Primary acquired melanosis/melanoma: utility of conjunctival map biopsy

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.

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.


2014 ◽  
Vol 2 (1) ◽  
pp. 9-14
Author(s):  
Sheikh Shawkat Kamal

Aim: To evaluate the role of newly created transcanal endoscopic antrostomy hole as a passage in restoring the aeration of epitympanum and mastoid antrum.Materials & Methods: Study design: Retrospective case series study. Study duration: From January 2013 to January 2014. Study place: Private tertiary care hospital. Patients: 27  ears of 23 adult patients (age ranging from 15years to 54years) underwent transcanal endoscopic tympanoplasty for their chronic middle ear diseases in presence of inflamed middle ear mucosa along with radiological shadows of stagnant fluid in their epitympanum and mastoid antrum. Structured three months follow-up was ensured in every case. Intervention: A hole was created at a selected site of posterior meatal wall purely through transcanal endoscopic approach that established direct communication between mastoid antrum and external auditory canal. Thereafter this newly created passage was used for three purposes- assessment of the condition of mastoid antrum, performing the water test for checking epitympanic patency and in few cases placement of temporary tube for postoperative ventilation and drainage of middle ear. Main out come measures: The feasibility, performance and management of transcanal endoscopic antrostomy hole as passage for reestablishing the aeration of epitympanum and mastoid antrum.Results: The chosen site for antrostomy hole was found effective and safe in providing convenient entrance into the mastoid antrum in every case in this study with out facing technical complexity and failure. Postoperative healing of skin over antrostomy hole was found complete in all ears without any inward growth of skin in to mastoid antrum. Available post operative CT scan imaging of temporal bones showed improved aeration in their epitympanum and mastoid antrum.Conclusions: The role of transcanal antrostomy hole has been proved worthy in restoring ventilation pathway to epitympanum and mastoid antrum during tympanoplasty. This antrostomy hole has the potentiality to be considered in future for placement of long term mastoid ventilation tube in order to treat persistent atelectatic middle ear.


2021 ◽  
pp. 239698732110594
Author(s):  
Antonio Ochoa-Ferraro ◽  
Subadra Wanninayake ◽  
Charlotte Dawson ◽  
Adam Gerrard ◽  
Mary Anne Preece ◽  
...  

Background Cerebral venous thrombosis (CVT) is an important cause of stroke particularly in younger patients and potentially fatal if diagnosis is delayed. The presentation of symptoms is highly variable and consequently the diagnosis and underlying cause is often delayed or overlooked. Homocystinuria, a rare autosomal recessive disorder is an identified risk factor for CVT. Purpose A timely diagnosis and treatment of the underlying cause of CVT could result in improved outcome and prevent further events. This case series describes the clinical course of six adults presented with unprovoked CVT, in whom the diagnosis of underlying homocystinuria was delayed with adverse consequences. We aim to highlight the importance of recognising homocystinuria as an underlying cause of CVT and offer a practical approach to the diagnosis and management. Methods This is a retrospective case series of a cohort of 30 consecutive patients seen in a UK tertiary referral centre. Result Six out of 30 patients presented with CVT prior to homocystinuria diagnosis. The mean and range of age at the time of the first CVT episode was 22.6 (range 11–31) years. The mean ±SD age at diagnosis of homocystinuria as the underlying cause was 26 ± 4.2 years. The time between first CVT and diagnosis of homocystinuria ranged from 1.6 to 11 years resulting in a delay to introduction of effective treatment and, in some cases, a further large vessels thrombotic event. Conclusion Physician awareness of homocystinuria as an underlying cause for an unprovoked CVT will facilitate timely introduction of effective treatment to prevent a further event.


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.


2014 ◽  
Vol 7 (5) ◽  
pp. 377-386 ◽  
Author(s):  
Stephen A. Brigido ◽  
Nicole M. Protzman ◽  
Melissa M. Galli ◽  
Scott T. Bleazey

Cystic talar shoulder defects are particularly challenging osteochondral lesions. A retrospective chart review was performed on 13 adults that previously failed microfracture, presented with medial cystic osteochondral lesions of the talus, and were treated with malleolar osteotomy and subchondral allograft reconstruction. The aim of the study was to evaluate the effect of a medial malleolar osteotomy and allograft subchondral bone plug on pain and function. We hypothesized that following surgery, pain and function would significantly improve. Compared with preoperative measures, pain (first step in the morning, during walking, at the end of the day) and function (descending the stairs, ascending the stairs, and ambulating up to 4 blocks) improved postoperatively at 6 and 12 months ( P ≤ .001). During each activity, pain improved postoperatively from 6 to 12 months ( P ≤ .006). Postoperatively, from 6 to 12 months, the level of disability improved while descending the stairs ( P = .004), and the level of disability experienced while ascending the stairs and ambulating up to 4 blocks was maintained ( P ≥ .02). Multiple regression analyses identified body mass index as a predictor of preoperative function ( R2 = .34, P = .04). No variables were identified as significant predictors of postoperative pain or function. With all osteotomies healing, no graft rejection, and a single deep venous thrombosis, allograft subchondral plugs appear to successfully treat osteochondral lesions of the talus with improvements in pain and function as well as an acceptable complication rate. Level of Evidence: Therapeutic, Level IV: Retrospective Case Series.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 10504-10504 ◽  
Author(s):  
Jason R. Fangusaro ◽  
Arzu Onar-Thomas ◽  
Tina Young-Poussaint ◽  
Shengjie Wu ◽  
Azra H Ligon ◽  
...  

10504 Background: A greater understanding of the Ras-MAP kinase-signaling pathway in pediatric low-grade glioma (LGG) paired with the availability of potent selective inhibitors has enhanced the ability to target this pathway with therapeutic intent. Methods: The PBTC conducted a multi-institutional phase II study (NCT01089101) evaluating selumetinib (AZD6244, ARRY-142886), a MEK I/II inhibitor, in children with recurrent/refractory LGG assigned to 6 strata and treated at 25 mg/m2/dose PO BID for up to two years. Here we present the data from three of these strata. The remaining strata are still accruing patients. Results: Stratum I included children with non-NF-1 and non-optic pathway recurrent/refractory pilocytic astrocytoma (PA) harboring BRAF aberrations (BRAF V600e mutation or the BRAF-KIAA 1549 fusion). Eight of 25 (32%) patients achieved a partial response (PR) with 2-year PFS of 66+/-11%. Two of 7 (29%) patient tumors with a BRAF V600e mutation and 6/18 (33%) with a BRAF KIAA-1549 fusion had a PR. Stratum 3 enrolled NF-1-associated LGG. Tissue for tumor BRAF evaluation was not required for eligibility. Ten of 25 (40%) achieved PR with a 2-year PFS of 96+/-4%. Only one patient progressed while on treatment. Stratum 4 included children with non-NF-1 optic pathway/hypothalamic LGG. Tissue for tumor BRAF evaluation was not required for eligibility. Two of 16 (12.5%) had a PR with a 2-year PFS of 65+/-13%. The BRAF aberration status of the responders in strata 3 and 4 is mostly unknown. All responses were confirmed centrally and seven patients remain on treatment. The most common toxicities were grade 1/2 CPK elevation, diarrhea, hypoalbuminemia, elevated AST and rash. Rare grade 3/4 toxicities included elevated CPK, rash, neutropenia, emesis and paronychia. Conclusions: Selumetinib was effective in treating children with recurrent/refractory LGG, including those with NF-1 associated LGG and PA harboring BRAF V600e mutation or BRAF-KIAA 1549 fusion. Larger prospective studies are necessary to determine the future, specific role of this agent in treating children with LGG harboring specific molecular aberrations. Clinical trial information: NCT01089101.


2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e13538-e13538
Author(s):  
Marc C. Chamberlain ◽  
Bryan T. Kim

e13538 Objective: A single institution retrospective evaluation of nivolumab following disease progression on bevacizumab in adults with recurrent glioblastoma (GBM) with an objective of determining progression free survival (PFS). Background: There is no accepted therapy for recurrent GBM after failure of bevacizumab. Methods: 16 adults, ages 52-72 years (median 62), with recurrent GBM were treated. All patients had previously been treated with surgery, concurrent radiotherapy and temozolomide, and post-radiotherapy temozolomide. Bevacizumab (with or without lomustine) was administered to all patients at first recurrence. Patients were treated with nivolumab only (3mg/kg) once every 2 weeks at second recurrence. One cycle of nivolumab was defined as 2 treatments. Neurological evaluation was performed bi-weekly and neuroradiographic assessment every 4 weeks. Results: A total of 37 treatment cycles (median 2) were administered of nivolumab in which there were 14 Grade 2 adverse events (AEs) and Grade 3 AEs in 2 patients. No Grade 4 or 5 AEs were seen. Following 1 month of nivolumab, 7 patients’ demonstrated progressive disease and discontinued therapy. No patient demonstrated a response though 9 patients demonstrated neuroradiographic stable response. Survival in the entire cohort ranged from 2 - 6 months with a median of 3.5 months (CI: 2.8, 4.2). Median and 6-month PFS at 6 months was 2.0 months (range 1-5 months; CI: 1.3, 2.7) and 0% respectively. Conclusions: Nivolumab salvage therapy demonstrated no survival advantage in patients with recurrent bevacizumab refractory GBM emphasizing a continued unmet need in neuro-oncology.


2013 ◽  
Vol 11 (8) ◽  
pp. 714-715 ◽  
Author(s):  
Annalise Katz-Summercorn ◽  
Clare Weaver ◽  
Mikael Södergren ◽  
Sarah Huf ◽  
Krishna Moorthy

2004 ◽  
Vol 118 (1) ◽  
pp. 31-33 ◽  
Author(s):  
Achamma Balraj ◽  
Mary Kurien ◽  
Anand Job

Treatment of the predisposing factors that are identified in the nose and throat in several ENT diseases is mandatory prior to the definitive management of the latter. When surgical management is indicated it has been traditional to use staged procedures. This study was undertaken to assess the role of concurrent surgical procedures in ENT and evaluate their cost-effectiveness compared to similar staged procedures. This was a retrospective case series of 100 consecutive patients undergoing concurrent and similar staged ENT surgical procedures. On analysis, it was noted that the average duration of surgery, anaesthesia and hospital stay was significantly less in the concurrent procedures group than in the staged procedures (2.35/3.1.hours; 3.05/3.30.hours and 2.5/6.5 days, respectively). The average hospital bill for the concurrent procedures was also lower than for the staged procedures. Hence, in patients requiring multiple ENT surgical procedures for definitive treatment, concurrent procedures are more cost-effective than staged procedures and should be considered the treatment of choice in a tertiary care centre.


F1000Research ◽  
2015 ◽  
Vol 4 ◽  
pp. 1103 ◽  
Author(s):  
Xiao Li ◽  
Josh Golubovsky ◽  
Joyce Hui-Yuen ◽  
Ummara Shah ◽  
Ewa Olech ◽  
...  

Objectives: Acthar Gel is a long-acting formulation of adrenocorticotropic hormone (ACTH) with anti-inflammatory effects thought to be mediated in part through melanocortin receptor activation. This study was initiated to understand the role of Acthar Gel in SLE treatment in rheumatology practices.Methods: This is a retrospective case series of nine adult female patients treated with Acthar Gel for at least six months at five academic centers. Treating physicians completed a one-page questionnaire on lupus medications, disease activity, and outcomes. Clinical response was defined using SLEDAI 2K and improvement in the clinical manifestation(s) being treated.Results: The most common clinical SLE manifestations/indications requiring therapy with Acthar Gel were arthritis, rash, and inability to taper corticosteroids. The mean SLEDAI 2K score at baseline was 5.8 (range 0-16). Six patients were concomitantly treated with corticosteroids (mean dose 18.3mg/day). All patients were on background SLE medications including immunosuppressives. Seven of nine patients had an overall improvement, with a decrease in SLEDAI 2K to 3.5; four of five patients had improvement or resolution in arthritis, and one of two patients had resolution of inflammatory rash. Four patients discontinued corticosteroids and one patient tapered below 50% of the initial dose by 3 months of treatment with Acthar Gel. No adverse events were reported.Conclusions: This study suggests a role for Acthar Gel as an alternative to corticosteroids in the treatment of SLE. Acthar Gel appears to be safe and well-tolerated after 6 months of treatment, with a significant reduction in disease activity.


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