A rare giant cardiac lymphangioma in adults.
Ontology highlight
ABSTRACT:
SUBMITTER: Zhao W
PROVIDER: S-EPMC10006123 | biostudies-literature | 2023 Mar
REPOSITORIES: biostudies-literature
Publications
A rare giant cardiac lymphangioma in adults.
Zhao Wenjing W Ma Peiqing P Zhang Nan N Liu Jiayi J Liu Dongting D Sun Zhonghua Z Xu Lei L Wen Zhaoying Z Li Yu Y
Quantitative imaging in medicine and surgery 20230102 3
Similar Datasets
Project description:IntroductionCystic lymphangioma is a benign tumor originating from the lymph vessels. It commonly occurs in childhood, in the head or neck regions. However, abdominal Cystic lymphangioma is extremely rare in adult patients and often asymptomatic. Considering abdominal space, it may attain huge sizes whilst causing minimal symptoms. Due to this insidious presentation, these tumors become massive and can be diagnosed late at the complication stage.Presentation of caseThis case report describes a rare and exceptional case of giant cystic lymphangioma of the stomach presented with a perforation in the abdominal cavity. The diagnosis was suspected following an abdominal CT scan, but could not confirm that the lesion was derived from the stomach. Therefore, an exploratory laparotomy found a multi-cystic mass occupying most of the abdominal space, adherent to the small gastric curvature and without delimitation line. This mass presents a small perforation responsible for an ascites of medium abundance. Then, the patient underwent a subtotal gastrectomy removing the entire cystic mass. Pathological analysis of the surgical specimen confirmed the diagnosis of cystic lymphangioma of the stomach.The postoperative recovery was uneventful, and the patient was discharged after 6 days. At the 3-month follow-up, the patient was in good health.DiscussionThe cystic lymphangioma of the stomach is rare and exceptionally described in the literature. However, if this tumor is benign, it has the potential to grow, invade vital structures, and develop life-threatening complications.ConclusionWe stress the importance of complete surgical excision to prevent cyst complications and to reduce the recurrence risk.
| S-EPMC7750175 | biostudies-literature
Project description:Mycosis fungoides is the most common cutaneous T-cell lymphoma. Although myocardial infiltration is frequent in advanced stages, symptomatic cardiac involvement is rare. We report an unusual case of rapidly progressing acute heart failure due to cardiac affection by mycosis fungoides manifested as an intracavitary mass in the right atrium. The patient received cytoreductive surgery and adjuvant radiotherapy, presenting an excellent cardiovascular outcome during follow-up.Learning objectives•Recognize mycosis fungoides as a potential cause of cardiac mass due to its visceral progression.•Note the role of cytoreductive surgery with adjuvant radiotherapy in order to relieve heart failure symptoms and improve the prognosis of the disease in the exceptional case of mycosis fungoides presenting as an isolated cardiac mass.
| S-EPMC9449774 | biostudies-literature
Project description:Introduction: Lymphangioma is a sporadic benign tumor of the bladder. It is a congenital disorder and based on the size of lymphatic spaces, it is divided into 3 types of capillary, cavernous, and cystic. Case Report: In this paper, we presented a 40-year-old woman with microscopic hematuria and a normal urinary ultrasound. Urethrocystoscopy showed a flat 4 mm highlighted strawberry-like lesion on the right lateral wall of the bladder. After a cold cup biopsy, the lesion was coagulated by the holmium: YAG (Ho: YAG) laser. Conclusion: In Bladder Lymphangioma Based on the size of the lesion, partial cystectomy or minimally invasive surgeries such as laser modality would be the principal treatment.
| S-EPMC7118501 | biostudies-literature
Project description:BackgroundCardiac blood cysts are exceedingly rare cardiac tumours usually found on cardiac valves in infants. We report and discuss a rare unique case wherein a giant atrial septal cardiac blood cyst was found in an adult.Case summaryA 59-year-old Chinese lady with history of hypertension, hyperlipidemia and transient ischaemic attack presented with atypical chest pain. Her ECG suggested right ventricular strain. Echocardiography revealed a large right atrial intracardiac mass attached to the interatrial septum which prolapsed into the right ventricle during each cardiac cycle with associated dilatation of the right atrium and right ventricle. Computed tomography scans showed no evidence of malignancy or metastasis. Considering our patient's profile and the characteristics of the mass, it was opined to likely be a cardiac myxoma. After consultation with our cardiothoracic surgeons, she underwent surgical resection of the intracardiac mass. Histology revealed that the intracardiac mass was a cardiac blood cyst.DiscussionFirst described by Elsasser in 1844, cardiac blood cysts are extremely rare benign cardiac tumours very rarely seen in adults. Though benign, they have been associated with outflow tract obstruction, valvular regurgitation and embolization. Symptomatic patients do well with surgical resection, while long-term outcomes in asymptomatic patients followed up with serial echocardiograms are mixed. Due to its rarity, existing literature is limited to case reports and small case series, with a lack of guidelines on management till recently. Our case report serves to highlight this unique presentation of an exceedingly rare intracardiac tumour.
| S-EPMC11384889 | biostudies-literature
Project description:BACKGROUND:Sudden cardiac death occurs in ∼220,000 U.S. adults annually, the majority of whom have no prior symptoms or cardiovascular diagnosis. Rare pathogenic DNA variants in any of 49 genes can pre-dispose to 4 important causes of sudden cardiac death: cardiomyopathy, coronary artery disease, inherited arrhythmia syndrome, and aortopathy or aortic dissection. OBJECTIVES:This study assessed the prevalence of rare pathogenic variants in sudden cardiac death cases versus controls, and the prevalence and clinical importance of such mutations in an asymptomatic adult population. METHODS:The authors performed whole-exome sequencing in a case-control cohort of 600 adult-onset sudden cardiac death cases and 600 matched controls from 106,098 participants of 6 prospective cohort studies. Observed DNA sequence variants in any of 49 genes with known association to cardiovascular disease were classified as pathogenic or likely pathogenic by a clinical laboratory geneticist blinded to case status. In an independent population of 4,525 asymptomatic adult participants of a prospective cohort study, the authors performed whole-genome sequencing and determined the prevalence of pathogenic or likely pathogenic variants and prospective association with cardiovascular death. RESULTS:Among the 1,200 sudden cardiac death cases and controls, the authors identified 5,178 genetic variants and classified 14 as pathogenic or likely pathogenic. These 14 variants were present in 15 individuals, all of whom had experienced sudden cardiac death-corresponding to a pathogenic variant prevalence of 2.5% in cases and 0% in controls (p < 0.0001). Among the 4,525 participants of the prospective cohort study, 41 (0.9%) carried a pathogenic or likely pathogenic variant and these individuals had 3.24-fold higher risk of cardiovascular death over a median follow-up of 14.3 years (p = 0.02). CONCLUSIONS:Gene sequencing identifies a pathogenic or likely pathogenic variant in a small but potentially important subset of adults experiencing sudden cardiac death; these variants are present in ∼1% of asymptomatic adults.
| S-EPMC7067308 | biostudies-literature
Project description:IntroductionCystic lymphangioma (CL) is a benign tumor originating from the lymph vessels. Lymphangiomas in the abdominal cavity are extremely rare, particularly in adults.This article was designed to study the epidemiological, diagnostic difficulties, and therapeutic principles of intra-abdominal cystic lymphangioma (ACL) in adults.Material and methodsWe conducted a single-center, retrospective study of 32 adult patients with ACL admitted to surgical department "A" in "La Rabta Hospital" in Tunis, from January 1998 through December 2020. The demographic, clinical, biological, radiological characteristics, histopathologic, and therapeutic data were collected, as well as the surgical intervention used and the postoperative immediate and late complications.ResultsThirty-two adult patients with ACL were recruited, including 20 females and 12 males. The median age at treatment was 47 (range 14-80) years. The most prevalent sites were the retroperitoneum (25%), the mesentery (21.9%), and the paracolic gutters (n = 18. 7%). Twenty patients underwent open surgery (62.5%), whereas 12 cases (37.5%) had laparoscopic surgery. Twenty-eight patients received total cystectomy (87%). Three recurrences were observed during follow-up (9.4%).ConclusionThe clinical features of CL in adults remain unclear. The diagnosis is only confirmed by histopathological examination after complete surgical resection. The laparoscopic approach is considered safe and feasible.
| S-EPMC9486738 | biostudies-literature
Project description:IntroductionMucocele is a slow growing, benign but locally aggressive cystic structure lined by true epithelium. It often results due to obstructed sinus outflow or obstruction of gland-like mucous retention cyst. It can cause bony destruction and might result in orbital symptoms like diplopia, orbital displacement, visual disturbances. Other clinical features are facial numbness, dental problems, etc. Radiological evaluation is the preferred diagnostic modality. Surgical removal is the treatment of choice both endoscopic and open (could well luc) approach or combined approach are preferred. Here we report a very typical case of maxillary mucocele who presented with subtle symptoms of nasal obstruction. The study was done in compliance with SCARE guidelines.[1].Case presentationWe present a very unique case of 24 years man with complaints of nasal obstruction and swelling over the right cheek for 2 years. He had a history of facial trauma two years back. Diagnosis was made on the basis of radiological examination CT (Computed Tomography) scan. He underwent enucleation via Cold well Luc's approach with good postoperative results.ConclusionMaxillary mucoceles are slow growing benign lesions. However, they are locally aggressive and cause bony destruction resulting into orbital and dental symptoms. Thus early recognistion with regular folllowr up and planning for surgical intervention can help avoid complications.
| S-EPMC6556524 | biostudies-literature
Project description: Not available
| S-EPMC11874422 | biostudies-literature
Project description:Enchondromas, common benign intramedullary cartilaginous tumors, typically arise asymptomatically in metaphysis and diaphysis. We report a case diagnosed and treated for a giant sinonasal enchondroma, an extremely uncommon localization. Few cases have been reported in this location, and additional data is needed to draw conclusions regarding the potential for malignancy.Supplementary informationThe online version contains supplementary material available at 10.1007/s12070-024-04620-z.
| S-EPMC11306484 | biostudies-literature
Project description:Cardiovascular disease (CVD) remains the most common cause of adult morbidity and mortality in developed nations. As a result, predisposition for CVD is increasingly important to understand. Ankyrins are intracellular proteins required for the maintenance of membrane domains. Canonical ankyrin-G (AnkG) has been shown to be vital for normal cardiac function, specifically cardiac excitability, via targeting and regulation of the cardiac voltage-gated sodium channel. Noncanonical (giant) AnkG isoforms play a key role in neuronal membrane biogenesis and excitability, with evidence for human neurologic disease when aberrant. However, the role of giant AnkG in cardiovascular tissue has yet to be explored. Here, we identify giant AnkG in the myocardium and identify that it is enriched in 1-week-old mice. Using a new mouse model lacking giant AnkG expression in myocytes, we identify that young mice displayed a dilated cardiomyopathy phenotype with aberrant electrical conduction and enhanced arrhythmogenicity. Structural and electrical dysfunction occurred at 1 week of age, when giant AnkG was highly expressed and did not appreciably change in adulthood until advanced age. At a cellular level, loss of giant AnkG results in delayed and early afterdepolarizations. However, surprisingly, giant AnkG cKO myocytes display normal INa, but abnormal myocyte contractility, suggesting unique roles of the large isoform in the heart. Finally, transcript analysis provided evidence for unique pathways that may contribute to the structural and electrical findings shown in giant AnkG cKO animals. In summary, we identify a critical role for giant AnkG that adds to the diversity of ankyrin function in the heart.
| S-EPMC8040283 | biostudies-literature