The clinical data of 10 patients with pathologically confirmed primary hepatic neuroendocrine neoplasms (PHNENs) were retrospectively analyzed. The cohort included 8 males and 2 females, with a median age of 63 years. None presented with carcinoid syndrome. Three cases were detected incidentally during health check-ups, 2 presented with painless jaundice, and 5 reported abdominal distension or pain (1 with concurrent jaundice). Elevated tumor markers included carbohydrate antigen 19-9 in 4 cases, alpha-fetoprotein in 2 cases, and neuron-specific enolase in 1 case. All patients underwent surgical resection, including hepatectomy and hilar cholangiocarcinoma resection, combining with resection and reconstruction of right hepatic artery, resection of liver metastases and pancreaticoduodenectomy according to the extent of tumor invasion.Preoperative imaging failed to diagnose neuroendocrine neoplasms in all cases. Final pathological diagnoses were neuroendocrine tumor (NET) G2 in 5 cases, NET G3 in 1 case, and neuroendocrine carcinoma (NEC) in 4 cases. During the follow-up, 4 patients died and 6 survived. The study demonstrates that PHNENs lack specific clinical or imaging features, and the diagnosis relies on pathological examination after excluding metastatic disease. Radical resection remains the primary treatment, with prognosis varying significantly by tumor grade.
Humans ; Middle Aged ; Male ; Female ; Neuroendocrine Tumors/pathology* ; Liver Neoplasms/pathology* ; Retrospective Studies ; Aged ; Adult

Neuroendocrine carcinoma (NEC) represents a category of malignant tumors originating from neuroendocrine cells. Given that NEC cells exhibit characteristics of both neural and endocrine cells, they can hijack neuronal signaling pathways and dynamically regulate the expression of neuronal lineage markers during tumor metastasis, thereby constructing a microenvironment conducive to tumor growth and metastasis. Conversely, alterations in the tumor microenvironment can enhance the interactions between neurons and tumor cells, ultimately synergistically promoting the metastasis of NEC. This review highlights recent advancements in the field of cancer neuroscience, uncovering neuronal lineage markers in NEC that facilitate tumor dissemination through mediating crosstalk, bidirectional communication, and synergistic interactions between tumor cells and the nervous system. Consequently, the latest findings in tumor neuroscience have enriched our understanding of the biological mechanisms underlying tumor metastasis, opening new research avenues for a deeper comprehension of the complex biological processes involved in tumor metastasis, particularly brain metastasis. This review provides a comprehensive review of the crosstalk between tumor cells and neural signaling in the metastasis of NEC.
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Humans ; Carcinoma, Neuroendocrine/metabolism* ; Signal Transduction ; Animals ; Neoplasm Metastasis ; Neurons/pathology* ; Tumor Microenvironment ; Cell Communication

Pulmonary large cell neuroendocrine carcinoma (LCNEC) is a high-grade neuroendocrine tumor with unique characteristics, and its treatment regimens are primarily derived from those for small cell lung cancer (SCLC) and non-small cell lung cancer (NSCLC). In recent years, the incidence rate has been on the rise, and the prognosis are affected by the interaction of multiple factors such as individual, clinical stage and treatment mode, and the heterogeneity is significant. In the study of molecular subtypes, multiple subgroups were divided according to key gene mutations such as RB1 and TP53, and genomic subtypes were associated with survival, chemotherapy response, and efficacy of precision therapy. Targeted therapy excavates multiple targets, and the efficacy of drugs is different. Immunotherapy has made remarkable progress, and immune checkpoint inhibitors (ICIs) have been effective in all stages of chemotherapy alone or in combination with chemotherapy or radiation therapy, but there is a risk of hyperprogressive diseases, and accurate prognostic markers need to be explored urgently. This review reviews the latest research progress in the study of molecular subtypes and precision therapies such as targeted therapy and immunotherapy of pulmonary LCNEC, and points out that pulmonary LCNEC treatment will develop in the direction of precision and individualization in the future.
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Humans ; Lung Neoplasms/drug therapy* ; Carcinoma, Neuroendocrine/drug therapy* ; Precision Medicine ; Immunotherapy ; Carcinoma, Large Cell/drug therapy*

Neuroendocrine carcinoma (NEC), a subtype of neuroendocrine tumors with high proliferative activity, is characterized by strong invasiveness and poor prognosis. This article reports a previously healthy female non-smoker who developed NEC occurring sequentially in different lobes of both lungs. The lesions were pathologically diagnosed by hematoxylin-eosin (HE) staining as large cell neuroendocrine carcinoma (LCNEC) and small cell lung cancer (SCLC), respectively. Next-generation sequencing (NGS) performed on both lesions revealed the presence of echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase (EML4-ALK) fusion mutations in both lesions. Notably, the patient achieved a significant therapeutic response to ALK-tyrosine kinase inhibitors (TKIs) targeted therapy.
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Humans ; Oncogene Proteins, Fusion/metabolism* ; Female ; Lung Neoplasms/enzymology* ; Carcinoma, Neuroendocrine/pathology* ; Middle Aged

OBJECTIVE: To investigate the effect of intraoperative blood salvage autotransfusion on local recurrence and long-term metastasis of patients after carotid body tumor resection. METHODS: We retrospectively reviewed a consecutive series of 61 patients undergoing elective carotid body tumor resection from August 2009 to December 2020. Among them, 14 received intraoperative blood salvage autotransfusion (autotrasfusion group) and 47 did not (non-autotransfusion). Data of general information, surgical status and postoperative follow-up results were collected. RESULTS: The proportion of Shamblin Ⅲ in the autotransfusion group was 85.7%, which was significantly higher than 31.9% in the non-autotransfusion group (P=0.003). The average operation time of the 14 patients in the autotransfusion group was (264±84) min, intraoperative blood loss was 1 200 (700, 2 700) mL, and autologous blood transfusion was 500 (250, 700) mL. Of these, 8 patients (57%) required concomitant allogeneic blood with 400 (260, 400) mL of allogeneic blood. The average operation time of the 47 patients in the non-autotransfusion group was (153±75) min, and the intraoperative blood loss was 300 (100, 400) mL. Of these, 6 (13%) required allogeneic blood transfusion, and 520 (400, 520) mL of allogeneic blood was used. Compared with the non-autotransfusion group, the average operation time in the autologous blood transfusion group was significantly longer (P < 0.001), and the intraoperative blood transfusion volume was larger (P=0.007). Of the 14 patients undergoing autotransfusion, 8 (57%) needed allogeneic blood at the same time; while in the 47 non-autologous transfusion patients, 6 (13%) needed allogeneic blood transfusion. The proportion of autotransfusion group using allogeneic blood at the same time was even higher (P=0.002). The incidence of nerve injury within 30 days after surgery was 29.5%, and there was no significant difference between the two groups. No early deaths occurred. The average follow-up was (76±37) months. One case of local recurrence occurred in the non-autotransfusion group. There was no distant metastasis. There were no tumor-related deaths. The estimated 5-year and 10-year overall survival rates were 96.4% and 83.8%, respectively. There was no significant difference in overall survival between the two groups (P=0.506). CONCLUSION The use of intraoperative blood salvage autotransfusion increased no risk of local recurrence and distant metastasis in patients with carotid body tumor, which is safe and effective in carotid body tumor resection.
Humans ; Blood Transfusion, Autologous/methods* ; Operative Blood Salvage/methods* ; Retrospective Studies ; Male ; Female ; Carotid Body Tumor/pathology* ; Middle Aged ; Prognosis ; Neoplasm Recurrence, Local ; Blood Loss, Surgical ; Aged ; Adult ; Operative Time

Neoplasms characterized by the expression of markers of neuroendocrine differentiation in neoplastic cells are defined as neuroendocrine neoplasms. A case of neuroendocrine carcinomas (NECs) with a small amount of papillary adenocarcinoma and significantly vacuolar nucleus at the esophagogastric junction was reported in this article. A 77-year-old male had dysphagia for one week. Endoscopy revealed early-stage esophagogastric junction carcinoma, and biopsy was diagnosed as poorly differentiated carcinoma. Endoscopic submucosal dissection was performed. Histologically, the papillary adenocarcinoma progresses from typically branching papillary structures (well-differentiated) to hyperplasia of the lining epithelium of the papilla to form a cribriform structure (moderately differentiated), to solid area lacking papillary structures (poorly differentiated). There was a continuous process, and during this process, the vacuoles in the nuclei of tumor cells showed progressive changes from mild to obvious and finally to significant vacuoles. The tumor was mainly composed of solid areas (about 95%), with single cell, large cell, round or oval to irregular nuclei, and significantly vacuolar nuclei, nuclei with larger vacuoles appeared in a loop, a few thin weakly basophilic or weakly eosinophilic fine particles could be seen in the vacuoles, and the vacuoles had rough edges. The nucleus chromatin at the outer edge of the vacuoles was fine particles, and mitosis was common (20-30/mm²), atypical mitosis could be seen, and nucleoli could be seen easily, the cytoplasm was weakly eosinophilic, and the boundaries of cells were unclear. The cells were arranged in a nested, trabecular, or diffuse sheet shape, with some arranged in a glandular tube shape. Tumor thrombus was found in the vein of submucosa; the interstitial tissue rich in capillaries within the tumor was accompanied by a large number of neutrophil infiltration. Immunohistochemical staining showed that the solid area of the tumor was positive for synaptophysin (Syn) and chromogranin A (CgA), while papillary adenocarcinoma was negative. Mucin 5AC (MUC5AC) was diffusely positive in papillary adenocarcinoma, while the proportion of positive cells in the solid area of the tumor was about 10% to 15%. In a word, this case showed the extreme situation of the vacuolar nuclear characteristics of NECs, extremely rare, in a sense, this case expanded the boundary of the morphological spectrum of NECs. Understanding the extreme vacuolar features of this nucleus is helpful to make a correct pathological diagnosis.
Humans ; Male ; Esophagogastric Junction/pathology* ; Aged ; Carcinoma, Neuroendocrine/pathology* ; Vacuoles/pathology* ; Esophageal Neoplasms/pathology* ; Cell Nucleus/pathology* ; Adenocarcinoma, Papillary/pathology* ; Stomach Neoplasms/pathology*

Objective:Paragangliomas （PGLs） are chromaffin cell tumors originating from paraganglia and are classified as neuroendocrine neoplasms.They predominantly occur along the distribution area of the paraganglia, commonly occurring between the ages of 20 and 40, with a slight male predominance.They are most frequently found in the axial regions from the skull base to the pelvic cavity. Paragangliomas in the head and neck region typically lack endocrine functionality and primarily manifest through local mass effects. However, clinical signs and symptoms alone cannot reliably distinguish between metastatic and non-metastatic cases. Clinically apparent metastatic paragangliomas are relatively rare. Herein, we present a case of a paraganglioma located in the region of the jugular foramen with liver, bone, and lymph node metastases, and discuss the treatment and prognosis of head and neck paragangliomas.
Humans ; Head and Neck Neoplasms/pathology* ; Jugular Foramina/pathology* ; Liver Neoplasms/secondary* ; Lymphatic Metastasis ; Paraganglioma/pathology*

Objective:To analyze the clinical characteristics and risk factors of postoperative first bite syndrome（FBS） after parapharyngeal space surgery, and to provide evidence-based recommendations for the diagnosis and management of this complication. Methods:A retrospective analysis was conducted on 142 patients undergoing parapharyngeal space surgery from March 2016 to November 2024, including 12 cases of postoperative FBS. Univariate analysis compared differences in tumor location, pathological type, and surgical approach between FBS and non-FBS groups. Multivariate logistic regression identified independent risk factors. A systematic literature review evaluated the preventive value of transoral endoscopic surgery for FBS, followed by proposed clinical optimization strategies. Results:The incidence of FBS was 8.45%（12/142）. Univariate analysis revealed significant associations with: Superior parapharyngeal space tumors（OR=3.21, 95%CI 1.12-9.21, P=0.029）; Schwannoma pathology（OR=4.05, 95%CI 1.35-12.18, P=0.013）; Traditional lateral cervical approach（OR=5.67, 95%CI 1.89-17.02, P=0.002）. Multivariate analysis confirmed lateral cervical approach（aOR=4.98, 95%CI 1.62-15.31, P=0.005） and schwannoma（aOR=3.75, 95%CI 1.22-11.51, P=0.021） as independent risk factors. Literature review suggested lower FBS rates with transoral endoscopic approaches. The overall effect of the drug on FBS is poor. Conclusion:FBS is a frequent complication of parapharyngeal space surgery, significantly associated with tumor location, pathology, and surgical approach. Transoral endoscopic surgery can effectively reduce the risk of FBS through precise anatomical dissection that minimizes sympathetic nerve injury. Minimally invasive approaches are recommended for eligible cases.
Humans ; Retrospective Studies ; Postoperative Complications/etiology* ; Risk Factors ; Endoscopy ; Parapharyngeal Space/surgery* ; Male ; Female ; Adult ; Middle Aged ; Neurilemmoma/surgery*

Objective:To summarize the initial experience and evaluate the technical feasibility of the endoscopic transcanal transpromontorial approach（TTA） for vestibular schwannoma resection by analyzing long-term follow-up outcomes. Methods:A retrospective analysis was conducted on the perioperative and long-term follow-up data（mean follow-up time: 5 years） of patients who underwent endoscopic TTA for vestibular schwannoma resection in the Department of Otorhinolaryngology Head and Neck Surgery at Xiangya Hospital, Central South University, between January 2020 and December 2020. Long-term outcomes were systematically evaluated. Results:This study included two patients（one 41-year-old male and one 51-year-old female）. According to the AAO-HNS hearing classification system, preoperative hearing was Class C in one patient and Class D in the other. Preoperative imaging confirmed Koos stageⅠ tumors in both cases. Postoperative transient facial nerve paralysis（House-Brackmann Grade Ⅲ） recovered to Grade Ⅰ within 4 months. No complications such as cerebrospinal fluid leakage, intracranial infection, or intracranial hemorrhage occurred. No tumor recurrence was observed during the 5-year follow-up period. Conclusion:The endoscopic transcanal transpromontorial approach is minimally invasive, facilitates rapid recovery, and demonstrates satisfactory technical feasibility and safety when strict patient selection criteria（Koos stageⅠtumors with non-serviceable hearing） are applied.
Humans ; Neuroma, Acoustic/surgery* ; Male ; Adult ; Middle Aged ; Retrospective Studies ; Female ; Endoscopy/methods* ; Follow-Up Studies ; Treatment Outcome

Sigmoid colon schwannoma is a rare benign gastrointestinal tumor that is challenging to be diagnosed preoperatively.This paper reported a case of sigmoid colon schwannoma that was preoperatively misdiagnosed as a gastrointestinal stromal tumor on ultrasonography.Intraoperative frozen section analysis identified it as a spindle cell tumor,with definitive diagnosis confirmed as gastrointestinal schwannoma via immunohistochemistry.This paper summarizes the similarities and differentiating features of intestinal stromal tumors and schwannomas on ultrasonographic imaging.By analyzing and discussing previous literature,we aim to enhance the diagnostic and differential diagnostic capabilities of ultrasonographers in preoperative evaluation of intestinal schwannomas,thereby providing reliable evidence for clinicians in the diagnosis and management of this condition.
Humans ; Neurilemmoma/diagnostic imaging* ; Ultrasonography ; Sigmoid Neoplasms/diagnostic imaging* ; Male ; Colon, Sigmoid/diagnostic imaging* ; Middle Aged ; Female ; Diagnosis, Differential