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1.
Front Endocrinol (Lausanne) ; 13: 907415, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35873006

RESUMO

Background: Small non-functional neuroendocrine tumors (NF-PNETs) are a heterogeneous subset of tumors with controversy regarding their optimal management. We aimed to analyze the prognostic factors of patients with small NF-PNETs and create a risk score for lymph node metastasis (LNM). Methods: Data of 751 patients with NF-PNETs ≤ 2 cm were obtained from the Surveillance, Epidemiology, and End Results (SEER) database. Multivariate survival analysis was performed to analyze the prognostic factors. Logistic regression was used to identify risk factors for LNM. Results: Of the 751 patients, 99 (13.2%) were confirmed to have LNM. In multivariate survival analysis, LNM (hazard ratio [HR], 2.12; 95% CI, 1.04-4.32, p = 0.040) was independently associated with disease-specific survival. Logistic regression identified that tumor location in the head of the pancreas (odds ratio [OR], 4.33; 95% CI, 2.75-6.81; p < 0.001), size ≥ 1.5-2 cm (OR, 1.84; 95% CI, 1.17-2.87; p = 0.009), and grade III-IV (OR, 7.90; 95% CI, 1.79-34.90; p = 0.006) were independent risk factors of LNM. According to the OR value, the risk of LNM was scored as follows: a score of 1 for tumors located in the body/tail of the pancreas and 4 for those located in the head; a score of 1 for tumors <1 cm and 2 for those ≥1.5-2 cm; and a score of 1 for tumors with grade I-II and 8 for those with grade III-IV. Finally, the median score for this cohort was 4, with an interquartile range of 3-6. Therefore, patients were classified as three groups based on the risk score system: a total score of 1-3 for low risk, 4-6 for intermediate risk (OR, 2.98; 95% CI, 1.59-5.60; p = 0.001), and 7-14 for high risk (OR, 8.94; 95% CI, 4.50-17.7; p < 0.001), with an incidence of LNM 5.0%, 13.5%, and 31.8%, respectively (p < 0.001). Conclusion: Surgical resection with regional lymphadenectomy is recommended for small NF-PNETs with malignant potential of LNM. A risk score for LNM based on tumor grade, location, and size may preoperatively predict LNM of small NF-PNETs and guide clinical practice.


Assuntos
Tumores Neuroectodérmicos Primitivos , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Humanos , Metástase Linfática , Tumores Neuroendócrinos/patologia , Neoplasias Pancreáticas/epidemiologia , Neoplasias Pancreáticas/patologia , Prognóstico
2.
BMC Urol ; 22(1): 103, 2022 Jul 11.
Artigo em Inglês | MEDLINE | ID: mdl-35821028

RESUMO

BACKGROUND: Ewing sarcoma/primitive neuroectodermal tumor (PNET) of the renal is extremely rare. The common cause of ectopic ACTH syndrome is pulmonary neuroendocrine tumors, such as small cell carcinomas and carcinoid tumors. Here, we present an unusual case of ectopic ACTH syndrome and hypothyroidism caused by Ewing sarcoma/PNET of the right kidney. CASE PRESENTATION: A 19-year-old girl presented with a history of right lumbar pain and discomfort for 2 months, aggravated for 2 days. Abdominal contrast-enhanced computed tomography and computed tomography angiography showed an upper pole occupancy of the right kidney occupancy with subepithelial hemorrhage. Preoperative hormone levels including plasma total cortisol (PTC), adrenocorticotrophic hormone (ACTH) and thyroid hormone measurements were abnormal, indicating that the patient had Cushing syndrome and hypothyroidism. The patient underwent right radical nephrectomy. Histopathological analysis revealed a renal small round blue cell tumor (consistent with a primitive neuroectodermal tumor), with positive immunohistochemistry for CD99 and Ki67 (about 10%) and molecular pathology for EWSR1 gene fusions. PTC, ACTH and thyroid hormone returned to normal after surgery. CONCLUSIONS: We report a rare ectopic ACTH syndrome and hypothyroidism due to renal Ewing sarcoma/PNET. The clinical manifestation of renal Ewing sarcoma/PNET is non-specific and the diagnosis relies on pathological morphology, immunohistochemistry and fusion gene detection. At present, surgery combined with radiotherapy and chemotherapy is used in the treatment, but the prognosis is still not optimistic.


Assuntos
Síndrome de ACTH Ectópico , Hipotireoidismo , Neoplasias Renais , Tumores Neuroectodérmicos Primitivos Periféricos , Tumores Neuroectodérmicos Primitivos , Sarcoma de Ewing , Hormônio Adrenocorticotrópico/uso terapêutico , Adulto , Feminino , Humanos , Neoplasias Renais/cirurgia , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Sarcoma de Ewing/patologia , Adulto Jovem
3.
J Am Coll Surg ; 235(2): 315-330, 2022 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-35839409

RESUMO

BACKGROUND: Resection of pancreatic neuroendocrine tumors (PNETs) may be associated with adverse perioperative outcomes compared with pancreatic adenocarcinoma given the high-risk nature of soft glands with small pancreatic ducts. The effect of minimally invasive surgery (MIS) pancreatectomy on outcomes of PNETs remains to be examined, which is the aim of this study. STUDY DESIGN: Between 2009 and 2019, 1,023 patients underwent pancreatectomy for PNETs at 4 institutions. Clinicopathologic data and perioperative outcomes of patients who underwent MIS (n = 447) and open resections (n = 576) were compared. RESULTS: Of the 1,023 patients, 51% were male, the mean age was 58, the median tumor size was 2.1 cm, and 73% were grade 1 PNETs. There were 318 (31%) pancreatoduodenectomies (PDs), 541 (53%) distal pancreatectomies (DPs), 80 (7.8%) enucleation (ENs), 72 (7%) central pancreatectomies (CPs), and 12 (1.2%) total pancreatectomies. Almost half of the patients (N = 447, 44%) had MIS operations, of which 230 (51%) were robotic and 217 (49%) were laparoscopic. Compared with open operations, MIS PDs had significantly lower operative blood loss (150 vs 400 mL, p < 0.001) and rate of clinically relevant postoperative pancreatic fistulas (CR-POPFs; 13% vs 27%, p = 0.030), and MIS DPs had a shorter length of stay (5 vs 6 days, p < 0.001). Although MIS DPs and ENs had CR-POPFs comparable with open operations, MIS CPs had a higher CR-POPF rate (45% vs 15%, p = 0.013). After adjusting for pathological differences, MIS pancreatectomy was associated with recurrence-free survival and overall survival comparable with open pancreatectomy. CONCLUSIONS: MIS pancreatectomy for PNETs is associated with improved outcomes or outcomes comparable with open resection.


Assuntos
Adenocarcinoma , Laparoscopia , Tumores Neuroectodérmicos Primitivos , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Procedimentos Cirúrgicos Robóticos , Adenocarcinoma/cirurgia , Feminino , Humanos , Laparoscopia/efeitos adversos , Masculino , Pessoa de Meia-Idade , Procedimentos Cirúrgicos Minimamente Invasivos , Tumores Neuroectodérmicos Primitivos/etiologia , Tumores Neuroectodérmicos Primitivos/cirurgia , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/cirurgia , Pancreatectomia/efeitos adversos , Neoplasias Pancreáticas/patologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Procedimentos Cirúrgicos Robóticos/efeitos adversos , Resultado do Tratamento
4.
Front Endocrinol (Lausanne) ; 13: 925632, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35837305

RESUMO

Introduction: The European Neuroendocrine Tumor Society, ENETS, reports variables of prognostic significance in pancreatic neuroendocrine tumors (PNET). However, studies have short follow-ups, and the optimal treatment remains controversial. We aimed to determine overall survival (OS), progression-free survival (PFS) after conservative treatment, and recurrence-free survival (RFS) after surgery and further to find predictors of aggressive PNET behavior to support treatment decisions. Methods: 174 patients with PNET treated at Aarhus University Hospital from 2011 to 2021 were included in a retrospective cohort study. Patients were divided into surgically resected (SUR, n=91) and medically or conservatively treated (MED, n=83). Variables were tested in univariate and multivariate survival analysis. Median follow-up time was 3.4 years in the MED group and 4.5 years in the SUR group. Results: The 5-year OS was 95% and 65% for the SUR and MED groups, respectively. The 5-year RFS in the SUR group was 80% whereas the 5-year PFS in the MED group was 41%. Larger tumor size, Ki67 index, tumor grade, and stage were predictive of shorter OS, RFS, and PFS. Further, chromogranin A was a predictor of OS. Larger tumor size was associated with higher stage and grade. Only 1 of 28 patients with stage 1 disease and size ≤2 cm developed progression on a watch-and-wait strategy during a median follow-up of 36 months. Conclusion: This study supported the ENETS staging and grading system to be useful to predict OS, PFS, and RFS in PNET. Further, our data support that small, localized, low-grade PNETS can be followed with active surveillance.


Assuntos
Tumores Neuroectodérmicos Primitivos , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Intervalo Livre de Doença , Humanos , Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/cirurgia , Estudos Retrospectivos
5.
Medicine (Baltimore) ; 101(29): e29564, 2022 Jul 22.
Artigo em Inglês | MEDLINE | ID: mdl-35866758

RESUMO

To investigate the characteristics and factors that impact the prognosis of Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) patients. We retrospectively analyzed ES/pPNET patients diagnosed at our hospital from January 2011 to December 2020. We used 1-way analysis of variance to investigate whether the age differences between different subgroups were statistically significant and used the Kaplan-Meier method and Cox regression model for the survival analysis. Of the 67 included patients, 13 had central nervous system PNET, and 54 had ES/pPNET. The median survival time of the 54 ES/pPNET patients was 18 months; the 1-year, 3-year and 5-year progression-free survival rates were 37.0% and 9.3% and 1.9%, respectively; and the 1-year, 3-year and 5-year overall survival (OS) rates were 66.7%, 27.8%, and 7.4%, respectively. The 1-way analysis of variance results showed no statistically significant age difference between the different subgroups (P = .127 between the central nervous system PNET and ES/pPNET groups, P = .764 between different subgroups within the ES/pPNET group). The univariate survival analysis showed that metastasis at diagnosis and the treatment method were independent factors affecting the OS rate of ES/pPNET patient (P = .003 and 0.000, respectively). The multivariate survival analysis also showed that the treatment method and metastasis at diagnosis were related factors affecting OS (P = .025 and 0.001, respectively). The prognosis of patients with primitive neuroectodermal tumors is poor. The treatment method and metastasis at the time of diagnosis influences ES/pPNET patient survival time, but there is no significant tumor site-dependent correlation with patient age or sex.


Assuntos
Neoplasias Embrionárias de Células Germinativas , Tumores Neuroectodérmicos Primitivos Periféricos , Tumores Neuroectodérmicos Primitivos , Sarcoma de Ewing , Humanos , Tumores Neuroectodérmicos Primitivos/terapia , Prognóstico , Estudos Retrospectivos , Sarcoma de Ewing/patologia
6.
Clin Nutr ESPEN ; 50: 162-169, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35871919

RESUMO

BACKGROUND AND AIMS: Childhood cancer survivors are at risk of unwanted late effects. The primary aim of this study was to assess bone mineral density Z-scores (BMDz) in long-term survivors of childhood medulloblastoma (MB) or central nervous system supratentorial primitive neuroectodermal tumor (CNS-PNET). Secondary aims were to describe nutrient intake, vitamin D status, physical activity and explore potential risk factors for decreased BMDz. METHODS: All MB and CNS-PNET survivors treated at Oslo University Hospital from 1974 to 2013 were invited to participate in a cross-sectional study. Dual-energy x-ray absorptiometry (Lunar Prodigy) assessed BMDz lumbar spine, BMDz total body, and lean body mass. Decreased BMDz was defined as a combination of low BMDz -1 to -1.99 and very low BMDz ≤-2. Lean body mass index (LMI) was calculated by dividing lean body mass by the squared height. Nutrient intake was assessed by a 3-day food record. Serum 25(OH)D was analyzed. Physical activity was reported by a questionnaire. Descriptive statistics and multivariable Cox regression analyses were applied. RESULTS: Fifty survivors with a median age of 25.5 years (5.5-51.9) and a median follow-up time of 19.5 years (3.2-40.5) were included. Mean BMDz lumbar spine was -0.8 (SD 1.1, 95% CI: -1.1 to -0.4), and BMDz total body was -0.6 (SD 1.1, 95% CI: -0.9 to -0.3). Decreased BMDz was detected in 48% of the lumbar spine and 34% of the total body measurements. In all, 62% had low calcium, and 69% had low vitamin D intake. 26% of participants had serum 25(OH)D < 50 nmol/L, and 62% reported an inactive lifestyle. Male sex, higher age at diagnosis, and lower LMI were potential risk factors for decreased BMDz. CONCLUSIONS: Long-term survivors of childhood MB and CNS-PNET had decreased BMDz, and risk factors were male sex, higher age at diagnosis, and lower LMI. Inadequate calcium and vitamin D intake, an inactive lifestyle, and a high prevalence of 25(OH)D ≤ 50 nmol/L were detected.


Assuntos
Neoplasias Encefálicas , Tumores Neuroectodérmicos Primitivos , Adulto , Densidade Óssea , Cálcio , Estudos Transversais , Feminino , Humanos , Masculino , Estado Nutricional , Sobreviventes , Vitamina D
7.
Artigo em Russo | MEDLINE | ID: mdl-35758080

RESUMO

Embryonal tumor with multilayered rosettes (ETMR) is a rare and highly malignant brain tumor that develops in children younger 4 years old. This neoplasm is characterized by extremely aggressive course, low sensitivity to chemotherapy and radiotherapy. Thanks to the progress of pathologists, diagnosis of ETMR is now available using immunohistochemical examination with LIN28A and SALL4 antibodies. Moreover, detection of microRNA amplification in the 19q13.42 locus by fluorescent hybridization in situ allows an unmistakable diagnosis.The authors describe clinical course and treatment outcome in a 2-year-old patient with a giant tumor of the right parietotemporal region. Postoperative histological examination verified ETMR. Despite adjuvant treatment, we observed fast progression of disease and unfavorable outcome after 5 months. Case report is supplemented by literature review. CONCLUSION: ETMR is very rare and poorly understood neoplasm. The authors present a giant hemispheric ETMR in a 2-year-old boy with an extremely aggressive course of disease. Despite the advances in diagnosis and treatment of CNS tumors in children, there are currently more questions than answers regarding ETMR. Pooled analysis of all available data with large-scale studies is needed. It is necessary to emphasize an exceptional role of each clinical case for global study of this tumor. Timely adjuvant/neoadjuvant therapy in highly specialized centers is also essential.


Assuntos
Neoplasias Encefálicas , Neoplasias do Sistema Nervoso Central , MicroRNAs , Neoplasias Embrionárias de Células Germinativas , Tumores Neuroectodérmicos Primitivos , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/genética , Neoplasias Encefálicas/cirurgia , Criança , Pré-Escolar , Humanos , Masculino , MicroRNAs/genética , Tumores Neuroectodérmicos Primitivos/genética , Tumores Neuroectodérmicos Primitivos/patologia , Tumores Neuroectodérmicos Primitivos/terapia
8.
BMC Urol ; 22(1): 89, 2022 Jun 22.
Artigo em Inglês | MEDLINE | ID: mdl-35733141

RESUMO

BACKGROUND: Pediatric non-Wilms renal tumors (NWRTs), which comprise a small proportion of renal tumors, are a heterogeneous group of neoplasms with variable malignant potential, mortality, and response to treatment. We performed this study to determine the clinical characteristics, management and prognosis of children with Pediatric NWRTs. METHODS: Medical records of all patients (n = 139) treated for NWRTs over a 12-year period (2008.01-2019.10) at a single center were reviewed retrospectively. RESULTS: The histopathological groups of NWRTs included malignant rhabdoid tumor of the kidney (MRTK) (n: 30, 21.6%), renal cell cancer (RCC) (n: 26,18.7%), clear cell sarcoma of the kidney (CCSK) (n: 24,17.3%), congenital mesoblastic nephroma (CMN) (n: 21,15.1%), cystic nephroma (CN) (n: 16,11.5%), metanephric tumors (n: 12, 8.6%), renal angiomyoliporma (RAML) (n: 3, 2.2%), renal primitive neuroectodermal tumor (n: 2, 1.4%), renal hemangioma (n: 2, 1.4%), inflammatory myofibroblastic tumor (n: 2, 1.4%), ossifying renal tumor of infancy (ORTI) (n: 1, 0.7%). The distribution of all malignant NWRTs, including MRTK, CCSK, RCC and PNET, according to stage was as follows: stages I (n = 26), II (n = 16), III (n = 29), and IV (n = 11). The summary table shows the treatment offered to children with NWRTs. A total of 123 children were followed up for an average of 42 months. Sixteen children were lost to follow-up. Tumor-free survival was observed in 94 children. One patient who suffered from RCC is currently receiving targeted therapy and survives with the tumor. Twenty-eight children (22.8%) died. CONCLUSIONS: Pediatric NWRTs comprise 19.1% of all renal tumors in our single center. Most NWRTs can readily be distinguished using a range of immunohistochemical markers. Molecular genetic profiling has allowed much progress in the understanding of this group of tumors, making diagnosis and classification less difficult. The mainstay treatment of malignant NWRTs, including MRTK, CCSK, RCC and PNET, is comprehensive treatment. The mainstay treatment of benign NWRTs, including RAML, CN, ORTI, CMN, metanephric tumors, and renal hemangioma, is surgical resection alone and when the tumor diameter is smaller than 7 cm and the tumor locates in one pole, NSS can be performed.


Assuntos
Carcinoma de Células Renais , Hemangioma , Neoplasias Renais , Nefroma Mesoblástico , Tumores Neuroectodérmicos Primitivos , Tumor Rabdoide , Sarcoma , Tumor de Wilms , Carcinoma de Células Renais/patologia , Criança , Humanos , Lactente , Rim/patologia , Neoplasias Renais/patologia , Nefroma Mesoblástico/congênito , Nefroma Mesoblástico/patologia , Nefroma Mesoblástico/cirurgia , Estudos Retrospectivos , Tumor de Wilms/diagnóstico
9.
Orphanet J Rare Dis ; 17(1): 219, 2022 06 13.
Artigo em Inglês | MEDLINE | ID: mdl-35698198

RESUMO

OBJECTIVE: To investigate the clinical characteristics of patients with multiple endocrine neoplasia type 1 (MEN1)-related insulinoma and their relationship with specific biochemical changes and to summarize the features of treatment options for the Chinese population with this disease and the impact on long-term prognosis. METHODS: "MEN1" and "insulinoma" were used when searching the Peking Union Medical College Hospital (PUMCH) medical record retrieval system to obtain clinical information about patients. We identified patients diagnosed with MEN1-associated insulinoma based on endocrinological, radiological, and pathological examinations, and subsequently analyzed their clinical data. RESULTS: A total of 55 patients with MEN1-associated insulinoma were included, including 29 (52.7%) men and 26 (47.3%) women. The parathyroid gland was the most commonly affected (78.2%), followed by the pituitary gland (69.1%) and adrenal gland (16.4%). Insulinoma was the first manifestation of MEN1 in at least 23.6% (13/55) of patients. Nineteen (34.5%) patients presented with initial symptoms of hypoglycemia before the age of 22 years. Among the 24 Patients with high serum calcium (Ca) had significantly lower serum insulin levels than those with normal serum Ca levels (p < 0.001) during hypoglycemic episodes. However, serum C-peptide level at 0.5 h and serum insulin level at 1 h was higher in patients with hypercalcemia than in patients with normal serum Ca levels in the oral glucose tolerance test (OGTT), although the differences were not statistically significant. Multifocal pancreatic neuroendocrine tumors (pNETs) were present in 38 (69.1%) patients; most of them (55.6%, 20/36) underwent multiple enucleations, and 45% (9/20) had a postoperative recurrence. Five patients (10%) who underwent distal pancreatectomy developed pancreatic insufficiency after an average of seven years. patients who underwent genetic testing, 23 (95.8%) were positive for MEN1 mutation, with mutations most commonly found in exons 2 (21.7%) and 3 (13%). CONCLUSIONS: In our study, the rates of postoperative recurrence and long-term complications in patients with MEN1 with multifocal pNETs were significantly different from those in other international centers and might be related to the choice of surgical method. In addition, elevated serum Ca levels in patients with primary hyperparathyroidism may affect insulin secretion.


Assuntos
Hipoglicemia , Insulinoma , Insulinas , Neoplasia Endócrina Múltipla Tipo 1 , Tumores Neuroectodérmicos Primitivos , Neoplasias Pancreáticas , Adulto , Feminino , Humanos , Insulinoma/diagnóstico , Insulinoma/patologia , Insulinoma/cirurgia , Masculino , Neoplasia Endócrina Múltipla Tipo 1/genética , Neoplasia Endócrina Múltipla Tipo 1/patologia , Neoplasias Pancreáticas/genética , Neoplasias Pancreáticas/patologia , Adulto Jovem
10.
Clin Nucl Med ; 47(7): e498-e499, 2022 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-35675141

RESUMO

ABSTRACT: Dual-tracer PET/CT with both 18F-FDG and 68Ga-DOTA-conjugated peptides is currently used in clinical routine for characterizing pNET (pancreatic masses suspicious for neuroendocrine tumor). We describe here the case of a 81-year-old man with a pancreatic lesion showing high 68Ga-DOTATOC uptake and mild 18F-FDG avidity, thus suggesting a well-differentiated pNET, which resulted at endoscopic ultrasound-guided fine-needle aspiration to be a clear cell renal cell carcinoma metastasis. In fact, the patient had right nephrectomy for clear cell renal cell carcinoma 27 years earlier. This case puts light on the role of PET/CT with 68Ga-DOTATOC in imaging RCC, a field which deserves to be further explored.


Assuntos
Carcinoma de Células Renais , Neoplasias Renais , Tumores Neuroectodérmicos Primitivos , Tumores Neuroendócrinos , Compostos Organometálicos , Neoplasias Pancreáticas , Idoso de 80 Anos ou mais , Carcinoma de Células Renais/diagnóstico por imagem , Fluordesoxiglucose F18 , Humanos , Neoplasias Renais/diagnóstico por imagem , Masculino , Tumores Neuroendócrinos/diagnóstico por imagem , Tumores Neuroendócrinos/patologia , Octreotida/análogos & derivados , Neoplasias Pancreáticas/diagnóstico por imagem , Neoplasias Pancreáticas/patologia , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Compostos Radiofarmacêuticos
11.
J Surg Oncol ; 126(1): 168-174, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35689580

RESUMO

BACKGROUND AND OBJECTIVES: Incidence of pancreatic neuroendocrine tumors (pNETS) seems to be rising over the years, with many cases incidentally diagnosed. Surgery and active surveillance are current treatment modalities for small pNETS. We review our institutional series and compare outcomes for small asymptomatic and nonfunctioning tumors. METHODS: This retrospective cohort study included patients with 2 cm or less and well differentiated pNETS at a single Brazilian Cancer Center. From 2002 to 2020, patients received active surveillance or surgery as a treatment strategy. Short and long-term results were compared. RESULTS: Sixty-four patients were included, 41 in surgical strategy and 23 in the active surveillance approach. Baseline group characteristics were comparable. More patients on active surveillance underwent abdominal magnetic resonance imaging (MRI) and had tumors located in the pancreatic head (41% vs. 17%, p = 0.038). Minimally invasive procedure was chosen in 80.1% of the surgical patients. No patient died after surgery. Median follow-up period was 38.6 and 46.4 months for active surveillance and surgery cohorts, respectively. No difference in disease progression rate was observed. CONCLUSION: Both approaches seem to be safe for small pNETs. Long-term outcome and quality of life should be considered when discussing such options with patients.


Assuntos
Tumores Neuroectodérmicos Primitivos , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Brasil/epidemiologia , Estudos de Coortes , Humanos , Tumores Neuroendócrinos/patologia , Pancreatectomia/métodos , Neoplasias Pancreáticas/patologia , Qualidade de Vida , Estudos Retrospectivos , Conduta Expectante
12.
Int J Radiat Oncol Biol Phys ; 113(5): 996-1002, 2022 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-35568246

RESUMO

PURPOSE: Our aim was to assess the effect of radiation therapy (RT) dose escalation on outcomes in surgically unresectable Ewing sarcoma (ES)/primitive neuroectodermal tumor (PNET). METHODS AND MATERIALS: Patients with nonmetastatic unresectable ES/PNET (excluding intracranial/chest wall) receiving vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide chemotherapy, planned for definitive RT, were accrued in this single-institution, open-label, phase 3 randomized controlled trial. Randomization was between standard dose RT (SDRT; 55.8 Gy/31 fractions/5 days a week) versus escalated dose RT (EDRT; 70.2 Gy/39 fractions/5 days a week) with a primary objective of improving local control (LC) by 17% (65%-82%). Secondary outcomes included disease-free survival (DFS), overall survival (OS), and functional outcomes by Musculoskeletal Tumor Society score. RESULTS: Between April 2005 and December 2015, 95 patients (SDRT 47 and EDRT 48) with a median age of 17 years (interquartile range, 13-23 years) were accrued. The majority of patients were male (59%). Pelvis was the most common site of primary disease (n = 60; 63%). The median largest tumor dimension (9.7 cm) and the median maximum standardized uptake value (8.2) on pretreatment fluorodeoxyglucose positron emission tomography-computed tomography were similar. At a median follow-up of 67 months, the 5-year LC, DFS, and OS for the entire cohort was 62.4%, 41.3%, and 51.9%, respectively. The 5-year LC was significantly better in EDRT compared with SDRT (76.4% vs 49.4%; P = .02). The differences in DFS and OS at 5 years (for EDRT vs SDRT) did not achieve statistical significance (DFS 46.7% vs 31.8%; P = .22 and OS 58.8% vs 45.4%; P = .08). There was a higher incidence of Radiation Therapy Oncology Group grade >2 skin toxic effects (acute) in the EDRT arm (10.4% vs 2.1%; P = .08) with excellent functional outcomes (median Musculoskeletal Tumor Society score = 29) in both arms. CONCLUSIONS: EDRT results in improved LC with good functional outcomes without a significant increase in toxic effects. Radiation dose escalation should be considered for surgically unresectable nonmetastatic ES/PNET.


Assuntos
Tumores Neuroectodérmicos Primitivos , Sarcoma de Ewing , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Ciclofosfamida , Etoposídeo , Feminino , Humanos , Ifosfamida , Masculino , Sarcoma de Ewing/tratamento farmacológico , Sarcoma de Ewing/patologia , Sarcoma de Ewing/radioterapia , Adulto Jovem
13.
Radiographics ; 42(4): 1145-1160, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35622491

RESUMO

Extraskeletal Ewing sarcoma (EES) is a rare subtype in the Ewing sarcoma family of tumors (ESFT), which also includes Ewing sarcoma of bone (ESB) and, more recently, primitive neuroectodermal tumors. Although these tumors often have different manifestations, they are grouped on the basis of common genetic translocation and diagnosis from specific molecular and immunohistochemical features. While the large majority of ESFT cases occur in children and in bones, approximately 25% originate outside the skeleton as EES. Importantly, in the adult population these extraskeletal tumors are more common than ESB. Imaging findings of EES tumors are generally nonspecific, with some variation based on location and the tissues involved. A large tumor with central necrosis that does not cross the midline is typical. Despite often nonspecific findings, imaging plays an important role in the evaluation and management of ESFT, with MRI frequently the preferred imaging modality for primary tumor assessment and local staging. Chest CT and fluorine 18 fluorodeoxyglucose PET/CT are most sensitive for detecting lung and other distant or nodal metastases. Management often involves chemotherapy with local surgical excision, when possible. A multidisciplinary treatment approach should be used given the propensity for large tumor size and local invasion, which can make resection difficult. Despite limited data, outcomes are similar to those of other ESFT cases, with 5-year survival exceeding 80%. However, with metastatic disease, the long-term prognosis is poor. ©RSNA, 2022.


Assuntos
Neoplasias Ósseas , Tumores Neuroectodérmicos Primitivos , Sarcoma de Ewing , Adulto , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/patologia , Criança , Humanos , Imagem Multimodal , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Sarcoma de Ewing/diagnóstico por imagem , Sarcoma de Ewing/terapia , Dedos do Pé/patologia
14.
Oncologist ; 27(2): e116-e125, 2022 03 04.
Artigo em Inglês | MEDLINE | ID: mdl-35641209

RESUMO

BACKGROUND: Pulmonary neuroendocrine tumors (pNETs) include typical carcinoid (TC), atypical carcinoid (AC), large cell neuroendocrine carcinoma (LCNEC), and small cell lung carcinoma (SCLC). The optimal treatment strategy for each subtype remains elusive, partly due to the lack of comprehensive understanding of their molecular features. We aimed to explore differential genomic signatures in pNET subtypes and identify potential prognostic and therapeutic biomarkers. METHODS: We investigated genomic profiles of 57 LCNECs, 49 SCLCs, 18 TCs, and 24 ACs by sequencing tumor tissues with a 520-gene panel and explored the associations between genomic features and prognosis. RESULTS: Both LCNEC and SCLC displayed higher mutation rates for TP53, PRKDC, SPTA1, NOTCH1, NOTCH2, and PTPRD than TC and AC. Small cell lung carcinoma harbored more frequent co-alterations in TP53-RB1, alterations in PIK3CA and SOX2, and mutations in HIF-1, VEGF and Notch pathways. Large cell neuroendocrine carcinoma (12.7 mutations/Mb) and SCLC (11.9 mutations/Mb) showed higher tumor mutational burdens than TC (2.4 mutations/Mb) and AC (7.1 mutations/Mb). 26.3% of LCNECs and 20.8% of ACs harbored alterations in classical non-small cell lung cancer driver genes. The presence of alterations in the homologous recombination pathway predicted longer progression-free survival in advanced LCNEC patients with systemic therapy (P = .005) and longer overall survival (OS) in SCLC patients with resection (P = .011). The presence of alterations in VEGF (P = .048) and estrogen (P = .018) signaling pathways both correlated with better OS in patients with resected SCLC. CONCLUSION: We performed a comprehensive genomic investigation on 4 pNET subtypes in the Chinese population. Our data revealed distinctive genomic signatures in subtypes and provided new insights into the prognostic and therapeutic stratification of pNETs.


Assuntos
Tumor Carcinoide , Carcinoma de Células Grandes , Carcinoma Neuroendócrino , Carcinoma Pulmonar de Células não Pequenas , Neoplasias Pulmonares , Tumores Neuroectodérmicos Primitivos , Tumores Neuroendócrinos , Carcinoma de Pequenas Células do Pulmão , Biomarcadores , Tumor Carcinoide/patologia , Carcinoma de Células Grandes/genética , Carcinoma de Células Grandes/patologia , Carcinoma Neuroendócrino/genética , Carcinoma Neuroendócrino/patologia , Carcinoma Pulmonar de Células não Pequenas/patologia , China , Genômica , Humanos , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/patologia , Tumores Neuroendócrinos/genética , Tumores Neuroendócrinos/patologia , Prognóstico , Carcinoma de Pequenas Células do Pulmão/genética , Fator A de Crescimento do Endotélio Vascular
15.
JNMA J Nepal Med Assoc ; 60(247): 303-305, 2022 Mar 11.
Artigo em Inglês | MEDLINE | ID: mdl-35633271

RESUMO

Primitive neuroectodermal tumour is a poorly differentiated small round cell neoplasm that primarily affects children and is very rarely seen in adults. Peripheral primitive neuroectodermal tumours are rare compared to the central type and resemble soft tissue sarcoma. Primitive neuroectodermal tumours involving the subcutaneous tissue are rare and only a few cases involving the subcutaneous tissue of the anterior abdominal wall have been reported. However, no cases involving the subcutaneous tissue of the shoulder region have been reported. We report the case of a peripheral primitive neuroectodermal tumour arising from subcutaneous tissue of the right shoulder in a young adult. Keywords: case report; magnetic resonance imaging; neuroectodermal tumour; neuron-specific enolase; subcutaneous tissue.


Assuntos
Tumores Neuroectodérmicos Primitivos Periféricos , Tumores Neuroectodérmicos Primitivos , Criança , Humanos , Imageamento por Ressonância Magnética , Tumores Neuroectodérmicos Primitivos/diagnóstico , Tumores Neuroectodérmicos Primitivos/metabolismo , Tumores Neuroectodérmicos Primitivos/patologia , Tumores Neuroectodérmicos Primitivos Periféricos/diagnóstico por imagem , Tumores Neuroectodérmicos Primitivos Periféricos/patologia , Ombro/patologia , Tela Subcutânea/metabolismo , Tela Subcutânea/patologia , Adulto Jovem
16.
Pediatr Blood Cancer ; 69(9): e29736, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35570402

RESUMO

BACKGROUND: An unexplained regional difference in survival was observed in previous publications on outcome for children treated for medulloblastoma and supratentorial primitive neuroectodermal tumor (CNS-PNET) in Norway. We aimed now to reevaluate and perform a retrospective molecular-based risk stratification of all embryonal brain tumors (excluding atypical teratoid rhabdoid tumors [ATRT]) in pediatric patients, who underwent surgery and treatment at Oslo University Hospital between 2005 and 2017. PROCEDURE: Specimens from all patients <20 years of age with initial diagnosis of medulloblastoma or CNS-PNET were reviewed. Molecular analyses comprised NanoString gene expression, molecular inversion probe profiling, Sanger sequencing, and 850K-methylation analysis. Whole chromosomal aberration signatures were assessed in standard-risk non-WNT/non-SHH medullobastomas for molecular risk stratification. RESULTS: We identified 53 non-ATRT embryonal tumors among which 33 were medulloblastomas. Molecular genetic parameters including whole chromosomal aberration signatures allowed classification of 17 medulloblastomas as molecular high risk. These patients had a significantly worse 5-year overall survival than the remaining 16 medulloblastoma patients (52.9% vs. 87.1% p = 0.036). Five patients in our cohort had tumors that are considered as new entities in the 2021 classification of tumors of the central nervous system. Five tumors were re-classified as nonembryonal tumors after review. CONCLUSION: Molecular-based risk stratification of standard-risk non-WNT/non-SHH medulloblastoma enabled superior identification of medulloblastomas with dismal prognosis. Our cohort demonstrated a significantly increased fraction of standard-risk non-WNT/non-SHH medulloblastoma with molecular high-risk profile compared to other studies, which might have contributed to previously reported unfavorable outcome data.


Assuntos
Neoplasias Encefálicas , Neoplasias do Sistema Nervoso Central , Neoplasias Cerebelares , Meduloblastoma , Tumores Neuroectodérmicos Primitivos , Tumor Rabdoide , Neoplasias Encefálicas/patologia , Neoplasias do Sistema Nervoso Central/genética , Neoplasias do Sistema Nervoso Central/terapia , Neoplasias Cerebelares/genética , Neoplasias Cerebelares/metabolismo , Neoplasias Cerebelares/terapia , Criança , Aberrações Cromossômicas , Humanos , Meduloblastoma/genética , Meduloblastoma/metabolismo , Meduloblastoma/terapia , Tumores Neuroectodérmicos Primitivos/patologia , Estudos Retrospectivos , Tumor Rabdoide/genética
17.
BMC Surg ; 22(1): 160, 2022 May 10.
Artigo em Inglês | MEDLINE | ID: mdl-35538535

RESUMO

BACKGROUND: Although some factors that predict the prognosis in pancreatic neuroendocrine tumor (pNET) have been confirmed, the predictive value of lymph node metastasis (LNM) in the prognosis of pNETs remains conflicting and it is not clear whether regional lymphadenectomy should be performed in all grades of tumors. METHODS: We included pNET patients undergoing surgery in Shanghai pancreatic cancer institute (SHPCI). The risk factors for survival were investigated by the Kaplan-Meier method and Cox regression model. We evaluated the predictors of LNM using Logistic regression. RESULTS: For 206 patients in the SHPCI series, LNM was an independent prognostic factor for entire cohort suggested by multivariate Cox regression analysis. LNM (P = 0.002) predicted poorer overall survival (OS) in grade 2/3 cohort, but there is no significant association between LNM and OS in grade 1 cohort. Grade (P < 0.001) and size (P = 0.049) predicted LNM in entire cohort. Grade (P = 0.002) predicted LNM while regardless of size in grade 2/3 cohort. CONCLUSIONS: Based on our own retrospective data obtained from a single center series, LNM seems to be associated with poorer outcome for patients with grade 2/3 and/or grade 1 > 4 cm tumors. On the other way, LNM was seems to be not associated with prognosis in patients with grade 1 tumors less than 4 cm. Moreover, tumor grade and tumor size seem to act as independent predictors of LNM. Thus, regional lymphadenectomy should be performed in grade 2/3 patients but was not mandatory in grade 1 tumors < 4 cm. It is reasonable to perform functional sparing surgery for grade 1 patients or propose a clinical-radiological monitoring.


Assuntos
Tumores Neuroectodérmicos Primitivos , Tumores Neuroendócrinos , Neoplasias Pancreáticas , China/epidemiologia , Humanos , Excisão de Linfonodo , Linfonodos/patologia , Metástase Linfática , Tumores Neuroectodérmicos Primitivos/cirurgia , Tumores Neuroendócrinos/patologia , Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/patologia , Prognóstico , Estudos Retrospectivos
19.
Pancreas ; 51(3): 282-287, 2022 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-35584387

RESUMO

OBJECTIVES: During the last decades, significant progress has been made in the management of patients with pancreatic neuroendocrine tumors (pNETs). It is unclear how the type of the treating health care facility alters patient outcomes. METHODS: Data from pNETs reported to the National Cancer Database between 2004 and 2016 were examined. Types of institutions were as follows: academic/research cancer program (ARP), comprehensive community cancer program (CCCP), integrated network cancer program (INCP), and community cancer program (CCP). RESULTS: A total of 17,887 patients with pNETs were analyzed. Treatment at ARPs was significantly associated with receipt of surgery (ARP, 61.9%; CCCP, 45.6%; CCP, 29.9%; INCP, 55.5%; P < 0.001), both for patients with very early tumors ≤2 cm (ARP, 74.7%; CCCP, 66.5%; CCP, 52.4%; INCP, 71.6%; P < 0.001) and for patients with liver metastases (ARP, 21.3%; CCCP, 10.6%; CCP, 5%; INCP, 16.8%; P < 0.001). Treatment at ARPs was associated with improved survival (median overall survival: ARP, 91 mo; CCCP, 47 mo; CCP, 24.5 mo; INCP, 72 mo; P < 0.001). CONCLUSIONS: Treatment of pNETs at academic/research programs is associated with more frequent resections and best survival outcomes. This survival benefit exists for early and late stages and after adjusting for known cofactors.


Assuntos
Tumores Neuroectodérmicos Primitivos , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Carbonil Cianeto m-Clorofenil Hidrazona , Instalações de Saúde , Humanos , Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/cirurgia , Estudos Retrospectivos
20.
Crit Rev Oncol Hematol ; 175: 103713, 2022 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-35598829

RESUMO

AIM: A range of CT characteristics with potential prognostic value have previously been identified for gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs). Still, there is no widely accepted consensus on which characteristics should be reported as prognostic factors. This systematic review therefore aims to provide an overview of the available literature regarding CT characteristics and their prognostic significance for GEP-NET patients. MATERIALS AND METHODS: PubMed, Embase, and Scopus/Cochrane Library databases were searched and a forward and backward reference check of the identified studies was executed. Eligible studies were conducted in patients with GEP-NET, and reported on the prognostic significance (in terms of tumor grade, spread of disease, and survival) of CT-based biomarkers. Study selection, quality assessment and data extraction were performed by two reviewers independently, resolving disagreement by consensus. RESULTS: In total, 5074 unique studies were identified, of which 37 were included. Given the paucity of data on GEP-NETs other than PNET, data extraction and analyses was restricted to PNETs. Fourteen CT characteristics were correlated to prognostic outcomes. Larger tumor size, hypo-enhancement, irregular shape and ill-defined margins, presence of locally invasive growth, lymphadenopathy and metastases were predictors of poorer prognosis according to 65-89% of the available studies. Most studies were regarded as having a low (65%) or moderate (24%) risk of bias. CONCLUSION: Evidence regarding prognostic value of CT-based biomarkers for PNETs is limited to heterogeneous, retrospective studies. Nonetheless, heterogeneity in data is more likely to obscure than to overestimate any correlation. Therefore, we feel that the before-mentioned characteristics should be regarded and reported as clinically relevant predictors of poorer prognosis.


Assuntos
Tumores Neuroectodérmicos Primitivos , Tumores Neuroendócrinos , Neoplasias Pancreáticas , Neoplasias Gástricas , Humanos , Neoplasias Intestinais , Tumores Neuroendócrinos/diagnóstico por imagem , Tumores Neuroendócrinos/patologia , Neoplasias Pancreáticas/diagnóstico por imagem , Neoplasias Pancreáticas/patologia , Prognóstico , Estudos Retrospectivos , Neoplasias Gástricas/patologia , Tomografia Computadorizada por Raios X
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