uterine corpus epithelioid leiomyoma

Uterine Corpus Epithelioid Leiomyoma: A Rare Variant

Uterine corpus epithelioid leiomyoma is a rare variant of leiomyoma, which is the most common type of benign smooth muscle tumor in the uterus. This condition is characterized by the presence of round or polygonal epithelioid smooth muscle cells forming clusters.

Histological Features

The histological features of uterine corpus epithelioid leiomyoma are distinct from those of typical spindle-shaped leiomyomas. The smooth muscle cells are round or polygonal with eosinophilic or clear cytoplasm, typically arranged in sheets, nests, or long cords. Mixtures of epithelioid, clear cells, and plexiform patterns occur frequently enough to suggest that they may be a variant of the same tumor.

Incidence

Epithelioid leiomyoma of the uterus is a rare variant, accounting for approximately 10% of all leiomyoma cases [1]. The exact incidence of this condition is difficult to determine due to few longitudinal studies.

Clinical Features

The clinical features of uterine corpus epithelioid leiomyoma are similar to those of typical leiomyomas. They can manifest with symptoms such as pain or uterine bleeding, and are often asymptomatic [4].

Classification

Uterine corpus epithelioid leiomyoma is classified as a morphologic variant of uterine corpus leiomyoma characterized by the presence of round or polygonal epithelioid smooth muscle cells forming clusters [5]. It is also referred to as a rare variant of leiomyoma composed of round or polygonal clear cells rather than typical spindle-shaped cells [15].

References

[1] - Leiomyomas are classified on the basis of their anatomic location and morphology, with epithelioid leiomyoma being a rare variant accounting for approximately 10% of all leiomyoma cases. [4] - Clinical features of uterine corpus epithelioid leiomyoma include symptoms such as pain or uterine bleeding, often asymptomatic. [5] - Uterine corpus epithelioid leiomyoma is classified as a morphologic variant of uterine corpus leiomyoma characterized by the presence of round or polygonal epithelioid smooth muscle cells forming clusters. [15] - Epithelioid leiomyoma of the uterus is a rare variant of leiomyoma composed of round or polygonal clear cells rather than typical spindle-shaped cells.

Symptoms and Signs of Epithelioid Leiomyoma of Uterine Corpus

Epithelioid Leiomyoma of Uterine Corpus is a rare type of tumor that can cause various symptoms, depending on its location, size, and whether it's single or multiple. While some women may not show any signs or symptoms at all

Based on the provided context, here are the diagnostic tests for uterine corpus epithelioid leiomyoma:

• Complete physical examination with thorough evaluation of medical history [3]
• Ultrasound scan of the pelvis: It is a noninvasive procedure that uses high frequency sound waves to produce real-time images [3]

Additionally, the following information can be inferred from the context:

• The diagnosis may also involve ruling out other conditions such as PEComa versus epithelioid leiomyoma (Genes Chromosomes Cancer 2020 Oct 24 [E

Treatment Options for Uterine Corpus Epithelioid Leiomyoma

Uterine corpus epithelioid leiomyoma is a rare and aggressive form of uterine cancer. While surgery is often the primary treatment approach, drug therapy may also be considered in certain cases.

• Mifepristone: This medication has been shown to be effective in reducing fibroid size and alleviating symptoms [14]. It works by blocking the action of progesterone, a hormone that can contribute to leiomyoma growth.
• Asoprisnil: Another selective progesterone receptor modulator, asoprisnil has also demonstrated efficacy in treating uterine leiomyomas [14].
• Ulipristal acetate: This medication has been found to be effective in reducing fibroid size and alleviating symptoms [12]. It works by blocking the action of progesterone.
• Epigallocatechin gallate (EGCG): A natural compound found in green tea, EGCG has been shown to have anti-cancer properties and may be effective in treating uterine leiomyomas [14].

Other Treatment Options

In addition to these medications, other treatment options for uterine corpus epithelioid leiomyoma may include:

• Surgery: This is often the primary treatment approach for uterine leiomyomas.
• Radiation therapy: May be used in combination with surgery or as a standalone treatment option.

Important Considerations

When considering drug treatment for uterine corpus epithelioid leiomyoma, it's essential to discuss the following factors with your healthcare provider:

• Size of the leiomyoma/fibroid
• Severity of symptoms
• Location of the fibroids
• Age and overall health

These factors will help determine the most effective treatment approach for your individual situation.

References:

[14] - Currently, mifepristone, asoprisnil, ulipristal acetate, and epigallocatechin gallate have been shown to be effective for fibroid regression and symptomatic improvement which are all in clinical trial.

The differential diagnosis for uterine corpus epithelioid leiomyoma involves a range of conditions that can present with similar histological features.

Key considerations:

• Leiomyosarcoma: This is a malignant smooth muscle tumor that can be difficult to distinguish from benign leiomyomas, especially in cases where unusual features are present [3].
• Epithelioid leiomyosarcoma: A rare and aggressive variant of leiomyosarcoma that can mimic epithelioid leiomyoma histologically [5].
• High-grade endometrial stromal sarcoma (ESS): This is a malignant tumor of the uterine corpus that can present with similar features to epithelioid leiomyoma, including hypercellularity and severe nuclear atypia [6].
• Uterine GIST: A rare type of gastrointestinal stromal tumor that can occur in the uterus and may be difficult to distinguish from epithelioid leiomyoma based on histology alone [5].

Other conditions to consider:

• Carcinomas: Malignant tumors of epithelial origin that can present with similar features to epithelioid leiomyoma, especially in cases where there is significant nuclear atypia and hypercellularity.
• PEComa: A rare type of tumor that can occur in the uterus and may be difficult to distinguish from epithelioid leiomyoma based on histology alone [7].
• Malignant melanoma: A rare type of cancer that can present with similar features to epithelioid leiomyoma, especially in cases where there is significant nuclear atypia and hypercellularity.
• Other sarcomas: Various types of malignant tumors that can occur in the uterus and may be difficult to distinguish from epithelioid leiomyoma based on histology alone.

Diagnostic approaches:

• Transcervical core needle biopsy: This is a reliable diagnostic test for distinguishing between uterine sarcoma and leiomyoma [8].
• Histological examination: A thorough examination of the tumor tissue is essential for making an accurate diagnosis.
• Immunohistochemical staining: This can be used to help distinguish between different types of tumors, especially in cases where there is significant overlap in histological features.

References:

[1] by A Setia · 2012 · Cited by 11 [3] Apr 1, 2008 [5] by T Kubo · 2017 · Cited by 7 [6] by S Sun · 2019 · Cited by 121 [7] Sep 17, 2021 [8] by N Kawamura · 2002 · Cited by 127