Well-Differentiated Neuroendocrine (Carcinoid) Tumors of the Extrahepatic Biliary Ducts

2010 ◽  
Vol 134 (7) ◽  
pp. 1075-1079
Author(s):  
Yvonne Saldanha Noronha ◽  
Anwar Sultana Raza

Abstract Primary, well-differentiated neuroendocrine (carcinoid) tumors of the extrahepatic biliary ducts are an uncommon cause of biliary obstruction. As compared to cholangiocarcinomas, which are more commonly seen at this location, these tumors tend to behave less aggressively, and only one-third metastasize. Tumor size (>2 cm) appears to be the best predictor of aggressive behavior. Surgery is the mainstay of treatment and complete resection offers prolonged disease-free survival. Accurate preoperative diagnosis is therefore important and can be made by examining brush cytology specimens obtained during endoscopic retrograde cholangiopancreatography and/or endoscopic ultrasound-guided fine-needle aspiration. It is important to keep this entity in mind, especially when examining cytologic or small biopsy specimens, so that appropriate immunohistochemical stains can be used to arrive at the correct diagnosis.

2018 ◽  
Vol 108 (2) ◽  
pp. 109-120 ◽  
Author(s):  
Atsuko Kasajima ◽  
Björn Konukiewitz ◽  
Naomi Oka ◽  
Hiroyoshi Suzuki ◽  
Akira Sakurada ◽  
...  

The clinicopathological features of lung neuroendocrine neoplasms (NEN) with a high proliferative index at the border area between atypical carcinoid and neuroendocrine carcinoma have not been investigated so far. The aim of this study was, therefore, to search for lung NENs which are well differentiated but show Ki67 values that overlap with those of poorly differentiated (PD)-NENs. Resected lung NENs from 244 Japanese patients were reviewed, and Ki67 indices were assessed in all tumors. The data were then correlated to clinicopathological parameters and patient outcome. Among 59 (24%) well-differentiated (WD)-NENs and 185 (76%) lung PD-NENs, 7 were defined as WD-NENs with Ki67 indices > 20%. The Ki67 indices of these tumors (mean 29%, range 24–36) were significantly lower than those of PD-NENs (mean 74%, range 34–99). All WD-NENs with Ki67 > 20% lacked abnormal p53 and loss of retinoblastoma 1 (Rb1) expression. In contrast, many PD-NENs expressed p53 (48%) and showed loss of Rb1 (86%). The 2- and 5-year disease-free survival rates in WD-NEN patients with Ki67 > 20% were lower than those of WD-NEN patients with Ki67 ≤20% (p < 0.01 for disease-free and overall survival). No statistical differences were detected between outcome of WD-NEN patients with Ki67 > 20% and those of PD-NEN. It is concluded that WD-NEN patients with Ki67 > 20% share the morphological and immunohistochemical features of WD-NEN patients with Ki67 ≤20%, but they have a worse prognosis, suggesting that this tumor group requires particular attention in future classifications and probably new therapeutic regimes.


2012 ◽  
Vol 30 (4_suppl) ◽  
pp. 406-406
Author(s):  
Joshua S. Hill ◽  
Safia Rafeeq ◽  
Matthew H.G. Katz ◽  
Michael J. Overman ◽  
Laura A. Lambert ◽  
...  

406 Background: Well-differentiated appendiceal adenocarcinomas (WDAA) are rare tumors characterized by peritoneal spread. Lymph node metastasis can occur, yet the association between nodal spread and recurrence is poorly understood. Methods: A single institution retrospective review of patients seen between August 1993 and January 2010 with a pathologic diagnosis of WDAA who underwent colectomy was conducted. Patients with zero lymph nodes found during pathologic review were excluded. Parameters evaluated included demographics, presence of lymph node metastasis, completeness of cytoreduction and time to recurrence. Results: Of 688 patients with appendiceal neoplasms, 160 (23.3%) had WDAA. The mean age at diagnosis was 50.7 years and 81 (50.6%) were male. Median follow-up after diagnosis was 58.5 months. One hundred patients (62.5%) had regional or distant metastasis present at the time of colectomy. Seventy-eight (48.8%) colectomies were performed at outside institutions. The median number of nodes examined was 12. Twelve patients (7.5%) were found to have nodal metastasis. The rate of peritoneal metastasis did not correlate with the presence of nodal metastasis (node positive 9/12, 75% versus node negative 91/148, 61.5%; p=0.35). No difference in the ability to perform complete cytoreduction existed for those with and without nodal metastasis (66% versus 51% p=0.19). Examining patients with complete cytoreduction and ≥ 12 months of follow-up, there was an increased risk of recurrence among patients with lymphatic metastasis compared to those without (5/8, 62.5% versus 15/61, 24.6%; p=0.03). In this subset, median disease-free-survival in lymph node positive patients was 53 months compared to 109.1 months in patients without nodal metastasis (p=0.08). Conclusions: Patients with WDAA tumors have an overall favorable prognosis; however, patients with lymph node metastasis appear to have an increased risk of recurrence and apparent shortened disease free survival. Right colectomy may be warranted in this patient population.


Blood ◽  
1974 ◽  
Vol 43 (2) ◽  
pp. 181-189 ◽  
Author(s):  
Philip S. Schein ◽  
Bruce A. Chabner ◽  
George P. Canellos ◽  
Robert C. Young ◽  
Costan Berard ◽  
...  

Abstract The evaluation of the results of CVP and MOPP chemotherapy in 80 patients with advanced stages of non-Hodgkin’s lymphoma indicates that 36 (45%) achieved a complete remission. Twenty-eight per cent of the entire group of patients remain free of disease for periods ranging from 4 mo to over 7 yr, with a projected median duration of complete remission of 3½ yr. Significant differences in prognosis relative to histologic categories were found. Well-differentiated and nodular histology were positive determinants for improved median survival, confirming the over-all clinical validity of the Rappaport classification system for the non-Hodgkin’s lymphomas. The median survival for patients in the most clinically aggressive subgroups with diffuse histology is inferior to those with nodular patterns or well-differentiated cells. In this study it was demonstrated that it was possible to achieve a significant number of complete remissions even in the most aggressive histologic subgroups using combination chemotherapy, and these responses can be correlated with an extended disease-free survival without further therapy.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e22531-e22531
Author(s):  
Daniela Greto ◽  
Carlotta Becherini ◽  
Calogero Saieva ◽  
Giulio Francolini ◽  
Domenico Andrea Campanacci ◽  
...  

e22531 Background: Liposarcoma (LPS) are classified into subtypes: well-differentiated (WDLPS), de-differentiated (DDLPS), myxoid (MLPS) and pleomorphic (PLS). We report a single institution cohort of patients with LPS, undergoing surgery and radiotherapy, to explore prognostic factors related to outcome and toxicity. Methods: This retrospective analysis included 186 LPS patients. Patient, tumor, and treatment variables were analyzed for local recurrence (LR-DFS) and distant metastasis (DM-DFS) disease free survival and overall survival (OS). Results: At a median follow-up of 8.6 years (range, 0.1-27.3 years), Kaplan-Meier (KM) survival analysis showed that LR, DM and OS were 75.5%, 76.6% and 48.1%, respectively. KM analysis showed that Age>56, DDLPS and lower limb localization were related to LR (p=0,001, p=0,0001 and p=0,0001, respectively). Association between LR, Age and DDLPS persisted both at univariate (p=0,003 and p=0,0001, respectively) and multivariate Cox regression (CR) analysis (p=0,024 and p=0,002). Age, tumor depth and grading influenced distant recurrence, both at KM (p=0,023, p = 0.026 and p = 0.016) and univariate CR (p=0,026, p=0,042 and p=0,012). Age and grading were confirmed at multivariate analysis (p=0,009 and p 0,017). Patients with WDLPS and wide excision had significantly better OS (p=0,001 and p=0,03, respectively), while histologic G3 and age>56 were related with worse OS (p= 0,008 and p=0,0001, respectively). Age, DDLPS and Grade were related to OS at univariate (p=0,0001, p=0,0001 and p=0,03, respectively) and multivariate CR analysis (p=0,031, p=0,0001 and p=0,001, respectively). Conclusions: Our analysis confirmed that Grade, tumor depth and histological subtype influenced survival. Further studies are needed in order to explore the influence of hystopathologic features on treatment outcomes. [Table: see text]


Sarcoma ◽  
2001 ◽  
Vol 5 (4) ◽  
pp. 209-213 ◽  
Author(s):  
Robert U. Ashford ◽  
Jeremy Stanton ◽  
Farid Khan ◽  
Jean A. S. Pringle ◽  
Stephen R. Cannon ◽  
...  

Purpose:We reviewed all tumours of the sternum referred to The London Bone and Soft Tissue Tumour Service between 1956 and 1997 inclusive.Patients and results:There were eight patients with this pathology, the male to female ratio was 3:1 and their mean age was 53 years. Of these patients, three are alive and disease free, one is alive with recurrence, and four have died, two of the consequences of the disease and two of unrelated causes. Surgery is the principal treatment of these tumours both for excision and subsequent reconstruction.Discussion:Extended disease-free survival is possible with correct diagnosis, complete excision at the first operation, appropriate skeletal reconstruction, adequate skin cover and appropriate postoperative support and follow-up.


2010 ◽  
Vol 76 (7) ◽  
pp. 759-763 ◽  
Author(s):  
Paul Avenel ◽  
Alasdair McKendrick ◽  
Sumet Silapaswan ◽  
Ramachandra Kolachalam ◽  
William Kestenberg ◽  
...  

Carcinoid tumors are slow-growing and usually become symptomatic late in the course of the disease. We evaluated our 10-year experience in the management of GI carcinoid tumors. The records of 133 patients with GI carcinoids were reviewed. The rectum was the most common site for carcinoid tumors with an incidence of 30 per cent followed by jejunoileal at 29.3 per cent. Other sites of carcinoid tumors were the appendix (8.3%), colon (8.3%), and duodenum (3.8%). Endoscopy was the most helpful modality in diagnosing GI carcinoids. CT was not helpful in preoperative diagnosis of carcinoid tumor. Fifteen patients died in follow-up with eight deaths related to carcinoid tumors, in the small bowel (6), rectum (1), and colon (1). Overall survival was 68.7 per cent and mortality rate was 19.5 per cent from carcinoid tumors. Most of the deaths occurred in patients with carcinoid syndrome, synchronous malignancy, and malignant carcinoid tumors. The mean disease-free survival was 51 months (range, 15 to 138 months). Screening colonoscopy, in addition to decreasing colorectal adenocarcinoma mortality, is useful in diagnosing carcinoid tumors at an earlier stage and in decreasing mortality from malignant colorectal carcinoid tumors.


1997 ◽  
Vol 15 (12) ◽  
pp. 3458-3463 ◽  
Author(s):  
K Omura ◽  
A Nagasato ◽  
E Kanehira ◽  
H Kinsen ◽  
S Amaya ◽  
...  

PURPOSE We analyzed retinoblastoma protein (pRB) and proliferating-cell nuclear antigen (PCNA) expression in primary tumors and recurrent lesions of well-differentiated papillary thyroid carcinoma (PTC) to clarify the relationship between their expression and recurrent disease. PATIENTS AND METHODS The study included 93 patients with PTC. No recurrent disease had developed in 60 patients within 10 years after surgery (group N). Thirty patients in whom recurrent disease had developed after surgery were enrolled in group R. Levels of pRB and PCNA expression were quantified using the CAS 200 system (Cell Analysis Systems, Elmhurst, IL) following immunohistochemical staining. RESULTS Mean pRB expression level in the primary tumors in group R was significantly lower than that in group N (P < .0001). pRB expression in the tumors with a diameter up to 20 mm was significantly lower than that in tumors larger than 20 mm in group R (P < .01). There were no significant differences in the levels of expression of PCNA in the primary tumors between group N and group R. Univariate analysis demonstrated that the disease-free survival was significantly correlated with pN category, pRB, and PCNA expression level. The subgroup with high-level expression of pRB (> 25%) showed significantly long disease-free survival (P < .001). Furthermore, the subgroup with low-level expression of PCNA (< 35%) showed significantly longer disease-free survival (P < .05). Multivariate analysis showed pRB expression and pN category to be independent prognostic factors for disease-free survival in PTC. CONCLUSION pRB expression level can be used as a reliable predictor for recurrence of PTC.


2015 ◽  
Vol 64 (02) ◽  
pp. 159-165 ◽  
Author(s):  
Nonso Osakwe ◽  
Navnett Narula ◽  
Jeffrey Port ◽  
Subroto Paul ◽  
Brendon Stiles ◽  
...  

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