Primary Prostatic Carcinoma With De Novo Diffuse Neuroendocrine Differentiation

2021 ◽  
pp. 106689692110358
Author(s):  
Laurence A. Galea ◽  
Christopher Mow ◽  
Samson W. Fine ◽  
Paul Manohar

The 2016 World Health Organization classification of prostate cancer with neuroendocrine (NE) differentiation includes NE cells in usual prostate cancer, adenocarcinoma with Paneth cell-like NE differentiation, well-differentiated NE tumor (carcinoid), small cell NE carcinoma, and large cell NE carcinoma. In this article, we report a rare case of primary prostatic carcinoma with de novo diffuse NE differentiation presenting with bilateral hydronephrosis in a 79-year-old man. This case did not fit into any of the existing classifications. The clinical, radiological, morphological, and immunohistochemical findings and response to androgen deprivation therapy (ADT) are presented. The proposed pathogenesis of NE differentiation via transdifferentiation from conventional prostatic adenocarcinoma whereby genomic alterations, coupled with ADT can induce lineage plasticity resulting in NE differentiation is described.

2019 ◽  
Vol 27 (8) ◽  
pp. 893-899
Author(s):  
Laura G. Pastrián ◽  
Ignacio Ruz-Caracuel ◽  
Raul S. Gonzalez

Primary neuroendocrine neoplasms of the liver have occasionally been reported in the liver, though many reports do not convincingly exclude metastases. In this article, we report 2 “giant” hepatic neuroendocrine lesions without evidence of a primary elsewhere after clinical workup. One occurred in a 21-year-old male; the lesion was a large cell neuroendocrine carcinoma measuring 24 cm. The patient died of disease in 10 months. The other occurred in a 25-year-old patient, was 18 cm wide, and was diagnosed as a well-differentiated neuroendocrine tumor, World Health Organization grade 3. The patient died of disease after 30 months. Molecular testing demonstrated only the presence of TP53 mutations in common. These cases expand our knowledge of seemingly primary neuroendocrine neoplasms of the liver, in particular, giant cases measuring more than 8 cm. Guidelines for clinical workup and therapy for these lesions remain unclear, but future thorough workup of such cases is necessary for specific characterization.


2017 ◽  
Vol 7 (2) ◽  
pp. 1221-1223 ◽  
Author(s):  
Nirajan Mainali ◽  
Niraj Nepal ◽  
Prabesh Kumar Choudhary ◽  
Amrita Sinha ◽  
Saroj Rajbanshi ◽  
...  

A mixed adenoneuroendocrine carcinoma is a tumor composed of both adenocarcinoma and neuroendocrine carcinoma components, with each comprising  at least one-third of the lesion, as defined by the World Health Organization classification of neuroendocrine neoplasms in 2010.. A 67-years-old male was admitted to the hospital with symptoms suggesting gastric cancer. Histopathology examination from endoscopic biopsy revealed adenocarcinoma. Later partial gastrectomy specimen examination the lesion show presence of well differentiated adenocarcinoma along with neuro endocrine carcinoma.


2007 ◽  
Vol 131 (7) ◽  
pp. 1110-1116
Author(s):  
M. Tarek Elghetany

Abstract Context.—Pediatric myelodysplastic syndromes (MDSs) are uncommon disorders, which may be difficult to diagnose, particularly in the absence of increased blasts. Pediatric MDSs have several unique features including their association with inherited/constitutional disorders in approximately one third of patients. The classification of pediatric MDSs has undergone significant evolution in the past 20 years. Objective.—To critically review existing classifications of pediatric MDSs and to evaluate their applicability on previously published large series. Data Sources.—Previously published pediatric MDS series containing more than 10 patients from the English literature between 1982 and 2005. Conclusions.—Data were available on 887 patients from 13 published series. Most cases (68.7%) were idiopathic/ de novo, 23.9% were associated with constitutional/inherited disorder, and 7.4% were therapy related. Approximately 10% of cases could not be classified by the French-American-British classification. Eighty-seven percent of unclassified cases were appropriately classified using the World Health Organization classification (2001), whereas 96% of them were classified with the modified World Health Organization classification for pediatric MDSs (2003). The impact of cytogenetics and constitutional/inherited disorders on the biology and outcome of the disease needs to be studied further.


Blood ◽  
2020 ◽  
Vol 136 (1) ◽  
pp. 24-35 ◽  
Author(s):  
Anna L. Brown ◽  
Christopher N. Hahn ◽  
Hamish S. Scott

Abstract Recognition that germline mutations can predispose individuals to blood cancers, often presenting as secondary leukemias, has largely been driven in the last 20 years by studies of families with inherited mutations in the myeloid transcription factors (TFs) RUNX1, GATA2, and CEBPA. As a result, in 2016, classification of myeloid neoplasms with germline predisposition for each of these and other genes was added to the World Health Organization guidelines. The incidence of germline mutation carriers in the general population or in various clinically presenting patient groups remains poorly defined for reasons including that somatic mutations in these genes are common in blood cancers, and our ability to distinguish germline (inherited or de novo) and somatic mutations is often limited by the laboratory analyses. Knowledge of the regulation of these TFs and their mutant alleles, their interaction with other genes and proteins and the environment, and how these alter the clinical presentation of patients and their leukemias is also incomplete. Outstanding questions that remain for patients with these germline mutations or their treating clinicians include: What is the natural course of the disease? What other symptoms may I develop and when? Can you predict them? Can I prevent them? and What is the best treatment? The resolution of many of the remaining clinical and biological questions and effective evidence-based treatment of patients with these inherited mutations will depend on worldwide partnerships among patients, clinicians, diagnosticians, and researchers to aggregate sufficient longitudinal clinical and laboratory data and integrate these data with model systems.


2002 ◽  
Vol 10 (3) ◽  
pp. 175-177 ◽  
Author(s):  
Slobodan Dozic ◽  
Dubravka Cvetkovic-Dozic ◽  
Milica Skender-Gazibara ◽  
Branko Dozic

(Conclusion) Classifications of the nervous system tumors should be neither static nor definitive. The most recent, third, current WHO classification of nervous system tumors was published in 2000. Many substantial changes were introduced. New entities include the chordoid glioma of the third ventricle, the atypical teratoid/rhabdoid tumor, cerebellar liponeurocytoma (the former lipomatous medulloblstoma of the cerebellum), solitary fibrous tumor and perineurioma. The new tumor variants include the large cell medulloblastoma, tanacytic ependymoma and rhabdoid meningioma. Several essential changes were introduced in the meningiomas regarding histological subtypes, grading and proliferation index. In addition to new entities described in the 2000 WHO classification there are newly brain tumor entities and tumor variants, which are not included in the current classification due to the insufficient number of reporeted cases, for example papillary glioneuronal tumor, rosetted glioneuronal tumor, lipoastrocytoma and lipomatous meningioma. They will be probably accepted in the next WHO classificaton. In the current WHO classification the importance of cytogenetic and molecular biologic investigation in the understanding and further classifications of these tumors has been emphasized.


2016 ◽  
Vol 60 (2) ◽  
Author(s):  
I.C. Šoštarić-Zuckermann ◽  
K. Severin ◽  
M. Huzak ◽  
M. Hohšteter ◽  
A. Gudan Kurilj ◽  
...  

<p>Circumanal gland tumors are very common neoplasms of dogs. Their classification relies on microscopic examination and is further supported by a few immunohistochemical markers that help indicate their prognosis. However, new additional tests would be highly useful. The purpose of this study was to develop such a test using fractal analysis which is increasingly being applied in science, especially in the field of biomedicine. A total of 53 circumanal gland tumors were chosen from our department archives. After a precise histological classification according to the World Health Organization classification, the number of <em>de novo</em> classified samples was as follows: 15 adenomas, 11 epitheliomas, 21 well differentiated carcinomas, 6 poorly differentiated carcinomas. Ten samples of normal circumanal gland were also included as control. All samples were immunohistochemicaly stained with vimentin. All immunohistochemical reactions were photographed at two different magnifications -100X and 400X and converted to 1 bit in black and white (bitmap) images thus enhancing the positive vimentin reactions. These images were used for the assessment of fractal dimension applying the <em>box counting method</em> and computer software <em>Fractalyse</em>. To determine the significance of results, conventional statistics were performed using Statistica software. The overall vimentin stain score was significantly higher in epitheliomas and carcinomas than in normal circumanal glands (CG) or adenomas. Mean values of fractal dimension estimated at magnification 100X and 400X were as follows: normal CG 1.318 and 1.372, CG adenomas 1.384 and 1.408, CG epitheliomas 1.547 and 1.597, CG well differentiated carcinomas 1.569 and 1.607, CG poorly differentiated carcinomas 1.679 and 1.723. Significant differences (at level of 5%) of these values were observed between individual groups of CG adenomas or normal CG, and epitheliomas or carcinomas. The above results indicate vimentin immunohistochemistry staining and assessment of fractal dimension as an ancillary diagnostic method of choice when discerning between benign and malignant tumors of circumanal glands. Additional development of the method of fractal dimension assesment may yield a possibility for this tool to successfully discern between all of the types of CG tumors.</p>


2003 ◽  
Vol 6 (6) ◽  
pp. 564-567 ◽  
Author(s):  
Megan J. DiFurio ◽  
Aaron Auerbach ◽  
Keith J. Kaplan

Well-differentiated fetal adenocarcinoma (WDFA) is a rare tumor of the lung, which has gone by many names over the years. The lesion was first described by Kradin et al., in 1982, who called it “pulmonary blastoma with argyrophil cells and lacking sarcomatous features (pulmonary endodermal tumor resembling fetal lung).” Since then, there have been at least 65 cases reported in the literature. Although there has been no consensus in the literature as to the best pathological term for this entity, the most recent World Health Organization classification of lung and pleural tumors uses the term well-differentiated fetal adenocarcinoma. Characteristically, this lesion consists of an epithelium, which recapitulates fetal lung at 3–5 months of gestation and demonstrates neuroendocrine differentiation. Although the classic age range is 30–40 years, there have been seven reports of WDFA in the pediatric age. We report an additional pediatric case of this tumor and review the pediatric cases in the existing literature.


2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Hideo Tomihara ◽  
Kazuhiko Hashimoto ◽  
Tomoko Wakasa ◽  
Hajime Ishikawa ◽  
Tomoyuki Tsujimoto ◽  
...  

Abstract Background Gallbladder neuroendocrine tumors (GB-NETs) are extremely rare, representing only 0.5% of all NETs because no neuroectodermal cells are present in the gallbladder. In 2019, the World Health Organization updated the classification of NETs based on their molecular differences. The mutation status of DAXX and ATRX has been added to the criteria for well-differentiated NETs. Case presentation A 50-year-old man presented to our hospital for further examination of a gallbladder polyp. He had no right quadrant pain, fever, jaundice, weight loss, or carcinoid syndrome-related symptoms. The patient hoped to avoid cholecystectomy. During the 3-year observation period, the polyp gradually increased in size from 8.3 to 9.9 mm. He decided to undergo surgery, and whole cholecystectomy was successfully performed. Immunohistochemical staining revealed positivity for chromogranin A, synaptophysin, and CD56. The Ki-67 index was < 3%. Taken together, these results led to a diagnosis of a grade 1 GB-NET. We also performed immunohistochemical staining of DAXX and ATRX, which revealed that DAXX protein expression was negative. The patient’s postoperative course was uneventful, and he developed no recurrence for 8 years after surgery. Conclusion We experienced a very rare case of GB-NET. Obtaining a correct preoperative diagnosis is quite difficult at the first evaluation. A GB-NET should be considered as a differential diagnosis of gallbladder tumors.


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