The predominant types of lymphangioma are lymphangioma circumscriptum (LC), cavernous
lymphangioma, cystic hygroma, and lymphangioendothelioma. These entities account for
approximately 26% of benign vascular tumors in children but are rarer in adults [1]. LC is the most common form of cutaneous lymphangioma and is characterized by superficial
lymphatic ducts protruding through the epidermis. Whimster postulated that lymph sacs
that are lined by muscle fibers contract and result in outpouching, which eventually
causes skin protrusions to become what are clinically described as vesicles [2]. This condition results in clusters of clear fluid-filled vesicles resembling frog
spawn and associated tissue edema. It is usually found on the proximal extremity,
trunk, and axilla, which has an abundant lymphatic system, but rarely involves the
vulva, penis, scrotum, gingiva, tongue, and even the conjunctiva. Vulvar LC is a debilitating
condition, causing lymphedema, itching, eczema, oozing, sexual dysfunction, and infection.
It is very rare and either a congenital or an acquired condition following damage
to previously normal lymphatics. Radical hysterectomy with subsequent radiotherapy
appears to be the only recently recognized acquired risk factor. Although there are
many other treatment modalities, surgery, particularly major labiectomy, has been
reported to be the best treatment option for the potential of early rehabilitation
and low recurrence. A 71-year-old female presented to our outpatient clinic with persistent
edema of both lower limbs, clusters of pink labial papules, pruritus, and watery lymph
oozing. Her condition had been deteriorating for 5 years without any treatment. These
cutaneous symptoms began with a few vesicles 24 years after radical hysterectomy with
adjuvant pelvic radiation therapy under the diagnosis of stage IIb squamous cell carcinoma
of the cervix ([Fig. 1]). There was no history of sexually transmitted disease or gastrointestinal disease.
Bilateral vulvar edema with vesicular skin eruption was found on colposcopy and a
cervical smear showed normal findings. Radioisotope lymphoscintigraphy showed a decreased
lymphatic uptake in the pelvis and the dermal back flow of lymphatics in both lower
legs, which implied lymphatic obstruction ([Fig. 2]). Preoperative biopsy revealed probable cavernous lymphangioma. Surgery was performed
under general anesthesia in the lithotomy position with a urinary catheter. We removed
all the papules by performing bilateral major labiectomy down to the level of Colles'
fascia, sparing the clitoris and fourchette ([Fig. 3]). The extent of the subcutaneous excision was determined by preoperative pelvic
computed tomography and intraoperative findings such as wetness, presence of cysts,
and fibrous components. After the excision, the wound was closed primarily. Surgical
biopsy showed multiple dilated lymph vessels, which is a histological feature of LC.
There was no endothelial swelling representing lymphangitis or a granulomatous reaction.
The postoperative course was uneventful. There was a clear improvement in the symptoms
such as oozing, itching, and rubbing by the time of follow-up at 6 months after surgery,
yet further follow-up is considered necessary ([Fig. 4]). Vulvar LC is an uncommon entity with various clinical mimics like genital warts,
herpes zoster, molluscum contagiosum, leiomyoma, and recurrent carcinoma. Further,
this makes the biopsy of the lesion essential to differentiate various infective diseases
and other tumors. Since the lesions may appear papillary upon histological analysis,
a specific lymphatic endothelial marker such as D2-40 is necessary to reduce the diagnostic
difficulty [3]. In our case, we used D2-40 stains as is and obtained positive findings ([Fig. 5]). When lymph nodes are affected, fluid formation exceeds the lymphatic transport
capacity, and this makes the affected region more prone to infection and the consequent
fibrosis. However, it is not known why most patients who have undergone regional node
dissection do not develop lymphedema. Although radical hysterectomy with or without
radiotherapy is believed to be the only recognized cause of LC, there are various
other reported conditions that lead to LC, such as Crohn's disease, urogenital tuberculosis,
bacterial infections, and non-gynecologic tumors. Thus, thorough patient history-taking
is essential to identify factors suggesting this rare condition and prevent delay
in treatment. Thus far, there is no definite treatment of choice for LC because the
number of reported LC cases is too small to conclusively determine the best treatment
modality. There are various non-surgical treatments ranging from conservative management
with massage, exercise, compression, and sclerotherapy, to CO2 laser therapy depending on the type and extent of LC. Recently, CO2 laser therapy has been used often as the main alternative to surgery. However, in
some cases, particularly in those with deep lesions, it caused pain and exacerbation
of vesicles and vulvar keloid formation [4]. Unlike conservative treatment options, surgical management offers a more permanent
solution. Although many other surgical treatment modalities, including the insertion
of polythene tubes, lymphovenous anastomosis, and lymph angioplasties have been reported,
labial reduction or excision of edematous tissue seems to show more promising results
since it can be repeated as necessary until both the functional and the aesthetic
goals are achieved. It is known that the size of the lesion also affects the surgical
outcome and the recurrence rate. Browse et al. [5] reported a high recurrence rate after radical excision when the initial lesion was
larger than 7 cm compared with a lesion less than 7 cm in size for which only a local
excision was performed. However, in our case, no recurrence was found despite the
fact that the lesion was approximately 9 cm×3 cm in size. We reported our experience
of major labiectomy as the surgical management of LC in one patient who had remission
with no recurrence. This might be just another case report of surgical resection of
LC, but because of a lack of cases, we believe that this report can contribute to
finding the best modality for the management of LC.
Fig. 1
A 71-year-old female with bilateral vulvar lymphangioma circumscriptum.
Fig. 2
Radioisotope lymphoscintigraphy showing dermal back flow (red arrows) of the lymphatics
in both legs.
Fig. 3
Operation on bilateral vulvar lymphangioma circumscriptum with major labiectomy.
Fig. 4
Follow-up at 6 months after major labiectomy; no recurrence or adverse effects.
Fig. 5
D2-40 staining marking lymphatic endothelial cells lining the dilated intraepidermal
lymphatic space (×200).
