Carcinoma vulva

Small-cell neuroendocrine carcinomas (SCNECs) of the female genital tract are rare and aggressive tumors that are characterized by a high rate of recurrence and poor prognosis. They can arise from various sites within the female genital tract, including the cervix, endometrium, ovary, fallopian tube, vagina, and vulva. They are composed of cells with neuroendocrine features, such as the ability to produce and secrete hormones and peptides, and a high mitotic rate. Immunohistochemical staining for neuroendocrine markers, such as chromogranin A, synaptophysin, and CD56, can aid in the diagnosis of these tumors...

INTRODUCTION: Cancer of unknown primary (CUP) is metastatic disease with no identifiable site of origin. Retroperitoneal squamous cell carcinoma of unknown primary is extremely rare. There are limited reports regarding treatment recommendations and outcomes for women with this disease. PRESENTATION OF CASE: Here we present the case of a woman with human papillomavirus (HPV)-related metastatic retroperitoneal pelvic squamous cell carcinoma of unknown primary previously treated for endometrial cancer as well as a review of the existing reports on this topic...

Adenoid cystic carcinoma (ACC) of the vulva represents a highly uncommon type of female malignancy. Due to the absence of specific treatment guidelines, such cases are typically managed by the treatment protocols for vulvar cancer. Here, we report the case of a 52-year-old woman who presented with a painful right vulvar mass, leading to a diagnosis of ACC of the vulva after biopsy and immunohistochemical analysis. She underwent vulvectomy, bilateral inguinal lymphadenectomy, and targeted radiotherapy, and no evidence of recurrence has been found for three years, with ongoing monitoring for post-radiation effects...

PURPOSE: To assess efficacy and toxicity of cisplatin (C) and gemcitabine (G) with intensity-modulated radiation therapy (IMRT) in patients with locally advanced vulvar cancer not amenable to surgery. METHODS: Patients enrolled in a single-arm phase II study. Pretreatment inguinal-femoral nodal assessment was performed. Sixty-four Gy IMRT was prescribed to the vulva, with 50-64 Gy delivered to the groins/low pelvis. Radiation therapy (RT) plans were quality-reviewed pretreatment...

OBJECTIVE: To evaluate predictors of recurrence and the risk of progression to carcinoma in patients with dVIN. METHODS: 36 self-identified White patients with dVIN from 2011 to 2022 were identified. Demographics, treatment and clinical course were abstracted. Histopathologic features and IHC stains were reviewed by 2 subspecialty pathologists. Standard statistical analyses were applied. RESULTS: Median cohort age was 70 years (range 39-91)...

Vulvar cancer is annually diagnosed in an estimated 6,470 individuals and the vast majority are histologically squamous cell carcinomas. Vulvar cancer accounts for 5% to 8% of gynecologic malignancies. Known risk factors for vulvar cancer include increasing age, infection with human papillomavirus, cigarette smoking, inflammatory conditions affecting the vulva, and immunodeficiency. Most vulvar neoplasias are diagnosed at early stages. Rarer histologies exist and include melanoma, extramammary Paget's disease, Bartholin gland adenocarcinoma, verrucous carcinoma, basal cell carcinoma, and sarcoma...

INTRODUCTION: The etiology and clinical presentation of vulvar carcinomas, especially vulvar lesions, are not fully understood. Because the vulva and cervix are anatomically connected, human papillomavirus (HPV) is the main cause of cervical lesions. Thus, this study explored the potential characteristics and effects of specific HPV infection types across vulvar lesions and concurrent cervical lesions. METHODS: This retrospective, cross-sectional study analyzed patients with cervical HPV or cytological results and concurrent vulvar biopsy who were seen in our hospital colposcopy clinic in Shanxi Province, China, between 2013 and 2023...

OBJECTIVES: Ensuring high-quality radiotherapy requires peer-reviewing target volumes. The Royal College of Radiologists recommends peer review specifically for individual target volumes in cases of gynaecological cancers. This study presents the outcomes of implementing an on-demand peer review system for gynaecological cancers within our institute. METHODS: The peer review process was planned for gynaecological cancer cases intended for curative radiotherapy. After junior clinical oncologists (COs) completed the segmentation, two senior COs specializing in gynaecological cancers conducted the peer review...

T-cell lymphoma is an extremely rare form of malignancy in the female genital tract. Most of the reported cases of lymphoma are B-cell lymphomas. A few cases of primary T-cell lymphomas involving the vagina or the vulva have been reported. We are reporting the first case of anaplastic large cell lymphoma (ALCL) presenting as a uterine cervical mass. The patient is a 24-year-old female who presented to the emergency room with a history of menorrhagia, night sweats and 40-pound weight loss. The diagnosis of ALCL was confirmed through immunohistochemical studies with strong CD30 and ALK expression...

Human papillomavirus-independent vulvar squamous cell carcinoma has a peak incidence in about the eighth decade of life. A variable portion of the vulvar squamous cell carcinoma are human papillomavirus-independent comprising 20% to 80% of all cases. Verrucous carcinoma (VC) is part of the spectrum of human papillomavirus-independent carcinomas and its combination with well-differentiated squamous cell carcinoma with sarcomatous differentiation is an extremely unusual neoplasm. The available literature on VC is currently limited to case reports and small single-institution studies...

BACKGROUND: Increased malignancy frequency is well documented in adult-systemic lupus erythematosus (SLE), but with limited reports in childhood-onset SLE (cSLE) series. We explored the frequency of malignancy associated with cSLE, describing clinical and demographic characteristics, disease activity and cumulative damage, by the time of malignancy diagnosis. METHOD: A retrospective case-notes review, in a nationwide cohort from 27 Pediatric Rheumatology centres, with descriptive biopsy-proven malignancy, disease activity/damage accrual, and immunosuppressive treatment were compiled in each participating centre, using a standard protocol...

There have been no reported cases of neuroendocrine carcinoma (NEC) of the cervix with pagetoid spread (Pag-S). A 44-year-old woman came to our department because of abnormal cytology that persisted immediately after a radical hysterectomy for NEC of the cervix. A mapping biopsy in a large area from the vaginal wall to the vulva revealed that synaptophysin/Ki-67-positive tumor cells were scattered within the epithelium in multiple areas, suggesting a wide Pag-S of NEC. Because tumor cells were found beyond the vaginal wall, the anterior pelvic exenteration was performed...

Adenoid cystic carcinoma (ACC) is a rare neoplasm most frequently observed in the salivary glands, that can occur in other organs, including the vulva and vagina. Oncogenic mechanisms involving MYB, NFIB, and MYB-NFIB rearrangements have been described, but evidence in the vulva and vagina remains scarce. Our aim is to report the clinicopathologic features, immunohistochemical, and molecular findings in a series of vulvar and vaginal ACCs. Five cases were included. Medical records and slides were reviewed. Formalin-fixed paraffin-embedded material was available in 4 cases, where additional immunohistochemical and molecular studies were carried out...

A 52-year-old female presented with labial ulcer of 4-month duration. Examination showed 1 cm × 1 cm single superficial ulcer in the right labium majus. Excision was done, and histopathologic examination revealed surface ulceration and dermal invasion by epithelial neoplasm formed of biphasic proliferation of squamoid and gland-forming cells. Immunohistochemical staining with p63 showed nuclear staining of the squamoid nests and was negative in areas with glandular differentiation, while epithelial membrane antigen and carcinoembryonic antigen highlighted the glandular elements...

Vulvar squamous cell carcinoma with sarcomatoid features is an extremely rare histological variant of squamous cell carcinoma with co-existence of both epithelial and mesenchymal features. A 70-year-old woman presented with genital lesions for 4 months with associated burning and pain. Examination revealed well-defined bilaterally symmetrical hyperpigmented plaques on labia majora, fleshy erythematous growth on labia minora. Biopsy from the fleshy mass was suggestive of sarcomatoid malignancy. However, immunohistochemistry was positive for cytokeratin AE1/AE3 and negative for desmin and smooth muscle actin...

Adenoid cystic carcinoma (AdCC) metastasis to kidney is rare. We identified 10 patients with metastatic AdCC in multi-institutional collaboration. Core needle biopsy was the most common specimen (n = 6). Patients were predominately female (n = 7) with a median age of 48 years (35-62 years). The most common primary location of the AdCC was head and neck (n = 6, among them parotid gland = 4), followed by lung (n = 2), breast (n = 1), and vulva (n = 1)...

Gynecological neuroendocrine neoplasms are rare entities and can be divided into two groups: carcinoids and neuroendocrine carcinomas. Due to their rarity their management is not standardized. The aim of this work is to summarize and discuss the current literature evidence on this pathology. A scoping literature review was performed in multiple databases. Thirty-one studies were included: 30 case reports and one case series. Patients' age ranged between 28-92 years. Surgery was the most used treatment and the surgical approach included local excision (N=16/31; 51...

•Sebaceous carcinoma is rare on the vulva and uncommonly associated with HPV.•Pregnancy may play a role in onset or exacerbation of HPV-associated vulvar cancers.•Treatment of vulvar sebaceous carcinoma is local excision and sentinel lymph node dissection with close follow-up.

BACKGROUND: Despite being a disease of mainly older women, little is known about the clinical management of older women with vulvar squamous cell carcinoma (VSCC). We evaluated their daily clinical management compared with younger women, and established the prevalence of comorbidities and its impact on overall survival (OS). METHODS: All Dutch women diagnosed with VSCC from 2015 to 2020 (n = 2249) were selected from the Netherlands Cancer Registry. Women aged ≥80 years (n = 632, 28%) were defined as "older" patients, women <80 years were considered as "younger"...

Vulvar carcinoma is a relatively rare malignancy and there is a paucity of data, especially from India and other developing countries regarding the prognostic factors impacting recurrence and survival. A retrospective observational study was conducted in the Department of Gynecologic Oncology at a tertiary care, regional cancer institute, including all patients with carcinoma vulva who underwent surgery between 2009 and 2018. Demographic profile, surgical-pathological information, details of neo-adjuvant chemotherapy, adjuvant radiation and chemotherapy, and peri-operative complications were analyzed...