Biological Therapy ; Carcinoma, Neuroendocrine ; Thyroid Neoplasms ; therapy

Neuroendocrine carcinoma (NEC) has been reported to comprise 25% of lung cancer cases. NEC is classified as typical carcinoid, atypical carcinoid, large-cell neuroendocrine carcinoma, and small-cell lung cancer. Carcinoid tumors are less aggressive and surgery is the mainstay of treatment; however, patients with metastatic or inoperable disease need systemic therapy to control carcinoid symptoms and improve survival. Somatostain analogues, targeted agents including everolimus, sunitinib, bevacizumab, and conventional chemotherapy have emerged as treatment options for such patients. Large-cell neuroendocrine carcinoma exhibits more aggressive behavior and has a poorer prognosis than carcinoid tumors. No standard adjuvant and palliative chemotherapeutic agents have yet been established. This review sets out the treatment options for neuroendocrine tumors.
Adenocarcinoma* ; Carcinoid Tumor ; Carcinoma, Neuroendocrine* ; Drug Therapy* ; Humans ; Lung Neoplasms ; Lung* ; Neuroendocrine Tumors ; Prognosis ; Bevacizumab ; Everolimus

Pulmonary neuroendocrine tumors (PNETs) are a kind of epithelial tumors originating from pulmonary neuroendocrine cells, accounting for about 20% of primary lung tumors, including typical carcinoid, atypical carcinoid, small cell carcinoma, and large cell neuroendocrine carcinoma. The morphologic and clinical characteristics of these four types of PNETs are relatively highly heterogeneous. Immune checkpoint inhibitors (ICIs) have been shown robust antitumor activity in a variety of solid tumors. Treatment regimens of advanced PNETs have developed greatly in the past decade, but ICIs are still in their infancy in the field of PNETs. This review focuses on the landscape of current clinical trials and research as well as the situation of ICIs-related biomarkers in PNETs.
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Carcinoid Tumor ; Carcinoma, Neuroendocrine ; Humans ; Immune Checkpoint Inhibitors ; Lung Neoplasms/drug therapy* ; Neuroectodermal Tumors, Primitive ; Neuroendocrine Tumors/drug therapy*

Neuroendocrine tumor in thymus is rare and has poor prognosis due to frequent recurrence and distant metastasis. Approximately half of thymic carcinoids are hormonally active and Cushing's syndrome is seen in 33% of affected patients. Treatment of choice is surgical excision of tumor and role of chemotherapy and radiotherapy is controversal. We report 2 cases of thymic neuroendocrine carcinoma associated with Cushing's syndrome.
Carcinoid Tumor ; Carcinoma, Neuroendocrine ; Cushing Syndrome* ; Drug Therapy ; Humans ; Neoplasm Metastasis ; Neuroendocrine Tumors* ; Prognosis ; Radiotherapy ; Recurrence ; Thymus Gland ; Thymus Neoplasms

A 57-year-old male with periampullary duodenal mass was diagnosed as grade 3 duodenal neuroendocrine carcinoma with multiple liver metastasis. After nine cycles of cisplatin and etoposide, abdominal computed tomography (CT) findings showed complete regression of primary duodenal mass with marked size reduction of liver metastasis. Positron emission tomography findings showed metabolic complete response in both duodenal and liver mass. Pylorus-preserving pancreaticoduodenectomy was done and pathologic finding showed 5 mm sized remnant neuroendocrine tumor. The patient has remained alive with no evidence of disease for 43 months after initial diagnosis. This case suggests the possibility of heterogeneous nature of grade 3 neuroendocrine carcinoma and selected population may have extreme sensitivity to cisplatin and etoposide chemotherapy leading to complete response.
Carcinoma, Neuroendocrine ; Cisplatin ; Diagnosis ; Drug Therapy ; Etoposide ; Humans ; Liver ; Male ; Middle Aged ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Pancreaticoduodenectomy ; Positron-Emission Tomography

OBJECTIVE: To evaluate the clinical and pathologic factors associated with survival in patients with neuroendocrine cervical carcinoma (NECC). METHODS: The records of 61 patients with NECC diagnosed between 2000 and 2014 at Seoul National University Hospital and the National Cancer Center were retrospectively reviewed. Kaplan-Meier and Cox regression methods were used for analyses. RESULTS: Of the 61 patients, 67.2% were diagnosed at early stage (I to IIA) with a median age of 49 years. Of those, 78% underwent surgery and 75.6% received postoperative adjuvant treatment. For patients diagnosed at advanced stage, 60.0% received chemotherapy only and 25.0% received concurrent chemoradiation therapy. In the univariate analysis, advanced stage (77 vs. 40 months, P=0.013), tumor size ≥2 cm (133 vs. 47 months, P=0.002) and mixed tumor (101 vs. 34 months, P=0.004) were shown to be poor prognostic factors. In the multivariate analysis, tumor stage, tumor size and tumor homology were shown to be independent prognostic factors for overall survival. Of the total, 39.3% of the patients experienced recurrence, and 54.1% of the patients had metastasis. Of the patients diagnosed at early stage, 51.2% experienced recurrence. CONCLUSION: Tumor stage, tumor size and tumor homology were found to be independent prognostic factors in patients with NECC. Even in patients diagnosed at early stage, recurrence and distant metastasis were frequently observed.
Carcinoma, Neuroendocrine ; Carcinoma, Small Cell ; Drug Therapy ; Humans ; Multivariate Analysis ; Neoplasm Metastasis ; Recurrence ; Retrospective Studies ; Seoul

Objective: To investigate the clinicopathological features, treatment and prognosis of maxillofacial neuroendocrine carcinoma. Methods: A total of 11 patients with maxillofacial neuroendocrine carcinoma diagnosed in the Department of Pathology of The First Affiliated Hospital of Zhengzhou University from December 2010 to July 2022 were retrospectively enrolled, including 8 males and 3 females, aged (65.2±9.5) years (ranged from 49 to 87 years), with a disease course of 0.5 to 6.0 months. The clinicopathological data including head and neck CT, MRI and treatment methods were analyzed. Results: Submandibular gland and maxilla were involved in 3 cases, parapharynx in 2 cases, and face, tongue root and soft palate in 1 case respectively. Clinically, the initial symptom is a rapidly growing painless or tender mass, which may be accompanied by restricted mouth opening, dysphagia, and local numbness after invasion of masticatory muscles and nerves. The tumors were all invasive and low-density, with unclear boundaries from the surrounding tissues. Among the patients, 9 received surgical treatment, and 5 received adjuvant treatment after surgery (2 received chemotherapy, 3 received radiotherapy+chemotherapy). According to the 5th edition of the World Health Organization classification of head and neck tumors in 2022, there were 1 case (1/11) with poorly differentiated large cells and 10 cases (10/11) with poorly differentiated small cells. Histologically, the macrocell type is composed of large cells with rough chromatin, obvious vacuolar nucleolus, protruding nucleolus, and necrosis. The small cell type is dominated by small blue round cells with neuroendocrine characteristics, with active growth and multifocal necrosis. Immunohistochemical staining showed that cytokeratin (CK), epithelial membrane antigen (EMA) and synaptophysin (Syn) were diffusively expressed, 10 cases expressed CD56, 8 cases expressed p63, 6 cases expressed weakly punctated chromograin-A (CgA), and S-100 was not expressed. The Ki-67 index ranges from 20 to 90 percent. By the end of follow-up (0.5 to 127.0 months), 3 patients were alive, and the mean progression-free survival (21.0 months) of postoperative chemoradiotherapy patients was significantly longer than that of surgery and/or chemotherapy alone (3.3 months). Conclusions: Maxillofacial neuroendocrine carcinoma is characterized by low differentiation of small cells, high degree of malignancy and poor prognosis. Radical surgery combined with chemoradiotherapy has better local control effect.
Male ; Female ; Humans ; Carcinoma, Small Cell/therapy* ; Retrospective Studies ; Carcinoma, Neuroendocrine/pathology* ; Prognosis ; Tongue

Neuroendocrine tumor is a very heterogenous group arising from the neuroendocrine cells. Especially, large cell neuroendocrine tumor of the ovary is a extremely rare aggressive neoplasm, characteristically arising in association with a surface epithelial tumor. This report describes a mixed type of large cell neuroendocrine carcinoma and adenocarcinoma of the ovary. A 63-year old woman presented with abdominal distension and discomfort underwent staging laparotomy under the impression of ovarian cancer. The operation revealed an small ovarian mass with invasion of multiple region in peritoneal cavity by the tumor. Immunohistochemical and ultrastructural analysis confirmed the neuroendocrine nature of the tumor. The adenocarcinoma in this case is mixture of mucinous and endometrioid type. A diagnosis of stage IIIc mixed large cell neuroendocrine tumor and adenocarcinoma of the ovary was rendered. She is subsequently being treated with Paclitaxel and Carboplatin combination chemotherapy.
Adenocarcinoma* ; Carboplatin ; Carcinoma, Neuroendocrine ; Diagnosis ; Drug Therapy, Combination ; Female ; Humans ; Laparotomy ; Middle Aged ; Mucins ; Neuroendocrine Cells ; Neuroendocrine Tumors* ; Ovarian Neoplasms ; Ovary* ; Paclitaxel ; Peritoneal Cavity

The clinical data of one case of laryngeal neuroendocrine carcinoma in our hospital were respectively analyzed. The patient was a 61-year-old male, and complained of progressive pain of right ear for 7-year, it was misdiagnosed as glossopharyngeal neuralgia and larynx hemangioma. This patient was achieved total laryngectomy and bilateral cervical lymph nodes dissection. As of 12 months after surgery, the patient remains under follow-up observation, and no findings indicating obvious recurrence or metastasis has been observed. This disease is a rare and highly malignant tumor that is lack of specific feature in clinical performances. Recognition at larynx is essential so that proper patient management can be initiated.
Carcinoma, Neuroendocrine ; diagnosis ; therapy ; Humans ; Laryngeal Neoplasms ; diagnosis ; therapy ; Male ; Middle Aged

Extrapulmonary small cell carcinoma is a highly malignant and poorly differentiated neuroendocrine tumor. The overall incidence in the United States is approximately 0.1~0.4%. Moreover, small cell carcinoma of the liver is extremely rare and few cases have been reported in the literature. We experienced a 65-year-old woman with a 15 cm hepatic mass, which was localized in the left lobe of a noncirrhotic liver. The mass was confirmed as small cell carcinoma by immunohistochemical staining of a biopsy specimen. Other possible primary sites were excluded by radiologic and endoscopic evaluations. The patient was treated with combination chemotherapy. She was in partial response on the last follow-up visit.
Aged ; Biopsy ; Carcinoma, Neuroendocrine ; Carcinoma, Small Cell ; Drug Therapy, Combination ; Female ; Follow-Up Studies ; Humans ; Incidence ; Liver ; Neuroendocrine Tumors ; United States