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Well-differentiated papillary mesothelioma of the peritoneum is genetically defined by mutually exclusive mutations in TRAF7 and CDC42.
Well-differentiated papillary mesothelioma is an uncommon mesothelial neoplasm that most frequently arises in the peritoneal cavity of women of reproductive age. Whereas malignant mesothelioma is an aggressive tumor associated with poor outcome, well-differentiated papillary mesothelioma typically exhibits indolent behavior. However, histologically differentiating between these two entities can be challenging, necessitating the development of distinguishing biomarkers. While the genetic alterations that drive malignant mesothelioma have recently been determined, the molecular pathogenesis of well-differentiated papillary mesothelioma is unknown. Here we performed genomic profiling on a cohort of ten well-differentiated papillary mesothelioma of the peritoneum. We identified that all tumors harbored somatic missense mutations in either the TRAF7 or CDC42 genes, and lacked alterations involving BAP1, NF2, CDKN2A, DDX3X, SETD2, and ALK that are frequent in malignant mesothelioma. We recently identified that another mesothelial neoplasm, adenomatoid tumor of the genital tract, is genetically defined by somatic missense mutations in the TRAF7 gene, indicating a shared molecular pathogenesis between well-differentiated papillary mesothelioma and adenomatoid tumors. To the best of our knowledge, well-differentiated papillary mesothelioma is the first human tumor type found to harbor recurrent mutations in the CDC42 gene, which encodes a Rho family GTPase. Immunohistochemistry demonstrated intact BAP1 expression in all cases of well-differentiated papillary mesothelioma, indicating that this is a reliable marker for distinguishing well-differentiated papillary mesothelioma from malignant mesotheliomas that frequently display loss of expression. Additionally, all well-differentiated papillary mesothelioma demonstrated robust expression of L1 cell adhesion molecule (L1CAM), a marker of NF-kB pathway activation, similar to that observed in adenomatoid tumors. In contrast, we have previously shown that L1CAM staining is not observed in normal mesothelial cells and malignant mesotheliomas of the peritoneum. Together, these studies demonstrate that well-differentiated papillary mesothelioma is genetically defined by mutually exclusive mutations in TRAF7 and CDC42 that molecularly distinguish this entity from malignant mesothelioma
Renal papillary carcinoma developed in a kidney transplant recipient with late IgA-nephropathy
With improvements in immunosuppressive therapy, patient and graft survival in renal transplant recipients have been prolonged. Increasing donor age and patient survival rates have been related to an increase in the number of de novo tumors. Posttransplant malignancy in these patients is an important cause of graft loss and death in these patients. Among cancers occurring after a kidney transplant, renal cell carcinoma is the fifth most common malignancy after lymphoproliferative disorders, and skin, gastrointestinal, and lung cancers. When nonmelanoma skin cancers and in situ carcinoma of the cervix are excluded from malignancies, renal cell carcinoma accounts for 2% of all cancers in the general population, which increases to 5% in solid-organ recipients. The majority of renal cell carcinomas found in transplant recipients develop in the recipient 's native kidneys, but only 9% of tumors develop in the allograft itself. Tumors transmitted by donors represent only 0.02% to 0.2% of cases. Most de novo allograft renal cell carcinomas are single tumors. The mechanisms of development of renal cell carcinoma in renal grafts are not completely understood
Iodine and Thyroid Cancer in Goa
There is a low papillary to follicular ratio in iodine deficient areas. A study of malignant thyroid tumors done over a period of 4 years in Goa shows that the ratio of papillary to follicular carcinoma in Goa conforms to a iodine deficient status of the population
Villoglandular papillary adenocarcinoma of the uterine cervix with lymph node metastasis - A case report and review of the literature
Villoglandular papillary adenocarcinoma was first described by Young and Scully in 1989 as a distinct entity with the histological features of exophytic growth proliferation, villous and papillary architecture and mild to moderate nuclear atypia. We report one case of villoglandular papillary adenocarcinoma (VGPA) of the uterine cervix with lymph node metastasis and reviewed the clinicopathological features of six other cases reported in the literature. Our patient is the seventh similar reported cases. They ranged in age from 29 to 54 (mean, 41) years. Five had lymphovascular invasion. All except our patient were treated with radical hysterectomy and radiotherapy. She was followed-up for 7 months and unfortunately died due to chest infectio
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Metachronous Intraductal Papillary Neoplasm of the Bile Duct and Intraductal Papillary Mucinous Neoplasm of the Pancreas in a Patient Diagnosed With Mucinous Adenocarcinoma.
Intraductal papillary neoplasm of the bile duct (IPNB) is a rare biliary tumor, which shares some radiologic and histologic similarities with pancreatic intraductal papillary mucinous neoplasm (IPMN). IPNB is a recognized precursor lesion of invasive adenocarcinoma. We present a case of metachronous IPNB and IPMN lesions in a patient with mucinous adenocarcinoma of the pancreas who presented with jaundice and abdominal pain. The patient was treated with surgery and adjuvant chemotherapy
Diffuse sclerosing variant of thyroid carcinoma presenting as Hashimoto thyroiditis: a case report [Difuzno sklerozirajući oblik papilarnog karcinoma štitnjače nalik Hashimoto tireoiditisu: prikaz slučaja]
The aim of report is to present a case of a rare diffuse sclerosing variant of a papillary thyroid carcinoma. A 15-year old girl referred for ultrasound examination because of painless thyroid swelling lasting 10 days before. An ultrasound of the neck showed diffusely changed thyroid parenchyma, without nodes, looking as lymphocytic thyroiditis Hashimoto at first, but with snow-storm appearance, predominantly in the right lobe. Positive thyroid peroxidase antibodies (TPO-AT) also suggested Hashimoto thyroiditis. Repeated US-FNAB (fine needle-aspiration biopsy) of the right lobe revealed diffuse sclerosing variant of papillary thyroid carcinoma and patient underwent total thyreoidectomy. Patohistologic finding confirmed diffuse sclerosing variant of a papillary thyroid carcinoma in the both thyroid lobes and several metastatic lymph nodes. Two months later patient recived radioablative therapy with 3700 MBq (100 mCi) of 1-131 followed by levothyroxine replacement. At the moment, patient is without evidence of local or distant metastases and next regular control is scheduled in 6 months. In conclusion, a diffuse sclerosing variant is rare form of papillary thyroid carcinoma that echographically looks similar to Hashimoto thyroiditis and sometimes could be easily overlooked
Cytological Characteristics of Postoperative Metastases of Papillary Thyroid Cancer During the Development of Secondary Radioiodine Refractoriness
Radioiodine refractoriness is the main problem in the diagnosis and treatment of papillary thyroid carcinoma. The aim of the study was to investigate the cytological and immunocytochemical changes of thyrocytes in fine-needle aspiration smears of thyroid papillary cancer metastases in the course of the development of secondary radioiodine resistance. A total of 70 postoperative metastases of thyroid papillary cancer (secondary radioiodine refractory metastases, previously responsive to radioiodine, that eventually loses the ability to radioiodine accumulation, radioiodine-avid metastases, primary radioiodine-refractory metastases), immunohistochemical staining of thyroid peroxidase, thyroglobulin, cytokeratin 17 and cytological analysis were performed. Revealing the presence of specific cellular phenotypes and structures in punctuates, a low percentage of thyroid peroxidase and thyroglobulin-positive thyrocytes allows the development of the method of cytological prediction of the radioiodine therapy effectiveness
Comparative analysis of tumor capsule thickness and other histologic features in encapsulated follicular variant of papillary thyroid carcinoma (EFVPTC) and noninvasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP)
Recent reclassification of a subset of non-invasive encapsulated follicular variant of papillary thyroid carcinoma (EFVPTC) tumors as non-invasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) has led to important changes in the clinical management of patients with these indolent lesions. Although there are established diagnostic criteria to differentiate NIFTP from EFVPTC, our objective was to determine further differences in histological characteristics between NIFTP and noninvasive EFVPTC. Additionally, we sought to identify histological differences between non-invasive and invasive EFVPTC lesions beyond the key finding of invasion. 68 encapsulated follicular lesions with papillary-like nuclear features from patients treated at Thomas Jefferson University Hospital were subcategorized into NIFTP, non-invasive EFVPTC, and invasive EFVPTC based on current diagnostic criteria. Histological characteristics such as capsule thickness, lesion size, fibrosis, and presence of established exclusionary criteria for NIFTP were recorded. Capsule thickness was significantly smaller in NIFTP (
Simultaneous Papillary Carcinoma in Thyroglossal Duct Cyst and Thyroid
Thyroglossal duct cyst (TDC) is a cystic expansion of a remnant of the thyroglossal duct tract. Carcinomas in the TDC are extremely rare and are usually an incidental finding after the Sistrunk procedure. In this report, an unusual case of a 36-year-old woman with concurrent papillary thyroid carcinoma arising in the TDC and on the thyroid gland is presented, followed by a discussion of the controversies surrounding the possible origins of a papillary carcinoma in the TDC, as well as the current management options
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