Acquired Lymphangioma Circumscriptum Research Articles

High-grade Undifferentiated Pleomorphic Sarcoma Presenting as Acquired Lymphangioma Circumscriptum

A previously healthy woman in her 40s presented to the dermatology clinic with a 1-year history of a tender, draining, enlarging mass on the left buttock. Physical examination revealed an extensively indurated hyperpigmented nodule with numerous overlying clustered exophytic verrucous papules on the L medial buttock (see figure 1). Initial skin biopsy of verrucous lesions was notable for lymphangioma with overlying condyloma, D2-40 positive. MRI abdomen and pelvis with contrast demonstrated a left gluteal enhancing subcutaneous soft tissue mass, 10.3 x 8.2 x 8.8 cm in size, overlying and involving the left gluteus maximus, along with satellite nodules present in the medial gluteus maximus and paraspinal muscles, with bilateral pelvic and inguinal adenopathy. Excisional deep tissue biopsy revealed a diagnosis of a high-grade, multifocal spindle and undifferentiated pleomorphic sarcoma. The patient subsequently underwent 4 cycles of neoadjuvant chemotherapy AIM (doxorubicin, iphosphamide, MESNA), followed by radical resection of the left gluteal region, with positive margins, followed by repeat surgical resection and reconstruction with left-sided latissimus dorsi myocutaneous free flap. The patient has now completed radiation therapy.
 Soft tissue sarcoma is a heterogeneous group of rare malignancies of mesenchymal origin, that represents 0.8% of all adult cancers. Prognostic factors associated with poor outcome include high histologic grade, large tumor size, presence of metastases, and unresectability1. Herein, we report a novel case of high-grade soft-tissue undifferentiated pleomorphic sarcoma, masquerading as acquired lymphangioma circumscriptum (ALC), as noted on initial skin biopsy of grouped flesh-colored verrucous papules overlying an indurated tender mass. ALC is a lymphatic malformation occurring as sequela to any process which interrupts previously normal lymphatic drainage. As such, subcutaneous lymphatic cisterns are unable to drain completely into the general lymphatic system, resulting in lymphatic dilation and subsequent development of vesicular and papular lesions on the trunk, extremities, and anogenital areas.2 ALC can mimic common infectious growths including molluscum contagiosum and condyloma accuminata, and accordingly be mismanaged. Etiologic causes of ALC including malignancy, and has previously been reported in the setting of cervical carcinoma, vulvar carcinoma, endometrial carcinoma, Hodgkin lymphoma, rhabdomyosarcoma, rectal carcinoma, melanoma, and high-grade penile dysplasia2,3,4. Other etiologic causes can include surgery, trauma, radiation therapy, chronic inflammatory conditions including hidradenitis suppurativa, and inflammatory bowel disease.
 To date, acquired lymphangioma circumscriptum has not been reported in the setting of underlying high-grade pleomorphic soft tissue sarcoma. As such, clinical presence of grouped frogspawn-like or verrucous papules, in an area of tender induration or lymphedema, should prompt a work-up to exclude an underlying malignant etiology as new onset ALC may be masquerading an underlying malignancy.

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.

Acquired lymphangioma circumscriptum of the vulva (secondary to lymphatic obstruction) occurring ten years after a hysterectomy and radiotherapy: A case report

Acquired lymphangioma circumscriptum of the vulva (secondary to lymphatic obstruction) occurring ten years after a hysterectomy and radiotherapy: A case report

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.

Acquired lymphangioma circumscriptum of the vulva treated with ablative radiofrequency

(LC) is a benign disorder involving the lymphatic system of skin and subcutaneous tissue. A case of acquired LC of the vulva in a57-year-old woman with a 10 -15 year history of lymphoedema of the left leg and vulvar lesions (vesicles and papulovesicles) following cervical cancer operation and subsequent radio therapy is presented. The patient was treated with ablative radiofrequency and was free of lesions for 6 months.

Acquired lymphangioma circumscriptum of the vulva secondary to severe herpes simplex infection

A 43-year-old woman presented to genito-urinary medicine with skin-coloured, papillomatous papules on the mons pubis and superior labia majora (figure 1). Routine sexually transmitted infection screening was normal. Given the...

A rare case report: Acquired vulva lymphangioma in a young female post tubercular lymphadenitis

Lymphangioma are rare benign proliferations of the lymphatic system. Acquired lymphangioma circumscriptum of the vulva is a superficial lymphatic malformation, presenting as lymph-filled micro-to macroscopic vesicles. We describe a rare case of acquired vulva lymphangioma resulting from tubercular lymphadenitis. A 20-year-old female came to Dermatology and Venereology Outpatient Clinic of Dr. Soetomo General Hospital with chief complain there were multiple clusters vesicles on her genitalia since three months ago. The vesicles followed with discomfort and slightly itchy. There was a lymphedema on both of her inguinal since 5 years ago. She was diagnosed with tubercular lymphadenitis in 13 years old of age. No history of sexual transmitted disease. She had 5 times of cryotherapy and it nearly got remission.

Lymphangioma circumscriptum of the scrotum following vasectomy

Lymphangioma circumscriptum is a congenital lymphatic hamartoma and rarely occurs in the male genital organs. Here we report a case of acquired lymphangioma circumscriptum of scrotum following vasectomy, which has not been reported till date. High clinical suspicion index is the clue to the clinician for diagnosis; histopathological confirmation and adequate surgical excision with deep lymphatic cisterns give the best result.

Lymphangiectasias of the skin: victims of confusing nomenclature

Lymphangiectasias are known by a variety of names, in the dermatology literature, including lymphangiectasis, acquired lymphangiomas, secondary lymphangiomas and acquired lymphangioma circumscriptum, which has led to confusion. The condition itself, especially in the genital region, is difficult to diagnose. This article attempts to resolve the issues of the confusing nomenclature and reviews the condition, which can arise under a variety of clinical circumstances. The relevant anatomical and histological details are described, with relevant clinical illustrations, to facilitate understanding of the aetiopathogenesis of this enigmatic condition. The available medical and non-medical treatments are discussed.

Lymphangioma Circumscriptum Of Vulva Due To Tuberculosis

We report a case of a 17-year old female patient presented with huge swelling of the vulva following tubercular lymphadenitis of the inguinal region. The case was diagnosed as acquired lymphangioma circumscriptum of the vulva due to destruction of the inguinal lymph nodes by tuberculosis. The vulval swelling did not respond after antitubercular chemotherapy and required a cosmetic surgery. The role of surgery is emphasized in this case.

Acquired lymphangioma circumscriptum of the vulva

Lymphangiomas are rare benign proliferations of the lymphatic system. Acquired lymphangioma circumscriptum of the vulva is induced by impaired lymph flow. We describe a new case of lymphangioma circumscriptum resulting from tuberculosis involving a lymph node. A 45-year-old female presented with swelling of the vulva for the past 8 years. She had a history of multiple left inguinal swellings successfully treated with four antituberculosis drugs. Her vulval swelling did not respond and was diagnosed as acquired lymphangioma circumscriptum. She becomes the fifth reported case of acquired lymphangioma circumscriptum of the vulva with tubercular lymphadenitis. The single antituberculosis treatment is insufficient to control lymphangioma. Ablative methods seem encouraging.

Lymphangioma Circumscriptum of the Vulva

In Brief OBJECTIVE To promote proper identification and management of this rare entity by presenting a review of the literature, a case report, and illustrations of its presentation and pathology. DATA SOURCES Sources searched included the literature contained in the National Library of Medicine's PubMed database, using the term “lymphangioma circumscriptum” combined with “vulva.” Other search terms used were “capillary lymphangioma,” “lymphangiectasia,” and “dermal lymphangioma.” Sources also included articles predating the PubMed database that were cited by other writers. METHODS OF STUDY SELECTION Selections were restricted to the English-language medical literature published since 1960, and the search resulted in retrieval of 3272 published papers about lymphangioma. TABULATION, INTEGRATION, AND RESULTS Congenital lymphangioma circumscriptum of the vulva has been reported in 11 patients, including one case reported and illustrated in this paper, and acquired lymphangioma circumscriptum has been reported in 20. It affects females 9–76 years old (mean 42.5 years). Clinically, it is characterized by persistent clusters of thin-walled vesicles filled with clear fluid. The diagnosis is usually made by biopsy, as these lesions often mimic such infectious diseases as molluscum contagiosum. Management options have included surgical excision of the skin and subcutaneous tissue, surface abrasion by laser or sclerosing therapy, and observation. CONCLUSION From this comprehensive review, which includes the clinicopathologic features of both the congenital and acquired forms, illustrations from the rarest form, and a summary of treatment approaches, we conclude that lymphangioma circumscriptum poses a diagnostic challenge the risks of which are misdiagnosis and mistreatment. These risks would likely be reduced were a database of cases accessible that permitted long-term follow-up and better assessment of presenting characteristics and treatment options. Vulvar lymphangioma circumscriptum, whether acquired orcongenital, is a rare benign condition whose proper identification may prevent inappropriate treatment.