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Ceren D Durmaz, Gareth Evans, Miriam J Smith, Pelin Ertop, Bengü N Akay, Timur Tuncalı
Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is a rare multisystemic autosomal dominant disorder typically presenting with cutaneous basal cell carcinomas, multiple keratocysts, and skeletal anomalies. NBCCS is caused by heterozygous mutations in the PTCH1 gene in chromosome 9q22, in the PTCH2 gene in 1p34, or the SUFU gene in 10q24.32. Here, we report on an 18-month-old boy presenting with medulloblastoma, frontal bossing, and multiple skeletal anomalies and his father who has basal cell carcinomas, palmar pits, macrocephaly, bifid ribs, calcification of falx cerebri, and a history of surgery for odontogenic keratocyst...
March 16, 2018: Cytogenetic and Genome Research
S Al-Benna, T G Arayathinal
No abstract text is available yet for this article.
March 15, 2018: Annals of the Royal College of Surgeons of England
Christian Doll, Kevin Dauter, Korinna Jöhrens, Stefan Hartwig, Jan Oliver Voss, Martin Klein, Max Heiland, Jan-Dirk Raguse
OBJECTIVE: the aim of this study was to evaluate clinical parameters and expression patterns of Ki-67, cyclin D1 and p53 in odontogenic keratocysts. MATERIAL AND METHODS: In this study, fifty-three patients with 80 odontogenic keratocysts were included. The medical records of these patients were reviewed retrospectively. To elucidate the molecular pathogenesis of the disease, the expression of p53, Ki-67 and cyclin D1 was analyzed using immunohistochemistry. RESULTS: A total of 53 patients (mean age 38 years) with a median follow-up of 4...
March 9, 2018: Journal of Stomatology, Oral and Maxillofacial Surgery
Jéssica Araújo Figueira, Fábio Roberto de Souza Batista, Karina Rosso, Vanessa Cristina Veltrini, Angelo José Pavan
Gorlin-Goltz syndrome (GGS), also known as nevoid basal cell carcinoma syndrome, is an autosomal dominant inherited disorder with high penetrance and variable expressivity. The classic triad originally described by Gorlin and Goltz in 1960 is composed of multiple nevoid basal cell carcinomas (NBCCs), odontogenic keratocysts (OKCs) in the jaws and bifid ribs. in 1977, this triad was modified by Rayner et al, and to GGS diagnosis, the OKCs had to appear in combination with calcification of the cerebellar falx or palmar and plantar pits...
March 8, 2018: Journal of Craniofacial Surgery
Tomasz Kaczmarzyk, Konrad Kisielowski, Rafał Koszowski, Magdalena Rynkiewicz, Ewa Gawełek, Karolina Babiuch, Anna Bednarczyk, Bogna Drozdzowska
OBJECTIVES: Odontogenic keratocyst (OKC) presents considerable variation in aggressiveness and propensity for recurrence, yet hitherto, no explicit clinicopathological features have been determined to clearly demonstrate the potential for relapse. This retrospective study aims to investigate the prognostic relevance of various clinicopathological features as well as immunoexpression of COX-2, bcl-2, PCNA, and p53 in sporadic OKC. MATERIALS AND METHODS: Among 41 patients with OKC treated by enucleation, the frequency of recurrence for various clinicopathological features as well as immunoexpression for COX-2, bcl-2, PCNA, and p53 was evaluated...
March 5, 2018: Clinical Oral Investigations
Vesna Musani, Petar Ozretić, Diana Trnski, Maja Sabol, Sanja Poduje, Mateja Tošić, Mirna Šitum, Sonja Levanat
We describe a case of twins with sporadic Gorlin syndrome. Both twins had common Gorlin syndrome features including calcification of the falx cerebri, multiple jaw keratocysts, and multiple basal cell carcinomas, but with different expressivity. One brother also had benign testicular mesothelioma. We propose this tumor type as a possible new feature of Gorlin syndrome. Gorlin syndrome is a rare autosomal dominant disorder characterized by both developmental abnormalities and cancer predisposition, with variable expression of various developmental abnormalities and different types of tumors...
February 28, 2018: Croatian Medical Journal
Annaji Araleri Gopalkrishna, B N Tejasree, M Manjunath, D P Ashwin
Odontogenic keratocyst(OKC)is a cyst oftooth origin with an aggressive behavior including a high recurrence rate, it has been rechristened to keratocystic odontogenic tumor(KCOT) as it be the reflects its neoplastic nature. We report a case of KCOT in association with an impacted supernumerary tooth along with Trigeminal Neuralgia, that subsided by itself after removal of the cyst.
January 2018: Journal of Oral and Maxillofacial Pathology: JOMFP
Mamata Kamat, Sampada Kanitkar, Uma Datar, Sanjay Byakodi
Orthokeratinized odontogenic cyst (OOC) is a relatively rare odontogenic cyst, distinct from odontogenic keratocyst (OKC). In the 4th edition of WHO Classification of Head and Neck Tumors (2017), OOC has been included as a separate entity in the category of developmental odontogenic cysts. It presents as a unilocular radiolucent lesion in the posterior mandible and is frequently related to impacted teeth, thus mimicking dentigerous cyst. Due to low local aggressiveness and no association with nevoid basal cell carcinoma syndrome, it does not show tendency to recur...
January 2018: Journal of Oral and Maxillofacial Pathology: JOMFP
J-R Tenório, T Santana, S-I-M-L Queiroz, D-H-I-P de Oliveira, L-M-G Queiroz
BACKGROUND: Ameloblastoma (AMB), odontogenic keratocyst (OKC) and adenomatoid odontogenic tumor (AOT) are epithelial odontogenic lesions with diverse biologic profiles. Defects in regulation of apoptosis and cell cycle may be involved in the development and progression of those lesions, therefore we aimed to investigate the expression of Bcl-2, Bax and p53 to better understand the possible role of these proteins in AMBs, OKCs and AOTs. MATERIAL AND METHODS: The studied sample consisted of 20 AMBs, 20 OKCs and 20 AOTs...
February 24, 2018: Medicina Oral, Patología Oral y Cirugía Bucal
Taynara Asevedo Campos de Resende, Vanessa de Fátima Bernardes, Jéssica Carolina da Silva, Luiz Armando De Marco, Ricardo Santiago Gomez, Carolina Cavalieri Gomes, Marina Gonçalves Diniz
OBJECTIVES: The odontogenic keratocyst (OKC) is an aggressive odontogenic cyst that has a high recurrence rate. Apart from PTCH1 mutations, few molecular alterations are described in OKCs. Low expression of microRNAs (miRNAs) miR-15a and/or miR-16-1 in association with increased expression of their target, Bcl-2, have been previously found in OKC. In humans, MIR15A and MIR16-1 are clustered at chromosome position 13 q14, and loss of heterozygosity (LOH) at this locus occurs in different tumors...
January 31, 2018: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Maryam Akbari, Harold Chen, Grace Guo, Zachary Legan, Ghali Ghali
In this article, we present three clinical case reports on Basal Cell Nevus Syndrome (Gorlin Syndrome). Gorlin syndrome is an inherited medical condition with challenges that manifest in multiple body systems and complicate early diagnosis. We examine the epidemiology of the disease and benefits of genetic testing, molecular pathophysiology, and advancement in the molecular-based therapy of Basal Cell Nevus syndrome. The goal of this paper is to shed light on both unmet challenges and advancements in the management of Gorlin syndrome and to provide a new clinical perspective and guidance for future research...
January 31, 2018: Pathophysiology: the Official Journal of the International Society for Pathophysiology
Puangwan Lapthanasupkul, Rachai Juengsomjit, Sopee Poomsawat, Tawepong Arayapisit
Polycomb group (PcG) proteins are repressive chromatin modifiers required for proliferation and development. PcG proteins form two large repressive complexes, namely, Polycomb Repressive Complex 1 and 2. These proteins have been shown to drive tumorigenesis by repressing cell-type specific sets of target genes. Using immunohistochemistry, we investigated the expression patterns of five human PcG proteins, including Bmi-1, Ring1b, Mel-18, Ezh2, and Suz12, in various cellular components of odontogenic keratocysts (OKCs), ameloblastomas and, pericoronal follicles (PFs)...
February 4, 2018: Acta Histochemica
Khamisah Awang Kechik, Chong Huat Siar
BACKGROUND AND AIMS: The odontogenic keratocyst (OKC) remains the most challenging jaw cyst to treat because of its locally-aggressive behaviour and high recurrence potential. Emerging evidence suggests that osteopontin, its receptors CD44v6 and integrin αv, and podoplanin, have a role in the local invasiveness of this cyst. However the spatial distribution characteristics of these pro-invasive markers in the lining epithelium of OKC, and their association with the clinicopathologic parameters of OKC are largely unexplored...
February 2018: Annals of Diagnostic Pathology
Sailendra Nath Biswas, R R Paul, Jay Gopal Ray, Sumit Majumdar, Divya Uppala
Context: The most common and important odontogenic cyst involving jaws is the odontogenic keratocyst (OKC) or primordial cyst, the dentigerous cyst and the radicular cyst. These cysts all though do not show similar behavior, they all have the potentiality to recur. Silver nitrate staining of the nucleolar organizer regions (AgNORs) of the benign and malignant lesions is becoming very useful as a diagnostic indicator. Thus, the aim of this study is to assess the diagnostic potential of AgNORs in the cystic epithelium of common odontogenic cysts...
September 2017: Journal of Oral and Maxillofacial Pathology: JOMFP
Vinay Kumar Dandena, Satish Yadava Thimmaiah, Mohammad Asif Kiresur, Prahlad Hunsigi, Swathi Roy, M Rashmi
Aim: This study aimed to demonstrate and evaluate the expression of stromal myofibroblasts (MFs) and epithelial cell proliferation using α-smooth muscle actin (α-SMA) and Ki67 markers, respectively, in odontogenic keratocyst (OKC) and orthokeratinized odontogenic cyst (OOC) to correlate their aggressive behavior. Materials and Methods: Twenty cases of OKC and twenty cases of OOC were stained with α-SMA and Ki67 markers for demonstration of stromal MFs and epithelial cell proliferation, respectively, and ten cases of well-differentiated squamous cell carcinoma were used as positive control...
September 2017: Journal of Oral and Maxillofacial Pathology: JOMFP
Atul Bhagwat, S R Barpande, J D Bhavthankar, M S Mandale, Jayanti Humbe, Preeti Singh
Introduction: Odontogenic tumors (OTs) are a group of heterogeneous lesions derived from epithelial or ectomesenchymal tissues or both, which are part of the tooth-forming apparatus. They range from hamartomatous or nonneoplastic tissue proliferations to malignant neoplasms with metastatic capacity. OTs are comparatively rare, comprising about 4.79% of all oral and maxillofacial biopsy specimens diagnosed. Several retrospective studies carried out in Africa, Asia, Europe and America, show that differences exist in the relative frequency of the various histologic types...
September 2017: Journal of Oral and Maxillofacial Pathology: JOMFP
B K Akshatha, Karpagaselvi Karuppiah, G S Manjunath, Jayalakshmi Kumarswamy, Lokesh Papaiah, Jyothi Rao
Introduction: The three common odontogenic cysts include radicular cysts (RCs), dentigerous cysts (DCs), and odontogenic keratocysts (OKCs). Among these 3 cysts, OKC is recently been classified as benign keratocystic odontogenic tumor attributing to its aggressive behavior, recurrence rate, and malignant potential. The present study involved qualitative and quantitative analysis of inducible nitric oxide synthase (iNOS) expression in epithelial lining of RCs, DCs, and OKCs, compare iNOS expression in epithelial linings of all the 3 cysts and determined overexpression of iNOS in OKCs which might contribute to its aggressive behavior and malignant potential...
September 2017: Journal of Oral and Maxillofacial Pathology: JOMFP
Naghmeh Jeiroodi, Mahshid Bagherpour, Razieh Zare, Shima Torabi-Ardakani, Azadeh Andisheh-Tadbir
OBJECTIVE: Midkine is a heparin-binding growth factor whose expression is increased in most tumors, namely ameloblastomas. This study aimed to compare Midkine expression in different odontogenic lesions. MATERIAL AND METHOD: This analytical cross-sectional study was performed on 52 definitely diagnosed odontogenic lesions including 15 dentigerous cysts, 13 odontogenic keratocysts, and 17 unicystic and 5 multicystic ameloblastomas archived from 1997 to 2015. Midkine expression was examined in tissue samples through immunohistochemistry...
January 31, 2018: Türk Patoloji Dergisi
Yookyeong Carolyn Sim, Gu-Hwan Kim, Sung-Weon Choi, Kang-Min Ahn
The purpose of this study was to report clinical characteristics, surgical results, and new PTCH1 gene mutations in nevoid basal cell carcinoma syndrome (NBCCS). Five patients were referred to the Department of Oral and Maxillofacial Surgery from local dental clinics between 2006 and 2016 to treat multiple keratocystic odontogenic tumors (KOTs). The cystic lesions were enucleated and peripheral ostectomy was performed to obtain safety margin. Recurrence and/or de novo development of KOT were assessed. Gene analysis using peripheral blood was performed in all patients to identify the mutation of PTCH1 gene...
January 29, 2018: Journal of Craniofacial Surgery
I Palacios-Álvarez, R González-Sarmiento, E Fernández-López
Gorlin syndrome is a rare autosomal dominant disease caused by mutations in the sonic hedgehog signaling pathway. Of particular importance is the PTCH1 gene. The disease is characterized by the development of multiple basal cell carcinomas at young ages. These tumors may present with other skin manifestations such as palmoplantar pits and with extracutaneous manifestations such as odontogenic keratocysts and medulloblastoma. Although the dermatologist may be key for recognizing clinical suspicion of the syndrome, a multidisciplinary team is usually necessary for diagnosis, treatment, and follow-up...
January 17, 2018: Actas Dermo-sifiliográficas
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