Intracardiac Leiomyomatosis Arising from a Uterine Leiomyoma - A Rare Case Requiring a Multidisciplinary Laparotomy-Thoracotomy Approach.

A 37-year-old lady, when being evaluated in gynecology for pain in the abdomen, was found to have a pelvic mass suspicious of ovarian cancer with markers negative. There was an ovarian vein thrombosis extending to the right atrium in the contrast-enhanced computed tomography scan. A fluorodeoxyglucose positron emission tomography-computed tomography ruled out any other lesions. The patient underwent surgery under general anesthesia with transesophageal echocardiography to monitor the atrial thrombus. Intraoperatively, a retroperitoneal mass is seen arising from the right adnexal region of the uterus extending to the lumbar area. After the hysterectomy, bilateral salpingo-oophorectomy, tumor resection, and baring and looping of the retroperitoneal vessels, a sternotomy was done, and she was put on cardiopulmonary bypass. The tumor thrombus had two limbs both arising from the mass, one through the ovarian and the second through the iliac veins and joining together inside the inferior vena cava (IVC). With the excision of the ovarian vein at its junction, atrial incision, and incisions over the iliac veins and IVC, the thrombus was removed completely in a single sitting. Final histopathology revealed intravenous leiomyomatosis and no malignancy. We report this case as a rare disease, with both ovarian and iliac thrombus being a further rarity and a multidepartment joint effort with a successful outcome.

Tumor in Transit: Intracardiac Leiomyomatosis.

Intravenous leiomyoma is a rare condition that occurs when there is a vascular invasion of a pre-existing uterine leiomyoma. The tumor can metastasize to structures such as the heart and lungs. We discuss a case of metastasis to the heart resulting in severe tricuspid regurgitation. Surgical intervention is the primary modality; usually a staged approach involving cardiac surgery along with abdominal and/or pelvic surgery. We want to highlight the importance of fully investigating right-sided cardiac masses. While there are common etiologies for these masses, one must maintain a high degree of suspicion for an intravenous leiomyoma, especially if a female has certain risk factors such as a prior history of fibroids or a hysterectomy. We also stress the importance of a multi-disciplinary team approach when providing care to these patients, along with reviewing all modalities of imaging.

Intravenous leiomyomatosis involving the right heart: Comprehensive evaluation using echocardiography combined with abdominal ultrasonography.

Intravenous leiomyomatosis (IVL) is a rare condition characterized by a tumor that originates within a pelvic vein wall or the uterine smooth muscle, with extension into the right heart (referred to as intracardiac leiomyomatosis) in approximately 10% of all IVL cases. Usually, computed tomography (CT) or magnetic resonance imaging (MRI) is performed for diagnostic imaging of IVL. Notably, this neoplasm presents with characteristic ultrasonographic findings. In this report, we present the case of a 49-year-old woman with IVL, which extended into the right heart. Echocardiography combined with abdominal ultrasonography was useful to outline the course of the tumor from the right heart to the uterus. Our findings suggest that in addition to CT or MRI, ultrasonography shows high diagnostic value in cases of IVL, and ultrasonography combined with CT or MRI can further improve the preoperative diagnosis rate of IVL.

Intravenous leiomyomatosis: Case report and review of the literature / Leiomiomatosis intravenosa: a propósito de un caso y revisión de la literatura

Introduction: We retrospectively reviewed a case of a 42-year-old woman with intravenous leiomyomatosis. Clinical findings: The present study describes a case of IVL extending into the right internal and common iliac veins. Diagnosis: The patient was diagnosed by definitive pathological examination after a hysterectomy and double salpingectomy. Hypovolaemic shock due to delayed intraperitoneal bleeding from the ovarian vessels pedicle was observed. An emergent laparotomy for haemostasis was performed. A follow-up MRI, 2 months later, showed a polylobulated mass extending inside the right internal and common iliac veins. Intervention and result: The patient underwent a third laparotomic procedure with the removal of the right pelvic mass together with the involved veins. The postoperative course was uneventful, and the patient remains well at 3-month follow-up, with no signs of lower limb edema or venous disorders. Conclusion: Intracardiac leiomyomatosis is mostly diagnosed in premenopausal women. The most severe manifestation could be a vascular thrombosis or a right atrial tumor in the case of intracardiac involvement. Early and appropriate diagnosis is essential for optimal treatment. Surgery is the best treatment.(AU)

Introducción: Presentamos el caso de una paciente de 42 años con leiomiomatosis intravenosa. Hallazgos clínicos: El estudio presente describe un caso de IVL con afectación de las venas ilíaca común e interna derecha. Diagnóstico: La paciente fue diagnosticada mediante el examen histológico definitivo tras una histerectomía total con doble salpingectomía. Se objetivó un shock hipovolémico por sangrado intraperitoneal tardío del pedículo de vasos ováricos. Se realizó una laparotomía urgente para hemostasia. Una RMN durante el seguimiento, 2 meses después, mostró una masa polilobulada con afectación de la vena ilíaca común y la vena ilíaca interna derecha. Intervención y resultado: A la paciente se le realizó una tercera laparotomía con la extirpación de la masa pélvica derecha. El curso postoperatorio fue satisfactorio y la paciente permaneció asintomática a los 3 meses de seguimiento, sin signos de edema en extremidades inferiores ni otras afectaciones vasculares. Conclusión. La leiomiomatosis intracardíaca se diagnostica más frecuentemente en mujeres premenopáusicas. La forma de manifestación más severa sería una trombosis vascular o un tumor intracardíaco en la aurícula derecha, en el caso de afectación intracardíaca. Un diagnóstico precoz es esencial para un tratamiento óptimo. La cirugía es el mejor tratamiento.(AU)

Treatment of intravascular leiomyomatosis: Case report and literature review.

Intravascular leiomyomatosis is a rare disease characterized by the proliferation of a smooth muscle tumor in the vascular lumen and possible cardiac extension. The best surgical procedure is yet to be identified. The present study aimed to explore the optimal surgical strategies. Fourpatients with intravascular leiomyomatosis in our hospital were analyzed retrospectively. All patients underwent one-stage surgery with multidisciplinary collaboration and were followed for 2 to 56 months. Based on the preliminary results, we found that one-stage surgery is safe and effective. We recommended Ma's staging system as a guide for selecting the surgical procedures.

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

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.

One-stage resection of intravascular leiomyomatosis involving the right heart chamber through a single laparotomy.

Objectives: This retrospective study aimed to summarize the feasibility and experience of utilizing a one-stage operation via single laparotomy to treat intracardiac leiomyomatosis (ICL). Materials and methods: A retrospective study of 13 patients with ICL who underwent one-stage resections was conducted at Peking Union Medical College Hospital from June 2015 to December 2021. All patients had their tumors removed by single laparotomy and were divided into a short venotomy group (6 cases) and an extensive venotomy group (7 cases). We reviewed the patient characteristics, surgical procedures, postoperative pathology, and perioperative and follow-up outcomes of all patients. Results: All patients underwent surgery for ICL resection using single laparotomy with a 100% success rate. Two patients had tumors distal to the right ventricle (RV), 2 patients had tumors that protruded into the RV in diastole and were confined to the right atrium (RA) in systole, and the other 9 patients had tumors confined to the RA that did not involve the tricuspid valve. The tumor was completely resected in 10 patients, yet 3 patients had a residual tumor. Six patients completed the surgery with short venotomy, 7 completed the surgery with extensive venotomy, and 9 underwent simultaneous total hysterectomy and bilateral adnexal resection. The mean operative time was 370.8 ± 111.0 min, and the mean blood loss was 992.3 ± 994.5 mL. Intraoperative blood loss was lower (483.3 ± 213.7 ml vs. 1429.2 ± 1208.0 ml; P = 0.020) and operative time was shorter (286.5 ± 71.9 min vs. 443.1 ± 84.4 min; P=0.004) in the short venotomy group than in the extensive venotomy group. At a mean follow-up of 26.3 ± 18.8 months, 1 patient had a local recurrence in the pelvis, and 1 patient died of pancreatic cancer, while the remaining patients had no recurrence during follow-up. Conclusion: One-stage resection of ICL patients by means of a single laparotomy is feasible and effective.

A rare case of intracardiac leiomyomatosis in a patient who underwent complete tumor resection only using the laparotomic approach.

Intracardiac leiomyomatosis (ICLM) is a rare tumor that usually originates from the mesenchymal cells of the uterus. If left untreated, it may lead to cardiac symptoms and sudden death. The present case was a 45-year-old female patient who presented with occasional palpitations. The imaging methods revealed a heterogeneous mass likely to be leiomyoma with intense enhancement in the arterial phase in the uterus, extending from the ovarian and uterine veins to the heart through the inferior vena cava. The tumor council attributed these findings to intravenous leiomyomatosis with intracardiac extension. Accordingly, a multidisciplinary surgical team performed complete excision of the intracardiac leiomyomatosis in the patient presented herein using only the laparotomic approach. The gold standard in the treatment of ICLM is complete tumor excision. Excision of intracardiac tumors can be performed through controlled traction from the abdominal region.

A Case of Syncope Due to Intracardiac Leiomyomatosis.

We present a case of a 46-year-old female presenting with syncope. Echocardiography initially showed a right atrial mass. Further evaluation revealed a mass arising from the fundus of the uterus, with a tumor thrombus in the left gonadal vein, extending into the left renal vein and through the inferior vena cava (IVC) into the right heart across the tricuspid valve. She was managed with surgical resection, and postoperative pathology was consistent with intravenous leiomyomatosis (IVL). IVL is a rare uterine smooth muscle cell neoplasm which extends into the venous system. Gynecological tumors are often overlooked in differential diagnosis for atrial masses. A benign tumor like fibroid, in rare circumstances, can extend into the right side of the heart causing dynamic obstruction to outflow tract, thus increasing mortality. The objective of this article is to present such a case and highlight the broad differentials of atrial masses, including IVL.

Multidisciplinary approach to pelvic leiomyomatosis with intracaval and intracardiac extension: A case report and review of the literature.

Intravenous leiomyomatosis (IVL) is an uncommon variant of leiomyoma characterized by intravascular proliferation of a histologically benign smooth muscle tumor extending beyond the uterus into the distant great vessels. Leiomyomatosis may reach the inferior vena cava, right atrium, and pulmonary arteries. Owing to its rare occurrence, intracardiac leiomyomatosis has been reported as isolated case reports and small case series. Early diagnosis and prompt surgical intervention are vital to prevent cardiac symptoms, pulmonary embolism, and sudden death. Complete tumor resection is essential for a favorable outcome, usually assisted with multimodal surgical imaging and multidisciplinary surgical planning. Herein, we report the case of a 50-year-old female that presented with a three-month history of abdominal pain and lower extremity edema with evidence of IVL extending to the inferior vena cava and right atrium. The patient was managed with a single-stage surgery involving cardiopulmonary bypass and excision of the right atrial and inferior vena cava tumors, as well as modified radical total abdominal hysterectomy and bilateral salpingo-oophorectomy.

Extrapelvic intravenous uterine leiomyomatosis mimicing cardiac myxoma and deep vein thrombosis.

Extrapelvic intravenous uterine leiomyomatosis is a rare smooth muscle neoplasm. Uterine leiomyomatosis is a histologically benign pathology. Rarely, it can be confused with a cardiac mass. A 44-year-old female patient was admitted with increasing severity of pain and swelling in both legs for the past week. The patient was initially diagnosed with bilateral deep vein thrombosis. After further evaluation, we decided that the patient had cardiac myxoma. However, we intraoperatively observed that the lesion in the right atrium was arising from the inferior vena cava. In the final postoperative histopathological evaluation, the definite diagnosis was extrapelvic intravenous leiomyomatosis. The patient was discharged uneventfully following her second operation.

The role of estrogen deprivation therapy in premenopausal women with primary unresectable intracardiac leiomyomatosis: a systematic review and meta-analysis.

BACKGROUND: Intracardiac leiomyomatosis (ICLM) is a rare life-threatening form of intravenous leiomyomatosis (IVLM). The incomplete resection and recurrence are associated with high morbidity and mortality. The objective of this study is to identify that whether estrogen deprivation therapies, including bilateral salpingo-oophorectomy (BSO)-based surgery and gonadotrophin releasing hormone agonists (GnRHa) administration, could bring benefits to patients with primary unresectable ICLM. METHODS: PubMed/MEDLINE (Ovid) was searched (up to May 2021) for studies reporting individual patient data on demographics, clinicopathological features, treatment, and follow-up information. Exclusion criteria were patients who may have been included in two or more publications. This study was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. RESULTS: A total of 114 patients from 70 papers were included. Several reports showed that the tumor in the right atrium and inferior vena cava shrank dramatically after BSO-based surgery, or GnRHa administrated preoperatively in premenopausal women. The rate of complete resection was 64.04% in patients with ICLM, which was 85.25% in no/slight adhesion and no pulmonary nodules group, while 22.22% in firm/extensive adhesion and/or pulmonary nodules group (p < 0.0001). Meanwhile, the recurrence rates in patients with complete resection and incomplete resection were 4.29% and 37.84% respectively (p < 0.0001). Furthermore, complete resection with BSO had the lowest recurrence rate of 3.13%, incomplete resection with BSO had a progression rate of 45.45%, while incomplete resection with ovarian preservation had the highest progression rate of 75.00%. CONCLUSIONS: The recurrence rate of ICLM was closely related to firm/extensive adhesion in IVC or above, and/or pulmonary nodules. BSO-based surgery might reduce the recurrence rate no matter ICLM could be completely resected or not. In addition, estrogen deprivation therapies could decrease tumor burden as a primary treatment, and further make a secondary complete resection feasible in premenopausal women with initially unresectable ICLM.

Surgical treatment strategies for extra-pelvic intravenous leiomyomatosis.

BACKGROUND: Extra-pelvic intravenous leiomyomatosis (IVL) extending into inferior vena cava (IVC) or heart (i.e. intracardiac leiomyomatosis, ICL) is an extremely rare benign disease. No consensus has been reached on the optimal surgical strategy. The aim of this study is to introduce four types of one-stage surgical strategies including less invasive options and a guideline to select patient-specific strategy for this disease. METHODS: Twenty-four patients of extra-pelvic IVLs receiving one-stage resections at the Zhongshan Hospital from July 2011 to November 2019 were reviewed retrospectively. Base on the initial experiences of the indiscriminate choices of tumor thrombectomies through sterno-laparotomy under cardiopulmonary bypass (CPB) in 6 ICLs, an anatomy-based guideline for four types of surgical strategies was developed and applied for the next 18 patients. RESULTS: Under the direction of guideline, tumor thrombectomies through single laparotomy were applied without CPB in 2 ICLs and 4 IVLs confined in IVC, or with CPB in 7 ICLs. Guideline-directed double-incisions with CPB were applied in only 5 ICLs, including 1 receiving mini-thoracotomy and 4 receiving sternotomy because of tumor adherences with right atriums in 2 and with pulmonary arteries in 2. All 24 patients accomplished one-stage panhysterectomy, bilateral adnexectomy and complete resections of intracaval and intracardiac tumors. For residual pelvic intravenous tumors in 19 patients, 17 received macroscopically complete resections while the other 2 failed because of high risk of hemorrhage. Intraoperative blood losses, operation time and hospitalization expense in the single-laparotomy non-CPB group were significantly lesser than the other groups. In CPB groups, inpatient stay and hospitalization expense in the single-incision group were significantly lesser than the double-incisions group. All patients were alive and free of recurrences during a mean follow-up of 35.4 ± 27.2 months (range, 1-100 months). The pelvic tumor residues in 2 patients remained unchanged for 51 and 52 months since operation, respectively. CONCLUSIONS: For various extra-pelvic IVLs, the 4 types of surgical strategies including less invasive options are feasible, providing these are selected by a guideline base on the tumor extension and morphology. The proposed guideline is believed to accommodate more patients receiving less invasive surgery without compromising the curative effect.

Long-term outcomes of surgical treatment for intravascular leiomyomatosis.

BACKGROUND: Intravascular leiomyomatosis (IVL) is relatively rare. The optimal surgical method and long-term outcomes are not completely understood. METHODS: Medical records between 2007 and 2017 in our hospital were analyzed to identify IVL cases with surgical intervention. Their medical records, operative details, and follow-up were collected by chart review and telephone communication. RESULTS: Eight patients with IVL were included in the study, accounting for 0.26% of all uterine leiomyoma cases. Primary IVL was confined to pelvic cavity in three patients, extended to the inferior vena cava (IVC) below renal vein in one, reached IVC and right atrium in three, and reached main pulmonary artery in one. One-stage operation was performed for seven patients. Cardiopulmonary bypass was done in four patients, and aortic cross-clamp and temporary circulatory arrest was performed in two patients. None of the four patients with intrapulmonary tumors received concomitant pulmonary tumor resection. There was no operative mortality and four morbidities, including ureter injury (2), bladder injury (1), and femoral vein thrombosis (1). During follow-up, two patients exhibited local recurrence of the tumor in the pelvis, and one patient had rapidly growing intrapulmonary tumor three months post-operatively. Intrapulmonary tumors in the other three patients remained stationary at 6, 84, and 120 months post-operatively. CONCLUSION: One-stage operation to completely remove IVL is feasible and with good long-term outcomes, which is recommended if the patient can tolerate the operation. Concomitant intrapulmonary tumors can be followed up watchfully except when associated with pleural effusion or the pathology indicating trend of increasing malignancy.

Massive pelvic recurrence of uterine leiomyomatosis with intracaval-intracardiac extension: video case report and literature review.

BACKGROUND: Uterine leiomyomas represent the gynecological neoplasm with the highest prevalence worldwide. This apparently benign pathological entity may permeate into the venous system causing the so-called intravenous leiomyomatosis of the uterus (IVL). IVL may seldom extend to large caliber veins and reach the right cardiac chambers or pulmonary arteries and cause signs of right sided congestive heart failure and sudden death. Due to its low incidence, however, IVL with intracardiac extension is often misdiagnosed resulting in deferred treatment. No consensus has been obtained regarding the standard surgical approach to be used for this rare condition. We describe the case of a massive pelvic recurrence of uterine leiomyomatosis with intracardiac extension and provide a review of the literature, analyzing management and surgical outcomes. CASE PRESENTATION: We present the case of a 46-year-old premenopausal woman presenting with lower-extremity edema, recurrent syncopes and a history of subtotal hysterectomy for multiple uterine fibroids. She was diagnosed with pelvic recurrence of uterine leiomyomatosis and IVL with cardiac involvement. A two-stage surgical excision of the intracardiac-intracaval mass and pelvic leiomyomatosis was performed. The patient had an uneventful recovery and no evidence of recurrence was observed on follow-up. CONCLUSIONS: By virtue of the rarity of the present pathology, awareness is widely scarce and diagnosis is often delayed. Early recognition is difficult due to initial aspecific and subtle clinical manifestations. Nevertheless, suspicion should be held high in premenopausal women with known history of uterine leiomyomata, presenting with cardiovascular symptoms and evidence of a free-floating mass within the right cardiac chambers. In-depth imaging is crucial for defining its anatomical origin and relations. Prompt surgical treatment with radical excision of pelvic and intravenous leiomyomatosis guarantees favorable outcomes and excellent prognosis with low rates of recurrence, whereas delayed diagnosis and treatment exposes to increased risk of congestive heart failure and sudden death.

Intracardiac Leiomyomatosis - an Unusual Cause of Syncope in a Middle-Aged Woman.

Intracardiac leiomyomatosis is a rare complication that occurs when a uterine leiomyoma (fibroid) undergoes vascular invasion and propagates within the inferior vena cava to reach the right atrium. This article describes a case of intracardiac leiomyomatosis in a middle-aged woman, exploring the presentation, diagnosis and surgical management of this condition. In this case the presenting complaints were syncope and atrial fibrillation, illustrating the importance of performing a transthoracic echocardiogram in patients presenting with their first episode of atrial fibrillation. Clinicians should consider intracardiac leiomyomatosis when evaluating women with right heart masses, especially those with a history of uterine leiomyomas.

Intracardiac leiomyomatosis presenting as an intraoperative consultation.

Intravenous leiomyomatosis (IVL) is an extremely rare variant of leiomyoma in which nodular masses of tumor grow within venous channels. Rarely, the tumor can reach the vena cava and right heart. We present a case of a 45-year-old woman, admitted with rapidly evolving exertional dyspnea. Cardiac ultrasonography revealed a "mass in the right chambers". She was submitted to right atriotomy plus tumorectomy, with intraoperative consultation requested. Grossly, the tumor was polypoid, firm, with a smooth surface. The frozen section showed a lesion composed of tortuous vessels and some areas with a fibrillar eosinophil extracellular matrix and others with spindle cells, without significant atypia, mitosis or necrosis. The diagnosis was deferred for definitive paraffin sections. In the definitive H&E and immunohistochemical stains, the case was diagnosed as an IVL and confirmed in the hysterectomy specimen. This is the first case report describing an intraoperative consultation of an intracardiac leiomyomatosis. Clinical information and pathologist awareness to this entity are essential for the correct diagnosis in frozen section.