We report a rare case involving a 52-year-old female diagnosed with an atypical bronchial carcinoid tumor with metastases to the mediastinum, hilar lymph nodes, breast, and pancreas. In additional, the patient had metastases to the iris and ciliary body, resulting in progressive vision loss in her left eye. Treatment was successful by intravitreal injections of anti-vascular endothelial growth factor.
Humans ; Female ; Middle Aged ; Endothelial Growth Factors ; Intravitreal Injections ; Case Management ; Carcinoid Tumor/pathology*

Objective: To investigate the clinical features, morphological characteristics, immunophenotype, and differential diagnosis of goblet cell adenocarcinoma (GCA) in the digestive system. Methods: The clinicopathological data, morphological characteristics, immunophenotypes of 22 cases of GCA in the digestive system diagnosed from January 2010 to January 2021 were collected. Meanwhile, 25 cases of neuroendocrine neoplasm (NEN) and 24 cases of adenocarcinoma were used as controls. Relevant literature was also reviewed. Results: There were 16 males and 6 females, aged from 36 to 79 years with an average of 56 years. The anatomical sites of the 22 GCA were mostly appendix (17 cases) and occasionally extra-appendix (5 cases), including 3 cases in stomach, 1 case in duodenum and 1 case in anal. All 17 cases of appendiceal GCA were pure GCA. Among the 5 cases of extra-appendiceal GCA, One case of gastric GCA was pure, two cases of gastric GCA with NEN or adenocarcinoma, duodenal GCA with NEN and adenocarcinoma, anal GCA with NEN.Low-grade GCAs were composed of goblet, Paneth and neuroendocrine cells, which were arranged in intestinal crypt tubular or cluster structures and distributed in the wall of digestive system. The tubular and cluster structures lacked adhesion. Goblet cells were columnar, located in the base, with clear cytoplasm, small nuclei, inconspicuous atypia, and uncommon mitoses. Extracellular mucus and signet-ring cells with nuclear variations could be seen in some cases. Nerve fiber bundle invasion and tumor thrombus in vessels were often present. High-grade GCAs lacked tubular and cluster structures, and their histological structures were more complex. Tumor cells expressed mixed neuroendocrine and glandular epithelial markers. Similar to the expression patterns of synaptophysin and chromogranin A, CD200 and INSM1 were also dot-like or patch-positive in GCA. Conclusions: GCA is an infrequent tumor of the digestive system and shows the bi-directional differentiation characteristics of neuroendocrine and glandular epithelium. Accurate diagnosis and staging are related to its prognosis.
Adenocarcinoma/pathology* ; Appendiceal Neoplasms/surgery* ; Carcinoid Tumor/surgery* ; Chromogranin A ; Female ; Goblet Cells/pathology* ; Humans ; Male ; Neuroendocrine Tumors/pathology* ; Repressor Proteins ; Synaptophysin

Carcinoid Tumor/pathology* ; Carcinoma, Neuroendocrine/pathology* ; Humans ; Lung Neoplasms/pathology* ; Neuroendocrine Tumors/pathology*

Objective: To investigate the clinical and pathological features, treatments and prognosis of laryngeal neuroendocrine carcinoma (LNEC). Methods: We conducted the retrospective analysis of the clinical data of 12 patients with LNEC admitted to the Department of Otorhinolaryngology Head and Neck Surgery, Second Hospital of Shanxi Medical University from May 2014 to December 2021, including 9 males and 3 females, aged 50-77 years. There were 4 cases of typical carcinoid tumour (highly differentiated), 5 cases of atypical carcinoid tumour (moderately differentiated) and 3 cases of neuroendocrine small cell carcinoma (hypofractionated). The clinical features, diagnosis, treatment and prognosis of LNEC were analysed. Results: The clinical manifestations of LNEC varied according to the tumour type but did not correlate with the pathological types. The supraglottic type was characterized by sore throat, foreign body sensation in the pharynx, coughing, obstructive sensation when eating and choking on water. The treatments were determined according to the pathological types, lesion location and invasion scope. Of 12 patients 4 underwent horizontal partial laryngectomy plus elective lymphatic dissection plus postoperative radiotherapy/chemotherapy, 4 underwent vertical partial laryngectomy (3 of them with cervical lymphatic dissection), 3 underwent supported laryngoscopic plasma laryngectomy for laryngeal cancer, and 1 abandoned for treatment. With the follow-up of 8 -78 months, 5 patients were alive, 1 died from chemotherapy reactions, 3 died from other diseases, 1 died from lung metastasis, 1 died from lung infection and 1 was lost to follow-up. Conclusion: LNEC is clinically rare, the clinical manifestations are less specificity, diagnosis relies on pathological and immunohistochemical examinations, and treatment modalities and prognoses are closely related to the pathological subtypes of LNEC.
Humans ; Male ; Female ; Laryngeal Neoplasms/pathology* ; Retrospective Studies ; Carcinoma, Neuroendocrine/pathology* ; Laryngectomy ; Carcinoid Tumor/pathology*

Goblet cell carcinoid (GCC) is a rare neoplasm of the vermiform appendix and can be mistaken as a typical neuroendocrine tumour (TNET). The natural history of this disease is more aggressive compared to TNETs and requires a more aggressive approach. We report a case of a 37-year-old male who was initially diagnosed with TNET, but subsequently revised as Tang's A GCC. He underwent appendectomy and right hemicolectomy. Aside from a persistently elevated carcinoembyrogenic antigen (CEA) result, his 18F-fluorodeoxyglucose (FDG) PET/CT and a 68-Gallium DOTATATE PET/CT scan showed no FDG or DOTATATE avid lesions. 

PURPOSE: Primary hepatic neuroendocrine tumor (PHNET) is a very rare neoplasm, requiring strict exclusion of metastasis from possible extrahepatic primary sites for its diagnosis. METHODS: We reviewed our clinical experience of 13 patients with primary hepatic NET who underwent liver resection from January 1997 to December 2015. RESULTS: The mean age of the 13 patients (8 males and 5 females) was 51.1 ± 12.8 years; the most common clinical manifestation was vague, nonspecific abdominal pain (n = 9). Of them, 11 patients underwent preoperative liver biopsy, 7 of which correctly diagnosed as neuroendocrine tumor (NET). Ten patients underwent R0 resection, and 3 underwent R1 resection. Diagnosis of PHNET was confirmed both immunohistochemically and by absence of extrahepatic primary sites. All tumors were single lesions, with a mean size of 9.6 ± 7.6 cm and a median size of 4.3 cm; all showed positive staining for synaptophysin and chromogranin. During a mean follow-up period of 95.1 ± 86.6 months, 7 patients died from tumor recurrence, whereas the other 6 remain alive to date, making the 5-year tumor recurrence rate 56.0% and the 5-year patient survival rate 61.5%. When confined to R0 resection, 5-year recurrence and survival rates were 42.9% and 70.0%, respectively. Univariate analysis showed that Ki-67 proliferative index was the only risk factor for tumor recurrence. CONCLUSION: PHNET is a very rare tumor with no specific clinical features, and its final diagnosis depends primarily on pathology, immunohistochemistry, and exclusion of metastasis from other sites. Aggressive surgical treatment is highly recommended for PHNET because of acceptably favorable postresection outcomes.
Abdominal Pain ; Biopsy ; Carcinoid Tumor ; Diagnosis ; Follow-Up Studies ; Hepatectomy ; Humans ; Immunohistochemistry ; Liver ; Male ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Pathology ; Prognosis ; Recurrence ; Risk Factors ; Survival Rate ; Synaptophysin

Pulmonary neuroendocrine tumors are common in pathological practice and its pathological classification and histological grading are not exactly the same as that of those in the digestive tract and pancreas. In 2015 edition of World Health Organization classification, pulmonary neuroendocrine tumors are classified as carcinoid tumors (including typical carcinoid and atypical carcinoid), small cell lung carcinoma, large cell neuroendocrine carcinoma, and precursor lesion diffuse idiopathic neuroendocrine cell hyperplasia; each category has distinctive morphological and immunohistochemical features. The morphologic features including growth patterns and cytological appearances are keys for the diagnosis of neuroendocrine tumor, and immunohistochemical findings are also critical for its diagnosis. Furthermore, the diagnostic criteria vary for different types of specimen. In this article, we present a concise review and summary of the update of clinicopathological characterizations of pulmonary neuroendocrine tumor, with an emphasis on its diagnostic criteria and differential diagnosis.
Carcinoid Tumor ; diagnosis ; pathology ; Diagnosis, Differential ; Humans ; Hyperplasia ; Lung Neoplasms ; diagnosis ; pathology ; Neuroendocrine Tumors ; classification ; diagnosis ; pathology

Neuroendocrine tumor (NET) is a cancer-like tumor that occurs mostly in the gastrointestinal system. Within the gastrointestinal tract, NET most commonly occurs in the rectum whereas appendix is very rarely involved. In most cases of appendiceal NET, it is found at a relatively early stage compared to other NETs because appendiceal NET frequently presents with acute appendicitis because appendiceal NET frequently presents with acute appendicitis even when the size is smaller than 1 cm. Therefore, it is very rare for lymph node metastasis to occur in a young adult. Herein, we report a rare case of grade 1 appendiceal NET with lymph node metastasis which developed in a teenage male.
Adolescent ; Appendiceal Neoplasms/*diagnosis/pathology/surgery ; Carcinoid Tumor/diagnosis/pathology ; Colectomy ; Colonoscopy ; Humans ; Lymphatic Metastasis ; Male ; Neuroendocrine Tumors/*diagnosis/pathology ; Tomography, X-Ray Computed

PURPOSE: To report the first case of pigmented choroidal metastases without enucleation diagnosed with fine-needle aspiration biopsy. CASE SUMMARY: A 47-year-old male was referred to our clinic with a suspected diagnosis of choroidal melanoma in his left eye. Positron emission tomography-computer tomography used to evaluate systemic metastases revealed lung cancer. Pathology of the detected lung mass was primary lung neuroendocrine tumor. To differentiate choroidal metastasis and primary choroidal melanoma, we performed a fine-needle aspiration biopsy for choroidal tumors. The cytology showed results favoring metastatic atypical carcinoid and the patient was started on systemic chemotherapy. CONCLUSIONS: All pigmented choroidal tumors are not choroidal melanomas and choroidal metastases may be pigmented. The present case suggests that proper systemic evaluation and biopsy for suspected choroidal tumor could be helpful in diagnosis.
Biopsy ; Biopsy, Fine-Needle ; Carcinoid Tumor ; Choroid* ; Diagnosis ; Drug Therapy ; Electrons ; Humans ; Lung ; Lung Neoplasms ; Male ; Melanoma* ; Middle Aged ; Neoplasm Metastasis* ; Neuroendocrine Tumors ; Pathology

PURPOSE: Several endoscopic resection therapies have been applied for the treatment of rectal carcinoid tumors. However, there is currently no consensus regarding the optimal strategy. We performed a meta-analysis to compare the efficacy and safety of endoscopic mucosal resection (EMR) or modified EMR (m-EMR) versus endoscopic submucosal dissection (ESD) for the treatment of rectal carcinoid tumors. MATERIALS AND METHODS: PubMed, Web of Science, Medline, Embase and CNKI were searched up to the end of January 2014 in order to identify all studies on the effects of EMR (or m-EMR) and ESD on rectal carcinoid tumors. RESULTS: A total of fourteen studies involving 782 patients were included. The pooled data suggested a significantly higher rate of pathological complete resection among patients treated with ESD or m-EMR than those treated with EMR [odds ratio (OR)=0.42, 95% confidence interval (CI): 0.25-0.71; OR=0.10, 95% CI: 0.03-0.33, respectively], while there was no significant difference between the m-EMR group and ESD group (OR=1.19, 95% CI: 0.49-2.86); The procedure time of ESD was longer than EMR or m-EMR groups [mean differences (MD)=-11.29, 95% CI: -14.19 - -8.38, MD= -10.90, 95% CI: -18.69 - -3.11, respectively], but it was insignificance between the EMR and m-EMR groups. No significant differences were detected among the treatment groups with regard to complications or recurrence. CONCLUSION: The results of this meta-analysis suggest that treatment of rectal carcinoid tumors with ESD or m-EMR is superior to EMR, and the efficacy of m-EMR is equivalence to ESD treatment. However, more well-designed studies are needed to confirm these findings.
Carcinoid Tumor/pathology/*surgery ; Dissection/adverse effects ; Endoscopy, Gastrointestinal/*adverse effects ; Humans ; Intestinal Mucosa/pathology/surgery ; Intestinal Neoplasms/pathology/*surgery ; Middle Aged ; Neoplasm Recurrence, Local/pathology ; Postoperative Complications/etiology ; Publication Bias ; Rectal Neoplasms/pathology/*surgery ; Time Factors ; Treatment Outcome ; Tumor Burden