scholarly journals Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Nasir Ud Din ◽  
Shabina Rahim ◽  
Tamana Asghari ◽  
Jamshid Abdul-Ghafar ◽  
Zubair Ahmad

Abstract Background Hepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor which is often multifocal and, in many cases, discovered incidentally. Here, we describe the clinicopathological features of hepatic epithelioid hemangioendothelioma cases seen in our practice and present a detailed review of the published literature. Methods All cases of hepatic epithelioid hemangioendothelioma diagnosed in Department of Pathology and Laboratory Medicine, Aga Khan University Hospital between January 1, 2006 and December 31, 2019 were included in the study. Slides were reviewed and follow up was obtained. Results Seven cases were reported during the study period. There were 4 females and 3 males. Age range was 20 to 77 years, mean age was 45 years. Three patients presented with right upper abdominal pain; 1 patient presented with jaundice while 3 patients were asymptomatic. In all 7 cases, lesions were identified on imaging studies. In 5 cases, liver lesions were multifocal. Clinical differential diagnosis in all cases was metastatic carcinoma and multifocal hepatocellular carcinoma. Liver function tests were normal in 5 cases. In 1 patient, tumor had already metastasized to the right lung. On histological examination of liver core biopsies performed in all 7 cases, classic histological features of epithelioid hemangioendothelioma were seen. Tumor cells expressed positivity for vascular markers (CD 34, CD31 and ERG) and were negative for cytokeratins, Hep par 1 and Glypican 3. Surgical resection was not performed in any of the 7 cases and all patients were treated by chemotherapy. Follow up was available in 5 cases. Of these, 3 patients died of disease and another patient was alive with metastases in both lungs, omentum and colon. Conclusion Clinicopathological features of the 7 cases in our series and detailed review of published literature is presented. Prognosis was bad in our cases most likely due to fact that surgical resection could not be performed in any of the cases owing to lack of surgical expertise for liver tumor surgery in most parts of the country.

Biology ◽  
2021 ◽  
Vol 10 (11) ◽  
pp. 1160
Author(s):  
Kasey J. McCollum ◽  
Rami N. Al-Rohil

Neoplasms of uncertain biological behavior present physicians with a genuine conundrum in practice. Cutaneous vascular neoplasms within this category are exceedingly rare, possessing significant gaps and uncertainty in many facets of clinical practice. Firstly, lesions were selected for review based on their categorization as indeterminate behavior, indicating the potential for local recurrence and rarely metastasize. After identification of the target lesions, a comprehensive review of the literature using national databases produced several landmark studies and case series regarding these neoplasms. Limiting the review to only cutaneous limited tumors narrowed the pool of studies; however, quite a large sum of papers remained. Examination of each paper yielded beneficial results on diagnosing, effective treatments, follow-up findings, and prognosis for each indeterminate lesion discussed. Overall, the literature search combined the molecular, histologic, immunohistochemical, surgical strategies to develop an up-to-date and comprehensive framework to guide physicians when encountering such lesions. The tumors reviewed include: kaposiform hemangioendothelioma, endovascular papillary angioendothelioma, pseudomyogenic hemangioendothelioma, retiform hemangioendothelioma, epithelioid hemangioendothelioma, and composite hemangioendothelioma.


2021 ◽  
Vol 15 (10) ◽  
pp. 3479-3481
Author(s):  
Anila Mujadid Qureshi ◽  
Azra Parveen Rajpar ◽  
Ishrat Saba Mari ◽  
Khalida Avesi ◽  
Kousar Fatima ◽  
...  

Introduction: Vesicovaginal fistulae is abnormal communication between bladder and vagina that cause continous dribling of urine. It is physically, mentally and socially distressing condition. There are various approaches for surgeries of these urogenital fistulae with different success-rate that depend upon the experience of surgeon and surgical procedures. This study can help us to estimate the success rate of layered repair with graft in vaginal route to make stragedy to adopted in severe patient. Objective: To determine frequency of success in surgery on vesic-ovaginal fistulae by layered closured with graft repair procedures among patients admitted in Isra University. Setting: Obstetrics & Gynecology department in Isra university hospital Duration: 6 months from 10.2.2014 to 10.8.2014 Study Design: Case series Subject and methods: A total of 100 patients after having surgery for vesicovaginal fistula by layered closure with graft repair was included in this study. History and examination of all subjects were taken. The follow up visit was planned after 3 weeks of surgery. All women was questioned for recurrence of continuous urinary leakage and that without such symptoms proved by absence of leakage on methylene blue dye test was labeled as ‘success’. Results: - Frequency of success in surgery on vesic-ovaginal fistulae by layered closured with graft repair procedures was observed in 88% cases. Conclusion: The success rate of VVF repair by layered closured with graft repair procedures is high. It is concluded that obstetric urogenital fistula is a preventable condition. Keywords: Vesicovaginal fistulae, Layered closured, Graft repair, urogenital fistula


2018 ◽  
Vol 06 (03) ◽  
pp. E335-E339 ◽  
Author(s):  
Naoki Asayama ◽  
Shinji Nagata ◽  
Kenjiro Shigita ◽  
Taiki Aoyama ◽  
Akira Fukumoto ◽  
...  

AbstractBenign colonic anastomotic stenosis sometimes occurs after surgical resection and usually requires surgical or endoscopic dilation. Limited data are available on the effectiveness and safety of the endoscopic radial incision and cutting (RIC) method at sites other than the esophagus. The aim of this retrospective study was to investigate the effectiveness and safety of RIC dilation for severe benign anastomotic colonic stenosis. Subjects were 3 men (median age 72 years, range 65 – 76 years) who developed severe benign anastomotic stenosis after surgical resection for colorectal carcinoma and were subsequently treated by RIC dilation at Hiroshima City Asa Citizens Hospital between May 2014 and December 2016. Severe anastomotic stenosis was defined as a narrowed anastomosis through which a standard colonoscope could not be passed. The median interval from surgery to RIC was 21 months (range 9 – 29 months). RIC was successful in all 3 patients and reduced the severity of dyschezia postoperatively; 2 patients experienced improvement after a single RIC session and the other after 6 RIC sessions. No treatment-related adverse events or re-stenosis requiring repeat dilation was noted during a median follow-up of 27 months (range 8 – 37 months). Our findings indicate that the RIC technique can be applied safely and effectively to various sites in the colon, avoiding the need for reoperation.


2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Fulvia Zappulo ◽  
Gabriele Donati ◽  
Giorgia Comai ◽  
Claudia Bini ◽  
Andrea Angeletti ◽  
...  

Abstract Background and Aims Survival of patients with Multiple Myeloma (MM), Light Chain Amyloidosis (LCA) and Monoclonal Gammopathies of renal significance (MGRS) on chronic renal replacement therapy (RRT) is poor. The gold standand treatment of plasma cell dyscrasias (PCD) is high-dose chemotherapy followed by Autologous Stem Cell Transplantation (ASCT) which can induce complete remission and longer survival than chemotherapy alone. Kidney transplantation (KT) after ASCT could represent an option for patients with PCD and End Stage Renal Disease (ESRD). There is no evidence about the time of follow up required from MM remission and KT. Method We present a case series of 5 patients who underwent KT after ASCT and remission of MM among 2,500 transplant recipients followed at the Nephrology Dialysis and Renal Transplantation Unit of S.Orsola University Hospital from 1967 untill now. As in case of recovery from solid cancers, the feasibility of KT after MM was considered when no signs of relapse were assessed. In our cohort 3 patients were affected by Light Chain Deposition Disease (LCDD), 1 patient presented Myeloma Cast Nephropathy (MCN) and one patient Light Chain Amyloidosis (LCA). They all required RRT and underwent KT after ASCT. Results Time between ASCT and KT ranged from 3 and 11 years and clinical outcome was very different. The mean follow up period ranged from 2 to 4 years. In the first case (LCDD) KT was performed 11 years after ASCT, the graft failure occurred 6 years later because of chronic allograft nephropathy requiring RRT. In the second case (LCDD) patient received KT 3 years after ASCT. He developed Bence-Jones proteinuria requiring specific therapy with Dexametasone and Bortezomib determining progressive graft failure. In the third case (LCDD) KT was performed 4 years after ASCT and the 4 year follow up is negative for relapse of MM or ESRD. The fourth patient presented MCN and received KT 8 years after ASCT. MCN relapsed 6 years later; it caused ESRD requiring RRT. In the last patient (LCA) KT was performed 4 years after ASCT. No recurrence occurred in a 2-year follow up. Conclusion MM is the most frequent malignancy in dialytic population; the need for KT in MM remains high. ASCT improves the quality of life and offers higher survival in patients with myeloma/MGRS/amyloidosis-related ESRD. Therefore the combination of chemotherapy/ASCT and KT is pivotal to pursue renal restoring. Since high risk of recurrence larger study are required to clarify the better follow up period after MM remission and KT.


2021 ◽  
Vol 12 ◽  
Author(s):  
Vladimir Gavrilovic ◽  
Annarita Dapoto ◽  
Nicola Marotti ◽  
Andrea Pellegrin ◽  
Alessandro Pauro ◽  
...  

Background and Purpose: Flow diverting stents are designed to divert blood flow from the aneurysm sac, allowing for eventual occlusion following endovascular therapy. This case series reports clinical experience using the Silk Vista Baby (SVB, Balt Extrusion, Montmorency, France), a flow diverter (FD) designed to treat intracranial aneurysms in small, distal vessels.Methods: All patients who underwent treatment with SVB at the University Hospital “Santa Maria della Misericordia” of Udine between July 2018 and September 2020 were retrospectively identified. Baseline patient and aneurysm characteristics, intraprocedural technical outcomes, periprocedural complications, modified Rankin Scale (mRS) at discharge, magnetic resonance imaging (MRI) results at 3-month follow-up, and angiographic results at 6-month follow-up were collected.Results: A total of 18 patients (55.6% [10/18] male; mean age 62.6 years, range: 42–77 years) were retrospectively identified, receiving treatment for 22 aneurysms. Most patients were symptomatic (14/18, 77.8%) and approximately half had subarachnoid hemorrhage (10/18, 55.6%). Sufficient aneurysm coverage was achieved in 88.9% (16/18) of patients with a single device. Mortality did not occur (0/18, 0%); adverse device-related events included side branch occlusion (1/18, 5.6%) and in-stent thrombosis (1/18, 5.6%). At discharge, 77.8% (14/18) had an mRS of 0. In most cases, patients showed complete occlusion (10/15, 66.7%) or a small aneurysmal remnant (3/15, 20.0%) upon MRI; upon angiography, most showed complete occlusion (10/13, 76.9%) or only a small aneurysmal remnant (2/13, 15.4%).Conclusion: This case series showed that the SVB FD is safe and feasible to use in patients with aneurysms in small, distal vessels. Additional randomized, prospective studies with larger cohorts are needed for the SVB.


2019 ◽  
Vol 90 (6) ◽  
pp. 695-703 ◽  
Author(s):  
Anshit Goyal ◽  
Lorenzo Rinaldo ◽  
Redab Alkhataybeh ◽  
Panagiotis Kerezoudis ◽  
Mohammed Ali Alvi ◽  
...  

ObjectiveThere is a paucity of literature investigating the clinical course of patients with spinal intramedullary cavernous malformations (ISCMs). We present a large case series of ISCMs to describe clinical presentation, natural history and outcomes of both surgical and conservative management.MethodsWe retrospectively reviewed the clinical course of patients diagnosed with ISCMs at our institution between 1995 and 2016. Haemorrhage was defined as clinical worsening in tandem with imaging changes visualised on follow-up MRI. Outcomes assessed included neurological status and annual haemorrhage rates.ResultsA total of 107 patients met inclusion criteria. Follow-up data were available for 85 patients. While 21 (24.7%) patients underwent immediate surgical resection, 64 (75.3%) were initially managed conservatively. Among this latter group, 16 (25.0%) suffered a haemorrhage during follow-up and 11 (17.2%) required surgical resection due to interval bleeding or neurological worsening. The overall annual risk of haemorrhage was 5.5% per person year. The rate among patients who were symptomatic and asymptomatic on presentation was 9.5% and 0.8%, respectively. Median time to haemorrhage was 2.3 years (0.1–12.3). Univariate analysis identified higher ISCM size (p=0.024), history of prior haemorrhage (p=0.013) and presence of symptoms (p=0.003) as risk factors for subsequent haemorrhage. Multivariable proportional hazards analysis revealed presence of symptoms to be independently associated with haemorrhage during follow-up (HR 9.39, CI 1.86 to 170.8, p=0.013).ConclusionLarge, symptomatic ISCMs appear to be at increased risk for subsequent haemorrhage. Surgery may be considered in such lesions to prevent rebleeding and subsequent neurological worsening.


2020 ◽  
Vol 3 (Supplement_1) ◽  
pp. 155-156
Author(s):  
S Li ◽  
M Monachese ◽  
P James

Abstract Background Endoscopic ultrasound guided fine needle aspiration (EUS-FNA) is the primary method of sampling pancreatic cystic lesions with reported specificity near 100% for diagnosing malignancy. Discrepant positive malignant cytopathology with final surgical pathology of pancreatic cystic lesions has not previously been described. Aims To present a case series and review the literature regarding the implications of positive malignant cytology with discrepant surgical pathology for high risk pancreatic mucinous cystic lesions. Methods Patient demographics, clinical history, procedure details, pathology evaluations and follow-up were collected. A thorough literature review was performed. Results Three patients with high-risk pancreatic cystic lesions on cross-sectional imaging underwent EUS-FNA evaluation. None of these patients had a history of pancreatitis. Cytology was reported as positive for adenocarcinoma in all patients by separate gastrointestinal cytopathologists. All patients underwent surgical resection. The pathology for all resected specimens were reported as intraductal papillary mucinous neoplasm. The resected cysts for two patients demonstrated foci of high-grade dysplasia and the third noted low grade dysplasia. All surgical pathology underwent consensus review by three separate gastrointestinal pathologists. None of the patients were treated with adjuvant chemotherapy. All patients have been followed post-operatively with surveillance magnetic resonance imaging with no evidence of recurrence to date (median follow-up time 239 days, range 133 – 447 days). Conclusions This phenomenon sheds light on the potential for variable interpretations of EUS-FNA cytopathology and surgical resection pathology for high risk pancreatic cystic neoplasms. EUS-FNA may identify foci of adenocarcinoma that is not seen on surgical pathology specimens. Further research is required to examine the long-term outcomes of these patients. Funding Agencies None


2009 ◽  
Vol 46 (3) ◽  
pp. 262-267 ◽  
Author(s):  
Marion Devambez ◽  
Alexis Delattre ◽  
Pierre Fayoux

Objectives: To review diagnosis and treatment modalities in congenital nasal pyriform aperture stenosis in a newborn population. Study Design: A 6-year retrospective case series review. Mean follow-up of 21.8 months. Setting: University hospital, tertiary care center. Patients: Twenty-one consecutive patients treated for congenital nasal pyriform aperture stenosis. Diagnosis was suspected on physical examination and confirmed by computed tomography scan. Associated abnormalities were present in 15 patients. Surgical Treatment: Nineteen patients underwent surgical treatment focused on external bony margins of pyriform aperture and the osseous anterior edge of the inferior turbinate, drilling by a sublabial approach and associated with a partial turbinectomy in seven cases. A stent was placed for 7 to 10 days. A conservative treatment based on topical nasal decongestants and gastroesophageal reflux treatment was proposed for two patients. Results: Postoperative follow-up revealed septal ulceration in five cases with septal perforation in one case and development of synechiae in two cases. Follow-up revealed normal nasal breathing and pyriform aperture growth after surgery. Conclusion: Our experience confirmed the link described between congenital nasal pyriform aperture stenosis and holoprosencephaly, but associated extracraniofacial malformations suggest that congenital nasal pyriform aperture stenosis should be integrated in a systemic malformative syndrome or genetic disorder and lead us to propose an exhaustive dysmorphology assessment. Some infants may be treated by conservative management, and severely affected patients may undergo surgery by a sublabial approach.


2020 ◽  
Vol 163 (6) ◽  
pp. 1150-1152 ◽  
Author(s):  
Elizabeth Floyd ◽  
Scott S. Harris ◽  
Jessica W. Lim ◽  
David R. Edelstein ◽  
Briana Filangeri ◽  
...  

Thirty-eight tracheostomies were performed on patients with respiratory failure secondary to SARS-CoV-2 infection over the month of April at North Shore University Hospital and Lenox Hill Hospital (members of Northwell Health System in Long Island and New York City). Follow-up by May 14 revealed that 21 (55.2%) had been weaned from ventilators and 7 (18.4%) underwent decannulation. Two patients (5.3%) expired in the weeks following tracheostomy. Between the 2 institutions, 10 attending surgeons performed all of the tracheostomies using appropriate personal protective equipment, and none demonstrated seroconversion within 1 to 2 weeks of this article.


2012 ◽  
Vol 126 (9) ◽  
pp. 913-917 ◽  
Author(s):  
J M Bernstein ◽  
S M Jones ◽  
P H Jones

AbstractObjective:We present a case series with airway compromise due to bilateral abductor vocal fold paralysis or fixation, treated with unilateral transverse cordotomy.Methods:Of eight consecutive patients with dyspnoea due to bilateral paramedian vocal fold immobility, seven underwent unilateral transverse cordotomy between August 2006 and April 2010 at University Hospital of South Manchester, UK. Airway and voice outcomes were compared before and after surgery.Results:All seven treated cases derived subjective airway function improvement; there was no aspiration. The eighth case had inadequate access. None of the seven treated patients required contralateral cordotomy or permanent tracheostomy. One treated case required a temporary tracheostomy; unilateral transverse cordotomy facilitated eventual decannulation. Two patients died of cancer at five and six weeks, variously. At a mean follow up of 22 months, four cases showed unchanged or slightly worse Voice Symptom Scale and Grade-Roughness-Breathiness-Asthenia-Strain scale scores.Conclusion:In patients with bilateral abductor vocal fold immobility, unilateral transverse cordotomy results in improved dyspnoea with either no voice change or only slight worsening. This is a more conservative procedure than bilateral transverse cordotomy, with the potential for better preservation of voice and breath support.


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