BACKGROUND: Medullary thyroid carcinoma (MTC) is a rare disease, but it exhibits more aggressive behaviors. The aim of this study was to improve the diagnostic accuracy of MTC before surgery by analyzing the clinical and ultrasonic data of patients with MTC. METHODS: The study included 71 patients (96 lesions) with histopathologically proven MTC between April 2011 and September 2016 in the Cancer Hospital of Chinese Academy of Medical Sciences and Peking Union Medical College. The clinical characteristics and sonographic findings were retrospectively reviewed and compared between the ultrasonic correct diagnosis group and the ultrasonic misdiagnosis group with the t test or Mann-Whitney U test for quantitative parameters and the χ test or Fisher exact test for qualitative parameters. RESULTS: Compared with the ultrasonic correct diagnosis group, the proportion of the cystic change in the ultrasonic misdiagnosed group was high (25.0% vs. 4.2%), the uncircumscribed margin and irregular shape proportions were low (20.8%, 58.3% vs. 74.7%, 87.3%), calcification was relatively rare (20.8% vs. 56.3%), and rich vascularity was relatively rare (25.0% vs. 78.9%). CONCLUSIONS In the case of atypical MTC, such as cystic change, circumscribed margin, regular shape, no calcification, no rich vascularity, and normal cervical lymph nodes, MTC is easily misdiagnosed as benign by ultrasound. Therefore, ultrasound, cytology and serum calcitonin should be comprehensively evaluated for a preoperative diagnosis of MTC.
Adult ; Carcinoma, Neuroendocrine ; diagnosis ; diagnostic imaging ; surgery ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Thyroid Neoplasms ; diagnosis ; diagnostic imaging ; surgery ; Ultrasonography

The liver is one of the most common sites to which malignancies preferentially metastasize. Although a substantial number of liver malignancies are primary tumors, including hepatocellular carcinoma and intrahepatic cholangiocarcinoma, the metastasis of carcinomas to the liver is relatively common and frequently encountered in clinical settings. Representative carcinomas that frequently metastasize to the liver include colorectal carcinoma, breast carcinoma, neuroendocrine tumors, lung carcinoma, and gastric carcinoma. The diagnostic confirmation of suspected metastatic lesions in the liver is generally achieved through a histopathologic examination of biopsy tissues. Although morphology is the most important feature for a pathologic differential diagnosis of metastatic carcinomas, immunohistochemical studies facilitate the differentiation of metastatic carcinoma origins and subtypes. Useful immunohistochemical markers for the differential diagnosis of metastatic carcinomas in the liver include cytokeratins (CK7, CK19, and CK20), neuroendocrine markers (CD56, synaptophysin, and chromogranin A), and tissue-specific markers (CDX2, SATB2, TTF-1, GCDFP-15, mammaglobin, etc.). Here, we provide a brief review about the pathologic differential diagnosis of major metastatic carcinomas in the liver.
Biopsy ; Breast Neoplasms ; Carcinoma, Hepatocellular ; Cholangiocarcinoma ; Colorectal Neoplasms ; Diagnosis, Differential ; Immunohistochemistry ; Keratins ; Liver Neoplasms ; Liver ; Lung ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Pathology ; Synaptophysin

Small cell carcinomas are the most aggressive, highly malignant neuroendocrine tumors; among these, gastric small cell carcinoma (GSCC) is extremely rare. Here we report a case of a patient with primary GSCC, presenting as linitis plastic, who was diagnosed using endoscopic ultrasound (EUS)-guided biopsy. With undiagnosed linitis plastica, an 80-year-old woman was referred to our institution. Abdominal computed tomography revealed irregular wall thickening extending from the gastric body to the antrum. Endoscopy suspected to have Borrmann type IV advanced gastric cancer. EUS of the stomach showed diffuse submucosal thickening of the gastric wall, mainly the antrum. EUS-guided bite-on-bite biopsy confirmed the diagnosis of GSCC. In general, GSCC is difficult to diagnose and careful examination is necessary to determine the therapeutic strategy; however, EUS is particularly helpful in the differential diagnosis of a lesion presenting as linitis plastica.
Aged, 80 and over ; Biopsy ; Carcinoma, Small Cell ; Diagnosis ; Diagnosis, Differential ; Endoscopy ; Endosonography ; Female ; Humans ; Linitis Plastica ; Neuroendocrine Tumors ; Plastics ; Stomach ; Stomach Neoplasms ; Ultrasonography

OBJECTIVE: To compare the clinicopathologic features and survival outcomes of neuroendocrine tumor of the uterine corpus (NET-U) to endometrioid type endometrial carcinoma (EC). METHODS: From 1993 to 2012, the Surveillance, Epidemiology and End Results cancer registry was queried for women diagnosed with EC or NET-U. Data regarding stage, grade, presence of extra-uterine disease, lymph node metastasis, receipt of adjuvant radiation, surgical intervention and overall survival (OS) was extracted. Chi-square tests, t-tests and Kaplan Meir curves were used for statistical analysis. RESULTS: A total of 98,363 patients were identified: 98,245 with EC and 118 with NET-U. The mean age at diagnosis for EC was 61.7 years and 64.8 years for NET-U (p=0.01). NET-U cases were more likely to be poorly differentiated (97.0% vs. 15.6%; p≤0.01) and have nodal metastasis (56.4% vs. 11.1%; p≤0.01) when compared to EC. Presence of extrapelvic disease at the time of diagnosis was observed more frequently in NET-U compared to EC, 49.1% vs. 4.8%, respectively (odds ratio=18; 95% confidence interval=13.1–27.2; p≤0.01). Significant improvement in OS was observed in NET-U patient who received radiation (OS: 7.7 vs. 3.3 years; p≤0.01) or underwent surgical management (5.6 vs. 0.9 years; p≤0.01). The OS for EC was 14.4 vs. 4.6 years for NET-U (p≤0.01). CONCLUSION: NET-U represents an aggressive form of uterine malignancy. When compared to EC, patients with NET-U present at more advanced stage, have more frequent extra-uterine disease and lower OS.
Carcinoma, Endometrioid ; Diagnosis ; Endometrial Neoplasms ; Epidemiology ; Female ; Humans ; Lymph Nodes ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Uterine Neoplasms

OBJECTIVE: To evaluate the histological and stage characteristics of cervical cancer in women under 25 years old, and to compare them with older women. METHODS: Cross-sectional study of cases from the Hospital Cancer Registry of São Paulo State/Brazil from 2000 to 2015. Variables were age, International Federation of Gynecology and Obstetrics stage and histological type. Prevalence ratio (PR) and its 95% confidence interval (CI) were calculated. RESULTS: Out of 18,423 cervical cancer cases 204 (1.1%) were in women under 25 years old. The most frequent stage was stage I in women under 25 (36.2%) and between 25 and 34 (43.4%), and stage III in older women (31.8%). No statistically significant difference was observed in stages by age group. Squamous carcinomas were the most frequent in 73.5% of women under 25 and 78.5% of older women. In women under 25 the following histological types were more frequent: neuroendocrine carcinomas (PR=6.10, 95% CI=2.03–18.35), malignant germ cell tumors (PR=54.98, 95% CI=26.53–113.95), mesenchymal tumors (sarcomas) (PR=5.67, 95% CI=2.58–12.45) and hematopoietic/lymphoid tumors (PR=0.72, 95% CI=2.90–36.69). CONCLUSION: In women under 25 years old cervical cancer was an uncommon diagnosis and in about one third occurred at early stage. Squamous carcinoma was the most frequent histological type regardless age, but rare histological types were more frequent in young women.
Carcinoma, Neuroendocrine ; Carcinoma, Squamous Cell ; Cross-Sectional Studies ; Diagnosis ; Female ; Gynecology ; Humans ; Neoplasm Staging ; Neoplasms, Germ Cell and Embryonal ; Obstetrics ; Prevalence ; Sarcoma ; Uterine Cervical Neoplasms ; Young Adult

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

OBJECTIVETo investigate clinicopathological features and prognostic factors of appendiceal neuroendocrine neoplasms(a-NEN).

METHODSClinical data of 20 patients diagnosed with a-NEN at Zhongshan Hospital of Fudan University between January 2000 and December 2016 were retrospectively analyzed. Pathological diagnosis was based on the WHO classification criteria of digestive system tumors (2010 edition). Based on the mitotic count and Ki-67 index, a-NENs were divided into grade 1 neuroendocrine tumor (NET G1), grade 2(G2) NET G2) and grade 3 (neuroendocrine carcinoma, NEC). Some special types of a-NEN (e.g. goblet cell carcinoid) and mixed adenoneuroendocrine neoplasms were classified as mixed adenoneuroendocrine carcinoma (MANEC). Follow-up was conducted by telephone or return visits. Univariate analysis was carried out using the Kaplan-Meier method, and the log-rank test was used to draw survival curves.

RESULTSOf 20 patients, 14 were male and 6 were female with median age of 54 years. Seventeen cases presented acute right lower quadrant abdominal pain, 1 chronic right lower quadrant abdominal pain, 1 persistent abdominal discomfort with outburst whole abdominal pain and 1 was found during body check without symptoms. Twenty cases comprised 8 G1 patients, 4 G2 patients, 3 G3 patients, and 5 MANEC patients. When diagnosed, there was 1 patient with liver metastasis, 1 patient with abdominal and pelvic metastases, and 2 patients with postoperative pathological findings of lymph node metastasis. Six patients underwent appendectomy, 12 underwent right hemicolectomy, 1 underwent right hemicolectomy plus small intestine resection, and 1 underwent partial hepatectomy plus right hemicolectomy. The follow-up time was 7-187 months(average, 36 months). The total 1- and 3-year survival rates were 94.7% and 60.2%, respectively. Univariate analysis showed that age >50 years (χ=7.036, P=0.008), pathology grade as MANEC (χ=5.297, P=0.021), and metastasis (χ=6.558, P=0.010) indicated lower 5-year survival rate.

CONCLUSIONSMost a-NEN patients have no typical symptoms, and the main complaint at consultation is acute right lower quadrant abdominal pain. Prognosis is poor for patients with age >50 years, MANEC pathology grade and metastasis.

Appendiceal Neoplasms ; complications ; diagnosis ; surgery ; Carcinoma, Neuroendocrine ; complications ; diagnosis ; therapy ; Female ; Gastrointestinal Neoplasms ; Humans ; Male ; Middle Aged ; Neuroendocrine Tumors ; complications ; diagnosis ; surgery ; Prognosis ; Retrospective Studies

Merkel cell carcinoma (MCC) is a rare aggressive neuroendocrine carcinoma of the skin that shows locoregional or distant metastasis. Metastasis of MCC to body cavity effusion is extremely rare; only three cases have been reported so far. Metastatic MCC in effusion cytology shows small blue round cells with fine stippled chromatin like other small blue round cell tumors such as small cell lung carcinoma or lymphoma. The diagnosis of metastatic MCC can grant patients good chances at recently advanced therapeutic options. Here, we present a case of metastatic MCC to pleural effusion with characteristic single file-like pattern.
Carcinoma, Merkel Cell ; Carcinoma, Neuroendocrine ; Chromatin ; Diagnosis ; Financing, Organized ; Humans ; Lymphoma ; Neoplasm Metastasis ; Pleural Effusion ; Skin ; Small Cell Lung Carcinoma

PURPOSE: To report a case of poorly differentiated neuroendocrine carcinoma of the eyelid. CASE SUMMARY: A 70-year-old male presented with a 5-month history of a right upper eyelid mass. The mass appeared as 1.2 × 1.2 cm on the right upper eyelid. A mass excision was performed under frozen section control. The tumor was completely excised with a safety margin clearance and an upper eyelid reconstruction was performed. Histopathological examination revealed a tumor composed of small atypical cells which showed a high nuclear/cytoplasm ratio, nuclear molding, and increased mitotic activity. Immunohistochemical examination revealed positive reactivity for Ki-67, synaptophysin, CD56, and negative reactivity for chromogranin, cytokeratin 20, and thyroid transcription factor-1. CONCLUSIONS: Primary neuroendocrine carcinoma of the eyelid is extremely rare, but the tumor has high malignancy and readily metastasizes. Poorly differentiated neuroendocrine carcinoma should be considered in the differential diagnosis of a rapidly growing eyelid mass.
Aged ; Carcinoma, Merkel Cell ; Carcinoma, Neuroendocrine ; Carcinoma, Small Cell ; Diagnosis, Differential ; Eyelids ; Frozen Sections ; Fungi ; Humans ; Keratin-20 ; Male ; Synaptophysin ; Thyroid Gland

Primary neuroendocrine tumors originating from the extrahepatic bile duct are rare. Among these tumors, large cell neuroendocrine carcinomas (NECs) are extremely rare. A 59-year-old man was admitted to Sanggye Paik Hospital with jaundice that started 10 days previously. He had a history of laparoscopic cholecystectomy, which he had undergone 12 years previously due to chronic calculous cholecystitis. Laboratory data showed abnormally elevated levels of total bilirubin 15.3 mg/dL (normal 0.2–1.2 mg/dL), AST 200 IU (normal 0–40 IU), ALT 390 IU (normal 0–40 IU), and gamma-glutamyl transferase 1,288 U/L (normal 0–60 U/L). Serum CEA was normal, but CA 19-9 was elevated 5,863 U/mL (normal 0–37 U/mL). Abdominal CT revealed a 4.5 cm sized mass involving the common bile duct and liver hilum and dilatation of both intrahepatic ducts. Percutaneous transhepatic drainage in the left hepatic duct was performed for preoperative biliary drainage. The patient underwent radical common bile duct and Roux-en-Y hepaticojejunostomy for histopathological diagnosis and surgical excision. On histopathological examination, the tumor exhibited large cell NEC (mitotic index >20/10 high-power field, Ki-67 index >20%, CD56 [+], synaptophysin [+], chromogranin [+]). Adjuvant concurrent chemotherapy and radiotherapy were started because the tumor had invaded the proximal resection margin. No recurrence was detected at 10 months by follow-up CT.
Bile Duct Neoplasms ; Bile Ducts, Extrahepatic* ; Bilirubin ; Carcinoma, Neuroendocrine* ; Cholecystectomy, Laparoscopic ; Cholecystitis ; Common Bile Duct ; Diagnosis ; Dilatation ; Drainage ; Drug Therapy ; Follow-Up Studies ; Hepatic Duct, Common ; Humans ; Jaundice ; Liver ; Middle Aged ; Neuroendocrine Tumors ; Radiotherapy ; Recurrence ; Synaptophysin ; Tomography, X-Ray Computed ; Transferases