scholarly journals Appendiceal neuroendocrine neoplasms: diagnosis and management

2015 ◽  
Vol 23 (1) ◽  
pp. R27-R41 ◽  
Author(s):  
Krystallenia I Alexandraki ◽  
Gregory A Kaltsas ◽  
Simona Grozinsky-Glasberg ◽  
Eleftherios Chatzellis ◽  
Ashley B Grossman
Keyword(s):  
Right Hemicolectomy ◽  
Proliferation Index ◽  
Neuroendocrine Neoplasms ◽  
Distinct Entity ◽  
Ki 67 ◽  
Prospective Data ◽  
Diagnosis And Management ◽  
Management Protocol ◽  
Consensus Statements

Gastrointestinal neuroendocrine neoplasms (GI-NENs) are increasingly being recognised, while appendiceal NENs (aNENs) currently constitute the third most common GI-NEN. Appendiceal NENs are generally considered to follow an indolent course with the majority being localised at diagnosis. Thus, the initial surgical approach is not that of a planned oncological resection. Due to the localised nature of the disease in the majority of cases, subsequent biochemical and radiological assessment are not routinely recommended. Histopathological criteria (size, mesoappendiceal invasion, Ki-67 proliferation index, neuro- and angio-invasion) are mainly used to identify those patients who are also candidates for a right hemicolectomy. Goblet cell carcinoids are a distinct entity and should be treated as adenocarcinomas. Despite the absence of any substantial prospective data regarding optimal management and follow-up, recent consensus statements and guidelines have been published. The purpose of this review is to overview the published studies on the diagnosis and management of appendiceal NENs and to suggest a possible management protocol.

scholarly journals Ghost cell odontogenic carcinoma: Role of p53 as predictor of progression

2020 ◽  
Vol 10 (1) ◽  
pp. 1675-1678
Author(s):  
Pallavi Srivastava ◽  
Nidhi Anand ◽  
Nuzhat Husain
Keyword(s):  
Distant Metastases ◽  
Proliferation Index ◽  
Ghost Cell ◽  
Ki 67 ◽  
Local Recurrences ◽  
Odontogenic Carcinoma ◽  
Fibular Flap ◽  
Slow Growing

Ghost cell odontogenic carcinoma (GCOC) a rare malignant Odontogenic Carcinoma with an unpredictable behaviour presenting with local recurrences and distant metastases, to best of our knowledge about 38 cases have been reported in the past. This is an additional case of GCOC in a 25-year old female presented with a slow-growing mandibular swelling since 9 months with restricted jaw mobility. The CT scan showed an ill-defined osteodestructive lesion in the mandible. The histological examination confirms the diagnoses as a GCOC. Immunohistochemical examination was performed for Ki-67 proliferation index and p53 a predictor of progression. This case was managed by wide surgical resection of tumor and reconstruction of the defect by free fibular flap. Six months follow- up period shows no signs of recurrence. GCOC is rare Odontogenic Carcinoma with unpredictable behaviour however p53 & Ki67 proliferation index can predict the progression of tumor and help in differentiation from benign precursor lesions as early diagnosis & treatment of GCOC is necessary to prevent local recurrences & distant metastases.

scholarly journals Changing biological behaviour of NETs during the evolution of the disease: progress on progression

2021 ◽  
Vol 28 (5) ◽  
pp. R121-R140
Author(s):  
Krystallenia I Alexandraki ◽  
Ariadni Spyroglou ◽  
Stylianos Kykalos ◽  
Kosmas Daskalakis ◽  
Georgios Kyriakopoulos ◽  
...  
Keyword(s):  
Cochrane Library ◽  
Neuroendocrine Neoplasms ◽  
Disease Course ◽  
Review Of The Literature ◽  
Biological Behaviour ◽  
Ki 67 ◽  
Future Studies ◽  
Biological Phenotype ◽  
Shed Light

Following improvements in the management and outcome of neuroendocrine neoplasms (NENs) in recent years, we see a subset, particularly of pancreatic NENs, which become more aggressive during the course of the disease. This is reflected by an increase in the Ki-67 labelling index, as a marker of proliferation, which may lead to an occasion of increase in grading, but generally does not appear to be correlated with histologically confirmed dedifferentiation. A systematic review of the literature was performed in PubMed, Cochrane Library, and Embase until May 2020 to identify cases that have behaved in such a manner. We screened 244 articles: only seven studies included cases in their cohort, or in a subset of the cohort studied, with a proven increase in the Ki-67 during follow-up through additional biopsy. In addition to these studies, we have also tried to identify possible pathophysiological mechanisms implicated in advanced NENs, although currently no studies appear to have addressed the mechanisms implicated in the switch to a more aggressive biological phenotype over the course of the disease. Such progression of the disease course may demand a change in the management. Summarising the overall evidence, we suggest that future studies should concentrate on changes in the molecular pathways during disease progression with sequential biopsies in order to shed light on the mechanisms that render a neoplasm more aggressive than its initial phenotype or genotype.

scholarly journals Multifocal recurrence of medulloblastoma: a long follow-up case study

2020 ◽  
Vol 29 (1) ◽  
pp. 93-9
Author(s):  
Benny Zulkarnaien ◽  
Edwin Suharlim ◽  
Eka Susanto ◽  
Soehartati Argadikoesoema Gondhowiardjo
Keyword(s):  
Immunohistochemical Staining ◽  
Pediatric Patients ◽  
Proliferation Index ◽  
Ki 67 ◽  
Negative Results ◽  
Axial Mass ◽  
Positive Results ◽  
Multifocal Recurrence

Medulloblastoma is the most common malignant brain tumor in pediatric patients. Leptomeningeal dissemination often occurs in the spinal canal and rarely into the supratentorial as an extra-axial lesion. This study followed an 8-year progression of multifocal medulloblastoma recurrence as supratentorial metastasis that mimics an intra-axial mass of the temporal lobe. Pathologic examination of the temporal mass showed densely packed small round blue cells that are distributed in a ribboning pattern with areas of necrosis, rosette formation, and mitosis. Ki-67 immunohistochemical staining of the tumor showed a high proliferation index of 40% and revealed positive results for synaptophysin and negative results for glial fibrillary acidic protein and β-catenin. A long follow-up is essential to diagnose the recurrence and elucidate the progression of medulloblastoma.

Primary Cutaneous Small/Medium CD4+ T-Cell Lymphomas: A Heterogeneous Group of Tumors With Different Clinicopathologic Features and Outcome

2008 ◽  
Vol 26 (20) ◽  
pp. 3364-3371 ◽  
Author(s):  
Adriana Garcia-Herrera ◽  
Luis Colomo ◽  
Mireia Camós ◽  
Joaquín Carreras ◽  
Olga Balague ◽  
...  
Keyword(s):  
T Cell ◽  
Prognostic Significance ◽  
Heterogeneous Group ◽  
Automated Image Analysis ◽  
Proliferation Index ◽  
Image Analysis System ◽  
Ki 67 ◽  
Cutaneous Lymphomas ◽  
Analysis System

Purpose To define the clinical and pathologic characteristics of primary cutaneous small/medium CD4+ T-cell lymphoma (PCSM-TCL) and identify parameters of prognostic significance. Patients and Methods We have investigated 24 patients with primary cutaneous lymphomas composed of small/medium mature T-cells with a βF1, CD3, CD4+ and/or noncytotoxic, CD8– and CD30– phenotype. The proliferation index and CD8+ infiltrating cells were quantified with an automated image analysis system. Results Sixteen patients presenting with solitary or localized plaques or small nodules (< 3 cm) had an indolent course. Only three patients experienced repeated cutaneous relapses, and none of them died as a result of the disease after 1 to 168 months (median, 17 months) of follow-up. The tumors had a low proliferation (median Ki-67, 9% ± 5%) and an intense infiltrate of reactive CD8+ (median, 20% ± 11.7%). Five patients presenting with rapidly evolving large tumors or nodules (≥ 5 cm) had an aggressive disease and died with extracutaneous dissemination 18 to 36 months after diagnosis (median, 23 months). These tumors had a significantly higher proliferation (median Ki-67, 22% ± 11.3%; P < .05) and lower number of infiltrating CD8+ (median, 1% ± 3%; P < .05) than the previous group. A third group of three patients had a peculiar clinical presentation with multifocal relapsing lesions without extracutaneous dissemination after a long period of follow-up ranging from 41 to 92 months. Histologically, these cases had an intense infiltrate of eosinophils. Conclusion PCSM-TCL is a heterogeneous group of tumors with differentiated clinical and pathological characteristics with impact in the outcome of the patients.

Cytokeratin 7 negative invasive breast carcinoma: clinicopathological and immunohistochemical analysis of 14 cases with clinical follow-up

2015 ◽  
Vol 68 (6) ◽  
pp. 484-487 ◽  
Author(s):  
Neda A Moatamed ◽  
Annie Wu ◽  
Khaled Sarah ◽  
Sophia K Apple
Keyword(s):  
Lymph Node ◽  
Lymph Node Metastases ◽  
Proliferation Index ◽  
Axillary Lymph ◽  
Cytokeratin 7 ◽  
Ki 67 ◽  
Her 2 ◽  
Node Metastases ◽  
Work Up

Cytokeratin 7 (CK 7) negative breast tumours are reported to occur rarely. We studied 14 CK 7 negative cases of primary invasive ductal carcinoma (IDC) detected during sentinel lymph node metastases work-up and immunohistochemistry panel in the work-up of metastatic carcinoma of unknown origin. Axillary lymph node metastases were present in seven patients (50%). Oestrogen receptor (ER) was strongly positive in all cases: progesterone receptor in 78%, Her-2/neu in 7% and high proliferation index with Ki-67 >20% was seen in 71% of the cases.Metastatic and/or recurrence were found in 8 of 14 patients (57%) with the mean clinical follow-up of 55 months. Metastatic sites include multiple bones, brain, spinal cord, liver, pancreas, ovary, lung, lymph node other than ipsilateral axillary and skin. 12 of 14 patients received adjuvant chemotherapy. All 14 patients received hormonal therapy and radiation therapy. Morphologically, IDC with neuroendocrine features was noted in 57%. Synaptophysin stain was positive in 57% and chromogranin was positive in 21% of the cases. In conclusion, these CK 7 negative breast carcinomas were ER positive, mostly Her-2/neu negative, had high Ki-67 and frequently showed neuroendocrine differentiation. More than half of these cases had a poor outcome.

scholarly journals Diagnostic and Interventional Role of Endoscopic Ultrasonography for the Management of Pancreatic Neuroendocrine Neoplasms

2021 ◽  
Vol 10 (12) ◽  
pp. 2638
Author(s):  
Giuseppinella Melita ◽  
Socrate Pallio ◽  
Andrea Tortora ◽  
Stefano Francesco Crinò ◽  
Antonio Macrì ◽  
...  
Keyword(s):  
Tumor Burden ◽  
Pancreatic Tumors ◽  
Proliferation Index ◽  
Neuroendocrine Neoplasms ◽  
Ki 67 ◽  
Ethanol Injection ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Reduce Tumor Burden ◽  
Precise Diagnosis

Pancreatic neuroendocrine neoplasms (PanNENs) are relatively rare, but their incidence has increased significantly in the last decades. Precise diagnosis and prognostic stratification are crucial for proper patient management. Endoscopic ultrasound (EUS) is the modality of choice for diagnosis of solid pancreatic tumors, showing a higher tumor detection rate than other imaging modalities, especially for small size lesions. EUS also serves as a guide for preoperative sampling and other interventions. EUS-tissue acquisition is a safe and highly accurate technique for cyto/histological diagnosis of PanNENs with a well-demonstrated correlation between Ki-67 proliferation index values and tumor grading on EUS and surgical specimens according to the WHO 2017 classification. Furthermore, the possibility of a preoperative EUS-guided fine needle tattooing or fiducial markers placement may help the surgeon to locate small and deep tumors, thus avoiding formal pancreatic resections in favor of parenchymal-sparing surgery. Finally, locoregional ablative treatments using either ethanol injection or radiofrequency ablation have been proposed in recent studies with promising results in order to control symptoms or reduce tumor burden in selected patients unfit for surgery with functioning or non-functioning PanNENs. This article review highlights the current role of EUS in PanNENs management, focusing on the present and future applications of EUS-guided interventions.

scholarly journals Correlation of Somatostatin Receptor 1–5 Expression, [68Ga]Ga-DOTANOC, [18F]F-FDG PET/CT and Clinical Outcome in a Prospective Cohort of Pancreatic Neuroendocrine Neoplasms

Cancers ◽  
2021 ◽  
Vol 14 (1) ◽  
pp. 162
Author(s):  
Susanna Majala ◽  
Tiina Vesterinen ◽  
Hanna Seppänen ◽  
Harri Mustonen ◽  
Jari Sundström ◽  
...  
Keyword(s):  
Prospective Study ◽  
Fdg Pet ◽  
Somatostatin Receptor ◽  
Proliferation Index ◽  
Neuroendocrine Neoplasms ◽  
Ki 67 ◽  
Pet Ct ◽  
Sstr Subtype ◽  
Node Metastases ◽  
Fdg Pet Ct

Purpose: The aim of this study was to correlate immunohistochemical (IHC) tissue levels of SSTR1-5 with the receptor density generated from [68Ga]Ga-DOTANOC uptake in a prospective series of NF-PNENs. Methods: Twenty-one patients with a total of thirty-five NF-PNEN-lesions and twenty-one histologically confirmed lymph node metastases (LN+) were included in this prospective study. Twenty patients were operated on, and one underwent endoscopic ultrasonography and core-needle biopsy. PET/CT with both [68Ga]Ga-DOTANOC and [18F]F-FDG was performed on all patients. All histological samples were re-classified and IHC-stained with monoclonal SSTR1-5 antibodies and Ki-67 and correlated with [68Ga]Ga-DOTANOC and [18F]F-FDG PET/CT. Results: Expression of SSTR1-5 was detected in 74%, 91%, 80%, 14%, and 77% of NF-PNENs. There was a concordance of SSTR2 IHC with positive/negative [68Ga]Ga-DOTANOC finding (Spearman’s rho 0.382, p = 0.043). All [68Ga]Ga-DOTANOC-avid tumors expressed SSTR2 or SSTR3 or SSTR5. Expression of SSTR5 was higher in tumors with a low Ki-67 proliferation index (PI) (−0.353, 95% CI −0.654–0.039, p = 0.038). The mean Ki-67 PI for SSTR5 positive tumors was 2.44 (SD 2.56, CI 1.0–3.0) and 6.38 (SD 7.25, CI 2.25–8.75) for negative tumors. Conclusion: SSTR2 was the only SSTR subtype to correlate with [68Ga]Ga-DOTANOC PET/CT. Our prospective study confirms SSTR2 to be of the highest impact for SST PET/CT signal.

Surgical Management of Patients with Neuroendocrine Neoplasms of the Appendix: Appendectomy or More

2017 ◽  
Vol 106 (3) ◽  
pp. 242-251 ◽  
Author(s):  
Nikhil Pawa ◽  
Ashley K. Clift ◽  
Helai Osmani ◽  
Panagiotis Drymousis ◽  
Andrzej Cichocki ◽  
...  
Keyword(s):  
Lymph Node ◽  
Lymph Node Metastases ◽  
Residual Disease ◽  
Disease Recurrence ◽  
Right Hemicolectomy ◽  
Neuroendocrine Neoplasms ◽  
Number Of Patients ◽  
Node Metastases ◽  
Current Guidelines

Background: Appendiceal neuroendocrine neoplasms (ANEN) are mostly indolent tumours treated effectively with simple appendectomy. However, controversy exists regarding the necessity of oncologic right hemicolectomy (RH) in patients with histologic features suggestive of more aggressive disease. We assess the effects of current guidelines in selecting the surgical strategy (appendectomy or RH) for the management of ANEN. Methods/Aims: This is a retrospective review of all ANEN cases treated over a 14-year period at 3 referral centres and their management according to consensus guidelines of the European and the North American Neuroendocrine Tumor Societies (ENETS and NANETS, respectively). The operation performed, the tumour stage and grade, the extent of residual disease, and the follow-up outcomes were evaluated. Results: Of 14,850 patients who had appendectomies, 215 (1.45%) had histologically confirmed ANEN. Four patients had synchronous non-ANEN malignancies. One hundred and ninety-three patients had index appendectomy. Seventeen patients (7.9%) had lymph node metastases within the mesoappendix. Forty-nine patients underwent RH after appendectomy. The percentages of 30-day morbidity and mortality after RH were 2 and 0%, respectively. Twelve patients (24.5%) receiving completion RH were found to have lymph node metastases. Two patients had liver metastases, both of them synchronous. The median follow-up was 38.5 months (range 1-143). No patient developed disease recurrence. Five- and 10-year overall survival for all patients with ANEN as the only malignancy was both 99.05%. Conclusions: The current guidelines appear effective in identifying ANEN patients at risk of harbouring nodal disease, but they question the oncological relevance of ANEN lymph node metastases. RH might present an overtreatment for a number of patients with ANEN.

scholarly journals Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management

2018 ◽  
Vol 7 (2) ◽  
pp. 268-277 ◽  
Author(s):  
Ashley K Clift ◽  
Oskar Kornasiewicz ◽  
Panagiotis Drymousis ◽  
Omar Faiz ◽  
Harpreet S Wasan ◽  
...  
Keyword(s):  
Goblet Cell ◽  
Fdg Pet ◽  
Right Hemicolectomy ◽  
Ca 125 ◽  
Neuroendocrine Neoplasms ◽  
Class A ◽  
Heated Intraperitoneal Chemotherapy ◽  
Pet Ct ◽  
Fdg Pet Ct

Goblet cell carcinomas (GCC) are a rare, aggressive sub-type of appendiceal tumours with neuroendocrine features, and controversy exists with regards to therapeutic strategy. We undertook a retrospective review of GCC patients surgically treated at two tertiary referral centres. Clinical and histopathological data were extracted from a prospectively maintained database. Survival analyses utilised Kaplan–Meier methodology. Twenty-one patients were identified (9 females). Median age at diagnosis was 55 years (range 32–77). There were 3, 6 and 9 grade 1, 2 and 3 tumours, respectively. One, 10, 5 and 5 patients had stage I, II, III and IV disease at diagnosis, respectively. There were 8, 10 and 3 Tang class A, B and C tumours, respectively. Index operation was appendectomy (n = 12), right hemicolectomy (n = 6) or resections including appendix/right colon, omentum and the gynaecological system (n = 3). Eight patients underwent completion right hemicolectomy. Surgery for recurrence included small bowel resection (n = 2), debulking with peritonectomy and heated intraperitoneal chemotherapy, and hysterectomy and bilateral salpingo-oophorectomy (all n = 1). Median follow-up was 30 months (range 2.5–123). One-, 3- and 5-year OS was 79.4, 60 and 60%, respectively. Mean OS (1-, 3-, and 5-year OS) for Tang class A, B and C tumours were 73.1 months (85.7, 85.7, 51.4%), 83.7 months (all 66.7%) and 28.5 months (66.7, 66.7%, not reached), respectively. Chromogranin A/B and 68Ga-DOTATATE PET/CT were not useful in follow-up, but CEA, CA 19-9, CA 125 and 18F-FDG PET/CT identified tumour recurrence. GCC must be clearly discriminated from relatively indolent appendiceal neuroendocrine neoplasms. 18F-FDG PET/CT and CEA/CA19-9/CA 125 are useful in detecting recurrence of GCC.

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