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Fibroepithelial polyps are benign lesions that may appear in the vulvovaginal region. They usually occur in women of reproductive age and tend to grow up to 5 cm, but there are some rare cases in which they grow up to 20 cm. We report a case of a 22-year-old woman in the third trimester of her first pregnancy with spontaneous bleeding from a pedunculated mass measuring 15 cm in the widest diameter on the right side of the vulva. Features of this case are discussed as well as its implications, especially regarding the decision of labour. Due to the big size of the mass and its propensity to bleed, we decided to perform an elective caesarean section as well as its excision.

Cellular angiofibroma (CA)is a rare benign mesenchymal tumor. In women, it occurs mainly in the vulvovaginal region, with vulvar location in 70% of the cases. Its clinical presentation is nonspecific and similar to several other vulvar tumors of different cellular origins. Thus, its histological and immunohistochemical features allow distinction from other tumors. Cellular angiofibromas have good prognosis, despite some risk of relapse. The authors present the case of a 49-year-old woman with a bulky right vulvar lesion, for which the preoperative diagnosis was a Bartholin cyst, but the histological and immunohistochemical evaluation yielded a CA.O angiofibroma celular é um tumor mesenquimatoso benigno e raro. Nas mulheres, surge principalmente na região vulvo-vaginal, com localização vulvar em 70% dos casos. A sua apresentação clínica é inespecífica e semelhante a vários outros tumores vulvares de diferentes origens celulares. Assim, são as suas características histológicas e imunohistoquímicas que permitem a diferenciação entre eles. Os angiofibromas celulares têm bom prognóstico, embora apresentem algum risco de recidiva. Os autores apresentam o caso de uma mulher de 49 anos de idade com uma lesão vulvar direita volumosa, cujo diagnóstico pré-operatório era de quisto da glândula de Bartholin, mas cujo exame histológico e imunohistoquímico revelaram tratar-se de um angiofibroma celular.

Background: The category of the “stromal tumors of the lower female genital tract” encompasses a wide spectrum of lesions with variable heterogeneity, which can be nosologically classified on the basis of their morphologic and immunohistochemical profiles as deep (aggressive) angiomyxoma (DAM), cellular angiofibroma (CAF), angiomyofibroblastoma (AMFB) or myofibroblastoma (MFB). Despite the differential diagnosis between these entities being usually straightforward, their increasingly recognized unusual morphological variants, along with the overlapping morphological and immunohistochemical features among these tumours, may raise serious differential diagnostic problems. Methods and Results: The data presented in the present paper have been retrieved from the entire published literature on the PubMed website about DAM, CAF, AFMB and MFB from 1984 to 2021. The selected articles are mainly represented by small-series, and, more rarely, single-case reports with unusual clinicopathologic features. The present review focuses on the diagnostic clues of the stromal tumours of the lower female genital tract to achieve a correct classification. The main clinicopathologic features of each single entity, emphasizing their differential diagnostic clues, are discussed and summarized in tables. Representative illustrations, including the unusual morphological variants, of each single tumour are also provided. Conclusion: Awareness by pathologists of the wide morphological and immunohistochemical spectrum exhibited by these tumours is crucial to achieve correct diagnoses and to avoid confusion with reactive conditions or other benign or malignant entities.

El paraganglioma es una neoplasia neuroendocrina extraadrenal derivada de precursores de la cresta neural, que surgen en asociación con los ganglios autónomos y, en ocasiones, también son denominados feocromocitomas extraadrenales. Se definen por su sitio anatómico y si son o no hormonalmente funcionales. Los paragangliomas son neoplasias poco frecuentes del tracto genital femenino y pueden aparecer en ovarios, útero o cuello uterino. Los que aparecen en la región vulvovaginal son extremadamente raros. Las manifestaciones clínicas dependen de la secreción no regulada de catecolaminas y la localización. El diagnóstico se basa en hallazgos morfológicos e inmunohistoquímicos. La resección quirúrgica es un tratamiento primario para este tumor, ya que no responde bien a la quimio-radioterapia. Debido a su baja frecuencia y sintomatología inespecífica, puede mostrar dificultades para realizar el diagnóstico. Se presenta un caso de paraganglioma vulvovaginal primario.

Aggressive angiomyxoma (AA) is a rare mesenchymal neoplasm occurring almost exclusively in the vulvovaginal region and which has a wide differential diagnosis. It has previously been suggested that the nuclear transcription factor HMGA2 is a useful marker of AA, although the number of studies is limited. We investigated HMGA2 immunoreactivity in a large series (n=284) of vulvovaginal mesenchymal lesions. HMGA2 nuclear staining was classified as diffuse (≥50%), focal (

Cellular angiofibroma is a rare type of benign mesenchymal tumour that arises mostly in middle-aged women. It needs to be distinguished from other neoplasms and has a predilection for the vulvovaginal region. To our knowledge, this is the first case of a cervical cellular angiofibroma. A 34-year-old nulligravid woman was referred with a large mass bulging in the fornix posterior. Ultrasound scanning and MRI showed a large solid mass projecting in the pouch of Douglas. Laparoscopic surgical excision was performed. Histopathological examination showed a well-demarcated, unencapsulated tumour, consisting of short fascicles of spindle cells in-between thick-walled medium-sized vessels. On immunohistochemistry, there was strong reactivity with antibodies against CD34 and oestrogen receptor. Angiofibromas are benign mesenchymal tumours mostly occurring in middle-aged women. They can cause abnormal swelling and uterine bleeding and need to be distinguished from other (malignant) neoplasms.

Angiomyofibroblastoma (AMF) represents a rare, benign mesenchymal tumor with a predilection for the vulvovaginal region, which may be misdiagnosed as aggressive angiomyxoma (AAM). Herein, we report a case of a 20-year-old nulliparous Chinese woman with a unique pedunculated and polypoidal mass, which had been developing within the previous 6 months in the left labium majus, exhibiting the AAM clinical impression but diagnosed as AMF. The mass measured 18 × 10 × 6 cm, and contained diffuse ulcerated areas and purulent discharge. A complete excision of the mass was performed. There was no subsequent evidence of recurrence, according to a 13-month follow-up. As a rare benign vulvovaginal tumor, AMF can present on patients of an early reproductive age with rapidly growing, polypoidal pattern. The whole exon sequencing analysis revealed the genomic alterations, which may contribute to the occurrence of AMF.

The wide variety of mesenchymal lesions that occur in the vulvovaginal region can result in diagnostic difficulties for pathologists. This is because of their relative rarity and overlapping morphological features. They can be divided into those lesions which are specific to or characteristic of this site and those which can occur at any site with no predilection for the vulvovaginal region. Many of the site specific or characteristic lesions are thought to arise from the specialized subepithelial stroma of the lower female genital tract that extends from the cervix to the vulva; the stromal cells of this region are hormone responsive and, as a consequence, most of the site specific mesenchymal lesions are positive with oestrogen receptor and progesterone receptor. Immunoreactivity with desmin, CD34 and CD99 is also common and this constitutes an unusual immunophenotype. The best known of the site specific vulvovaginal mesenchymal lesions is aggressive (deep) angiomyxoma, an infiltrative neoplasm with a marked propensity for local recurrence following excision. Most of the other site specific mesenchymal lesions are well circumscribed and exhibit little tendency for local recurrence. These include fibroepithelial stromal polyp (the most common), angiomyofibroblastoma, superficial myofibroblastoma of the lower female genital tract and cellular angiofibroma. Smooth muscle tumours also occur in this region and exhibit a propensity for local recurrence. Relatively recently described mesenchymal lesions in the vulvovaginal region include vulval oedema, prepubertal vulval fibroma and reactive fibroblastic and myofibroblastic proliferation of the vulva (cyclist's nodule). A wide range of other mesenchymal lesions potentially occur in this region and it is stressed that when dealing with a vulvovaginal mesenchymal lesion, pathologists should consider a wide range of diagnoses since many mesenchymal lesions potentially occur in this region.

Aggressive angiomyxoma is a rare and locally aggressive mesenchymal tumour, predominantly occurring in women of reproductive age group. The term aggressive is attributed to the infiltrative nature and frequent local recurrences. They arise commonly from the vulvovaginal region, perineum or pelvis and are usually misdiagnosed as other common entities in these regions. Radiological investigations aid in the diagnosis and planning of surgery. However, the final diagnosis in most of the cases is established by histopathological examination. We herein report a case of a middle-aged woman presenting with recurrent large right vulvar mass highlighting the surgical challenges posed by its intrapelvic extension.

The main objective was to evaluate the association between vulvovaginal laceration and uterine diseases in dairy cows. The secondary objectives were to evaluate the association between vulvovaginal laceration and cyclicity, and reproductive performance. The vulvovaginal region of 660 Holstein cows from a 5,000 lactating-cows herd was inspected at 4d in milk (DIM) for the presence of lacerations, and scored (VLS) as follows: 0=no laceration; 1=laceration

Physiologic alterations of the oral and vulvovaginal mucosal surfaces result from the profound hormonal and immunologic changes of gestation. High estrogen levels are responsible for the vascular changes noted on mucosal surfaces. Gingival hyperemia and edema, gingivitis and pyogenic granuloma are the most common alterations of the oral mucosa during gestation. Physiologic changes of the vulvovaginal area are mainly of vascular nature, and include among others varicose veins. The oral and vulvovaginal mucosal surfaces can be affected by diseases that can worsen or develop in pregnancy. Oral lesions are encountered in a large spectrum of diseases including aphthosis, pemphigus vulgaris, systemic lupus, and Behçet disease. Pregnancy dermatoses such as impetigo herpetiformis and gestational pemphigoid can exceptionally affect the oral mucosa. Infections of the vulvovaginal region by Candida species, Trichomononas vaginalis, human papilloma virus, and herpes simplex virus have been associated with fetal risks. The dermatologist, obstetric medicine physician, and neonatologist should be familiar with the above physiologic changes as well as maternal/fetal risks relevant to disease affecting these mucosal surfaces during gestation.

Background: Cellular Angiofibroma (CA) is an uncommon benign tumor that generally occurs in the vulvovaginal region in women. Extra-vulvar cases have previously been described but are exceptionally rare. Case: A 45-year-old female with an incidentally noted pelvic mass in the Space of Retzius that was presumed to be a fibroid. Excision was complicated by significant haemorrhage from the surgical bed. Conclusion: Extra-vulvar cases of CA are rare and have never been described in the Space of Retzius. We also discuss preoperative MRI findings to aid in diagnosis and surgical planning. Additionally, this CA was highlyvascularized, resulting in massive haemorrhage after removal. This is the first case to identify haemorrhage as a source of morbidity in CA.

Addressing vaginal laxity, atrophic vaginitis, stress urinary incontinence (SUI), and different manifestations of sexual dysfunction has always been problematic due to women's traditional difficulty discussing these issues with doctors as well as the societal attitude of resignation toward these conditions. The recent rise of non-invasive feminine rejuvenation using energy-based modalities to vaginal tissue has its origins in aesthetic medicine. Transcutaneous temperature-controlled radiofrequency therapy at the vulvovaginal region has shown promising results in giving a more youthful appearing vulva, restoration of vaginal elasticity and 'tightness', considerable improvement in SUI, reduction in overactive bladder symptoms, and reduction in sexual dysfunction. It is also emerging as the non-invasive treatment modality for mild to moderate SUI. It seems that the time has come, when women shall ever be grateful to their gynecologist for management of SUI with ThermiVa without an incision.

Fibroepithelial polyps of the vulva are specific mesenchymal lesions that are commonly found in the vulvovaginal region in reproductive period and in premenopausal females. We present a case of a 36 year old female, who came for examination because of interruption of unwanted pregnancy. During the examination we revealed a large flank mass on the stalk, which started from the right labia. Tumor formation was measuring 165x120x10mm, soft consistency, painfully insensitive to palpation. The tumor was removed by excision, cutting the stem at the base and the material was sent for histopathological examination. Histopathological findings confirmed that this is a fibroepithelial polyp of the vulva.

Background Fibroepithelial polyps (FEPs) are benign tumors, of possibly hormone-dependent nature, found in the vulvovaginal region of women of reproductive age. Case A 15-year-old adolescent girl, receiving hormonal contraceptive therapy, who presented with multiple vulvar masses with histopathology consistent with FEP. Summary and Conclusion The spectrum of the morphology of FEPs might make their diagnosis challenging. We describe a rare presentation of vulvar FEPs in an adolescent girl receiving hormonal contraceptive therapy.

Introduction: Angiomyofibroblastoma (AMFB) is an extremely rare disease. It commonly occurs in middle-aged females and mainly involves the vulvovaginal region. Pathological examination plays an important role in differentiating from other tumors. But far less published literature focus on the imaging characteristics of AMFB. Clinical findings/diagnoses: We reported a case of AMFB in a 73-year-old male, involving the mediastinum with computed tomography (CT) imaging and pathologic findings. Preoperative unenhanced CT scan of chest revealed a 6.9 × 7.4 × 9.3 cm mass with equal density, located in the posterior mediastinum. On contrast-enhanced CT images, the tumor presented moderate, heterogeneous enhancement. Due to the unclear interface between the tumor and adjacent tissues, this patient underwent operative partial resection of the tumor. The pathologic diagnosis was AMFB. Conclusions: This tumor represents a further example of unusual location different from conventional AMFB. This case adds to the experience with AMFB by summarizing its characteristics, and also reviewing the literature.

Schwannoma is a rare benign encapsulated tumor derived from neural sheath cells. The tumor is more common in the head and neck regions and the extremities. Its location in the vulvovaginal region is a rare phenomenon with a few reported cases worldwide. We report a case of a large vulvar schwannoma occurring in an 18‑year‑old woman, primigravidae at 35 weeks gestation. The ulcerated tumor mass which measured 10.5 × 7 × 6.5 cm and weighed 249.2 grams was located on the right labium majus with extension into the vagina. The tumor evoked strong immunohistochemical reactivity to S‑100. There was no evidence of recurrence after several weeks of excision of the neoplasm. Key words: Pelvic nerve sheath tumor; schwannoma; vulva mass.

Fibroepithelial polyps (FEPs) are rarely seen lesions of the lower female genital tract with polypoid proliferations of stroma. These tumors usually present in the vulvovaginal region of the reproductive aged women. In this presentation, we report a case of a psoriatic woman who developed unusual multiple polypoid lesions approximately 15 cm in size arising from both left and right labia minora and unique connection of FEPs with psoriasis disease.

This report describes a case of aggressive angiomyxoma (AAM), arising in the vulvovaginal region, which was initially misdiagnosed as lymphedema due to a vaginal ulcer caused by Behcet’s disease. AAM is a very rare disease, usually found only in women of reproductive age, occurring predominantly in the pelviperineal region. AAM is basically a benign tumor usually treated by surgical resection. However, even after apparently complete resection, local recurrence of the tumor often occurs, thus its characterization as “aggressive” angiomyxoma. In contrast, vulvar edema is a condition with a very similar appearance that is observed relatively commonly, and which has many causes, e.g., obesity, inflammation, and benign or malignant tumors. Here we report a case of AAM which was initially mistaken for vulvovaginal edema, and initially believed to arise as a result of an ulcerative vaginal lesion associated with the patient’s underlying Behcet’s disease. We have reviewed the literature concerning vulvovaginal edema. We present herein this work to others as a precautionary tale, to prevent similar misdiagnosis and delay of treatment.

Summary We analyzed the clinicopathologic features of 10 cases of vulvovaginal myofibroblastoma to widen its morphological and immunohistochemical spectrum. Most tumors (8/10 cases) were located in the vagina. The patients' age ranged from 44 to 77 years, and tumor size ranged from 0.4 to 3 cm. Histologically, 5 tumors had the characteristics of vulvovaginal myofibroblastoma. In addition, we identified 3 cases composed of spindle-shaped cells arranged in short fascicles with intervening thick collagen bands, closely reminiscent of mammary myofibroblastoma. Notably, 1 case resembled Sertoli cell tumor, sclerosing type, because of its predominant cord-like arrangement. In another case, there were highly cellular areas composed of uniform-packed, rounded cells that, at low magnification, looked like a malignant "small round blue cell tumor." A variably thick band of native connective tissue separated tumors from the overlying squamous epithelium even if, in 3 cases, tumor cells extended up to the epithelium. In 7 cases, a variable number of vessels showed perivascular hyalinization. Only rare mitotic figures were identified. All tumors were diffusely positive for vimentin, desmin, and CD99. A variable staining intensity was observed for CD34, Bcl-2, B-cell lymphoma 2 (Bcl-2) CD10, estrogen receptor, and progesterone receptor in most cases, but none expressed α -smooth muscle actin. We emphasize that vulvovaginal myofibroblastoma encompasses a morphological spectrum wider than previously described. The overlapping morphological and immunohistochemical features of vulvovaginal and mammary myofibroblastomas led us to speculate that these are related entities with morphological variations on a common basic theme likely dependent on anatomical location.

Angiomyofibroblastoma is a rare, benign and recently defined soft tissue tumor. It generally arises from superficial soft tissues, especially from mesenchymal cells in the subepithelial myxoid stromal zone of the vulvovaginal region. The tumor is usually positive for estrogen and progesterone receptor. We report a pregnant woman who developed vulvar angiomyofibroblastoma within 2 months. The tumor was negative for estrogen and progesterone receptor.

Angiomyofibroblastoma (AMFB) is a rare benign mesenchymal tumour that occurs almost exclusively in the vulvovaginal region of women but can also occur occasionally in the inguinoscrotal region of men. It is a well-circumscribed lesion that clinically is often thought to represent a Bartholin's gland cyst and usually does not form a pedunculated mass. To our knowledge, only five cases of vulvar AMFB with pedunculated mass have been reported in the English literature and all cases involving the labia majora and middle-aged women. We report the first case of pedunculated AMFB of the vulva occurring in a young woman of 21 years old and involving the left labia minora. After excluding the most common diseases, pedunculated AMFB should be part of differential diagnosis in the workup of any pedunculated vulvar mass even in young women with a lesion involving the labia minora. We reviewed the literature and summarized all reported cases.

Fibro epithelial stromal polyps which are benign mesenchymal lesions that typically occur in skin, oral cavity, urinary tract and genital area pose diagnostic problems because of bizarre cytomorphology. Genital presentation has a greater predilection for the vulvovaginal region and rarely the cervix. Cases of fibro epithelial polyp in vulva have been rarely reported and usually they do not grow larger than 5 cm in diameter and are found incidentally during routine gynecologic exams. Diagnosis mainly based on histopathology has to be done to rule out malignancy and also to differentiate from other lesions like angiomyxoma which mimics fibro epithelial polyps. The case report presented is a rarest of its kind as regards to its size because the polyp in this patient measured 35x23x11 cm and weighing 10 kg which becomes the largest polyp reported ever.

ngiomyofibroblastoma (AMF) is described as a superficial tumor of the vulva that occurs almost exclusively in the vulvovaginal region. It may also occur on rare occasions in the inguinoscrotal region. The first case was described in 1998, 1 in the form of a mesenchymal tumor of the male genital tract. It was histomorphologically similar to female AMF and to spindle cell lipoma. We report a case of AMF that was confirmed pathologically, along with a review of the cases available in the literature.

Mucosal lichen planus (LP) is a rare variant of LP, itself a common skin disorder. Two-thirds of those affected have an erosive form of LP that causes severe vulvovaginal and, in some cases, oral mucosal symptoms, including burning, pain, and pruritus. The disease affects the vaginal orifice and often, the labia minora. Patients present with erythematous, partly erosive, desquamative lesions that may have a pale border. Erosive LP is thought to be an autoimmune disorder. In time, vulvovaginal LP causes scarring and narrowing of the vagina. Treatment of vulvovaginal LP has proved to be challenging. The authors report 5 women with severe symptoms of vulvar scarring and resultant stenosis or obliteration of the vagina, all of whom responded, at least in part, to treatment with surgical dilatation, methotrexate, and local preparations such as tacrolimus and a potent steroid cream. Methotrexate, given in a weekly dose of 7.5 mg, also benefited oral lesions. Stenotic changes decreased during methotrexate therapy, suggesting that it be started as soon as possible after surgical dilatation of the vagina. Topical steroids proved helpful in some patients having exacerbations of the disorder. Tacrolimus, a topical immunosuppressant, is active against vulvar LP and has helped to prevent narrowing of the vulva and vagina in patients failing to respond to a potent local steroid. Local treatment with pimecrolimus and cyclosporin has reportedly been effective. In addition, patients were asked to wash the genital area with emollients, and local estrogen treatment was given to prevent vaginal atrophy. In the present patients, methotrexate appeared to exert positive effects even after it was discontinued. The only adverse side effect was hair loss in 1 patient. All the women took folic acid while receiving methotrexate. This experience endorses the use of low-dose methotrexate in patients with severe stenosing vulvovaginal LP if surgical dilatation and local treatment have failed. It should be continued until symptoms have resolved, and may be d if the disorder recurs. These patients require close follow-up because of the possibility that vulvar LP will undergo malignant transformation.

Aggressive angiomyxoma (AAM), a distinctive neoplasm occurring in the female perineum and pelvis, was initially described in 1983. Subsequently reported to occur in the inguinoscrotal region in men, this uncommon specialized genital stromal tumor is characterized by a paucicellular spindle cell proliferation separated by loose myxoid stroma with a prominent thick-walled, hyalinized vascular component. Despite the bland histologic appearance, AAM is an infiltrative tumor with a propensity for aggressive local and regional recurrences, especially when incompletely excised. Due to involvement of the deep pelvic soft tissues and retroperitoneal regions, these tumors often attain a large size and may not be amenable to complete surgical excision. While AAM can be very difficult to distinguish histologically from other myxoid soft tissue lesions of the vulvovaginal region, especially from the so-called benign angiomyofibroblastoma, differences in the clinical behavior between these various neoplasms require accurate diagnosis to ensure proper treatment. A variety of immunohistologic strategies have been proposed to be of value in differentiating these various tumors, but in many instances their relative contribution is overemphasized. In problematic cases, careful evaluation of a few key histologic features, in concert with attention to a series of practical considerations, including patient age, determination of adequacy of biopsy and tumor sectioning, tumor size, borders, and location (superficial versus deep), should permit accurate classification.

Aggressive angiomyxoma (AA) is a soft-tissue tumor of mesenchymal origin, not so common, but it occurs and exhibits marked tendency for local recurrence with extremely low risk of distant metastasis. The most common sites of origin include vulvovaginal region, perineum, and pelvis of reproductive age females. We report a case of AA in a 36-year-old female presenting with polypoidal vulval growth on the right side with a previous history of surgical resection at the same site 4 years before. Fine needle aspiration cytology of the growth revealed only blood and no cellular component. The tumor was excised and submitted for histopathological examination. A diagnosis of aggressive angiomyxoma was made based on characteristic histological features

Angiomyofibroblastoma (AMF) is a rare, benign, soft-tissue tumor, which predominantly occurs in the vulvovaginal region of middle-aged women. It is clinically important to distinguish an AMF from other stromal cell lesions. Here, we report the case of a 32-year-old woman with a rare, giant pelvic AMF, which showed a benign clinical course. The tumor was located in the cul-de-sac of Douglas. It was well demarcated, hypocellular, edematous and composed of spindle-shaped and oval stromal cells aggregating around thin-walled blood vessels. The tumor cells had abundant eosinophilic cytoplasm, and expressed estrogen receptors, progesterone receptors and desmin. Mitotic figures were absent. It is important to distinguish AMFs from aggressive angiomyxomas because both occur at similar sites but show different clinical behaviors. Most AMFs and aggressive angiomyxomas have the same immunohistochemical phenotype. The well-circumscribed borders of AMF are the most important characteristic that distinguish it from aggressive angiomyxomas. AMFs rarely recur after complete surgical excision. The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/5510813471244189 .

The vulvovaginal region is a very complex area because of its anatomy and the fact that it can be source of a large number of benign tumors and less frequent malignant tumors. In recent decades, there have been significant gains in the diagnosis and treatment of vulvovaginal tumors, thanks to the contributions of cellular and molecular biology. The purpose of this review article is to serve as a practical reference for clinical and pathological diagnosis as well as in the outpatient treatment and monitoring of these benign tumors.La región vulvovaginal es una zona muy compleja por todos los elementos que la conforma y puede ser origen de un gran número de tumores benignos y en menor frecuencia de tumores malignos. En las últimas décadas, los tumores vulvovaginales han tenido un avance significativo en cuanto a su diagnóstico y tratamiento, dado por las aportaciones de la biología celular y molecular. El presente artículo pretende ser una referencia práctica para el diagnóstico clínico e histopatológico de estos tumores benignos así como en el tratamiento ambulatorio de algunos de ellos y su vigilancia.

We report an 84-year-old Japanese woman who presented with a pedunculated malignant melanoma of the vaginal mucosa. Mucosal melanoma is believed to be more common in Japan than other countries, but such tumors of the vulvovaginal region are quite unusual. In our patient, three tumors were connected by a narrow pedicle. Three black tumors measuring 5-10 mm in diameter with a common pedicle were seen on the vaginal mucosa at five o'clock from the cervix. The tumors were hanging from the narrow pedicle. On histologic examination, they were diagnosed as malignant melanoma. Resection was done with a distal margin of 3 cm from the tumors and a margin of 1 cm from the cervix. The patient has had no evidence of local recurrence or distant metastasis. In our patient, the three main tumors had a common pedicle, which seems to be a unique finding. Since pedunculated malignant melanomas are rare, making a clinical diagnosis is difficult. Although pedunculated melanomas are recognized as having a high malignant potential because these lesions are generally thick, a relatively good outcome is sometimes reported. In our patient, there was no tumor infiltration into the dermis of the pedicle, and this may be one reason for the good outcome at present. There has been no previous report of a mucosal melanoma consisting of three tumors like those in the present patient.

Differences in biological behaviour make familiarity with (and accurate diagnosis of) vulvovaginal soft tissue tumours essential. Since ancillary studies such as immunoperoxidase staining and electron microscopy may not always be helpful in their distinction, one must often rely on morphological features to distinguish between different tumour types. This is particularly pertinent with regard to the relatively site-specific stromal tumours of this region. The purpose of this review article is to reacquaint the reader with these specific types of vulvovaginal soft tissue tumour, particularly focusing upon the salient morphological features that help in their distinction, as well as to review their clinical aspects and pathogenesis. The following soft tissue lesions are described: fibroepithelial stromal polyp, cellular angiofibroma, angiomyofibroblastoma, superficial angiomyxoma and aggressive angiomyxoma. Because of continued difficulty in predicting their behaviour, a discussion of vulvar smooth muscle tumours is also included, with a particular focus upon a practical approach to their diagnosis.

Fibroepithelial stromal polyps of the vulvovaginal region display a wide range of morphologic appearances. This morphologic spectrum allows the correct diagnosis to be made in some cases but when histology reveals unusual features, the lesion may be misinterpreted as malignant. We present a case of a 35-year-old woman with psoriasis who developed a 15-cm polypoid lesion localized in the left labium. The labial lesion was resected and no recurrence was detected 12 months after the initial treatment. Histologically, the lesion exhibited the characteristics of a fibroepithelial stromal polyp with focal myxoid areas and underlying vulval psoriasis disease. The clinical, microscopic, and immunohistochemical findings of this case suggest a fibroepithelial polyp of the vulva. This vulval lesion represents a unique example of giant fibroepithelial stromal polyp developed in association with psoriasis.

Angiomyofibroblastoma represents a rare, benign mesenchymal tumor with a predilection for the vulvovaginal region. Lipomatous change may occur but rarely comprises a substantial component of the lesion. There are only eight reports in the English language literature describing the lipomatous variant of this tumor. We describe a further lipomatous angiomyofibroblastoma that occurred on the labium majus of a 49-year-old woman. The histopathologic and immunohistochemical features are described, and the collective experience in the literature is reviewed.

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.

Ischemic fasciitis is a benign reactive lesion that most commonly occurs in elderly, immobile patients in weight-bearing areas that are subject to intermittent ischemia with subsequent tissue breakdown and regenerative changes. The lesion can be clinically and pathologically mistaken for malignancy. Here we describe the first reported case of ischemic fasciitis of the vulvovaginal region in a 20-year-old paraplegic woman who presented with a clinically suspicious vulvar swelling. Histologic examination showed surface ulceration and underlying fibrinoid necrosis with surrounding reactive atypical fibroblast-like cells and small blood vessels, the characteristic histologic features of ischemic fasciitis.

The wide variety of mesenchymal lesions that involve the vulvovaginal region can result in diagnostic difficulties for pathologists, in part because of their relative rarity but also because of overlapping morphological features. They can be divided into those lesions which are specific to or characteristic of this site and those which can occur at any site with no predilection for the vulvovaginal region. Many of the site-specific or characteristic lesions are thought to arise from the specialized subepithelial stroma of the lower female genital tract that extends from the cervix to the vulva; the stromal cells of this region are hormone responsive and exhibit positive immunohistochemical staining with estrogen receptor (ER) and progesterone receptor (PR). As a consequence, most of the site-specific mesenchymal lesions are positive with ER and PR. Immunoreactivity with both desmin and CD34 is also common; this constitutes an unusual immunophenotype since mesenchymal lesions at other sites are uncommonly positive with both markers. The best known of the site-specific lesions is aggressive angiomyxoma, an infiltrative neoplasm with a marked propensity for local recurrence following excision. Recent developments with regard to aggressive angiomyxoma include the description of occasional metastasizing cases, the potential value of gonadotropin-releasing hormone agonists in management and the emergence of HMGA2 as a valuable diagnostic marker. Most of the other site-specific mesenchymal lesions are well circumscribed and exhibit little tendency for local recurrence. These include angiomyofibroblastoma, fibroepithelial stromal polyp, superficial myofibroblastoma of the lower female genital tract, and cellular angiofibroma. Smooth muscle tumors also occur in this region and are more likely than their uterine counterparts to have an epithelioid or myxoid appearance. Vulvovaginal smooth muscle neoplasms exhibit a propensity for local recurrence and the morphological features which predict malignant behavior differ from uterine smooth muscle neoplasms. Relatively, recently described mesenchymal lesions in the vulvovaginal region include massive vulval edema, prepubertal vulval fibroma, and reactive fibroblastic and myofibroblastic proliferation of the vulva (cyclist’s nodule). Gastrointestinal stromal tumors have been described as primary neoplasms in the rectovaginal septum and vagina (termed extragastrointestinal stromal tumors). A wide range of other mesenchymal lesions potentially occur in this region, and it is stressed that when dealing with a vulvovaginal mesenchymal lesion, as well as considering the site-specific lesions, pathologists should consider a wide range of diagnoses since many mesenchymal lesions potentially occur in this region.

Aggressive angiomyxoma is a rare mesenchymal neoplasm, which almost always occurs in the vulvovaginal region and which exhibits a marked tendency for local recurrence after excision. A wide range of other mesenchymal lesions occur in this region, which potentially mimic aggressive angiomyxoma to a variable extent. Because rearrangement of the HMGA2 gene has been shown in a significant percentage of aggressive angiomyxomas, we examined the expression of HMGA2 protein in this neoplasm and in a large number of other mesenchymal lesions occurring at this site, including many which were referred with a possible diagnosis of aggressive angiomyxoma. There was positive staining of tumor cell nuclei, mostly with a diffuse distribution, in all but 2 aggressive angiomyxomas (n=12). Blood vessel endothelial cells within the neoplasms, entrapped tissues, and surrounding normal tissues were negative. Ten of 23 leiomyomas were positive, as were occasional other neoplasms. Based on our study, we conclude that HMGA2 is positive in most aggressive angiomyxomas and is useful in diagnosis as most mesenchymal lesions which closely mimic this are negative. However, caution should be exercised as some other vulvovaginal mesenchymal lesions, especially, but not exclusively leiomyomas can be HMGA2-positive. As well as being of value in primary diagnosis, HMGA2 is useful in evaluating margins and in reexcision specimens of aggressive angiomyxoma in identifying foci of residual or recurrent tumor.

This review discusses recent developments in vulvovaginal pathology. A variety of morphologically bland mesenchymal lesions occur at this site with considerable histological and immunohistochemical overlap. Aggressive angiomyxoma exhibits HMGA2 immunoreactivity in approximately 50% of cases, and this nuclear transcription factor is emerging as a useful and relatively specific marker for aggressive angiomyxoma, although occasional vulvovaginal smooth muscle neoplasms are positive. HMGA2 is useful in the diagnosis of aggressive angiomyxoma and its distinction from mimics, in the evaluation of resection margins and in the assessment of the presence or absence of residual disease in re-excisions. Aggressive angiomyxoma is almost invariably positive with oestrogen and progesterone receptors, and there have been several reports of a dramatic reduction in size following gonadotropin releasing hormone agonist therapy. Recent series of the relatively newly described entities cellular angiofibroma and superficial myofibroblastoma of the lower female genital tract have expanded upon the morphological spectrum of these neoplasms. Recently described mesenchymal lesions at this site include massive oedema and prepubertal vulval fibroma. Gastrointestinal stromal tumours have been described as primary neoplasms in the vagina, and rectovaginal septum and extragastrointestinal stromal tumour should be added to the differential diagnosis of a vulvovaginal mesenchymal lesion. Many mesenchymal lesions in the vulvovaginal region exhibit immunoreactivity with both CD34 and desmin, a somewhat unusual immunophenotype in mesenchymal lesions at other sites. It is now established that there are two distinct types of vulval intraepithelial neoplasia (VIN), most commonly termed classic and differentiated VIN, the former associated with human papillomavirus (HPV) infection. There are two corresponding types of vulval squamous carcinoma with HPV-associated and non-HPV-associated variants, the latter often arising in a vulval dystrophy and associated with p53 mutation. However, in some cases there is clinicopathological overlap between HPV-associated and non-HPV-associated squamous carcinomas, and immunohistochemistry with p16 is more reliable than morphology in predicting the presence of HPV. There have been new developments regarding Paget's disease of the vulva with the identification of markers that are useful in diagnosis and evidence that the neoplastic cells represent a proliferation of adnexal stem cells residing in sebaceous units. The newly described entity vaginal tubulo-squamous polyp typically exhibits immunopositivity with prostatic markers, possibly indicating derivation from displaced periurethral Skene's glands.

Rhabdomyoma is a rare benign tumor characterized histologically by striated skeletal muscle. Rhabdomyomas can be classified into adult and fetal variants. The majority of adult variants arise in the head and neck region and are derived from the skeletal muscles of the pharyngeal (branchial) arches. The majority of cases of fetal variant rhabdomyomas have been described in the postauricular region of young children and the vulvovaginal region in middle-aged women. We present a case of a 15-month-old infant with a circumferential rhabdomyoma of the cricopharyngeus muscle requiring excision of the esophageal inlet and reconstruction with a free microvascular jejunal interposition graft. This case represents the youngest reported case of adult variant rhabdomyoma. The clinical, radiographic, and histologic findings of this unusual case and a review of reported cases of rhabdomyoma in the pediatric population will be presented.

Vulvodynia was defined by the International Society for the Study of Vulvovaginal Disease (ISSVD) in 2003 as vulval discomfort, described as burning, stinging or irritation within the vulvovaginal region. This definition was to be in the absence of visible findings or identified clinical signs of neurological conditions. The condition can either be localised or general, with the discomfort experienced, being spontaneous or provoked by physical contact. Other causative potential diagnosis include infections and dermatological issues. Importantly psychosexual concerns are to be ruled out as a causative nature. More often than not, the woman ends up on a long and arduous journey from General Practitioner, to Gynaecologist, onto a Physiotherapist and to a Psychologist. Too infrequently, she may be referred to a Sexologist. Although a multidisciplinary approach is ideal to address the various often layered problems that gives rise to such a diagnosis of vulvodynia, from a retrospective clinical observation, it would be more beneficial to introduce the concept of a sexologist directly. Moreover, many women appear to be labelled with this diagnosis when in fact, it is often a complex psychosexual issue peppered with relationship difficulties. The label of vulvodynia has the negative ability to cause increased emotional trauma, fear, anxiety and guilt. Subsequently, causing sexual problems rather than answers. So who begins this discussion with the woman? Who opens the line of communication up to explore the layers? More importantly, why should we? Disclosure of interest statement Nothing to Declare.

Background The relatively site-specific mesenchymal lesions of the vulvovaginal region can exhibit superficially overlapping histological features and can be diagnostically challenging. Fibroepithelial stromal polyp is generally an easily recognizable entity, but certain cases cause differential diagnostic problems. Case We present a case of a 16-year-old girl with a 10-cm polypoid lesion localized to the left labium. The patient has therapy resistant congenital lymphedema localized to the left arm and leg. The labial lesion was resected and recurred after 12 months and 6 years following the initial treatment. Histologically, it exhibited characteristics of a fibroepithelial stromal polyp with scattered bizarre multinucleated giant cells and ectatic tortuous lymphatic spaces. Conclusion This vulvar lesion represents a unique example of giant fibroepithelial stromal polyp developed in association with Nonne–Milroy–Meiges syndrome.

Background Solitary fibrous tumour (SFT) is a ubiquitous mesenchymal neoplasm which rarely occurs in the vulvovaginal region. There are a variety of other vulvovaginal mesenchymal lesions which exhibit morphological overlap with SFT and which often express CD34. Recently NAB2STAT6 gene fusions have been demonstrated in the vast majority of SFTs. This results in nuclear expression of STAT6 which is considered to be a sensitive and specific immunohistochemical marker of SFTs. Aims To evaluate STAT 6 immunohistochemical expression in a series of vulvovaginal mesenchymal lesions to ascertain whether this marker is of value in distinguishing SFT from other lesions. Methods and results STAT6 immunohistochemical staining was performed on 50 vulvovaginal mesenchymal lesions. Four of four SFTs (100%) exhibited diffuse nuclear immunoreactivity. Other positive lesions were cellular angiofibroma (50%), aggressive angiomyxoma (85%), leiomyoma (33%), superficial cervicovaginal myofibroblastoma (88%), fibroepithelial polyp (62%), prepubertal fibroma (50%) and angiomyofibroblastoma (50%). All other lesions were STAT6 negative. Conclusions While STAT6 is positive in all vulvovaginal SFTs, it is commonly positive in a variety of other mesenchymal lesions at this site limiting its diagnostic value. Future studies should investigate NAB2STAT6 gene fusion status in vulvovaginal mesenchymal lesions which are STAT6 positive.

Primary appearance of a malignant lymphoma in the vulvovaginal region is extremely rare. In this paper, we report on three such cases we observed during the last few years. There was no difference in clinical manifestation compared to cancer of the vulva/vagina. The diagnosis was made using histological and immunohistochemical methods. Choice of the treatment depended upon the stage of malignancy and spreading of the tumourous changes (radiation, chemotherapy). On reviewing the literature of the last 15 years, we could not find any description of primary malignant lymphoma in the vulva and vaginal region.

Extracardiac rhabdomyomas are rare benign neoplasms that in the past have been divided by histologic criteria into adult and fetal types. In this series, 15 previously unpublished cases are presented and analyzed in combination with 51 acceptable cases from the literature. The adult type of rhabdomyoma occurs almost exclusively in the head and neck region (93% of all cases), particularly in the larynx and pharynx of adult males. The fetal type of rhabdomyoma may be subdivided on histologic grounds into myxoid and cellular variants. The fetal myxoid type usually occurs in the vulvovaginal region of middle aged women or in the postauricular region of male infants. The fetal cellular type has not been previously emphasized. This type tends to occur in the head and neck region of adult males. It may be mistaken for a sarcoma because of its high degree of cellularity. Local recurrence of a rhabdomyoma is rare and has only been occasionally reported in the adult type of rhabdomyoma. In no instance has a rhabdomyoma been shown to possess aggressive local growth or metastatic potential. Therefore, it is essential that these neoplasms be recognized histologically and that they be treated conservatively by local excision.

Iodine chloride hydroxyquinoline and chlorquinaldol are antimicrobial agents added to topical pharmaceutical preparations, including external preparations containing corticosteroids. Derivatives of hydroxyquinoline are also used as disinfectants for the pharynx (lozenges, mouthwashes, and gargles), the intestinal tract, the vulvovaginal region, and wound cavities. Some compounds are used to disinfect the hands and floors and to preserve sera and tobacco.

The frequency with which gonococcal vulvovaginitis is seen in the young, the difficulty often experienced in demonstrating the specific micro-organism, and the extreme chronicity of this form of neisserian infection are known to most workers in this field of medicine. Vulvovaginitis may be due to various organisms, but, unfortunately, in the large majority of cases occurring among children, the gonococcus is the exciting factor. The combined statistics of Hurdon, 1 Koplik, 2 Romniceanu and Robin, 3 Dukelski 4 and Plomley 5 show that, in a large series of cases, 80.29 per cent. were due to the gonococcus of Neisser. For this reason, and because of the difficulty with which, as a rule, the organism can be demonstrated, all suspicious cases of inflammation in the vulvovaginal region should be treated as if they were of specific origin, until they are proved to be nonspecific. This is especially important in institutional work