[Elastofibroma dorsi: Diagnosis and follow-up in primary care]. / Elastofibroma dorsi: diagnóstico y seguimiento en atención primaria.

OBJECTIVE: To present a series of patients with elastofibroma dorsi (ED) in order to recall the pathology and justify its diagnostic and therapeutic management in the primary care setting. MATERIALS AND METHODS: Retrospective observational and longitudinal study of 12 patients with 18ED. Epidemiological, clinical and imaging characteristics were analysed. Treatment outcomes were assessed in terms of pain (VAS scale), shoulder function (Constant and Murley scale) and quality of life (EuroQol-5 dimension scale, EQ-5D). The mean follow-up of the cases was 60.5months (5years, range 1-161months). RESULTS: Six patients were male and six were female, with a mean age at diagnosis of 59years. The diagnosis of suspicion in all cases was based in clinical and imaging findings. Only three required surgery. The results of all were satisfactory. CONCLUSIONS: Both the diagnosis and the indication for treatment of an ED can be made in the primary care setting. In typical cases, which are the majority, the clinical history and an ultrasound study allow a diagnosis of certainty. The indication for conservative or surgical treatment depends on the informed patient. When there are diagnostic doubts or the patient wishes surgical resection of the tumour, the patient should be referred to a hospital specialist.

Non-ossifying fibromas and fibrous cortical defects around the knee - an epidemiologic survey in a Japanese pediatric population.

BACKGROUND: The aim of the present study was to evaluate the prevalence of non-ossifying fibroma (NOF) and fibrous cortical defect (FCD) in a Japanese pediatric population and the association between the lesion size and pain. METHODS: This retrospective study, conducted across 10 Japanese institutions, included patients aged 5-15 years who had undergone standard antero-posterior and lateral view radiography of the knee. Using these radiographs, we diagnosed the lesion as a NOF or FCD. Patient demographics, including age, sex, the size and location of the NOF, and chief complaint were recorded. The lesion size was determined using radiographs. Student's t-test was used to compare the associations between the lesion size and spontaneous pain. RESULTS: A total of 6222 subjects (3567 boys and 2455 girls) were included in this study. The number of NOF and FCD cases was 143 and 437, respectively, and the prevalence of NOF and FCD was 2.3% and 7.0%, respectively. The average size of NOF and FCD was 22.1 mm (range: 4-102 mm) and 13.2 mm (range: 5-21 mm), respectively. Three patients (2.1%) had pathological fractures due to NOF. Of the 140 NOFs and 437 FCDs, we obtained complaints from the medical records of 126 and 393 patients, respectively. The number of patients with spontaneous pain or other problems with NOF was 68 (54%) and 58 (46%), respectively, that of patients with FCD was 195 (50%) and 198 (50%) patients, respectively. The lesion size was not associated with spontaneous pain in either lesion (p = 0.67 and p = 0.27, respectively). CONCLUSION: The prevalence of NOF and FCD around the knee was lower than that reported in previous studies. The prevalence of NOF increased and that of FCD decreased with advancing age. In both lesions, the lesion size may not be associated with pain.

Prevalence of elastofibroma dorsi found incidentally upon chest computed tomography scan: A tertiary care center experience.

OBJECTIVES: To determine the prevalence and demographic data regarding incidentally encountered elastofibroma dorsi (EFD) in the Saudi population-based on chest CT (computed tomography) scans. METHODS: This retrospective study was carried out on 4,435 chest CT examinations that were collected between January 2014 and December 2016 in Riyadh, Saudi Arabia. One musculoskeletal radiologist who was unaware of the patients' clinical presentations or reasons for CT study reviewed the 4,435 chest CTs prospectively, searching for soft tissue tumors in the subscapular region. RESULTS: Elastofibroma dorsi was incidentally confirmed via CT scan in 36 of the 4,435 patients, including 8 males and 28 females, with a mean age of 76.6 years, yielding a prevalence of 0.8%. Elastofibroma dorsi was more common in females, with a 3.3-fold higher prevalence (77.8%) as compared to males (22.2%); however, this difference was not statistically significant (p>0.05). Twenty-five (69.44%) patients had unilateral EFD and 11 (30.56%) had bilateral EFD. Right-sided EFD was encountered in 60% of females and 50% of males. Furthermore, there was no statistically significant correlation between the size of EFD and the patient's gender or age (p>0.05). CONCLUSION: Elastofibroma dorsi is a rare but uncommon incidental finding on routine chest CT examination encountered in 0.8% of elderly Saudi patients.

Clinical and echocardiographic characteristics of patients with pathology proven right-sided papillary fibroelastomas.

BACKGROUND: Papillary fibroelastoma (PFE) are one of the most common primary cardiac neoplasms. They are most commonly found on cardiac valves especially on the left side of the heart and have been associated with an increased risk of embolization. To date, no large studies looked at the clinical significance of right-sided PFE (R-PFE). Therefore, we sought to better characterize patients with R-PFE and its clinical sequelae. METHODS: We retrospectively identified patients with pathology-proven PFEs at a single center between January 1995 and December 2018 (n = 279). Patients with R-PFE were analyzed. Medical records and echocardiograms were reviewed for clinical and PFE characteristics. RESULTS: Twenty-nine patients with R-PFE (mean age 70 ± 11 years; 62% women) were included in the analysis. PFEs were located on the right atrial (RA) wall in 31%, tricuspid valve in 34%, right ventricular (RV) wall in 7%, RV outflow tract in 4%, and pulmonary valve in 10% of patients. The remaining patients (14%) had multifocal PFEs. Around 38% of patients had cancer diagnosed before PFE diagnosis, and 34% had associated congenital heart disease. Seventeen (59%) patients had angina or dyspnea at the time of the presentation, and 3 (10%) had embolic symptoms. One patient with PFE located on the RA with associated patent foramen ovale had a stroke at time of presentation. Two patients, one with PFE on the RA wall and another patient with PFE at the tricuspid valve, had pulmonary embolism at the time of presentation. Median maximal length for PFE varied by location, ranging from a media of 8 mm to 16 mm. Of the 12 patients with follow up echocardiogram 1 year after PFE removal; 3/12 (25%) had documented PFE recurrence. CONCLUSION: R-PFE can be present in patients thromboembolic events when there is a right-to-left shunt. They can be a rare cause of pulmonary embolism, and at least in those that had follow-up echocardiograms, had a significant recurrence rate.

Prevalence of Merkel Cell Polyomavirus (MCPyV) in the Oral Cavity Biopsies in Northern Iran.

OBJECTIVE: Infection with human tumor viruses is one of the hypothesized causes of cancer. The current investigation aimed to explore the presence and quantitative analysis of a new human tumor virus, Merkel cell polyomavirus (MCPyV) in tissue samples of 114 patients with oral cavity lesions including oral squamous cell carcinoma (OSCC), oral lichen planus (OLP), Dysplasia and oral irritation fibroma (OIF) in Northern Iran. METHODS: From 114 formalin fixed paraffin embedded samples; 35 with SCC, 29 with OLP, 14 with dysplasia and 36 with OIF were cut, deparaffinized and DNA was extracted. Quantitative detection of MCPyV large T antigen was performed by absolute quantitative Real-Time PCR. RESULT: MCPyV DNA was detected in 30.6% (n: 11/36) of IF, 24.1% (n; 7/29) of OLP, 21.4% (n:3/14) of dysplasia and 20% (n;7/35) of OSCC samples. The mean MCPyV DNA copy number was 2.32×10-2 ± 3.97 ×10-2, 2.02×10-2 (SD=3.13×10-2), 2.69×10-4 (SD=2.51×10-4), and 2.56×10-4 (SD=6.73×10-4) per cell in OSCC, dysplasia and both of OLP and OIF samples, respectively (P=0.76). CONCLUSION: This study provides the first data from Iran regarding the presence of MCPyV genome in oral cavity lesions and oral cancer. These results also emphasize that MCPyV has an active role in the occurrence of oral lesions and progression to cancer. Further studies should be carried out to clarify the role of MCPyV in oral cavity lesions.

The Natural History of Benign Bone Tumors of the Extremities in Asymptomatic Children: A Longitudinal Radiographic Study.

BACKGROUND: Benign bone tumors are common incidental findings in the pediatric population during radiographic evaluation. Counseling these patients requires reassurance and raises questions about the natural history of these tumors over time. The purpose of this study was to estimate the prevalence and observe the behavior of benign childhood bone tumors in an asymptomatic population. METHODS: A historical, longitudinal radiographic collection of healthy children was reviewed, which included comprehensive left-sided radiographs of the extremities at yearly intervals. In this study, 262 subjects with 25,555 radiographs were screened for benign bone tumors at a median age of 8 years (range, 0 to 18 years). All potential tumors were reviewed by a multidisciplinary panel, which confirmed the radiographic diagnosis of each lesion, the age at which the lesion first appeared, and the age at which it had resolved. Prevalence rates were calculated using the number of distinct subjects available for each radiographic location and age. RESULTS: Thirty-five tumors were identified in 33 subjects, including 19 nonossifying fibromas, 8 enostoses, 6 osteochondromas, and 2 enchondromas. The prevalence rate for all tumors combined increased with age and was 18.9% overall. The overall prevalence rates for specific tumor types were 7.5% for nonossifying fibromas, 5.2% for enostoses, 4.5% for osteochondromas, and 1.8% for enchondromas. Nonossifying fibromas demonstrated a bimodal distribution of prevalence, with a peak at 5 years (10.8%) and another after skeletal maturity (13.3%). The median age at the first appearance for all tumors combined was 9 years (range, 2 to 15 years), but varied by tumor type. Nonossifying fibromas often resolved (7 [37%] of 19), with further resolution possible beyond the last available radiograph. Enostoses, osteochondromas, and enchondromas persisted until the last available radiographs in all subjects. CONCLUSIONS: The prevalence of benign childhood bone tumors of the extremities was 18.9% in a historical asymptomatic population. Longitudinal radiographs allowed observation of the timing of the first appearance and the potential for resolution for each tumor type. These findings provide unique evidence to answer many commonly encountered questions when counseling patients and their families on benign bone tumors. LEVEL OF EVIDENCE: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Fibromatosis of the breast: a 10-year multi-institutional experience and review of the literature.

BACKGROUND: Breast fibromatosis is a rare clinical entity, but poses significant diagnostic and therapeutic challenges. In light of recent changes in management practices, the aim was to review our institutional experience of breast fibromatosis and provide a review of current available literature on such management. METHODS: A search of pathological databases within two tertiary institutions for all patients diagnosed with fibromatosis of the breast over a 10-year period (2007-2016) was performed. Clinicopathological characteristics and modes of treatment were recorded for each patient. Concurrently a comprehensive literature search was performed and studies relating to breast fibromatosis and its management were identified and reviewed. RESULTS: Sixteen patients were identified. Median age at diagnosis was 42 (range 21-70) and all patients were diagnosed with core biopsy. The most useful imaging modality in diagnosis was ultrasonography and magnetic resonance imaging. 13/16 were treated surgically whilst 3/16 were treated using a watch-and-wait approach. 6/13 (46%) required re-excision of margins and 2/13 (15%) had recurrence after surgery. On review of the literature, there is no dedicated guideline in place for the management of breast fibromatosis. Currently a 'watch and wait' approach is favoured over surgical intervention due to high levels of recurrence and associated surgical morbidity. All cases should be discussed at a sarcoma multidisciplinary team meeting and tyrosine kinase inhibitors should be considered in advanced cases. CONCLUSIONS: Breast fibromatosis is rare but affects young patients. Active surveillance is now favoured over surgical resection due to high recurrence rates and extensive morbidity. Dedicated guidelines are required to ensure best outcomes.

An epidemiological review of 623 foot and ankle soft tissue tumours and pseudo-tumours.

BACKGROUND: In evaluating foot and ankle soft tissue masses, comprehensive epidemiological data, especially clinical predictors of malignancy, is essential knowledge. Our aim was to assess these data in a cohort of patients that have undergone surgical excision of foot and ankle soft tissue tumours and pseudo-tumours over a 10-year period. METHODS: A retrospective review of foot and ankle soft tissue tumours and pseudo-tumours excised in a tertiary hospital from 1 Jan 2006 to 31 Dec 2016 was performed. Uni- and multivariable analyses via logistic regression were conducted for all independent variables to identify their relationship with malignancy. Applying receiver operating characteristic (ROC) curves and Youden's Index to significant variables, we attempted to identify optimal threshold values to predict malignancy of the soft tissue mass. RESULTS: A total of 623 tumours and pseudo-tumours were analysed, and majority were benign (n = 605, 97.1%). The most common pseudo-tumour, benign, and malignant tumours were ganglion cysts (n = 289, 90.3%), plantar fibromas (n = 54, 18.9%) and pleomorphic undifferentiated sarcomas (n = 4, 22.2%), respectively. Increasing age (P = 0.036), larger size of mass (P < 0.001) and male gender (P = 0.017) were significant predictors of malignancy. ROC and Youden's Index analyses identified optimal threshold values of 4.0 cm (area under curve [AUC] = 87.6%) for size of mass and 66 years (AUC = 60.7%) for age. CONCLUSION: Majority of foot and ankle soft tissue masses are benign. Increasing age, larger size of mass and male gender are significant predictors of malignancy. The threshold value in predicting malignancy is 4.0 cm for size of mass and 66 years for age.

Retrospective analysis of 73 cases of elastofibroma.

OBJECTIVE: Elastofibroma is a rare soft-tissue tumour. This study retrospectively analysed and summarised the clinical, imaging and typical pathological features, together with the short- and long-term surgical outcomes of patients with pathologically confirmed soft-tissue elastofibroma to improve their management. MATERIALS AND METHODS: We enrolled 73 patients with pathologically confirmed soft-tissue elastofibroma from January 2010 to December 2018. The general, clinical, diagnostic and treatment-related data, operation notes, pathological examination results and follow-up status were obtained by reviewing inpatient medical records. Disease onset age, sex, tumour location and size were statistically analysed using the chi square and rank sum tests. RESULTS: A total of 90 lesions from 73 patients were examined. Among these, 56 patients had single lesions: 27 were under the right scapula, 26 were under the left scapula, 1 at the umbilicus, 1 on the aortic valve, 1 on the right hip and 17 at the bilateral inferior angles of the scapula. The average age at onset was 56.4 years (range: 6-82 years). The male-to-female incidence ratio was about one to three. Tumour diameter and follow-up duration ranged from 2cm to 12cm and from one month to nine years, respectively; recurrence was not observed. The main postoperative complication was wound effusion, occurring in 24 sites among the 90 lesions, corresponding to an incidence rate of 26.7%. CONCLUSIONS: A correct diagnosis of elastofibroma can be made prior to surgical resection by examining typical clinical features and characteristic imaging findings. Short- and long-term outcomes of local excision are good, with no further recurrence.

Pattern of distribution of reactive localised hyperplasia of the oral cavity in patients at a tertiary health institution in Nigeria.

BACKGROUND: Reactive localized hyperplastic lesions of the oral cavity (RHLs) are relatively common peripheral lesions which present as a range of clinically similar lesions at dental centers. Diagnosis can be challenging if dentists are unfamiliar with their clinicopathological across various populations. OBJECTIVE: This study reviews the pattern of distribution of RHLs of the oral mucosa in a hospital- the Obafemi Awolowo University Teaching Hospital Complex (OAUTHC), Ile-Ife. MATERIALS AND METHODS: We reviewed 10 years data from the archives of the Department of Oral Maxillofacial Surgery and Oral Pathology, Obafemi Awolowo University, Nigeria. Information on RHLs were extracted and recorded on standardized data forms and analyzed using STATA. RESULTS: The most common lesions were pyogenic granuloma (43.7%) and focal fibrous hyperplasia (39.7%), respectively. RHLs were found to be more frequent in women (66.7%) than men (33.3%). The most common locations of involvement was the gingivae (84.6%), and lesions were more common in the 9-29 year age group and the mean age was 37.7 (±21.1) years. The relationship between age group and reactive lesions was however not statistically significant. CONCLUSION: The major benefit of this study is an improved knowledge of the frequency and distribution of oral reactive lesions in sub-Saharan Africa which may be highly beneficial when establishing a diagnosis and treatment plan in clinical practice.

Prevalence of elastofibroma dorsi on CT: Is it really an uncommon entity?

OBJECTIVE: The aim of this study was to determine the prevalence, demographic data of elastofibroma dorsi (ED) in adult population who had undergone chest CT examination and to discuss clinical, and radiological presentations, and treatment options of ED. METHODS: We retrospectively reviewed 4074 chest CT examinations for ED from July 2014 to April 2015. Lesion size, side, and patient demographics were analyzed for positive cases of ED. The initial radiology reports of patients with ED were also reviewed. RESULTS: Of the 4074 patients, 111 patients (2.73%) (77 women and 34 men; mean age: 68.2 years; range: 35-91 years) had a total of 168 ED. The females had a 1.96 -fold higher prevalence of ED than the males (OR, 1.96; 95% CI, 1.481-2.59). The mean lesion thickness was found to be significantly greater in the female patients compared with the male patients (p = 0.001). The prevalence of the disease was estimated to be 4.98 times higher in patients aged 65 years or older (CI 95%, 3.25-7.36). In 111 ED patients, the lesions were only noted in 9 patients' initial radiology report. CONCLUSION: Here, we present a prevalence study with the largest population in the literature concerning ED. Our study shows that ED is not as uncommon as previously thought and should be especially suspected in females and older age groups. LEVEL OF EVIDENCE: Level IV, Diagnostic Study.

Clinical features and management of superficial fibromatoses.

Fibromatoses are a group of benign connective tissue tumors characterized by the infiltrative, aggressive proliferation of well-differentiated fibroblasts, leading to frequent local recurrence. Within this heterogeneous disease group, superficial fibromatoses show slower growth and more benign infiltration of surrounding tissues than deep fibromatoses. Superficial fibromatoses relevant to dermatology include palmar, plantar, and penile fibromatosis, knuckle pads, pachydermodactyly and infantile digital fibromatosis. They present clinically with subcutaneous nodules or cords that lead to local infiltration and limited mobility of the affected areas. Treatment options vary from watchful waiting, non-invasive methods such as radiotherapy and intralesional corticosteroid/collagenase injections to radical surgical procedures. Early intervention may disrupt disease progression and may even restore functional ability. These disorders should therefore be recognized and treated early in the course of the disease.

Acquired digital fibrokeratoma: review of its clinical and dermoscopic features and differential diagnosis.

Acquired digital fibrokeratoma (ADFK) is a rare, benign, fibrous tumor that most often occurs on fingers. It may resemble a rudimentary supernumerary digit and is often misdiagnosed as another common benign condition, such as common wart. It is typically asymptomatic and occurs as a solitary nodule less than 1 cm in diameter. Oftentimes ADFK shares clinical and/or histologic resemblance with other benign and malignant cutaneous conditions so it is crucial that careful examination is undertaken. In this article, we will discuss the clinical presentation, epidemiology, etiology, dermoscopic and histologic findings, management, and differential diagnoses for ADFK, with the hope that this review will facilitate timely diagnosis and management for this distinct condition.

Oral benign neoplasms: A retrospective study of 790 patients over a 14-year period.

INTRODUCTION AND OBJECTIVE: Oral benign neoplasms (OBNs) exhibit some features that can guide the professionals to the correct diagnosis and best treatment. Through retrospective studies, medical records can be reviewed to better describe a given population and, furthermore, help clinicians in routine practice. In this context, the objective of this paper was to analyze the cases of OBNs of an oral pathology referral department, from 2003 to 2017, in order to better understand their epidemiological and clinicopathological characteristics. METHODS: A total of 8355 histopathological reports were analyzed. Lesions diagnosed as OBNs were selected and the following variables were recorded: gender, age, histological type of the lesion, anatomical location, rate and pattern of growth, type of base, color, symptomatology and diagnostic hypotheses on clinical examination. RESULTS: OBNs represented 9.4% of all lesions diagnosed. The most frequent histopathological types were fibroma (39.9%), papilloma (22%), fibroblastoma (13.1%), lipoma (10.2%) and hemangioma (6.1%). Overall, most cases affected females (n=518; 65.6%) and in the fifth decade of life (n=148; 18.7%). The oral mucosa was the most common site (n=265; 33.5%). The most common features of each OBN were also highlighted. CONCLUSION: The most common OBNs were fibroma, papilloma, fibroblastoma, lipoma and hemangioma. Overall, the OBN presented common clinical features; however, in particular cases, there are some characteristics that can lead the professionals to the correct diagnosis. Nevertheless, in general, histopathological analysis must be performed to confirm diagnosis. Intraosseous tumors and large lesions may require imaging tests to help diagnosis.

[A clinicopathological retrospective epidemiological analysis of benign tumors and tumor-like lesions in the oral and maxillofacial region, diagnosed at the University of Szeged, Department of Oral Medicine (1960-2014)]. / A szegedi Fogászati és Szájsebészeti Klinikán diagnosztizált, az orofacialis régiót érinto jóindulatú daganatok és daganatszeru laesiók klinikopatológiai retrospektív epidemiológiai analízise (1960­2014).

INTRODUCTION: In a clinicopathological retrospective epidemiological study we investigated benign tumors and tumor-like lesions located in the orofacial region, diagnosed at the Universiy of Szeged, Department of Oral Medicine. METHOD: During a 54-year period (1960-2014), 14 661 biopsies were taken. The included subjects were 7491 patients diagnosed with benign tumors and tumor-like lesions. RESULTS: The average age of patients was 55.3 years, 2823 (37.7%) patients were male and 4668 (62.3%) female. The male : female ratio was 1 : 1.65. Most of the patients included in the study were aged 51-60 (1280, 17.1%). The number of children was 1014 (13.6%) and the number of adults was 6477 (86.3%). The number of non-neoplasms was 6420 (85.7%), being significantly higher than the number of neoplasms (1071, 14.3%). Most of the lesions were of mesenchymal origin (5574, 77.4%); the number of lesions of non-mesenchymal origin was 982 (13.1%). The most prevalent type of lesions was traumatic fibroma (fibrosis): 1806 (32.4%). The most common lesion type in the group of lesions of infectious/inflammational origin was pyogenic granuloma, the number of which was 465 (8.3%). The most common cystic lesion was mucocele (805, 10.7%). Hemangioma was the most frequent lesion type among developmental anomalies with the number of 815 (14.6%). The most common location of the lesions was the lip in 2081 cases (27.8%), followed by the gingiva in 2024 cases (27.0%), bucca in 1069 cases (14.3%), tongue in 981 cases (13.1%), and the facial skin in 695 cases (9.3%). After taking biopsy, the majority of benign lesions were treated with cryo-, laser-, or combined (cryo and laser) surgery. CONCLUSION: The present computer-aided study showed that irritational fibroma was the most common orofacial benign tumor, and the lip was the most frequent location. The diagnostic classification and the methodology are considerably different in the majority of the studies, which may hinder the exact comparison with other surveys from different regions of the world. Orv Hetil. 2018; 159(37): 1516-1524.

Granular cell tumors of the tongue: fibroma or schwannoma.

BACKGROUND: Granular cell tumors are benign lesions that typically occur in the oral cavity, but can also be found in other sites. However, the characteristics of these tumors are unclear. Thus, the present study aimed to investigate the immunohistological characteristics of these tumors of the tongue. METHODS: Seven patients were treated for granular cell tumors of the tongue at our institution during 2003-2017. Paraffin-embedded specimens were available for all cases; thus, retrospective immunohistochemical analyses were performed. RESULTS: All cases exhibited cytoplasmic acidophilic granules in the muscle layer of the tumor. Both the normal nerve cells and tumor cells also stained positive for PGP9.5, NSE, calretinin, and GFAP. A nucleus of tumor cells was typically present in the margin. The PAS-positive granules were also positive for CD68 (a lysozyme glycoprotein marker). Various sizes of nerve fibers were observed in each tumor, and granular cells were observed in the nerve fibers of a representative case. CONCLUSIONS: Based on our immunohistological findings, granular cell tumors may be derived from Schwann cells, and the presence of CD68 indicates that Wallerian degeneration after nerve injury may be a contributor to tumor formation. Thus, a safe surgical margin is needed to detect the infiltrative growth of granular cell tumors.

MRI findings and demographics of elastofibroma dorsi: assessment of diffusion-weighted imaging and contrast enhancement patterns.

Background Elastofibroma dorsi is a rare pseudotumoral lesion. Thus, there is no report of magnetic resonance imaging (MRI) findings that investigates multiple patients particularly with respect to diffusion-weighted imaging (DWI) findings and contrast enhancement patterns. Purpose To describe the imaging findings of elastofibroma on MRI, particularly DWI findings and contrast enhancement patterns, and to further investigate patient demographics. Material and Methods Forty-four patients with elastofibroma that underwent MRI were enrolled in this retrospective study. All images were evaluated by two radiologists to visually assess the signal intensity for each sequence. Enhanced elastofibromas were classified into four categories to assess the enhancement pattern. Differences in gender and laterality were also assessed statistically. Results An equal number of men and women were included (n = 22 each). There was no significant difference in laterality ( P = 0.783). All lesions (73 lesions) had low signal intensity on both T1-weighted (T1W) and T2-weighted (T2W) images: heterogeneous in 56, homogeneous in 17. None of the 41 lesions with DWI had true abnormal diffusion restriction. The average ADC value was 1.36 × 10-3 ± 0.29 mm2/s. All 31 lesions that had contrast-enhanced MRI were classified according to enhancement pattern: homogeneous (three lesions, 9.7%); heterogeneous (15 lesions, 48.4%); streak-like (three lesions, 9.7%); and rim-like (ten lesions, 32.2%). Conclusion There were no statistically significant differences in gender or laterality. Elastofibroma showed homogeneous to heterogeneous low signal intensity on T1W and T2W images. No lesion showed abnormal diffusion restriction, and all lesions demonstrated enhancement on MRI.

Prevalence of Oral Mucosal Lesions in a Tertiary Care Dental Hospital of Kathmandu.

INTRODUCTION: Oral mucosa can be affected by a variety of soft tissue lesions and conditions. Benign as well as malignant lesions of oral cavity are common. Squamous cell carcinoma is one of the commonest malignancies. The present retrospective study was undertaken to study the prevalence of various oral mucosal lesions in a tertiary care dental hospital of Kathmandu. METHODS: This retrospective study was carried out in the Department of Oral and Maxillofacial Pathology at Kantipur Dental College and Teaching hospital from January 2015 to January 2017. The study included biopsy specimen from the oral cavity. The parameters included in the study were age, gender, site of the lesion and histopathological diagnosis. The data collected was statistically analyzed. RESULTS: A total of 111 biopsy cases were included in the present study. Out of which, there were 16 (14.4%) cases of non-neoplastic and 16 (14.4%) cases of benign pathology. The oral cavity lesions were commonly seen in age range between 6-74 years where males 59 (53.2%) were mostly affected. The most common site for oral lesion was buccal mucosa 23 (20.7%) and anterior gingiva 23 (20.7%). Total 15 (13.5%) cases of oral squamous cell carcinoma were seen as the predominant malignancy affecting in the vestibular region 8 (7.2%). CONCLUSIONS: The present study shows benign as well as the non-neoplastic lesions were more prevalent in oral mucosa with the buccal mucosa and anterior gingiva as the commonest site of occurrence.

Multiple Endocrine Neoplasia and Hyperparathyroid-Jaw Tumor Syndromes: Clinical Features, Genetics, and Surveillance Recommendations in Childhood.

Children and adolescents who present with neuroendocrine tumors are at extremely high likelihood of having an underlying germline predisposition for the multiple endocrine neoplasia (MEN) syndromes, including MEN1, MEN2A and MEN2B, MEN4, and hyperparathyroid-jaw tumor (HPT-JT) syndromes. Each of these autosomal dominant syndromes results from a specific germline mutation in unique genes: MEN1 is due to pathogenic MEN1 variants (11q13), MEN2A and MEN2B are due to pathogenic RET variants (10q11.21), MEN4 is due to pathogenic CDKN1B variants (12p13.1), and the HPT-JT syndrome is due to pathogenic CDC73 variants (1q25). Although each of these genetic syndromes share the presence of neuroendocrine tumors, each syndrome has a slightly different tumor spectrum with specific surveillance recommendations based upon tumor penetrance, including the age and location for which specific tumor types most commonly present. Although the recommended surveillance strategies for each syndrome contain similar approaches, important differences do exist among them. Therefore, it is important for caregivers of children and adolescents with these syndromes to become familiar with the unique diagnostic criteria for each syndrome, and also to be aware of the specific tumor screening and prophylactic surgery recommendations for each syndrome. Clin Cancer Res; 23(13); e123-e32. ©2017 AACRSee all articles in the online-only CCR Pediatric Oncology Series.