Vulvar Paget's disease: A population-based cancer registry descriptive study.

BACKGROUND: Although vulvar Paget's Disease (VPD) is a rare skin cancer associated with an excellent prognosis, high recurrence rates are associated with impaired quality of life. OBJECTIVES: Our objective was to investigate the epidemiological and clinical features of VPD diagnosed in a French administrative area (Franche Comté). METHODS: This retrospective study investigated clinical, histologic, therapeutic and follow-up data of patients with VPD diagnosed between 1981 and 2021, including data from the Doubs cancer registry. RESULTS: Among the 21 patients included (19 intra-epithelial and 2 invasive VPD), the median time to diagnosis was 24 months [0-110 months], with a median age of 72 years [38-88 years]. An associated cancer was present in 6 patients (29 %). At 5 years of follow-up, the recurrence rate was 26 %, but then increased to 42 % after a median follow-up of 145 months [31-503 months]. Among the 14 patients first surgically treated, incomplete resection (positive margins) was observed in all patients (100 %), associated with a postoperative recurrence rate of 86 % which was much higher than the rate observed in patients first topically treated (20 %). Postoperative adjuvant therapy (surgical revision, laser, imiquimod) significantly increased the recurrence-free survival (p < 0.001). CONCLUSIONS: Postoperative recurrence of VPD is frequent, mainly after 5 years, proving the importance of prolonged follow-up. Recurrence-free survival was significantly higher after postoperative adjuvant treatment.

Syphilitic gumma presenting as squamous cell carcinoma of Vulva: A case report.

Introduction: Tertiary syphilis, a late form of the disease with neurologic, cutaneous, and cardiovascular manifestations, is rarely seen in the United States in modern times. Also called the "great imitator," syphilis tends to mimic other disease processes, which can lead to a delay in proper diagnosis and treatment. Case: We present the case of a 59-year-old woman who presented for evaluation of possible squamous cell carcinoma of the vulva. After multiple inconclusive biopsies and extensive workup with multiple providers, she was instead found to have cutaneous and neurologic tertiary syphilis. A correct diagnosis was delayed in this patient due to a presentation not consistent with classical teachings and due to a lack of access to care. Conclusion: Syphilis has characteristic signs and symptoms, but in practice, it can have an indolent presentation that may differ from traditional presentations. To our knowledge, this is the first comprehensive case report of vulvar cutaneous syphilis masquerading as squamous cell carcinoma of the vulva. This case also provides additional evidence for the necessity of comprehensive gynecologic care and sexual history-taking in the post-menopausal populations and in rural communities.

Therapeutic effects of focused ultrasound on vulvar squamous intraepithelial lesions in rat.

OBJECTIVE: In this study, we established a Sprague-Dawley rat model of vulvar squamous intraepithelial lesions and investigated the impact of focused ultrasound on the expression of hypoxia-inducible factor-1α (HIF-1α), vascular endothelial growth factor (VEGF) and mutant type p53 (mtp53) in the vulvar skin of rats with low-grade squamous intraepithelial lesions (LSIL). MATERIALS AND METHODS: The vulvar skin of 60 rats was treated with dimethylbenzanthracene (DMBA) and mechanical irritation three times a week for 14 weeks. Rats with LSIL were randomly allocated into the experimental group or the control group. The experimental group was treated with focused ultrasound, while the control group received sham treatment. RESULTS: After 14 weeks treatment of DMBA combined with mechanical irritation, LSIL were observed in 44 (73.33%) rats, and high-grade squamous intraepithelial lesions (HSIL) were observed in 14 (23.33%) rats. 90.91% (20/22) of rats showed normal pathology and 9.09% (2/22) of rats exhibited LSIL in the experimental group at four weeks after focused ultrasound treatment. 22.73% (5/22) of rats exhibited LSIL, 77.27% (17/22) of rats progressed to HSIL in the control group. Compared with the control-group rats, the levels of HIF-1α, VEGF and mtp53 were significantly decreased in experimental-group rats (p < 0.05). CONCLUSIONS: These results indicate that DMBA combined with mechanical irritation can induce vulvar squamous intraepithelial lesion in SD rats. Focused ultrasound can treat LSIL safely and effectively, prevent the progression of vulvar lesions, and improve the microenvironment of vulvar tissues by decreasing the localized expression of HIF-1α, VEGF, and mtp53 in rats.

A Rare Case of Vulval Leiomyoma.

Vulval leiomyomas are exceedingly rare benign smooth muscle tumours, with limited cases reported in the literature. They often present a diagnostic challenge due to their uncommon location and nonspecific clinical presentation, mimicking other vulval pathologies. We present the case of a 40-year-old female who reported a painless, slowly enlarging mass on the left side of the vulva. An excisional biopsy was performed, and histopathological analysis confirmed the diagnosis of vulval leiomyoma. Vulval leiomyomas are rare and may be misdiagnosed due to their similarity to other vulval lesions such as Bartholin cysts, lipomas, or fibromas. The standard treatment is surgical excision, which is often curative.

Ultrasonic features of hidradenoma papilliferum: An unexpected mass in the vulvar.

Hidradenoma papilliferum is a rare superficial mass with distinct ultrasonic features. It originates from mammary structures and is commonly observed in the anogenital region of women. We report a hidradenoma papilliferum with clear ultrasound images which have never be described before.

A rare non-malignant cauliflower vulvar mass in a post-menopausal woman: Case report and insight into vulval fibromatosis.

INTRODUCTION AND IMPORTANCE: Vulvar fibromas are benign tumours that primarily occur in women of reproductive age but very rarely among postmenopausal women. Evidence of its occurrence in Sub-Saharan Africa is scant, with hardly any data among postmenopausal women. CASE PRESENTATION: A 54-year-old multiparous (para 4) Ghanaian female presented at the Gynaecology Outpatient Department of Korle Bu Teaching Hospital, with a three-year history of a painless vulval mass. Her general condition was satisfactory. Vulvar examination revealed a prominent, 20 cm × 15 cm cauliflower-like mass originating from the right labium majus, attached by a 5 cm long and 1 cm thick stalk. There was no inguinal lymphadenopathy. Mass was excised under regional anesthesia and histology confirmed benign vulva fibromatosis. The patient made a satisfactory post-operative recovery. CLINICAL DISCUSSION: This case was managed successfully surgically, and histology confirmed a benign tumour. These benign vulval tumours typically occur in younger premenopausal women, but very rarely after menopause as was in the case of our patient who was 7 years postmenopausal. This further emphasizes the exceptional nature of this pathology. CONCLUSION: Our report adds valuable insight to the limited literature on vulvar fibromatosis, particularly in postmenopausal patients, emphasizing the need for careful diagnostic and management strategies for best patient outcomes.

A Rare Case of Primary Vulval Amelanotic Melanoma Involving the Urethra.

A 60-year-old woman came to the Emergency Department complaining of a vaginal formation. The urologist suspected a urethral caruncle: the patient was discharged with vaginal oestrogen cream to relieve symptoms and a follow-up was suggested. After two months the patient returned to the Emergency Department since the mass was increasing in volume and complaining of dysuria and haematuria. Ultrasound, contrast-enhanced computed tomography, and contrast-enhanced magnetic resonance revealed a mass arising from the mucosa and involving the vulva and the urethra, suspicious of malignancy. We present a challenging diagnosis of an infiltrative and rapidly progressive primary vulval amelanotic melanoma with a complete imaging evaluation and a confirmed histological diagnosis.

MR imaging of benign vulvar lesions: a pictorial essay.

Benign vulvar lesions can be difficult to differentiate with few publications on their imaging appearances. While many vulvar lesions may be clinically diagnosed and treated, more are being detected incidentally with the increasing prevalence of magnetic resonance imaging (MRI) of the pelvis. In addition, clinicians may find imaging of benign vulvar lesions helpful for greater anatomical correlation. After reviewing the important MRI sequences for vulvar imaging and the anatomy of the vulva on MRI, this pictorial essay illustrates variety of cystic and solid benign vulvar lesions to familiarize radiologists with their common MRI appearances. Other miscellaneous pelvic lesions that can affect the vulva are also described.

Simultaneous Endometrial Cancer with Extensive Pelvic and Vulvar Endometriosis: A Case Report.

Background: Endometrial cancer represents the most prevalent malignant genital tract neoplasm in high-income countries and is the second most common cancer worldwide following cervical cancer. Endometriosis is a benign condition wherein endometrial glands and stroma are found outside the uterine cavity. Case Presentation: During a routine care and ultrasound examination of the uterus and adnexa of a 64-year-old woman, an increased endometrial thickness (22 mm) was noted. In 2023, according to ultrasound report, the patient underwent diagnostic curettage with immunohistochemistry, revealing a pathological diagnosis of endometrial cancer (endometrioid adenocarcinoma) with positive staining for p16, estrogen receptor (ER), and vimentin. Subsequently, after one week, she underwent complete surgical staging. Extensive superficial endometriosis disseminated in the pelvis and vulva was noted during surgery and preoperative examinations. Final pathology confirmed a well-differentiated typical endometrioid carcinoma (grade 1) with 40% myometrial invasion and positive lymphovascular invasion. The patient was considered to be at stage 1A. Conclusion: Despite some studies suggesting an unclear association between endometriosis and endometrioid or clear-cell ovarian cancers, the correlation between endometriosis and endometrial cancer and its prognosis remains ambiguous. Additionally, although infertility has been linked to both endometrial cancer and endometriosis in various studies, the presented case exhibited no signs of infertility. Extensive pelvic endometriosis with vulvar involvement was present, yet the patient did not exhibit any symptoms. This is in contrast to the typical initial manifestation of endometrial cancer, which is abnormal uterine bleeding. The patient's condition was incidentally detected through routine care due to an abnormal increase in endometrial thickness, prompting this presentation.

Triangular fossa of the third cerebral ventricle - an original 3D model and morphometric study.

Introduction: The triangular recess (TR), also called triangular fossa or vulva cerebri, represents the anterior extension of the diencephalic ventricle, located between the anterior columns of the fornix and the anterior white commissure. Over time, this structure of the third cerebral ventricle generated many disputes. While some anatomists support its presence, others have opposite opinions, considering that it only becomes visible under certain conditions. The aim of the study is to demonstrate the tangible structure of the triangular recess. Secondly, the quantitative analysis allowed us to establish an anatomical morphometric standard, as well as the deviations from the standard. Materials and methods: Our study is both a quantitative and a qualitative evaluation of the triangular fossa. We dissected 100 non-neurological adult brains, which were fixed in 10% formaldehyde solution for 10 weeks. The samples are part of the collection of the Institute of Anatomy, "Grigore T. Popa" University of Medicine and Pharmacy, Iasi. We highlighted the triangular fossa by performing dissections in two stages at the level of the roof of the III ventricle. Results: The qualitative analysis is a re-evaluation of the classical data concerning the anatomy of the fossa triangularis. We proposed an original 3D model of the triangular recess in which we described a superficial part called vestibule and a deep part called pars profunda. We measured the sides of the communication between the two proposed segments, as well as the communication with the III ventricle. By applying the Heron's formula, we calculated the area of the two communications. Statistical evaluations have shown that these communications are higher than they are wide. In addition, there is a statistical difference between the surfaces of the two communications: 34.07 mm2 ± 7.01 vs. 271.43 mm2 ± 46.36 (p = 0.001). Conclusion: The outcome of our study is both qualitative and quantitative. Firstly, we demonstrated the existence of the triangular fossa and we conceived a spatial division of this structure. Secondly, the measurements carried out establish an anatomo-morphometric norm of the triangular recess, which is useful in assessing the degree of hydrocephalus during the third endoscopic ventriculoscopy.

Genital calcinosis cutis: Microscopic evidence supporting a dystrophic origin.

Calcinosis cutis (CC) is characterized by the deposition of calcium salts in the skin and subcutaneous tissues. CC involving the vulva or foreskin (prepuce) is uncommon. We present a 9-year-old female with vulvar CC and a 15-year-old male with preputial CC. Microscopic review of excisional specimens revealed calcification associated with follicular cysts in the vulvar case and lichen sclerosus in the preputial case, suggesting a dystrophic origin to a subset of cases of genital CC that might otherwise be classified as idiopathic. The clinical implication of these findings is the need for close histopathologic scrutiny and ongoing clinical surveillance of patients with genital CC initially deemed idiopathic.

Modified clock mapping biopsy sec. Della Corte-Bifulco in the preoperative assessment of excisional surgery for vulvar Paget's disease.

We have developed a biopsy technique aimed at preoperative evaluating the extent of Paget's vulvar disease in order to plan subsequent radical vulvar surgery. The aim is to find all possible lesion sites that are not visible macroscopically, to obtain a clear evaluation of the disease spread and to tailor the radical surgical procedure to remove even microscopic lesions, avoiding recurrences and excessively destructive surgery, adopting as conservative an approach as possible. We used this procedure for the first time to establish the radicality of the surgical intervention in a 68-year-old patient initially suffering from a single invasive vulvar Paget's lesion.

Unveiling the Truth: Fibroepithelial Polyp of the Vulva and Its Misdiagnosis as Cancer.

Fibroepithelial polyps (FEPs) are peculiar benign lesions that typically present as painless, pedunculated growths in various regions of the body, including the lower female genital tract. We discuss a case of a 45-year-old menopausal female who presented with an FEP in her vulva. The patient reported noticing a painless growth in her vulvar region for the past seven years, which had gradually increased in size. Clinical examination revealed a polypoidal, pedunculated, fleshy mass measuring approximately 11x8x7 cm in diameter. The lesion was excised under anesthesia, and histopathological examination confirmed the diagnosis of FEP. The patient had an uneventful postoperative course and showed no evidence of recurrence.

Myoepithelioma-Like Tumor of the Vulvar Region: A Clinicopathologic Study of Four Cases.

Myoepithelioma-like tumors of the vulvar region (MELTVR) are solid tumors found in the vulva of adult women. They have a similar histopathology to myoepithelioma but differ in immunohistochemical phenotype and genetic changes. In this study, we report four examples of MELTVR, occurred in the external genitalia and mons pubis of adult women aged 32 to 39 years. The tumors presented as subcutaneous masses without obvious tenderness. The tumors were composed of a mixture of myxoid and nonmyxoid components, and myxoid areas accounted for 5% to 80% of the tumor volume. The tumor cells were spindle-shaped or epithelioid, with abundant cytoplasm, vesicular nuclei, and small nucleoli. The nuclear atypia was mild to moderate, with 0 to 10 mitotic figures per 10 high-power fields. Immunohistochemically, all four tumors showed consistent positivity for EMA, calponin and ER; three tumors exhibited PR expression. All tumors were negative for S100 protein and SMA. AE1/AE3 expression was absent in all except one tumor, which showed rare positivity. SMARCB1/INI1 expression was deficient in all tumors. EWSR1 and FUS rearrangements were absent. All tumors were treated through surgery. All patients were alive without recurrence on most recent follow-up. Together, this overview of four additional tumors of MELTVR offers further insight into this rare and poorly understood disease.

The Rarity in the Rarity: Presentation of Two Rare and Unusual Cases of Nodular Fasciitis and Proliferative Fasciitis.

Nodular fasciitis (NF) and proliferative fasciitis (PF) are benign, reactive mesenchymal neoplasms that can mimic malignancies due to their rapid growth and histological characteristics. NF typically affects the subcutaneous tissue, occasionally involving muscles and fascia, predominantly in young adults, and appears frequently in the upper extremities, trunk, and head/neck. PF, a pseudosarcomatous lesion, primarily occurs in the subcutaneous tissue of adults aged 40-70 years and is uncommon in younger populations. This article presents two pediatric cases of NF and PF in unusual locations: a six-year-old girl with a vulvar NF and a 10-year-old girl with a gluteal PF. Both cases demonstrated rapid growth and distinct histological features, confirmed by immunohistochemical analyses and fluorescence in situ hybridization (FISH). These cases underscore the importance of accurate histological recognition to avoid misdiagnosis and ensure appropriate treatment, highlighting the rarity of such occurrences in children and the need for awareness among clinicians and pathologists.