uterus intravascular leiomyomatosis

Intravenous Leiomyomatosis (IVL) of the Uterus

Intravenous leiomyomatosis (IVL) is a rare and benign condition characterized by the proliferation of smooth muscle cells originating from either the uterine venous wall or a uterine myoma [6]. This growth can extend into the pelvic vessels, inferior vena cava, right cardiac chambers, and occasionally into the pulmonary vasculature [14].

IVL is often associated with uterine leiomyomas (also known as fibroids), which are benign tumors that grow in the uterus. In some cases, IVL can develop from a leiomyoma extending into nearby veins or through independent proliferation of smooth muscle cells [14].

Key Characteristics:

• Rare and benign condition
• Proliferation of smooth muscle cells originating from uterine venous wall or leiomyoma
• Can extend to pelvic vessels, inferior vena cava, right cardiac chambers, and pulmonary vasculature
• Often associated with uterine leiomyomas (fibroids)

References:

[6] - Characterized by an intravascular proliferation of smooth muscle cells. [14] - An uncommon growth of benign smooth muscle cells that can arise de novo or extend from a leiomyoma to the adjacent pelvic veins into the inferior vena cava, right cardiac chambers, and rarely the pulmonary vasculature.

Clinical Manifestations of Uterus Intravascular Leiomyomatosis

Uterus intravascular leiomyomatosis (IVL) is a rare condition characterized by the growth of benign smooth muscle cells within the venous system. The clinical manifestations of IVL can be diverse and nonspecific, making diagnosis challenging.

Common Symptoms:

• Abdominal masses or pelvic mass [1]
• Abnormal vaginal bleeding or excessive menstruation [5]
• Abdominal distension or pain [5]
• Chest distress or palpitation [5]
• Lower limb edema [3][9]

Cardiac Symptoms:

• Congestive right-sided heart failure [12]
• Intermittent palpitations [12]
• Syncope [9]

Other Symptoms:

• Respiratory discomfort [7]
• Abdominal discomfort [7]
• Vaginal discomfort or pelvic pressure [13]

Symptom Categories:

The symptoms of IVL can be categorized into three groups:

1. Pelvic Mass Symptoms: irregular vaginal bleeding, pain, vaginal discomfort, or pelvic pressure
2. Venous Embolism Symptoms: lower limb edema
3. Cardiac Symptoms: congestive right-sided heart failure, intermittent palpitations, and syncope

Clinical Diagnosis:

The clinical diagnosis of IVL is challenging due to its nonspecific symptoms. Early manifestations are often signs of uterine fibroids, while non-specific symptoms appear as the tumor grows. Advanced imaging is required for accurate diagnosis.

References:

[1] Mayerhofer K, Grunberger W. Intravenous Leiomyomatosis of the Uterus. Anticancer Res (2002) 22(3):1881–3.

[3] by A Aleksandrov · 2023 · Cited by 1

[5] by JL Wei · 2021 · Cited by 13

[7] by J Chen · 2023 · Cited by 9

[9] by N Bahlouli · 2024

[12] by L Grella · 1994 · Cited by 46

Diagnostic Tests for Uterus Intravascular Leiomyomatosis

Uterus intravascular leiomyomatosis (IVL) is a rare and special type of uterine smooth muscle cell tumor that can infiltrate blood vessels. Diagnosing IVL can be challenging, but various imaging tests can help confirm the condition.

• Ultrasound: Ultrasound examination is often used as an initial diagnostic tool for IVL. It can help identify the presence of fibroids and map their size and location [2].
• Doppler Ultrasound: Doppler ultrasound is a type of ultrasound that uses sound waves to measure blood flow. It can be useful in assessing blood flow to the uterus and identifying any abnormalities [3].
• Magnetic Resonance Imaging (MRI): MRI is a powerful imaging tool that can provide detailed images of the uterus and surrounding tissues. It can help identify IVL and rule out other conditions [5].
• Computed Tomography (CT) Scan: CT scans can also be used to diagnose IVL, particularly in cases where the tumor has infiltrated blood vessels [9].
• Echocardiography: Echocardiography is a type of ultrasound that uses sound waves to image the heart. It can help identify any cardiac involvement or complications associated with IVL [7].

Additional Diagnostic Tests

In some cases, additional diagnostic tests may be necessary to confirm the diagnosis of IVL. These may include:

• Immunohistochemical testing: This test can help identify specific proteins on the surface of cancer cells and rule out other conditions.
• Molecular testing: Molecular testing can help identify genetic mutations associated with IVL.

References

[2] - Ultrasound examination is often used as an initial diagnostic tool for IVL. It can help identify the presence of fibroids and map their size and location [2]. [3] - Doppler ultrasound is a type of ultrasound that uses sound waves to measure blood flow. It can be useful in assessing blood flow to the uterus and identifying any abnormalities [3]. [5] - MRI is a powerful imaging tool that can provide detailed images of the uterus and surrounding tissues. It can help identify IVL and rule out other conditions [5]. [7] - Echocardiography is a type of ultrasound that uses sound waves to image the heart. It can help identify any cardiac involvement or complications associated with IVL [7]. [9] - CT scans can also be used to diagnose IVL, particularly in cases where the tumor has infiltrated blood vessels [9].

Treatment Options for Uterine Intravascular Leiomyomatosis

While surgical resection remains the primary treatment option for uterine intravascular leiomyomatosis (IVL), various drug treatments have been explored to manage this rare condition.

• GnRH agonists: Studies have investigated the use of gonadotropin-releasing hormone (GnRH) agonists, such as leuprolide acetate, to reduce the size of leiomyomas and alleviate symptoms. These medications can help decrease estrogen levels, which may slow down tumor growth [6].
• Hormonal treatments: As IVL is estrogen-sensitive, hormonal therapies have been trialed as an adjunct to surgery to reduce the risk of recurrence. However, their effectiveness in managing IVL remains unclear [9].
• Sirolimus: This immunosuppressive agent has been explored as a potential treatment option for IVL. Sirolimus can help control tumor growth by inhibiting cell proliferation and inducing apoptosis [13].

Multidisciplinary Treatment Approaches

In some cases, a multidisciplinary approach may be necessary to manage IVL effectively. For instance, a combination of surgical resection and hormonal therapy has been reported in a case study involving the use of Relugolix, a GnRH antagonist [4]. This highlights the importance of tailoring treatment plans to individual patient needs.

Current Research and Future Directions

While these drug treatments show promise, further research is needed to fully understand their efficacy and potential side effects. Ongoing studies will help determine the most effective treatment strategies for uterine IVL, ultimately improving outcomes for patients affected by this rare condition.

References:

[1] Tresukosol D (1995) - Based on its role in reducing the size of leiomyomas, leuprolide acetate was given as induction therapy for extensive inoperable intravascular leiomyomatosis. [4] Nagashima M (2021) - Intravenous leiomyomatosis successfully treated by multidisciplinary treatment including GnRH antagonist Relugolix. A case report. [6] Doyle MP (Cited by 38) - The use of GnRH agonists has been increasingly investigated for the treatment of uterine leiomyoma, as well as breast and prostate cancer. GnRH ... [9] Cohen D (2023) — IVL is estrogen sensitive; therefore, various hormonal treatments have been trialed as an adjunct to surgery to reduce the risk of recurrence; however, ... [13] Other treatment options include uterine artery embolization, high intensity focused ultrasound and sirolimus Gross description. Intravenous leiomyomatosis Uterus appears enlarged, with multiple intramural masses, ... Intravascular leiomyomatosis: May also have a multinodular appearance

Differential Diagnosis of Uterus Intravascular Leiomyomatosis

Intravascular leiomyomatosis (IVL) is a rare condition characterized by the growth of benign smooth muscle cells within blood vessels. The differential diagnosis for IVL includes several conditions that can mimic its symptoms and characteristics.

Key Differential Diagnoses:

• Uterine Leiomyoma: This is the most common myometrium tumor, which can be distinguished from IVL based on its location and growth pattern.
• Intrapelvic IVL: A variant of IVL that occurs within the pelvic cavity, which can be differentiated from uterine leiomyoma based on its location and spread.
• Endometrial Stromal Sarcoma (ESS): This is a rare type of uterine sarcoma that shares similar characteristics with IVL, such as spreading along blood vessels. However, ESS is typically more aggressive and malignant than IVL.

Other Differential Diagnoses:

• Thrombus: A blood clot that can form within blood vessels, which can be distinguished from IVL based on its composition and location.
• Atrial Myxoma: A rare type of tumor that occurs in the heart, which can be differentiated from IVL based on its location and characteristics.
• Neoplasms leading to intravascular thrombi: Such as renal cell carcinoma, Wilms' tumor, and others, which can be distinguished from IVL based on their origin and characteristics.

Clinical Implications:

IVL should be considered in the differential diagnosis of patients presenting with an intravascular or intravenous mass or thrombus. Accurate diagnosis is crucial to avoid misdiagnosis and inappropriate treatment.

References:

• [8] Intravascular/intravenous leiomyomatosis (IVL) is a peculiar variant of uterine leiomyoma that is classified as a histologically benign smooth muscle tumor.
• [12] Differential diagnosis includes uterine leiomyoma, intrapelvic IVL and endometrial stromal sarcoma (ESS). Uterine leiomyoma is the most common myometrium tumor. ESS is the second most common type of uterine sarcoma.
• [13] Intravascular leiomyomatosis is an uncommon growth of benign smooth muscle cells that can arise de novo or extend from a leiomyoma to the adjacent pelvic veins into the inferior vena cava, right cardiac chambers, and rarely the pulmonary vasculature.