Well-differentiated grade 3 digestive neuroendocrine tumors: Myth or reality? The PRONET study group.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 4129-4129 ◽  
Author(s):  
Jean-Yves Scoazec ◽  
Anne Couvelard ◽  
Genevieve Monges ◽  
Emmanuelle Leteurtre ◽  
Genevieve Belleannee ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Who Classification ◽  
Epidemiologic Study ◽  
Histologic Grade ◽  
World Health ◽  
Ki67 Index ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Incident Cases

4129 Background: In contrast to the 2000 World Health Organization (WHO) classification of digestive neuroendocrine tumors (NET) in which morphologic differentiation was the first criterion, the 2010 WHO classification of NET is based mostly on histologic grade. NET are now classified into three main categories: NET G1 (mitotic count <2/10 HPF and/or ≤2% Ki67 index), NET G2 (2-20/10 HPF and/or 3-20%), and neuroendocrine carcinoma (NEC) of small or large cell type. While NET G1 and G2 are well-differentiated tumors, NEC are considered poorly differentiated G3 tumors. We looked at the agreement between grade and differentiation to determine whether all NET can be readily classified according to the 2010 WHO classification. Methods: We designed a 1-year prospective, epidemiologic study to assess the characteristics of newly diagnosed NET, including diagnostic pathology. From August 2010 to July 2011, all pathology laboratories in France were invited to register all incident cases of gastroenteropancreatic (GEP) and thoracic NET, excluding small cell carcinoma. For GEP-NET, investigators were asked to indicate morphologic differentiation (according to WHO 2000) and elements of histologic grade (mitotic index, Ki67 index), according to ENETS. Results: Of 500 invited centers, 80 participated; 1417 incidental cases were included and 77 excluded (duplicates or exclusion criteria), totaling 1340 cases; 778 (58.1%) were GEP-NET; 660/778 (85%) were well differentiated, 72 (9%) poorly differentiated, and 46 (6%) adenocarcinoid, nonclassified, or not evaluable; 422 (54.2%) were G1, 220 (28%) G2, 104 (13.5%) G3, and 32 (4.1%) had missing grades. Of those deemed G3, 72 (69%) were described as poorly differentiated, 21 (20%) as well differentiated (mean Ki67 index 35%, range 25%-60%), and 11 (10.5%) as adenocarcinoid. Conclusions: In this prospective, epidemiologic study, overall agreement between grade and differentiation was good. However, a significant proportion of G3 NET were classified as well differentiated and thus unclassifiable by 2010 WHO classification. This group of tumor deserves to be included in future classifications to help the clinician decide whether they should be treated as NET G1/G2 or NEC G3.

scholarly journals Mixed adenoneuroendocrine carcinoma of the hepatic bile duct: a case report and review of the literature

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Sulai Liu ◽  
Zhendong Zhong ◽  
Meng Xiao ◽  
Yinghui Song ◽  
Youye Zhu ◽  
...  
Keyword(s):  
Bile Duct ◽  
Neuroendocrine Tumors ◽  
Radical Surgery ◽  
Malignant Tumors ◽  
World Health ◽  
Ki 67 ◽  
Mixed Adenoneuroendocrine Carcinoma ◽  
Hilar Bile Duct ◽  
Hilar Tumor

Abstract Background The World Health Organization's updated classification of digestive system neuroendocrine tumors in 2010 first proposed the classification of mixed adenoneuroendocrine carcinoma (MANEC). The incidence of biliary malignant tumors with neuroendocrine tumors accounts for less than 1% of all neuroendocrine tumors. Moreover, the incidence of hilar bile duct with MANEC is very rare. Case presentation A 65-year-old female patient came to our hospital for repeated abdominal pain for more than 4 months and skin sclera yellow staining for 1 week. Contrast-enhanced computed tomography imaging and magnetic resonance results suggested a hilar tumor for Bismuth-Corlette Type II. The patient underwent radical surgery for hilar cholangiocarcinoma. Finally, the patient was diagnosed with hilar bile duct MANEC, staged 1 (pT1N0M0) based on the eighth edition of the AJCC. Histopathology showed that the tumor was a biliary tumor with both adenocarcinoma and neuroendocrine carcinoma. No evidence of recurrence and metastasis after 20 months of follow-up. Conclusions We first reported a MANEC that originated in the hilar bile duct. As far as we known, there were few reports of biliary MANEC, and the overall prognosis was poor. We also found that the higher the Ki-67 index, the worse the prognosis of this type of patient. Radical surgery is the most effective treatment.

Lung Tumors With Neuroendocrine Morphology: Essential Radiologic and Pathologic Features

2008 ◽  
Vol 132 (7) ◽  
pp. 1055-1061 ◽  
Author(s):  
Teri J. Franks ◽  
Jeffrey R. Galvin
Keyword(s):  
Neuroendocrine Tumors ◽  
Classification Systems ◽  
World Health ◽  
National Library ◽  
Molecular Features ◽  
Distinct Subset ◽  
Pathologic Features ◽  
The World ◽  
Health Organization

Abstract Context.—Tumors with neuroendocrine morphology are a distinct subset of lung neoplasms sharing characteristic histologic, immunohistochemical, ultrastructural, and molecular features. Objective.—To review the current histologic classification and the diagnostic criteria for the major categories of neuroendocrine tumors of the lung. Data Sources.—Published classification systems from the World Health Organization and pertinent peer-reviewed articles indexed in PubMed (National Library of Medicine) form the basis of this review. Conclusions.—Accurate classification of the neuroendocrine tumors of the lung requires knowledge of specific criteria separating the major categories, which is essential for determining prognosis and treatment.

Hemorrhagic Suprasellar Central Nervous System Embryonal Tumor in an Adult: Uncommon Features of an Extremely Rare Neoplasm

2021 ◽  
Author(s):  
Martina Piloni ◽  
Filippo Gagliardi ◽  
Michele Bailo ◽  
Lina Raffaella Barzaghi ◽  
Marcella Callea ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Who Classification ◽  
Optic Chiasm ◽  
World Health ◽  
Embryonal Tumors ◽  
Embryonal Tumor ◽  
First Case ◽  
Diffusion Pattern

Abstract Background Occurrences of suprasellar central nervous system (CNS) embryonal tumors in adults are extremely rare. Hemorrhagic onset is further uncommon, with only anecdotic cases reported in the literature. The authors describe the case of a 57-year-old man affected by a suprasellar CNS embryonal tumor, with hemorrhagic onset and a unique diffusion pattern along the optic pathways. Material and Methods A 57-year-old man presenting with acute visual acuity worsening and left homonymous hemianopia was referred to our hospital. Neuroradiologic studies demonstrated an infiltrating, high-grade lesion involving the optic chiasm and right retrochiasmatic pathways with a hemorrhagic area in the ipsilateral pulvinar. Results The patient underwent microsurgical biopsy. Pathologic assessment confirmed the diagnosis of CNS embryonal tumor, not otherwise specified (NOS) according to the 2016 World Health Organization (WHO) classification of CNS tumors. The patient was referred to a multimodal adjuvant treatment; he eventually died 4 months after surgery. Competent literature has been systematically reviewed in the light of the relevant changes made in the last version of the WHO classification. Conclusion Embryonal tumors should be considered in the differential diagnosis for sellar and suprasellar space-occupying lesions, despite the rarity of the disease and the uncommon features at time of presentation. As per our knowledge, this is the first case ever described of hemorrhagic suprasellar embryonal tumor with a diffusion pattern along white matter fibers. Histogenesis, biomolecular and neuroradiologic features, and classification of embryonal tumors are an open field of research, with considerable implications for the definition of better diagnostic pitfalls and therapeutic regimens.

Acute Leukemia

2016 ◽  
Author(s):  
Richard A. Larson ◽  
Roland B Walter
Keyword(s):  
Acute Myeloid Leukemia ◽  
Acute Lymphoblastic Leukemia ◽  
Acute Leukemia ◽  
Myeloid Leukemia ◽  
Who Classification ◽  
Lymphoblastic Leukemia ◽  
World Health ◽  
Acute Leukemias ◽  
Acute Myeloid

The acute leukemias are malignant clonal disorders characterized by aberrant differentiation and proliferation of transformed hematopoietic progenitor cells. These cells accumulate within the bone marrow and lead to suppression of the production of normal blood cells, with resulting symptoms from varying degrees of anemia, neutropenia, and thrombocytopenia or from infiltration into tissues. They are currently classified by their presumed cell of origin, although the field is moving rapidly to genetic subclassification. This review covers epidemiology; etiology; classification of leukemia by morphology, immunophenotyping, and cytogenetic/molecular abnormalities; cytogenetics of acute leukemia; general principles of therapy; acute myeloid leukemia; acute lymphoblastic leukemia; and future possibilities. The figure shows the incidence of acute leukemias in the United States. Tables list World Health Organization (WHO) classification of acute myeloid leukemia and related neoplasms, expression of cell surface and cytoplasmic markers for the diagnosis of acute myeloid leukemia and mixed-phenotype acute leukemia, WHO classification of acute lymphoblastic leukemia, WHO classification of acute leukemias of ambiguous lineage, WHO classification of myelodysplastic syndromes, European LeukemiaNet cytogenetic and molecular genetic subsets in acute myeloid leukemia with prognostic importance, cytogenetic and molecular subtypes of acute lymphoblastic leukemia, terminology used in leukemia treatment, and treatment outcome for adults with acute leukemia. This review contains 1 highly rendered figure, 9 tables, and 117 references.

Haematopathology of classic myeloproliferative neoplasms

2020 ◽  
pp. 45-62
Author(s):  
Hans Michael Kvasnicka ◽  
Jürgen Thiele
Keyword(s):  
Continuous Improvement ◽  
Who Classification ◽  
Myeloproliferative Neoplasms ◽  
Primary Myelofibrosis ◽  
World Health ◽  
Polycythaemia Vera ◽  
The World ◽  
Health Organization ◽  
Diagnostic Importance

The classification of the World Health Organization (WHO) continues to advocate the diagnostic importance of bone marrow (BM) morphology in the diagnostic workup of myeloproliferative neoplasms (MPN). In this regard, distinctive histological BM patterns characterize specific subtypes of MPN and are the key to a meaningful clinical and molecular-defined risk stratification of patients. In this regard, the morphological denominator includes a characteristic megakaryocytic proliferation along with variable changes in the granulopoiesis and erythropoiesis. Importantly, diagnosis of MPN requires absence of relevant dysgranulopoiesis or dyserythropoiesis. In terms of clinical practice, the concept of precursor stages provides the possibility of an early intervention by appropriate therapeutic regimens that might prevent fatal complications like thrombosis and haemorrhage, especially in early stages of polycythaemia vera or in primary myelofibrosis. However, the WHO classification is not aimed to capture all biological true cases of MPN or guarantee a complete diagnostic specificity and thus might be in need of continuous improvement following clinical experience.

Intestinal-Type Adenocarcinoma of the Sinonasal Tract: A Clinicopathologic Study of 18 Cases

1993 ◽  
Vol 79 (3) ◽  
pp. 205-210 ◽  
Author(s):  
Carmelo Urso ◽  
Maria Benedetta Ninu ◽  
Alessandro Franchi ◽  
Milena Paglierani ◽  
Roberto Bondi
Keyword(s):  
Intestinal Type ◽  
Presenting Symptoms ◽  
Clinicopathologic Study ◽  
Clinicopathologic Findings ◽  
Poorly Differentiated ◽  
Intestinal Type Adenocarcinoma ◽  
Aggressive Tumors ◽  
Well Differentiated ◽  
Colonic Adenocarcinomas

Background Intestinal-type adenocarcinoma (ITAC) of the nose and paranasal sinuses is a relatively rare tumor. It commonly affects subjects exposed to wood or leather dust. Methods The authors present the clinicopathologic findings of 18 cases of sinonasal ITACs and review the proposed histologic classifications. Results All patients, except one, were males; mean age was 60 years (range, 41-79); in 9 cases an occupational exposure to wood or leather dust was found. Common presenting symptoms were epistaxis, nasal obstruction and rhinorrhea. Histologically, tumors were divided into four groups: well-differentiated (G1) ITACs = 3 cases; moderately differentiated (G2) ITACs = 8 cases; poorly differentiated (G3) ITACs = 2 cases; mucinous (M) ITACs = 5 cases. Immunocytochemically, 16/17 cases were positive for carcinoembryonal antigen, 1/17 for somatostatin, and 0/16 cases for gastrin. Conclusions Sinonasal ITACs are aggressive tumors, often diagnosed in a relatively advanced stage. Owing the close similarity of the microscopic aspects, a histologic classification of ITACs analogous to that of colonic adenocarcinomas is proposed.

scholarly journals Congenital eruption cyst: a case report

2010 ◽  
Vol 21 (3) ◽  
pp. 259-262 ◽  
Author(s):  
Ramón Manuel Alemán Navas ◽  
María Guadalupe Martínez Mendoza ◽  
Mário Roberto Leonardo ◽  
Raquel Assed Bezerra da Silva ◽  
Henry W. Herrera ◽  
...  
Keyword(s):  
Case Report ◽  
Tooth Extraction ◽  
Who Classification ◽  
World Health ◽  
Close Monitoring ◽  
Cystic Lesions ◽  
Separate Entity ◽  
Epithelial Cysts ◽  
Health Organization

Congenital pathologies are those existing at or dating from birth. Occurrence of congenital cystic lesions in the oral cavity is uncommon in neonates. Eruption cyst (EC) is listed among these unusual lesions. It occurs within the mucosa overlying teeth that are about to erupt and, according to the current World Health Organization (WHO) classification of epithelial cysts of the jaws, EC is a separate entity. This paper presents a case of congenital EC successfully managed by close monitoring of the lesion, without any surgical procedure or tooth extraction. Eruption of the teeth involved, primary central incisors, occurred at the fourth month of age. During this time neither the child nor mother had any complication such as pain on sucking, refusal to feed, airway obstruction, or aspiration of fluids or teeth.

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