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1.
Zhonghua Wei Chang Wai Ke Za Zhi ; 23(1): 38-43, 2020 Jan 25.
Artigo em Chinês | MEDLINE | ID: mdl-31958929

RESUMO

Objective: To investigate clinicopathological features and prognostic factors of gastric neuroendocrine tumors (G-NEN). Methods: Clinical and pathological data of patients with G-NEN diagnosed by pathological examination in Chinese PLA General Hospital from January 2000 to June 2018 were retrospectively analyzed in this case-control study. Patients with complicated visceral lesions, other visceral primary tumors, mental disorders and incomplete clinicopathological data were excluded. Finally, 240 hospitalized patients who met the inclusion criteria were enrolled. Physical examination information, tumor characteristics and pathological characteristics of patients were summarized. The Cox regression models were used to analyze the risk factors affecting G-NEN and the survival conditions were described by Kaplan-Meier survival curves and log-rank test. Results: In 240 patients with G-NEN, the mean age was (60.3±10.1) years; 181 were male (75.4%) and 59 females (24.6%); mean tumor diameter was (4.2±2.8) cm; 51 cases (21.2%) were neuroendocrine tumor (NET), 139 cases (57.9%) neuroendocrine carcinoma (NEC), 50 cases (20.8%) mixed neuroendocrine carcinoma (MANEC); 28 cases (11.7%) were G1 low grades, 34 cases (14.2%) G2 medium grades, and 178 cases (74.2%) G3 high grades; tumor infiltration depth T1 to T4 were 44 cases (18.3%), 27 cases (11.2%), 60 cases (25.0%) and 109 cases (45.4%) respectively; 163 cases (67.9%) developed lymphatic metastasis and 46 patients (19.2%) distant metastasis; tumor stage from stage I to stage IV were 55 cases (22.9%), 42 cases (17.5%), 94 cases (39.2%) and 53 cases (22.1%) respectively. Of the 240 G-NEN patients, 223 cases (92.9%) were followed up. The median survival time of the patients was 39.2 (95% CI: 29.1 to 47.5) months. Univariate survival analysis showed that age ≥ 60 years, tumor diameter ≥ 4.2 cm, tumor grade G3, lymphatic metastasis, distant metastasis, and tumor stage III-IV were risk factors for G-NEN patients. Multivariate survival analysis revealed that lymphatic metastasis (HR=1.783, 95%CI: 1.007-3.155, P=0.047) and distant metastasis (HR=2.288, 95% CI: 1.307-4.008, P=0.004) were independent risk factors of the prognosis. Further analysis of the G3 subgroup of G-NEN showed that the 5-year survival rate of NET-G3 was 76.19%, which was significantly higher than that of NEC-G3 and MANEC-G3 (15.60% and 24.73%, P=0.012). Conclusions: Most G-NEN patients are in advanced stage at diagnosis. Lymphatic metastasis and distant metastasis indicate poor prognosis. The prognosis of high proliferation NET-G3 patients is better as compared to those of NEC-G3 and MANEC-G3. This classification is worth further attention.


Assuntos
Tumores Neuroendócrinos/diagnóstico , Neoplasias Gástricas/diagnóstico , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Tumores Neuroendócrinos/mortalidade , Tumores Neuroendócrinos/patologia , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Neoplasias Gástricas/mortalidade , Neoplasias Gástricas/patologia , Taxa de Sobrevida
2.
Eur J Endocrinol ; 182(4): R29-R58, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31999619

RESUMO

Ectopic ACTH syndrome (EAS) is rare but is frequently a severe condition because of the intensity of the hypercortisolism that may be dissociated from the tumoral condition. EAS should often be considered as an endocrine emergency requiring an emergency response both in terms of diagnostic procedures and therapeutic interventions. Patient management is complex and necessitates dual skills, in the diagnosis and treatment of CS and in the specific management of neuroendocrine tumors (NET). Therefore, initial management should be performed ideally by experienced endocrinology teams in collaboration with specialized hormonal laboratory, modern imaging platforms and intensive care units. Diagnostic procedures vary according to the endocrine and tumoral contexts but should be reduced to a minimum in intense hypercortisolism. Preventive and curative treatments of cortisol-induced comorbidities, non-specific management of hypercortisolism and etiological treatments should be considered simultaneously. Therapeutic strategies vary according to (1.) the intensity of hypercortisolism, the general condition of the patient and associated comorbidities and (2.) the tumoral status, ranging from resectable ACTH secreting tumors to non-resectable metastatic endocrine tumors or occult tumors. The ideal treatment is complete excision of the ACTH-secreting tumor that can be performed rapidly or after preoperative preparation using cortisol-lowering drugs. When this is not possible, the therapeutic strategy should be discussed by a multidisciplinary experienced team in a personalized perspective and include variable combinations of pharmacological agents, bilateral adrenalectomy and non-specific tumoral interventions. Here we discuss the diagnosis and therapeutic strategies including the modern, currently available tools and emphasize on the operational effectiveness of care.


Assuntos
Síndrome de ACTH Ectópico/diagnóstico , Síndrome de ACTH Ectópico/terapia , Síndrome de Cushing/diagnóstico , Síndrome de Cushing/terapia , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/terapia , Síndrome de ACTH Ectópico/complicações , Síndrome de Cushing/etiologia , Endocrinologia/métodos , Humanos , Tumores Neuroendócrinos/complicações , Resultado do Tratamento
3.
Int J Cancer ; 146(5): 1316-1323, 2020 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-31509608

RESUMO

Due to the increasing incidence and prevalence of neuroendocrine tumors (NETs), there is a need to assess any gaps in awareness and care. A survey was undertaken in 2017 to identify perceived unmet needs from the perspectives of patients/families, patient advocates and health care professionals (HCPs). The survey consisted of 33-37 questions (depending on type of respondent) across four areas: information, care, treatments and research. In total, 443 participants from 26 countries responded: 338 patients/families, 35 advocates and 70 HCPs. Perceived unmet needs regarding provision of information at diagnosis differed between groups. While 59% of HCPs believed they provided sufficient information, informational needs were mostly/fully met for only 30% of patients and 18% of advocates. Additionally, 91% of patients and 97% of advocates felt that patients had to search for information themselves. Availability of Gallium-68-Dotatate PET/CT scan was limited for the majority of patients (patients: 73%; advocates: 85%; HCP: 86%), as was access to treatments, particularly peptide receptor radionuclide therapy (patients: 42%; advocates: 95%; HCPs: 77%). All groups felt that standards of care, including psychological needs and diagnosis of mental health, were not fully met. Although about two-thirds of patients were managed by a multidisciplinary team, 14% of patients reportedly did not have enough contact. All groups supported more patient involvement in research; patients and advocates prioritized improvement in diagnosis and HCPs focused on clinical trials. This survey revealed significant unmet needs but differing perceptions regarding these among the groups. There is a need for investigation and collaboration to improve standards of care for NET patients.


Assuntos
Saúde Global , Necessidades e Demandas de Serviços de Saúde/estatística & dados numéricos , Tumores Neuroendócrinos/terapia , Participação do Paciente/estatística & dados numéricos , Lacunas da Prática Profissional/estatística & dados numéricos , Adolescente , Adulto , Carga Global da Doença , Comunicação em Saúde , Pessoal de Saúde/estatística & dados numéricos , Humanos , Incidência , Comportamento de Busca de Informação , Oncologia/organização & administração , Oncologia/estatística & dados numéricos , Pessoa de Meia-Idade , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/epidemiologia , Neuroendocrinologia/organização & administração , Neuroendocrinologia/estatística & dados numéricos , Defesa do Paciente/estatística & dados numéricos , Prevalência , Relações Profissional-Paciente , Inquéritos e Questionários/estatística & dados numéricos , Adulto Jovem
4.
Oncology ; 98(1): 10-15, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31505502

RESUMO

INTRODUCTION: Neuroendocrine tumors (NETs) are a heterogeneous group of malignancies with various clinical presentations and growth rates. NET incidence has been estimated to 2.5-5 per 100,000 people per year, and NET prevalence is 35 per 100,000. They are frequently associated with synchronous or metachronous second primary malignancies (SPM). METHODS: We retrospectively reviewed our institutional database on NET patients. We report on 30 patients with NETs and SPMs from a series of 262 patients with NETs: 10 patients with synchronous NETs (33.3%) and 20 with metachronous SPMs (66.6%). RESULTS: The median patient age was 67 years. Of the 10 synchronous lesions, 50% were observed in the GI tract. The most common locations of these lesions were the colon (15%) and pancreas (25%). In 2 patients, there was an association of prostate neoplasia with a subsequent NET of the pancreas. CONCLUSIONS: Only few studies have examined the association between NETs and SPMs. Our study showed that the risk of second cancer following NETs is increased. In this single-institution retrospective review, our incidence of additional malignancies in patients with NET was 11.4%.


Assuntos
Neoplasias Primárias Múltiplas/epidemiologia , Segunda Neoplasia Primária/epidemiologia , Tumores Neuroendócrinos/epidemiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/diagnóstico , Segunda Neoplasia Primária/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Estudos Retrospectivos
6.
Am J Clin Pathol ; 153(1): 99-104, 2020 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-31587038

RESUMO

OBJECTIVES: We investigated the ability of routine flow cytometry (FC) to detect nonhematologic neoplasms (non-HN) using antibody panels routinely used for the diagnosis of hematologic neoplasms. METHODS: FC analyses of 4,000 various diagnostic samples were retrospectively reviewed to identify cases in which an aberrant, viable CD45-negative, nonhematologic neoplastic population was detected by FC panels designed to evaluate hematologic neoplasms. RESULTS: A total of 57 (1.4%) diverse non-HNs were identified, representing neuroendocrine tumors (33/57) and carcinomas (9/57), as well as other malignancies (15/57) such as sarcoma and melanoma. The majority of neoplasms were positive for at least one antibody, typically CD56 (43/51, 84.3%), followed by CD117 (15/34, 44.1%) and CD138 (6/33, 18.2%). CONCLUSIONS: Our findings highlight the importance of carefully inspecting CD45-negative events to identify non-HNs by routine FC analysis. This can help expedite further downstream immunophenotypic analysis of specimens and triage samples for appropriate genetic and molecular studies.


Assuntos
Citometria de Fluxo , Neoplasias Hematológicas/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Medula Óssea/patologia , Neoplasias Hematológicas/patologia , Humanos , Imunofenotipagem , Antígenos Comuns de Leucócito/metabolismo , Tumores Neuroendócrinos/patologia
7.
BMC Surg ; 19(1): 197, 2019 Dec 18.
Artigo em Inglês | MEDLINE | ID: mdl-31852474

RESUMO

BACKGROUND: Ectopic insulinoma is a rare entity that is difficult to diagnose before surgery. This article reports two cases of ectopic insulinoma. CASE PRESENTATION: Two patients manifested recurrent hypoglycemia with a typical Whipple triad. In terms of the qualitative diagnosis, the oral glucose tolerance test (OGTT) suggested a diagnosis of hyperinsulinemic hypoglycemia. However, preoperative imaging did not show a significant mass in the pancreas. In one patient, preoperative abdominal enhanced volume perfusion computed tomography (CT), somatostatin receptor imaging and 99mTc-HYNIC-TOC SPECT/CT revealed a mass with a rich blood supply anterior to the duodenum. In the other patient, preoperative enhanced CT, magnetic resonance imaging (MRI) and 68Ga-Exendin-4 PET/CT showed a mass above the spleen. After surgical removal of the tumor, both patients received a confirmed diagnosis of neuroendocrine tumors by postoperative pathology. The symptoms of hypoglycemia were relieved after surgery, and the blood glucose level was significantly increased. CONCLUSION: Ectopic insulinoma is difficult to locate before surgery. 68Ga-Exendin-4 PET/CT has a high diagnostic value. Surgical removal of the lesion is main treatment.


Assuntos
Insulinoma/diagnóstico , Insulinoma/cirurgia , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/cirurgia , Feminino , Humanos , Hipoglicemia/etiologia , Pessoa de Meia-Idade , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Tomografia Computadorizada por Raios X
8.
Medicine (Baltimore) ; 98(50): e18278, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31852101

RESUMO

RATIONALE: Primary hepatic neuroendocrine tumors (PHNET) are extremely rare, which makes it difficult for doctors not deeply to be aware of their imaging and pathological characteristics. Therefore, it is challenging to diagnose PHNET accurately without biopsy or surgical excision. The purpose of this study is PATIENT CONCERNS:: A 52-year-old male patient came to our outpatient department with intermittent upper abdominal pain. DIAGNOSES: PHNET. INTERVENTIONS: Biochemical examination and imaging examination were performed prior to operation. Liver tumors were removed by ultrasound scalpel under laparoscopy. Pathology examination of liver tumors was performed after operation. Symptomatic supportive treatment was performed after operation as well, including anti-inflammation and rehydration. OUTCOMES: The results of biochemical examination were generally normal. The results of MRI showed low signal on T1WI, slightly high signal on T2WI/FS and DWI manifestation of high signal. Immunohistochemistry (IHC) showed that synaptophysin (Syn) was positive, CD56 was positive, chromaffin A (CgA) was positive, and Ki-67 was 15%. The patient was generally in good condition and no discomfort or recurrence was reported during 15 months of follow-up. LESSONS: The incidence of PHNET is extremely low. Sometimes the patient has no cirrhosis or hepatitis, and alpha-fetoprotein is not high, but imaging examination shows solid occupation and clear boundaries of the liver tumor, for which doctors should consider the primary liver nerve tumor. The diagnosis of PHNET depends on pathological characteristics. Surgical excision is the main method to treat the disease.


Assuntos
Hepatectomia/métodos , Neoplasias Hepáticas/diagnóstico , Fígado/diagnóstico por imagem , Tumores Neuroendócrinos/diagnóstico , Biópsia , Diagnóstico Diferencial , Seguimentos , Humanos , Neoplasias Hepáticas/cirurgia , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/cirurgia , Tomografia Computadorizada por Raios X , Ultrassonografia
9.
BMC Cancer ; 19(1): 1226, 2019 Dec 16.
Artigo em Inglês | MEDLINE | ID: mdl-31842791

RESUMO

BACKGROUND: Neuroendocrine tumours (NETs) are rare malignancies characterised by its capacity to synthesise and secrete monoamines, due to its neuroendocrine origin. Its varied locations and symptoms have traditionally been responsible for extended delays in their diagnosis. The interest of this study was to characterise the patient population diagnosed with NETs in Spain and to revise how the disease is managed, together with the hospitalisation costs of these patients. METHODS: The database included records of all patients diagnosed with a NET between 2010 and 2015. Admission records were used to evaluate hospitalisation, disease management data and costs, and single-patient files were used to characterise the population. RESULTS: Nine Thousand One Hundred Twenty patients were diagnosed with a neuroendocrine tumour between 2010 and 2015, with a 2 fold increase in the diagnosis rate over the study period. 42.25% of the patients were females, while 57.75% were males, and mean diagnosis age was 62.58 years (SD = 14.65). Considering all the registered neuroendocrine neoplasms, 46.86% of the patients had malignant well-differentiated NETs, 32.02% had a malignant poorly differentiated neuroendocrine carcinoma and 42.93% of patients developed metastatic NETs. In addition, 18.59% of patients were diagnosed with benign well-differentiated NETs. The most common tumour sites were the bronchus, lung and other sites, including pancreatic tumours; metastasis was found in the liver and distant lymph nodes. Pancreatic resection was the most common surgical procedure utilised in these patients, summing 19% of total expenses, the injection of an unspecified therapeutic substance (including targeted therapies) was registered in 11.40% of admissions, while chemotherapy was registered in only 6.85% of admissions. The annual healthcare cost of NETs was €15,373,961, corresponding to €9092 per patient. CONCLUSIONS: The implementation of standard diagnosis procedures should be prioritised, with a focus on the pancreas and lung, and taking into account that 42.93% of the patients develop a metastatic tumour. The presence of comorbidities and multimorbidities should be considered in order to develop more efficient disease management protocols.


Assuntos
Gerenciamento Clínico , Tumores Neuroendócrinos/epidemiologia , Sistema de Registros , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Feminino , Custos de Cuidados de Saúde , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/economia , Tumores Neuroendócrinos/terapia , Espanha , Adulto Jovem
10.
Pan Afr Med J ; 34: 48, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31762914

RESUMO

Neuroendocrine tumors of the cervix are rare accounting for only 0.9-1.5% of these tumors. The non-specificity of the clinical signs and the absence of a screening test delay diagnosis. Immunohistochemical and histological studies play a crucial role to confirm the diagnosis. Today, therapeutic management is difficult and prognosis is unfavorable. We report a case of neuroendocrine tumor of the cervix and we will outline the peculiarities of this rare disease.


Assuntos
Tumores Neuroendócrinos/diagnóstico , Neoplasias do Colo do Útero/diagnóstico , Feminino , Humanos , Pessoa de Meia-Idade , Tumores Neuroendócrinos/patologia , Prognóstico , Neoplasias do Colo do Útero/patologia
11.
Chirurgia (Bucur) ; 114(5): 550-563, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31670630

RESUMO

Digestive cancers diagnosed during pregnancy are rare and are sporadically reported. The shift of pregnancy towards the age interval of 30-39 years old (and even 40-49 years old) could explain the more and more frequent superposition of pregnancy and cancer. Pancreatic Neuroendocrine Tumours (PNET) originate in the insular endocrine tissue and are extremely rare; they have a slow, less aggressive neoplastic development, with the capacity of secreting and storing different peptides and neuroamines. From the large group of NET tumours, approximately 70% have a gastroenterohepatic (GET) localisation and represent less than 2% of the digestive tract tumours. PNET incidence is extremely reduced, of approximately 1/100.000. The association of pregnancy and PNET in literature is exceptional until now only 39 cases were reported. The most frequent PNET in pregnant patients is the insulinoma with the starting point in pancreatic B-cells, with 27 out of 39 PNET worldwide reported cases until 2012. Establishing a diagnosis in pregnant patients is difficult in the first period of pregnancy because physiologically, the glucose tends to be low. This may be a consequence of the increased secretion of insulin and sensibility to insulin, probably as an effect of increased oestrogen levels. Furthermore, there are other signs which could suggest the existence of a "problem", which are relatively common during the first trimester of pregnancy: unexplained fatigue, vomiting, hypotension, rare episodes of hypoglycaemia. Performing paraclinical tests on these patients is difficult; an essential indicator of the opportunity for the investigation is the pregnancy trimester. The precaution about a foetal injury when indicating an invasive exploration (imaging, endoscopy, etc.) delays the diagnosis. The insulinomas have a rather benign potential than an aggressive nature, and their prognosis is usually favourable, with therapeutic possibilities easy to administrate and control in pregnant patients. Medical treatment is used in patients when there is a positive diagnosis but the topography of the insulinoma was not identified, when the symptomatology is easy to control through conserving therapy until the foetus is mature enough or immediately after birth, in pregnant patients who refuse the surgical procedure, or in the case of metastasis of malignant insulinoma. Surgical treatment indeed remains the only therapeutic method, but most often it is scheduled usually after birth, or if the situation requires, as late as possible after the foetus has a convenient age (after 28 weeks).


Assuntos
Insulinoma/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Complicações Neoplásicas na Gravidez/diagnóstico , Adulto , Feminino , Humanos , Insulinoma/cirurgia , Pessoa de Meia-Idade , Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/cirurgia , Gravidez , Complicações Neoplásicas na Gravidez/cirurgia , Resultado do Tratamento
12.
Chirurgia (Bucur) ; 114(5): 639-649, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31670640

RESUMO

Pancreatic neuroendocrine tumors (PNETs) are rare and characterized by widely variable clinical presentation and often challenging surgical management. Methods: Retrospective study conducted during the last 15 years at the First Surgical Clinic of the Iasi St Spiridon University Hospital, and which included all the patients diagnosed with pancreatic endocrine tumors by immunohistochemistry. Results: There were 26 cases diagnosed with PNET. The male/female ratios was 7/19 and mean age 41.93 +- 2.48 years (range 20-79 years). Of the PNET cases 13 were insulinomas, 5 gastrinomas, 2 gastrinomas associated with other endocrine neoplasms (Wermer syndrome), 5 non-functional endocrine pancreatic tumors and 1 ACTHoma. Clinical manifestations depended on tumor type: hypoglycemia and Whipple triad for insulinoma, Zollinger Ellison syndrome and complicated peptic ulcer (hemorrhage, perforation) for gastrinoma, Cushing syndrome for ACTHoma. Biological diagnosis included biological markers (e.g. insulin, gastrin and cortisol). Tumor site and size at diagnosis were determined by ultrasound, CT-scan, angiography, PETscan, octreoscan and intraoperative ultrasound. Surgical procedures for PNET insulinomas were: tumor resection - 6 cases; left splenopancreatectomy - 3 cases; left spleen-preserving pancreatectomy - 2 cases; pancreaticoduodenectomy - 2 cases. We also present 4 cases of gastrinoma with multiple ulcers and multiple surgical interventions for hemorrhage and perforation with peritonitis. The two patients with Wermer syndrome also had ulcers complicated with hemorrhage and peritonitis and parathyroid adenoma. Nonfunctional pancreatic endocrine tumors were diagnosed in 5 women of which in 3 the tumors were located in the pancreatic tail (in which splenopancreatectomy and left pancreatectomy with spleen preservation were performed) and in 2 in the pancreatic head (in which pancreaticoduodenectomy and Beger type operation were performed). Conclusions: Knowledge of clinical signs of secreting tumors and exploring the patients are of crucial importance for management of PNETs. Immunohistochemistry is mandatory for confirming the diagnosis and assessing the proliferation and biological behavior of the tumor, thus facilitating the administration of specific therapy. Aggressive surgical treatment is indicated, even in advanced stages.


Assuntos
Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/cirurgia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/diagnóstico , Pancreatectomia/métodos , Neoplasias Pancreáticas/diagnóstico , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
13.
Pan Afr Med J ; 33: 238, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31692695

RESUMO

Multiple endocrine neoplasia type 1 (MEN1) is a rare disease, defined as a tumor developing in at least two endocrine glands including the anterior pituitary gland, the parathyroid glands and the duodenopancreatic endocrine tissue. This disorder, inherited in an autosomal dominant pattern, is caused by mutations in the MEN1 gene encoding the tumor suppressor menin and located on chromosome 11q13. However, sporadic cases account for 8-14%. The first endocrine lesion may be solitary in approximately 75% of cases. However, all major alterations can be inaugural. We here report a case of multiple endocrine neoplasia type 1 revealed by aggressive somatoprolactinic pituitary adenoma which didn't respond to conventional treatment. The detection of primary hyperparathyroidism as well as neuroendocrine tumor of the pancreas seven years later make this a very particular case. Therapeutic options are discussed within the multidisciplinary team specialized in endocrine diseases.


Assuntos
Neoplasia Endócrina Múltipla Tipo 1/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Neoplasias Hipofisárias/diagnóstico , Adenoma/diagnóstico , Adenoma/patologia , Adulto , Humanos , Hiperparatireoidismo Primário/diagnóstico , Masculino , Neoplasia Endócrina Múltipla Tipo 1/patologia , Tumores Neuroendócrinos/patologia , Neoplasias Pancreáticas/patologia , Neoplasias Hipofisárias/patologia , Prolactina/metabolismo
14.
Arkh Patol ; 81(5): 11-21, 2019.
Artigo em Russo | MEDLINE | ID: mdl-31626200

RESUMO

An important role in the differentiation of tissues in different organs is played by transforming factors (TFs); pancreatic and duodenal homebox 1 (PDX-1) is one of the earliest factors for pancreatic cells. Many malignant tumors, including neuroendocrine tumors (NETs), are similar in structure, and therefore the actual problem of oncomorphology is to search for narrow-specific markers and TFs. AIM: to comparatively analyze and assess the value of the expression of the TF PDX-1 in NETs and non-NETs of different localization and histogenetic origin. MATERIAL AND METHODS: Anti-PDX-1 antibodies were used to study 528 tumors divided into 3 groups: Group 1 included 394 NETs, among them there were those of the pancreas (n=173), stomach (n=46), bowel (n=65), lung (n=40), thymus (n=8), kidney (n=6), Merkel's cell carcinomas (n=14), NETs of the breast (n=3), larynx (n=2), trachea (n=2), bladder (n=1), and metastatic NETs (n=34) of unknown primary site; Group 2 consisted of 16 tumors, of them there were paragangliomas (n=6), medullary thyroid cancers (MTC) (n=6) and adrenal pheochromocytomas (APCC) (n=4); Group 3 comprised 118 non-NETs, among them there were tumors of the pancreas (n=54), stomach (n=26), bowel (n=17), lung (n=11), breast (n=3), kidney (n=4), adrenal glands (n=2), and bladder (n=1). RESULTS: PDX-1 was positive in 75.1% (130/173) of pancreatic NETs, all insulinomas (50/50), gastrinomas (11/11), somatostatinomas (3/3), ACTH-producing tumors (2/2); PDX-1 was positive in the non-functioning pancreatic NETs, all PPomas (19/19), 76.1% (35/46) of NETs without the hormone detected, 50% (2/4) of calcitoninomas, and 21.1% (8/38) of silent glucagonomas. PDX-1 was positive in 32.4% (11/34) of carcinoids and 50% (6/12) of neuroendocrine carcinomas, all duodenal NETs (18/18), 90% (9/10) of rectal carcinoids and 30.8% (4/13) colonic carcinoids, 37.5% (3/8) of thymic/mediastinal carcinoids, 66.7% (4/6) of kidney carcinoids, and 37.5% (9/24) of metastatic NETs of unknown primary site. PDX-1 was negative in all carcinoids of the colon and sigmoid (0/5), ileum and jejunum (0/24), lung (0/40), trachea (0/2), larynx (0/2), Merkel's cell carcinoma (0/14), breast (0/3), bladder (0/1), as well as MTC (0/6), APCC (0/4), and paragangliomas (0/6). PDX-1-positive non-NETs included 81.8% (18/22) of adenocarcinomas (AC) and all serous cystic, mucinous cystic, intraductal and acinar cell tumors of the pancreas (4/4, 3/3, 2/2, and 3/3), 57.1% of AC (8/14) and 83.3% of signet ring cell carcinomas of the stomach (10/12), 56.2% AC of the bowel (9/17), bladder cancer (1/1). PDX-1 was negative in all anaplastic cancers (0/2) and solid pseudopapillary tumors of the pancreas (0/20), cancers of the lung (0/11), kidney (0/4), breast (0/3), and adrenal glands (0/2). CONCLUSION: The expression of PDX-1 is very specific for most digestive tract NETs and non-NETs. Pancreatic ductal and acinar cell tumors and gastric signet ring cell carcinomas are most commonly PDX-1-positive. Most tumors that do not originate from the digestive tract have a PDX-1 negative immunophenotype.


Assuntos
Proteínas de Homeodomínio/metabolismo , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Transativadores/metabolismo , Biomarcadores Tumorais/metabolismo , Humanos
15.
Int J Mol Sci ; 20(19)2019 Oct 08.
Artigo em Inglês | MEDLINE | ID: mdl-31597249

RESUMO

Despite being infrequent tumors, the incidence and prevalence of pancreatic neuroendocrine tumors (P-NETs) has been rising over the past few decades. In recent years, rigorous phase III clinical trials have been conducted, allowing the approval of several drugs that have become the standard of care in these patients. Although various treatments are used in clinical practice, including somatostatin analogues (SSAs), biological therapies like sunitinib or everolimus, peptide receptor radionuclide therapy (PRRT) or even chemotherapy, a consensus regarding the optimal sequence of treatment has not yet been reached. Notwithstanding, sunitinib is largely used in these patients after the promising results shown in SUN111 phase III clinical trial. However, both prompt progression as well as tumor recurrence after initial response have been reported, suggesting the existence of primary and acquired resistances to this antiangiogenic drug. In this review, we aim to summarize the most relevant mechanisms of angiogenesis resistance that are key contributors of tumor progression and dissemination. Furthermore, several targeted molecules acting selectively against these pathways have shown promising results in preclinical models, and preliminary results from ongoing clinical trials are awaited.


Assuntos
Inibidores da Angiogênese/uso terapêutico , Antineoplásicos/uso terapêutico , Terapia de Alvo Molecular , Neovascularização Patológica/tratamento farmacológico , Tumores Neuroendócrinos/tratamento farmacológico , Neoplasias Pancreáticas/tratamento farmacológico , Inibidores da Angiogênese/farmacologia , Animais , Antineoplásicos/farmacologia , Biomarcadores , Ensaios Clínicos como Assunto , Gerenciamento Clínico , Resistencia a Medicamentos Antineoplásicos/genética , Humanos , Gradação de Tumores , Neovascularização Patológica/etiologia , Neovascularização Patológica/metabolismo , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/etiologia , Tumores Neuroendócrinos/metabolismo , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/etiologia , Neoplasias Pancreáticas/metabolismo , Sunitinibe/farmacologia , Sunitinibe/uso terapêutico , Resultado do Tratamento
16.
Virchows Arch ; 475(6): 687-692, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31578606

RESUMO

The 2017 World Health Organization (WHO) classification proposes to type and subtype primary adenohypophyseal tumours according to their cell lineages with the aim to establish more uniform tumour groups. The definition of atypical adenoma was removed in favour of high-risk adenoma, and the assessment of proliferative activity and invasion was recommended to diagnose aggressive tumours. Recently, the International Pituitary Pathology Club proposed to replace adenoma with the term of pituitary neuroendocrine tumour (PitNET) to better reflect the similarities between adenohypophyseal and neuroendocrine tumours of other organs. The European Pituitary Pathology Group (EPPG) endorses this terminology and develops practical recommendations for standardised reports of PitNETs that are addressed to histo- and neuropathologists. This brief report presents the results of EPPG's consensus for the reporting of PitNETs and proposes a diagnostic algorithm.


Assuntos
Glucosiltransferases/metabolismo , Glicoproteínas/metabolismo , Tumores Neuroendócrinos/diagnóstico , Neoplasias Hipofisárias/diagnóstico , Neoplasias Hipofisárias/patologia , Consenso , Humanos , Tumores Neuroendócrinos/patologia , Sistemas Neurossecretores/patologia , Organização Mundial da Saúde
17.
Int J Comput Assist Radiol Surg ; 14(11): 1981-1991, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31555998

RESUMO

PURPOSE: The World Health Organization (WHO) grading system of pancreatic neuroendocrine tumor (PNET) plays an important role in the clinical decision. The rarity of PNET often negatively affects the radiological application of deep learning algorithms due to the low availability of radiological images. We tried to investigate the feasibility of predicting WHO grades of PNET on contrast-enhanced magnetic resonance (MR) images by deep learning algorithms. MATERIALS AND METHODS: Ninety-six patients with PNET underwent preoperative contrast-enhanced MR imaging. Fivefold cross-validation was used in which five iterations of training and validation were performed. Within every iteration, on the training set augmented by synthetic images generated from generative adversarial network (GAN), a convolutional neural network (CNN) was trained and its performance was evaluated on the paired internal validation set. Finally, the trained CNNs from cross-validation and their averaged counterpart were separately assessed on another ten patients from a different external validation set. RESULTS: Averaging the results across the five iterations in the cross-validation, for the CNN model, the average accuracy was 85.13% ± 0.44% and micro-average AUC was 0.9117 ± 0.0053. Evaluated on the external validation set, the average accuracy of the five trained CNNs ranges between 79.08 and 82.35%, and the range of micro-average AUC was between 0.8825 and 0.8932. The average accuracy and micro-average AUC of the averaged CNN were 81.05% and 0.8847, respectively. CONCLUSION: Synthetic images generated from GAN could be used to alleviate the difficulty of radiological image collection for uncommon disease like PNET. With the help of GAN, the CNN showed the potential to predict the WHO grades of PNET on contrast-enhanced MR images.


Assuntos
Meios de Contraste/farmacologia , Aprendizado Profundo , Imagem por Ressonância Magnética/métodos , Gradação de Tumores/métodos , Tumores Neuroendócrinos/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Algoritmos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Organização Mundial da Saúde
18.
Chirurgia (Bucur) ; 114(4): 461-466, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31511132

RESUMO

Background: Appendicular neuroendocrine neoplasms (ANEN) are rare tumours diagnosed incidentally on histology after an appendicectomy carried out for acute appendicitis. Five years survival rate of node-negative an early ANEN is over 90%. However, the growing interest, among clinicians, to treat appendicitis by antibiotics only, could potentially miss these indolent tumours. The aim of our study was to look at the incidence and management of ANEN discovered in appendicectomy specimens carried out for acute appendicitis. Methods: A retrospective review was performed for all patients undergone appendicectomy, at Barnsley hospital from 2009 to 2015, as suspected acute appendicitis, with particular focus on incidence, investigations and outcome of ANEN. Results: During 2009-2015, 1562 appendicectomies were carried out for acute appendicitis, with 11 specimens showed neuroendocrine tumours (0.70%). Mean age of patients was 31.73 years, with 81.8% female. 8 patients had laparoscopic appendicectomy (72.7%), 2 had open appendicectomy (18.2%), while one had laparoscopic converted to open appendicectomy. Tumour size was less 10 mm in 6 patients (54.5%), between 10-20 mm in 4 (36.4%). Majority were located at the body/base of appendix (54.5%). All tumours were grade 1 well differentiated tumours. 9 patients had clear resection margins, while two patients needed further surgery as an open right hemicolectomy as a curative procedure. In our series, 3 patients (27.27%) had follow up scans 2 years after surgery and showed no tumour recurrence. Conclusion: Appendicectomy is the curative procedure for the majority of ANEN. As shown in our study, if acute appendicitis is managed with conservative treatment only, there is less than 1% risk of missing the diagnosis of ANEN. Also, two of our patients had locally advanced diseases. These could definitely have a poor prognosis without ap-pendectomy.


Assuntos
Neoplasias do Apêndice/complicações , Neoplasias do Apêndice/diagnóstico , Apendicite/complicações , Apendicite/diagnóstico , Tumores Neuroendócrinos/complicações , Tumores Neuroendócrinos/diagnóstico , Doença Aguda , Adulto , Apendicectomia , Neoplasias do Apêndice/cirurgia , Apendicite/cirurgia , Apêndice/cirurgia , Erros de Diagnóstico , Feminino , Humanos , Laparoscopia , Masculino , Recidiva Local de Neoplasia , Tumores Neuroendócrinos/cirurgia , Estudos Retrospectivos
19.
Medicine (Baltimore) ; 98(37): e17154, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31517859

RESUMO

INTRODUCTION: Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are a heterogeneous group of epithelial neoplasms originating from the diffuse neuroendocrine cell system of the gastrointestinal tract and pancreas. They are very rare, especially in pediatric age, and vary widely in terms of clinical presentation, malignant potential, and prognosis. PATIENT CONCERNS: A 9 years' old, white female child presented with abdominal pain and diarrhea mixed with bright red blood lasting 2 days followed by hematemesis. DIAGNOSIS: Routine laboratory tests revealed microcytic anemia. Upper endoscopy showed a 20-mm polypoid lesion in the posterior wall of the duodenal bulb. Biopsy specimens were taken and histologic analysis showed a well-differentiated neuroendocrine tumor G1, with a ki-67 index <2%, an expression of chromogranine A (CgA), synaptophysin and somatostatin receptor type 2A (SSTR2A). Endoscopic ultrasound showed a 21-mm hypoechoic, hypervascular lesion involving the mucosal, submucosal, and muscular layers and a 15-mm hypoechoic round periduodenal lymph node. Gallium-68-somatostatin receptor positron emission tomography (PET with Ga-DOTATOC) showed one area of tracer uptake in the duodenum and other one near the duodenum compatible with the primary tumor site and a lymph node respectively. All the tests confirmed the diagnosis of a GEP-NET of the duodenal bulb, with a single lymph-node metastasis. INTERVENTIONS: The patient underwent an open duodenal wedge resection. OUTCOMES: The follow-up at 6, 24, and 36 months and then yearly after surgery for a total of 42 months showed no evidence of recurrence. CONCLUSION: Duodenal neuroendocrine tumors represent 1% to 3% of all GEP-NETs. They are rare in adults and extremely rare in children. Therefore, the diagnostic and therapeutic approach should be multidisciplinary, including laboratory, endoscopic, and specific imaging tests and strictly follows guidelines, to avoid misdiagnosis and inadequate treatments. Although the prognosis is benign in most cases, they can present with metastases. Therefore, a careful follow-up is extremely important.


Assuntos
Neoplasias Intestinais/diagnóstico , Neoplasias Intestinais/cirurgia , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/cirurgia , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/cirurgia , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/cirurgia , Criança , Diagnóstico Diferencial , Duodeno , Feminino , Humanos , Neoplasias Intestinais/patologia , Metástase Linfática/diagnóstico , Tumores Neuroendócrinos/patologia , Neoplasias Pancreáticas/patologia , Neoplasias Gástricas/patologia
20.
Cir Cir ; 87(S1): 17-21, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31501629

RESUMO

Introduction: Patients with inflammatory bowel disease (IBD) have a higher risk of developing gastrointestinal tumors, the adenocarcinoma is the most frequently associated, and neuroendocrine tumor (NET) the most rare. Clinical cases: We present two patients, one with Crohn's disease and the other with ulcerative colitis, who present nonspecific symptoms, and after resection of an intestinal lesion, a gastrointestinal NET (GINET) is diagnosed. Discussion and conclusion: The GINET have an insidious clinic and these can be confused with those of the IBD. There could be an association between both pathologies; an important role of the chronic intestinal inflammatory process is suggested. The best treatment for GINET is the resection.


Assuntos
Colite Ulcerativa/complicações , Doença de Crohn/complicações , Neoplasias do Íleo/diagnóstico , Tumores Neuroendócrinos/diagnóstico , Neoplasias Retais/diagnóstico , Idoso , Carcinoma de Células Renais , Colite Ulcerativa/tratamento farmacológico , Colite Ulcerativa/cirurgia , Pólipos do Colo/complicações , Pólipos do Colo/tratamento farmacológico , Colonoscopia , Doença de Crohn/tratamento farmacológico , Doença de Crohn/cirurgia , Diagnóstico Tardio , Diagnóstico Diferencial , Suscetibilidade a Doenças , Humanos , Neoplasias do Íleo/complicações , Neoplasias do Íleo/cirurgia , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Achados Incidentais , Inflamação , Neoplasias Renais , Masculino , Pessoa de Meia-Idade , Segunda Neoplasia Primária/complicações , Segunda Neoplasia Primária/diagnóstico , Tumores Neuroendócrinos/complicações , Tumores Neuroendócrinos/cirurgia , Neoplasias Retais/complicações , Neoplasias Retais/cirurgia
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