scholarly journals Necrolytic migratory erythema: an important visual cutaneous clue of glucagonoma

2022 ◽  
Author(s):  
Wei Li ◽  
Xue Yang ◽  
Yuan Deng ◽  
Yina Jiang ◽  
Guiping Xu ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Symptom Relief ◽  
Somatostatin Analogues ◽  
Tumor Control ◽  
Somatostatin Analogue ◽  
Imaging Studies ◽  
Presenting Symptoms ◽  
The Past ◽  
Necrolytic Migratory Erythema

Abstract Objective: Glucagonoma is an extremely rare neuroendocrine tumor that arises from pancreatic islet alpha cells. Although glucagonoma is usually accompanied by a variety of characteristic clinical symptoms, early diagnosis is still difficult due to the scarcity of the disease. Methods: In this study, we present the cumulative experiences, clinical characteristics and treatments of seven patients diagnosed with glucagonoma during the past 10 years at the First Affiliated Hospital of Xi’an Jiaotong University. Results: The seven patients in our cohort consisted of six females and one male with an average diagnosis age of 40.1 years (range 23-51). The average time from onset of symptoms to diagnosis of glucagonoma was 14 months (range 2-36 months). All the patients visited dermatology firstly for necrolytic migratory erythema (NME) 7/7 (100%), other presenting symptoms included: diabetes mellitus (DM) 4/7 (57%), stomatitis 2/7 (28%), weight loss 4/7 (57%), anemia 4/7 (57%), diarrhea 1/7 (14%), DVT1/7 (14%). Plasma glucagon levels were increased in all patients (range 216.92–3155 pg/mL), and declined after surgery. Imaging studies revealed that four of seven patients had liver metastasis. Six of seven patients received surgical resection, and all of them received somatostatin analogue therapy. Symptoms improved significantly in 6 out of 7 patients. Three of seven patients died of this disease by the time of follow-up. Conclusion: Our data suggest that if persistent NME is associated with DM and high glucagon levels, timely abdominal imaging should be performed to confirm glucagonoma. Once diagnosed, surgery and somatostatin analogues are effective for symptom relief and tumor control.

scholarly journals Treatment and therapeutic strategies for pituitary apoplexy in pregnancy: a case series

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Yuya Kato ◽  
Yoshikazu Ogawa ◽  
Teiji Tominaga
Keyword(s):  
Pituitary Apoplexy ◽  
Clinical Symptoms ◽  
Case Series ◽  
Optimal Timing ◽  
Presenting Symptoms ◽  
Pathological Evaluation ◽  
Mother And Child ◽  
Life Threatening ◽  
In Pregnancy

Abstract Background Pregnancy is a known risk factor for pituitary apoplexy, which is life threatening for both mother and child. However, very few clinical interventions have been proposed for managing pituitary apoplexy in pregnancy. Case presentation We describe the management of three cases of pituitary apoplexy during pregnancy and review available literature. Presenting symptoms in our case series were headache and/or visual disturbances, and the etiology in all cases was hemorrhage. Conservative therapy was followed until 34 weeks of gestation, after which babies were delivered by cesarean section with prophylactic bolus hydrocortisone supplementation. Tumor removal was only electively performed after delivery using the transsphenoidal approach. All three patients and their babies had a good clinical course, and postoperative pathological evaluation revealed that all tumors were functional and that they secreted prolactin. Conclusions Although the mechanism of pituitary apoplexy occurrence remains unknown, the most important treatment strategy for pituitary apoplexy in pregnancy remains adequate hydrocortisone supplementation and frequent hormonal investigation. Radiological follow-up should be performed only if clinical symptoms deteriorate, and optimal timing for surgical resection should be discussed by a multidisciplinary team that includes obstetricians and neonatologists.

scholarly journals Isolated Tuberculous Tenosynovitis of the Anterior Tibial and Extensor Digitorum Longus Tendons

2013 ◽  
Vol 3 ◽  
pp. 37 ◽  
Author(s):  
Berhan Genç ◽  
Aynur Solak ◽  
Aslan Mayda ◽  
Nazime Şen
Keyword(s):  
Clinical Symptoms ◽  
Granulomatous Inflammation ◽  
Tendon Sheath ◽  
Imaging Studies ◽  
Rare Form ◽  
Common Diagnosis ◽  
Anterior Tibial ◽  
Histopathological Studies ◽  
Tuberculous Tenosynovitis

Musculoskeletal system is involved in 1-5% of extrapulmonary cases of tuberculosis. Tuberculous tenosynovitis is a rare form of musculoskeletal tuberculosis. Tuberculosis of the tendon sheath in the hand has been seen in a few cases. Involvement of the tendons of the leg is less common. Diagnosis is not easy as there are no specific clinical symptoms or signs. A 33-year-old male presented with painful swelling in the distal right lower limb that caused restriction of movement. Imaging studies showed inflammation and infection of the extensor digitorium longus and tibialis anterior tendons. Histopathological studies showed a necrotizing granulomatous inflammation in the synovial tissue. A diagnosis of tuberculosis was made and medical treatment was initiated that proved successful. Patient remained infection-free at 26-month follow-up examination.

scholarly journals The Changing Paradigm for the Surgical Treatment of Large Vestibular Schwannomas

2018 ◽  
Vol 79 (S 04) ◽  
pp. S362-S370 ◽  
Author(s):  
Constantin Tuleasca ◽  
Alda Rocca ◽  
Mercy George ◽  
Etienne Pralong ◽  
Luis Schiappacasse ◽  
...  
Keyword(s):  
Functional Outcome ◽  
Residual Tumor ◽  
University Hospital ◽  
Tumor Control ◽  
Subtotal Resection ◽  
Consecutive Series ◽  
Cochlear Function ◽  
Nerve Preservation ◽  
The Past

Objective Planned subtotal resection followed by Gamma Knife surgery (GKS) in patients with large vestibular schwannoma (VS) has emerged during the past decade, with the aim of a better functional outcome for facial and cochlear function. Methods We prospectively collected patient data, surgical, and dosimetric parameters of a consecutive series of patients treated by this method at Lausanne University Hospital during the past 8 years. Results A consecutive series of 47 patients were treated between July 2010 and January 2018. The mean follow-up after surgery was 37.5 months (median: 36, range: 0.5–96). Mean presurgical tumor volume was 11.8 mL (1.47–34.9). Postoperative status showed normal facial nerve function (House–Brackmann I) in all patients. In a subgroup of 28 patients, with serviceable hearing before surgery and in which cochlear nerve preservation was attempted at surgery, 26 (92.8%) retained serviceable hearing. Nineteen had good or excellent hearing (Gardner–Robertson class 1) before surgery, and 16 (84.2%) retained it after surgery. Mean duration between surgery and GKS was 6 months (median: 5, range: 3–13.9). Mean residual volume as compared with the preoperative one at GKS was 31%. Mean marginal dose was 12 Gy (11–12). Mean follow-up after GKS was 34.4 months (6–84). Conclusion Our data show excellent results in large VS management with a combined approach of microsurgical subtotal resection and GKS on the residual tumor, with regard to the functional outcome and tumor control. Longer term follow-up is necessary to fully evaluate this approach, especially regarding tumor control.

scholarly journals Volumetric changes and clinical outcome for petroclival meningiomas after primary treatment with Gamma Knife radiosurgery

2018 ◽  
Vol 129 (6) ◽  
pp. 1623-1629 ◽  
Author(s):  
Zjiwar H. A. Sadik ◽  
Suan Te Lie ◽  
Sieger Leenstra ◽  
Patrick E. J. Hanssens
Keyword(s):  
Gamma Knife ◽  
Clinical Symptoms ◽  
Cranial Nerves ◽  
Gamma Knife Radiosurgery ◽  
Local Tumor Control ◽  
Tumor Control ◽  
Local Tumor ◽  
Tumor Control Rate ◽  
Petroclival Meningiomas

OBJECTIVEPetroclival meningiomas (PCMs) can cause devastating clinical symptoms due to mass effect on cranial nerves (CNs); thus, patients harboring these tumors need treatment. Many neurosurgeons advocate for microsurgery because removal of the tumor can provide relief or result in symptom disappearance. Gamma Knife radiosurgery (GKRS) is often an alternative for surgery because it can cause tumor shrinkage with improvement of symptoms. This study evaluates qualitative volumetric changes of PCM after primary GKRS and its impact on clinical symptoms.METHODSThe authors performed a retrospective study of patients with PCM who underwent primary GKRS between 2003 and 2015 at the Gamma Knife Center of the Elisabeth-Tweesteden Hospital in Tilburg, the Netherlands. This study yields 53 patients. In this study the authors concentrate on qualitative volumetric tumor changes, local tumor control rate, and the effect of the treatment on trigeminal neuralgia (TN).RESULTSLocal tumor control was 98% at 5 years and 93% at 7 years (Kaplan-Meier estimates). More than 90% of the tumors showed regression in volume during the first 5 years. The mean volumetric tumor decrease was 21.2%, 27.1%, and 31% at 1, 3, and 6 years of follow-up, respectively. Improvement in TN was achieved in 61%, 67%, and 70% of the cases at 1, 2, and 3 years of follow-up, respectively. This was associated with a mean volumetric tumor decrease of 25% at the 1-year follow-up to 32% at the 3-year follow-up.CONCLUSIONSGKRS for PCMs yields a high tumor control rate with a low incidence of neurological deficits. Many patients with TN due to PCM experienced improvement in TN after radiosurgery. GKRS achieves significant volumetric tumor decrease in the first years of follow-up and thereafter.

Stereotactic radiosurgery for cerebellopontine angle meningiomas

2014 ◽  
Vol 120 (3) ◽  
pp. 708-715 ◽  
Author(s):  
Seong-Hyun Park ◽  
Hideyuki Kano ◽  
Ajay Niranjan ◽  
John C. Flickinger ◽  
L. Dade Lunsford
Keyword(s):  
Stereotactic Radiosurgery ◽  
Cerebellopontine Angle ◽  
Tumor Volume ◽  
Progression Free Survival ◽  
Tumor Control ◽  
Improvement Rate ◽  
Free Survival ◽  
Neurological Improvement ◽  
Presenting Symptoms

Object To assess the long-term outcomes of stereotactic radiosurgery (SRS) for cerebellopontine angle (CPA) meningiomas, the authors retrospectively reviewed data from a 20-year experience. They evaluated progression-free survival as well as improvement, stabilization, or deterioration in clinical symptoms. Methods Seventy-four patients with CPA meningiomas underwent SRS involving various Gamma Knife technologies between 1990 and 2010. The most common presenting symptoms were dizziness or disequilibrium, hearing loss, facial sensory dysfunction, and headache. The median tumor volume was 3.0 cm3 (range 0.3–17.1 cm3), and the median radiation dose to the tumor margin was 13 Gy (range 11–16 Gy). The median follow-up period was 40 months (range 4–147 months). Results At last imaging follow-up, the tumor volume had decreased in 46 patients (62%), remained stable in 26 patients (35%), and increased in 2 patients (3%). The progression-free survival after SRS was 98% at 1 year, 98% at 3 years, and 95% at 5 years. At the last clinical follow-up, 23 patients (31%) showed neurological improvement, 43 patients (58%) showed no change in symptoms or signs, and 8 patients (11%) had worsening symptoms or signs. The neurological improvement rate after SRS was 16% at 1 year, 31% at 3 years, and 40% at 5 years. The post-SRS deterioration rate was 5% at 1 year, 10% at 3 years, and 16% at 5 years. A multivariate analysis demonstrated that trigeminal neuralgia was the symptom most likely to worsen after SRS (HR 0.08, 95% CI 0.02–0.31; p = 0.001). Asymptomatic peritumoral edema occurred in 4 patients (5%) after SRS, and symptomatic adverse radiation effects developed in 7 patients (9%). Conclusions Stereotactic radiosurgery for CPA meningiomas provided a high tumor control rate and relatively low risk of ARE. Tumor compression of the trigeminal nerve by a CPA meningioma resulted in an increased rate of facial pain worsening in this patient experience.

scholarly journals Same-day stereotactic aspiration and Gamma Knife surgery for cystic intracranial tumors

2012 ◽  
Vol 117 (Special_Suppl) ◽  
pp. 45-48 ◽  
Author(s):  
Xiaomin Liu ◽  
Qi Yu ◽  
Zhiyuan Zhang ◽  
Yipei Zhang ◽  
Yanhe Li ◽  
...  
Keyword(s):  
Gamma Knife ◽  
Tumor Volume ◽  
Symptom Relief ◽  
Gamma Knife Surgery ◽  
Tumor Control ◽  
Intracranial Tumors ◽  
Glial Tumor ◽  
Stereotactic Aspiration ◽  
Cyst Aspiration

Object The goal of this study was to evaluate the efficacy and safety of same-day stereotactic aspiration and Gamma knife surgery (GKS) for cystic intracranial tumors. Methods Between 1996 and 2007, 77 patients harboring cystic intracranial tumors underwent a same-day procedure of MRI-guided cyst aspiration followed by GKS. The diagnoses were metastatic tumor in 43 patients, glial tumor in 12 patients, vestibular schwannoma in 10 patients, craniopharyngioma in 9 patients, and hemangioblastoma in 3 patients. Results An improvement in symptoms was achieved in 68 patients (88.3%) immediately after cyst aspiration. The mean tumor volume in this group of patients was 25.1 cm3 before aspiration and 11.1 cm3 afterward. Hemorrhage during the course of aspiration was encountered in 1 patient. Transient nausea after cyst aspiration developed in 3 patients. There was no treatment-related hematoma, seizure, neurological deficit, or infection. The median follow-up period was 16 months (range 6–108 months). Tumor control was achieved in 50 (80.6%) of 62 patients who participated in follow-up for at least 6 months. Conclusions The same-day stereotactic aspiration and GKS procedure was safe in patients with cystic brain tumors. Prompt symptom relief was obtained after cyst aspiration. The decrease in tumor volume following aspiration made GKS more effective because a higher prescription dose could be administered with a lower possibility of radiation-induced side effects.

Simultaneous Septal Perforation Repair with Septorhinoplasty: A 31-Year Experience

2018 ◽  
Vol 34 (03) ◽  
pp. 298-311 ◽  
Author(s):  
Sean Delaney ◽  
Russell Kridel
Keyword(s):  
Symptom Relief ◽  
Advancement Flap ◽  
Open Approach ◽  
Septal Perforation ◽  
Cosmetic Results ◽  
Presenting Symptoms ◽  
The Mean ◽  
Four Quadrant ◽  
Anterior Posterior

AbstractSeptal perforation repair and septorhinoplasty (SRP) each present unique surgical challenges. However, in many instances, these procedures may be performed together successfully. In this study, the authors aim to determine the safety and effectiveness of combining primary or revision SRP and septal perforation repair via an open approach. A retrospective review was carried out of all consecutive patients who had SRP and septal perforation repair via an open approach between 1986 and 2017 in the senior author's practice. Perforation closure in surgery and at the patient's last follow-up, resolution of presenting symptoms, cosmetic results, and complications were analyzed. Records for 141 patients who had simultaneous septal perforation repair and SRP via an open approach, with a mean follow-up of 3.24 years, were reviewed. The mean anterior–posterior perforation dimension was 1.41 ± 0.89 cm, and the mean vertical perforation dimension was 1.16 ± 0.59 cm. The most common etiologies for septal perforation were previous SRP (35.4%) and septoplasty (24.1%). An overall 93.6%, perforation closure, 91.1% symptom relief, and 91.2% patient satisfaction with cosmetic results were achieved. Septal perforations under 1.5 cm in height were closed in 96.7% of patients as opposed to 71.4% of patients with perforations 1.5 cm or taller. Minor revision rhinoplasties were performed in 7.0% of patients. Postoperative infections were rare and noted in only two (1.4%) patients. In the largest study of its kind to date, the authors have shown that in experienced hands septal perforation repair may be performed simultaneously with primary or revision SRP via an open approach without compromising the perforation repair outcome. The vertical dimension of a septal perforation and presence of mucosa above and below a perforation are important considerations for the difficulty of a perforation closure, as septal mucosa is recruited from these locations in our technique of four-quadrant intranasal bipedicled mucosal advancement flap closure.

scholarly journals Clinical course and surgical outcomes in middle-age adults with anomalous aortic origin of a coronary artery

2021 ◽  
Vol 22 (Supplement_3) ◽  
Author(s):  
A Javaid ◽  
S Sehgal ◽  
BK Khetarpal ◽  
A Singh ◽  
J Diep ◽  
...  
Keyword(s):  
Young Adults ◽  
Coronary Artery ◽  
Stress Testing ◽  
Clinical Course ◽  
Symptom Relief ◽  
Middle Aged ◽  
Coronary Anatomy ◽  
Presenting Symptoms ◽  
Anomalous Artery

Abstract Funding Acknowledgements Type of funding sources: None. Background Patients with anomalous aortic origin of a coronary artery (AAOCA) present with a wide range of clinical manifestations, including ischemic symptoms (chest pain or dyspnea) and sudden cardiac death (SCD). Studies have identified coronary anatomic characteristics associated with a higher risk of SCD. However, most of the published literature consists of studies in adolescents and young adults. There is a paucity of data regarding outcomes in middle-aged patients. Current guidelines reveal gaps in evidence for identification of adults are at risk for SCD, and for whom surgery is beneficial. Purpose To study the clinical course and rate of major adverse cardiac events (MACE) in middle-aged adults with AAOCA based on presenting symptoms, coronary anatomy on coronary computed tomography angiography (CCTA), stress test results, and surgical management. Methods We included all patients from January 2013 to December 2019 age > 18 at our institution who were found to have AAOCA.  Patients with the following were excluded to minimize confounding factors which could cause MACE: coronary artery disease (CAD) with >50% stenosis in any coronary vessel, CAD requiring revascularization, heart failure with ejection fraction <40%, history of heart transplant, and non-AAOCA congenital heart disease. All patient charts were reviewed for demographics, coronary anatomy on CCTA, presenting symptoms, rationale for pursuing stress testing and CCTA, nature of surgical interventions, post-surgical course, and MACE (cardiovascular death, myocardial infarction, and need for coronary revascularization). All patients underwent PET as well as treadmill stress testing. Results Of 19,367 patients who underwent CCTA, 47 met inclusion criteria, with median age at diagnosis of 54 and median follow-up of 48 months. No patients suffered MACE. Twenty-five patients had AAORCA and 22 had AAOLCA (Table 1). Ten patients with AAORCA and 8 patients with AAOLCA presented with ischemic symptoms and had coronary anatomy characteristics associated with higher risk of SCD, as well as ischemia corresponding to the anomalous artery on stress testing and did not undergo surgery due to personal preference. Five symptomatic patients with stress-induced ischemia corresponding to the anomalous artery underwent surgery and all achieved symptom relief over a median follow up of 5 years. Conclusion As AAOCA is a significant cause of SCD in young adults, it is compelling to observe this adult cohort in which no patients experienced MACE, including 18 symptomatic patients with high-risk anatomy and stress-induced ischemia, as well as a Class I recommendations for surgery.  The results suggest that although surgery may be beneficial for symptom relief, it does not necessarily improve mortality over an intermediate follow-up period.  Future studies should examine surgical outcomes in middle-aged cohorts with larger sample sizes.

The safety and efficacy of using large woven stents to treat vertebrobasilar dolichoectasia

2019 ◽  
Vol 11 (11) ◽  
pp. 1162-1166 ◽  
Author(s):  
Xuying He ◽  
Chuanzhi Duan ◽  
Jianbo Zhang ◽  
Xifeng Li ◽  
Xin Zhang ◽  
...  
Keyword(s):  
Digital Subtraction Angiography ◽  
Clinical Symptoms ◽  
Symptom Relief ◽  
Symptomatic Improvement ◽  
Digital Subtraction ◽  
Safety And Efficacy ◽  
Intracranial Stent ◽  
Partial Reconstruction ◽  
Vertebrobasilar Dolichoectasia

ObjectivesTo characterize the safety and efficacy of large woven stents in the treatment of vertebrobasilar dolichoectasia (VBD).MethodsWe retrospectively reviewed 19 consecutive patients with VBD treated with large woven intracranial stent (Leo stents) between January 2016 and December 2018. The clinical symptoms and angiograms of all the patients were recorded.ResultsThe patients were treated with 1–3 large Leo stents (5.5 mm x 75 mm, 5.5 mm x 50 mm, or 4.5 mm x 40 mm), with or without coiling. They had follow-up angiography and MRI between 3 months and 1 year. Digital subtraction angiography showed 16 patients with complete reconstruction of the target vessels, one patient with almost complete reconstruction, and two patients with partial reconstruction. All patients had symptomatic improvement shortly after treatment, but two patients developed recurrent dysphagia at 8 and 18 months, respectively.ConclusionsDeployment of woven stents with or without supportive coiling may offer symptom relief and reconstruction in patients with VBD.

Outcomes in pediatric patients with Chiari malformation Type I followed up without surgery

2011 ◽  
Vol 7 (4) ◽  
pp. 375-379 ◽  
Author(s):  
David Benglis ◽  
Derek Covington ◽  
Ritwik Bhatia ◽  
Sanjiv Bhatia ◽  
Mohamed Samy Elhammady ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Pediatric Patients ◽  
Neurological Deficits ◽  
Type I ◽  
Imaging Studies ◽  
Chiari Malformation Type I ◽  
Presenting Symptoms ◽  
Asymptomatic Patients ◽  
Number Of Patients

Object The natural history of untreated Chiari malformation Type I (CM-I) is poorly defined. The object of this study was to investigate outcomes in pediatric patients with CM-I who were followed up without surgical intervention. Methods The authors retrospectively reviewed 124 cases involving patients with CM-I who presented between July 1999 and July 2008 and were followed up without surgery. The patients ranged in age from 0.9 to 19.8 years (mean 7 years). The duration of follow-up ranged from 1.0 to 8.6 years (mean 2.83 years). Imaging findings, symptoms, and findings on neurological examinations were noted at presentation and for the duration of follow-up. Results The mean extent of tonsillar herniation at presentation was 8.35 mm (range 5–22 mm). Seven patients had a syrinx at presentation. The syrinx size did not change in these patients on follow-up imaging studies. No new syrinxes developed in the remaining patients who underwent subsequent imaging. The total number of patients with presenting symptoms was 81. Of those 81 patients, 67 demonstrated symptoms that were not typical of CM-I. Of the 14 patients with symptoms attributed to CM-I, 9 had symptoms that were not severe or frequent enough to warrant surgery, and surgery was recommended in the remaining 5 patients. Chiari malformation Type I was also diagnosed in 43 asymptomatic patients who had imaging studies performed for various reasons. No new neurological deficits were noted in any patient for the duration of follow-up. Conclusions The majority of patients with CM-I who are followed up without surgery do not progress clinically or radiologically. Longer follow-up of this cohort will be required to determine if symptoms or new neurological findings develop over the course of many years.

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