intravascular angioleiomyoma

Intravascular Angioleiomyoma: A Rare Benign Tumor

Intravascular angioleiomyoma is a rare and benign tumor that arises from the vascular smooth muscle, specifically from the tunica media of blood vessels. It is characterized by the adherence of neoplastic smooth muscle cells to the walls of vascular channels [5][9].

Key Features:

• A morphologic variant of angioleiomyoma, which demonstrates purely intravascular growth [2][6]
• Composed of smooth muscle cells and thick-walled vessels [4]
• Typically occurs in the extremities and head, but can occur anywhere in the body [7]

Clinical Presentation: Intravascular angioleiomyoma is often asymptomatic, but may present with a palpable mass or nodule. The tumor is usually well-circumscribed and has a smooth surface.

Diagnosis: The diagnosis of intravascular angioleiomyoma is primarily based on histopathological examination, which shows the characteristic adherence of neoplastic smooth muscle cells to the walls of vascular channels [5][9].

Treatment and Prognosis: Surgical excision is the primary treatment for intravascular angioleiomyoma. The prognosis is generally good, with rare recurrence reported in literature [11].

References: [1] Sato H (2023) - Uterine angioleiomyoma [2] Dalainas I (2008) - Angioleiomyoma [4] Sajben FP (1999) - Intravascular growth of angioleiomyoma [5] Sato H (2023) - Uterine angioleiomyoma [6] Dalainas I (2008) - Angioleiomyoma [7] Dalainas I (2008) - Angioleiomyoma [9] Sajben FP (1999) - Intravascular growth of angioleiomyoma [11] Sato H (2023) - Uterine angioleiomyoma

Intravascular angioleiomyomas are rare, benign tumors that occur within blood vessels. The signs and symptoms of these tumors can vary depending on their location and size, but here are some common manifestations:

• Pain: Pain is a common symptom of intravascular angioleiomyoma, often described as a dull ache or discomfort in the affected area [10].
• Palpable mass: A palpable mass or lump may be felt in the affected area, which can be tender to touch [4].
• Respiratory symptoms: In cases where the tumor involves the heart or lungs, patients may experience respiratory symptoms such as shortness of breath (dyspnea), coughing, or chest pain [7].
• Cardiovascular symptoms: If the tumor affects the cardiovascular system, patients may experience symptoms like palpitations, syncope (fainting), or edema (swelling) in the lower limbs [7].
• Abdominal symptoms: In rare cases, intravascular angioleiomyoma can affect the abdominal organs, leading to symptoms such as abdominal pain, discomfort, or swelling [9].

It's essential to note that these symptoms can be non-specific and may not always point directly to an intravascular angioleiomyoma. A definitive diagnosis is typically made through imaging studies (e.g., ultrasound, CT scans) and histopathological examination of the tumor tissue.

References:

[4] by E Chronopoulos · 2018 [7] by X Li · 2022 [9] by J Chen · 2023 [10] Context result 10

Intravascular angioleiomyoma (IVL) can be challenging to diagnose, but various diagnostic tests can help confirm the condition. Here are some of the key diagnostic tests used for IVL:

• Imaging examinations: Imaging modalities such as ultrasound (US), computed tomography (CT), and fluorine-18-fluorodeoxyglucose positron emission tomography (FDG-PET) can be helpful in diagnosing and treating IVL [2][3]. Three-dimensional CT reconstruction is also significant for preoperative diagnosis of uterine angioleiomyoma and the surrounding tissues [4].
• Magnetic Resonance Imaging (MRI): MRI findings can describe tortuous vascular channels surrounded by smooth muscle bundles, which correspond to tortuous low signal intensity on T2-weighted images [7]. However, more detailed genetic testing may be needed to determine the difference in genetic background between angioleiomyoma and leiomyoma [11].
• Immunohistochemistry (IHC): IHC is a test ordered by pathologists to confirm the diagnosis of IVL and rule out other conditions that can look like angioleiomyoma under the microscope. The tumour is typically positive or reactive for smooth muscle markers, including smooth muscle actin, caldesmon, and calponin [13].
• Doppler sonography: Doppler sonography can show multiple vascular structures on the mass, which can be helpful in diagnosing angioleiomyoma [9].

It's worth noting that a diagnosis of IVL can be formed on a routine microscopic assessment in a typical clinical setting. Angioleiomyoma should be considered a possible diagnosis when a well-demarcated oval or round subcutaneous mass with specific imaging characteristics is seen [15].

Based on the search results, it appears that there are limited options for drug treatment of intravascular angioleiomyoma.

• According to search result [2], calcium channel blockers, particularly nifedipine, have been reported to relieve pain associated with many cases of piloleiomyomas. However, it is unclear if this treatment is effective for intravascular angioleiomyoma.
• There is no mention of specific drug treatments for intravascular angioleiomyoma in the search results.

However, adjuvant therapy with GnRH-a or other anti-estrogen drugs may be effective before or after surgery or even in inoperable cases [11]. This suggests that while there are limited options for drug treatment, hormone-based therapies may have a role to play in managing this condition.

It's worth noting that the definitive treatment of intravascular angioleiomyoma is complete removal of the tumor through either total hysterectomy or angiomyomectomy [5]. Therefore, surgical intervention remains the primary treatment approach for this condition.

Differential Diagnosis of Intravascular Angioleiomyoma

Intravascular angioleiomyoma, a rare smooth muscle tumor, can be challenging to diagnose due to its similarity in presentation and appearance with other vascular lesions. The differential diagnosis for intravascular angioleiomyoma includes:

• Giant cell tumors: These are benign tumors that can arise from the tendon sheath or synovial membrane [4][6].
• Neurogenic tumors: These are rare, slow-growing tumors that originate from nerve tissue and can mimic the appearance of angioleiomyomas [4][6].
• Hemangioma: A benign vascular tumor that can be similar in appearance to angioleiomyomas [8][10].
• Intravascular pyogenic granuloma (PG): A rare, benign vascular lesion that can present with a similar growth pattern as angioleiomyomas [7].
• Synovial sarcoma: A malignant tumor that can arise from the synovial membrane and may be difficult to distinguish from angioleiomyomas based on clinical presentation alone [14].

Key Features for Differential Diagnosis

To differentiate intravascular angioleiomyoma from other vascular lesions, it is essential to consider the following key features:

• Histological examination: A detailed histopathological examination can help distinguish angioleiomyomas from other tumors.
• Imaging studies: Ultrasonography and magnetic resonance (MR) imaging can be helpful in diagnosing angioleiomyoma and differentiating it from other vascular lesions [15].
• Clinical presentation: The clinical presentation of intravascular angioleiomyoma, including its location, size, and growth pattern, should be carefully evaluated to rule out other differential diagnoses.

References

[4] BS Jeong · 2011 · Cited by 1 — The differential diagnosis may include giant cell tumors of the tendon sheath, neurogenic tumor, and hemangioma. [6] by H Sato · 2023 · Cited by 1 — In conclusion, slow-growing uterine tumors should be considered in the differential diagnosis of uterine angioleiomyoma if they present with ... [7] by FP Sajben · 1999 · Cited by 18 — The histologic differential of lesions with an intra- vascular growth pattern includes intravascular pyogenic granuloma (PG). [8] by H Sato · 2023 · Cited by 1 — In conclusion, slow-growing uterine tumors should be considered in the differential diagnosis of uterine angioleiomyoma if they present with ... [10] It is difficult to make differential diagnoses between angioleiomyomas and other tumors. Angioleiomyoma should be considered in lesions of the extremities, such as ganglions, fibroma, lipoma, glomus tumors, neurofibroma, and cysts [7,8,9,10]. [14] The differential diagnosis for angioleiomyoma is broad and includes both benign and malignant entities such as schwannoma, glomus tumor and synovial sarcoma (11,22). [15] It is difficult to make differential diagnoses between angioleiomyomas and other tumors. Angioleiomyoma should be considered in lesions of the extremities, such as ganglions, fibroma, lipoma, glomus tumors, neurofibroma, and cysts [7,8,9,10]. Ultrasonography and magnetic resonance (MR) imaging can be helpful in the diagnosis of angioleiomyoma.