Cervical angioleiomyoma: A rare tumor behind persistent menorrhagia and pelvic pain

Epidemiology

Angioleiomyomas are rare benign tumors of mesenchymal origin. While leiomyomas are the most common benign uterine tumors, their angioleiomyomatous variant is much less frequently observed in the uterus, cervix, broad ligament and any other part of the reproductive tract [2, 3]. About 0.3–0.4% of leiomyomas are angioleiomyomas [4]. The occurrence of angioleiomyoma in the female genital tract is exceedingly rare, with published reports limited to 15 cases in the uterine corpus and 6 cases in the cervix [5, 6]. According to World Health Organization classification of soft tissue tumors in 2020, angioleiomyomas belong to the group of perivascular tumors [7]. Pathogenesis of angioleiomyomas is not clear. Angioleiomyomas arise from perivascular smooth muscle cells possibly due to some endothelial injuries or venous obstruction or due to sex hormone stimulation while leiomyomas arise from myometrial smooth muscle cells [7, 8].

Clinical presentation

Angioleiomyomas present in the reproductive age group. In most cases, angioleiomyomas in the extremities present as painful, subcutaneous masses. In contrast, cervical angioleiomyomas, though extremely rare, can manifest with symptoms similar to other cervical or uterine pathology like heavy menstrual bleeding, pelvic pain, dysmenorrhea and pressure symptoms. There are case reports of severe haemorrhage and disseminated intravascular coagulation in uterine angioleiomyomas [9]. Presentation like pseudo- Meig’s syndrome, coagulopathy and even rupture of uterus have also been reported [9–11]. Pain is mainly due to ischemia or vascular obstruction while abnormal bleeding is due to dysregulated angiogenesis and increase in growth receptors.

Histopathologically these tumors are of cavernous, venous or solid subtypes. Solid types have abundant smooth muscle bundles compactly surrounding the vascular channels. Cavernous type tumors have dilated vascular channels with scanty smooth muscle tissue component. Venous types have vascular channels with thick walls and less muscle tissue. These tumors may undergo hyaline or myxoid degeneration [2]. Malignant transformation of these tumors is extremely rare [12].

In the index case, the patient’s primary symptoms were chronic heavy menstrual bleeding and intermittent pelvic pain, both of which are common presentations for many gynecological disorders such as fibroids, adenomyosis, and endometriosis. This can make diagnosis challenging, as these symptoms are nonspecific and common in various benign conditions of the reproductive tract. Reproductive age, chronic heavy menstrual bleeding, pelvic pain, and the tumor’s vascular predominance predisposed the index patient to anaemia and hemorrhagic complications.

Diagnostic imaging

Ultrasound is typically the first-line imaging modality for evaluating abnormal bleeding and pelvic masses. Angioleiomyomas typically appear as hypoechoic masses on ultrasound, similar to leiomyomas. However, MRI is more definitive, as the vascular component of these tumors can often be highlighted with hyper intensity on T2-weighted images.

Histopathology

Histopathologically, angioleiomyomas are characterized by interlacing bundles of smooth muscle cells with numerous thick-walled blood vessels. The mass is usually well circumscribed or encapsulated in nature. There is no significant hypercellularity, nuclear atypia, increased mitosis or necrosis noted in this tumor.

Immunohistochemistry (IHC) often shows positivity for SMA, desmin, and sometimes vimentin and h-caldesmon, confirming smooth muscle origin [8].

Differential diagnoses for such a tumor include angiomyolipoma, endodermal stromal tumor, low grade perivascular epithelioid cell tumor and other vascular tumors. Histopathological study and IHC helps to differentiate these tumors.

Treatment

The definitive treatment for cervical angioleiomyomas is surgical excision, either through hysterectomy or localized excision, depending on the size and location of the tumor and the patient’s reproductive preferences. In our case, complete excision via a vaginal approach successfully resolved the patient’s symptoms.

Prognosis

The prognosis for cervical angioleiomyomas is excellent, with complete resection being curative. Recurrence is extremely rare, and there have been no reports of malignant transformation in these tumors [12]. Periodic clinical and imaging surveillance is recommended to confirm complete excision, monitor hematological recovery, and assess fertility outcomes.

Clinical implication

This case highlights the critical importance of considering angioleiomyoma in the differential diagnosis of cervical masses, especially in reproductive – age women presenting with chronic heavy menstrual bleeding and pelvic pain. Although rare, angioleiomyoma can mimic common gynecological conditions like fibroids or adenomyosis, leading to potential misdiagnosis and delayed treatment. In this patient, advanced imaging with MRI revealed a hyperintense lesion suggestive of vascularity, prompting surgical excision and histological confirmation of angioleiomyoma. The tumor’s prominent vascular component explained the patient’s anaemia and heavy bleeding, and its benign nature allowed for conservative management with complete symptom resolution. IHC played a pivotal role in distinguishing this tumor from other vascular tumors or stromal neoplasms, reinforcing the need for thorough in distinguishing this tumor from other vascular or stromal neoplasms, reinforcing the need for thorough pathological evaluation. Clinicians should be aware that while angioleiomyomas are uncommon, their inclusion in diagnostic considerations can guide appropriate imaging, prevent complications, and support fertility-preserving treatment strategies.