Incidence of brain metastases and overall survival in patients with primary cardiac sarcoma: A retrospective case series.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. 10584-10584
Author(s):  
Maryann Shango ◽  
Lili Zhao ◽  
Monika Leja ◽  
Jonathan B. McHugh ◽  
Scott Schuetze ◽  
...  

10584 Background: Primary cardiac sarcoma (PCS) is the most common primary cardiac malignancy, but is a rare primary site of sarcoma. We present 21 cases from a tertiary care center to better understand this uncommon malignancy. Methods: A cancer center-based registry and pathology database were searched to identify pts diagnosed with PCS from 1992-2013 at University of Michigan. Kaplan-meier method was used to estimate survival. Cox proportional hazard model was used to associate variables to occurrence of metastases (mets) or death. Results: Atotal of 21 pts (F12, 9M) with PCS were identified, median age 36 (range 11-74). The most common presenting symptoms included dyspnea (16) and chest pain (6; 5 with associated pericardial effusion). Histologies included: angiosarcoma (9), leiomyosarcoma (4), undifferentiated pleomorphic (3), spindle cell (2), fibrosarcoma (1), rhabdomyosarcoma (1) and synovial (1). Sites of origin were R atrium (7), R ventricle (2), L atrium (10) and pericardium (2). Ten pts presented with mets; most common sites were lung (8), liver (2), brain (2), pancreas (2) and bone (2). Surgery was attempted in 12 pts, achieving 1 R0 resection. Pts received a median of 1 (0-7) systemic therapies. Median overall (OS) was 12.6 mos (range 3-79) from diagnosis. Pts without prior surgery were more likely to have mets or death (p=0.038). Brain mets were common, occurring in 7 of 21 pts after a median of 7 mos (range 1-75) from diagnosis. Median OS after diagnosis of brain mets was 8 mos. Of the 7 pts who developed brain metastasis, 5 had PCS originating in the left heart. Of the 2 pts with PCS in the right heart, one was evaluated for and had a right to left shunt. The likelihood of developing brain mets did not correlate with age, chemotherapy, or surgery. Conclusions: PCS portends an extremely poor prognosis, marked by inability to achieve complete resection and a high incidence of disseminated disease at diagnosis. Metastatic disease to the brain was much more common in PCS (33%) as compared to STS of any origin (approximately 1-8%), particularly in pts with PCS originating in the left heart. Clinicians should have a low threshold for brain imaging evaluation of PCS pts.

2019 ◽  
Vol 162 (2) ◽  
pp. 234-240 ◽  
Author(s):  
Claire M. Lawlor ◽  
Natasha D. Dombrowski ◽  
Roger C. Nuss ◽  
Reza Rahbar ◽  
Sukgi S. Choi

Objective To discuss the presentation, evaluation, and management of pediatric laryngeal web. Study Design Retrospective case series. Setting Single tertiary care center. Subjects All patients with laryngeal web at Boston Children’s Hospital in the past 22 years. Methods No exclusion criteria. Charts mined for age at presentation, presenting symptoms, degree/location of web, associated syndromes, number/type of surgical procedures, and postoperative outcomes. Results Thirty-seven patients were included (13 male, 24 female). Average age at diagnosis was 3.7 years (0-19.5 years). Mean follow-up was 4.4 years (range, 0-16.4 years). There were 26 congenital webs (70.2%) and 11 acquired webs (29.8%). Presenting symptoms were vocal (29 patients, 78.4%) and respiratory (22 patients, 60%). Underlying syndromes or synchronous airway lesions included the following: premature (n = 5), congenital heart disease (n = 18), subglottic stenosis (n = 5), 22q11.2 deletion syndrome (n = 10), and recurrent respiratory papillomatosis (n = 4). There were 20 type 1 webs, 6 type 2 webs, 8 type 3 webs, and 3 type 4 webs; 10 had subglottic extension of the laryngeal web. Twelve patients were managed conservatively with observation. Eighty-four interventions were performed: 18 open and 66 endoscopic (sharp division, 32; dilation, 33; mitomycin C, 14; laser, 5; keel, 6; triamcinolone injection, 8; stent, 15; removal of granulation tissue, 5). Tracheotomy was required in 11 patients, and 5 patients were decannulated. Voice improved in 12 patients, with respiratory symptoms in 12 patients. Web recurred in 17 patients. One patient died due to airway complications. Conclusions Pediatric laryngeal web is an uncommon but challenging lesion. Patients need to be evaluated for comorbid syndromes and synchronous airway lesions. Management includes open and endoscopic procedures. Procedures should be tailored to the child’s presentation.


2020 ◽  
Vol 130 (1) ◽  
pp. 38-46
Author(s):  
Geoffrey Casazza ◽  
Matthew L. Carlson ◽  
Clough Shelton ◽  
Richard K. Gurgel

Objective: Describe the outcomes of treatment for patients with cholesteatomas that are medially invasive to the otic capsule, petrous apex, and/or skull base. Study Design: Retrospective case series Setting: Two tertiary care academic centers. Patients: Patients surgically managed for medially-invasive cholesteatoma at two tertiary care institutions from 2001 to 2017. Interventions: Surgical management of medially-invasive cholesteatomas. Main Outcome Measures: The presenting symptoms, imaging, pre- and post-operative clinical course, and complications were reviewed. Results: Seven patients were identified. All patients had pre-operative radiographic evidence of invasive cholesteatoma with erosion into the otic capsule beyond just a lateral semicircular canal fistula. Five patients had a complex otologic history with multiple surgeries for recurrent cholesteatoma including three with prior canal wall down mastoidectomy surgeries. Average age at the time of surgery was 41.3 years (range 20-83). Two patients underwent a hearing preservation approach to the skull base while all others underwent a surgical approach based on the extent of the lesion. Facial nerve function was maintained at the pre-operative level in all but one patient. No patient developed cholesteatoma recurrence. Conclusions: The medially-invasive cholesteatoma demonstrates an aggressive, endophytic growth pattern, invading into the otic capsule or through the perilabyrinthine air cells to the petrous apex. Surgical resection remains the best treatment option for medially-invasive cholesteatoma. When CSF leak is a concern, a subtotal petrosectomy with closure of the ear is often necessary.


Author(s):  
Shashidhar S. Suligavi ◽  
Mallikarjun N. Patil ◽  
S. S. Doddamani ◽  
Chandrashekarayya S. Hiremath ◽  
Afshan Fathima

<p class="abstract"><strong><span lang="EN-US">Background:</span></strong>Tracheo- bronchial foreign bodies have always posed a challenge to the ENT surgeon as they present with varied symptomatology ranging from a simple cough and fever to more grave respiratory distress. It requires a strong suspicion, early diagnosis and timely intervention to reduce the overall morbidity and mortality .This study was undertaken to highlight our experiences in handling cases of tracheo- bronchial foreign bodies (FB) at our setup.</p><p class="abstract"><strong><span lang="EN-US">Methods:</span></strong>It is a retrospective case series study conducted in S. Nijalingappa Medical College between January 2011 and January 2015.  </p><p class="abstract"><strong><span lang="EN-US">Results:</span></strong>Most commonly affected were children between 1year to 3years of age. Chronic cough and wheeze were the commonest presenting symptoms. Vegetative foreign body was found to be the commonest variety of foreign body. The mortality rate in our study was 4.7% (n=3).</p><p class="abstract"><strong><span lang="EN-US">Conclusions:</span></strong>A good clinical acumen, team work, early diagnosis and timely intervention are all needed to reduce the overall mortality and morbidity associated with tracheo- bronchial foreign bodies.</p>


2020 ◽  
Vol 9 (02) ◽  
pp. 105-108
Author(s):  
Manasi C. Mundada ◽  
Faiq Ahmed ◽  
Rachna Khera ◽  
Sudha Murthy ◽  
Senthil Rajappa ◽  
...  

Abstract Background Plasmablastic lymphoma (PBL) is a rare aggressive B cell lymphoma that is commonly encountered in patients with human immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS). In this case series, we describe the clinicopathological features of cases of PBL seen at a tertiary care center in South India. Materials and Methods Medical records of patients diagnosed with PBL between January 2009 and November 2017 were reviewed. PBL was defined as per the World Health Organization 2016 classification for hematopoietic and lymphoid neoplasms. The slides were reviewed with hematoxylin and eosin along with immunohistochemistry (IHC) including CD45, CD20, PAX5, CD79a, CD3, CD5, CD138, MUMI, EMA, ALK, and Ki67. Epstein-Barr virus (EBV) association was documented by rapid in situ hybridization (RISH) studies wherever possible. The demographic data, clinical presentation, treatment details, and outcomes are elaborated using descriptive statistics. Results During the study period, nine patients with PBL were identified. The median age at presentation was 47 years (range: 36–54 years). All patients had associated HIV/AIDS, eight (89%) had extranodal disease, and six (66%) had advanced clinical stage (stage III). All biopsies were positive for CD45, CD138, and MUM1, and negative for CD79a and T cell markers with a high Ki67 proliferation index (85–90%); CD20 was faint positive in one patient, and CD56 was positive in one (11%) patient. EBV-RISH was tested in two patients and was positive in one. Bone marrow was uninvolved in all the cases. At the time of last follow-up, three patients were alive. Treatment details were available in six patients. With frontline therapy, four patients achieved a complete remission (CR) and one patient developed progressive disease. Three of four patients in CR are alive till the last follow-up. Conclusion PBL is a rare form of lymphoma with predominant association with HIV, extranodal location, and characteristic IHC pattern.


Cephalalgia ◽  
2021 ◽  
pp. 033310242199635
Author(s):  
Dev G Mehta ◽  
Ivan Garza ◽  
Carrie E Robertson

Objective To review characteristics and outcomes of all cases of visual snow seen at our institution, with attention to possible triggering events or comorbidities. Methods This is a retrospective case series of patients seen at our tertiary care center from January 1994 to January 2020. Charts were reviewed if they contained the term “visual snow”. Results Of the 449 charts reviewed, 248 patients described seeing visual snow in part or all of their vision. Thirty-eight reported transient visual snow as their typical migraine aura. Of the remaining 210 patients, 89 were reported to have either an inciting event or contributing comorbidity for their visual snow symptoms, including: Post-concussion (n = 15), dramatic change in migraine or aura (n = 14), post-infection (n = 13), hallucinogen persisting perception disorder (n = 10), ocular abnormalities (n = 7), idiopathic intracranial hypertension (n = 4), neoplastic (n = 1), and posterior cortical atrophy (n = 1). Some patients had partial improvement with benzodiazepines (n = 6), lamotrigine (n = 5), topiramate (n = 3) and acetazolamide (n = 3). Presenting characteristics were similar, but patients with visual snow attributed to an inciting event or contributing comorbidity were more likely to have some improvement in their symptoms by last follow-up compared to spontaneous visual snow ( p < .001). Conclusions Though most cases of visual snow are spontaneous, potential secondary causes should be recognized by clinicians. Patients who develop visual snow after an inciting event or related to an underlying comorbidity may have a better prognosis than those in whom it develops spontaneously. In select cases, treatment of the suspected underlying cause may significantly alleviate the otherwise typical intractable visual disturbances associated with visual snow.


2020 ◽  
Vol 35 (9) ◽  
pp. 591-599 ◽  
Author(s):  
Alice Rutatangwa ◽  
Nikita Mittal ◽  
Carla Francisco ◽  
Kendall Nash ◽  
Emmanuelle Waubant

Autoimmune encephalitis is the third most common cause of encephalitis in children. We provide a detailed account of presenting symptoms, diagnosis, and response to treatment in pediatric autoimmune encephalitis patients evaluated at University of California San Francisco within a 2.5-year period. Eleven were identified: anti– N-methyl-d-aspartate receptor (NMDAR) encephalitis (n = 4), antibody-negative autoimmune encephalitis (n = 4), steroid-responsive encephalopathy associated with thyroiditis (SREAT) (n = 2), and glial fibrillary acidic protein (GFAP)–associated encephalitis (n = 1). Most common presenting symptoms included seizures and behavior changes (54%). More than 90% of patients showed improvement following first-line immunotherapy (high-dose corticosteroids, intravenous immunoglobulin, and/or plasma exchange). A total of 64% received second-line treatment with rituximab, cyclophosphamide, or mycophenolate mofetil. One patient with NMDAR encephalitis died despite escalating immunotherapy. None of the patients showed complete recovery after median follow-up of 9 months (range 0.5-66). Children with autoimmune encephalitis have a diverse clinical presentation and may lack an identifiable autoantibody. Majority of patients show a good response to immunotherapy; however, recovery can be delayed.


2021 ◽  
pp. 019459982098266
Author(s):  
Emily E. Karp ◽  
Linda X. Yin ◽  
Eric J. Moore ◽  
Anna J. Elias ◽  
Thomas J. O’Byrne ◽  
...  

Objective Failure to recognize symptoms of human papillomavirus–associated oropharyngeal squamous cell carcinoma (HPV(+)OPSCC) at presentation can delay diagnosis and treatment. This study aims to identify patient factors and provider patterns that contribute to delayed diagnosis. Study Design Retrospective case series. Setting Tertiary care center. Methods Patients with HPV(+)OPSCC receiving intent-to-cure treatment from 2006 to 2016. Clinical data, workup, and care timelines were abstracted. Univariate and multivariable linear regressions were performed to determine associations. Results Of 703 included patients, 627 (89%) were male, and mean (SD) age at diagnosis was 59 (9) years. The mean (SD) delay to diagnosis was 148.8 (243.51) days, with an average delay of 63 (154.91) days from symptom onset to first presentation and 82.8 (194.25) days from first presentation to diagnosis. Most patients visited at least 2 providers (n = 546, 78%) before diagnosis and saw their primary care physician at first presentation (n = 496, 71%). The most common imaging and biopsy obtained before diagnosis was neck computed tomography (n = 391, 56%) and neck fine-needle aspiration (n = 423, 60%), respectively. On multivariable linear regression, being a homemaker, being a current smoker, seeing 3 or more providers, and getting a magnetic resonance imaging scan were associated with significant delays in diagnosis ( P < .01, all). Treatment with antibiotics and a suspicion for HPV(+)OPSCC at first presentation were associated with decreased delays in diagnosis ( P < .01, both). Conclusions Patient delays in seeking medical attention and provider delays in recognizing the appropriate diagnosis both contribute to delays of care in HPV(+)OPSCC. Improved patient and provider education is necessary to expedite the diagnosis of HPV(+)OPSCC.


2021 ◽  
Vol 12 (02) ◽  
pp. 067-070
Author(s):  
Manish Kumar ◽  
Sanjeev Sachdeva ◽  
Ajay Kumar ◽  
Ujjwal Sonika ◽  
Manish Gupta ◽  
...  

Abstract Objective Gastric foreign bodies are a common problem brought to an endoscopist. Prisoners are more prone to ingesting unusual foreign bodies for a secondary gain. The objective of this study was to study the profile of foreign body ingestion among the prisoners brought to a tertiary care center. Methods This is a retrospective case series. All the prisoners brought for endoscopic removal of foreign bodies between January 2018 to December 2019 were included in the analysis. Patients’ baseline characteristics, type of foreign body, management, and outcome were noted Results A total of eight inmates presented with ingestion of foreign body to our department. Most common foreign bodies ingested were drug packets and mobile phones. All ingestions were for secondary gain. Endoscopic removal was successful in all cases. Conclusion Endoscopic removal can be tried with all necessary precautions by an expert endoscopist.


2020 ◽  
pp. 112067212092645
Author(s):  
Tina Felfeli ◽  
Kamiar Mireskandari ◽  
Asim Ali

Purpose To report the characteristics and longitudinal visual outcomes of traumatic cataracts and retinal detachments in children with self-inflicted injury. Methods A retrospective case series of pediatric patients at a tertiary care center who sustained ocular trauma due to self-inflicted injury between 2000 and 2014. Results A total of 11 children, all with an intellectual disability and a mean age of 10 ± 4.8 years (range, 2.8–16.2), were identified with traumatic cataract secondary to ocular self-inflicted injury over the study period. Five eyes had a concurrent unilateral retinal detachment. Over the course of follow-up, one additional eye developed a cataract and five eyes developed a retinal detachment. Patients underwent an average of 2.5 ± 2.3 procedures and 17.1 ± 10.7 eye examinations over 11.7 ± 4.2 years of follow-up; 36% required general anesthesia for examination. Visual acuity was improved or preserved in 77% of the eyes that underwent surgical interventions, but was worse in the remaining cases due to development of traumatic retinal detachment. The use of protective helmets, eye shields, and immobilizing orthoses were essential in management of active self-inflicted injury. Conclusion Traumatic cataracts and retinal detachments due to self-inflicted injury may cause severe visual loss. Visual prognosis although poor in children with severe intellectual disabilities may be optimized with risk awareness for early detection and customized interventions.


Sign in / Sign up

Export Citation Format

Share Document