LARGE CELL METASTATIC PANCREATIC NEUROENDOCRINE CARCINOMA TREATED WITH SOMATOSTATIN ANALOGUES - CASE REPORT AND LITERATURE REVIEW.

We report the case of a 55-year-old-male with a large cell metastatic pancreatic neuroendocrine carcinoma treated for 14 months with lanreotide autogel having a stable disease (SD) and not responding to chemotherapy. The somatostatin analogues (SSA) were introduced after an episode of diarrhea and controlled the disease. Progression-free survival (PFS) as determined by Computerized Tomography (CT) scans was obtained for 14 months. After more than a year, the patient's health state deteriorated along with progressive disease. The capecitabine-temozolomide regimen was challenged, but after three cycles, a rapid clinical decline was noted. CONCLUSION: This unexpected event (diarrhea) in the course of the disease could represent the beginning of carcinoid syndrome. While the lanreotide autogel helped the episode of diarrhea pass, it also helped gain control over the disease itself.

PROGNOSTIC FACTORS IN PATIENTS WITH SURGICAL RESECTION OF PANCREATIC NEUROENDOCRINE TUMOURS.

CONTEXT: Pancreatic neuroendocrine tumours (PanNETs) are rare pancreatic neoplasms. PanNETs can be treated by multimodal approach including surgery, locoregional and systemic therapy. OBJECTIVE: The aim of the present study is to evaluate predictive factors of overall survival in patients with PanNETs surgically treated at a single center. SUBJECTS AND METHODS: The study group consisted of 120 patients with PanNETs who had undergone surgery at the Center of Digestive Diseases and Liver Transplantation of Fundeni Clinical Institute, Bucharest, Romania. Surgical resection of the primary tumor was performed in 110 patients. RESULTS: Tumor size > 2 cm (p=0.048) (90% CI) lymph node involvement (p=0.048), ENET grade (p<0.001), distant metastases (p<0.001), Ki 67 index (<2%, 2-5%, 5-10%, 10-20%, >20%) (p<0.001) were identified as significant prognostic factors for OS on univariate analysis. Using multivariate Cox proportional regression model we found that distant metastases and Ki 67 index were independent risk factors for the survival outcome. CONCLUSIONS: Surgery with curative intent should be considered in all cases if clinically appropriate and technically feasible. High grade (Ki67 index ≥10%) tumours were associated with a 2- fold increase in risk of death as compared to those with a Ki67 <10%.

Rare cause of intestinal obstruction - submucous lipoma of the sigmoid colon.

The lipoma of the colon is a benign and rare tumor. Most lipomas are asymptomatic, their discovery being fortuitous. The diagnosis is ussualy easy by colonoscopy associated with biopsies. The abdominal CT scan also has its role in the diagnostic process and in the assesment of the tumoral extension.The treatment depends essentially on the clinical picture, on the size and location of the lipoma and involves endoscopic or surgical excision. We present the case of a 56 years old woman in which a random colonoscopic and than tomographic diagnosis of a sigmoidian lipoma was made 2 years ago when the patient presented with different symptoms, the submucosal lipoma being small sized at the time; the surgical treatment(sigmoidectomy including the tumor) was currently indicated by the sub-occlusive syndrome and haematochezia, due to the intraluminal proliferation of the tumor.

The Todani classification for bile duct cysts: an overview.

Bile duct cysts are a rare medical condition and are more frequent in children. However, the disease is becoming increasingly common in adults. The modified Todani classification, which is based on anatomical characteristics, is the current standard classification method. However, this classification does not take the following factors into consideration: different epidemiology, pathogenesis, risk of malignant transformation, clinical and imaging aspects, and different therapeutical approaches for all the bile duct cysts. Thus, some clinicians denied its clinical significance and viability. Moreover, some rare variants (i.e., cystic duct cysts) of bile duct cysts were initially not included and were subsequently categorized as type VI. Although it clusters different diseases, the Todani classification of bile duct cysts should also be used in clinical practice because it is simple, reproducible and widely agreed upon, thereby allowing an appropriate comparative analysis between different series of patients who are classified based on this scheme. Exceptional, cystic duct cysts should be included in the Todani classification (as a subtype of type II BDC rather than as a "new" type VI) so that the gastroenterologists, radiologists and surgeons are aware of this variation.

Fibromuscular dysplasia--a rare cause of renovascular hypertension. Case study and overview of the literature data.

Renal artery stenosis (RAS) is associated with increased cardiovascular mortality and morbidity and may constitute a treatable cause of secondary hypertension. Fibromuscular dysplasia is frequently affecting children as the main cause of RAS, but is very rare in adults. We present the case of a 19-year-old overweight patient, with no known pathological conditions in her medical history or family background, admitted for severe, pulsing headaches during the past 3 months and increased blood pressure (BP) values for about a month (maximum BP 220/140 mmHg). The initial clinical exam and first-line imagistic methods did not provide a high suspicion for RAS. However, the invasive methods established the diagnosis of right renal artery medial dysplasia. Balloon angioplasty was the treatment of choice.

Updates in MRI characterization of the thymus in myasthenic patients.

PURPOSE: To evaluate the imaging appearance of the thymus in the myasthenic patients by using chemical shift magnetic resonance imaging, and, to correlate the chemical shift ratio (CSR) with pathologic findings after surgical excision. MATERIALS AND METHODS: In the past year, a total of 11 myasthenic patients (4 males, 7 females; age range of 26-65 years), have been investigated by MRI centered at the thymic lodge. Our protocol included a Dual-Echo technique, T1-weighted In-phase/Opposed-phase MR images in all patients. A chemical shift ratio (CSR) was calculated by comparing the signal intensity of the thymus gland with that of the chest wall muscle for quantitative analysis. For this purpose, we have used standard region-of-interest electronic cursors at a slice level of the maximum axial surface of the thymus. We have identified two patients groups: a thymic hyperplasia group and a thymic tumoral group. RESULTS: With the decrease in the signal intensity of the thymus gland at chemical shift, the MR imaging was evident only in the hyperplasia group. The mean CSR in the hyperplasia group was considerably lower than that in the tumor group, 0,4964 ± 0,1841, compared with 1,0398 ± 0,0244. The difference in CSR between the hyperplasia and tumor groups was statistically significant (P=0,0028). CONCLUSION: MR imaging using T1-weighted In-phase/Opposed-phase images could be a useful diagnostic tool in the preoperative assessment of the thymic lodge and may help differentiate thymic hyperplasia from tumors of the thymus gland.

The thymus in myasthenic patients: correlation between mediastinal CT imaging and histopathological findings.

PURPOSE: To correlate computed tomographic (CT) appearance of the thymus with results from histologic examination of thymic tissue in myasthenic patients who underwent thymectomy. MATERIALS AND METHODS: A retrospective study, based on case series report, between January 2000 and December 2010 on 247 patients with generalized myasthenia gravis or myasthenic syndrome explored by CT using a specific protocol, in the Radiology and Medical Imaging Department of Fundeni Clinical Institute. All subjects in the study were operated and had a histologic evaluation. RESULTS: CT examinations of these 247 subjects showed remaining thymic mass, remnant of thymic tissue, fatty infiltration of the thymus and tumoral thymus or focal thymic mass. The results of histologic examination showed normal thymus, thymic lymphoid follicular hyperplasia, thymic atrophy, fatty infiltration and thymoma. CONCLUSION: It was a 100% correlation between CT examinations and intra-operative findings regarding the presents of focal thymic masses in our study. In the diagnostic of focal thymic mass, the only inter-disciplinary correlation is between radiological and macroscopic aspects. CT examination has a limited value in differential diagnosis between thymic lymphoid follicular hyperplasia and thymoma.

Updates in CT characterization of thymic epithelial tumors in patients with myasthenia gravis.

Thymic epithelial tumors have been traditionally classified into two main types: noninvasive and invasive thymoma. Several classifications have been proposed for thymic tumors, but according to these classifications, the prognosis of patients with thymomas varies considerably. Our purpose is to present different CT aspects according to various subtypes of thymic epithelial neoplasms based on the simplified World Health Organization classification. In this article, we will discuss and illustrate histologic and functional features of the thymus and a spectrum of thymic tumors associated with Myasthenia Gravis. Smooth contours and a round shape are the most suggestive of type A thymic epithelial tumors, whereas irregular contours and heterogeneous enhancement are the most suggestive of type C tumors. Calcifications are suggestive of type B tumors. CT findings may serve as predictors of postoperative recurrence or metastasis for the thymic epithelial tumors.

Isolated splenic metastasis of endometrial adenocarcinoma--a case report.

The spleen in rarely the place for solid, non-haematological tumors, isolated splenic metastases from adenocarcinomas being extremely rare findings, regardless of the origin and the histological type of the primary tumor. We present the case of a female patient with isolated splenic metastasis diagnosed by abdominal computer tomography at only 20 months after curative surgery for endometrial adenocarcinoma, in which the final diagnosis has been established by histological and immunohistochemical examination of the splenectomy piece. The haematogenous dissemination of the endometrial cancer occurs most commonly in the lungs, liver or bones, the spleen being rarely affected. In the medical literature there are cited up to date only 12 cases of solitary splenic metastasis from endometrial adenocarcinoma. The particularity of the case presented by us is the early appearance of an isolated splenic metastasis, at less than two years after curative surgery (compared to an average of 4-5 years cited in the literature), from an endometrial cancer which was classified histologicaly in the group with low-risk for relapse (well differentiated endometrioid adenocarcinoma). In conclusion, although solitary splenic secondary determinations are very rare, the incidence of the reported cases in the medical literature is increasing, their late appearance (a few years after the primary tumor's resection) and the lack of symptoms until the tumor reaches appreciable size or it complicates with necrosis, justifies the periodic abdominal imaging examination, on long-term, for postoperative monitorisation after the initial curative surgery. Their treatment of choice is open, classical splenectomy that must be followed by chemotherapy in order to prevent the development of other possible micrometastases.

[Insulinoma of the pancreas: analysis of a clinical series of 30 cases]. / Insulinomul pancreatic--analiza unei serii clinice de 30 de cazuri.

UNLABELLED: Insulinoma is the most frequent neuroendocrine pancreatic tumor and is the main cause for hypoglicemia due to endogenous hyperinsulinism. We performed an analysis of a clinical series in order to study the clinical and biological spectrum of presentation, the preoperatory imagistic diagnosis and results of the surgical approach. Between 1986-2009, 30 patients with symptoms suggesting an insulinoma were hospitalized in our department. Preoperatory localization of insulinomas was possible in 16 patients. The most sensitive imagistic methods were ecoendoscopy and magnetic resonance. Intraoperatory ultrasound was performed in 16 patients and its sensitivity in detection of insulinomas was 93%; the combination between intraoperative ultrasound and manual exploration of pancreas by the surgeon reached a 100% sensitivity. Before the intraoperatory ultrasound was used the tumor excision was predominantly done by extensive pancreatic resection, while after this was available in our centre more conservative (enucleo-resection) procedures were chosen. In 1 patient the resection was done by laparoscopy, and in 1 patient by robotic surgery. The dimensions of the tumor were less than 2 cm in most of the patients; 2 had nesidioblastosis and 2 had multiple insulinomas; all 28 patients proved to have benign insulinomas at histological specimens. Following surgery, the symptoms disappear in all patients. The most common complication following extensive pancreatic resections was acute pancreatitis, while after enucleation pancreatic fistula occurred more frequently. CONCLUSIONS: Due to small dimensions, the preoperative diagnosis of insulinomas is usually difficult, ecoendoscopy being the most sensitive method. Intraoperative ultrasound is essential for insulinoma localization and for chosing the optimal type of excision. Enucleation is the resection method to be chosen whenever this it is technical possible. In benign insulinomas the prognosis is excellent, surgical resection being curative in all cases.

[Helical CT and diagnostic evaluation of cranio-facial fibrous dysplasia]. / Scanner spiralé et bilan diagnostique de dysplasie fibreuse cranio-faciale.

PURPOSE: To evaluate the role of helical CT in the evaluation of skull abnormalities seen in fibrous dysplasia. MATERIALS AND METHODS: Prospective study on 6 cases with cranio-facial fibrous dysplasia (3 males and 3 females) aged 8 to 59 years old, evaluated with plain radiographs, helical CT and, in 1 case, MRI. Helical CT was performed in 3 cases on a CT Twin scan (Elscint), in 3 cases on a Somatom Plus scan (Siemens). Multiplanar reconstructions and 3D reconstructions were done in all cases. 4 cases had a histopathological confirmation of fibrous dysplasia lesions by bone-biopsy (3 cases) or surgical biopsy (1 case). RESULTS: The 6 cases included sclerotic form in 3 cases, cystic variety in 1 case, polyostotic variety in 1 case, mixed form in 1 case. Topographic localizations of fibrous dysplasia were skull base involvement in 1 case, multizonal skull involvement in 4 cases, maxillo-mandibulary involvement (cherubism) in 1 case. CONCLUSION: Helical CT is an optimal method to evaluate the skull lesions of fibrous dysplasia. The review of source images must always be done. 3-dimensional bone reconstructions are better to visualize the anterior cranial vault and skull base dysmorphy. Helical acquisition is a compromise in case by case between the dose of irradiation and the quality of source images.