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Mesoblastic nephroma

Lily Marsden, Lawrence J Jennings, Samantha Gadd, Min Yu, Elizabeth J Perlman, Mariana M Cajaiba
Metanephric stromal tumors are rare renal stromal tumors that predominantly affect children. They belong to the metanephric family of tumors along with metanephric adenofibroma and metanephric adenoma. The previous documentation of BRAF exon 15 mutations in 88% of metanephric adenomas and in isolated cases of metanephric adenofibroma prompted us to investigate the prevalence of these mutations in metanephric stromal tumors and in other pediatric renal stromal tumors. In this study, 17 metanephric stromal tumors, 22 congenital mesoblastic nephromas and 6 ossifying renal tumors of infancy were selected for BRAF exon 15 testing...
October 18, 2016: Human Pathology
Surbhi Goyal, Kiran Mishra, Urvee Sarkar, Satendra Sharma, Anita Kumari
BACKGROUND & OBJECTIVES: Renal tumours constitute about 7 per cent of all neoplasms in children. It is important to differentiate Wilms' tumour (commonest tumour) from non-Wilms' tumours. The aim of this study was to evaluate the immunoexpression and diagnostic role of Wilms' tumour-1 protein (WT1) in paediatric renal tumours. METHODS: A total of 53 cases of renal tumours in children (below 18 yr) who underwent total nephrectomy were included in this retrospective study...
May 2016: Indian Journal of Medical Research
H Wang, Y Y Ma, D W Zhang, B Y Wu, C Fang, L Chen
No abstract text is available yet for this article.
September 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Evsen Apaydin, Yasemin Ozluk, Secil Yuksel, Basak Erginel, Deniz Tugcu, Alaaddin Celik, Isin Kilicaslan
OBJECTIVE: We aimed to define the histopathologic features and proliferative rate of congenital mesoblastic nephroma (CMN) as a risk factor for recurrence. METHODS: Fourteen cases of CMN among 138 registered pediatric renal tumors were retrospectively reviewed. The prognostic impact for mitotic rate and Ki67 index was investigated. RESULTS: There were four (28.6%) classic, six (42.9%) cellular, and four (28.6%) mixed type CMNs, with average Ki-67 counts of 16...
August 2, 2016: Fetal and Pediatric Pathology
Goutam Bera, Ram Narayan Das, Janki Bisht, Prafulla Kumar Mishra, Mamata Guha Mallick, Chhanda Datta, Manoj Kumar Chaudhuri, Uttara Chatterjee
Congenital mesoblastic nephroma (CMN) is a rare pediatric renal neoplasm, occurring most commonly in the first few months of life, with a favourable clinical outcome. Accurate pre-operative cytological diagnosis of this entity is important as pre-operative chemotherapy is not recommended and surgery is the treatment of choice. Cytodiagnosis of this rare tumor is discussed in only a few case reports. Here two cases of CMN and one case of cellular congenital mesoblastic nephroma (CCMN) diagnosed on FNAC along with their morphological differential diagnoses has been reported...
October 2016: Diagnostic Cytopathology
Kartik Chandra Mandal, Madhumita Mukhopadhyay, Shibsankar Barman, Pankaj Halder, Biswanath Mukhopadhyay, Rajarshi Kumar
AIMS: Scrutiny over the clinical behaviors, management, and the final outcome of some rare renal neoplasm in order to find out some hidden facts about these tumors which are playing an important role in the disease course and its management. MATERIALS AND METHODS: Retrospective evaluation of uncommon (non-Wilms') renal neoplasm in the pediatric population in a tertiary care center. Fifteen cases of uncommon renal tumors were treated in our institution over the last 5 years (January 2008 to December 2012)...
April 2016: Journal of Indian Association of Pediatric Surgeons
Dina El Demellawy, Caitlin A Cundiff, Ahmed Nasr, John A Ozolek, Nancy Elawabdeh, Shelley A Caltharp, Pourya Masoudian, Katrina J Sullivan, Joseph de Nanassy, Bahig M Shehata
Mesoblastic nephroma (MN) is the most common renal tumour in the first 3 months of life and accounts for 3-5% of all paediatric renal neoplasms. To further understand the morphological variants of MN, we identified 19 cases of MN (five classic, eight cellular and six mixed) and examined each case for markers known to be important in urogenital embryological development (PAX8, WT1 and RCC), stem cell associated markers (Oct 4, CD34 and c-kit), muscle/myofibroblastic markers (muscle specific actin, calponin and h-caldesmon), aberrant transcription factors, cell cycle regulation and other oncogenic proteins (p16, cyclin D1 and beta-catenin)...
January 2016: Pathology
Sydney Correia Leão, Diego Marques Fernandes, Bruno Garcia Dias, Wlisses Ramon Oliveira, Simone Maria de Oliveira, Margareth Rose Uchoa Rangel
A male child born at 27 weeks, weighting 1305 g and presenting with a right-sided abdominal tumor. Computed tomography scan demonstrated the presence of a solid mass compressing the right kidney. Puncture biopsy revealed congenital mesoblastic nephroma. The patient underwent total right nephroureterectomy, and died on the second day after surgery.
November 2015: Radiologia Brasileira
Eliza Lamin, Dana A Weiss, Kassa Darge, Fumiko Dekio, Douglas A Canning
A 25 4/7 week boy was born with a prenatal diagnosis of polyhydramnios and enlarged left kidney. Over the next 2 months serial ultrasounds demonstrated abnormal growth of the kidney, with 28.9% split function. At gestational age 39 4/7, he underwent a left radical nephrectomy. Pathology revealed congenital mesoblastic nephroma with mixed classic and cellular features. This case was puzzling due to prenatally diagnosed renal enlargement in a premature infant and inconclusive post-natal ultrasonographic imaging...
September 2015: Urology Case Reports
S A Islam, M S Rahman, S Habib, M A Salam
Mesoblastic nephroma is an uncommon renal tumor of infancy and rarely occurs in adults. We report a case of mesoblastic nephroma in adult. A 22-year-old woman, who presented with left flank pain, was found to have a left renal mass by abdominal ultrasonography. Computed tomography revealed a heterogeneous tumor. Left radical nephrectomy was performed. The tumor was a creamy white solid mass. Microscopically, the tumor was composed of spindle cell proliferation. Atypia and mitoses were not identified. Among the tumor cells, there were tubular arranged epithelial elements...
October 2015: Mymensingh Medical Journal: MMJ
Senthil Kamaraj, Susan Arbuckle, Denise Warner, Grahame Smith, Jonathan Karpelowsky
Congenital mesoblastic nephroma (CMN) is the most frequent renal neoplasm of newborns and young infants. Four cases presenting with hemorrhagic manifestations have been reported in the English literature (Hu et al, 2006; Bolande et al, 1967). We report the unusual clinical and radiographic findings of a 2-day-old neonate with hematuria secondary to a CMN. The first ultrasound was equivocal. Repeat ultrasound followed by magnetic resonance imaging confirmed the diagnosis. He underwent a right nephroureterectomy with histopathology revealing a cellular variant of CMN without classical translocation (t12:15)...
February 2016: Urology
H Takahashi, A Ohkuchi, T Kuwata, R Usui, S Takahashi, S Matsubara
To characterise congenital mesoblastic nephroma (CMN), with special emphasis on polyhydramnios and the neonatal prognosis, we summarise 31 CMN patients (30 reported patients and the present patient). CMN was detected at a median of 30 weeks' gestation, and infants were delivered at a median of 34 weeks' gestation. Of 27 patients with available data, 19 (70%) had polyhydramnios, of which 8 required amnio- drainage. Women with amnio-drainage gave birth significantly earlier (30.4 weeks' gestation) than those without polyhydramnios (36...
2016: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
A Young Do, Jung-Sun Kim, Suk-Joo Choi, Soo-Young Oh, Cheong-Rae Roh, Jong-Hwa Kim
Congenital mesoblastic nephroma is a rare renal tumor that is diagnosed during pregnancy and is associated with polyhydramnios, prematurity, and neonatal hypertension. Differential diagnoses include Wilms tumor, adrenal neuroblastoma, and other abdominal tumors. We report a case of congenital mesoblastic nephroma detected by prenatal ultrasonography as a large fetal renal mass with polyhydramnios at 32 weeks of gestation. Ultrasonography showed a 6×6-cm complex, solid, hyperechoic, round mass in the right kidney...
September 2015: Obstetrics & Gynecology Science
Sze Jet Aw, Chik Hong Kuick, Min Hwee Yong, Derrick Wen Quan Lian, Shi Wang, Alwin Hwai Liang Loh, Sharon Ling, Guat Lian Peh, Shui Yen Soh, Amos Hong Pheng Loh, Puay Hoon Tan, Kenneth Tou En Chang
Pathological diagnosis of clear cell sarcoma of the kidney (CCSK) is challenging as it resembles blastemal Wilms tumor (WT) and other pediatric sarcomas, and does not have any distinctive immunophenotype. The YWHAE-FAM22 translocation t(10;17)(q22;p13) has been reported in a subset of CCSK. This translocation also occurs in high-grade endometrial sarcoma, in which it is associated with cyclin D1 overexpression. Hence we seek to determine YWHAE-FAM22 translocation status and cyclin D1 immunoreactivity in a series of local CCSK cases...
July 2015: Pediatric and Developmental Pathology
Dominique Berrebi, Jean-Christophe Fournet, Françoise Boman, Monique Fabre, Pascale Philippe-Chomette, Sophie Branchereau, Paul Fréneaux, Dorothée Bouron-Dal Soglio, Jean Michon, Michel Peuchmaur
Congenital/infantile fibrosarcoma (IFS) is a relatively rare form of fibrosarcoma diagnosed at birth or during early years of life and that differs from its adult counterpart because of a more favorable behavior. IFS is also known as cellular congenital mesoblastic nephroma, when it affects the kidney and is often but not always characterized by the ETV6-NTRK3 fusion transcript. We report herein the first series of an exceptional tumor of the small intestine occurring in newborns. The four patients shared a stereotyped clinico-pathological presentation with early and acute onset, intestinal perforation, and an infiltration by a highly cellular spindle cell tumor within the dilated intestinal wall exhibiting pathologic features typical of IFS...
April 2015: Pediatric Surgery International
Gábor Máté, István Kertész, Kata Nóra Enyedi, Gábor Mező, János Angyal, Nikolett Vasas, Adrienn Kis, Éva Szabó, Miklós Emri, Tamás Bíró, László Galuska, György Trencsényi
PURPOSE: Aminopeptidase N (APN/CD13) plays an important role in tumor neoangiogenic process and the development of metastases. Furthermore, it may serve as a potential target for cancer diagnosis and therapy. Previous studies have already shown that asparagine-glycine-arginine (NGR) peptides specifically bind to APN/CD13. The aim of the study was to synthesize and investigate the APN/CD13 specificity of a novel (68)Ga-labeled NOTA-c(NGR) molecule in vivo using miniPET. METHODS: c[KNGRE]-NH2 peptide was conjugated with p-SCN-Bn-NOTA and was labeled with Ga-68 ((68)Ga-NOTA-c(NGR))...
March 10, 2015: European Journal of Pharmaceutical Sciences
Jelena Mirkovic, Monica Calicchio, Christopher D Fletcher, Antonio R Perez-Atayde
AIMS: Distinguishing clear cell sarcoma of the kidney (CCSK) from other paediatric malignancies, particularly blastema-rich Wilms tumour (WT) and congenital mesoblastic nephroma (CMN), is challenging. Specific immunohistochemistry for CCSK does not exist, and diagnosis rests upon histopa thology. Recently, the YWHAE-FAM22 rearrange ment, identical to that in endometrial stromal sarcoma (ESS), has been identified in CCSKs. As this fusion results in overexpression of cyclin D1 in ESS, we postulated that overexpression would also occur in CCSK; cyclin D1 immunohistochemistry could then be used to differentiate CCSK from other tumours...
September 2015: Histopathology
Alpin D Malkan, Amos Loh, Armita Bahrami, Fariba Navid, Jamie Coleman, Daniel M Green, Andrew M Davidoff, John A Sandoval
Renal masses in children may be discovered during routine clinical examination or incidentally during the course of diagnostic or therapeutic procedures for other causes. Renal cancers are rare in the pediatric population and include a spectrum of pathologies that may challenge the clinician in choosing the optimal treatment. Correct identification of the lesion may be difficult, and the appropriate surgical procedure is paramount for lesions suspected to be malignant. The purpose of this article is to provide a comprehensive overview regarding the spectrum of renal tumors in the pediatric population, both benign and malignant, and their surgical management...
January 2015: Pediatrics
Maria Tretiakova, Debra L Zynger, Chunyan Luan, Nicole K Andeen, Laura S Finn, Masha Kocherginsky, Bin T Teh, Ximing J Yang
Glypican 3 (GPC3), a heparan sulfate proteoglycan, plays a role in cell growth and differentiation. Mutations of the GPC3 gene are responsible for Simpson-Golabi-Behmel syndrome, which is characterized by anomalies of postnatal overgrowth and an increased risk of developing pediatric malignancies, mostly Wilms tumor and liver cancer. In order to understand the possible role of GPC3 in renal development and Wilms tumor formation, we analyzed messenger RNA (mRNA) and protein levels of GPC3 in sporadic Wilms tumors and compared it to normal kidneys and other common renal epithelial tumors...
January 2015: Virchows Archiv: An International Journal of Pathology
Zuo-Peng Wang, Kai Li, Kui-Ran Dong, Xian-Min Xiao, Shan Zheng
Congenital mesoblastic nephroma (CMN) is a mesenchymal renal tumor. The aim of the present study was to review the clinical characteristics and outcome of CMN in infants. A retrospective file review was conducted of eight cases of CMN treated at the Children's Hospital of Fudan University between 2004 and 2012. Ultrasound and computerized tomography scans had been performed on all eight patients. Two cases presented with a solid tumor and exhibited pathological features consistent with those of classic CMN, five cases exhibited cystic, hemorrhagic and necrotic characteristics, with calcification and pathology consistent with the cellular variant of CMN and one case presented with a solid tumor, which exhibited pathological features consistent with ceullular CMN...
November 2014: Oncology Letters
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