initially presented with subarachnoid hemorrhage and the other two, with mass effect. Eight aneurysms presented with rebleeding and 16 aneurysms were found on follow-up CT angiogram (n = 12) or catheter angiogram (n = 4). The interval between clipping and coiling ranged from 8 days to 114 months (mean, 31 months). Twelve were treated by using single-catheter, 6 by stent-assisted, 4 by multicatheter, 1 by both balloon- and catheter-assisted, and 1 by balloon-in-stent technique. Immediate postembolization angiogram revealed complete obliteration (n = 19) or residual neck (n = 5). Procedure-related permanent morbidity and mortality rates were 4.2% (1 of 24) and 0%, respectively. There was no rebleeding during clinical follow-up for 3 to 82 months (mean, 24 months). Presentation with rupture after clipping was the only significant predictor of poor outcome (P < .05). CONCLUSION Coiling seems to be a safe and effective retreatment option for remnant/ recurred aneurysm after clipping. Presentation with rupture after clipping is the only predictor of poor outcome. For routine/regular follow-up after clipping, CT angiography may be the imaging modality advisable for detection of remnant/recurred aneurysm.

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Six Cycles of BEACOPP Tailored Based on Interim Scintigraphy Provide Favorable Outcome for Patients with Standard and Advanced Risk Hodgkin Lymphoma and Female Fertility Is Preserved: Results of a 10-Year Follow-up

Blood ◽

10.1182/blood.v118.21.1564.1564 ◽

2011 ◽

Vol 118 (21) ◽

pp. 1564-1564

Author(s):

Eldad J Dann ◽

Zeev Blumenfeld ◽

Rachel Bar-Shalom ◽

Irit Avivi ◽

Menachem Ben-Shachar ◽

...

Keyword(s):

Prognostic Factors ◽

Hodgkin Lymphoma ◽

Gnrh Agonist ◽

Ovarian Function ◽

Prognostic Score ◽

Individual Risk ◽

Term Survival ◽

Pet Ct

Abstract Abstract 1564 Background: Therapy of Hodgkin lymphoma (HL) is designed to prolong survival and minimize toxicity; however, an intense debate is ongoing over whether ABVD or BEACOPP should be used upfront and whether a long-term survival benefit is obtained with the BEACOPP regimen. The current study was aimed to personalize therapy based on individual risk factors and interim scintigraphy. Design and Methods: 124 patients with newly diagnosed HL and adverse prognostic factors were prospectively studied between 7/1999 and 8/2005. Patients with early unfavorable and advanced disease were eligible for the study. Study participants were assigned to therapy based on their International Prognostic Score (IPS). Those with an IPS of ≥3 were assigned to 2 cycles of escalated BEACOPP (EB). All others received 2 cycles of standard BEACOPP (SB). Subsequent therapy was prospectively allocated according to the results of early interim GA67 or PET/CT. Four cycles of EB or SB were administered following a positive or negative scan, respectively. Results: Complete remission (CR) rate, 10-year progression free (PFS) and overall survival (OS) were 97%, 87% and 88%, respectively, at a median follow-up of 89 months (5–144). Only 38% of patients, mostly with a bulky mediastinal mass, received radiation therapy. PFS and OS were similar in both groups. Predictive value of negative interim Ga67 or PET/CT was 87% and 93%, respectively. Fertility status was assessed in 38 females aged <40 years (Table). Twenty four of these patients received 6 cycles of SB, 8 patients had 2 cycles of EB and 4 cycles of SB, 3 patients were treated with 2 cycles of SB and 4 cycles of EB and one patient received 6 cycles of EB. Thirty six females did not have disease progression and 34 of them were found to preserve their cyclic ovarian function (COF). Twenty six females were co-treated with 6 cycles of GNRH agonist triptorelin (Decapeptyl 3.75 mg) for 6 months. 19 patients conceived during the follow-up (30 pregnancies), delivering 23 babies. Deliveries were reported for up to seven years from diagnosis. Notably, the female receiving 6 cycles of EB, co-administered with the GNRH agonist, was among those who conceived and delivered a healthy baby. Conclusion: Six cycles of tailored BEACOPP administered to HL patients with adverse prognostic factors provide encouraging long-term PFS and OS, and fertility is preserved in most females following reduction in the number of chemotherapy cycles and GNRH agonist co-administration. Disclosures: Off Label Use: GNRH agonist triptorelin was simultaneously administered for fertility preservation.

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The criteria of aggressiveness and other prognostic factors for predicting relapses of primary tumors and imatinib (IM) treatment outcomes in advanced KIT immunopositive gastrointestinal stromal tumors (GIST): A report of the Polish Clinical GIST Registry (PCGR)

Journal of Clinical Oncology ◽

10.1200/jco.2006.24.18_suppl.9544 ◽

2006 ◽

Vol 24 (18_suppl) ◽

pp. 9544-9544

Author(s):

P. Rutkowski ◽

Z. I. Nowecki ◽

W. Michej ◽

M. Debiec-Rychter ◽

J. Limon ◽

...

Keyword(s):

Prognostic Factors ◽

Mitotic Index ◽

Primary Tumor ◽

Risk Group ◽

Primary Tumors ◽

Kit Mutation ◽

Free Survival ◽

Group I ◽

Group Ii

9544 Background: The development of adjuvant treatment trials with imatinib in GIST has raised the debate about the accuracy of NIH risk criteria consensus and about the significance of other prognostic factors. Methods: We analyzed the criteria of aggressiveness and other clinico-pathological and genetic factors influencing disease-free survival (DFS) in patients with primary CD117-positive tumors (group I: 274 patients; median follow-up 29 months; calculated from primary tumor resection) and progression-free survival (PFS) in metastatic/unresectable GIST patients treated with IM (group II: 179 patients; median follow-up 19 months; calculated from the start of imatinib therapy) enrolled into PCGR. Results: In group I statistically significant (p<0.05) factors negatively influencing DFS both in univariate (log-rank test) and multivariate (Cox’s model) analysis were: primary tumor size > 5 cm, mitotic index > 5/50HPF, male gender, primary tumor R1 resection or tumor rupture, non-gastric primary tumor localization and intermediate/high risk group (3-year DFS for high, intermediate and low/very low risk group was: 28%, 75% and 99%, respectively). In group II we identified 5 factors negatively affecting PFS statistically significant both in univariate and multivariate analyses (p<0.05): tumor genotype indicating other than exon 11 KIT mutation, mitotic index > 10/50HPF, age below 45 years at diagnosis, high baseline pre-IM granulocyte count and poor WHO performance status ≥ 2. Conclusions: We validated the value of criteria of risk groups for the assessment of the natural course of primary GIST, but we also identified additional independent prognostic factors. For the prediction of PFS during IM therapy for advanced GIST we detected 5 different, independent biological and pathological factors. No significant financial relationships to disclose.

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Long-term overall survival (os) and prognostic factors of patients (pts) with surgery of liver metastases from colorectal cancer origin (lm/crc): Retrospective analysis of a Spanish single institution

Journal of Clinical Oncology ◽

10.1200/jco.2009.27.15_suppl.e15052 ◽

2009 ◽

Vol 27 (15_suppl) ◽

pp. e15052-e15052

Author(s):

I. R. Quispe ◽

F. J. Ramos ◽

I. Bilbao ◽

T. Macarulla ◽

S. Cedres ◽

...

Keyword(s):

Prognostic Factors ◽

Liver Resection ◽

Primary Tumor ◽

Disease Free Survival ◽

Extrahepatic Disease ◽

Term Survival ◽

Free Survival ◽

Tumor Number

e15052 Background: Hepatectomy is the only potentially curative treatment for LM/CRC. Long-term survival following liver resection (LR) has improved. The aim of this study was to analyze prognostic factors and OS after LR Methods: A retrospective review of the medical records of pts who underwent LR for LM/CRC from 1997 to 2007 was conducted. Multiple potential prognostic factors for survival were analyzed: synchronic vs. metachronic, post-op chemotherapy (CT) vs. no, lymph node (LN) status of primary tumor, R0 vs. R1–2 resection, gender, location of primary tumor, number and size of metastases, and unilobar vs. bilobar disease. Results: 190 pts (colon 130, rectum 60) underwent LR. Pts’ characteristics: 123 male, 67 female; median age 63 yrs (32–85); 95 pts synchronic, 95 metachronic; 145 pts (76%) received post-op CT; LN status was pN0 in 61 (32%) pts, pN1 in 74 (39%) and pN2 in 52 (27%). A R0 resection was performed in 164 (86%) and R1 in 25 (13%). Post-op mortality was 2.6%.Median OS was 68 months with 5- and 10-year OS of 52% and 31%, and 5- and 10-year disease free survival of 36% and 18%. 5-year OS differed between metachronic and synchronic (64% vs. 39%; p<0.001); LN-status (73% for pN0 vs. 41% for pN1–2; p<0.001); R0/R1–2 surgery (58% for R0 vs. 21% for R1- 2; p=0.002). With a median follow-up of 70 months, 71 (37%) pts remained free of disease, 60 (32%) had new metachronic LM/CRC, 18 (9.5%) had LM/CRC and extrahepatic disease and 36 (19%) had extrahepatic disease alone. Non-significant factors for prognosis were gender, location of primary tumor, number and size of metastases, bilobar disease and adjuvant CT. Conclusions: Pts with LM/CRC who undergo liver resection can achieve long-term OS, our data identifying as prognostic factors: LN status, synchronic/metachronic disease and Type of resection (R0). No significant financial relationships to disclose.

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The impact of tuberous sclerosis complex – a parent’s perspective

Advances in Autism ◽

10.1108/aia-12-2015-0026 ◽

2016 ◽

Vol 2 (2) ◽

pp. 70-75

Author(s):

Helen Willacy

Keyword(s):

Tuberous Sclerosis ◽

Tuberous Sclerosis Complex ◽

Professional Service ◽

Content Type ◽

Her Family ◽

Accessible Information ◽

Wide Range ◽

The Impact

Purpose – The purpose of this paper is to describe the impact of having a child with tuberous sclerosis complex (TSC). Design/methodology/approach – The author explains the process her family has been through on their TSC journey. Findings – Several themes emerge from the author’s story: a lack of awareness about relatively common conditions such as epilepsy; a dearth of accessible information provided to families; delays in identification/assessment and a lack of follow up on parents’ concerns; the wide range in the quality of advice provided from one professional/service to another; the extent to which parents have to advocate for their children and the importance of social support. Originality/value – Few journal articles explore the impact of complex health conditions on families from their perspective and in their own words. This thought provoking viewpoint piece provides a powerful insight into the reality of having a child with TSC.

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