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Abstract: Lymphangioma circumscriptum (LC) is a rare benign condition, with marked dilation of surface lymphatic vessels in the deep and subcutaneous layers. Vulvar LC can become a highly disabling condition with vulvar discomfort, itching, burning and lymph seeping being the dominant symptoms. Biopsy is mandatory for the diagnosis. There is no consensus on the standard treatment for vulvar LC and recurrence is frequent. In complex cases with wide disease location, combination of different treatment options, such as abrasive methods and surgery, may lead to the best clinical and aesthetical result, with extended disease-free periods. We present a patient with a long history of Crohn disease with multiple pelvic surgeries who developed an extensive vulvar LC., Competing Interests: The authors have declared they have no conflicts of interest., (Copyright © 2023, ASCCP.)

Background: 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)., Methods: 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., Results: 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)., Conclusion: 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., (© 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

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.

Competing Interests: Competing interests: None declared.

Lymphangioma circumscriptum is a benign hamartomatous malformation involving the lymphatic system of skin and subcutaneous tissue. It can involve any part of the body with maximum predilection for proximal parts of limbs. Vulvar involvement is quite infrequent. Based on the aetiopathogenesis, it can be either primary or secondary, with the secondary form being relatively more common in vulva. We report an exceedingly rare case of primary lymphangioma circumscriptum in a post-menopausal female presenting with multiple warty, papulo-nodular lesions on both labia majora. The patient was diagnosed histopathologically and managed surgically by wide local excision with primary closure., (© 2019 The Australasian College of Dermatologists.)

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., Competing Interests: The authors declare that they have no conflicts of interest.

Objective: To 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., Methods: A 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., Results: All 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., Conclusion: It 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.

Objective: To describe the clinicopathological spectrum of Lymphangioma Circumscriptum (LC)., Study Design: Observational case series., Place and Duration of Study: Department of Pathology and Microbiology, AKUH, Karachi, from 2002 to 2012., Methodology: All reported cases of LC were retrieved from medical record. Clinical and pathological features were noted. Frequency percentages were determined., Results: There were 29 cases of LC predominantly males (62%). The mean age was 27.17 ±15.5 years. The commonest sites was anal/perianal region (24%) followed by extremities (17%) and tongue, (14%). Vulval LC was seen in 3 patients. Two cases were described on scrotum. The lesions were most commonly suspected as viral warts, mole or polyp (in anal region). Vesicles with erosions and bleeding and localized growth were the usual clinical presentations. Four of the patients presented with swelling since birth. All were treated with surgical excision. Microscopic examination revealed acanthotic squamous epithelium with papillomatosis. The subepithelial region had collections of lymphatic channels composed of ectatic dilated vessels with serum and inflammatory cells in their lumina. The lymphatic channels were seen in deeper layers along with lymphocytic aggregates., Conclusion: Lymphangioma circumscriptum is a malformation of abnormal lymphatic channels with feeding cisterns in subcutaneous tissue. It is a benign lesion usually occurring in anal/perianal region and confused with warts. Surgical excision is preferred mode of treatment.

Objective: To add a new case of primary vulvar lymphangioma circumscriptum to the literature and to review the current literature., Materials and Methods: We searched the PubMed/MEDLINE databases for previous case reports using the key words "vulvar lymphangioma circumscriptum" and "primary vulvar lymphangioma circumscriptum.", Results: We found 21 cases of primary vulvar lymphangioma circumscriptum reported until 2013. Mean age was 32 years (range = 3-76 y), and 62 % of the cases had been treated surgically., Conclusions: 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.

Lymphangioma circumscriptum (LC) results from the development of abnormal lymphatic vasculature and is characterized by the presence of grouped vesicles filled with clear or colored fluid. Vulvar localization is uncommon. Abnormalities of the lymphatic system, such as lymphedema and cystic hygroma, are well-known features of genetic disorders such as Noonan syndrome (NS) and Turner syndrome. We report the case of a patient with NS who presented with LC of the vulva. We also discuss the expanding spectrum of clinical anomalies associated with the presentation of NS.

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.

Objective: To evaluate the efficacy of major labiaectomy as a surgical management of vulvar lymphangioma circumscriptum, we report two cases of this rare clinical entity., Case Reports: Two female patients, aged 56 and 68 years, presented with persistent edema of the lower limbs, papule-like condyloma of the labia majora, and lymph oozing from these papules of the vulva, which had developed 24 and 10 years, respectively, after radical hysterectomy with adjuvant pelvic radiation therapy for cervical cancer. After major labiaectomy was performed, symptoms in the first case, of extensive resected skin margin, improved clearly, in the second case, vulvar lymphangioma circumscriptum was more severe than in the first case, and a small amount of lymph oozing occurred from residual papules of the labia majora. In both cases, histology revealed lymphangioma circumscriptum of the vulva., Conclusion: Major labiaectomy is an effective therapy for vulvar lymphangioma circumscriptum. Particularly, in the case which was extensive and deep resected skin margin, symptoms such as papules of the labia majora and lymph oozing from these papules of the vulva associated with lymphangioma seemed to be clearly improved.

The authors report a case of acquired lymphangioma circumscriptum (LC), localised in the right labium majus of vulva and characterised by periodic symptoms for 8 years; however, 3 months after the right major labiectomy, the same symptoms developed in both the left labium majus and the right labium minus. To the best of our knowledge, this is the first case of acquired LC with this type of localisation and symptoms without any known aetiologic factor.

Background: Lymphangioma circumscriptum, a rare, benign disease that can be either congenital or acquired, involves the deep dermis and subcutaneous lymphatics., Objective: This study aims to analyse the efficacy of surgical resection of vulva lymphangioma circumscriptum (VLC)., Materials and Methods: Between January 2000 and December 2008, eight consecutive women referred to our centre and treated surgically for VLC were included in the study. VLC was responsible for recurrent lymph oozing in seven cases. All women were treated by the same plastic surgeon specialising in lymphatic diseases., Results: The first surgery was performed after a median interval of 5.4 years since VLC onset. The first cutaneous resection included the labia majora of all women and labia minora of five and clitoral hood of four. Five women experienced rapidly recurrent vesicles associated with lymph oozing and underwent resection again (once: two women, twice: three women). The second resection was performed 4-6 months after the first, whereas the third took place 1-6 years after the second. Five women had moderate and transitory post-surgical lymph oozing. After a median follow-up of 53 months after the last surgery, seven of the eight women were free from symptom., Conclusion: Surgical resection is an effective and well-tolerated therapy for VLC in most women. Lesion recurrence is frequent but resection can be repeated several times with no adverse effects., (Copyright © 2009 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Background: Lymphangioma circumscriptum (LC) is a benign lesion of lymphatic origin. Vulvar involvement occurs in various clinical settings., Methods: We present 12 cases, and compare lesions in patients with Crohn's disease and those associated with pelvic radiation., Results: 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., Conclusions: 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 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.

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.

Background: 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., Case: 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., Conclusion: Vulvar lymphangioma may mimic other vulvar tumors. Excision may be the best treatment of extensive vulvar LC.

Lymphangioma circumscriptum (LC) is a defect of lymphatics in deep dermis. This abnormal saccular dilatation of lymphatics occurs in dermal papillae visible as vesicles clinically. The entity may be acquired or congenital. The occurrence of congenital variety of LC in vulva is extremely rare, with only few cases reported in the literature. We communicate one such case presenting as recurrent vulval growth along with a review of literature and treatment modalities.

Genital warts can mimic a variety of conditions. We report a middle-aged pregnant woman with lymphangiectatic lesions on the vulva resembling warts.

Background: Lymphangioma is a rare cause of a vulvar mass. Two types of lympangioma involving the vulva have been described: lymphangioma circumscriptum and cavernous lymphangioma. Cavernous lymphangioma has been reported much less frequently than lymphangioma circumscriptum in association with the vulva., Case: A cavernous lymphangioma was diagnosed in a 19-year-old nullipara who had a history of a gradually expanding vulvar mass. The mass was removed surgically, with no postoperative complications or recurrences to date., Conclusion: Cavernous lymphangioma should be considered in the differential diagnosis of a vulvar mass and can be treated successfully with surgical excision.

Background: Lymphangioma circumscriptum of the vulva rarely develops after postoperative pelvic irradiation., Goal: The goal was to describe two cases of lymphangioma circumscriptum and their treatment and present a brief review of the literature., Study: Two female patients, aged 75 years and 46 years, presented with persistent edema, papules, and vesicles of the labia majora, which had developed 15 and 9 years after hysterectomy, lymph node dissection, and subsequent irradiation of cervical cancer. The external diagnosis was genital warts., Results: In both cases histology revealed lymphangioma circumscriptum of the vulva. Whereas the older woman's condition responded well to laser treatment, keloids developed in the second patient at the site of carbon dioxide laser vaporization., Conclusion: CO2 laser treatment recently has been recommended for vulvar lymphangioma circumscriptum and is effective in vaporizing the communicating vessels to deeper cisterns. To our knowledge this is the first description of keloid development after laser therapy for vulvar lymphangioma circumscriptum, and such an effect should be considered before CO2 laser surgery is applied for this particular entity.

Lymphangioma circumscriptum (LC) is an uncommon dermatologic problem that rarely affects the vulva and it is considered to be a localised developmental defect of lymphatic tissue in the dermis. We report a case of vulval LC, clinically diagnosed as genital wart, in a 48-year-old woman without evidence of secondary lymphatic damage. The patient required extensive vulval surgery and there was no recurrence after 16 months.

Lymphangioma circumscriptum is a benign disease of the lymph ducts and an unusual pathologic process that rarely affects the vulva. The etiology of this lesion is not clear, but obstruction of the lymph vessels has been suggested as a possible cause in some cases. We report the case of a 44-year-old woman with lesions similar to lymphangioma circumscriptum of the vulva and chronic idiopathic lymphedema of the lower right limb. Because there was no obvious cause, we propose that the lymphangioma was caused by the lymphedema.

Lymphangiomas of the vulva are rare clinical entities. Acquired or secondary lymphangiomas have characteristically been reported after radiation therapy for cervical carcinoma and appear on the vulva years after this treatment. Local surgery, scrofuloderma, and Crohn's disease may also damage vulvar lymphatic flow and lead to the development of vulvar lymphangiomas. We report a case of acquired vulvar lymphangiomas that occurred in a patient 15 years after she received radiation therapy for squamous cell carcinoma of the uterine cervix.

A 44-year-old female developed confluent, dusky red, pruritic labial papules clinically suspected to be genital warts. She had a long-standing history of Crohn's disease with vulvar fistulae. The papular eruption developed after several bouts of cellulitis in a region of vulvar lymphedema. Shave biopsy of a papule exhibited papillated epidermal hyperplasia overlying a dermis with a 'Swiss-cheese' appearance secondary to lymphedema and superficial ectatic thin-walled vascular spaces characteristic of lymphangiectasias. Review of published cases reveals that acquired lymphangiomas often affect the vulva compared to other cutaneous sites and can be associated with surgery, radiation therapy, infection (e.g., erysipelas, tuberculosis), Crohn's disease, congenital dysplastic angiopathy and congenital lymphedema. Rather than translucent vesicles ('frog spawn') typical of extragenital cutaneous lymphangiomas, vulvar lymphangiomas often present as verrucous papules that can be mistaken for genital warts. In this case, we believe that the combination of vulvar Crohn's disease and recurrent cellulitis resulted in local lymphatic destruction, lymphedema and ultimately symptomatic lymphangiectasias that mimicked genital warts.

The lymphangioma of the vulva offers difficulties in differential diagnosis because of its wart-like appearance [7]. Is the diagnosis histologically confirmed, there often arise uncertainties about an adequate therapy [7, 2, 15]. By this case report these problems will be explained. Vaporisation with the CO2-Laser showed to be the predominant therapeutical procedure.

A 46-year-old woman developed multiple papules on the right labium majus. A histological examination revealed acanthotic epidermis and dilated vessels in the papillary dermis. The clinical and histological features were compatible with those of lymphangiectasis or acquired lymphangioma of the vulva, which occurs after surgery or irradiation for cervical cancer. This patient, however, had no such past history. Acquired lymphangioma of the vulva arising without obvious causes seems to be unusual.