scholarly journals Atypical Enostoses—Series of Ten Cases and Literature Review

Medicina ◽  
2020 ◽  
Vol 56 (10) ◽  
pp. 534
Author(s):  
Thomas Bedard ◽  
Mujtaba Mohammed ◽  
Serenella Serinelli ◽  
Timothy A. Damron

Bone islands (BI; enostoses) may be solitary or occur in the setting of osteopoikilosis (multiple bone islands) and are sometimes associated with Gardner’s Syndrome (osteopoikilosis and colonic polyposis). Characteristic features of bone islands are (1) absence of pain or local tenderness, (2) typical radio dense central appearance with peripheral radiating spicules (rose thorn), (3) Mean CT (computerized tomography) attenuation values above 885 Hounsfield units (HU) (4) absence of uptake on bone scan and (5) radiographic stability over time. However, when enostoses display atypical features of pain, unusual radiographic appearance, aberrant HU, increased radiotracer uptake, and/or enlargement, they can be difficult to differentiate from more sinister bony lesions such as osteoblastic metastasis, low grade central osteosarcoma, osteoid osteoma and osteoblastoma. In this retrospective case series, the demographic, clinical, radiographic, treatment and outcome for ten patients with eleven atypical bone islands (ABI) are presented, some showing associated pain (5), some with atypical radiographic appearance (3), some with increased activity on BS (4), some with documented enlargement over time (7), one with abnormal CT attenuation value, some in the setting of osteopoikilosis (2), one in the setting of Gardner’s Syndrome and one osteoid osteoma simulating a bone island. This series represents the spectrum of presentations of ABI. Comprehensive review of the literature reveals that the previous largest series of ABI showing enlargement as the atypical feature was in younger patients with jaw BI. Hence, this represents one of the largest series reported of ABI of all types in adults.

Cancers ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 1453
Author(s):  
Chiara Fabbroni ◽  
Giovanni Fucà ◽  
Francesca Ligorio ◽  
Elena Fumagalli ◽  
Marta Barisella ◽  
...  

Background. We previously showed that grading can prognosticate the outcome of retroperitoneal liposarcoma (LPS). In the present study, we aimed to explore the impact of pathological stratification using grading on the clinical outcomes of patients with advanced well-differentiated LPS (WDLPS) and dedifferentiated LPS (DDLPS) treated with trabectedin. Patients: We included patients with advanced WDLPS and DDLPS treated with trabectedin at the Fondazione IRCCS Istituto Nazionale dei Tumori between April 2003 and November 2019. Tumors were categorized in WDLPS, low-grade DDLPS, and high-grade DDLPS according to the 2020 WHO classification. Patients were divided in two cohorts: Low-grade (WDLPS/low-grade DDLPS) and high-grade (high-grade DDLPS). Results: A total of 49 patients were included: 17 (35%) in the low-grade cohort and 32 (65%) in the high-grade cohort. Response rate was 47% in the low-grade cohort versus 9.4% in the high-grade cohort (logistic regression p = 0.006). Median progression-free survival (PFS) was 13.7 months in the low-grade cohort and 3.2 months in the high-grade cohort. Grading was confirmed as an independent predictor of PFS in the Cox proportional-hazards regression multivariable model (adjusted hazard ratio low-grade vs. high-grade: 0.45, 95% confidence interval: 0.22–0.94; adjusted p = 0.035). Conclusions: In this retrospective case series, sensitivity to trabectedin was higher in WDLPS/low-grade DDLPS than in high-grade DDLPS. If confirmed in larger series, grading could represent an effective tool to personalize the treatment with trabectedin in patients with advanced LPS.


2021 ◽  
Vol 108 (Supplement_7) ◽  
Author(s):  
Nicholas Bradley ◽  
Michael Wilson ◽  
Christopher Shearer ◽  
Timothy Heron ◽  
Katie Robertson ◽  
...  

Abstract Aims Gallbladder polyps are typically an incidental finding on sonographic or pathological examination with an estimated prevalence of 0.3 – 9.5%. Their role as a precursor to gallbladder malignancy is disputed. The 2017 European Joint Society Guidelines (ESGAR/EAES/EFISDS/ESGE) aim to standardise management of gallbladder polyps detected prior to cholecystectomy. We aim to describe our experience in the management of gallbladder polyps in a district general hospital. Methods This single centre retrospective case series included consecutive cholecystectomies over an 8-year period, identified through pathology records. Medical records were interrogated to identify the presence of gallbladder polyps identified pre-operatively and at subsequent histopathological analysis. Results 3835 cases were included. Four cases (0.1%) had an adenocarcinoma identified; none of these had pre-operatively identified polyps. Two cases (0.05%) were found to have lesions with high-grade dysplasia, 1 of which had a 17mm polyp preoperatively. One case (0.03%) had cellular atypia, without a pre-operative polyp. Forty six cases (1.2%) were found to have low-grade dysplasia; 1 (2.2%) of these had adenomyomatosis pre-operatively but none had pre-operative polyps. Overall, 53 patients (1.4%) had abnormal pathology with only 1 (1.9%) of these having a pre-operative polyp identified. Conclusions Our results suggest that in the real-world setting, intensive surveillance of gallbladder polyps has limited utility in identifying cases of gallbladder malignancy. The burden on services produced by adherence to strict surveillance guidelines is difficult to justify and a less arduous approach is unlikely to significantly influence the management or outcome in patients with gallbladder malignancy.


2020 ◽  
pp. bjophthalmol-2020-317772
Author(s):  
Hansell Soto ◽  
Randy C Bowen ◽  
Vishal Raval ◽  
Gabrielle Yeaney ◽  
Arun Singh

AimTo assess the role of map biopsy in patients with conjunctival primary acquired melanosis (PAM)/melanoma.MethodsRetrospective case series of 400 conjunctival biopsy samples of 51 unique patients in a tertiary referral centre.ResultsEach patient underwent one diagnostic biopsy and several additional map biopsies (range 2–7) providing a total of 400 samples for the analysis (55 diagnostic biopsies, 345 map biopsies). The median age was 63 years old (range 20–88) with women representing 67% of the cases. Histopathological findings were graded as negative for melanosis/normal (grade 0), melanosis without atypia (grade 1), melanosis with mild atypia (grade 2), melanosis with severe atypia (grade 3) or invasive melanoma (grade 4). Clinicopathologic concordance was observed in the majority of the map biopsies (313, 91%) (positive: clinical+/path+ (57,17%), negative: clinical−/path− (256, 74%)). Three discordant samples (clinical−/path+) represented PAM sine pigmento. The histopathological spectrum of atypia was absent (40, 73%) or limited (11, 20%) in the majority of cases with tendency to cluster as low-grade or high-grade atypia. Map biopsy led to the identification of six patients (11%) with severe atypia, requiring topical mitomycin (MMC). Similarly, in 29 cases, periodic observation without topical MMC was recommended. One case of invasive melanoma transformation occurred in the MMC-treated group.ConclusionsMap biopsy enhances overall assessment of the anatomic and pathologic extent, impacting use of adjuvant topical chemotherapy. In absence of map biopsy, it would be impossible to diagnose PAM sine pigmento. Additional corroborative work is needed to validate our observations.


2020 ◽  
Vol 132 (1) ◽  
pp. 159-167 ◽  
Author(s):  
Benjamin B. Whiting ◽  
Bryan S. Lee ◽  
Vaidehi Mahadev ◽  
Hamid Borghei-Razavi ◽  
Sanchit Ahuja ◽  
...  

OBJECTIVECurrent management of gliomas involves a multidisciplinary approach, including a combination of maximal safe resection, radiotherapy, and chemotherapy. The use of intraoperative MRI (iMRI) helps to maximize extent of resection (EOR), and use of awake functional mapping supports preservation of eloquent areas of the brain. This study reports on the combined use of these surgical adjuncts.METHODSThe authors performed a retrospective review of patients with gliomas who underwent minimal access craniotomy in their iMRI suite (IMRIS) with awake functional mapping between 2010 and 2017. Patient demographics, tumor characteristics, intraoperative and postoperative adverse events, and treatment details were obtained. Volumetric analysis of preoperative tumor volume as well as intraoperative and postoperative residual volumes was performed.RESULTSA total of 61 patients requiring 62 tumor resections met the inclusion criteria. Of the tumors resected, 45.9% were WHO grade I or II and 54.1% were WHO grade III or IV. Intraoperative neurophysiological monitoring modalities included speech alone in 23 cases (37.1%), motor alone in 24 (38.7%), and both speech and motor in 15 (24.2%). Intraoperative MRI demonstrated residual tumor in 48 cases (77.4%), 41 (85.4%) of whom underwent further resection. Median EOR on iMRI and postoperative MRI was 86.0% and 98.5%, respectively, with a mean difference of 10% and a median difference of 10.5% (p < 0.001). Seventeen of 62 cases achieved an increased EOR > 15% related to use of iMRI. Seventeen (60.7%) of 28 low-grade gliomas and 10 (30.3%) of 33 high-grade gliomas achieved complete resection. Significant intraoperative events included at least temporary new or worsened speech alteration in 7 of 38 cases who underwent speech mapping (18.4%), new or worsened weakness in 7 of 39 cases who underwent motor mapping (18.0%), numbness in 2 cases (3.2%), agitation in 2 (3.2%), and seizures in 2 (3.2%). Among the patients with new intraoperative deficits, 2 had residual speech difficulty, and 2 had weakness postoperatively, which improved to baseline strength by 6 months.CONCLUSIONSIn this retrospective case series, the combined use of iMRI and awake functional mapping was demonstrated to be safe and feasible. This combined approach allows one to achieve the dual goals of maximal tumor removal and minimal functional consequences in patients undergoing glioma resection.


2018 ◽  
Vol 54 (1) ◽  
pp. 50-59 ◽  
Author(s):  
Arathi Vinayak ◽  
Deanna R. Worley ◽  
Stephen J. Withrow ◽  
Dustin S. Adams ◽  
Barbara E. Powers

ABSTRACT This retrospective case series describes seven dogs and one cat diagnosed with dedifferentiated chondrosarcoma, an uncommon, aggressive variant of chondrosarcoma. The purpose of the study is to describe clinical, imaging, and histopathological findings of this tumor. Medical records and the diagnostic laboratory database at Colorado State University from 2000 to 2015 were reviewed and complete medical records were available for the eight animals in this report. Similar to what has been reported in people, poor long-term survival and high metastatic rate, particularly to the lungs, was observed in our case series. A bimorphic pattern on imaging (radiographs, computed tomography, and MRI) consisting of mineralized and nonmineralized areas was seen mirroring the high-grade sarcomatous component adjacent to a low-grade chondroid component seen histopathologically. A review of the human literature including suspected etiology, imaging findings, histopathology, and survival times with various treatment options is presented. This article describes the first reported cases of dedifferentiated chondrosarcoma in the veterinary literature. Early accurate recognition could lead to treatment plans tailored to this variant.


Cancers ◽  
2021 ◽  
Vol 14 (1) ◽  
pp. 196
Author(s):  
Yukinori Ozaki ◽  
Sakiko Miura ◽  
Ryosuke Oki ◽  
Teppei Morikawa ◽  
Keita Uchino

Breast tumors with neuroendocrine (NE) differentiation comprise an uncommon and heterogeneous group of tumors, including invasive breast cancer of no special type (IBC-NST) with NE features, neuroendocrine tumors (NETs), and neuroendocrine carcinoma (NEC). The most recent World Health Organization (WHO) classification in 2019 defined neuroendocrine neoplasms (NENs) of the breast (Br-NENs) as tumors in which >90% of cells show histological evidence of NE differentiation, including NETs (low-grade tumors) and NEC (high-grade). Due to the low prevalence of these tumors and successive changes in their diagnostic criteria over the years, only limited evidence of these tumors exists, derived mainly from case reports and retrospective case series. Breast tumors with NE differentiation are usually treated like the more commonly occurring IBC-NSTs. Immunohistochemistry (IHC) of breast tumors with NE differentiation usually shows a hormone receptor (HR)-positive and human epidermal growth factor type 2 (HER2)-negative profile, so that hormonal therapy with cyclin-dependent kinase (CDK)4/6 inhibitors or other targeted agents would be reasonable treatment options. Herein, we present a review of the literature on breast tumors with NE differentiation as defined in the latest WHO 2019 classification, and discuss the clinical management of these tumors.


Neurosurgery ◽  
2012 ◽  
Vol 72 (2) ◽  
pp. 216-220 ◽  
Author(s):  
Peter Kan ◽  
Shady Jahshan ◽  
Parham Yashar ◽  
David Orion ◽  
Sharon Webb ◽  
...  

Abstract BACKGROUND: Endovascular coil embolization of ruptured aneurysms is performed under general anesthesia at most centers for perceived improved image quality and patient safety. OBJECTIVE: To report the feasibility of and outcomes associated with endovascular treatment of subarachnoid hemorrhage (SAH) patients with ruptured cerebral aneurysms performed under conscious sedation with local anesthetics. METHODS: Between January 2005 and December 2009, 187 patients with aneurysmal SAH were treated with coil embolization at the authors' hospital. For each patient, procedural details, mode of anesthesia, and clinical and radiographic outcomes were reviewed retrospectively (retrospective case series). RESULTS: A total of 197 coil embolizations were performed: 112 under general anesthesia, 78 under conscious sedation with local anesthetics, and 7 converted from conscious sedation to general anesthesia. None of the patients who presented with Hunt & Hess grade IV or V were treated under conscious sedation. For patients who presented with Hunt & Hess grades I, II, and III, 79.2%, 66.7%, and 32.6% of patients, respectively, underwent successful completion of treatment under conscious sedation. The symptomatic procedural complication rate was 2.5% overall and 2.4% for the conscious sedation group alone. Among the 14 interventions with intraprocedural perforation, 11 were performed under general anesthesia and 3 were performed under conscious sedation. CONCLUSION: In the authors' experience, conscious sedation with local anesthetics for endovascular treatment of ruptured intracranial aneurysms is feasible and safe in most patients with low-grade SAH. It may allow direct evaluation of the patient's neurological status, potentially leading to earlier detection and response to intraprocedural complications.


2020 ◽  
Vol 5 (1) ◽  
pp. e000483
Author(s):  
Giannis A Moustafa ◽  
Allan K Topham ◽  
Mary E Aronow ◽  
Demetrios G Vavvas

ObjectiveTo investigate the incidence, clinicopathological characteristics and survival of ocular adnexal lymphoma (OAL) in the paediatric population.Methods and analysisIn this retrospective case series, the Surveillance, Epidemiology and End Results database was accessed to identify individuals with OAL ≤18 years of age, diagnosed between 1973 and 2015. OAL located in the eyelid, conjunctiva, lacrimal apparatus and orbit were included. Main outcome measures were the age-adjusted incidence rates (IRs) per 1 000 000 population at risk (calculated for the period 2000–2015) and descriptive statistics of demographic and clinicopathological features.ResultsThe IR of paediatric OAL was 0.12 (95% CI 0.08 to 0.16) per 1 000 000. Males (0.15; 95% CI 0.10 to 0.22) and blacks (0.24; 95% CI 0.13 to 0.42) had a higher tendency for OAL development. A total of 55 tumours in 54 children were identified. The majority were localised (78.4%), conjunctival (49.1%) lymphomas. Extranodal marginal zone lymphoma (EMZL, 45.5%, n=25) was the most frequent subtype, followed by diffuse large B-cell lymphoma (DLBCL, 9.1%, n=5), B lymphoblastic lymphoma (7.3%, n=4), follicular lymphoma (5.5%, n=3), Burkitt lymphoma (5.5%, n=3), anaplastic large cell lymphoma (ALCL, 3.6%, n=2), small lymphocytic lymphoma (1.8%, n=1), diffuse large B-cell lymphoma, immunoblastic (1.8%, n=1) and panniculitis-like T-cell lymphoma (1.8%, n=1). Localised, low-grade, conjunctival lymphomas were frequently treated with complete excision with or without radiation, while high-grade and distant tumours usually received chemotherapy. Only 29.1% of paediatric OAL cases were treated with radiation. Three out of five (60%) patients with DLBCL died of lymphoma at a median follow-up of 21 (range 10–86) months, and 1 out of 2 (50%) patients with ALCL died of lymphoma at 23 months from diagnosis.ConclusionOAL in the paediatric population is rare. The majority of OAL are EMZL and are characterised by excellent prognosis. The histological subtype was found to be the main predictor of outcome with cancer-specific deaths observed in patients with DLBCL and ALCL.


2008 ◽  
Vol 1 (2) ◽  
pp. 86-90
Author(s):  
Erdinç AYDIN ◽  
Seda TÜRKOĞLU ◽  
İsmail KIRBAŞ ◽  
Figen ÖZÇAY

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