Massive aggressive angiomyxoma of ischioanal region with relapse: A case report.

Background: Aggressive angiomyxoma (AA) is a rare and slow-growing tumor in the pelvic and perineal regions that might develop into other perineal structures. It can present variably, ranging from a painless mass to non-specific symptoms such as dyspareunia. Due to the high relapse rate, extensive tumoral resection is reasonably required to prevent recurrences. It is also commonly confused with other conditions such as lipomas, Bartholin's gland cysts, and hernias. Objective: A 43-yr-old female diagnosed with AA 10 yr ago was evaluated as a consequence of the tumor recurrence. She presented rare manifestations of a giant and cystic pelvic mass involving pararectal and paravaginal tissue in front of the sacrum. Case Presentation: Although AA is a rare and slow-growing tumor, close observation is recommended due to the high relapse rate. Furthermore, extensive tumoral resection and regular follow-up can reduce morbidity in these patients.

A Case of Systemic Chemotherapy With Paclitaxel/Cisplatin Followed by Wide Local Vulvectomy in Pelvic Lymph Nodes-Related Stage IVB Vulvar Cancer.

Multimodality treatments, including chemotherapy, radiation, and surgery, have been evaluated to reduce the extent of resection and morbidity in patients with advanced vulvar cancer. Here, we report the case of a 55-year-old woman diagnosed with advanced vulvar cancer with inguinal and pelvic lymph node metastasis. She exhibited cancerous labia, which were entirely covered with ulcerated and exophytic lesions of squamous cell carcinoma, and underwent systemic chemotherapy consisting of combined paclitaxel-cisplatin. After eight cycles of this regimen, the tumors had nearly regressed, and we performed a wide local vulvectomy with a plastic musculocutaneous flap. Pathological examination revealed no residual carcinoma in the excised labia, indicating that the chemotherapy elicited a pathological complete response. The paclitaxel-cisplatin regimen may provide sufficient efficacy for selected patients with stage IVB vulvar cancer. In addition, surgical strategies should be tailored to avoid complications associated with extensive surgery and more emphasis should be placed on the patient's expected quality of life.

Contribution of preoperative ultrasound-guided implantation of a magnetic seed for optimal localization and resection of vulvar angiosarcoma: A case report.

INTRODUCTION: Vulvar cancer is a rare cause of malignancy among women. It is key for surgeons to achieve negative resection margins, as it greatly impacts patient's prognosis. Unfortunately, additional surgical procedures are often performed due to the regional anatomical complexity. Based on non-palpable breast tumors, where image-guided preoperative localization tools have enhanced the complete resection rates, we aimed at evaluating the feasibility of magnetic seed technique for localizing perineal lesions. PRESENTATION OF THE CASE: We present the case of a 40-year-old female patient, who underwent iterative resections for a recurrent epithelioid angiosarcoma of the left labia major. Imaging revealed a suspicious regional involvement at 3 months of follow-up, for which another surgery was planned. We decided to target this non-palpable lesion with the Magnetic Seed technique to guide the intervention. A seed was inserted into the nodule under ultrasound guidance. Resection was then performed, with negative margins and no recurrence on last follow-up. DISCUSSION: Surgical procedures with minimal extension are recommended in vulvar cancer, to limit the aesthetic and functional complication. Unfortunately, recurrences and residual tumors remain frequent, even higher when surgical margin safety is not achieved. Many studies have suggested the benefit of image-guided localization tools in non-palpable breast tumors. By reducing the excising volume and focusing on the lesions, relapse and complications are rarer. We considered Magnetic Seed to be the most appropriated technique for perineal lesions. CONCLUSION: As for breast cancer, Magnetic Seed technique could be appropriate for non-palpable perineal lesions, optimizing resection margins with minimal procedures.

Angiomyofibroblastoma as a rare cause of vulvar mass: A case report and literature review.

Key Clinical Message: Angiomyofibroblastoma is a benign soft tissue tumor and a form of genital stromal mesenchymal tumor that primarily affects the vulva. It could possibly affect the reproductive-aged women's lower genital tract (vagina). Abstract: Angiomyofibroblastoma is a rare benign soft tissue tumor primarily affecting the vulva in reproductive-aged women. We report a 67-year-old female complaining of a painless mass in her right vulva spreading to the right inguinal region over the past 2 years. The first clinical impression was a canal of Nuck hernia, diagnostic laparoscopy was planned to rule hernia out. The vulvar mass was excised, and a histopathology examination revealed Angiomyofibroblastoma.

Outcomes and prognostic factors of surgically treated extramammary Paget's disease of the vulva.

OBJECTIVE: Extramammary Paget's disease (EMPD) of the vulva is a rare disease which predominantly presents in postmenopausal Caucasian women. As yet, no studies on Asian female patients with EMPD have been performed. This study aimed to identify the clinical features of patients with vulvar EMPD in Korea, and to evaluate the risk factors of recurrence and postoperative complications in surgically treated EMPD. METHODS: We retrospectively reviewed 47 patients with vulvar EMPD who underwent wide local excision or radical vulvectomy. The clinical data and surgical and oncological outcomes following surgery were extracted from medical records and analyzed. Univariate and multivariate analyses for predicting recurrence and postoperative complications were performed. RESULTS: 21.3% of patients had complications after surgery, and wound dehiscence was the most common. 14.9% of patients experienced recurrence, and the median interval to recurrence from initial treatment was 69 (range 33-169) months. Vulvar lesions larger than 40 mm was the independent risk factor of postoperative complications (odds ratio [OR]=7.259; 95% confidence interval [CI]=1.545-34.100; p=0.012). Surgical margin status was not associated with recurrence in surgically treated vulvar EMPD patients (OR=0.83; 95% CI=0.16-4.19; p=1.000). CONCLUSION: Positive surgical margin is a frequent finding in the patients with vulvar EMPD, but disease recurrence is not related with surgical margin status. Since EMPD is a slow growing tumor, a surveillance period longer than 5 years is required.

Challenges in management of Bartholin gland leiomyoma: A case report.

Leiomyomas are uncommon vulvar neoplasms often misdiagnosed as other Bartholin gland pathology. This case report describes a case of accelerating growth of a vulvar mass, initially diagnosed as Bartholin cyst. Surgical excision led to a histopathologic diagnosis of vulvar leiomyoma. The postoperative recovery was complicated by secondary hematoma and dehiscence of the surgical site. There was no recurrence at 2 years follow-up.

Vulvar malignant melanoma: A case report and review of its management.

Vulvar malignant melanoma (VMM) is uncommon and poses a significant management challenge. Here, we presented a case of VMM managed by surgery, chemoradiation, and planned for targeted therapy. A 70- year-old woman with underlying diabetes mellitus and hypertension presented with a black-colored exophytic growth around her left vulva for two months. Initial biopsy confirmed malignant melanoma with positive staining for S100, HMB 45, and Melan A. An imaging study showed that the disease was localized to the vulva. She underwent bilateral radical vulvectomy and bilateral inguinofemoral lymph node dissection followed by radiotherapy. She had a locoregional disease recurrence, which was subsequently managed by palliative perineal radiotherapy, chemotherapy, and planned for immunotherapy. Vulvar malignant melanoma is a rare and aggressive tumor, with a poor overall prognosis, and high recurrence rate. Adjuvant chemotherapy, radiotherapy, and immunotherapy may be beneficial for local recurrence and distant metastasis cases. Molecular Analysis has a potential role in targeted therapy to improve the survival and outcome of the patient.

Vulvar glomangioma: A case report and literature review.

GLMN is a gene that encodes a critical protein necessary for normal vascular development. Mutations of GLMN predispose individuals to development of glomangiomas, with nearly 100% penetrance by age 30. Glomangiomas are tumors of the glomus body, a thermoregulatory arterial-venous shunt composed of modified smooth muscle cells. Vulvar glomangioma is an exceedingly rare cause of chronic pelvic pain, that may be easily confused for other conditions such as Bartholin's gland abscess or deep angiomxyomas, thereby delaying diagnosis and treatment. Glomangiomas have characteristic pathologic and imaging findings which may aid diagnosis. We herein describe the case of a 24-year-old female who developed chronic pelvic pain in the setting of a vulvar glomangioma. We further delineate the magnetic resonance imaging and biopsy findings critical to her diagnosis, and the appropriate steps taken for surgical management. She was found to harbor a heterozygous GLMN mutation. To the best of our knowledge, this is the first description of such a case in the medical literature.

Myoepithelioma-like tumors of the vulvar region: A case report and review of the literature.

Myoepithelioma-like tumors of the vulvar region (MELTVR) is a kind of solid tumor newly recognized in recent years, which is characterized by mesenchymal tumors of adult female vulva. The histopathology is similar to myoepithelioma, but the immunohistochemical phenotype and genetic changes are different from myoepithelioma. It usually has clear boundary and partial capsule, mixed with two forms of cells (epithelioid and spindle), the cells are mild, the nucleoli are clear, mitoses are rare, some cases have myxoid differentiation. In this article, a case of MELTVR diagnosed in our hospital is discussed. The patient was a 43-year-old female who finds a neoplasm in the pubic tubercle 4 months ago. Local resection was performed. Pathological examination showed that the boundary of the tumor was clear with partial capsule. The cells were arranged in cords or nests, and partially infiltrated the surrounding adipocytes. The tumor cells had two morphologies, epithelioid or spindle shaped. The spindle type cells were dominant, with bright cytoplasm, obvious nucleoli, rare nuclear mitosis (about 1/10HPF), and no necrosis was observed. Immunohistochemically, the tumor cells were positive for vimentin, epithelial membrane antigen, estrogen receptor, progestogen receptor, calponin and were partially positive for cathepsin k. INI1/SMARCB1 expression was deficient. There was no recurrence or metastasis during the 8-month-long follow-up. The unique feature of this case was that the site of the disease was not the vulva, but in front of the pubic tubercle, there was no large amount of mucus production, and the cytoplasm of most tumor cells was transparent. Due to our limited knowledge of MELTVR, its pathogenesis and tissue origin are not clear. Clinicians should be aware of such potential patients.

[Extra Mammary Paget disease]. / Maladie de Paget extra-mammaire.

Extramammary Paget's disease is a rare malignancy. The underlying cause of VPD remains not well understood. We provide an overview of the histopathology of vulvar Paget's disease, with or without invasion.

Vulvar myoma: A case report and review of literature.

OBJECTIVE: To present a case of vulvar myoma and the factors differentiating this tumor from Bartholin's cyst. CASE REPORT: A 50-year-old woman presented with a nodule over the left labia majora. Pelvic examination showed swelling and redness of the left labia majora. A 2-cm nodule with firm consistency was found near the vaginal opening. There was no inguinal lymphadenopathy. Bartholin's cyst was suggested, and oral cephalexin was prescribed for 1 week, but no improvement was seen. Therefore, she underwent excision of the nodule. Pathology revealed it to be a benign vulvar myoma. The patient recovered well, and no recurrence was noted after 2 months of follow-up. CONCLUSION: Vulvar myoma is rare. Sexual history, nodule consistency, and imaging are helpful, but the final diagnosis of vulvar myoma is usually made following surgical excision and histopathological analysis.

Terms, definitions and measurements to describe sonographic features of lymph nodes: consensus opinion from the Vulvar International Tumor Analysis (VITA) group.

In centers with access to high-end ultrasound machines and expert sonologists, ultrasound is used to detect metastases in regional lymph nodes from melanoma, breast cancer and vulvar cancer. There is, as yet, no international consensus on ultrasound assessment of lymph nodes in any disease or medical condition. The lack of standardized ultrasound nomenclature to describe lymph nodes makes it difficult to compare results from different ultrasound studies and to find reliable ultrasound features for distinguishing non-infiltrated lymph nodes from lymph nodes infiltrated by cancer or lymphoma cells. The Vulvar International Tumor Analysis (VITA) collaborative group consists of gynecologists, gynecologic oncologists and radiologists with expertise in gynecologic cancer, particularly in the ultrasound staging and treatment of vulvar cancer. The work herein is a consensus opinion on terms, definitions and measurements which may be used to describe inguinal lymph nodes on grayscale and color/power Doppler ultrasound. The proposed nomenclature need not be limited to the description of inguinal lymph nodes as part of vulvar cancer staging; it can be used to describe peripheral lymph nodes in general, as well as non-peripheral (i.e. parietal or visceral) lymph nodes if these can be visualized clearly. The association between the ultrasound features described here and histopathological diagnosis has not yet been established. VITA terms and definitions lay the foundations for prospective studies aiming to identify ultrasound features typical of metastases and other pathology in lymph nodes and studies to elucidate the role of ultrasound in staging of vulvar and other malignancies. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Primary vulvar leiomyosarcoma localized in the Bartholin's gland area: A case report and review.

Vulvar sarcomas located in the Bartholin's gland area are extremely uncommon mesenchymal vulvar tumors. These neoplasms can be mistaken as Bartholin' gland benign lesions such as cysts or abscesses, leading to a delay in the diagnosis of underlying malignancy. Currently, only a few cases of these aggressive cancers have been reported in the literature. A 42-year-old female patient without any previous complaint presented to Obstetrics and Gynecology Department of 'G. Chaztikosta' General Hospital due to a vulvar lump in the area of the left Bartholin's gland with a 6-month history of progressive swelling. Pelvic examination showed a solid mass of 6.5-cm in maximum diameter, localized in the left Bartholin's gland. The patient underwent wide local excision and histopathological examination of hematoxylin and eosin-stained sections indicated intersecting fascicles of spindle cells, with moderate to severe atypia. The number of mitoses was up to 8 per 10 high power fields. The neoplasm to its greatest extent was circumscribed and in places had an invasive growth pattern. Tumoral necrosis was not seen. Involved Bartholin' gland by the tumor was identified. The tumor extended focally to the surgical margin. The neoplastic cells showed positive staining for smooth muscle actin, desmin, HHF35, caldesmon, vimentin and estrogen and progesterone receptors. Immunohistochemistry was negative for S100, myoglobulin, keratin 116, CD117, CD34 and CD31. The patient denied further surgery or/and local radiotherapy, although the mass was >5-cm and a focally infiltrative surgical margin was found. During the close follow-up, no local recurrences or metastases were observed 53 months after surgery. In conclusion, wide local tumor excision with free surgical margins is a good option of surgery for vulvar leiomyosarcomas. In recurrences, a new extensive surgical resection of the lesion and radiotherapy are suggested. Ipsilateral lympadenectomy is indicated when there is a pathologic lymph node. Chemotherapy is provided in cases of distal metastases.

Lymphoscintigraphy and sentinel lymph node biopsy in vulvar carcinoma: update from a European expert panel.

PURPOSE: This study aimed to update the clinical practice applications and technical procedures of sentinel lymph node (SLN) biopsy in vulvar cancer from European experts. METHODS: A systematic data search using PubMed/MEDLINE database was performed up to May 29, 2019. Only original studies focused on SLN biopsy in vulvar cancer, published in the English language and with a minimum of nine patients were selected. RESULTS: Among 280 citations, 65 studies fulfilled the inclusion criteria. On the basis of the published evidences and consensus of European experts, this study provides an updated overview on clinical applications and technical procedures of SLN biopsy in vulvar cancer. CONCLUSIONS: SLN biopsy is nowadays the standard treatment for well-selected women with clinically negative lymph nodes. Negative SLN is associated with a low groin recurrence rate and a good 5-year disease-specific survival rate. SLN biopsy is the most cost-effective approach than lymphadenectomy in early-stage vulvar cancer. However, future trials should focus on the safe extension of the indication of SLN biopsy in vulvar cancer. Although radiotracers and optical agents are widely used in the clinical routine, there is an increasing interest for hybrid tracers like indocyanine-99mTc-nanocolloid. Finally, it is essential to standardise the acquisition protocol including SPECT/CT images, and due to the low incidence of this type of malignancy to centralise this procedure in experienced centres for personalised approach.

Measuring the depth of invasion in vulvar squamous cell carcinoma: interobserver agreement and pitfalls.

AIMS: The depth of invasion is an important prognostic factor for patients with vulvar squamous cell carcinoma (SCC). The threshold of 1 mm distinguishes between FIGO stages IA and ≥IB disease and guides the need for groin surgery. Therefore, high interobserver agreement is crucial. The conventional and the alternative method are described to measure the depth of invasion. The aims of this study were to assess interobserver agreement for classifying the depth of invasion using both methods and to identify pitfalls. METHODS AND RESULTS: Fifty slides of vulvar SCC with a depth of invasion approximately 1 mm were selected, digitally scanned and independently assessed by 10 pathologists working in a referral or oncology centre and four pathologists in training. The depth of invasion was measured using both the conventional and alternative method in each slide and categorised into ≤1 and >1 mm. The percentage of agreement and Light's kappa for multi-rater agreement were calculated, and 95% confidence intervals were calculated by bootstrapping (1000 runs). The agreement using the conventional method was moderate (κ = 0.57, 95% confidence interval = 0.45-0.68). The percentage of agreement among the participating pathologists using the conventional method was 85.0% versus 89.4% using the alternative method. Six pitfalls were identified: disagreement concerning which invasive nest is deepest, recognition of invasive growth and where it starts, curved surface, carcinoma situated on the edge of the tissue block, ulceration and different measurement methods. CONCLUSIONS: Pathologists reached only moderate agreement in determining the depth of invasion in vulvar SCC, without a notable difference between the two measurement methods.

["Alone with the taboo" - The social support experienced by women with vulvar neoplasia: a qualitative study]. / "Allein mit dem Tabu" ­ Wie Frauen mit vulvären Neoplasien die Unterstützung durch ihr soziales Umfeld beschreiben ­ Eine qualitative Studie.

"Alone with the taboo" - The social support experienced by women with vulvar neoplasia: a qualitative study Abstract. BACKGROUND: Cancer of the vulva is a gynaecological disease often with relevant impact for these women's daily life. On top of the medical complications, psychological problems might arise as well, due to the impaired quality of life and the withdrawal from social life. We do not know whether and what type of support is given to these patients. AIM: The qualitative study aimed at describing how the affected women experience the support they receive from their social environment from the time of the diagnosis to six months postoperatively. METHODS: A qualitative design was used. In a secondary analysis, 20 transcribed interviews from the WOMN-PRO study were analysed by qualitative content analysis according to Mayring. RESULTS: The findings from the study resulted in four main categories: having a serious disease shows how the patients experience the diagnosis as a shock and are confronted with questions of guilt and taboos. The category feel alone was strongly represented with features of not-speaking out, not looking at it and not-acting. Do it on your own features descriptions of the strategies the women use to get out of their loneliness. In experience help, it is shown that women who try to get in touch with their social environment usually receive support. CONCLUSIONS: Patients have a high demand for information concerning the disease and its possible impacts on their daily life as well as emotional and practical support from their families / partners and especially from health professionals. We recommend designating someone who acts throughout the whole treatment process as a mediator and a person of trust.

Prognostic factors for local recurrence of squamous cell carcinoma of the vulva: A systematic review.

BACKGROUND: In patients treated for early-stage squamous cell vulvar carcinoma local recurrence is reported in up to 40% after ten years. Knowledge on prognostic factors related to local recurrences should be helpful to select high risk patients and/or to develop strategies to prevent local recurrences. OBJECTIVE: This systematic review aims to evaluate the current knowledge on the incidence of local recurrences in vulvar carcinoma related to clinicopathologic and cell biologic variables. DATA SOURCES: Relevant studies were identified by an extensive online electronic search in July 2017. STUDY ELIGIBILITY CRITERIA: Studies reporting prognostic factors specific for local recurrences of vulvar carcinoma were included. STUDY APPRAISAL AND SYNTHESIS METHODS: Two review authors independently performed data selection, extraction and assessment of study quality. The risk difference was calculated for each prognostic factor when described in two or more studies. RESULTS: Twenty-two studies were included; most of all were retrospective and mainly reported pathologic prognostic factors. Our review indicates an estimated annual local recurrence rate of 4% without plateauing. The prognostic relevance for local recurrence of vulvar carcinoma of all analyzed variables remains equivocal, including pathologic tumor free margin distance <8mm, presence of lichen sclerosus, groin lymph node metastases and a variety of primary tumor characteristics (grade of differentiation, tumor size, tumor focality, depth of invasion, lymphovascular space invasion, tumor localization and presence of human papillomavirus). CONCLUSIONS: Current quality of data on prognostic factors for local recurrences in vulvar carcinoma patients does not allow evidence-based clinical decision making. Further research on prognostic factors, applying state of the art methodology is needed to identify high-risk patients and to develop alternative primary and secondary prevention strategies.