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1.
Orv Hetil ; 161(8): 313-319, 2020 Feb.
Artigo em Húngaro | MEDLINE | ID: mdl-32073294

RESUMO

Small-cell lung carcinoma (SCLC) and the rare large-cell neuroendocrine carcinoma belong to the high grade pulmonary neuroendocrine carcinomas. Making the correct diagnosis and selection of treatment modalities require multidisciplinary meetings due to the morphological overlaps, aggressive behaviour and debated therapeutic guidelines of these entities. A 52-year-old woman was admitted to the hospital because of headache, nausea and tenebrous vision. The CT revealed metastatic tumour mass in the occipital lobe and in the cerebellum. Both tumours were removed and resulted in histological diagnosis of metastatic neuroendocrine carcinoma. Chest X-ray established contrast-enhancing lesion in the left lung. Bronchoscopy was performed and histological examination revealed large-cell neuroendocrine carcinoma. Postoperative skull irradiation and small-cell lung cancer chemotherapy protocol were utilized. Due to atelectasis and progression, chest irradiation was initiated, which was interrupted because of novel brain metastases. Further chemotherapy followed the non-small-cell lung cancer protocol. After 3 months, thoracic progression, brain and disseminated bone metastases were diagnosed. After a 14-month-long therapy, the patient deceased. Large-cell neuroendocrine carcinoma has a poor prognosis, the incidence of brain metastasis is 25-50%. In early stage large-cell neuroendocrine carcinoma, lobectomy is the standard treatment and adjuvant chemotherapy should also be considered. Although the non-small-cell lung cancer chemotherapy protocol is approved widely in the treatment of large-cell neuroendocrine carcinoma, the utility of SCLC scheme has also been suggested. Orv Hetil. 2020; 161(8): 313-319.


Assuntos
Carcinoma de Células Grandes/diagnóstico , Carcinoma de Células Grandes/tratamento farmacológico , Carcinoma Neuroendócrino/diagnóstico , Carcinoma Neuroendócrino/tratamento farmacológico , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica , Quimioterapia Adjuvante , Feminino , Humanos , Pessoa de Meia-Idade
2.
Ann R Coll Surg Engl ; 102(2): e33-e35, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31530165

RESUMO

Spindle epithelial tumour with thymus-like differentiation (SETTLE) is a very rare neck malignancy arising from thymic tissue or branchial pouch, mostly seen in the early decades of life. SETTLE is thought to be a low-grade neoplasm but distant metastases have been reported. Fewer than 50 cases have been reported in the PubMed database. We report on the four-year follow-up for a 13-year-old boy, with no signs of recurrence or distant metastasis. In addition, a 45-year-old woman was admitted to hospital with neck mass. Fine-needle aspiration biopsy was compatible with medullary thyroid carcinoma but serum levels of calcitonin were normal. Total thyroidectomy and central compartment lymph node dissection was planned with an initial diagnosis of medullary thyroid carcinoma. At surgery, frozen section analysis converted our diagnosis to SETTLE and the intraoperative strategy was changed. SETTLE should be considered in a patient with normal serum calcitonin levels in the presence of suspicious medullary thyroid carcinoma at fine-needle aspiration biopsy. Frozen section analysis may help to manage the intraoperative strategy. This report describes the first case of SETTLE successfully diagnosed by frozen section analysis.


Assuntos
Neoplasias Embrionárias de Células Germinativas/diagnóstico , Neoplasias da Glândula Tireoide/diagnóstico , Adolescente , Biomarcadores Tumorais/sangue , Biópsia por Agulha Fina , Calcitonina/sangue , Carcinoma Neuroendócrino/diagnóstico , Diferenciação Celular , Diagnóstico Diferencial , Feminino , Seguimentos , Secções Congeladas , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Embrionárias de Células Germinativas/patologia , Neoplasias Embrionárias de Células Germinativas/cirurgia , Neoplasias da Glândula Tireoide/patologia , Neoplasias da Glândula Tireoide/cirurgia , Tireoidectomia/métodos
3.
Ann R Coll Surg Engl ; 102(3): e63-e66, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31841033

RESUMO

Calcitonin-negative medullary thyroid carcinoma is a rare, poorly understood primary neuroendocrine carcinoma of the thyroid characterised by classic medullary thyroid carcinoma morphology without raised serum calcitonin. A 24-year-old woman presented with a slow-growing, right-sided neck swelling. She underwent an ultrasound scan, cytopathological and histopathological examination, and tests for alternative diagnoses. The ultrasound showed a heterogeneous, hyperechoic nodule in the right thyroid lobe. Serum calcitonin was normal. Cytopathology and histopathology showed typical medullary thyroid carcinoma morphology but without calcitonin upon immunostaining and mRNA in situ hybridisation. A 'triple-negative' calcitonin-negative medullary thyroid carcinoma was diagnosed. A completion thyroidectomy with bilateral central lymph node dissection was performed. The patient remains well three-years post-surgery. When cytopathology suggests a medullary thyroid carcinoma, serum calcitonin, pro-calcitonin, carcinoembryonic antigen and calcitonin-gene-related peptide should be measured to identify cases of calcitonin-negative medullary thyroid carcinoma. They should also be measured post-treatment for monitoring purposes. This will aid future calcitonin-negative medullary thyroid carcinoma diagnoses and will inform prognostic stratification and influence treatment decisions.


Assuntos
Calcitonina/sangue , Carcinoma Neuroendócrino/sangue , Glândula Tireoide/diagnóstico por imagem , Neoplasias da Glândula Tireoide/sangue , Biomarcadores Tumorais/sangue , Biópsia , Carcinoma Neuroendócrino/diagnóstico , Carcinoma Neuroendócrino/cirurgia , Feminino , Humanos , Fenótipo , Glândula Tireoide/cirurgia , Neoplasias da Glândula Tireoide/diagnóstico , Neoplasias da Glândula Tireoide/cirurgia , Tireoidectomia/métodos , Tomografia Computadorizada por Raios X , Ultrassonografia , Adulto Jovem
4.
BMC Neurol ; 19(1): 304, 2019 Nov 29.
Artigo em Inglês | MEDLINE | ID: mdl-31783737

RESUMO

BACKGROUND: Paraneoplastic limbic encephalitis (PLE) is a rare autoimmune neurological syndrome observed in cancer patients. PLE is difficult to diagnose and presents a variable response to treatment, depending on the characteristics of the tumor and neuronal autoantibodies. CASE PRESENTATION: A 64-year-old, Caucasian, non-smoker man presented with a rapidly developing cognitive impairment, personality change, spatial disorientation, and short-term memory loss associated with anorexia and cervical and inguinal lymph nodes. The 18F-FDG PET scan documented intensely hypermetabolic lymph nodes, which histologically corresponded to a metastasis from a small cell neuroendocrine carcinoma. The brain MRI revealed a high T2-weighted FLAIR signal of the hippocamps, consisted with a PLE. The presence of anti-neuronal Hu antibodies confirmed the diagnosis. The patient underwent plasmapheresis, associated to a systemic chemotherapy resulting in a partial and temporary improvement of the neurological symptoms. Four cycles of intravenous immunoglobulins were also necessary. After six cures of chemotherapy, the lymph node metastases regressed. However, a new anorectal lesion was detected and was histologically confirmed as a primary small cell neuroendocrine carcinoma, which was treated with concomitant chemoradiotherapy. At the end of this treatment, the patient showed a rapid tumor progression leading to his death. CONCLUSIONS: This case highlights the rare entity, PLE, which is difficult to diagnose and manage. In addition, this is the first published case of PLE associated with an anorectal small cell neuroendocrine carcinoma, which appeared after completion of systemic chemotherapy.


Assuntos
Carcinoma Neuroendócrino/diagnóstico , Carcinoma de Células Pequenas/diagnóstico , Encefalite Límbica/diagnóstico , Autoanticorpos , Fluordesoxiglucose F18 , Hipocampo/patologia , Humanos , Encefalite Límbica/imunologia , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Tomografia por Emissão de Pósitrons
5.
J Ovarian Res ; 12(1): 95, 2019 Oct 15.
Artigo em Inglês | MEDLINE | ID: mdl-31615543

RESUMO

BACKGROUND: Large cell neuroendocrine carcinoma is a very rare ovarian neoplasm that has a poor clinical outcome even in the early stage, and there is as yet no established treatment. Diagnostic laparoscopy has been used to determine the possibility of primary optimal cytoreductive surgery or neoadjuvant chemotherapy in patients with advanced epithelial ovarian cancer. However, the role of diagnostic laparoscopy is still unclear in large cell neuroendocrine carcinoma due to its rarity. CASE PRESENTATION: A 31-year-old woman with abdominal distention was referred to our hospital. She was strongly suspected of having advanced ovarian cancer because of a huge pelvic mass, massive ascites, and their appearance on medical imaging. However, cytological examinations from ascitic fluid by abdominal paracentesis did not show any malignant cells. She underwent diagnostic laparoscopy to evaluate the possibility of primary optimal cytoreductive surgery, and only tissue sampling was performed for pathological diagnosis because of the countless disseminated lesions of various sizes in the intraperitoneal organs. The patient had no postoperative complications, leading to the early start of postoperative chemotherapy. CONCLUSIONS: To date, there have been no systematic reviews that focused on determining the treatment strategy using laparoscopy. Diagnostic laparoscopy can be helpful to determine the optimal treatment, including primary debulking surgery, neoadjuvant chemotherapy, or best supportive care, assisting in decision-making particularly for patients with advanced large cell neuroendocrine carcinoma with carcinomatous peritonitis.


Assuntos
Abdome/patologia , Carcinoma de Células Grandes/diagnóstico , Carcinoma Neuroendócrino/diagnóstico , Ovário/patologia , Adulto , Líquido Ascítico/patologia , Carcinoma de Células Grandes/patologia , Carcinoma de Células Grandes/cirurgia , Carcinoma Neuroendócrino/patologia , Carcinoma Neuroendócrino/cirurgia , Procedimentos Cirúrgicos de Citorredução , Feminino , Humanos , Ovário/diagnóstico por imagem , Paracentese/métodos , Peritonite/diagnóstico , Peritonite/patologia
6.
BMC Surg ; 19(1): 135, 2019 Sep 12.
Artigo em Inglês | MEDLINE | ID: mdl-31510991

RESUMO

BACKGROUND: Poorly differentiated neuroendocrine carcinomas (NECs) originating from the gastrointestinal (GI) tract are rare and very highly malignant disease with a poor prognosis. Poorly differentiated NECs most commonly arise in the esophagus and the large bowel; however, they may occur within virtually any portion of the GI tract. It is known, however, that they do not typically occur in the small intestine. CASE REPORT: A 21-year-old woman visited an emergency room with acute abdominal pain that commenced 2 days prior to her presentation. Thereafter, a computed tomography (CT) scan was notable for a small-intestine perforation, and huge masses were observed in the small intestine and the mesentery. The mass that was located at the ileum site is approximately 100 cm above the ileocecal (IC) valve, and while it is located on the anti-mesenteric border and it seems that luminal narrowing had occurred, an obstruction is absent. Also, a same-nature mass is on the mesentery. The pathologic reports confirmed a small-cell-type NEC with a mass size of 7.5 × 6.5 cm. The mitotic count is up to 24/10 high-power fields (HPFs), the results of the immunohistochemical stain are positive for CD56 and synaptophysin, and the Ki-67 level is 50%. %. After the operation, she was treated with Etoposide-Cisplatin (EP) chemotheraphy. Stable disease was seen during Etoposide-Cisplatin chemotheraphy. Liver metastasis was also confirmed after chemotheraphy. Additionally, Irinotecan and cisplatin were used for 3 cycles, but progression of disease, neutropenic fever, thrombocytopenia, general weakness persisted. Eventually, she died 1 year and 6 months after surgery. CONCLUSION: Ileum-located NECs are diagnosed very rarely. The most common locations for these tumors along the GI tract are the esophagus and the large intestine, but they can arise anywhere. The prognosis for NECs is poor due to the metastatic disease of most patients at the time of diagnosis. The role of adjuvant treatment requires further evaluation for the attainment of a better understanding of the overall treatment effect.


Assuntos
Carcinoma Neuroendócrino/diagnóstico , Carcinoma de Células Pequenas/diagnóstico , Neoplasias Hepáticas/secundário , Carcinoma Neuroendócrino/patologia , Feminino , Humanos , Íleo/patologia , Prognóstico , Tomografia Computadorizada por Raios X , Adulto Jovem
7.
Medicina (B Aires) ; 79(4): 271-275, 2019.
Artigo em Espanhol | MEDLINE | ID: mdl-31487246

RESUMO

There is much controversy about the benefits of the use of serum calcitonin (CT) in the initial evaluation of patients with thyroid nodules. The objective of the study was to early identify medullary thyroid carcinoma (MTC) through the routine measurement of CT in thyroid nodular pathology in a large cohort of patients from Buenos Aires, Argentina. Consecutive patients with nodular thyroid disease (n=1017) were studied. CT was measured by chemiluminescence, normal value: up to 18 pg/ml in men and 12 pg/ml in women. In two patients, hypercalcitoninemia was confirmed in repeated measurements. Fine needle aspiration with CT measurement in the needle wash fluid identified MTC in nodules with citology abnormalities. The genetic study was positive in one patient (mutation exon 14, Val804Met, MTC familiar). The other presented a polymorphism (exon 13 L769L heterozygous - exon 15 S904S heterozygous). In both cases, CT was normalized 3 months after surgery and remained normal after 6 years of follow-up. The routine measurement of CT in thyroid nodular pathology was useful to detect two cases of MTC, one of them sporadic and the other familiar in this cohort. The prevalence of MTC was 0.2%.


Assuntos
Calcitonina/sangue , Carcinoma Neuroendócrino/diagnóstico , Neoplasias da Glândula Tireoide/diagnóstico , Nódulo da Glândula Tireoide/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores/sangue , Biópsia por Agulha Fina , Carcinoma Neuroendócrino/sangue , Carcinoma Neuroendócrino/patologia , Estudos de Coortes , Diagnóstico Precoce , Feminino , Humanos , Imuno-Histoquímica , Luminescência , Masculino , Pessoa de Meia-Idade , Sensibilidade e Especificidade , Neoplasias da Glândula Tireoide/sangue , Neoplasias da Glândula Tireoide/patologia , Nódulo da Glândula Tireoide/sangue , Adulto Jovem
8.
BMJ Case Rep ; 12(7)2019 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-31371331

RESUMO

The ureter is an extremely rare site for small cell neuroendocrine carcinoma. We present a case of this disease in a patient who presented without urological symptoms. The multidisciplinary team proposed nephroureterectomy (if fit) or watchful waiting as management. After discussion with the patient a decision in favour of watchful waiting was made. We report her case including a review of the literature, and emphasise that although small cell neuroendocrine carcinomas can be very aggressive, they can remain asymptomatic.


Assuntos
Carcinoma Neuroendócrino/diagnóstico , Carcinoma de Células Pequenas/diagnóstico , Sintomas do Trato Urinário Inferior/diagnóstico , Neoplasias Ureterais/diagnóstico , Idoso de 80 Anos ou mais , Carcinoma Neuroendócrino/complicações , Carcinoma de Células Pequenas/complicações , Feminino , Humanos , Achados Incidentais , Sintomas do Trato Urinário Inferior/etiologia , Ureter/patologia , Neoplasias Ureterais/complicações
9.
J Ovarian Res ; 12(1): 69, 2019 Jul 25.
Artigo em Inglês | MEDLINE | ID: mdl-31345245

RESUMO

BACKGROUND: There is no consensus on the optimal chemotherapy regimen and the prognostic factors for ovarian large cell neuroendocrine carcinoma (LCNEC), a rare type of tumor. The objective of the present study is to present the case of a recent encounter of pure ovarian LCNEC and perform a brief review to summarize the clinicopathological features and prognostic factors of 57 cases of LCNEC patients that have been previously reported. METHOD: CASE PRESENTATION: Eligible studies were searched for online and 57 cases with clear follow-up data were found to have been reported. We present the 58th case, which is of a 70-year-old woman with stage IIIc primary pure LCNEC of the ovary. The initial symptom of this patient was abdominal distension (more than 2 months). A recent ultrasound test showed a solid-cystic mass occupying the pelvic and abdominal cavity. She received two courses of cisplatin-etoposide chemotherapy as an adjuvant therapy. No signs of nonclinical or radiological evidence of disease recurrence was found at follow-up examinations during the first 3 months after operation. A retrospective review of these 58 cases was conducted and survival curves were estimated. Using the Kaplan-Meier method. CONCLUSION: The patients included were aged between 18 and 80 years. A Kaplan-Meier survival curve revealed that the median overall survival was 10.000 months, while 26 (44.83%) patients died within 12 months. We compared the overall mean survival time of all patients with that of stage I patients (42.418 vs 42.047 months), which suggests that ovarian LCNEC has a very poor prognosis even at stage I. Mean survival was longer for patients who had undergone postoperative chemotherapy than for those without postoperative chemotherapy (48.082 vs 9.778 months). A small series, such as this, does not provide adequate data to establish a firm correlation between the postoperative chemotherapy and prognosis (p = 0.176). In our review of 58 cases with ovarian LCNEC, prognosis was unfavorable in most cases. Given the rarity of LCNEC, it is highly recommended that a global medical database of ovarian LCNEC and a standard system of diagnosis and treatment is established.


Assuntos
Carcinoma de Células Grandes/diagnóstico , Carcinoma Neuroendócrino/diagnóstico , Neoplasias Ovarianas/diagnóstico , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Carcinoma de Células Grandes/mortalidade , Carcinoma de Células Grandes/terapia , Carcinoma Neuroendócrino/mortalidade , Carcinoma Neuroendócrino/terapia , Quimioterapia Adjuvante , Diagnóstico por Imagem/métodos , Feminino , Humanos , Estimativa de Kaplan-Meier , Gradação de Tumores , Estadiamento de Neoplasias , Neoplasias Ovarianas/mortalidade , Neoplasias Ovarianas/terapia , Avaliação de Sintomas , Resultado do Tratamento
10.
Diagn Pathol ; 14(1): 64, 2019 Jun 22.
Artigo em Inglês | MEDLINE | ID: mdl-31228945

RESUMO

BACKGROUND: Primary grade 2 neuroendocrine tumors of the cervix in female patients are rare and have a highly aggressive clinical course. This study is aimed to analyse the diagnosis, genetic changes, management and prognosis of these tumors and investigate whether the genetic alterations could provide more useful information to guide the molecular characterization and potential individualized treatment of grade 2 cervical neuroendocrine tumors. METHODS: The clinical records of all three patients diagnosed as primary grade 2 neuroendocrine tumors of the cervix in Peking Union Medical College Hospital (PUMCH) from 2011 to 2018 were reviewed retrospectively. We investigated the morphology, immunophenotype and molecular abnormalities of all the cases. The follow-up data were also collected. RESULTS: The age of the patients ranged from 46 to 69 years. All cases were in stage II and treated with surgery. The microscopic examination showed that the tumors took the form of nest-like, trabecular, sheet-like, "single file" strands or rosette-like structures. The mitotic figures ranged from 2 to 5 in every 10 high-power fields, and necrotic foci were observed in one case. Immunohistochemically, the tumor cells were positive for AE1/AE3, Cg A, Syn, CD56, P16, CAM5.2, and PGP9.5 and negative for ER, PR, P63, P40, CK7, and CK20. The expression of P53 showed as normal/wild-type pattern, and the proliferation index of Ki-67 ranged from 2 to 7%. A total of 560 genes were sequenced by next-generation sequencing for each patient, and nonsynonymous somatic mutations were identified in the three cases. Non-frameshift insertions of the MAGI1 and SLC45A were both observed in case 1, while we only observed the non-frameshift insertion of the MAGI1 in case 2 and the non-frameshift insertion of the SLC45A in case 3. Case 1 was treated with chemoradiotherapy before and after surgery. Cases 2 and 3 were treated with chemotherapy before and after surgery. The follow-up time ranged from 27 to 74 months. Cases 2 and 3 survived, while case 1 died. CONCLUSION: Cervical grade 2 neuroendocrine tumors are extremely rare. We presented the first mutation profile revealed by whole exome sequencing in a series of grade 2 cervical NETs along with their clinicopathological characteristics. Their genetic changes are different from those that take place in the gastrointestinal tract, pancreas and lung, which have gene changes in VEGF, RTKs or the mTOR signalling pathway. While changes in MAGI1 and SLC45L3 were observed in two of our cases and the case who had the gene changes of both MAGI1 and SLC45L3 died because of metastases to the liver and bone. The genetic alterations may provide more useful information to guide the molecular characterization and potential individualized treatment of grade 2 cervical neuroendocrine tumors.


Assuntos
Carcinoma Neuroendócrino/patologia , Colo do Útero/patologia , Tumores Neuroendócrinos/patologia , Neoplasias do Colo do Útero/patologia , Adulto , Biomarcadores Tumorais/análise , Carcinoma Neuroendócrino/diagnóstico , Feminino , Humanos , Imuno-Histoquímica/métodos , Pessoa de Meia-Idade , Tumores Neuroendócrinos/diagnóstico , Prognóstico , Neoplasias do Colo do Útero/diagnóstico
11.
Rinsho Shinkeigaku ; 59(7): 442-447, 2019 Jul 31.
Artigo em Japonês | MEDLINE | ID: mdl-31243254

RESUMO

A 69-year-old man presented with a history of personality change for several years. He was admitted to our hospital due to partial seizure. A cerebrospinal fluid test and an electroencephalogram showed no specific abnormalities, but brain magnetic resonance imaging revealed abnormal findings in the right temporal pole, bilateral amygdala to hippocampus, and insular cortex. He was diagnosed with limbic encephalitis accompanied by partial seizure, and received infusion of an antiepileptic agent and acyclovir. Additional examinations for malignancy and autoimmune disease were performed, and neck CT and MRI revealed a neck tumor. Neck lymph node biopsy suggested lymph node metastasis of a neuroendocrine neoplasm derived from other organs. He did not want aggressive treatment involving surgical resection and chemotherapy, and thus, conservative treatment was chosen by an otorhinolaryngologist and immunotherapy was not used. After discharge, the neck tumor grew gradually. To manage the focal mass effect, chemotherapy and surgical resection followed by chemoradiotherapy were performed by the otorhinolaryngologist on days 244 and 325 of the disease course, respectively. Histology of resected tissues disclosed neck neuroendocrine carcinoma derived from a submandibular gland. His personality change improved temporarily after surgical resection, but then worsened again with regrowth of the tumor. He died on day 723. After death, a blood test revealed the presence of anti-amphiphysin antibody. This case suggests that neck neuroendocrine carcinoma can induce paraneoplastic limbic encephalitis, and in such cases, early surgical resection of the neck tumor with suspected lymph node metastasis is necessary both to control symptoms associated with encephalitis and to exclude carcinoma derived from the neck itself.


Assuntos
Carcinoma Neuroendócrino/complicações , Encefalite Límbica/etiologia , Neoplasias da Glândula Submandibular/complicações , Idoso , Autoanticorpos/sangue , Biomarcadores Tumorais/sangue , Carcinoma Neuroendócrino/diagnóstico , Carcinoma Neuroendócrino/patologia , Carcinoma Neuroendócrino/terapia , Terapia Combinada , Evolução Fatal , Humanos , Metástase Linfática , Proteínas do Tecido Nervoso/imunologia , Neoplasias da Glândula Submandibular/diagnóstico , Neoplasias da Glândula Submandibular/patologia , Neoplasias da Glândula Submandibular/terapia
12.
Endocr Relat Cancer ; 26(9): R499-R518, 2019 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-31252403

RESUMO

Medullary thyroid carcinoma (MTC) is a rare type of tumor that originates from thyroid C cells and accounts for 2-4% of all malignant thyroid neoplasms. MTC may occur sporadically or be inherited, as part of the MEN 2 syndrome. Germline mutations of the RET (REarranged during Transfection) proto-oncogene cause hereditary cancer, whereas somatic RET mutations and, less frequently, RAS mutations have been described in sporadic MTC samples. Since early surgery with complete resection of tumor mostly determines the likelihood of attaining cure for MTC, the broader use of RET genetic screening has dramatically changed the prognostic of gene carriers in hereditary MTC. Nevertheless, despite recent advances, the management of advanced, progressive MTC remains challenging. The multikinase inhibitors (MKI), vandetanib and cabozantinib, were approved for the treatment of progressive or symptomatic MTC, and several other compounds have exhibited variable efficacy. Although these drugs have been shown to improve progression-free survival, no MKI has been shown to increase the overall survival. As these drugs are nonselective, significant off-target toxicities may occur, limiting achievement of the required TK-specific inhibition. Recently, next-generation small-molecule TKI has been developed. These TKI are specifically designed for highly potent and selective targeting of oncogenic RET alterations, making them promising drugs for the treatment of advanced MTC. Here, we summarize the current understanding of the intracellular signaling pathways involved in MTC pathogenesis as well as the therapeutic approaches and challenges for the management of advanced MTC, focusing on targeted molecular therapies.


Assuntos
Carcinoma Neuroendócrino , Neoplasias da Glândula Tireoide , Antineoplásicos/uso terapêutico , Carcinoma Neuroendócrino/diagnóstico , Carcinoma Neuroendócrino/genética , Carcinoma Neuroendócrino/metabolismo , Carcinoma Neuroendócrino/terapia , Genótipo , Humanos , Imunoterapia , Terapia de Alvo Molecular , Fenótipo , Inibidores de Proteínas Quinases/uso terapêutico , Transdução de Sinais , Neoplasias da Glândula Tireoide/diagnóstico , Neoplasias da Glândula Tireoide/genética , Neoplasias da Glândula Tireoide/metabolismo , Neoplasias da Glândula Tireoide/terapia
13.
Interact Cardiovasc Thorac Surg ; 29(4): 517-524, 2019 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-31177277

RESUMO

OBJECTIVES: Sublobar resection (SLR) for early non-small-cell lung carcinoma (NSCLC) has been shown to have a survival rate similar to that of lobectomy. Large-cell neuroendocrine carcinoma (LCNEC) of the lung, although treated like an NSCLC, has a poor prognosis compared to NSCLC. We sought to determine if outcomes are poor in patients with early stage LCNEC treated with SLR versus lobectomy. METHODS: We searched for patients with pathological stage I LCNEC ≤3 cm within the National Cancer Database between 2004 and 2014. Propensity score matching was used to compare the 5-year overall survival rate of patients having SLR (wedge or segmentectomy) to that of patients having a lobectomy. Patients were matched for age, node sampling, comorbidity score, tumour size, insurance status and other factors. Patients who received neoadjuvant therapy were excluded. Kaplan-Meier methods were used for analysis. RESULTS: A total of 1011 patients met the inclusion criteria: 263 were treated with SLR (223 wedges and 40 segmentectomies) and 748 patients, with lobectomy. Patients who received SLR were older, had more comorbidities and smaller tumours. On unadjusted Kaplan-Meier analysis, patients who had SLR had decreased 5-year overall survival compared to those who had a lobectomy (37.9% vs 56.6%, P < 0.001). Propensity score matching (1:1) across 12 demographic and tumour variables yielded 185 patients per group with 34 segmentectomies and 151 wedge resections in the SLR cohort. On Kaplan-Meier analysis of the matched cohort, patients who had SLR had a worse 5-year overall survival rate compared to those who had a lobectomy (41.5% vs 60.3%; P = 0.001). CONCLUSIONS: SLR for early stage LCNEC is associated with a lower 5-year overall survival rate compared to lobectomy on unadjusted and propensity matched analyses.


Assuntos
Carcinoma Neuroendócrino/cirurgia , Neoplasias Pulmonares/cirurgia , Estadiamento de Neoplasias , Pneumonectomia/métodos , Idoso , Carcinoma Neuroendócrino/diagnóstico , Estudos de Coortes , Feminino , Humanos , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/mortalidade , Masculino , Pessoa de Meia-Idade , Taxa de Sobrevida/tendências , Estados Unidos/epidemiologia
14.
J Dermatol ; 46(8): 720-723, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31206216

RESUMO

A 61-year-old Japanese man had a gradually growing, red-colored nodule in his umbilicus from 3 years ago. He had no symptoms such as hot flush, diarrhea or wheezing. Computed tomography detected a 3.0 cm × 3.0 cm enhanced nodule on the umbilical portion, inguinal and axillary lymph node swelling, and peritoneal nodules. Upper and lower gastrointestinal endoscopy and cystoscopy did not show any other tumor. We resected the umbilical nodule and subjected the inguinal lymph node and peritoneal nodule to biopsy. Histopathological findings indicated that the cutaneous lesion was composed of variously sized nests that included small, monomorphic, round to polygonal cells, mainly in the dermis to the peritoneum. Mitotic figures were scant. The inguinal lymph node and peritoneal nodule were positive for metastasis. Immunohistochemistry was diffusely positive for cytokeratin (CK)-7, CD56, chromogranin A, synaptophysin, estrogen receptor-α, progesterone receptor, GATA3 and carcinoembryonic antigen, and focally positive for mammaglobin and gross cystic disease fluid protein 15. The Ki-67 labeling index was 1.5%. The patient was diagnosed with a case of low-grade neuroendocrine carcinoma of the skin (LGNECS) occurring on the umbilicus. This case exhibited distant peritoneal metastasis, as well as inguinal and axillary lymph node metastases; however, the patient is alive without chemotherapy at 23 months after the first visit. LGNECS is a newly proposed, extremely rare entity that has been reported under various names, including primary cutaneous carcinoid tumor. In the present case, this tumor shows a slow-growing nature and favorable prognosis, even though it harbors metastatic potential.


Assuntos
Carcinoma Neuroendócrino/diagnóstico , Metástase Linfática/diagnóstico , Neoplasias Peritoneais/diagnóstico , Neoplasias Cutâneas/patologia , Biópsia , Carcinoma Neuroendócrino/secundário , Humanos , Linfonodos/diagnóstico por imagem , Linfonodos/patologia , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Neoplasias Peritoneais/secundário , Peritônio/diagnóstico por imagem , Peritônio/patologia , Pele/patologia , Neoplasias Cutâneas/cirurgia , Umbigo
15.
Int J Surg Pathol ; 27(7): 744-752, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31195855

RESUMO

We aimed to determine the histopathological differences between primary breast carcinomas with neuroendocrine features (NEBC) and carcinomas mimicking neuroendocrine features (NEBC-like). Twenty-three cases with NEBC, all showing positive staining for synaptophysin and/or chromogranin-A in ≥50% of tumor cells and 36 cases with NEBC-like (no staining for neuroendocrine [NE] markers but suspicious for NE morphology in terms of solid/trabecular growth patterns) were included in the study. Significant differences were found between the groups in terms of the patients' ages, histologic/nuclear grade of tumor, lymphovascular invasion, comedo-type ductal carcinoma in situ (DCIS), microcalcification, Ki-67 proliferation index, nuclear shape, and level of peritumoral lymphocytic infiltration. The presence of large-size solid cohesive groups of tumor cells; plasmocytoid, spindle, and/or columnar shapes of tumor cells; and eosinophilic-granular appearance of cytoplasm were mostly noted in the NEBC group. The presence of small- to medium-sized solid cohesive groups of tumor cells; high-grade histologic and nuclear features; clear cytoplasm; and round to ovoid nucleus were mostly noted in the NEBC-like group. No significant differences were found in terms of tumor size, ER/PR/HER2 status, as well as the presence of DCIS, elastosis, extracellular/intracellular mucin, signet ring cells, apocrine features, and accompanying papilloma or ductal ectasia. In conclusion, small- to medium-sized solid cohesive groups of tumor cells, high-grade features, clear cytoplasm, round to ovoid shape of nucleus, lymphovascular invasion, comedo-type DCIS, microcalcification, high level of Ki-67 proliferation index (≥20%), and moderate/strong level of peritumoral lymphocytic infiltration might support non-NE features in breast carcinomas.


Assuntos
Neoplasias da Mama/diagnóstico , Mama/patologia , Carcinoma Intraductal não Infiltrante/diagnóstico , Carcinoma Neuroendócrino/diagnóstico , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Neoplasias da Mama/patologia , Carcinoma Intraductal não Infiltrante/patologia , Carcinoma Neuroendócrino/patologia , Proliferação de Células , Cromogranina A/análise , Diagnóstico Diferencial , Feminino , Humanos , Imuno-Histoquímica , Antígeno Ki-67/análise , Pessoa de Meia-Idade , Gradação de Tumores , Invasividade Neoplásica/patologia , Sinaptofisina/análise
16.
Gan To Kagaku Ryoho ; 46(4): 727-729, 2019 Apr.
Artigo em Japonês | MEDLINE | ID: mdl-31164516

RESUMO

We report a rare case ofrectal neuroendocrine carcinoma(NEC)following sigmoidectomy of sigmoid colon cancer. NEC of the rectum is a rare disease. It has an extremely poor prognosis and a high potential for malignancy with hematogenous and lymph node metastases. A 90-year-old man who had carcinoma ofthe sigmoid colon 2 years ago was found to have rectal NEC based on endoscopy findings. He underwent the Hartmann operation. Histological and immunohistochemical analyses showed NEC. Four months after the surgery, he developed local recurrence with lymph node metastasis. He was not administered chemotherapy because ofhis old age. Although the patient needed strict outpatient care, he remained symptom-free 4 months after the Hartmann operation.


Assuntos
Carcinoma Neuroendócrino , Neoplasias Retais , Neoplasias do Colo Sigmoide , Idoso de 80 Anos ou mais , Carcinoma Neuroendócrino/diagnóstico , Carcinoma Neuroendócrino/cirurgia , Humanos , Masculino , Recidiva Local de Neoplasia , Neoplasias Retais/diagnóstico , Neoplasias Retais/cirurgia , Reto , Neoplasias do Colo Sigmoide/diagnóstico , Neoplasias do Colo Sigmoide/cirurgia
17.
Diagn Cytopathol ; 47(9): 943-947, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31190466

RESUMO

Medullary carcinoma of thyroid (MCT) is a neuroendocrine neoplasm derived from the parafollicular cells or C cells. It constitutes 5% of thyroid carcinomas. We present a case of 36-year-old female with a left lower lobe thyroid swelling since 8 to 9 years. Swelling was smooth, nontender, and moving with deglutition. Contrast-enhanced computed tomography (CECT) findings were suggestive of malignant thyroid nodule. Fine-needle aspiration cytology (FNAC) smears were highly cellular comprising predominantly Hurthle cells arranged in sheets, clusters, and follicles with few singly scattered cells. Background was hemorrhagic with scant colloids. An impression of follicular neoplasm or suspicious of follicular neoplasm was given with an additional note mentioning that the possibility of Hurthle cell neoplasm could not be excluded. Left hemithyroidectomy was done and sections showed a well-circumscribed tumor suggestive of MCT, which was confirmed based on immunohistochemistry for calcitonin and carcinoembryonic antigen. It is difficult to differentiate MCT from Hurthle cell neoplasm. There may be cellular pleomorphism within a single aspirate, but the presence of mixed cell population is a diagnostic pointer for MCT. In the presence of predominant Hurthle cell population, distinguishing these entities by cytomorphology alone is difficult. Hence, clinical findings and histopathology with immunohistochemistry are mandatory in such cases to reach to a correct diagnosis to ensure proper management.


Assuntos
Adenoma Oxífilo , Neoplasias da Glândula Tireoide , Tireoidectomia , Adenoma Oxífilo/diagnóstico , Adenoma Oxífilo/metabolismo , Adenoma Oxífilo/patologia , Adenoma Oxífilo/cirurgia , Adulto , Biópsia por Agulha Fina , Carcinoma Neuroendócrino/diagnóstico , Carcinoma Neuroendócrino/metabolismo , Carcinoma Neuroendócrino/patologia , Carcinoma Neuroendócrino/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Imuno-Histoquímica , Neoplasias da Glândula Tireoide/diagnóstico , Neoplasias da Glândula Tireoide/metabolismo , Neoplasias da Glândula Tireoide/patologia , Neoplasias da Glândula Tireoide/cirurgia
18.
Arch Endocrinol Metab ; 63(2): 175-181, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31038596

RESUMO

Cushing's syndrome (CS) is an uncommon condition that leads to high morbidity and mortality. The majority of endogenous CS is caused by excessive ACTH secretion, mainly due to a pituitary tumor - the so-called Cushing's disease (CD) - followed by ectopic ACTH syndrome (EAS), an extra-pituitary tumor that produces ACTH; adrenal causes of CS are even rarer. Several methods are used to differentiate the two main etiologies: specific laboratory tests and imaging procedures, and bilateral inferior petrosal sinus sampling (BIPSS) for ACTH determination; however, identification of the source of ACTH overproduction is often a challenge. We report the case of a 28-year-old woman with clinical and laboratory findings consistent with ACTH-dependent CS. All tests were mostly definite, but several confounding factors provoked an extended delay in identifying the origin of ACTH secretion, prompting a worsening of her clinical condition, with difficulty controlling hyperglycemia, hypokalemia, and hypertension. During this period, clinical treatment was decisive, and measurement of morning salivary cortisol was a differential for monitoring cortisol levels. This report shows that clinical reasoning, experience and use of recent methods of nuclear medicine were decisive for the elucidation of the case.


Assuntos
Síndrome de ACTH Ectópico/diagnóstico , Carcinoma Neuroendócrino/diagnóstico por imagem , Neoplasias Pulmonares/diagnóstico por imagem , Síndrome de ACTH Ectópico/etiologia , Hormônio Adrenocorticotrópico/sangue , Adulto , Carcinoma Neuroendócrino/complicações , Carcinoma Neuroendócrino/diagnóstico , Diagnóstico Diferencial , Feminino , Humanos , Hidrocortisona/sangue , Neoplasias Pulmonares/complicações , Neoplasias Pulmonares/diagnóstico , Amostragem do Seio Petroso , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Saliva/metabolismo
20.
BMC Endocr Disord ; 19(Suppl 1): 45, 2019 May 29.
Artigo em Inglês | MEDLINE | ID: mdl-31142313

RESUMO

BACKGROUND: Medullary thyroid carcinoma is a neuroendocrine tumor belonging form a malignant growth of the thyroid parafollicular C-cells, representing from 1 to 10% of all thyroid cancer. The biochemical activity of medullary thyroid carcinoma includes the production of calcitonin and carcinoembryogenic antigen, which are sensitive tumor markers, facilitating the diagnosis, follow-up and prognostication. The diagnosis is reached through the identification of high basal calcitonin serum level or after pentagastrin stimulation test. Medullary thyroid carcinoma is able to produce other relevant biomarkers as procalcitonin, carcinoembryionic antigen and chromogranin A. In Literature are described few cases of medullary thyroid carcinoma without elevation of serum calcitonin, an extremely rare event. The aim of this study was to analyse the presentation, the main features and therapeutic management of medullary thyroid carcinoma associated with negative serum calcitonin levels. METHODS: Using the PubMed database, a systematic review of the current Literature was carried out, up to February 2018. Finally, nineteen articles met our inclusion criteria and were selected according to the modified Newcastle-Ottawa scale. RESULTS: Fourty-nine patients with definitive pathology confirming medullary thyroid carcinoma and with calcitonin serum level in the normal range were identified (24 female, 24 male and not reported gender in 1 case). Mean age was 51.7 years. Serum calcitonin levels were reported for 20 patients with a mean value of 8.66 pg/mL and a range of 0.8-38 pg/mL. Despite the low or undetectable calcitonin serum level, at immunochemistry in almost the half of the cases reported by the Authors, the tumors presented diffuse or focal positivity for calcitonin and carcinoembryionic antigen, while was reported a chromogranin A positivity in 41 of the 43 tested patients. CONCLUSIONS: Calcitonin negative medullary thyroid carcinoma is an extremely rare pathology. The diagnosis and the surveillance is often challenging and delayed, due to the lack of elevation of serum markers as calcitonin and carcinoembryionic antigen. Further studies are needed, to better define options for management of non secretory medullary thyroid carcinoma and to identify new and reliable biomarkers associated to diagnosis and relapse of this medical dilemma.


Assuntos
Calcitonina/sangue , Carcinoma Neuroendócrino/diagnóstico , Carcinoma Neuroendócrino/cirurgia , Neoplasias da Glândula Tireoide/diagnóstico , Neoplasias da Glândula Tireoide/cirurgia , Nódulo da Glândula Tireoide/diagnóstico , Nódulo da Glândula Tireoide/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Carcinoma Neuroendócrino/sangue , Feminino , Seguimentos , Humanos , Masculino , Metanálise como Assunto , Pessoa de Meia-Idade , Prognóstico , Neoplasias da Glândula Tireoide/sangue , Nódulo da Glândula Tireoide/sangue
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