Subcutaneous Nodules of the Vulva.

A 32-year-old patient complained of three slow-growing subcutaneous nodules on her right labius majus, present for 3 years. Her past medical history was unremarkable. Cutaneous examination revealed three subcutaneous nodules of 1 cm diameter firmly adherent to the underlying tissues, located on her right labium majorum (Figure 1). Regional lymph nodes were not enlarged. She underwent an excision biopsy of a subcuta-neous nodule under local anesthesia. The gross specimen was firm, white and fleshy in appearance. A skin biopsy was performed, and histological findings revealed a non-encapsulated dermal nodule composed of clusters of polygonal cells with small central nuclei and abundant eosinophilic cytoplasm (Figure 2a). The tumor cells formed sheets and nests irregularly infiltrating between collagen bundles. There was no significant cyto-logic atypia and mitotic features. There were no necrosis and hemorrhage. The cells were positive for S-100 immunostain (Figure 2b). Hence, the diagnosis of benign vulvar granular cell tumor was assessed. The patient underwent surgical excision of the subcutaneous nodules with no recurrence at 2 years.

Study of 138 vulvar lichen sclerosus patients and the malignant risk transformation.

Objective: To report the prevalence of malignant transformation of vulvar lichen sclerosus (VLS) and possible risk factors. Methods: This is a cohort study with data analysis from medical records of 138 patients with histological diagnosis of VLS registered at the Vulvar Pathology Outpatient Clinic of the University Hospital, between 2007 and 2017. Predominance of risk factors was performed using logistic regression analysis. The variables studied were the length of follow-up, age, regular or irregular follow up; presence of symptoms (dyspareunia, pruritus and/or vulvar burning); histology characteristics, the presence of epithelial hyperplasia; and the presence of autoimmune diseases. Results: There were 138 patients included in the study, and among them five progressed to malignant transformation. The patients had a median age of 59 years and 83% were symptomatic. The most frequent symptom was itching with 72%. Autoimmune diseases were present in 11.6%, the most prevalent being thyroid disease. All five case of malignant transformation (0.6%) had an irregular follow up. The logistic regression analysis was used among the studied variables, and no statistical significance was found among them (p ≥ 0.05). The relationship between hyperplasia and the clinical outcome of malignant transformation, in which non-significant but acceptable p value close to 0.05 was observed. Conclusion: The prevalence of malignant transformation in patients with VLS was 0.6%, and common factors were the lack of adherence to medical treatments and the loss of follow-up.

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.

Transmissible venereal tumor of the uterine stump following successful chemotherapy in a 5-year-old mixed-breed dog.

A 5-year-old spayed female mixed-breed dog was referred to the Atlantic Veterinary College (Charlottetown, Prince Edward Island) because of a 7-month history of intermittent pink, mucoid, vulvar discharge. The dog was imported from the Bahamas at 3.5 y of age and had a history of transmissible venereal tumor (TVT) of the vulva that was successfully treated with a course of vincristine chemotherapy. Complete remission was achieved with a disease-free interval of 6 mo before clinical signs recurred. Abdominal ultrasound and CT scan identified a large caudal abdominal mass thought to arise from the uterine stump. An exploratory laparotomy was performed and the mass grossly excised. Histopathology was consistent with a poorly differentiated round cell tumor, and immunohistochemical analysis confirmed TVT as the most likely diagnosis. No further treatment was carried out. Repeat abdominal ultrasound at 4 mo after surgery showed no evidence of mass recurrence. At 8 mo after surgery, the dog was reported to be doing well clinically. Key clinical message: Transmissible venereal tumor should be considered as a differential diagnosis for masses arising from the deep genital tissues of dogs in cases where there is a history of previous TVT. Transmissible venereal tumor should be considered even in dogs that have had complete resolution of a primary mass after chemotherapy.

Tumeur vénérienne transmissible du moignon utérin à la suite d'une chimiothérapie réussie chez un chien croisé de 5 ans.Une chienne de race mixte de 5 ans, stérilisée, a été référée au Atlantic Veterinary College (Charlottetown, Île-du-Prince-Édouard) en raison d'antécédents de pertes vulvaires roses, mucoïdes et intermittentes depuis 7 mois. Le chien a été importé des Bahamas à l'âge de 3,5 ans et avait des antécédents de tumeur vénérienne transmissible (TVT) de la vulve qui a été traitée avec succès par une chimiothérapie à la vincristine. Une rémission complète a été obtenue avec un intervalle sans maladie de 6 mois avant la réapparition des signes cliniques. L'échographie abdominale et la tomodensitométrie ont identifié une grosse masse abdominale caudale qui proviendrait du moignon utérin. Une laparotomie exploratoire a été réalisée et la masse excisée. L'histopathologie était compatible avec une tumeur à cellules rondes peu différenciée et l'analyse immunohistochimique a confirmé la TVT comme le diagnostic le plus probable. Aucun autre traitement n'a été effectué. Une échographie abdominale répétée 4 mois après la chirurgie n'a montré aucun signe de récidive massive. Huit mois après l'opération, la chienne se portait bien cliniquement.Message clinique clé:Les tumeurs vénériennes transmissibles doivent être considérées comme un diagnostic différentiel pour les masses provenant des tissus génitaux profonds des chiens dans les cas où il existe des antécédents de TVT. Une tumeur vénérienne transmissible doit être envisagée même chez les chiens dont la masse primaire a complètement disparu après chimiothérapie.(Traduit par Dr Serge Messier).

Extramammary breast cancer of the vulva.

A woman in her 70s was seen in the gynaecology outpatient clinic with a swelling on the right side of the vulva. Surgical excision of the lesion revealed unexpectedly an extensive ductal carcinoma in situ with a focus of a grade 2 invasive ductal carcinoma arising in extramammary breast tissue of the vulva. Postoperative staging studies showed normal breasts, with no evidence of disease elsewhere. The patient underwent a wider excision of the right vulva and sentinel node biopsy of the right inguinal region, which revealed no further disease. The patient is currently taking adjuvant hormonal therapy and has remained disease free at 2-year follow-up. This case underscores the importance of considering rare presentations of vulvar malignancies and the necessity for a multidisciplinary approach in managing such cases.

Impact of sentinel node use in lymphedema formation among gynecologic cancer patients.

PURPOSE OF REVIEW: The most common surgical procedure associated with lymphedema formation is the regional lymphadenectomy. One of the advantages of sentinel node biopsy is the reduction of the risk of lymphedema formation. The purpose of this review is to collect and analyze the most relevant and recent evidence of the use of sentinel node biopsy and its implication on the development of postoperative lymphedema in gynecological cancer. RECENT FINDINGS: The current evidence of the use of sentinel node biopsy in cervical cancer to reduce lymphedema is heterogeneous and more data is needed to establish its role.Sentinel lymph node biopsy in endometrial cancer is a staging procedure with lower surgical complications, as well as lymphedema formation; while the results of prospective trials evaluating its impact on quality of life are still lacking.Sentinel lymph node biopsy in vulvar cancer minimizes the need for extensive dissection and reduces the incidence of complications associated with overharvesting of lymph nodes such as lymphedema without compromising oncological outcomes. SUMMARY: The prevalence of lymphedema in gynecological cancer varies based on the surgical treatment or additional therapies applied. Over the past years, one of the most important surgical modifications to decrease lymphedema formation has been implementation of sentinel lymph node technique mainly in vulvar cancer patients.

Different tracers for sentinel node detection in gynecologic oncology.

PURPOSE OF REVIEW: In the past decade, sentinel lymph node (SLN) mapping has progressively substituted full lymphadenectomies in gynecologic oncology. In this article, we review the most relevant and the latest literature on this topic. RECENT FINDINGS: In endometrial and cervical cancer, the current evidence further support the value of indocyanine green (ICG) as tracer of choice for SLN mapping. Experience in vulvar cancer is more limited, with ICG used together with technetium-99 m (Tc-99m) as a dual tracer but ICG, so far, has not been a game changer in this setting as it has been for cervical and endometrial cancer. SUMMARY: For most gynecologic cancers, ICG fluorescence imaging is considered now a days the tracer of choice for lymphatic mapping. However, in early-stage vulvar cancer, SLN biopsy with radioactive tracer continues to be the standard-of-care in lymph node status assessment.

Monocyte infiltration is an independent positive prognostic biomarker in vulvar squamous cell carcinoma.

BACKGROUND: Vulvar squamous cell carcinoma (VSCC) arises after an HPV infection or the mutation of p53 or other driver genes and is treated by mutilating surgery and/or (chemo) radiation, with limited success and high morbidity. In-depth information on the immunological make up of VSCC is pivotal to assess whether immunotherapy may form an alternative treatment. METHODS: A total of 104 patient samples, comprising healthy vulva (n = 27) and VSCC (n = 77), were analyzed. Multispectral immunofluorescence (15 markers) was used to study both the myeloid and lymphoid immune cell composition, and this was linked to differences in transcriptomics (NanoString nCounter, 1258 genes) and in survival (Kaplan-Meier analyses). RESULTS: Healthy vulva and VSCC are both well infiltrated but with different subpopulations of lymphoid and myeloid cells. In contrast to the lymphoid cell infiltrate, the density and composition of the myeloid cell infiltrate strongly differed per VSCC molecular subtype. A relative strong infiltration with epithelial monocytes (HLADR-CD11c-CD14+CD68-CD163-CD33-) was prognostic for improved survival, independent of T cell infiltration, disease stage or molecular subtype. A strong infiltration with T cells and/or monocytes was associated with drastic superior survival: 5-year survival > 90% when either one is high, versus 40% when both are low (p < 0.001). CONCLUSION: A hot myeloid and/or lymphoid infiltrate predicts excellent survival in VSCC. Based on the response of similarly high-infiltrated other tumor types, we have started to explore the potential of neoadjuvant checkpoint blockade in VSCC.

Complications and adverse events in lymphadenectomy of the inguinal area: worldwide expert consensus.

BACKGROUND: Inguinal lymph node dissection plays an important role in the management of melanoma, penile and vulval cancer. Inguinal lymph node dissection is associated with various intraoperative and postoperative complications with significant heterogeneity in classification and reporting. This lack of standardization challenges efforts to study and report inguinal lymph node dissection outcomes. The aim of this study was to devise a system to standardize the classification and reporting of inguinal lymph node dissection perioperative complications by creating a worldwide collaborative, the complications and adverse events in lymphadenectomy of the inguinal area (CALI) group. METHODS: A modified 3-round Delphi consensus approach surveyed a worldwide group of experts in inguinal lymph node dissection for melanoma, penile and vulval cancer. The group of experts included general surgeons, urologists and oncologists (gynaecological and surgical). The survey assessed expert agreement on inguinal lymph node dissection perioperative complications. Panel interrater agreement and consistency were assessed as the overall percentage agreement and Cronbach's α. RESULTS: Forty-seven experienced consultants were enrolled: 26 (55.3%) urologists, 11 (23.4%) surgical oncologists, 6 (12.8%) general surgeons and 4 (8.5%) gynaecology oncologists. Based on their expertise, 31 (66%), 10 (21.3%) and 22 (46.8%) of the participants treat penile cancer, vulval cancer and melanoma using inguinal lymph node dissection respectively; 89.4% (42 of 47) agreed with the definitions and inclusion as part of the inguinal lymph node dissection intraoperative complication group, while 93.6% (44 of 47) agreed that postoperative complications should be subclassified into five macrocategories. Unanimous agreement (100%, 37 of 37) was achieved with the final standardized classification system for reporting inguinal lymph node dissection complications in melanoma, vulval cancer and penile cancer. CONCLUSION: The complications and adverse events in lymphadenectomy of the inguinal area classification system has been developed as a tool to standardize the assessment and reporting of complications during inguinal lymph node dissection for the treatment of melanoma, vulval and penile cancer.

Role of V-Y flap reconstruction in vulvar cancer patients: multicenter retrospective study.

OBJECTIVE: To assess if the use of a V-Y reconstructive flap after excisional radical surgery positively influences the surgical outcomes in patients with vulvar cancer. METHODS: This was a multicenter, retrospective, controlled study. Surgical outcomes and complication rates of women with invasive vulvar cancer who underwent radical surgery and vulvar reconstruction and those who underwent radical surgery without the reconstruction step were compared. Only patients who underwent bilateral or unilateral V-Y advancement fascio-cutaneous flaps were included in the reconstruction group. Univariate and multivariate logistic regression models were used to analyze predicting variables for their association with complication rates. RESULTS: Overall, 361 patients were included: 190 (52%) underwent the reconstructive step after the excisional radical procedure and were compared with 171 (47.4%) who did not undergo the reconstructive step. At multivariate analysis, body mass index >30 kg/m2 (odds ratio (OR) 3.36, p=0.007) and diabetes (OR 2.62, p<0.022) were independently correlated with wound infection. Moreover, increasing age (OR 1.52, p=0.009), body mass index >30 kg/m2 (OR 3.21, p=0.002,) and International Federation of Gynecology and Obstetrics (FIGO) stages III-IV (OR 2.25, p=0.017) were independent predictors of wound dehiscence. A significant reduction in the incidence of postoperative wound complications among patients who underwent V-Y reconstructive flaps was demonstrated. This was correlated more significantly in women with lesions >4 cm. CONCLUSIONS: The adoption of V-Y flaps in vulvar surgery was correlated with reduced surgical related complications, particularly in vulnerable patients involving large surgical defects following excisional radical procedures.

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.

A case of giant labia majora lipoma in an adult.

Lipomas are common benign adipose tissue tumors but are infrequently found in the labia majora, especially at a large size. We report a case of a giant lipoma of the labia majora in a 37-year-old woman diagnosed by imaging methods and successfully treated by surgery. This case highlights the importance of considering lipoma in the differential diagnosis of labial masses and underscores the role of imaging in diagnosis. They need to be differentiated from liposarcoma or omental herniation into Nuck's canal. It also demonstrates the effectiveness of surgical management for large lipomas in sensitive areas, with attention to both medical and aesthetic outcomes.

Nodular Vulvar Lesions.

A woman in her 30s presented with mildly itchy skin nodules in the vulvar region for 1 year, which occurred during pregnancy and increased gradually in size and number without any treatments. What is your diagnosis?

Feasibility study of focused ultrasound in the treatment of vulvar low-grade squamous intraepithelial lesions with persistent symptoms.

OBJECTIVE: This study aimed to investigate the feasibility, efficacy, and safety of focused ultrasound (FUS) for the treatment of vulvar low-grade squamous intraepithelial lesions (VLSIL) with persistent symptoms. METHODS: This retrospective analysis included 24 VLSIL patients who underwent FUS treatment. At each follow-up visit, the clinical response was assessed including changes in symptoms and signs. In addition, the histological response was assessed based on the vulvar biopsy results of the 3rd follow-up. Clinical and histological response were assessed to elucidate the efficacy. RESULTS: A total of 22 patients completed follow-up and post-treatment pathological biopsies. After treatment, the clinical scores of itching decreased from 2.55 ± 0.51 to 0.77 ± 0.81 (p < 0.05). Furthermore, the clinical response rate and histological response rate were 86.4% and 81.8%, respectively. Only two cured patients indicated recurrence in the 3rd and 4th year during the follow-up period and achieved cure after re-treatment. In terms of adverse effects, only one patient developed ulcers after treatment, which healed after symptomatic anti-inflammatory treatment without scarring, and no other treatment complications were found in any patients. None of the patients developed a malignant transformation during the follow-up period. CONCLUSION: This study revealed that FUS is feasible, effective, and safe for treating VLSIL patients with persistent symptoms, providing a new solution for the noninvasive treatment of symptomatic VLSIL.

Neoadjuvant chemotherapy with bevacizumab for locally advanced vulvar cancer.

OBJECTIVES: External beam radiation with sensitizing platinum is the recommended therapy for locally advanced vulvar cancers not amenable to curative surgery and is associated with considerable acute and chronic side effects. Radical vulvectomy post-radiation for persistent disease is often compromised with poor wound healing. We describe clinical outcomes for patients who received neoadjuvant chemotherapy plus bevacizumab followed by radical vulvectomy for locally advanced vulvar cancer. METHODS: We performed retrospective analyses of all patients at our institution who underwent radical vulvectomy from January 2015 to November 2023. Of 113 patients, 13 patients underwent neoadjuvant chemotherapy. Demographics and clinicopathologic data were extracted, and descriptive statistical analyses were performed. Cases with neoadjuvant chemotherapy plus bevacizumab were further evaluated for response, adverse effects, and survival. RESULTS: Neoadjuvant chemotherapy was administered to 13 patients with stage II-IV disease that involved the urethra, vagina, or anus. Lesion sizes ranged from 4 to 20 cm (median 7 cm). Patients received 2-6 cycles of carboplatin or cisplatin, paclitaxel, and bevacizumab. Nine (69.2%) patients had partial pathologic responses, and four patients had complete responses. All patients had negative surgical margins. Ten (76.9%) patients had radiographic evidence of inguinal lymph node metastasis prior to neoadjuvant chemotherapy, and four had residual nodal disease. Only one patient developed a superficial groin seroma. Three patients developed recurrence, two locally and one distant, and there was one death. The median follow-up was 23 months (range 6-84 months). CONCLUSIONS: Neoadjuvant chemotherapy using combination platinum/paclitaxel/bevacizumab was efficacious for locally advanced vulvar cancer, resulting in complete resections, negative margins, and excellent wound healing. A multi-institutional phase II trial is warranted to validate these findings.

Prognostic characteristics, recurrence patterns, and survival outcomes of vulval squamous cell carcinoma - A twelve-year retrospective analysis of a tertiary centre.

INTRODUCTION: Vulval cancer is a rare gynaecological malignancy. In this study, we present a tertiary centre case analysis to examine the recurrence patterns and survival outcomes of vulval squamous cell carcinoma (SCC). METHODS: This is a retrospective cohort study of women who received treatment at Oxford University Hospitals between February 2010 and July 2022 for primary vulval SCC. RESULTS: We included 98 cases. The median age at diagnosis was 68 years. Human Papillomavirus (HPV) infection and lichen sclerosis were observed in 21 and 50 cases, respectively. Surgical excision was the primary treatment. Recurrence within 2 years was more common with advanced stage (p = 0.047, RR = 2.26) and extracapsular lymph node spread (p = 0.013, RR = 2.88). Local recurrence was not associated with a specific cut-off value for tumour-free margin. Poor survival outcomes were observed with higher grade (p = 0.01), advanced FIGO stage (p < 0.001), HPV-independent cancer (p = 0.048), lymph node involvement (p < 0.001, HR = 7.14), extracapsular spread (p < 0.001, HR = 7.93), lymphovascular space invasion (p = 0.002, HR = 3.17), tumour diameter wider than 23 mm (p = 0.029, HR = 2.53) and depth of invasion more than 6 mm (p = 0.006, HR = 3.62). Perineural invasion is associated with shorter disease-free survival. Five-year cancer-specific survival rates for stages I, III, and IV were 90.2%, 40.8%, and 14.3%, respectively.