Skip Navigation LinksHome > May/June 2008 - Volume 18 - Issue 3 > Radiotherapy in congenital vulvar lymphangioma circumscriptu...
International Journal of Gynecological Cancer:
Correspondence and Brief Report

Radiotherapy in congenital vulvar lymphangioma circumscriptum


Free Access
Article Outline
Collapse Box

Author Information

Departments of *Radiation Oncology, †Radiology, ‡Pathology, and §Gynecologic Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey

Address correspondence and reprint requests to: Ferah Yildiz, MD, Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, 06100 Ankara, Turkey. Email:

Accepted for publication June 7, 2007

Collapse Box


Congenital lymphangioma circumscriptum (LC) of the vulva is a rare disorder with unknown etiology. Treatment options include ablative approaches such as laser therapy, sclerotherapy, and surgery. Radiotherapy has been shown to be effective in the management of congenital lymphangioma especially in the thoracic and abdominal lesions. In this report, we describe a patient with persistent vulvar LC despite sclerosing therapy and several surgical excisions. She was treated with a course of external radiotherapy and showed a dramatic objective response with relief of all symptoms

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.

Back to Top | Article Outline

Case report

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.

Figure 1
Figure 1
Image Tools

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.

Back to Top | Article Outline


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.

Back to Top | Article Outline


1 Peachey RD, Lim CC, Whimster IW. Lymphangioma of skin; a review of 65 cases. Br J Dermatol 1970;83:519–27.

2 Roy KK, Agarwall R, Kumar S. Recurrent vulval lymphangioma circumscriptum—a case report and literature review. Int J Gynecol Cancer 2006;16:884–943.

3 Tulasi NR, John A, Chauhan I et al. Lymphangioma circumscriptum. Int J Gynecol Cancer 2004;14:564–6.

4 Whimster IW. The pathology of lymphangioma circumscriptum. Br J Dermatol 1976;94:473–86.

5 Ochsendorf FR, Kaufmann R, Runne U. YAG laser ablation of acquired vulval lymphangioma. Br J Dermatol 2001;144:442–4.

6 Vlastos AT, Malpica A, Follen M. Lymphangioma circumscriptum of the vulva; a review of literature. Obst Gynecol 2003;101:946–54.

7 Horn LC, Kühndel K, Pawlowitsch T et al. Acquired lymphangioma circumscriptum of the vulva mimicking genital warts. Eur J Obstet Gynecol Reprod Biol 2005;123:117–24.

8 Harwood CA, Mortimer PS. Acquired vulval lymphangiomata mimicking genital warts. Br J Dermatol 1993;129:334–6.

9 Sah SP, Yadav R, Rani S. Lymphangioma circumscriptum of the vulva mimicking genital wart: a case report and review of the literature. J Obstet Gynaecol Res 2001;27:293–6.

10 Browse NL, Whimster I, Stewart G, Helm CW, Wood JJ. Surgical management of lymphangioma circumscriptum. Br J Surg 1986;73:585–8.

11 Schwab RA, McCollough ML. Acquired vulvar lymphangiomas: a sequel of radiation therapy. Cutis 2001;67:239–40.

12 Jappe U, Zimmermann T, Kahle B. Lymphangioma circumscriptum of the vulva following surgical and radiological therapy of cervical cancer. Sex Trans Dis 2002;29:533–5.

13 Kandil A, Rostom AY, Mourad WA et al. Successful control of extensive thoracic lymphangiomatosis by irradiation. Clin Oncol 1997;9:407–11.

14 Aristizabal SA, Runyon TD. Radiotherapy of unusual benign disease. Int J Radiat Oncol Biol Phys 1981;7:1437–40.

15 Yildiz F, Yilmaz M, Cengiz M et al. Radiotherapy in the management of Klippel-Trenaunay-Weber syndrome: report of two cases. Ann Vasc Surg 2005;19:566–71.

16 Atahan IL, Cengiz M, Ozyar E, Gurkaynak M. Radiotherapy in the management of Kasabach-Merritt syndrome: a case report. Pediatr Hematol Oncol 2001;18:471–6.

17 Johnson DW, Kalazynski PT, Gordon WH. Mediastinal lymphangioma and chylothorax. The role of radiotherapy. Ann Thorac Surg 1986;41:325–38.

18 Sklar CA, Mertens AC, Mitby P et al. Premature menopause in survivors of childhood cancer: a report from childhood cancer survivor study. J Natl Cancer Inst 2006;98:890–6.

19 Chiarelli AM, Marrett LD, Darlington G. Early menopause and infertility in females after treatment from childhood cancer diagnosed in 1964–1988 in Ontario, Canada. Am J Epidemiol 1999;150:245–54.

20 Boice JD, Day NE, Anderson A et al. Second cancers following radiation treatment for cervical cancer: an international collaboration among cancer registries. J Natl Cancer Inst 1985;74:955–75.

Cited By:

This article has been cited 4 time(s).

Turkiye Klinikleri Tip Bilimleri Dergisi
Lymphangioma Circumscriptum of the Vulva: Case Report
Yasar, SP; Dogan, Y; Serdar, ZA; Gunes, P
Turkiye Klinikleri Tip Bilimleri Dergisi, 32(5): 1396-1400.
Journal of Cutaneous Pathology
Acquired vulvar lymphangioma circumscriptum: a comparison of 12 cases with Crohn's associated lesions or radiation therapy induced tumors
Papalas, JA; Robboy, SJ; Burchette, JL; Foo, WC; Selim, MA
Journal of Cutaneous Pathology, 37(9): 958-965.
Indian Pediatrics
Congenital Lymphangioma Circumscriptum of the Vulva
Aggarwal, K; Gupta, S; Jain, VK; Marwah, N
Indian Pediatrics, 46(5): 428-429.

International Journal of Dermatology
Cutaneous lymphangioma circumscriptum: frog spawn on the skin
Patel, GA; Schwartz, RA
International Journal of Dermatology, 48(): 1290-1295.

Back to Top | Article Outline

lymphangiomatosis; radiotherapy; vulva

© 2008 by the International Gynecologic Cancer Society and the European Society of Gynaecological Oncology.


Article Tools



Article Level Metrics

Search for Similar Articles
You may search for similar articles that contain these same keywords or you may modify the keyword list to augment your search.

Connect With Us


For additional oncology content, visit LWW Oncology Journals on Facebook.