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1.
Ann Surg Oncol ; 31(1): 475-487, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-37768414

RESUMEN

BACKGROUND: Pancreatic solid pseudopapillary neoplasms (SPN) are generally indolent; however, some patients present with "malignant" SPN. An orthogonal analysis of multiple datasets was performed to investigate the utility of complete surgical resection (CSR) for malignant SPN. METHODS: A systematic review was performed for cases of malignant SPN, defined as T4, N1, and/or M1. Malignant SPN was analyzed within the National Cancer Database (NCDB) and compared with T1-3N0M0 SPN. Predictors of malignant SPN were assessed, and treatments were analyzed by using survival analysis. RESULTS: The systematic review yielded 164 cases of malignant SPN. Of 31 children, only one died due to malignant SPN. Among adults, CSR was associated with improved disease-specific survival (DSS) (P = 0.0002). Chemotherapy did not improve malignant SPN DSS, whether resected (P = 0.8485) or not (P = 0.2219). Of 692 adults with SPN within the NCDB, 93 (13.4%) had malignant SPN. Pancreatic head location (odds ratio [OR] 2.174; 95% confidence interval [CI] 1.136-4.166; P = 0.0186) and tumor size (OR 1.154; 95% CI 1.079-1.235; P < 0.0001) associated with the malignant phenotype. Malignant SPN predicted decreased overall survival (OS) compared with T1-3N0M0 disease (P < 0.0001). Resected malignant SPN demonstrated improved OS (P < 0.0001), including resected stage IV malignant SPN (P = 0.0003). Chemotherapy did not improve OS for malignant SPN, whether resected (P = 0.8633) or not (P = 0.5734). Within a multivariable model, resection was associated with decreased hazard of death (hazard ratio 0.090; 95% CI 0.030-0.261; P < 0.0001). CONCLUSIONS: Approximately 13% of patients with SPN present with a malignant phenotype. Pediatric cases may be less aggressive. Resection may improve survival for malignant SPN, which does not appear chemosensitive.


Asunto(s)
Carcinoma Papilar , Neoplasias Pancreáticas , Adulto , Humanos , Niño , Neoplasias Pancreáticas/cirugía , Neoplasias Pancreáticas/patología , Páncreas/cirugía , Pancreatectomía , Pancreaticoduodenectomía , Carcinoma Papilar/cirugía , Carcinoma Papilar/patología
2.
J Surg Oncol ; 2024 Aug 19.
Artículo en Inglés | MEDLINE | ID: mdl-39155692

RESUMEN

BACKGROUND AND OBJECTIVES: Solid pseudopapillary neoplasm (SPN) of the pancreas demonstrates an indolent disease course; however, some patients present with a "malignant" phenotype, including distant metastases resistant to chemotherapy. This analysis identifies molecular drivers of metastatic SPN using the world's largest clinicogenomics database. METHODS: The American Association for Cancer Research Project Genomics Evidence Neoplasia Information Exchange was queried for primary and metastatic SPN samples. Sample-level genomic alterations were compared. A pan-pancreatic cancer analysis assessed relevant mutations among all metastatic pancreatic malignancies. RESULTS: Among 28 SPN samples identified (n = 17 primary, n = 11 metastatic), the most commonly mutated gene was CTNNB1, (24/28 samples; 85.7%). Most mutations were missense (21/24; 87.5%) or in-frame deletions (3/24; 12.5%). The most common CTNNB1 mutations in primary SPN were exon 3 S37F/C missense mutations (6/16 profiled patients, 37.5%), contrasting exon 3 D32N/Y/H missense mutations in metastatic samples (6/11 profiled patients, 54.5%). Metastatic SPN had higher rates of CTNNB1 mutations than metastases from pancreatic ductal adenocarcinoma (72.7% vs. 1.1%; q < 0.0001), pancreatic neuroendocrine tumor (72.7% vs. 2.5%; q < 0.0001), and pancreatic acinar cell carcinoma (72.7% vs. 11.5%; q = 0.0254). CONCLUSIONS: Missense mutations along exon 3 of CTNNB1 predominate metastatic SPN, differentiating these patients from those with metastases from analogous pancreatic malignancies.

3.
Ann Surg Oncol ; 30(2): 738-747, 2023 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-36319871

RESUMEN

BACKGROUND: We sought to determine the impact of social determinants of health (SDoH) on outcomes of patients undergoing resection for hepatocellular carcinoma (HCC). PATIENTS AND METHODS: Patients with HCC in the National Cancer Database who underwent resection from 2009 to 2018 were identified. SDoH associated with length of stay (LOS), 30-day readmission, and 30-day mortality were analyzed using regression analyses adjusted for confounding variables. RESULTS: Among 9235 patients, the median age (range) was 65.0 (18-90) years, 72.1% were male, and 57.9% were White. A total of 3% were uninsured, 11.1% had Medicaid, 21% resided in regions with a median household income within the lowest quartile of the US population, and 27.0% resided in regions within the lowest quartile of education level. The odds for having longer LOS were lower among patients with the highest regional education level compared with those with the lowest level [odds ratio (OR) 0.86, 95% confidence interval (CI) 0.77-0.97]. The risk of readmission was lower among patients with Medicare (OR 0.52; 95% CI 0.33-0.81), Medicaid (OR 0.52; 95% CI 0.31-0.87), or private insurance (OR 0.56; 95% CI 0.35-0.88) compared with uninsured patients. Thirty-day overall mortality was less likely among patients with Medicare (OR 0.45; 95% CI 0.27-0.75), Medicaid (OR 0.53; 95% CI 0.30-0.93), or private insurance (OR 0.40; 95% CI 0.24-0.66), and among patients with high regional income (OR 0.58; 95% CI 0.44-0.77). CONCLUSIONS: Adjusted regression analyses identified SDoH that were associated with HCC outcomes. Increased awareness of how SDoH relate to outcomes may inform strategies that attempt to account for these associations and improve patient outcomes.


Asunto(s)
Carcinoma Hepatocelular , Neoplasias Hepáticas , Humanos , Masculino , Anciano , Estados Unidos/epidemiología , Femenino , Medicare , Carcinoma Hepatocelular/cirugía , Determinantes Sociales de la Salud , Neoplasias Hepáticas/cirugía , Medicaid
4.
J Surg Oncol ; 127(5): 815-822, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36629137

RESUMEN

BACKGROUND AND OBJECTIVES: Published data comparing peritoneal metastases from appendiceal cancers (pAC) and colorectal cancers (pCRC) remain sparse. We compared pAC and pCRC using comprehensive tumor profiling (CTP). METHODS: CTP was performed, including next-generation sequencing and analysis of copy number variation (CNV), microsatellite instability (MSI) and tumor mutational burden (TMB). RESULTS: One hundred thirty-six pAC and 348 pCRC samples underwent CTP. The cohorts' age and gender were similar. pCRC demonstrated increased pathogenic variants (PATHs) in APC (48% vs. 3%, p < 0.01), ARID1A (12% vs. 2%, p < 0.01), BRAF (12% vs. 2%, p < 0.01), FBXW7 (7% vs. 2%, p < 0.01), KRAS (52% vs. 41%, p < 0.05), PIK3CA (15% vs. 2%, p < 0.01), and TP53 (53% vs. 23%, p < 0.01), and decreased PATHs in GNAS (8% vs. 31%, p < 0.01). There was no difference in CNV, fusion rate, or MSI. Median TMB was higher in pCRC (5.8 vs. 5.0 mutations per megabase, p = 0.0007). Rates of TMB-high tumors were similar (pAC 2.1% vs. pCRC 9.0%, p = 0.1957). pCRC had significantly more TMB-high tumors at lower thresholds. CONCLUSIONS: Despite a reduced overall TMB, pAC demonstrated mutations distinct from those seen in pCRC. These may serve as discrete biomarkers for future study.


Asunto(s)
Neoplasias del Apéndice , Neoplasias Colorrectales , Neoplasias Peritoneales , Humanos , Neoplasias Colorrectales/genética , Neoplasias Colorrectales/patología , Neoplasias Peritoneales/genética , Neoplasias Peritoneales/secundario , Variaciones en el Número de Copia de ADN , Neoplasias del Apéndice/genética , Neoplasias del Apéndice/patología , Mutación , Inestabilidad de Microsatélites , Biomarcadores de Tumor/genética
5.
J Surg Oncol ; 128(8): 1268-1277, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37650827

RESUMEN

BACKGROUND: Children, adolescents, and young adults (CAYA) (age ≤39 years) with GIST have high rates of LNM, but their clinical relevance is undefined. This study analyzed the impact of LNM on overall survival (OS) for CAYA with GIST. METHODS: The National Cancer Database was queried for patients with resected GIST and pathologic nodal staging data from 2004-2019. Factors associated with LNM were identified. Survival was assessed stratified by presence of LNM. RESULTS: Of 4420 patients with GIST, 238 were CAYA (5.4%). When compared to older adults, CAYA more often had small intestine primaries (51.8% vs. 36.6%, p < 0.0001), T4 tumors (30.7% vs. 24.5%, p = 0.0275) and pN1 disease (11.3% vs. 4.7%, p < 0.0001). Within a multivariable Cox proportional hazards regression model adjusting for age, comorbid disease, mitotic rate, tumor size, and primary site, LNM were associated with increased hazard of death for older adults (hazard ratio [HR]: 1.83; confidence interval [CI]: 1.35-2.42; p < 0.0001), but not CAYA (HR: 3.38; CI: 0.50-14.08; p = 0.13). For CAYA, only high mitotic rate predicted mortality (HR: 4.68; CI: 1.41-18.37: p = 0.02). CONCLUSIONS: LNM are more commonly identified among CAYA with resected GIST who undergo lymph node evaluations, but do not appear to impact OS as observed in older adults. High mitotic rate remains a predictor of poor outcomes for CAYA with GIST.


Asunto(s)
Tumores del Estroma Gastrointestinal , Adulto Joven , Niño , Humanos , Anciano , Adolescente , Adulto , Metástasis Linfática/patología , Tumores del Estroma Gastrointestinal/patología , Tasa de Supervivencia , Ganglios Linfáticos/cirugía , Ganglios Linfáticos/patología , Modelos de Riesgos Proporcionales , Estadificación de Neoplasias , Estudios Retrospectivos , Pronóstico
6.
Surg Endosc ; 37(11): 8316-8325, 2023 11.
Artículo en Inglés | MEDLINE | ID: mdl-37679582

RESUMEN

BACKGROUND: Same day discharge (SDD) may be considered in some patients undergoing minimally invasive adrenalectomy (MIA). Recent studies have demonstrated similar outcomes between SDD and admitted patients; however, most excluded pheochromocytoma and adrenal metastases. This study evaluates 30-day complications and hospital readmission in a large cohort of patients undergoing MIA. METHODS: Adult patients undergoing MIA (2010-2020) for benign adrenal disorders, pheochromocytoma, and adrenal metastases were identified within the ACS-NSQIP database. Comparisons between patients having SDD versus admission were performed. Factors associated with 30-day complications and unplanned readmission were evaluated by multivariable regression modeling. RESULTS: Of 7316 patients who underwent MIA, 254 had SDD. Baseline characteristics were similar between groups, although SDD patients had lower ASA class (p < 0.001) and were more likely to undergo MIA for nonfunctioning adenoma or primary aldosteronism (p = 0.001). After adjusting for covariates, higher ASA class and presence of medical comorbidities were associated with increased complications (p < 0.001; p < 0.05) and unplanned readmission (p < 0.001; p < 0.05). Additionally, prolonged operative time was associated with 30-day complications (p < 0.001). Notably, SDD was not associated with increased complications (OR 0.78, 95% CI 0.38-1.61, p = 0.502) or unplanned readmission (OR 0.76, 95% CI 0.35-1.64, p = 0.490). The rate of SDD for MIA increased from 1.48% in 2017 to 10.81% in 2020. CONCLUSIONS: Not all patients undergoing MIA should have SDD; however, the current analysis demonstrates a trend toward SDD and supports its safety in select patients with adrenal metastases and benign adrenal disorders including pheochromocytoma.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Feocromocitoma , Adulto , Humanos , Estudios Retrospectivos , Alta del Paciente , Feocromocitoma/cirugía , Adrenalectomía/efectos adversos , Readmisión del Paciente , Neoplasias de las Glándulas Suprarrenales/cirugía , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología
7.
HPB (Oxford) ; 25(12): 1566-1572, 2023 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-37652810

RESUMEN

BACKGROUND: Utilization of minimally-invasive distal pancreatectomy (MIDP) for pancreatic adenocarcinoma has increased. While unplanned conversion to an open procedure during MIDP is associated with inferior short-term outcomes, the long-term consequences of conversion have not been adequately examined. METHODS: Patients with pancreatic adenocarcinoma undergoing MIDP were selected from the National Cancer Database (2010-2015) and subdivided based on the occurrence of unplanned conversion. Post-operative outcomes and overall survival (OS) were examined. Conversion was additionally compared to a matched group of planned open resections. RESULTS: Among 592 patients undergoing attempted MIDP, unplanned conversion occurred in 23.1%. Despite increased 90-day mortality among patients experiencing conversion, there was no difference in median OS between groups (25.0 vs 27.8 months, p = 0.095). For patients undergoing conversion, post-operative outcomes and long-term survival were similar when compared to a propensity-matched group of patients undergoing planned open resection. On multivariable analysis, treatment at an academic facility (OR 0.63) and a robotic approach (OR 0.50) were both significantly associated with completed MIDP. CONCLUSION: Despite inferior post-operative outcomes compared to successful MIDP, unplanned conversion did not result in significantly reduced long term survival. MIDP can be attempted selectively but treatment at experienced centers via a robotic approach should be considered.


Asunto(s)
Adenocarcinoma , Laparoscopía , Neoplasias Pancreáticas , Procedimientos Quirúrgicos Robotizados , Humanos , Neoplasias Pancreáticas/patología , Pancreatectomía/efectos adversos , Pancreatectomía/métodos , Adenocarcinoma/cirugía , Resultado del Tratamiento , Procedimientos Quirúrgicos Robotizados/efectos adversos , Procedimientos Quirúrgicos Robotizados/métodos , Laparoscopía/métodos , Complicaciones Posoperatorias/epidemiología , Estudios Retrospectivos , Neoplasias Pancreáticas
8.
HPB (Oxford) ; 25(11): 1288-1299, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-37423850

RESUMEN

INTRODUCTION: Data regarding oncologic outcomes of segmental bile duct resection (SBDR) versus pancreatoduodenectomy (PD) for bile duct cancers (BDC) are conflicting. We compared SBDR and PD for BDC utilizing pooled data analysis. MATERIALS AND METHODS: A comprehensive PRISMA 2020 systematic review was performed. Studies comparing SBDR with PD for BDC were included. Pooled mean differences (MD), odds ratios (OR), and risk ratios (RR) with 95% confidence intervals (CI) were calculated. Subgroup analyses were performed. Study quality, bias, heterogeneity, and certainty were analyzed. RESULTS: Twelve studies from 2004 to 2021 were included, comprising 533 SBDR and 1,313 PD. SBDR was associated with positive proximal duct margins (OR 1.56; CI 1.11-2.18; P = .01), and distal duct margins (OR 43.25; CI 10.38-180.16; P < .01). SBDR yielded fewer lymph nodes (MD -6.93 nodes; CI -9.72-4.15; P < .01) and detected fewer nodal metastases (OR 0.72; CI 0.55-0.94; P = .01). SBDR portended less perioperative morbidity (OR 0.31; CI 0.21-0.46; P < .01), but not mortality (OR 0.52; CI 0.20-1.32; P = .17). SBDR was associated with locoregional recurrences (OR 1.88; CI 1.01-3.53; P = .02), and lymph node recurrences (OR 2.13; CI 1.42-3.2; P = .04). SBDR yielded decreased 5-year OS (OR 0.75; CI 0.65-0.85; P < .01). CONCLUSIONS: Despite decreased perioperative morbidity, SBDR appears to provide inferior oncologic control for BDC.

9.
Ann Surg Oncol ; 29(13): 8641-8648, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-36197560

RESUMEN

BACKGROUND: Lymph node (LN) metastases are uncommon among gastrointestinal stromal tumors (GISTs), and their presence has not been utilized in disease prognostication. This study was designed to examine factors associated with GIST nodal metastases and their impact on survival. PATIENTS AND METHODS: Patients undergoing surgical resection of GIST with nodal evaluation were selected from the National Cancer Database. Logistic regression was utilized to evaluate factors associated with LN metastases. Survival was assessed for patients with and without nodal involvement and Cox regression was used to evaluate the impact of LN metastases while adjusting for other prognostic factors. RESULTS: Out of 5018 patients, 301 (6.0%) had LN involvement. Nodal metastases occurred most frequently among tumors of the stomach (49.5%), followed by the small bowel (43.2%), colorectum (6.0%), and esophagus (1.3%). On multivariable analysis, male sex (OR 1.34), high mitotic rate (OR 2.10), tumor size (OR 1.02), and a primary tumor located in the small bowel (OR 1.36) were all significantly associated with nodal metastases. LN metastases were significantly associated with decreased overall survival (OS) for tumors arising in the small bowel (log-rank p < 0.01) and the colorectum (log-rank p < 0.01). Within a multivariable model adjusting for established prognostic factors, LN metastases remained independently associated with decreased survival (HR 1.60, p < 0.001). CONCLUSIONS: For GISTs, LN metastases occur more often in males and were associated with tumor size and mitotic activity. Nodal involvement is associated with decreased survival, independent of other well-established prognostic factors.


Asunto(s)
Tumores del Estroma Gastrointestinal , Humanos , Masculino , Metástasis Linfática/patología , Tumores del Estroma Gastrointestinal/cirugía , Tumores del Estroma Gastrointestinal/patología , Ganglios Linfáticos/cirugía , Ganglios Linfáticos/patología , Estómago , Pronóstico , Estudios Retrospectivos
10.
J Surg Oncol ; 125(8): 1231-1237, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35234280

RESUMEN

BACKGROUND AND OBJECTIVES: Visceral angiosarcoma is rare and aggressive, accounting for 2% of soft tissue sarcomas. Using a national data set, we examine determinants of outcomes for patients presenting with this rare disease. METHODS: The 2004-2015 National Cancer Database was queried for patients with visceral angiosarcoma. Trends in treatment and outcomes were examined. Factors affecting overall survival (OS) were assessed with log-rank and Cox regression. RESULTS: Eight hundred and ninety-three patients with visceral angiosarcoma were identified (median age 65 years, male [63%], Charlson comorbidity index <1 [86%]). Tumor size was <5 cm in 20.7%, and 34.2% were moderate/high grade. Median OS was 3.8 months (95% CI: 3.4-4.4). By multivariate analysis, increased tumor grade and size, and liver/biliary origin demonstrated worse OS while surgery, radiation, and systemic chemotherapy demonstrated improved OS (all p < 0.001). Survival was similar between patients achieving R0 resection and those with R1/2 resection receiving chemotherapy by Kaplan-Meier analysis. CONCLUSIONS: Visceral angiosarcomas are rare tumors with poor outcomes. Liver/biliary origin, higher tumor grade, and larger tumor size demonstrate worse outcomes. While R0 resection remains the mainstay of treatment, patients with R1/R2 resection have improved survival with addition of chemotherapy. Consideration should be made for multimodal therapy in these patients.


Asunto(s)
Hemangiosarcoma , Sarcoma , Neoplasias de los Tejidos Blandos , Anciano , Hemangiosarcoma/patología , Humanos , Estimación de Kaplan-Meier , Masculino , Pronóstico , Estudios Retrospectivos , Sarcoma/patología
11.
Ann Surg Oncol ; 28(3): 1417-1427, 2021 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-32856227

RESUMEN

BACKGROUND: Perioperative chemotherapy is a standard-of-care treatment for patients with gastric cancer. However, the impact of the postoperative chemotherapy (postCTX) component on overall survival (OS) is not well defined. METHODS: The National Cancer Database (NCDB) 2006-2014 was queried for patients who received preoperative chemotherapy (preCTX) and resection for gastric cancer. Analysis was performed to identify factors influencing receipt of postCTX. The impact of postCTX on OS was evaluated in propensity-matched groups. RESULTS: Among 3449 patients who received preCTX and resection for gastric cancer, 1091 (31.6%) received postCTX. Independent predictors of receiving postCTX were diagnosis after 2010 (odds ratio [OR] 1.985), distal tumor location (OR 1.348), and 15 or more lymph nodes examined (OR 1.214). Predictors of not receiving postCTX were older age (OR 0.985), comorbidity score higher than 1 (OR 0.592), and black race (OR 0.791). After propensity-matching (1091 per group), the median OS was 56.8 months for those who did receive postCTX versus 52.5 months for those who did not (p = 0.131). Subset analysis according to tumor grade, lymphovascular invasion, number of lymph nodes evaluated, T and N class, and AJCC stage identified an improvement in OS for the patients with N1 disease who received postCTX compared with those who did not (79.6 vs 41.3 months; p = 0.025). However, no other subgroup had a significant survival benefit. CONCLUSIONS: Additional postCTX was administered to a minority of patients who received preCTX and gastrectomy for gastric cancer, and its influence on OS appeared to be limited. Future trials should aim to define patients who will benefit from postCTX.


Asunto(s)
Adenocarcinoma , Neoplasias Gástricas , Adenocarcinoma/tratamiento farmacológico , Adenocarcinoma/patología , Adenocarcinoma/cirugía , Anciano , Anciano de 80 o más Años , Quimioterapia Adyuvante , Femenino , Gastrectomía , Humanos , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Cuidados Posoperatorios , Cuidados Preoperatorios , Estudios Retrospectivos , Neoplasias Gástricas/tratamiento farmacológico , Neoplasias Gástricas/patología , Neoplasias Gástricas/cirugía , Análisis de Supervivencia
12.
Ann Surg Oncol ; 28(1): 492-501, 2021 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-32656720

RESUMEN

BACKGROUND: Although advocated by some, minimally invasive adrenalectomy (MIA) for adrenocortical carcinoma (ACC) is controversial. Moreover, the oncologic implications for patients requiring conversion to an open procedure during attempted MIA for ACC are not extensively reported. PATIENTS AND METHODS: The National Cancer Database was queried for patients undergoing resection for ACC. Overall survival (OS) for patients undergoing successful MIA was compared with those requiring conversion, and additionally evaluated with a multivariable Cox regression analysis including other factors associated with OS. After propensity matching, those experiencing conversion were further compared with patients who underwent planned open resection. RESULTS: Among 196 patients undergoing attempted MIA for ACC, 38 (19.4%) required conversion. Independent of 90-day postoperative mortality, conversion was associated with significantly reduced OS compared with successful MIA (median 27.9 months versus not reached, p = 0.002). Even for tumors confined to the adrenal, conversion was associated with worse median OS compared with successful MIA (median 34.2 months versus not reached, p = 0.003). After propensity matching for clinicopathologic covariates to establish well-balanced cohorts (N = 38 per group), patients requiring conversion during MIA had significantly worse OS than those having planned open resection (27.9 months versus 50.5 months, p = 0.020). On multivariable analysis for predictors of OS, conversion during MIA (HR 2.32, p = 0.003) was independently associated with mortality. CONCLUSIONS: ACC is a rare tumor for which adequate oncologic resection is the only chance for cure. Given the relatively high rate of conversion and its associated inferior survival, open resection should be considered standard of care for known or suspected ACC.


Asunto(s)
Neoplasias de la Corteza Suprarrenal , Adrenalectomía , Carcinoma Corticosuprarrenal , Neoplasias de la Corteza Suprarrenal/cirugía , Carcinoma Corticosuprarrenal/cirugía , Humanos , Laparoscopía , Procedimientos Quirúrgicos Mínimamente Invasivos , Periodo Posoperatorio
13.
J Natl Compr Canc Netw ; 19(7): 839-868, 2021 07 28.
Artículo en Inglés | MEDLINE | ID: mdl-34340212

RESUMEN

The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Neuroendocrine and Adrenal Gland Tumors focus on the diagnosis, treatment, and management of patients with neuroendocrine tumors (NETs), adrenal tumors, pheochromocytomas, paragangliomas, and multiple endocrine neoplasia. NETs are generally subclassified by site of origin, stage, and histologic characteristics. Appropriate diagnosis and treatment of NETs often involves collaboration between specialists in multiple disciplines, using specific biochemical, radiologic, and surgical methods. Specialists include pathologists, endocrinologists, radiologists (including nuclear medicine specialists), and medical, radiation, and surgical oncologists. These guidelines discuss the diagnosis and management of both sporadic and hereditary neuroendocrine and adrenal tumors and are intended to assist with clinical decision-making. This article is focused on the 2021 NCCN Guidelines principles of genetic risk assessment and counseling and recommendations for well-differentiated grade 3 NETs, poorly differentiated neuroendocrine carcinomas, adrenal tumors, pheochromocytomas, and paragangliomas.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales , Tumores Neuroendocrinos , Neoplasias de las Glándulas Suprarrenales/diagnóstico , Neoplasias de las Glándulas Suprarrenales/genética , Neoplasias de las Glándulas Suprarrenales/terapia , Humanos , Oncología Médica , Tumores Neuroendocrinos/diagnóstico , Tumores Neuroendocrinos/genética , Tumores Neuroendocrinos/terapia
14.
Ann Surg Oncol ; 27(5): 1329-1337, 2020 May.
Artículo en Inglés | MEDLINE | ID: mdl-32112212

RESUMEN

This first part of a two-part review of pheochromocytoma and paragangliomas (PPGLs) addresses clinical presentation, diagnosis, management, treatment, and outcomes. In this first part, the epidemiology, prevalence, genetic etiology, clinical presentation, and biochemical and radiologic workup are discussed. In particular, recent advances in the genetics underlying PPGLs and the recommendation for genetic testing of all patients with PPGL are emphasized. Finally, the newer imaging methods for evaluating of PPGLs are discussed and highlighted.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales/diagnóstico , Paraganglioma Extraadrenal/diagnóstico , Feocromocitoma/diagnóstico , Abdomen , Neoplasias de las Glándulas Suprarrenales/epidemiología , Neoplasias de las Glándulas Suprarrenales/genética , Neoplasias de las Glándulas Suprarrenales/fisiopatología , Arritmias Cardíacas/fisiopatología , Catecolaminas/sangre , Catecolaminas/orina , Células Cromafines/metabolismo , Ganglios Parasimpáticos , Ganglios Simpáticos , Pruebas Genéticas , Cefalea/fisiopatología , Humanos , Hipertensión/fisiopatología , Neoplasia Endocrina Múltiple Tipo 2a/diagnóstico , Neoplasia Endocrina Múltiple Tipo 2a/genética , Neoplasia Endocrina Múltiple Tipo 2b/diagnóstico , Neoplasia Endocrina Múltiple Tipo 2b/genética , Síndromes Neoplásicos Hereditarios/diagnóstico , Síndromes Neoplásicos Hereditarios/genética , Neurofibromatosis 1/diagnóstico , Neurofibromatosis 1/genética , Paraganglioma/diagnóstico , Paraganglioma/epidemiología , Paraganglioma/genética , Paraganglioma/fisiopatología , Paraganglioma Extraadrenal/epidemiología , Paraganglioma Extraadrenal/genética , Paraganglioma Extraadrenal/metabolismo , Pelvis , Feocromocitoma/epidemiología , Feocromocitoma/genética , Feocromocitoma/fisiopatología , Sudoración/fisiología , Enfermedad de von Hippel-Lindau/diagnóstico , Enfermedad de von Hippel-Lindau/genética
15.
Ann Surg Oncol ; 27(5): 1338-1347, 2020 May.
Artículo en Inglés | MEDLINE | ID: mdl-32112213

RESUMEN

This is the second part of a two-part review on pheochromocytoma and paragangliomas (PPGLs). In this part, perioperative management, including preoperative preparation, intraoperative, and postoperative interventions are reviewed. Current data on outcomes following resection are presented, including outcomes after cortical-sparing adrenalectomy for bilateral adrenal disease. In addition, pathological features of malignancy, surveillance considerations, and the management of advanced disease are also discussed.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales/cirugía , Hipertensión/tratamiento farmacológico , Hipoglucemia/terapia , Hipotensión/terapia , Paraganglioma Extraadrenal/cirugía , Atención Perioperativa/métodos , Feocromocitoma/cirugía , Complicaciones Posoperatorias/terapia , Neoplasias de las Glándulas Suprarrenales/complicaciones , Adrenalectomía/métodos , Antagonistas Adrenérgicos alfa/uso terapéutico , Antagonistas Adrenérgicos beta/uso terapéutico , Bloqueadores de los Canales de Calcio/uso terapéutico , Inhibidores Enzimáticos/uso terapéutico , Fluidoterapia , Humanos , Hiperinsulinismo/diagnóstico , Hiperinsulinismo/epidemiología , Hiperinsulinismo/terapia , Hipertensión/etiología , Hipoglucemia/diagnóstico , Hipoglucemia/epidemiología , Hipotensión/diagnóstico , Hipotensión/epidemiología , Laparoscopía , Neoplasia Endocrina Múltiple Tipo 2a , Neoplasia Endocrina Múltiple Tipo 2b , Recurrencia Local de Neoplasia/epidemiología , Paraganglioma/complicaciones , Paraganglioma/cirugía , Paraganglioma Extraadrenal/complicaciones , Feocromocitoma/complicaciones , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/epidemiología , Procedimientos Quirúrgicos Robotizados , Resultado del Tratamiento , Tirosina 3-Monooxigenasa/antagonistas & inhibidores , Vasoconstrictores/uso terapéutico , Enfermedad de von Hippel-Lindau
16.
Ann Surg Oncol ; 27(1): 171-178, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-30963398

RESUMEN

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare intra-abdominal soft tissue sarcoma affecting adolescents and young adults. Cytoreduction, hyperthermic intraperitoneal chemotherapy (CRS/HIPEC), and adjuvant radiotherapy may improve local control. We review our experience with patients who undergo CRS/HIPEC and adjuvant radiotherapy for DSRCT. METHODS: A retrospective review was performed for patients with DSRCT from 2013 to 2017 who underwent CRS/HIPEC. Clinicopathologic, operative, and outcome data were reviewed. RESULTS: Ten CRS/HIPEC procedures were performed for nine patients (7 males, 6 Caucasian, median age 19 years (range 10-24)). Four patients presented with extra-abdominal disease; five had liver involvement. The median peritoneal cancer index was 16 (range 5-20). All received neoadjuvant chemotherapy. CCR 0/1 resection was possible in nine patients. Major complications occurred in four with no operative mortalities. All received adjuvant chemotherapy, seven received radiation therapy, and three received stem-cell transplant. All but one patient recurred after treatment. The median recurrence-free and overall survival (OS) were 12 and 45 months (95% confidence interval 35.1-54.9) respectively, with a 3-year OS of 55%. Long-term parenteral nutrition was required in eight for a median of 261 days (range 37-997). Clinically significant long-term complications requiring further surgery included gastroparesis (N = 1), small bowel obstruction (N = 3) and hemorrhagic cystitis (N = 2). CONCLUSIONS: Multimodal therapy for DSRCT consisting of multiagent neoadjuvant chemotherapy, CRS/HIPEC, adjuvant chemotherapy, and radiation therapy is associated with potential cumulative toxicity. Recurrence after resection is common. Prolonged parenteral nutrition may be necessary, and late gastrointestinal and genitourinary complications may require additional treatment.


Asunto(s)
Procedimientos Quirúrgicos de Citorreducción/efectos adversos , Tumor Desmoplásico de Células Pequeñas Redondas/terapia , Hipertermia Inducida/efectos adversos , Neoplasias Peritoneales/terapia , Adolescente , Antineoplásicos/uso terapéutico , Quimioterapia Adyuvante , Niño , Terapia Combinada/efectos adversos , Tumor Desmoplásico de Células Pequeñas Redondas/mortalidad , Tumor Desmoplásico de Células Pequeñas Redondas/patología , Femenino , Estudios de Seguimiento , Humanos , Masculino , Recurrencia Local de Neoplasia , Estadificación de Neoplasias , Neoplasias Peritoneales/mortalidad , Neoplasias Peritoneales/patología , Complicaciones Posoperatorias , Estudios Retrospectivos , Tasa de Supervivencia , Adulto Joven
17.
Ann Surg Oncol ; 27(6): 1900-1905, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-31925595

RESUMEN

BACKGROUND: Fibrolamellar hepatocellular carcinoma (FLC) is a rare variant of hepatocellular carcinoma (HCC), with most clinical data stemming from single-institution series. The variability in the literature lends support for analysis using a large national dataset. In doing so, we sought to (1) define the characteristics and outcomes of patients with FLC; (2) determine factors associated with survival in patients undergoing resection; and (3) compare the overall survival (OS) of patients with FLC with a matched group of patients with HCC. METHODS: The National Cancer Database was queried for patients with FLC, and their clinicopathologic features were recorded. Univariate and multivariate analyses were performed to delineate factors associated with survival. RESULTS: Between 2004 and 2015, 496 patients were diagnosed with FLC, 229 of whom underwent a curative resection. The median OS for patients with FLC undergoing curative resection was 78.5 months. Factors associated with abbreviated OS in this surgical cohort include multiple tumors [hazard ratio (HR) 3.15, p = 0.025], positive regional lymph nodes (HR 2.83, p = 0.023), and elevated serum α-fetoprotein (AFP; HR 2.81, p = 0.034). When the OS of patients with FLC was compared with a matched group of patients with HCC, no difference was detected (p = 0.748); however, patients with FLC and elevated AFP had abbreviated OS compared with patients with HCC and elevated AFP (43 vs. 82 months, p ≤ 0.001). CONCLUSIONS: Elevations in serum AFP occur more frequently than previously documented for patients with FLC and are associated with abbreviated OS. AFP levels may help guide the decision for operative intervention in patients with FLC.


Asunto(s)
Biomarcadores de Tumor/sangre , Carcinoma Hepatocelular/mortalidad , Hepatectomía/mortalidad , alfa-Fetoproteínas/análisis , Adulto , Anciano , Anciano de 80 o más Años , Carcinoma Hepatocelular/sangre , Carcinoma Hepatocelular/patología , Carcinoma Hepatocelular/cirugía , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia
18.
J Surg Oncol ; 122(6): 1152-1162, 2020 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-32705677

RESUMEN

BACKGROUND: Adrenocortical carcinoma (ACC) is a rare tumor and the role of lymph node dissection remains ill-defined. This study evaluates the effect of nodal examination on prognosis and survival in patients undergoing curative-intent resection of ACC. METHODS: The National Cancer Database (2004-2015) was queried for patients undergoing margin-negative resection for ACC. Patients with distant metastases, neoadjuvant therapy, multivisceral resection and T4 tumors were excluded. RESULTS: Among 897 patients, 147 (16.4%) had lymph nodes examined. Factors associated with lymph node examination included increasing tumor size (P < .001), extra-adrenal extension (P < .001), open operation (P < .001), and resection at an academic facility (P = .003). Lymph node metastasis was significantly associated with extra-adrenal tumor extension (P = .04). Lymph node harvest, regardless of the number of nodes examined, was not associated with a survival benefit. Median overall survival was incrementally worse with increasing number of positive lymph nodes (88.2 months for N0, 34.9 months for 1-3 positive nodes, and 15.6 months for ≥4 positive nodes, P < .001). CONCLUSIONS: Lymph node harvest and lymph node metastasis were associated with more advanced tumors. Although nodal harvest did not offer a survival advantage, stratifying the nodal staging classification may provide important prognostic information.


Asunto(s)
Neoplasias de la Corteza Suprarrenal/patología , Carcinoma Corticosuprarrenal/patología , Escisión del Ganglio Linfático/mortalidad , Ganglios Linfáticos/patología , Neoplasias de la Corteza Suprarrenal/cirugía , Carcinoma Corticosuprarrenal/cirugía , Adulto , Femenino , Estudios de Seguimiento , Humanos , Ganglios Linfáticos/cirugía , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia
19.
J Surg Oncol ; 121(3): 494-502, 2020 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-31902137

RESUMEN

BACKGROUND: Socioeconomic disparities in gastric cancer have been associated with differences in care and inferior outcomes. We evaluated the presentation, treatment, and survival for patients with gastric cancer (GC) in a metropolitan setting with a large African American population. METHODS: Retrospective cohort analysis of patients with GC (2003-2018) across a multi-hospital system was performed. Associations between socioeconomic and clinicopathologic data with the presentation, treatment, and survival were examined. RESULTS: Of 359 patients, 255 (71%) were African American and 104 (29%) Caucasian. African Americans were more likely to present at a younger age (64.0 vs 72.5, P < .001), have state-sponsored or no insurance (19.7% vs 6.9%, P = .02), reside within the lowest 2 quintiles for median income (67.4% vs 32.7%, P < .001), and have higher rates of Helicobacter pylori (14.9% vs 4.8%, P = .02). Receipt of multi-modality therapy was not impacted by race or insurance status. On multivariable analysis, only AJCC T class (HR 1.68) and node positivity (HR 2.43) remained significant predictors of disease-specific survival. CONCLUSION: Despite socioeconomic disparities, African Americans, and Caucasians with GC had similar treatment and outcomes. African Americans presented at a younger age with higher rates of H. pylori positivity, warranting further investigation into differences in risk factors and tumor biology.


Asunto(s)
Infecciones por Helicobacter/complicaciones , Grupos Raciales/estadística & datos numéricos , Clase Social , Neoplasias Gástricas/mortalidad , Anciano , Terapia Combinada , Manejo de la Enfermedad , Femenino , Estudios de Seguimiento , Infecciones por Helicobacter/virología , Helicobacter pylori/aislamiento & purificación , Humanos , Renta , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Factores de Riesgo , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/etiología , Neoplasias Gástricas/terapia , Tasa de Supervivencia , Estados Unidos
20.
J Surg Oncol ; 121(8): 1320-1328, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32166754

RESUMEN

BACKGROUND AND OBJECTIVES: Peritoneal metastases (PM) from primary colorectal cancer (pCRC) are associated with poor outcomes; however, molecular differences are not well defined. METHODS: We compared unpaired tumor profiles of patients with pCRC and PM from Caris Life Sciences. Testing included next-generation sequencing of 592 genes, microsatellite instability (MSI) and tumor mutational burden (TMB). Mutations were test-defined as pathogenic (PATH). RESULTS: Six hundred seventeen pCRC and 348 PM patients had similar gender (55% male) and age (median 59). PATHs were similar between PM and pCRC in KRAS, BRAF, SMAD2, SMAD4, and PTEN. pCRC PATHs were increased in APC (76% vs 48%, P < .01), ARID1A (29% vs 12%, P < .05), TP53 (72% vs 53%, P < .01), PIK3CA (22% vs 15%, P < .05), and FBXW7 (13% vs 7%, P < .01) compared with PM. Mucinous PM had more PATHs in GNAS (19% vs 8%, P = .032) while nonmucinous PM had more PATHs in BRAF (13% vs 8%, P = .027). Right-sided PM had decreased PATHs in APC (39% vs 68%, P < .0001), ARID1A (7% vs 38%, P < .004), and TP53 (48% vs 65%, P = .033) while there were no difference for left-sided PM. Nine percent of pCRC and 6% of PM were MSI-high (P = NS). There was no difference in TMB-high, TMB-intermediate, or TMB-low between PM and pCRC. CONCLUSIONS: PM have similar rates of KRAS mutation with increased PATHs in GNAS (mucinous) and BRAF (nonmucinous) compared to pCRC. No differences in MSI or TMB were identified between PM and pCRC tumors. These findings inform future study into the molecular profile of PM.


Asunto(s)
Neoplasias Colorrectales/genética , Neoplasias Colorrectales/patología , Neoplasias Peritoneales/genética , Neoplasias Peritoneales/secundario , Adenocarcinoma/genética , Adenocarcinoma/metabolismo , Adenocarcinoma/patología , Adenocarcinoma/secundario , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias Colorrectales/metabolismo , Femenino , Secuenciación de Nucleótidos de Alto Rendimiento , Humanos , Inmunohistoquímica , Masculino , Inestabilidad de Microsatélites , Persona de Mediana Edad , Mutación , Neoplasias Peritoneales/metabolismo , Adulto Joven
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