Primary ovarian neuroendocrine tumor arising in association with a mature cystic teratoma: A case report

Primary ovarian carcinoid tumors are exceptionally rare entities accounting for approximately 0.1% of all ovarian neoplasms. This report describes a primary ovarian neuroendocrine tumor arising in association with a mature cystic teratoma in a 65 year-old woman. Macroscopically, the unilateral adnexal tumor was composed of cystic, solid and mucinous elements which resolved into a dual component lesion histologically. The majority of the tumor displayed an organoid architecture with mild to moderate pleomorphism and no discernible mitotic activity, while approximately 10% consisted of sheets and groups of cells with highly pleomorphic nuclei, necrosis and occasional mitoses. Features of a mature cystic teratoma were seen very focally. Immunohistochemistry revealed strong, diffuse positivity for CD56 and synaptophysin. Chromogranin immunonegativity was noted and there was an absence of nuclear β-catenin accumulation. Ki-67 index was 10–12%. Although there is no established diagnostic framework for primary ovarian carcinoid tumors, this case was diagnosed as a well-differentiated neuroendocrine tumor, Grade 2 (intermediate grade), arising in association with a mature cystic teratoma/dermoid cyst. This case highlights the need to develop ovarian diagnostic criteria in this area

Tumeur pancréatique rare de découverte fortuite chez un enfant en Côte d’Ivoire

La tumeur pseudo-papillaire et solide du pancréas (TPPSP) est une tumeur rare. Elle touche le plus souvent la femme jeune. Décrite la première fois par Frantz en 1959, sa pathogénie demeure peu claire. C’est une tumeur de bon pronostic qui nécessite une chirurgie radicale. Les auteurs rapportent un cas de TPPSP chez une fillette de 11 ans. La symptomatologie était aiguë, faite de syndrome de compression et d’épigastralgies. L’examen trouvait une masse solide de l’hypochondre gauche. Le scanner montrait une masse de structure mixte de la queue du pancréas. Une splénopancréatectomie gauche était réalisée. Le diagnostic était confirmé par l’examen histologique avec immunohistochimie. Le suivi à long terme ne montrait pas de récidive. Le recul est de deux ans et demi. À travers cette observation et une revue de la littérature, les auteurs discutent la contribution de la radiologie dans le diagnostic et insistent sur une chirurgie radicale dans le traitement de ces tumeurs de faible degré de malignité

Utility of histopathological revision in the management of gastro-entero-pancreatic neuroendocrine neoplasia

Background: Histological evaluation and grading assessment are key points in the diagnostic work-up of gastroentero-pancreatic neuroendocrine neoplasms (GEP-NENs). Aim: To analyze the impact of histopathological revision on the clinical management of patients with GEP-NEN. Materials and methods: Patients referred to our Center of Excellence between 2015 and 2021 were included in this study. Immunohistochemical slides at the time of initial diagnosis were reviewed to assess tumor morphology, diagnostic immunohistochemistry, and Ki67. Results: 101 patients were evaluated, with 65 (64.4%) gastrointestinal, 25 (24.7%) pancreatic, and 11 (10.9%) occult neoplastic lesions suspected to be of GEP origin. The main changes resulting from the revision were: first Ki-67 assessment in 15.8% of patients, Ki-67 change in 59.2% of patients and grading modification in 23.5% of patients. An additional immunohistochemical evaluation was performed in 78 (77.2%) patients, leading to a confirmation of GEP origin in 10 of 11 (90.9%) of unknown primary site neoplastic lesions and an exclusion of NEN diagnosis in 2 (2%) patients. After histopathological revision, a significant modification in clinical management was proposed in 42 (41.6%) patients. Conclusions: Histopathological revision in a referral NEN center is strongly advised in newly diagnosed GEP-NENs to properly plan prognostic stratification and therapeutic choice

Efficacy of a pre-S containing vaccine in patients receiving lamivudine prophylaxis after liver transplantation for chronic hepatitis B

Lamivudine monoprophylaxis against hepatitis B virus (HBV) reinfection after liver transplantation is associated with recurrence due to escape mutants and second generation recombinant HBV vaccine is not effective. We studied the efficacy of two courses each of three double-doses (20 ug) of third-generation recombinant pre-S containing vaccine (Sci-B-Vac™) in 20 patients on lamivudine prophylaxis at a median of 637 days (range, 390-2666 days) after transplantation. At enrollment, all patients were seronegative for HBsAg, anti-HBs and HBVDNA (by qPCR). Lamivudine (100 mg/day) was continued throughout the study. Five patients (25%) responded to the first course and five additional patients responded after the second course (overall response rate 50%). The response rate was 88% in patients younger than 50 years old and 25% in older patients (p = 0.02). The median peak anti-HBs titer was 153 mIU/mL with six responders having a titer >100 mIU/mL and seven sustained >6 months. Among seven previous nonresponders to second generation recombinant vaccine, three (44%) responded. At the end of the study, all patients remained seronegative for HBsAg. In conclusion, Sci-B-Vac™ is effective in about 50% of patients receiving lamividine prophylaxis and may prevent recurrence due to escape mutants. © 2006 The Authors.postprin

Relevance of angiogenesis in neuroendocrine tumors

While traditional cytotoxic drugs have shown limited efficacy in neuroendocrine tumors (NETs), their biological features have been characterized and can be exploited therapeutically. Their most prominent trait is an extraordinary vascularization in low-grade NETs and an hypoxia-dependent angiogenesis in high-grade NETs, which is associated to a significant expression of many pro-angiogenic molecules. Therefore, several antiangiogenic compounds have been tested in these malignancies and among these, sunitinib has demonstrated activity in pancreatic NET patients by dually targeting the VEGFR and PDGFR pathways. In spite of these efficacious clinical results, apparent resistance to antiangiogenic therapies has been described in NETs animal models and in clinical trials. Therefore, overcoming anti-angiogenic resistance is a crucial step in the subsequent development of antiangiogenic therapies. Several strategies have been postulated to fight resistance, but pre-clinical studies and clinical trials will investigate and address these therapeutic approaches in the coming years in order to overcome resistance anti-angiogenic therapies in NETs

Collagenous gastritis : a rare cause of anaemia in childhood

We report a thirteen year old boy presenting with severe iron deficiency anaemia. Initial extensive investigation failed to reveal an obvious cause. Subsequently a diagnosis of collagenous gastritis was made. To our knowledge this is the first report of a patient with this rare condition in the Maltese Islands.peer-reviewe

Colloid Carcinoma of Gallbladder - Incidental Finding of a Rare Entity

Majority gall bladder carcinomas are conventional adenocarcinomas of pancreatobiliary type. Mucinous carcinomas in gall bladder constitutes 2.5% of all gallbladder carcinomas. Pure mucinous (colloid carcinomas) are even rarer. Its rarity deserves this case being highlighted. Less than twenty-five cases of mucinous carcinomas of the gallbladder have been reported so far. A 46-year-old female presented with recurrent right upper quadrant pain along with nausea and vomiting for last one year. Liver function tests & kidney function tests were within range. USG Abdomen revealed an enlarged gallbladder measuring 8x5 cm with no stones. No other abnormalities were found on USG. A simple cholecystectomy was performed for chronic cholecystitis. Gross examination of specimen showed glistening, globular, soft and cystic gall bladder filled with mucoid tenacious yellowish material. There was no discernible mass lesion. Microscopic examination revealed pools of extracellular mucinous material dissecting muscular wall containing few signet ring cells. A diagnosis of colloid or pure mucinous adenocarcinoma of the gallbladder was made. Pure mucinous carcinomas as seen in exocrine glands like breast, pancreas and skin are very uncommon in gall bladder. Mucinous variant (>50% stromal mucin) are most of the time admixed with conventional type. Mucinous carcinomas most of the time are large and advanced at the time of diagnosis and are aggressive in nature than conventional type. This case is presented owing to its extreme rarity

Two Separated Ileal Adenocarcinomas in Neurofibromatosis Type 1

Patients with neurofibromatosis-1 (NF-1) have increased susceptibility to a variety of malignancies. Here, we document a rare case of two separated ileal adenocarcinomas in NF-1. The adenocarcinomas were surrounded by a diffuse tubular adenomatous lesion of the mucosa, and ganglion cells were scattered in the NF background. We found this case meaningful for several reasons: two separated adenocarcinomas arising in an unusual ileal segment, the association with precancerous tubular adenoma, and the presence of ganglion cells, which suggests ganglioneuromatosis in NF-1