Vulvar Lymphangioma Circumscriptum Research Articles

Acquired lymphangioma circumscriptum of the vulva in a middle aged woman with chronic hidradenitis suppurativa – A case report

Lymphangioma circumscriptum (LC) is a distention of lymphatic channels present in skin and subcutaneous tissues. LC is either primary (usually present at birth or develops in early childhood) or secondary due to diminished lymphatic outflow. It is commonly prone to develop in oral cavity, tongue, proximal regions of arms and legs, groin, axilla, and trunk. Primary vulvar involvement is unusual. Vulvar LC is a rare untoward outcome of hidradenitis suppurativa (HS), which causes significant physical and emotional discomfort. We report a case of acquired LC of vulva as a sequlae of HS in a 42 years old female who presented with painful papules, edema and thickening of the vulva with history of foul-smelling discharging sinuses. The patient was managed surgically by wide excision and reconstructive surgery by Plastic surgery unit.

Malignancy-associated acquired vulvar lymphangioma circumscriptum: A clinicopathologic study of 71 cases.

Acquired lymphangioma circumscriptum of the vulva is rare and can occur subsequent to malignancies of the anogenital and pelvic region. We sought to investigate the clinicopathologic characteristics of malignancy-associated acquired vulvar lymphangioma circumscriptum (AVLC). We identified all cases of AVLC within our institution with history of prior malignancy between 2005 and 2021. A similar search was performed in the PubMed database to identify published cases to date. The clinical and histopathologic information was recorded. A total of 71 cases were identified. The most common preceding malignancy was cervical carcinoma (71.8%, 51/71). Radiation therapy was given to 91.4% (64/70) of the patients and lymph node dissection was made on 70.2% (40/57). Median interval between the diagnosis of malignancy and the AVLC was 10 years (range 0-32 years). AVLC frequently presented as vesicular (31.6%, 18/57) or verrucous (28.1%, 16/57) lesions clinically. Common treatments for AVLC included excision (53.1%, 26/49) and laser therapy (16.3%, 8/49), with an overall recurrence rate of 42.9% (24/56) at a median follow-up interval of 1.8 years (range 0.04-32.3 years). AVLC is a rare, late complication of anogenital and pelvic malignancies causing debilitating physical symptoms and psychological stress. Further studies are warranted to determine the most effective treatment modalities to mitigate recurrence.

Radyofrekans ablasyonla tedavi edilen vulvar lenfanjioma sirkumskriptum: Olgu sunumu

Lymphangioma circumscriptum (LC) is a rare benign skin disease involving hamartomatous lymphatic malformation of the deep dermal and subcutaneous lymphatic ducts. Upon occuring in uncommon areas such as axilla, shoulder, groin, and vulva, it is a therapeutic challenge for the dermatologist. Various methods such as surgical excision, lasers, and sclerotherapy have been used in the past to treat this unsightly skin condition. In this article we report the efficacy of a radiofrequency ablation in a female patient with vulvar LC. The treatment efficiency of radiofrequency was satisfactory in our patient without recurrence during the 6-month follow-up period. Based on previous studies, radiofrequency technique is a safe and cost-effective treatment for LC management, as in our case.

Acquired lymphangiectasia: a rare mimic of genital warts

Acquired lymphangiectasia of the vulva is very uncommon. Owing to the non-specific papillomatous manifestation and the vast array of possible differential diagnoses, lymphangioma circumscriptum (LC) still presents a diagnostic challenge. In this report, we present a very rare form of acquired vulvar LC in a 71-year-old patient with a longstanding history of asymptomatic lesions over the labia majora that had been previously treated as genital warts. On examination, the patient had multiple clustered translucent papules up to 15mm in diameter, morphologically reminiscent of vesicles, that oozed clear fluid throughout her groin and swollen labia majora. The patient also suffered concomitant bilateral lower-extremity lymphedema. A skin biopsy showed multiple, irregular-shaped lumina containing eosinophilic material in the upper dermis. Dilated lymphatic channels were lined by a single layer of flattened endothelial cells and the overlying epidermis showed acanthosis, hyperkeratosis, focal mild pseudoepitheliomatous hyperplasia. There is still no consensus on the optimal management of LC. Our patient was referred to a plastic surgeon for further evaluation and treatment. Although there are a variety of therapeutic modalities for LC, positive results are few and relapses are observed.

High-grade squamous intraepithelial lesion arising adjacent to vulvar lymphangioma circumscriptum: a tertiary institutional experience.

Lymphangioma circumscriptum of the vulva occurs in patients who have undergone radical hysterectomy, lymph node dissection, or radiation therapy for management of advanced uterine cancer. Since vulvar lymphangioma circumscriptum typically presents as multiple, grossly verrucous vesicles of various sizes, it may be impossible to clinically distinguish vulvar lymphangioma circumscriptum from other vulvoperineal cutaneous diseases. In the present study, 16 (1.6%) out of the 1,024 vulvar biopsy or excision specimens were diagnosed as lymphangioma circumscriptum. In two (12.5%) out of the 16 cases, unusual histopathological findings were observed. Both patients had previously undergone radical hysterectomy with lymph node dissection and postoperative radiation therapy or concurrent chemoradiation therapy for advanced cervical cancer. Microscopic examination revealed high-grade squamous intraepithelial lesions, which were located immediately adjacent to the normal squamous epithelium covering the dilated subepithelial lymphatic vessels. Further, human papillomavirus genotyping confirmed that both patients were infected with high-risk human papillomavirus. High-grade squamous intraepithelial lesion cannot be grossly distinguished from vulvar lymphangioma circumscriptum because the multiple, verrucous vesicles that constitute the characteristic gross appearance of vulvar lymphangioma circumscriptum hinder its distinction. In this regard, our cases of high-grade squamous intraepithelial lesion, located adjacent to vulvar lymphangioma circumscriptum, support the notion that active surgical excision is necessary for the treatment of vulvar lymphangioma circumscriptum.

Congenital Vulvar Lymphangioma (Lymphoedema) Circumscriptum in a Young Female

Lymphangioma circumscriptum (LC) is a form of lymphangioma characterized by benign dilation of lymphatic channels, which affects the skin and subcutaneous tissues. The most common sites are mucosa of mouth, tongue, groin, axilla, trunk and proximal region of extremities. Vulva is a rare site of LC. These abnormal lymphatic malformations do not communicate to the normal lymphatics. A female patient, aged 17 years, presented with papule-like condyloma of the labia majora. After simple vulvectomy was performed, symptoms improved. Histology revealed lymphangioma circumscriptum of the vulva. LC is a rare benign pathology that should be kept in mind in any vulval condylomatous lesion. Till date, only a few cases of LC of vulva have been reported in the literature. The treatment of primary vulvar lymphangioma circumscriptum is mainly surgery. Depending on the age of the patient and the extent of lesion, surgical treatment might be in the form of the local excision, labiectomy, or vulvectomy.

Acquired vulvar lymphangioma circumscriptum

Lymphangioma circumscriptum is a benign dilatation of lymph channels localized to the skin and subcutaneous tissues. This dermatological condition is generally found localized to the oral mucosa, tongue, proximal parts of arms and legs, groin, axilla, and trunk. Primary vulvar involvement is very rare. This report dwells on a 61-year-old female presenting with multiple itchy translucent warty papules on the left vulva. These lesions had been present for the past 3 years, and were gradually increasing in number and size. She had undergone a hysterectomy for uterine carcinoma 13 years ago, and had subsequently developed lymphedema of the left leg. Histopathologic examination of the biopsied vulvar papule demonstrated hyperkeratotic epidermis and dilatation of thin-walled lymph vessels in superficial dermal papillae. The physiopathological features helped clinch the diagnosis of vulvar lymphangioma circumscriptum.

Congenital vulvar Lymphangioma Circumscriptum: an Unusual Benign Pathology in Young Female

A female patient, aged 17 years, presented with papule-like condyloma of the labia majora. After major labiaectomy was performed, symptoms improved. Histology revealed lymphangioma circumscriptum of the vulva. Simple vulvectomy is an effective therapy for vulvar lymphangioma circumscriptum. It is a rare begning pathology that should be kept in mind in any vulval condylomatous lesion.

Clinical outcomes of primary surgical treatment for acquired vulvar lymphangioma circumscriptum

ObjectiveTo assess the clinical outcomes of surgical treatment for acquired vulvar lymphangioma circumscriptum in patients who received radical surgery and/or adjuvant radiation therapy for cervical cancer.MethodsA retrospective chart review of eight patients was performed to assess the demographic information, chief complaints, treatment modality for cervical cancer, location, and primary treatment modality for vulvar LC, postoperative changes in symptoms, and/or signs, the development of local recurrence and the outcome of patients.ResultsAll eight patients were previously diagnosed with cervical cancer FIGO clinical stage IA to IIA and received surgery, radiation therapy, or concurrent chemoradiation therapy. Microscopic examination revealed multiple, dilated, D2-40-positive dermal vascular channels containing eosinophilic proteinaceous material, consistent with LC. Most chief complaints showed considerable improvements on assessment at the outpatient clinic after the primary surgery. No patient showed aggravation of symptoms. Two patients developed local recurrences. One patient developed recurrence on the opposite side 13 months after local excision. We performed a second wide local excision. Another patient developed recurrence 47 months after the primary surgery. Since the lesion was very small and localized, we decided to manage it conservatively, but monitor it very closely. The remaining six patients remained free of recurrence.ConclusionIt is not easy for gynecologists to have an initial clinical diagnosis of LC, because there are a number of diseases that exhibit similar clinical manifestation to that of vulvar LC. Even if it is diagnosed correctly, local recurrence often occurs. Relevant symptoms associated with LC are not only distressing, but also affect patients’ quality of life. Based on our data, we propose that surgical treatment could provide a more long-lasting answer compared to other treatment modalities, since it is beneficial in terms of clinical outcomes. In the future, a long-term follow-up investigation is required to assess the prognosis and to compare the efficacy and side effects of each modality.

Acquired vulvar lymphangioma circumscriptum: Clinicopathologic study of 11 patients from a single institution and 67 from the literature

Acquired vulvar lymphangioma circumscriptum: Clinicopathologic study of 11 patients from a single institution and 67 from the literature

Acquired Vulvar Lymphangioma

Vulvar lymphangioma circumscriptum (LC) is an unusual benign condition, congenital or acquired, related to interference in the lymph drainage. Acquired cases are usually related to oncological surgical procedures. We report 6 cases of vulvar LC: 3 have been caused by surgery and radiotherapy for gynecologic cancer, whereas the other 3 have originated under benign conditions--Crohn disease, recurring episodes of cellulits, and lower limb lymphedema. In 3 of them, the initial clinical diagnosis was genital wart. Surgical exeresis was performed as the treatment of choice in 5 patients and there were 3 cases of recurrences. Diagnosis of this unusual condition is not always easy even after histopathologic examination. The possibility of vulvar LC should be taken into account as a possible diagnosis in patients with previous oncological surgery or genital warts refractory to the conventional treatment. In these cases, a biopsy of not only the epidermis but also the superficial dermis is recommendable to rule out inflammatory or tumoral processes. Knowledge on the features of this lesion can avoid unnecessary clinical and therapeutic procedures.

Surgical Resection of Acquired Vulvar Lymphangioma Circumscriptum

Surgical Resection of Acquired Vulvar Lymphangioma Circumscriptum

Acquired vulvar lymphangioma circumscriptum: Series of cases in three hospitals and literature review

Acquired vulvar lymphangioma circumscriptum: Series of cases in three hospitals and literature review

Acquired vulvar lymphangioma circumscriptum: a comparison of 12 cases with Crohn's associated lesions or radiation therapy induced tumors

Lymphangioma circumscriptum (LC) is a benign lesion of lymphatic origin. Vulvar involvement occurs in various clinical settings. We present 12 cases, and compare lesions in patients with Crohn's disease and those associated with pelvic radiation. The average age at presentation was 49 years. Thirty-three percent of the patients had Crohn's disease, 58% had radiation therapy and 9% had no significant medical history. Sixty-seven percent of the patients had multifocal lesions in anatomically distinct regions. Patients presented on average 16 years after onset of predisposing factors. Presenting complaints were pruritus, wetness and vulvar edema. Lesions were clinically heterogeneous, often found on the labia majora. Lesions consisted of dilated lymphatic channels at the junction of the reticular and papillary dermis. The cells lining these spaces lacked cytologic atypicality or mitotic activity. All lesions so examined were immunoreactive for D240. Patients were most often treated with surgical excision followed by laser ablation. Four of twelve patients, all with radiation-associated lesions, experienced disease progression necessitating additional surgery. Patients with LC secondary to radiation, when compared to those with Crohn's disease, were 10 years younger, more likely to have associated co-morbidities, and frequently experienced disease progression needing additional surgeries. Acquired vulvar LC has multiple causes with differing prognosis.

Vulvar Lymphangioma Circumscriptum

Vulvar lymphangioma circumscriptum is a rare, benign proliferation of the lymphatic system that presents a diagnostic and management challenge. It may be confused with condyloma acuminata, molluscum contagiosum, or other vulvar disorders. Treatment options include observation, surgical excision, laser ablation, or sclerosing therapy. We report 3 cases, 2 associated with vulvar squamous cell carcinoma and 1 with hidradenitis suppurativa.

Lymphangioma Circumscriptum of the Vulva: A Case Report

This article does not have an abstract.

Radiotherapy in congenital vulvar lymphangioma circumscriptum

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.

Major labiaectomy as surgical management of vulvar lymphangioma circumscriptum: three cases and a review of the literature

Vulvar lymphangioma circumscriptum (LC) is a developmental defect of lymphatics in deep dermal and subcutaneous layers. The entity may be acquired that induced by surgical intervention and radiation therapy for malignancy or congenital. Lymphangiomas of the vulva are rare. Thirteen cases of the congenital and 24 cases of acquired form have been reported in the literature presently. To evaluate major labiaectomy as surgical management of vulval lymphangioma, we report our experiences on surgical treatment of three cases of this rare disease from Gynecologic Oncology Department of Vali-E-Asr Hospital. Three female patients, aged 18, 17 and 63 years, presented with persistent edema, papules, and vesicles of the labia major, among which two first cases had congenital LC and another case had developed 9 years after radical hysterectomy, lymph node dissection, and adjuvant radiotherapy of cervical cancer. Overall there was a clear improvement in the symptoms and signs associated with these conditions in all patients. In particular, all patients reported an improvement in the amount of edema following surgery. In particular, major labiaectomy seems to be more successful than methods such as lymphovenous anastomoses and lymph angioplasties. A single operation may prove beneficial; also, this approach has the potential to allow patients to rehabilitate to normal life and activity.

Surgical Management of Primary Vulvar Lymphangioma Circumscriptum and Postradiation: Case Series and Review of Literature

Vulvar lymphangioma circumscriptum is a defect of lymphatics in the reticular dermis. It can occur as either acquired—induced by radical hysterectomy and pelvic radiation for cervical cancer–or congenital. Lymphangiomas of the vulva are rare. Thirteen cases of congenital and 24 cases of the acquired form have been reported in the literature. To describe major labiaectomy as surgical management of primary vulval lymphangioma and postradiation, experiences about surgical treatment of 3 cases of this rare disease are reported. Three female patients (aged 17, 18, and 63 years) had persistent edema, papules, and vesicles of the labia major. Two patients had congenital lymphangioma circumscriptum. Another patient developed it 9 years after radical hysterectomy, lymph node dissection, and adjuvant pelvic radiation of cervical cancer. Qualitative analysis of signs and symptoms that occurred before and after surgery was performed. Clear improvement in symptoms and signs associated with these conditions occurred in all patients postoperatively. In particular, all patients reported reduction in the amount of edema after surgery. Our data suggest major labiaectomy is more successful than methods such as lymphovenous anastomoses, lymph angioplasties, and laser therapy. A single operation may provide benefit for a long period.

Giant Vulvar Lymphangioma Circumscriptum Mimicking Vulvar Tumor

Lymphangioma circumscriptum (LC) is a benign dilation of lymph channels localized to the skin and subcutaneous tissues. Primary vulvar involvement is very rare, with only 34 cases reported. A 17-year-old girl presented with pain and an 18-cm mass of the right labium majus that started 4 years earlier. She underwent wide local excision, and histological examination showed multiple dilated vascular channels in papillary dermis with a diagnosis of vulvar LC. Vulvar lymphangioma may mimic other vulvar tumors. Excision may be the best treatment of extensive vulvar LC.