Lymphangioma is a developmental defect of lymphatics located in the deep dermal layers and characterized by a local eruption of clustered thin-walled vesicles(1). Although lymphangioma circumscriptum (LC) of the vulva is a benign disorder, it may cause distressing symptoms of itching, pain, oozing of serous fluid, tendency to infections, psychosexual dysfunction, and cosmetic disfigurement(2). It is believed that the congenital form of LC results from a hamartomatous malformation. On the other hand, the acquired form is the result of a treatment-related obstruction of lymphatics and generally occurs after pelvic lymphadenectomy(3,4). The lesion in this form of the disease is generally small and well circumscribed, while the congenital one may be large and have deep lymphatic cavernous involvement.
Treatment approaches are similar for both congenital and acquired vulvar LC ranging from conservative modalities such as CO2 and YAG laser ablation to surgical removal of the lesion, which is generally in the form of simple or radical vulvectomy(3,5–7).
Herein, we report a case with congenital vulvar LC who was definitively treated with radiotherapy after several unsuccessful surgical excisions. To the best of our knowledge, our patient is the first case in the gynecology literature successfully managed with radiotherapy.
A 37-year-old premenopausal female patient with persistent vulvar swelling and watery secretion was referred to our department after several failed treatments with a variety of surgical excisions. She had pink-colored vaginal discharge beginning from the age of 16. Her complaints progressed gradually, and she discovered swelling, erythema, and tenderness on her left labium minor and major at the age of 25. Her first labor was at the age of 17. She had a history of four labors with three successful deliveries and one abortion.
Her first visit to the gynecology clinic was at the age of 27 due to these complaints. Several punch and excisional biopsies were performed. The first histopathological examination revealed a squamous epithelial hyperplasia, whereas the following ones were reported as angiokeratoma and hemangioma folliculitis. Pelvic and transvaginal ultrasonography (US) and Pap smear tests were normal.
On February 2005, she applied to the gynecology clinic with continuous watery discharge precluding her social life. She could not even walk short distances without getting wet. Her gynecological examination revealed hemorrhagic vesicular papillary lesions located on both labium major and minor that mimic a genital wart. A dilatation of periuterine venous plexus was prominent in transvaginal ultrasound (US). Color Doppler US and angiography of pelvis showed no abnormal findings in the bilateral iliac artery and venous systems. Pelvic magnetic resonance imaging (MRI) demonstrated enlarged lymphatics adjacent to the uterus and along the parailiac regions, which were predominant on the left side. Local injection of iodinated contrast was performed at the site of lymphangioma to delineate the lymphatic pathway, and small lymphatics were identified as draining into a deep inguinal lymph node. Subsequently, this lymph node was marked by a mammography localization needle. Then, partial vulvectomy and deep inguinal lymph node excision were performed. The histopathological diagnosis was lymphangioma and lymphangiectasia. The levels of glucose, lactate dehydrogenase, and triglyceride in the lymphatic discharge from vagina and vulva were within normal limits.
A course of external radiotherapy was planned due to persistent complaints of our patient, which were unresponsive to several surgeries. Pelvic MRI (Fig. 1A, B) prior to radiotherapy showed that dilated lymphatics in the bilateral parailiac regions were more pronounced compared to previous MRI studies that indicated the failure of previous surgical procedures. A total dose of 20 Gy with conventional daily fractions encompassing the abnormal lymphatic vessels with a safety margin of 2 cm was administered between June 26 and July 04, 2006.
The patient had a major suppression of her complaints at the first follow-up visit which was 1 month after radiotherapy. The fluid leakage decreased to an extent that she could go outside and was able to walk without any fear of getting wet. Her physical examination revealed a decrease in the swelling and erythema. Pelvic MRI taken 3 months after radiotherapy showed a significant decrease in the extent of the parailiac, parauterine, and cervical dilated lymphatics (Fig. 1C, D) when compared to the MRI findings of preradiotherapy period.
At this time of reporting, the patient is free of symptoms with a follow-up period of 5 months after radiotherapy. She has gained her self-confidence and returned to normal social activities.
Vulvar lymphangioma is a very rare disease. Patients generally are treated with ablative therapies such as sclerotherapy, electrocoagulation, or surgical excision(6). The conservative treatments as CO2 laser, liquid nitrogen, or electrocoagulation have advantages of being outpatient procedure and repeatability. In a review of various treatment approaches, Vlastos et al.(6) reported that the recurrence rates after these conservative approaches are very high. In the same review, it has been postulated that the recurrence rate was around 23% after surgical excision alone. The extent of the disease at presentation often affects the extent of surgery in the treatment of vulvar LC. Surgical approaches vary from local excision to radical vulvectomy(2,7–10). Although radical vulvectomy seems to have the least recurrence rate(2), such a mutilating surgery seems to be unfair considering the benign nature of this disease.
Radiotherapy is generally being accused of a causative factor for the acquired form of vulvar LC when there is a prior history of radiation treatment due to pelvic malignancies(5,11,12). It has been proposed that the architectural disruption of previously normal channels led to a sequestration and further dilatation of previously normal lymphatics(4). On the contrary, the role of radiotherapy in the management of congenital lymphangioma has been shown in the thoracic and abdominal types with long-lasting results(13,14). The efficacy of radiotherapy in the other benign vascular malformations has also been demonstrated(15,16). The mechanism of the radiotherapy action is assumed to be a radiation-induced lymphatic endothelial proliferation and edema leading to the obstruction of dilated vessels(17). Our patient had congenital vulvar LC that had no response to the various surgical excisions and sclerosing therapies. However, a total dose of 20 Gy external radiotherapy led to a surprisingly good response, supporting a possible role of radiation therapy in the management of congenital lymphangiomas.
Radiotherapy may cause premature menopause due to the suppression of ovarian functions and very rarely secondary malignancies. In a study by Childhood Cancer Survivor Study Group(18), the risk of premature menopause in 2819 survivors of childhood cancer was 15%. The cumulative incidence approached 30% when they were treated with alkylating agents and abdominopelvic irradiation. In a corresponding study by Chiarelli et al.(19), abdominopelvic irradiation alone led to 23% risk of fertility deficit with the highest risk in patients who received ≥35 Gy. Boice et al.(20) studied the risk of second malignancies as a consequence of the treatment by radiation of carcinoma of the uterine cervix in the largest study of its kind. They reported that very high doses increased the risk of cancer of the bladder, rectum, vagina, possibly bone, uterine corpus, and cecum and of non-Hodgkin's lymphoma, whereas lower doses increased the risk of stomach cancer and leukemia. In this aspect, our case was treated with 20 Gy external radiotherapy, which has a risk of infertility and secondary malignancy. In the light of current evidences, longer follow-up period is certainly needed for the safety of this approach in the management of LC, and patients should be informed about these possible late consequences of radiation. On the other hand, all other conservative approaches failed, and it was mandatory for us to find an efficient nonmutilating treatment modality to give a chance to our patient for a better quality of life.
In conclusion, though a rare and benign disorder, congenital vulvar LC can cause exhaustive complaints and may progress to huge dimensions. Radiotherapy may be a good alternative to mutilating surgery. Besides, it may be applied to the patients with persistent or progressive lesions after surgery or any other ablative treatment modalities.
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