vulval carcinoma

This review covers the significant new developments in the pathological classification of gynecological tumors. Many of these were included in the updated World Health Organization Classification of Female Genital Tract Tumours, published in 2020. Topics include the compelling evidence that a large majority of extrauterine high-grade serous carcinomas arise from the fallopian tube; the Cancer Genome Atlas (TCGA) Classification of endometrial carcinomas; the discovery that most so-called synchronous endometrial and ovarian endometrioid carcinomas represent metastasis from the endometrium to the ovary; and the division of cervical, vaginal, and vulval carcinomas into clinically meaningful HPV-associated and HPV-independent types...

Vulval lichen planus (VLP) is a rare, but often chronic, inflammatory disease whose symptoms include genital pain, discomfort, and dyspareunia. The clinical manifestations include erythema, erosions, and scarring. The aim of this study was to longitudinally investigate patient-reported outcomes and clinical findings in patients with VLP. Patients (>18 years) with histologically confirmed VLP were included in the retrospective analysis. Patient demographics, clinical features, symptomatology, quality of life, management, clinical outcomes, and comorbidities associated with VLP were analyzed...

Vulval extramammary Paget disease (vEMPD) is an uncommon epithelial malignancy that may arise within the vulva (primary vEMPD) or represent vulval skin involvement by a noncutaneous carcinoma (secondary vEMPD). Primary vEMPD is most often an in situ carcinoma arising within the epidermis but may be associated with dermal invasion (invasive vEMPD) or represent intraepidermal spread of an adenocarcinoma originating in vulval skin adnexa or anogenital mammary-like glands. The latter, termed mammary gland-like adenocarcinoma (MGLA), exhibits morphologic, immunohistochemical, and molecular features of various breast carcinomas but, as far as we are aware, the metaplastic variant of MGLA has not been reported on the vulva...

In a previous study, we showed that the Hedgehog (Hh) signalling pathway is aberrantly activated in vulval squamous cell carcinoma (VSCC). In this study, we further validated our findings on a prospective cohort of primary VSCC cases, where immunohistochemical staining confirmed that key Hh pathway components were overexpressed in VSCC compared to normal vulval epithelium. We also undertook a series of in vitro studies to determine the extent of Hh pathway activation in VSCC-derived cell lines, and examine the consequences of pathway inhibition on the growth of these cells...

Fanconi anaemia is a rare autosomal recessive disorder associated with bone marrow failure and congenital malformations. The impaired DNA repair pathways in Fanconi anaemia predispose patients to a high risk of cancers of squamous cell origin, particularly in the head and neck region. Cancers of the vagina and vulva are rare in Fanconi anaemia. Here, we report a case of a 44-year-old female with Fanconi anaemia who developed an ulcerated lesion on the clitoris that extended into the labia majora. A biopsy of the lesion showed well-differentiated squamous cell carcinoma...

A 47- year-old woman developed a rapidly enlarging vulvar mass. Although the clinical appearance suggested malignancy, its lack of atypia and invasion on initial superficial biopsy delayed the pathological diagnosis. It was not until a large incisional biopsy was performed that showed the diagnosis of verrucous squamous cell carcinoma (VSCC) involving pre-existing sinuses of hidradenitis suppurativa (HS). VSCC arising in HS is very rare and often leads to death in published cases. This case demonstrates the challenge in pathological diagnosis of this condition which impacted time to treatment...

Extramammary Paget's disease (EMPD) of the vulva is a rare disease that occurs mainly in postmenopausal women. It can be primary or secondary. Prognosis of primary EMPD is good, but when secondary, we need to find the underlying malignancy as the prognosis depends on it. Very few cases of secondary EMPD of the vulva have been reported till now. We hereby report a case of a 65-year-old postmenopausal woman with EMPD secondary to invasive adenocarcinoma with neuroendocrine features in the dermis and she was treated surgically followed by radiotherapy...

CONTEXT: Vulvar carcinoma accounts for 3%-5% of gynecologic malignancies. The past three decades has observed changes in the trends of clinical characteristics and treatment modalities used in managing this disease. AIMS: The aim of the present study is to analyze the clinic-pathological characteristics and survival of women with squamous cell carcinoma vulva who underwent primary surgical management. SETTINGS AND DESIGN: This was a retrospective observational study...

Cancer metastasis and recurrence are potentially lethal. A small number of cancer cell groups called cancer stem cells (CSCs) have both stem cell capacity and cancer-forming ability and are reported to play important roles in cancer metastasis and recurrence. These CSCs are considered to be radiation-resistant (RR). Therefore, understanding the biological effects of radiation on squamous cell carcinoma (SCC) cell lines in vitro and in vivo might be worthwhile to circumvent radiation resistance. Currently, there are no reports on the establishment of RR-SCC cells in serum-free defined culture, which mimics biological mechanisms and prevents instability of using serum in the culture medium...

Mutations and other molecular events involving subunits of the SWI/SNF chromatin remodelling complex are common in a wide variety of malignancies, including those arising at various sites in the female genital tract. Endometrioid and clear cell carcinomas in the uterine corpus and ovary not uncommonly contain mutations in ARID1A and these also occur in other endometriosis-associated ovarian neoplasms such as seromucinous tumours. In these organs, mutations in SMARCA4, SMARCB1, ARID1A and ARID1B (with subsequent loss of corresponding protein expression as a reliable surrogate) are relatively common in undifferentiated carcinomas, including the undifferentiated component of dedifferentiated carcinoma...

Adenoid cystic carcinoma of the Bartholin gland is a rare vulval cancer. Available literature suggests an aggressive nature with protracted clinical symptoms and local recurrence despite adequate surgical excision with or without adjuvant radiotherapy. This case demonstrates the role of neoadjuvant chemotherapy and interstitial brachytherapy with less radical surgery, in the treatment of adenoid cystic carcinoma of the Bartholin gland. A 63-year-old woman presented in the clinic four months after the removal of vulvar mass with histopathology report showing adenoid cystic carcinoma of the Bartholin gland with positive margins...

Verrucous carcinomas are a rare variant of squamous cell carcinoma. Vulval verrucous carcinoma comprises only 1% of vulval malignancies. This case report discusses the successful management of aggressive recurrent vulval verrucous carcinoma with acitretin, an oral synthetic retinoid used to manage severe psoriasis, after multiple surgical interventions and radiotherapy had failed. This is the first reported case in which verrucous carcinoma had recurred in a musculocutaneous graft. To manage the recurrence, the patient underwent radiotherapy, which did not resolve the condition...

The purpose of this guideline is to collate evidence and propose evidence-based guidelines for the diagnosis and management of adult patients with vulva carcinoma treated in the UK. Malignant melanoma may present via similar routes and will be discussed. The reader is referred to the Ano-uro-genital Mucosal Melanoma Full Guideline [1] for more detailed recommendations. The management of vulval sarcoma is outside of the scope of this guideline. For further information, including details of guideline development and GRADE of recommendations, please see BGCS website for details (https://www...

AIM: Vulval lichen sclerosus is an inflammatory genital skin condition associated with poor quality of life, sexual dysfunction and risk of squamous cell carcinoma. The aim of this study was to document the demographics of women with lichen sclerosus seen at specialist vulval clinics. METHOD: We performed a retrospective review of women with lichen sclerosus seen at a tertiary combined gynaecology/dermatology vulval clinic over 12 months and Auckland Regional sexual health vulval clinics over five years...

Objective . Vulvar lichen sclerosus (VLS) is a chronic inflammatory disorder, which affects women of all ages. With numerous controversies as regards to the nomenclature, diagnosis and its association with neoplastic conditions, we decided to conduct a scoping review on this subject. Data Source . A review protocol was developed, and the Knowledge Resource Services website was used to do a search of articles pertaining to VLS with keywords "Vulvar," "Vulval," "diagnosis," "lichen sclerosus et atrophicus," "kraurosis," "vulvar dystrophy," and "Lichen Sclerosus"...

Vulval cancer is rare. With Bartholin gland carcinomas representing <5% of all vulval carcinomas they present both diagnostic and management challenges. There are a small number of cases in the literature describing Bartholin gland carcinomas with unusual histology which necessitates the need to explore the possibility of metastases from elsewhere. We present a case of a 55-yr-old woman presenting with a vulval lesion within the Bartholin gland. Morphology demonstrated enteric type adenocarcinoma and the immunohistochemistry profile was positive for CK7, CK20, CDX2, CEA, and CA19-9...

Vulval squamous cell carcinoma (VSCC) can arise through two distinct pathways [human papillomavirus (HPV)-associated and HPV-independent], and these VSCC variants are recognised as different disease entities on the basis of different aetiologies, morphological features, molecular events during oncogenesis, precursor lesions, prognosis, and response to treatment. The precursor of HPV-associated VSCC, variously referred to as high-grade squamous intraepithelial lesion (HSIL) [vulvar intraepithelial neoplasia (VIN) 2/3] or usual-type VIN, is morphologically identical to the more common HSIL (cervical intraepithelial neoplasia 2/3) of the cervix...

Introduction: Abnormal uterine bleeding may be defined as bleeding pattern that differs in frequency, duration and amount from a pattern observed during a normal menstrual cycle or after menopause. Aim and Objective: The aim of this study was (1) to identify the different benign, premalignant and malignant conditions as the cause of abnormal uterine bleeding in perimenopausal and postmenopausal women and (2) to find out the different histopathological patterns of endometrium and cervix in these women...

Vulval basal cell carcinomas (BCC) are an important differential diagnosis of painful, itchy vulval lesions, which can occur at all ages but commonly affect women in their senium. BCC tend to grow locally in an invasive and destructive pattern and seldom metastasize. Here we describe the rare case of inguinal metastasis in a 70-year-old woman with vulval BCC who was treated with radical hemivulvectomy, bilateral inguinal node sampling and adjuvant external beam radiotherapy. We discuss the clinical management of vulval BCC and provide an overview of the previously reported cases in the literature...

Lichen planus (LP) is a chronic immune-mediated dermatosis mainly affecting skin, oral, and genital mucosa. The heterogeneous clinical presentation, spectrum of symptoms depending on subtype and overlap with other vulval and cutaneous disorders can lead to challenging in diagnosis. We report an unusual case of vulval SCC arising within a patient with initial oral mucosal lichen planus who later developed lichen planus of the vulva. Discussion of this case is important as it typifies the difficulties in diagnosis of vulvo-vaginal disorders and potential complications...