D efinition —A malignant neoplasm of adipocytes. Clinical Features Epidemiology Rare. Most commonly encountered in middle-aged women. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

D efinition —A benign adipocytic neoplasm. Clinical Features Epidemiology Rarely encountered in the vulva. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

D efinition —A painless, deep-seated, and slow-growing tumor with a deceptively bland histologic appearance and a propensity for local recurrence. Clinical Features Epidemiology Low-grade fibromyxoid sarcoma (LGFMS) is uncommon. Most often identified in children and young to middle-aged adults. The third and fourth decades of life are the most common age at presentation. You’re Reading a Preview Become a Clinical Tree membership for Full access and…

D efinition —A subcutaneous, locally aggressive spindle cell neoplasm with a storiform growth pattern and honeycomb-like infiltration into adipose tissue. Clinical Features Epidemiology Dermatofibrosarcoma protuberans involving the vulva is rare; however, cases have been reported. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

D efinition —A benign fibrohistiocytic tumor of dermal stroma, composed of spindle cells with a storiform (pinwheel) growth pattern. Clinical Features Epidemiology Occurs in adults. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

D efinition —A benign subcutaneous neoplasm composed of short fascicles of spindle cells with prominent small, thick-walled, blood vessels. Clinical Features Epidemiology Uncommon. Typically seen in middle-aged women (mean age ~55 years). You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

D efinition —A benign pedunculated myxoid soft tissue neoplasm with a tendency toward local recurrence. Clinical Features Epidemiology Rare. Occasionally occurs in the vulva. Most frequently seen in reproductive-age women in their 30s. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

PITFALL D efinition —A bland, hypocellular, spindle cell lesion of the vulvar/perineal region with a tendency for local infiltration and recurrence. Clinical Features Epidemiology Reproductive-age women in their 30s. Uncommon. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

D efinition —A benign, nonrecurring spindle cell lesion of the vulvovaginal region, characterized by alternating hypercellular and hypocellular areas. Clinical Features Epidemiology Reproductive-age women. Uncommon. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

D efinition —A highly malignant tumor of dermal melanocytes. Clinical Features Epidemiology Rare. Accounts for approximately 3% of all melanomas in women. The second most common vulvar malignancy. Most often in patients in their sixth decade of life. May be more common in patients with a BRCA2 mutation. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership…

D efinition —A melanocytic proliferation with architectural, cytologic, and clinical features that are intermediate between those seen in common nevi and melanoma. Clinical Features Epidemiology Patients have a higher risk of melanoma. Patients with first-degree relatives who have melanoma have an extremely high risk of developing melanoma (dysplastic nevus syndrome). You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles…

D efinition —A melanocytic proliferation seen in locations with redundant skin, characterized by large junctional melanocyte nests and transepidermal elimination of nests. Clinical Features Epidemiology Uncommon; estimated prevalence of 2.3% in women. Young women, most often in the third decade of life. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log…

D efinition —Benign pigmented lesion of mucosal surfaces. Clinical Features Epidemiology No distinct demographic associations. Increased numbers may be seen in Carney complex. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

D efinition —Secondary involvement of the vulva by metastatic carcinoma. Clinical Features Epidemiology Rare. Accounts for 5% to 8% of vulvar malignancies. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

D efinition —“Ectopic,” colonic-type neoplasia (adenoma/carcinoma) arising in the vaginal or vulvar mucosa. Clinical Features Epidemiology Origin is in colonic-type epithelium, presumably incorporated into the vaginal or vulvar region following formation of the urorectal septum in early development. Another possible scenario is intestinal metaplasia of vaginal adenosis as rare cases have been reported in diethylstilbestrol (DES)–exposed women. Typically seen in adults, implying an age-related transformation of…

D efinition —A malignant mucocutaneous neuroendocrine neoplasm. Clinical Features Epidemiology Very rare (~12 per year in the United States). Vulvar tumors account for about 3% of all Merkel cell carcinomas. Risk factors include age (>60 years) and sun exposure. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

D efinition —Infiltration of the squamous epithelium by mucin-producing neoplastic cells. Clinical Features Epidemiology Uncommon; represents only 1% to 2% of vulvar malignancies. The average age at presentation is 65 years; the majority of patients are over 50 years. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

D efinition —An aggressive variant of squamous cell carcinoma with conspicuous glandular differentiation. Clinical Features Epidemiology Uncommon. Mean age at diagnosis is 65 to 70 years. Associated with chronic vulvar inflammatory disease. Human papillomavirus (HPV) link is unclear but likely when coexisting with basaloid carcinomas. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are…

D efinition —An uncommon, nonmetastasizing (usually!) but locally destructive carcinoma of basal keratinocytes with characteristic peripheral nuclear palisading. Clinical Features Epidemiology Rare. Not human papillomavirus (HPV) associated. Elderly women in their 70s and 80s. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here

PITFALL D efinition —A neoplastic proliferation of keratinocytes arising in follicular epithelium. Clinical Features Epidemiology Typically occurs in the middle aged to elderly. Suspected causes include ultraviolet light, immunosuppression, genetic alterations, chemical exposure, viruses, and trauma. You’re Reading a Preview Become a Clinical Tree membership for Full access and enjoy Unlimited articles Become membership If you are a member. Log in here