Angiomiofibroblastoma vulvoperineal, presentación de un caso / Vulvoperineal angiomyofibroblatoma, a case report

An angiomyofibroblastoma is a benign tumor that may present itself in the genital tract of a reproductive age woman. Despite it being a benign neoplasia, unable to be invasive, it can reach large sizes, producing discomfort, pain, and anatomical deformation. A late diagnosis may result in increased surgical difficulty for resection. The following is the clinical case of a woman with a vulvo-perineal tumor of 6 years of evolution, with a final diagnosis of angiomyofibroblastoma, in which complete resection of the lesion was achieved with good cosmetic results. The highlight of this tumor is its slow, silent, but progressive growth, leading to diagnoses that are often confused and late, with a consequent delay in treatment. Among the common differential diagnoses are the Bartholin gland cyst and the aggressive angiomyxoma.

El angiomiofibroblastoma es un tumor benigno que puede presentarse en el tracto genital de la mujer en edad reproductiva. A pesar de ser una neoplasia benigna, sin capacidad invasora, puede alcanzar grandes tamaños, produciendo molestia, dolor y alteración importante de la anatomía. Si el diagnóstico es tardío, aumenta la dificultad quirúrgica en su resección. A continuación, se presenta el caso clínico de una mujer con un tumor vulvoperineal de 6 años de evolución, con diagnóstico final de angiomiofibroblastoma, en el que se logró la resección completa de la lesión con un buen resultado estético. La importancia de este tumor está dada por su crecimiento lento, silencioso, pero progresivo, llevando a que su diagnóstico sea en muchas ocasiones confuso y tardío, con el consecuente retraso en el tratamiento. Dentro de los diagnósticos diferenciales comunes se encuentran el quiste de la glándula de Bartholino y el angiomixoma agresivo.

Pediatric Vulvar Superficial Angiomyxoma: A Case Report With Clinical, Radiological, and Anatomopathological Characterization and a Comprehensive Review of the Literature.

Superficial angiomyxoma is characterized as a benign, slow-growing vascular cutaneous myxoma. A 6-year-old Arab girl with no medical history presented with a vulvar tumor located on the left labia majora. The lesion was present since birth, but it had significantly increased over the last 6 months. She did not have any associated symptoms. Physical examination revealed an exophytic tumor of the left labia majora, which measured 5 cm in its major axis. Doppler ultrasound study showed a mass with abundant arterial and venous vascularization, and magnetic resonance imaging showed a highly vascular contrast-enhanced mass with well-delimited margins, which depended on the labia majora. A macroscopically complete resection was performed, achieving a tension-free primary closure. Histologically, the lesion was characterized as a well-demarcated superficial tumor with thin-walled vessels and myxoid stroma, S100 (-), CD34 (+), vimentin (+), and actin (+). The final histopathological diagnosis was superficial angiomyxoma. The literature review of this entity in the pediatric population shows a predominance of this lesion in the vulvar location. Local recurrence has been described. Loss of PRKAR1A expression may be involved in the pathogenesis of superficial angiomyxoma.

Primary myoepithelial carcinoma of the Vulva: Case of a rare tumor with malignant potential and review of literature.

•Primary myoepithelial carcinoma is rare with a handful of cases reported from the vulva.•There is no standard of treatment for primary myoepithelial carcinoma.•Current recommendations suggest excision with wide margins to be the best therapy.•Chemotherapy and immunotherapy should be considered in combination with excision.

Experience with flap repair after vulvar carcinoma resection: a retrospective observational study of 26 cases.

Background: A defect caused by the radical resection of vulvar cancer requires repair with flap transplantation or vulvoplasty. However, in clinical practice, the surgeons encounter difficulties while using a flap to repair the wound. Therefore, this study aimed to present a review of our practice of post-surgical defect reconstruction in cases using different skin flaps. Methods: An observational study was performed involving 26 patients with vulvar cancer who were admitted to Sun Yat-Sen Memorial Hospital between February 2015 and February 2020 for surgical and reconstructive procedures. The clinical data of these 26 patients were analyzed. All patients underwent radical resection of vulvar cancer, followed by post-surgical defect repair using random flap or axial flap transplantation (even for very complex defects). The clinical variables collected and the assessment of efficacy included survival of the flap, history of dysfunction of the recipient area, such as scar contracture, and satisfaction of the patient with the shape after external vaginal surgery. Results: Among the 26 cases in this study, all patients underwent 38 soft tissue reconstruction procedures for vulvar perineal defects during the study period. Squamous cell carcinoma was the most commonly diagnosed cancer (80.8%). The average size of the defect was 9.3×7 cm2. Rhomboid flaps were the most commonly used flaps for performing reconstruction in both the primary and recurrent groups. Poor wound healing was the most commonly discovered complication, which occurred in three of the 38 flaps (7.9%) used. Previous surgery or radiotherapy did not increase the rate of complications following successful reconstruction. Conclusions: Different skin flaps are effective premium options for post-surgical defect reconstruction, and the selective use of skin flaps for treating vulvar defects preserves the vulvar morphology and allows for relatively better functionality.

Clinical characteristics and risk factors of invasion in extramammary Paget's disease of the vulva.

PURPOSE: This study aimed to evaluate the risk factors of recurrence and invasive disease in patients with extramammary Paget's disease of the vulva (EPDV). METHODS: We performed a retrospective analysis of patients who were initially diagnosed with EPDV in Fudan University Shanghai Cancer Center between May 2006 and March 2019. RESULTS: Thirty-eight patients were initially diagnosed with EPDV in our institution. Among them, 29 had intraepithelial EPDV, 8 had intraepithelial EPDV with stromal invasion, and 1 had an underlying vulvar adenocarcinoma. In total, 8 (21%) patients had 12 recurrences. Of these eight patients, four had one recurrence, while other four had two recurrences. Intraepidermal EPDV recurred nine times, while intraepidermal EPDV with invasive disease recurred thrice. The first and second recurrence intervals were 62.1 (9-146) months and 22 (15-28) months, respectively. The rate of invasive disease was 23.7% (9/38) for primary EPDV and 25% (3/12) for recurrent ones. We determined that the presence of invasive disease was associated with a history of more than 10 years (p = 0.02) and inversely correlated with positive margins (p = 0.037), However, invasive disease had no statistical relations with age (p = 0.438), recurrence (p = 0.642), and lesion diameter (p = 0.08). CONCLUSIONS: EPDV with a history of more than 10 years was associated with invasive disease. Close and long-term follow-up are recommended to identify those who require further treatment.

Vulvar intravascular papillary endothelial hyperplasia or Masson's tumor: A case report.

Masson's tumor, also known as intravascular papillary endothelial hyperplasia (IPEH), is a rare and benign vascular disease in which there is a reactive hyperplasia of intravascular endothelial cells. This tumor is most commonly found in soft tissues in the head, neck and upper extremities. We report a rare case of IPEH on the vulva. A Hispanic woman in her mid-thirties presented with a painful and pruritic left vulvar mass. On physical examination, a pedunculated mass was visualized on the left labia majora. Under pathologic examination, it was concluded the lesion was IPEH and it was surgically excised. This is a rare case of IPEH located on the vulva. However, despite this rarity, a simple local excision could be used to manage IPEH.

Pregnancy-associated gigantomastia recurrence and ectopic breast after reduction mammaplasty: A case report.

It has been reported that majority of cases of gigantomastia, also known as breast hypertrophy and macromastia, occur during either pregnancy or puberty. Gigantomastia is a rare disorder that does not have a clear etiology or well-established risk factors. We present a 26-year-old female patient who appeared to have pregnancy-associated gigantomastia recurrence, large accessory breast and, ectopic breast tissue at external genital three years after bilateral breast reduction surgery. The patient successively underwent bilateral mastectomy and vulvar tumor resection. Breast hypertrophy and progenital ectopic breast were pathologically confirmed. This the first case of gigantomastia reported worldwide.

Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 1.

Malignant tumors of the female reproductive system are a serious health and social problem, as they are the second most common cause of death among women, after breast cancer. Their incidence has increased dramatically during recent years, probably due to the different sexual habits and changes in the prevalence of HIV/ AIDS and HPV virus carriers, among other factors. Vulvar tumors represent only 4% of all gynecological neoplasms, and they are fourth in frequency after tumors of the cervix, uterus, and ovary. Ninety eight percent of all vulvar tumors are benign and only 2% are malignant. The overall incidence of tumors with vulvar location is between two and seven cases per 100,000 women, and it increases with age, while the death rate is estimated at 0.7 per 100,000 women. Sarcomas of the vulva comprise approximately 1-3% of all vulvar cancers, with leiomyosarcomas, epithelioid sarcomas, and rhabdomyosarcomas being the most common among them. They are characterized by rapid growth, high metastatic potential, frequent recurrences, aggressive behavior, and high mortality rate. In this paper, we present the most common forms of sarcomas of the vulva (leiomyosarcoma, epithelioid sarcoma, malignant rhabdoid tumor, rhabdomyosarcoma) in order to emphasize the broad differential diagnosis, rare appearance, non-specific clinical picture, aggressive course, and high mortality.

Epidermal cyst in an unusual site: A case report.

INTRODUCTION: Epidermoid cysts can occur in a variety of locations including face, trunk, neck, extremities and scalp. Up to now, those vulvar epidermal cysts reported in the literature were localized on the labia majora and the clitoris. This is the first case of epidermal cyst reported on the labia minora. PRESENTATION OF CASE: A 47-year-old, multiparous woman presented with a history of a palpable vulvar mass, without pain but causing difficulty in walking. The large mass was 6cm in diameter and located in the left labium minus. The labial mass was surgically removed. The final pathologic diagnosis was a vulvar epidermoid cyst. The patient was discharged from hospital without any complications. DISCUSSION: Total surgical excision of the mass is more appropriate for definitive histopathological diagnosis and for the prevention of future development of complications. MRI is very important in the localization of the mass and relationship with other tissues regarding treatment planning of larger vulvar masses. CONCLUSION: Epidermal cysts should be considered in the differential diagnosis of a vulvar mass.

Vulvar granular cell tumor.

Granular cell tumors are rare, usually benign, soft tissue neoplasms of neural origin. They occur more often in females than males, the peak age incidence is in the fourth through fifth decades. They can occur anywhere in the body with up to 15% situated in the vulva. The commonest presentation is as an asymptomatic mass. Microscopic findings are usually sufficient, but immunohistochemistry can also be helpful in confirming the diagnosis. The vulvar tumors are benign in 98% of cases with 2% reported as malignant. In this case report we describe a woman with a granular cell tumor confirmed by biopsy who underwent excision of the mass but with focal extension to the resection margin on microscopy. Our recommendation of re-excision was declined. Since it is not uncommon with these tumors to find groups of tumor cells extending beyond the macroscopic limits of growth, we conclude that it is advisable to have margins assessed intraoperatively by frozen section such that further excision can be performed for positive margins. Our patient has been followed for 18 mo without recurrence, should the tumor recur, re-excision, with frozen section control, is indicated. Recurrence rates are reported as 2%-8% with clear margins and 20% with positive margins.

Neurofibroma circunscrito solitario de la vulva / Solitary circumscribed neurofibroma of the vulva

Introdución: el neurofibroma es un tumor de comportamiento benigno que se origina de las células de la vaina nerviosa periférica, puede presentarse en forma solitaria o como lesiones múltiples como parte de la enfermedad de Von Recklingha³sen o neurofibromatosis. Los solitarios son de presentación rara y usualmente no se encuentran asociados a síntomas sistémicos. Objetivo: presentar el caso y la revisión bibliográfica en la literatura nacional y extranjera. Paciente: se presenta el caso de una paciente con un tumor solitario en la vulva tratado de forma quirúrgica en el servicio de Ginecología del Hospital Carlos Manuel de Céspedes y del Castillo de Bayamo, Granma, en octubre del 2011. Resultados: se procedió a retirar la lesión quirúrgicamente mediante cirugía escisional con enucleación del tumor y resección de la piel redundante. Conclusión: el diagnóstico histológico se correspondió con neurofibroma

Introduction: neurofibroma is a benign tumor that originates from cells of peripheral nerve sheath. It may occur as solitary or multiple lesions as part of Von Recklinghausen disease or neurofibromatosis. The solitary lesions are rare and usually they are not associated with systemic symptoms. Objective: case presentation and literature review in national and international literature. Patient: we report the case of a patient with a vulvar solitary tumor, treated surgically at the Department of Gynecology of Carlos Manuel de Céspedes y del Castillo Hospital in Bayamo, Granma, in October 2011. Results: we proceeded to surgically remove the lesion by excisional surgery with enucleation of the tumor and resection of redundant skin. Conclusion: the histological diagnosis was consistent with neurofibroma

Painful clitoromegaly caused by rare epithelioid hemangioma.

► Painful clitoromegaly may be caused by an infiltrating epithelioid hemangioma tumor. ► Epithelioid hemangiomas are usually benign. ► Rare forms are the aggressive epithelioid hemangioendotheliomas or epithelioid angiosarcomas. ► Endocrine and infectious causes of clitoromegaly must be ruled out.