Intravenous leiomyomatosis extending into right heart: A case report.
Ontology highlight
ABSTRACT:
SUBMITTER: Qiuying L
PROVIDER: S-EPMC7298371 | biostudies-literature | 2019 Jun
REPOSITORIES: biostudies-literature
Publications
Intravenous leiomyomatosis extending into right heart: A case report.
Qiuying Liao L Chengfeng Huang H Xiaoshen Zhang Z
Turk gogus kalp damar cerrahisi dergisi 20190601 2
Intravenous leiomyomatosis is a rare and special tumor which is exclusively seen in premenopausal women. Herein, we present a 46-year-old female case diagnosed with intravenous leiomyomatosis through an exploratory sternotomy and pathological examination. ...[more]
Similar Datasets
Project description:Intravenous leiomyomatosis (IVL) is a rare neoplasm characterized by histologically benign-looking smooth muscle cell tumor mass, which is growing within the intrauterine and extrauterine venous system. In this report we aimed to present an unusual case of IVL, which is originating from iliac vein and extended throughout to right cardiac chambers. A 49-year-old female patient, who was treated with warfarin sodium due to right iliac vein thrombosis, was admitted to our department with intermittent dyspnea, palpitation, and dizziness. Physical examination was almost normal except bilateral pretibial edema. On magnetic resonance venography, there was an intravenous mass, which is originated from right internal iliac vein and extended into the inferior vena cava. Transthoracic echocardiography and transesophageal echocardiography revealed a huge mass extending from the inferior vena cava through the right atrium, with obvious venous occlusion. Thoracic, abdominal, and pelvic MR showed an intravascular mass, which is concordant with leiomyomatosis. Surgery was performed through median sternotomy. A huge mass with 25-cm length and 186-gr weight was excised through right atrial oblique incision, on beating heart with cardiopulmonary bypass. Histopathologic assessment was compatible with IVL. Exact strategy for the surgical treatment of IVL is still controversial. We used one-stage approach, with complete resection of a huge IVL extending from right atrium to right iliac vein. In such cases, high recurrence rate is a significant problem; therefore it should be kept in mind.
| S-EPMC4465713 | biostudies-literature
Project description:BackgroundMalignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma mainly treated via surgical resection. Herein, we report a case of MPNST wherein a massive tumor thrombus extended to the major veins and heart.Case presentationA 39-year-old female with a history of neurofibromatosis type 1 developed MPNST from the right radial nerve. In addition to adjuvant chemotherapy, she underwent wide tumor resection and concomitant radial nerve resection, followed by postoperative radiotherapy. Histological evaluation revealed marked venous invasion. The 2-year follow-up CT revealed an asymptomatic recurrent tumor thrombus extending from the right subclavian vein to the heart. An urgent life-saving operation was performed to ligate the base of the right subclavian vein and remove the entire intravenous thrombus that extended to the right ventricle. The remaining tumor in the right subclavian vein increased in size 3 months after thrombectomy. After confirming the absence of any metastatic lesions, the patient underwent extended forequarter amputation to achieve surgical remission. One year later, a new metastasis to the right diaphragm was safely resected. The patient remains alive without any evidence of disease 2 years after the extended forequarter amputation.ConclusionsIn cases of a previous history of microscopic venous invasion, recurrence can occur as a massive tumor thrombus that extends to the great vessels.
| S-EPMC8742394 | biostudies-literature
Project description:Uterine leiomyoma (UL) is the most common benign smooth muscle tumour of the premenopausal women. Rarely it shows malignant behaviour by metastasizing through the pelvic veins into systemic veins, inferior vena cava (IVC), there it is termed as intravenous leiomyomatosis (IVL). IVL may restrict itself within the IVC or it may extend into right heart chambers reaching up to pulmonary arteries. Here we report a case of single staged excision of intracardiac(IC) extension of IVL of a 45 -year -old premenopausal women, who have undergone abdominal hysterectomy five years ago, with the complaints of shortness of breath aggravated on bending forward for the past two years.
| S-EPMC8617376 | biostudies-literature
Project description:Introduction:Myxomas are the most common type of cardiac benign tumors and most of them are located in the left atrium, followed by the right atrium. The majority of Myxomas is located in the left atrium and has a variable clinical presentation. Myxomas affect patients within a wide age range (15-80 years), and the average age is approximately 50 years. There is a female predominance in the sporadic form. Myxomas are usually pedunculated, solitary, and sporadic but may be associated with familial autosomal dominant syndromes. Case presentation:We report a 38-year-old female presented with large myxoma in the right atrium and atypical presentation and successfully underwent surgical excision of right atrial myxoma. The patient was asymptomatic on 6 months follow up. Conclusion:Cardiac myxomas are the most frequent finding among primary cardiac tumors. Clinical manifestations depend on the involvement in valvular obstruction, distant arterial embolisms, or nonspecific, constitutional symptoms. Transoesophageal Echocardiography is the cornerstone for diagnosis of atrial myxoma. Cardiac myxomas should be managed with surgical resection.
| S-EPMC6820116 | biostudies-literature
Project description:BackgroundIntraventricular masses are a relatively rare condition ranging from asymptomatic to potentially life-threatening situations.Case summaryHerein, we report a case of a 49-year-old woman under investigation for a massive right ventricular (RV) mass who underwent complete investigation for possible differential diagnosis, in the suspect of RV tumour. Multimodality imaging with cardiac computed tomography and magnetic resonance imaging showed the presence of a massive thrombus partially obliterating the right ventricle. Surgical removal of the mass showed a large area of stratified thrombosis with an underlying area of endocardial fibrosis. The patient has been then discharged in good clinical condition and with lifetime oral anticoagulation.DiscussionMassive RV thrombosis is a rare yet potentially fatal condition. Invasive management is preferable and lifetime anticoagulation is required to reduce possible downstream thrombotic complications.
| S-EPMC8343560 | biostudies-literature
Project description:Flood syndrome is a rare condition, where a patient with ascites suffers sudden umbilical hernia rupture and a subsequent drainage of ascitic fluid from the abdominal cavity. The cause of ascites is usually liver cirrhosis. Complications associated with cirrhosis of the liver make Flood syndrome difficult to manage. In these cases, conservative management has been associated with high morbidity. We describe, to our knowledge, for the first time a patient with Flood syndrome caused by right-sided heart failure. We also show that conservative treatment gives great initial outcome and is a viable option for this type of Flood syndrome.
| S-EPMC8790074 | biostudies-literature
Project description:Right ventricular diverticula (RVD) are very rare congenital anomalies and their association with constrictive pericarditis is even rarer. So far, only one case has been published in literature. We report a case of multiple congenital RVD with constrictive pericarditis and right heart failure which was incidentally identified on surveillance computed tomography (CT) for abdominal lymphangioma. Interval CT, echocardiography, and cardiac magnetic resonance imaging (CMR) studies were performed and reviewed. Computed tomography abdomen showed hepatic congestion with features of portal hypertension, increasing size of the RVD on review of serial CTs, and eccentric foci of pericardial calcification. Echocardiography performed for breathlessness demonstrated supranormal early diastolic tissue velocities with average of 19.8 cms-1 and a septal bounce phenomenon on m-mode imaging suggesting constrictive physiology, which triggered a CMR referral. Cardiac magnetic resonance imaging HASTE and right ventricular (RV) outflow tract imaging showed four outpouchings along RV free wall, the largest measuring 4.5 × 2 cm with a sizeable neck. These outpouchings displayed a trabecular network and/or were contractile aiding the diagnosis of diverticula as opposed to aneurysms. Right ventricular function was moderately compromised, whereas left ventricular function was preserved. Right ventricular diverticula can be associated with, and potentially be causative of, pericardial thickening and calcification eventually leading to constrictive pericarditis and heart failure.
| S-EPMC6601178 | biostudies-literature
Project description:Intra-cardiac extension of tumour thrombus of follicular carcinoma of thyroid is uncommon. We report a case of advanced thyroid carcinoma where tumour thrombus was well profiled with CT scan and transesophageal echo images and extension was noted from SVC into right atrium, with clinical features of superior vena cava syndrome. The clinical significance of the "ring sign" is discussed.
| S-EPMC5067792 | biostudies-literature
Project description:BackgroundMyxoma is an uncommon disease and its symptoms vary greatly depending on size, location and mobility. Right-sided myxoma, especially right ventricular myxoma, is much rarer, and the symptoms are alien and uncharacteristic. The lack of understandings poses challenges to prompt diagnosis and timely treatment.Case presentationA 44-year-old female patient was diagnosed with giant right ventricular tumor. Right heart failure and systemic congestion caused by right ventricular outflow tract obstruction were observed on this case. Surgery was performed to excise the mass which was measured at 9.5 * 5.0 cm and confirmed as myxoma pathologically.ConclusionsRight-side myxoma is easy to be unnoticed due to its low incident rate and atypical symptoms. Delay in surgical intervention might cause unrecoverable complications. More comprehensive understanding of the symptoms is expected to help improving the diagnose and treat of right-sided myxoma.
| S-EPMC7845124 | biostudies-literature
Project description:Intravenous leiomyomatosis (IVL) is an unusual uterine smooth muscle proliferation that can be associated with aggressive clinical behavior despite a histologically benign appearance. It has some overlapping molecular characteristics with both uterine leiomyoma and leiomyosarcoma based on limited genetic data. In this study, we assessed the clinical and morphological characteristics of 28 IVL and their correlation with molecular features and protein expression, using array comparative genomic hybridization (aCGH) and Cyclin D1, p16, phosphorylated-Rb, SMARCB1, SOX10, CAIX, SDHB and FH immunohistochemistry. The most common morphologies were cellular (n = 15), usual (n = 11), and vascular (n = 5; including 3 cellular IVL showing both vascular and cellular features). Among the immunohistochemical findings, the most striking was that all IVL showed differential expression of either p16 or Cyclin D1 in comparison to surrounding nonneoplastic tissue. Cytoplasmic phosphorylated-Rb was present in all but one IVL with hyalinization. SMARCB1, FH, and SDHB were retained; S0X10 and CAIX were not expressed. The most common genetic alterations involved 1p (39%), 22q (36%), 2q (29%), 1q (25%), 13q (21%), and 14q (21%). Hierarchical clustering analysis of recurrent aberrations revealed three molecular groups: Groups 1 (29%) and 2 (18%) with associated del(22q), and Group 3 (18%) with del(10q). The remaining IVL had nonspecific or no alterations by aCGH. Genomic index scores were calculated for all cases and showed no significant difference between the 14 IVL associated with aggressive clinical behavior (extrauterine extension or recurrence) and those without (median scores 5.15 vs 3.5). Among the 5 IVL associated with recurrence, 4 had a vascular morphology and 3 had alterations of 8q. Recurrent chromosome alterations detected herein overlap with those observed in the spectrum of uterine smooth muscle tumors and involve genes implicated in mesenchymal tumors at different sites with distinct morphological features.
| S-EPMC7483566 | biostudies-literature