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      Symptomatic uterine leiomyomatosis with intracaval and intracardiac invasion: Video case report

      case-report
      a , * , b , b , b , c , d , b
      Gynecologic Oncology Reports
      Elsevier
      Uterine leiomyoma, Intravenous leiomyomatosis (IVL), Intracardiac leiomyomatosis (ICLM), Cardiopulmonary bypass (CPB), Hysterectomy, CPB, Cardiopulmonary bypass, ICLM, Intracardiac Leiomyomatosis, ICU, Intensive Care Unit, IVC, Inferior vena cava, IVL, Intravenous Leiomyomatosis, MRI, Magnetic Resonance Imaging, PCOS, Polycystic Ovary Syndrome, PO, Postoperative, RCC, Red Cell Concentrate, SIRS, Systemic Inflammatory Reaction, VTE, Venous Thromboembolism, WBC, Warm Blood Cardioplegia

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          Highlights

          • Uterine leiomyoma is the most prevalent benign tumor of the female genital tract.

          • Most patients with uterine leiomyoma are asymptomatic and acute complications are rare.

          • In the case of intracardiac leiomyomatosis (ICLM), surgery is morbid but highly recommended.

          • Studies demonstrated that the one-stage procedure is safer from the patient perspective.

          • Cardiopulmonary bypass reduced the clinical repercussions of surgery and improved the patient's quality of life.

          Abstract

          Background

          Fibroid is the most prevalent benign tumor of the female genital tract. Intravenous and intracardiac leiomyomatosis (IVL and ICLM, respectively) are rare complications that present with symptoms of pulmonary thromboembolism and heart failure and whose etiology, despite controversial, is a direct vascular invasion by a primary uterine leiomyoma.

          Case presentation

          We present the case of a 31-year-old female patient with a previous history of pelvic pain and dysmenorrhea, whose ultrasound showed an enlarged and heterogeneous uterus. Complete hysterectomy was performed, and the anatomopathological examination showed leiomyomas without evidence of malignancy. One month later, the patient manifested dyspnea and chest pain. A neoplastic thrombus was identified, extending from the inferior vena cava to the right atrium, for which we proceeded with cavo-atrial thrombectomy under Normothermic Cardiopulmonary Bypass (CPB) with Warm Blood Cardioplegia (WBC). A metastatic lung injury of non-malignant histology was also detected.

          Discussion

          Uterine leiomyoma is a very common benign tumor of the female genital tract. IVL with ICLM are rare and difficult-to-treat complications, whose etiology is a direct vascular invasion by a primary uterine leiomyoma, although it is still controversial. The incidence of ICLM is 10 to 30% of IVL cases. The main symptoms of ICLM are dyspnea, syncope, edema of the lower extremities and palpitations. Treatment is based on complete surgical removal of the tumor thrombus. Studies demonstrated that the one-stage procedure is safer from the patient’s perspective and that CPB with WBC reduced intraoperative blood loss and total operative time, ensuring a less traumatic postoperative.

          Conclusions

          Most patients with uterine leiomyoma are asymptomatic and acute complications are rare. In ICLM clinical manifestations are related to heart failure and flow obstruction. Because of the severity of the condition and the curative potential of treatment, surgery is morbid but highly recommended. The use of CPB with WBC improved the postoperative period and increased the patient’s quality of life.

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          Most cited references12

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          Incidence, aetiology and epidemiology of uterine fibroids.

          Uterine fibroids are the most common benign tumour of the female genital tract. However, their true prevalence is probably under-estimated, as the incidence at histology is more than double the clinical incidence. Recent longitudinal studies have estimated that the lifetime risk of fibroids in a woman over the age of 45 years is more than 60%, with incidence higher in blacks than in whites. The cause of fibroids remains unclear and their biology poorly understood. No single candidate gene has been detected for commonly occurring uterine fibroids. However, the occurrence of rare uterine fibroid syndromes, such as multiple cutaneous and uterine leiomyomatosis, has been traced to the gene that codes for the mitochondrial enzyme, fumarate hydratase. Cytogenetic abnormalities, particularly deletions of chromosome 7, which are found in up to 50% of fibroid specimens, seem to be secondary rather than primary events, and investigations into the role of tumour suppressor genes have yielded conflicting results. The key regulators of fibroid growth are ovarian steroids, both oestrogen and progestogen, growth factors and angiogenesis, and the process of apoptosis. Black race, heredity, nulliparity, obesity, polycystic ovary syndrome, diabetes and hypertension are associated with increased risk of fibroids, and there is emerging evidence that familial predisposition to fibroids is associated with a distinct pattern of clinical and molecular features compared with fibroids in families without this prevalence.
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            Intracardiac leiomyomatosis: a comprehensive analysis of 194 cases.

            Intracardiac leiomyomatosis is rare but has been increasingly reported in recent years. Owing to its rarity, intracardiac leiomyomatosis has been reported only as isolated case reports and case series. This disorder is thought to be underestimated and easily overlooked in the clinic, while it is dangerous owing to the risk of sudden death caused by total outflow tract obstruction. We performed an electronic literature search for intracardiac leiomyomatosis and identified 194 cases that were reported in English from 1974 (the first reported case) to September 2012. Our aim is to provide a detailed and comprehensive review of the clinical presentation, diagnosis, histopathological characterization, treatment and prognosis of this disorder. According to our analysis, intracardiac leiomyomatosis is most common in the fifth decade, and the mean age of detection is ~50 years. Most patients had undergone previous hysterectomy/myomectomy or had a coexisting uterine leiomyoma when admitted. The most common clinical presentations were dyspnoea, syncope, oedema of the lower extremities and palpitation. Transoesophageal echocardiography, computed tomography and magnetic resonance imaging are helpful in the preoperative diagnosis and to guide the surgical management. Complete removal guarantees an excellent outcome, with no recurrence or postoperative death, while incomplete removal leads to recurrence in one-third of patients. Anti-oestrogen therapy is not imperative after incomplete removal owing to its inability to prevent recurrence.
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              Different surgical strategies of patients with intravenous leiomyomatosis

              Abstract Intravenous leiomyomatosis (IVL) is a rare benign tumor. The study aimed to assess outcomes of patients treated surgically for IVL. Between November 2002 and January 2015, 76 patients were treated for IVL. The stage of IVL was evaluated preoperatively by echocardiography and enhanced computerized tomography (CT) scan, and graded into 4 stages according to intravascular tumor progression. We recorded age, lower limb edema before surgery, surgical parameters, and hospitalization expenses. Patients were followed up every 6 months and tumor recurrence was assessed by CT and ultrasound. Patients were followed up for a mean of 4.5 ± 2.5 years (range 1–13 years) and there was no operative, hospital, or long-term mortality or were lost to follow-up. The rate of lower extremity edema, amount of blood loss, postoperative transfusion, length of intensive care unit (ICU) stay, postoperative hospitalization, and hospitalization expenses differed significantly between patients at different presurgery stages. Tumors recurred in 4 of 7 patients with stage I IVL that opted for surgery that preserved the ovaries and uterus. No recurrence was observed in patients graded stage II or more, in all of which the uterus and ovaries were removed. Recurrence was observed in only 4 of 76 cases of IVL, all of whom opted for surgery that spared the ovaries and uterus. Different surgical strategies should be decided based on the staging to completely remove the tumor and ensure the safety of patients. Removal of both ovaries is necessary for inhibiting tumor growth and avoiding recurrence.
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                Author and article information

                Contributors
                Journal
                Gynecol Oncol Rep
                Gynecol Oncol Rep
                Gynecologic Oncology Reports
                Elsevier
                2352-5789
                19 December 2022
                February 2023
                19 December 2022
                : 45
                : 101127
                Affiliations
                [a ]Medical Student at Faculdade de Medicina da Universidade de São Paulo (FMUSP), São Paulo, SP, Brazil
                [b ]III Surgical Clinic at Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, SP, Brazil
                [c ]Vascular Surgery at Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, SP, Brazil
                [d ]Cardiovascular Surgery at Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, SP, Brazil
                Author notes
                [* ]Corresponding author at: Av. Dr. Arnaldo, 455, Cerqueira César, CEP: 01246903, São Paulo, SP, Brazil. debora.cassol@ 123456fm.usp.br
                Article
                S2352-5789(22)00209-0 101127
                10.1016/j.gore.2022.101127
                9829743
                36636580
                f4c55eb1-746b-4e64-8228-07ee2daccef9
                © 2022 The Authors

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 7 August 2022
                : 26 November 2022
                : 16 December 2022
                Categories
                Case Report

                uterine leiomyoma,intravenous leiomyomatosis (ivl),intracardiac leiomyomatosis (iclm),cardiopulmonary bypass (cpb),hysterectomy,cpb, cardiopulmonary bypass,iclm, intracardiac leiomyomatosis,icu, intensive care unit,ivc, inferior vena cava,ivl, intravenous leiomyomatosis,mri, magnetic resonance imaging,pcos, polycystic ovary syndrome,po, postoperative,rcc, red cell concentrate,sirs, systemic inflammatory reaction,vte, venous thromboembolism,wbc, warm blood cardioplegia

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