Vulvar Mass Research Articles

Clinical Analysis of Primary Primitive Neuroectodermal Tumors in the Female Genital Tract

The aim of the study was to investigate the clinical manifestations, diagnosis, treatment, and prognosis of primitive neuroectodermal tumors (PNETs) in the female genital tract. From April 2001 to May 2013, the clinicopathologic characteristics, treatments, outcomes, and prognosis of 11 patients with PNET in the female genital tract were analyzed retrospectively at our hospital. The location of PNET in the 11 patients presented here included vulva (2 patients), cervix (2 patients), uterus and its ligament (5 patients), and the ovaries (2 patients). Ages ranged from 18 to 59 years (median, 31 years).The main clinical manifestations of PNET in the female genital tract are irregular vaginal bleeding (6 patients), pelvic mass, uterine enlargement, and rapidly increasing vulvar mass (8 patients), and vulvar pain and lower abdominal pain (5 patients). The CA125 levels of 8 patients were elevated before the operations and reduced to normal when the diseases were controlled, while the levels increased as the tumor was progressive. Results for the most commonly used immunohistochemistry studies revealed CD99 in 11 of the 11 tumors, synaptophysin in 6 of the 7 positive tumors, and neuron-specific enolase in 6 of the 6 tumors. Ten patients underwent surgical resection. Nine of them underwent preoperative or/and postoperative combination chemotherapy. The follow-up of 10 patients were available and ranged from 1 to 145 months (median, 30.5 months), 3 of whom experiencing recurrence. Primitive neuroectodermal tumor is very rare and can originate from any part of the female genital tract. The tumors had different manifestations but the same pathologic features. CA125 may be an important marker for prognosis and follow-up of PNET of the female internal genital tract.

Uterine and ovarian remnants in an incorrectly spayed bitch: a case report

A spayed Samoyed bitch, 12 years old, was presented to the Veterinary Clinical Hospital of the University of Cordoba (Spain) with abundant vulvar sanguineous discharge over the previous three days. The clinical examination revealed a remarkable vulvar mass, which protruded through the vulvar lips. Abdominal ultrasonography revealed the presence of structures compatible with uterus and ovary, which had been presumably removed eight years previously. An exploratory laparotomy was carried out, which confirmed the presence of the right ovary and a remnant of the uterus. The histological evaluation confirmed a granulosa cell tumour in the ovary, and an enlarged portion of the right uterine horn with brownish contents. The vulvar mass was also surgically removed and fibroma with some fibrosarcoma areas was diagnosed. This case shows the evolution of ovary and uterus into the abdomen, which were incorrectly removed after ovariohysterectomy eight years previously.  

An unusual gross appearance of vulval tuberculosis masquerading as tumor.

Tuberculosis of the vulva is very rare. It is found in about 0.2% of the cases of genital tract tuberculosis. It usually presents as small shallow ulcers and multiple sinus tracts or rarely as elephantiasis of vulva. Except for very rare cases of primary tuberculosis in the vulva, it is usually associated with tuberculosis elsewhere in the body leading to secondary tuberculosis. Here, we report a case of secondary vulval tuberculosis which presented as a vulval mass in a 40-year-old female patient. The rarity of this presentation in the female genital tract is emphasized.

Pedunculated Angiomyxofibroma of Vulva: A Rare Entity Case Report

Pedunculated Angiomyxofibroma of Vulva: A Rare Entity Case Report Angiomyxofibroma is a rare, slow growing mesenchymal neoplasm that occurs almost exclusively in the genital, perineal and pelvic regions of adult women of reproductive age. It commonly occurs in the women of reproductive age group, but can be seen between age of 16 to 70 years (median 34 years). This is a rare case of large vulval pedunculated ulcerated growth developing after the vaginal delivery and on histology confirmed as a case of angiofibromyxoma. It has a marked tendency for local recurrence but with a low tendency to metastasize. This case clearly depicts the classical clinic-pathological picture of the vulval mass and diagnosing & management strategy.

Aggressive angiomyxoma of the vulva: Case report and review of literature

Aggressive angiomyxoma (AAM) is a rare mesenchymal benign tumor that preferentially involves the pelvic and the perineal region of relatively young females. The diagnosis of AAM should be considered when a female presents with an atypical vulvoperineal mass. We report a perimenopausal multiparous lady with AAM who presented with a big vulval mass. Key word : AAM - Aggressive angiomyxoma

Ectopic Mammary Tissue as a Vulvar Mass in a Lactating Woman

Ectopic Mammary Tissue as a Vulvar Mass in a Lactating Woman

Prolapsed cecoureterocele presented as prenatal genital mass: a urological challenge

Prolapse of a cecoureterocele through the urethra presenting as a prenatal vulval mass is an extremely uncommon entity. We present a case of a newborn girl with a cecoureterocele extending through the urethra (diagnosed at 29 weeks' gestation) and we present its postnatal findings and outcomes.

Reconstructive Surgery for Female Genital Mutilation Starts Sexual Functioning in Sudanese Woman: A Case Report

Female genital mutilation (FGM) involves the partial or complete removal of the external female genitalia and/or other injury to the female genital organs whether for cultural or other nontherapeutic reasons. The study aims to describe the method of and findings from reconstructive surgery for FGM victims. We present a case of a 24-year-old Sudanese female, who had undergone ritual FGM type III as a young girl. She had suffered from a large, vulval mass for the last 6 years and came to the clinic because of apareunia. We performed mass excision and reconstructive surgery of the mutilated genital tissue. The giant mass was successfully removed. Remaining genital tissues were approximated and sutured, with hemostasis assured for the reconstructed organs on each side. Reconstructive surgery for women who suffer sexual consequences from FGM is feasible, with a high degree of client acceptance and satisfaction. It restores some of women's natural genital anatomy, and offers the potential for improved female sexuality.

Unusual Locations of Primary Subepithelial Squamous Cell Carcinomas of the Vulva

This study aimed to report 2 cases of squamous cell carcinoma (SCC) of the vulva, arising from unusual subepithelial locations. The first case was of an 85-year-old woman with a 65-year history of a mass in the left labium majus. The second case was of a 54-year-old woman who presented with a 1-week history of a painful left inguinal mass. She had previously sought medical attention for a 2-year history of a left-sided painless vestibular mass. In the first case, a simple excision showed a purely dermal SCC with no attachment to the epidermis. After the diagnosis of SCC, a wide, deep local excision of the left side of the vulva and left inguinofemoral lymph node dissection were performed. Pathological findings showed no residual tumor in the vulva, and the lymph nodes were clear. Based on the long history of a mass at the same site, the pathogenesis of the SCC was considered to be malignant degeneration of a previously benign epidermal cyst.In the second case, SCC was diagnosed on fine-needle aspirations of the vulvar and groin masses. The patient was treated with primary chemoradiation. Subsequently, wide, deep local excision of the left side of the vulva and left inguinofemoral lymph node dissection were performed. No residual tumor was found in the vulva, although atrophic Bartholin gland tissue was found at the site of the SCC. One 5-mm inguinofemoral lymph node metastasis was found in the groin node dissection. In the absence of any evidence of another pathogenesis, it was believed that the SCC had arisen from an unusually anterior located Bartholin gland. Subepithelial SCC should be considered in the differential diagnosis of unusually located vulvar masses.

Subpubic cartilaginous cyst: a rare cause of dysuria

A subpubic cartilaginous cyst is a rare lesion that may present as a vulvar mass or rarely with dysuria. This condition occurs predominantly in multiparous postmenopausal women and is believed to be secondary to degenerative changes in the fibrocartilaginous disc of the symphysis pubis. The midline location, close relationship with the undersurface of the symphysis pubis and the cystic nature are the clues to the correct diagnosis.

English

Introduction Cellular angiofibroma is a rare benign mesenchymal tumour that occurs in the subepithelial myxoid stromal zone of the vulvovaginal region. We report a case of painful angiofibroma in a 52-year-old morbidly obese female, in the left labia. Case report A 52-year-old morbidly obese (body mass index of 67) female was referred to our gynaecologic oncology clinic with a 3-year history of a large, painful, pendulous left vulvar mass. Magnetic resonance imaging of the lesion helped confirm that the mass did not represent a hernia and was fibrous in nature. Resection revealed a 12 × 12 × 9 cm, a 187-gram encapsulated mass. Histologically, it was a well circumscribed tumour composed of hyalinised blood vessels and collagen bundles, exhibiting characteristic immunohistochemical features of a cellular angiofibroma. Conclusion Angiofibroma of the vulva is a rare benign encapsulated mass, yet hypervascular. Its diagnosis in a morbidly obese patient is challenging and necessitates magnetic resonance imaging or CT scan. Its surgical removal needs precision and meticulousness especially in a patient with multiple comorbidities.

Androgen Insensitivity Syndrome with Gynandroblastoma and Vulvar Leiomyoma

This study aimed to describe a rare case of androgen insensitivity syndrome (AIS) in association with gynandroblastoma and vulvar leiomyomata. The patient was a 64-year-old phenotypically female person with 46 XY chromosomal karyotypes who presented with a chief complaint of abdominal distension of 1-month duration. She had 2 vulvar masses for more than 20 years and had experienced life-long (untreated) amenorrhea. History and physical examination findings showed the patient to be 175 cm in height, with normal breast development, and a lack of pubic or axillary hair. Serum levels were as follows: testosterone, 1,980 ng/dL; estradiol, 1,380.8 pg/mL. In combination with clinical findings, a diagnosis of complete AIS was made. The patient subsequently underwent surgery, during which the bilateral vulvar masses and the rudimentary uterus, right sex gland, and left side of the tumor were resected. Histological examination of the tumor revealed a mass composed primarily of juvenile-type granulosa cells, admixed with a Sertoli cell component that comprised less than 50% of the tumor. The right side of the gonad had an ovarian-like cortex and was considered to be an undeveloped ovary. The left and right side of the vulvar masses were diagnosed with leiomyomas. Patients with AIS should be monitored closely because these patients may also experience gonadal tumors. When confronted with gynandroblastoma, close attention should be paid to the patient's endocrinologic status, and comprehensive endocrinologic analyses should be conducted to make correct treatment decisions.

Bilateral Accessory Breast Tissue of the Vulva

We present the case of a 23-year-old female with bilateral ectopic breast tissue of the vulva, the repair of which necessitated a novel labiaplasty technique. Labiaplasty is becoming an increasingly frequent cosmetic procedure, and the popularity of brief didactic labiaplasty courses has risen in response to consumer demand. There is a paucity of detailed anatomic description of female sensory innervation patterns to the clitoris and surrounding structures. This places patients at risk for denervation of clitoral structures during labiaplasty procedures. Our novel technique proposes a method of individualized patient neurosensory mapping preoperatively, which allows for surgical planning to avoid injury to the sensory branches of the dorsal clitoral nerve. A 23-year-old female presented with bilateral vulvar masses that involved the clitoral complex, which had first become apparent during the second trimester of pregnancy, and failed to resolve in the postpartum period. We describe the preoperative planning and intraoperative approach and dissection to labiaplasty in this patient, which was complex given the size of the masses, and specifically designed to avoid injury to sensory branches of the dorsal clitoral nerve. As labiaplasty becomes more common, it is important to approach labiaplasty patients with a detailed understanding of the sensory innervation of the clitoris and surrounding structures, to avoid nerve injury and resultant sexual dysfunction. Traditional labiaplasty approaches may violate the sensory innervation patterns of the clitoral region, thus causing a sensory loss that affects patient sexual function. Our novel approach to preoperative clitoral nerve sensory mapping provides an alternative method of labiaplasty that may avoid denervation injury.

Lipoma Vulvar: Um Diagnóstico Raro

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Vulvar embryonal rhabdomyosarcoma: A case report

Embryonal rhabdomyosarcoma (ERMS) is a rare malignant neoplasm found in both male and female children. It typically presents as a protruding vaginal mass with vaginal bleeding in girls less than 8 years of age. It is often seen as the sarcoma botryoides histologic sub type. This report represents the first case study of ERMS presenting as a vulvar mass. The article further explores the etiologies of pediatric vulvar masses, including ERMS, and the suggested diagnostic and treatment modalities.

Cavernous Hemangioma Presenting as a Vulvar Mass in a 10 Year Old Female

Cavernous Hemangioma Presenting as a Vulvar Mass in a 10 Year Old Female

Canal of Nuck Cyst Presenting as a Symptomatic Vulvar Mass

Canal of Nuck Cyst Presenting as a Symptomatic Vulvar Mass

Endometriosis of the perineum

Endometriosis of the perineum and vulva is extremely rare, with the most common site being episiotomy scars. We report here a case of spontaneously developing perineal endometriosis successfully treated with local excision. A 39-year-old woman was admitted complaining of a painful vulvar lump with cyclic swelling. She had first noticed the mass 7 years before, and it had gradually increased in size. Gynecological examination showed a walnut-size, painful, subcutaneous mass in the left perineum. Magnetic resonance imaging revealed a multilobular cystic mass with inner hemorrhage, suggesting vulvar endometriosis. The patient was treated by local excision of the vulvar mass, and complete excision was achieved. The pathological diagnosis of the excised tissue was endometriosis. The patient is well without evidence of disease 5 months following the local excision. Spontaneous perineal and vulvar endometriosis is extremely rare. However, any lesion that evolves in response to the menstrual cycle should be considered endometriosis.

Case of lung carcinoma revealed by vulvar metastasis associated with systemic scleroderma and literature review

Metastatic carcinoma to the vulva is rare, where the incidence is believed to be between 5% and 8%.However, malignant tumors have been described in 3–11% of systemic scleroderma (SSc) cases.We report the case of one patient, a 66-year-old postmenopausal woman, whose medical history was marked with rheumatic vascular disease (systemic scleroderma) since 1993 without muscular, renal, cardiac lesions or HTA (arterial hypertension) and without tobacco history.The woman presented with a new vulvar mass of the right labia in December 2011 that had progressively enlarged in size.CT scan of the abdominopelvic region demonstrated a lobular mass of the right labia with central necrosis, 7cm on the wide axis, and the rectum and the vaginal wall were normal. No inguinal or iliac lymphadenopathy was noted.An outpatient excisional biopsy revealed a poorly differentiated malignant tumor suggestive of carcinoma.IHC: CK7+/CK20−, estrogen receptors−, AE 1 AE 3+, vimentine+, S100−, Desmina−, CD34−, KI 67: 20%.The thoracic scan revealed a large mass of 4cm×3cm in the right lung base with right paratracheal lymphadenopathy 3cm×2cm.A bronchoscopy revealed discrete stenosis of the mediastinal portion of the right bronchial tree.The bronchial biopsy also revealed poorly differentiated lung carcinoma, non-small cell, which was identical with the vulvar tumor. ConclusionThe presence of the single lung lesion with only one lymphadenopathy paratracheal with pathological and immunohistochemical (IHC) profile similar to the vulvar lesion, and a particular IHC profile with CK7+ and CK20− was detected – that is more specific to the primitive pulmonary cancer, and the presence of only one sarcoma marker vementine+, desmine and actine−. Also the presence of KI 67: 20%, predicted the proliferative and great metastatic power of the lung tumor was observed.Additionally, lung cancer was the most frequent type and may develop in scleroderma as reported in most studies.This allows to conclude for primitive lung carcinoma revealed with vulvar metastasis after elimination of the possibility of vulvar sarcoma.The patient was treated by chemotherapy (Taxol/Platin) with partial response from the lung after 3 cycles and palliative radiotherapy in the vulva with a good response.This case described primary lung carcinoma associated with scleroderma, revealed by a vulvar metastasis, which may be related to the aggressiveness of lung cancer when the lung fibrosis follow-up is not performed well to detect early the development of lung tumors in the patient with systemic scleroderma.

Low-Grade Fibromyxoid Sarcoma of the Vulva

The study aimed to describe a case of low-grade fibromyxoid sarcoma arising from the vulva and to discuss the diagnostic challenges, clinical management, and epidemiology of this rare malignancy. A 36-year-old woman presented to 3 separate emergency departments with complaints of a painful and slowly enlarging vulvar mass. Eventual gynecologic referral resulted in excision of a 6-cm, noncystic vulvar mass. Pathological diagnosis revealed low-grade fibromyxoid sarcoma. Later, a right radical hemivulvectomy ensured adequate margins, and 2 years later, the patient is free of recurrent and metastatic disease. Low-grade fibromyxoid sarcoma is a rare malignancy that may present in the lower genital tract. Definitive diagnosis is essential because low-grade fibromyxoid sarcoma may metastasize many years after diagnosis, thereby requiring indefinite clinical surveillance.