In the vulvar region, a wide range of benign, premalignant, and malignant tumors can arise. In reproductive-age women, unilateral swelling of the vulva with a mass is commonly regarded as a Bartholin's cyst (1, 2). While uterine leiomyomas are quite common, vulvar leiomyomas, frequently misinterpreted as Bartholin's cysts, are very rare (3). They constitute approximately 0.03% of all gynecological neoplasms and 0.07% of all vulvar tumors (4). Although case reports or series have been reported in the literature, there is no specific guideline determining the management approach of vulvar leiomyoma.

Hemangioma, papilloma, mucosal polyp, and leiomyoma are among the types of vaginal tumors that are seldom encountered (5). Vaginal leiomyomas are benign mesenchymal tumors with a very low incidence. Diagnosis is generally confirmed postoperatively through histological examination of the mass. Such tumors usually arise from the anteriorivaginal wall and are therefore often misdiagnosed as cystocele (5). They can cause various clinical presentations, even leading to damage to adjacent organs (5, 6).

In this article our aim was to provide an overview of the diagnosis, treatment, and follow-up management of vulvar and vaginal leiomyomas through the presentation of two rare cases.

Vulvar leiomyomas are uncommon, benign, monoclonal growths that commonly occur in the fourth and fifth decades of life (7). These are typically asymptomatic benign mesenchymal tumors, rarely causing swelling and local discomfort. The majority of vulvar leiomyomas are clinically misdiagnosed as Bartholin cysts or abscesses at first (8). Unlike solid tumors, which usually present as painless masses that progressively enlarge, Bartholin's abscess appears as a painful lump that may fluctuate over time, accompanied by local inflammation and fever. On the other hand, the less painful Bartholin's cyst, is commonly linked to a background of recurring Bartholin's abscesses and might produce local discomfort. Histological evaluation is indispensable for diagnosis, particularly when clinical features hint at malignancy, such as accelerated growth (8–13).

Vaginal leiomyomas are frequently observed in women aged between 35 and 50 (14). Typically, they manifest as a solitary, well-defined mass originating from the medial anterior wall, as demonstrated in our case, and less commonly from the posterior and lateral walls (15). While often asymptomatic, they may present with chronic pelvic pain, lumbar discomfort, vaginal bleeding, dyspareunia, urinary symptoms such as pollakiuria and dysuria, or other indications of urinary obstruction, depending on their location of origin (16). Because the tumor can be mistaken for Skene's ductiabscess, urethrocele, cystocele, urethralidiverticulum, Gartner's ductxcysts, vaginalxcysts, or a malignant vaginalxtumor, a clinicalxdiagnosis of vaginal leiomyoma necessitates a high degree of skepticism (17, 18). Most of such tumors typically range in diameter from 3 to 4 cm. They are commonly solitary, benign, and characterized by slow growth. However, instances of sarcomatous transformation have been documented (19).

Various reports emphasize the importance of imaging modalities for evaluating vaginal or vulvar masses, as they can confirm the presence, location, and size of the tumor, as well as aid in its characterization. Due to its accessibility, cost-effectiveness, and non-invasive nature, the most commonly employed diagnostic technique is ultrasonography. Pelvic magnetic resonance imaging and pelvic computeditomography are employed less frequently and typically reserved for challenging occasions or in case of the presence of suspicion for malignancy or local spread. In our cases, we did not utilize any of the aforementioned imaging technologies. Given that the tumor was solitary, well-circumscribed, and lacked suspicious features upon clinical evaluation, a benign neoplasm was strongly suspected, rendering imaging investigations unnecessary. On the other hand, the role of various imaging methods in both positive and differential diagnosis of vaginal or vulvar tumors remains unclear. There is currently no consensus regarding the preferred method or defined criteria for a positive diagnosis (4, 8, 20).

Opinions regarding the risk of recurrence of vulvar or vaginal leiomyomas are contentious. Researchers advocate for long-term close monitoring due to the heightened risk of recurrence. The prevailing approach in the literature suggests that complete enucleation or excision of the tumor, along with the surrounding normal tissue, can mitigate the recurrence rate and improve the 5-year survival rate. However, owing to the limited number of instances and the scarcity of follow-upidata available, it is unknown how vulvar or vaginal leiomyomas will behave clinically over the long run (4, 20, 21).

Spindle, epithelioid, and myxoid or myxohyaline are the three primary histological patterns of vulvar or vaginal leiomyomas that have been found, while mixtures of these may also exist (4). Management is similar for all histological types. The current spindle pattern in our instances is a rather typical kind of leiomyoma, which is defined by a lot of eosinophilic cytoplasm and fascicular proliferation of spindle-shaped cells with elongated nuclei (4, 21). The histological differentiation between benign and malignant forms, such as leiomyoma, atypical leiomyoma, and leiomyosarcoma, primarily relies on a set of criteria described in the literature (4). Smooth muscle actin, desmin, and caldesmon are positive immunohistochemical markers of smooth muscle cells seen in both leiomyomas and leiomyosarcomas. But leiomyosarcomas also show immunopositivity for cytokeratin and S-100. Histological examination and immunohistochemistry can be used to distinguish leiomyoma from other mesenchymal tumors, such as aggressive angiomyxoma and cellular angiofibroma. In particular, the absence of extensive staining for smooth muscle markers, notably h-caldesmon can be used to make this distinction (22, 23). The role of estrogen, progesterone, and androgen receptors in the formation of these cancers is not well understood, but some of these tumors may express these receptors (24, 25).

In conclusion, it should be kept in mind that extrauterine leiomyomas or their malignant transformations may be present in the differential diagnosis of only conventional diagnoses in our clinical practice, such as cystocele and Bartholin cyst. Although we did not use preoperative imaging methods in our study, they can be used both in differential diagnosis and in cases of suspicion of malignancy. The primary treatment approach includes surgical excision followed by histopathological evaluation. We did not detect any recurrence in the short term in our study. We will continue to follow-up patients in the long term. There are currently no established guidelines determining their management. Additional data are needed to improve understanding of prognostic considerations, best practices for care, and diagnostic standards. In general, since clinical experience regarding the diagnosis and treatment of vulva or vaginal leiomyomas is limited, it is best to carry out diagnosis, treatment and follow-up in experienced multidisciplinary centers. We believe that multicenter studies conducted with a standardized patient management method will contribute more to the literature.

The datasets presented in this study can be found in online repositories. The names of the repository/repositories and accession number(s) can be found in the article/supplementary material.

Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. Written informed consent was obtained from the participant/patient(s) for the publication of this case report.

OY: Formal analysis, Methodology, Project administration, Resources, Validation, Visualization, Writing – original draft, Writing – review & editing. AK: Data curation, Writing – original draft. ZB: Data curation, Supervision, Writing – original draft. NA: Data curation, Writing – original draft. KM: Methodology, Writing – original draft. AA: Writing – original draft.