Surgically Managed Acquired Vulvar Lymphangioma Circumscriptum.

Lymphangioma circumscriptum is a rare benign disorder of lymphatic channels in deep dermal and subcutaneous layers. It can occur either as a congenital abnormality or as acquired damage to previously normal lymphatic channels. It occurs in different parts of the body, and the vulva is one of the parts which is commonly affected. Here, we presented a 39 years old para 2 who presented with vulvar swelling. She was diagnosed with acquired lymphangioma circumscriptum of the vulva and superficial vulvectomy was done.

Congenital lymphangioma circumscriptum of the vulva.

Lymphangioma circumscriptum is a rare benign disorder of unknown etiology affecting lymph vessels in the deep dermal and subcutaneous layers. The commonest sites of presentation are the axilla, shoulders, groins, tongue and oral cavity. Lymphangioma circumscriptum of the vulva is very rare can be either congenital or secondary to lymphadenectomy or pelvic radiation in patients with genital cancer, genital tuberculosis, Crohn's disease, cellulitis, morbid obesity and immobilization. We report the case of congenital lymphangioma circumscriptum of the vulva in an adolescent with congenital lymphedema of the leg.

Congenital lymphangioma circumscriptum of vulva presenting as multiple giant mass lesions: a case report and literature review.

A G2L2 33-year-old woman presented to our clinic with large verrucous warty masses in labia major, perinea and the end portion of her spine measuring about 15 × 7, 9 × 7 and 8 × 8 cm, which had been enlarged following puberty and pregnancy. Her right upper and left lower limbs had gross congenital lymphedema. The masses were removed by superficial partial vulvectomy with a qualified margin and repaired without skin graft. Pathology report showed lymphangioma circumscriptum. Several months of follow up revealed normal healing and no recurrence. In conclusion, congenital vulvar lymphangioma, which is a rare disorder, can be highly triggered by hormonal-stimulating situations like puberty and pregnancy. Thus, it is better to visit the affected cases in a timely manner in order to excise these lesions before massive enlargement. In our experience, a superficial partial vulvectomy without skin graft can be a sufficient procedure. Obviously, an appropriate approach during and after surgery would be very important for obtaining a desirable healing.

[Rapid regression of infantile haemangioma with 2% propranolol ointment]. / Régression rapide d'un hémangiome infantile sous propranolol topique à 2 %

BACKGROUND: Oral propranolol produces dramatic results in severe infantile haemangioma. We report a case of rapid and significant regression of an infantile haemangioma in infant treated topically with 2% propranolol ointment. PATIENTS AND METHODS: A female infant aged 11 weeks was seen as an outpatient for two infantile haemangiomas on the front of the left knee and the vulva. After parental consent was given, the child was treated with a topical preparation of 2% propranolol ointment prepared by the pharmacy. Regression was rapid and on the 45th day, lesion size had been reduced by an estimated 75%. No adverse effects were observed. DISCUSSION: Topical propranolol appears to be effective and safe for the treatment of superficial haemangiomas. Clinical trials are required to determine the optimal dosage and pharmaceutical form, method of use and treatment duration.

Congenital vulvar lymphangioma circumscriptum.

Lymphangioma circumscriptum (LC) is a defect of lymphatic channels in the deep dermis and subcutaneous layers, characterized by grouped vesicles. This disorder rarely occurs in vulvar sites. We present a rare case of LC in a 72-year-old patient who presented with a vulvar mass and ipsilateral leg edema.

Congenital plexiform schwannoma of the clitoris.

Schwannomas are slow-growing nerve sheet neoplasms which are rarely found in the female genital system. In this article, we present a patient with Antony A congenital plexiform schwannoma of the clitoris. A 6-year-old girl was brought to our hospital with the history of a firm non-cystic clitoral mass from birth, which had been growing more rapidly during the previous year. The patient was scheduled for surgery. Histological studies revealed plexiform schwannoma of the clitoris post-operatively. Therefore, schwannoma should be considered in the differential diagnosis of clitoral masses.

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.

Recurrent vulval congenital lymphangioma circumscriptum--a case report and literature review.

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.

[Congenital botryoid rhabdomyosarcoma of the vulva]. / Rabdomiosarcoma botrioides congénito de vulva.

Rhabdomyosarcoma is the most frequent malignant soft tissue tumor in pediatric patients; however, the vulvar location and congenital appearance are exceptional. We present the case of a newborn girl with botryoid rhabdomyosarcoma of the vulva, treated with chemotherapy, conservative surgery and autologous transplant. Botryoid rhabdomyosarcoma is a variation of embryonal rhabdomyosarcoma that typically grows in mucosa-lined hollow organs, from where it can spread to the body surface. The treatment of botryoid rhabdomyosarcoma in the genito-urinary area is based on polychemotherapy, and it can be complemented with radiotherapy and conservative surgery if necessary, thus resulting in an excellent prognosis and few long-term functional sequelae.

Congenital vulvar teratoma in a newborn.

The most common site of teratomas in neonates is in the sacrococcygeal region. Herein the authors describe a congenital teratoma, and to their knowledge it is the first reported case of vulvar site in a newborn. Tumor was removed on the fourth day of life, and she was completely normal 18 months after the operation. Because of possible malignancy or recurrence, complete surgical excision of the tumor and careful follow-up is the treatment of choice. J Pediatr Surg 36:620-621.

Congenital epulis and granular cell tumor: a histologic and immunohistochemical study.

OBJECTIVES: Although it is now reasonably certain that granular cell tumors derive from Schwann cells, the histogenesis of congenital epulis, which is largely isomorphic with granular cell tumor, remains unclear. A study was undertaken to compare the immunophenotype of these tumors with particular emphasis on the expression of matrix proteins and macrophage markers because such information is not available in the literature. STUDY DESIGN: Four granular cell tumors and two congenital epulis were immunostained with a panel of 29 antibodies. Two congenital epulis and one granular cell tumor were investigated by electron microscopy, the latter also by immunoelectron microscopy. RESULTS: Many similarities in immunostaining were found, for example, both tumor types were CD68+, Ki-M1P+, lysozyme-, vimentin+, fibronectin+, laminin+, lectin PHAE+, and lectin WGA+. However, differences were also noted, for example, granular cell tumor was always S100 protein+, but only one congenital epulis case was reactive (weak reactivity after microwave treatment), and staining with the proliferation markers anti-proliferating cell nuclear antigen and MIB 1 was found only in congenital epulis. Both tumor types exhibited pericellular and diffuse cytoplasmic staining for fibronectin and laminin. CONCLUSIONS: The hypothesis that congenital epulis and granular cell tumor would exhibit similar reactivity for macrophage markers was confirmed: both were reactive with anti-CD68 and Ki-M1P and nonreactive with MAC387, anti-lysozyme, and 3A5. Intracytoplasmic staining for fibronectin and laminin, which has not been described previously in these tumors, appears to be a characteristic feature common to both tumors. This finding suggests that there could be a disturbance of synthesis and secretion of extracellular matrix proteins or a derangement of their receptor systems. This theory could be supported by the finding of intracytoplasmic CD49e-positive material in two cases.

Congenital hemangiopericytoma of the clitoris.

Hemangiopericytoma is a rare vascular tumor that is believed to arise from the pericyte of Zimmermann. Approximately 10% of cases occur in children and a third are congenital. To our knowledge we report the first case of a congenital hemangiopericytoma arising from the clitoris, describe its management and review the literature.

Vulvar congenital dysplastic angiopathy.

Congenital dysplastic angiopathy is a syndrome consisting of vascular angiomata, congenital varicosities, and trophic changes of the soft tissue and the skeleton. Frequently referred to as Klippel-Trenaunay or Klippel-Trenaunay-Weber syndrome, it rarely affects the female genitalia. An 18-year-old woman underwent evaluation and treatment for Klippel-Trenaunay syndrome with vulvar involvement. Preoperative evaluation included consultation with pediatric surgeons, gynecologic surgeons, and an interventional radiologist as well as individual and family psychological counseling. Attention to intraoperative detail resulted in minimal operative blood loss and preservation of normal anatomy. A postoperative hematoma was treated aggressively with surgical evacuation and drainage. Six-month follow-up revealed functionally and cosmetically normal vulvar anatomy.

Cáncer de vulva

Se analizaron 51 historias clínicas del HUSVP con diagnóstico de cáncer de vulva y a pesar de que el número de casos no es muy grande, nos permite sacar algunas conclusiones. El 86 por ciento se presenta después de los 40 años. El 76.6 por ciento consulta después de 1 año de la iniciación de los síntomas. Se encontró una asociación con otros tumores ginecológicos primarios en el III y IV. El tratamiento de elección en los tumores malignos de vulva es el quirúrgico. Los resultados del tratamiento son mejores en los estados iniciales. Las recurrencias son más frecuentes en los avanzados. El seguimiento a 5 años es del 66.6 por ciento, cifra que se debe tratar de mejorar para poder obtener una rata de supervivencia más ajustada a la realidad. Es importante la identificación más temprana de esta patología para un diagnóstico más precoz

Congenital dysplastic angiopathy (Klippel-Trenaunay-Weber syndrome) with vulvar involvement. A case report.

Congenital dysplastic angiopathy (Klippel-Trenaunay-Weber Syndrome) consists of angiomata and congenital varicosities with trophic changes of soft tissues and various parts of the skeletal system. Involvement of female genitalia is rare and has been reported to occur in only four patients. The authors treated a patient with a lymphangioma of the vulva associated with congenital dysplastic angiopathy.