Advanced vulvar apocrine carcinoma expressing estrogen receptors that responds to tamoxifen therapy.

Primary vulvar carcinoma is rare and thought to arise from either anogenital mammary-like glands or native apocrine sweat glands. The diagnosis is predominantly based on tumor morphology with supportive evidence from immunohistochemical staining and exclusion of a primary breast carcinoma. The primary modality of treatment is surgery, while optimal managment of advanced disease is unclear. We present the case of a lady who had metastatic recurrent apocrine carcinoma expressing estrogen receptors, who had a complete response assessed by PET-CT scanning after 7 months of tamoxifen therapy. The report includes a discussion of the histological diagnosis and assessment of response to treatment by PET-CT scanning.

Extremely large vulvar fibroma in a 15-year-old girl.

BACKGROUND: Fibromas and fibromyomas belong to the most common solid vulvar tumors. Their cause remains unknown. CASE: A 15-year-old girl arrived at our department for extirpation of a large pendulous vulval fibroma. For three years she had observed a gradually enlarging structure protruding from her external genitals. After a preoperative CT examination the tumor was extirpated with a histological diagnosis of benign soft fibroma. CONCLUSION: Our report describes a therapeutic management of a large vulval fibroma in a young girl. The extended time from first symptoms to final treatment deserves reflection.

Congenital vulvar lipoma within an accessory labioscrotal fold.

Perineal lipomas are uncommon congenital anomalies. They mainly develop in male patients with an accessory scrotum, posterior to the original scrotum. However, on rare occasions, these lipomas can occur in female patients with an accessory labioscrotal fold. We report on three such cases, where the lipomas were found in the anterior part of the right side of the labia majora. None had other anomalies, with the exception of one who had premature growth of pubic hair on the accessory labioscrotal fold. Skin with a rugated scrotal appearance was present in all cases.

Asymptomatic Isolated Vulvar Metastasis in Old Treated Case of Carcinoma Rectum-Diagnosis and Treatment Response Evaluation by 18F-FDG PET/CT Scan.

We report a clinically asymptomatic, old treated case of carcinoma rectum, presenting with raised serum carcinoembryonic antigen level, referred for F-FDG PET/CT scan for restaging. Whole-body PET/CT scan revealed an isolated FDG-avid lesion in the left labia majora region, which on histopathology was found to be metastasis from carcinoma rectum. Subsequent F-FDG PET/CT done after 4 cycles of chemotherapy revealed favorable response to therapy. F-FDG PET/CT facilitates the detection of unusual site of metastasis and its subsequent response evaluation in the index case.

Recurrent vulvar melanoma in a patient with neurofibromatosis and gastrointestinal stromal tumour.

We report a case of a 51-year-old woman with neurofibromatosis who presented in 2012 with postmenopausal bleeding. Excision biopsy of a pigmented lesion of the labia minora was consistent with an ulcerated vulvar BRAF wild type malignant melanoma (MM). Initial excision was followed by radical vulvectomy and adjuvant interferon. Local recurrence in January 2017 was further resected. Positron emission tomography (PET)-CT in May 2017 identified an FDG avid omental deposit; consistent histologically with MM when resected. Postoperative PET-CT in August 2017 demonstrated local recurrence. In the setting of resected stage IV disease and a third local recurrence, the decision was made to instigate immunotherapy. Vulvar melanoma is rare accounting for 0.2% of all melanoma. Presentation is typically a decade later than cutaneous melanoma with a tendency to late metastases and poorer prognosis. Given their rarity the treatment paradigm is less clearly defined and largely extrapolated from that of cutaneous melanomas.

ACR appropriateness criteria staging and follow-up of vulvar cancer

Vulvar cancer is an uncommon gynecologic tumor and one of several human papillomavirus-associated malignancies. Squamous cell carcinoma is the most prevalent histologic subtype of vulvar cancer, accounting for the majority of cases. Imaging plays an important role in managing vulvar cancer. At initial diagnosis, imaging is useful to assess the size and extent of primary tumor and to evaluate the status of inguinofemoral lymph nodes. If recurrent disease is suspected, imaging is essential to demonstrate local extent of tumor and to identify lymph node and distant metastases. In this publication, we summarize the recent literature and describe the panel's recommendations about the appropriate use of imaging for various phases of patient management including initial staging, surveillance, and restaging of vulvar cancer. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision include an extensive analysis of current medical literature from peer reviewed journals and the application of well-established methodologies (RAND/UCLA Appropriateness Method and Grading of Recommendations Assessment, Development, and Evaluation or GRADE) to rate the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where evidence is lacking or equivocal, expert opinion may supplement the available evidence to recommend imaging or treatment.