Initially described in 1895 by Klotz et al. [6], urethral hemangiomas are rare vascular anomalies that have historically demonstrated benign histopathological characteristics. To our knowledge, this represents the first documented case of urethral hemangioma coexisting with carcinoma in situ, which expands the recognized histopathological spectrum of this uncommon entity.

Hemangiomas of the urinary tract are uncommon, with the majority of cases located in the kidney, ureter, and bladder [7]. Urethral hemangiomas are considered to be congenital in origin, caused by the development of abnormal blood vessels from residual unipotent angioblastic cells in the embryo [8]. Some researchers have proposed a potential association with Klippel-Trenaunay syndrome [8, 9, 10]. Pathologically, urethral hemangiomas are characterized by vascular spaces lined by endothelium. They are histopathologically categorized into capillary or cavernous according to whether the vesselar spaces remain independent or anastomosing [12].

Carcinoma originating in the female urethra represents a rare malignancy, constituting less than 0.02% of all cancer cases diagnosed in the female population [13]. In a study, SC-CIS accounted for only 10.7% (6/56) of primary urethral tumors in women [14].In our case, based on the typical pathological changes of urethral hemangioma, SC-CIS was identified, suggesting shared molecular pathways between vascular proliferation and carcinogenesis. HIF-1α and VEGF are directly involved in the regulation of angiogenesis [15–16]. mTOR mediates VEGF-A expression via both HIF-1α-dependent and HIF-1α-independent mechanisms [17]. KRAS and TP53 mutations are associated with tumorigenesis, while mTOR is associated with KRAS and TP53 mutations. There may be a crosstalk between the VEGF/HIF-1α-driven angiogenesis pathway and tumorigenic KRAS/TP53 pathway. The PI3K-AKT-mTOR axis, activated in both processes, may represent the mechanistic link between urethral hemangioma and malignant transformation.

Urethral hemangiomas can occur at any age. Most cases have occurred in males, with only a few reported in females [9]. These vascular lesions may present as either focal or diffuse growth patterns, manifesting as solitary or multiple lesions [18]. The most common clinical manifestations include urethral bleeding, hematuria, and dysuria after ejaculation associated with blood clot discharge or hematospermia [12, 19]. In female patients predominantly exhibit a palpable urethral mass accompanied by contact bleeding [20]. Primary urethral tumors in female patients also present with overlapping clinical manifestations [14]. Our case exhibited classic diagnostic features, which demonstrated a urethral mass associated with recurrent bleeding episodes.

Current therapeutic strategies for urethral hemangiomas demonstrate significant practice variation. For these benign entities, the primary therapeutic goal is complete resection without compromising urinary function. Lesions of the outer urethra can be directly excised, while prostate hemangiomas and small isolated lesions are treated endoscopically by electrocoagulation, fulguration, or laser ablation (Nd: YAG, Argon, or KTP ) [9, 12, 21]. When hemangiomas are extensive, open resection and urethroplasty may be required. Large posterior urethral lesions can be excised through suprapubic cystostomy [22–23]. Other treatment options include topical and oral steroids, sclerotherapy, and cryoablation [18].

Although current data remain limited, local excision is recommended as the primary treatment for carcinoma in situ. In cases of residual disease following incomplete excision, postoperative brachytherapy sources should be applied [12–13]. In female patients, radiotherapy was applied at a median cumulative dose of 65 Gy (prescription range: 40–106 Gy). The 5-year local control rate reached 64%, with a 7-year cancer-specific survival (CSS) rate of 49% [24]. In our patient, the coexistence of SC-CIS necessitated adjunctive therapy per urethral malignancy protocols. The patient declined reoperation and underwent localized radiotherapy (50 Gy in 25 fractions), achieving sustained disease control.

We recommend that surgical excision should be the primary therapeutic modality for histologically confirmed urethral hemangiomas. Careful evaluation of resection margins for malignant cells is essential. If resection margins are positive for malignant cells, adjuvant radiotherapy should be considered to improve outcomes, as evidenced by our long-term follow-up data.