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Glandular and Other Malignancies of the Vulva

Introduction By far, the most common epithelial malignancies of the vulva are squamous carcinomas and their precursor lesions. The remaining tumors are more rare and include adenosquamous carcinomas; adenocarcinomas, including metastatic ( Box 7.1 ) Bartholin gland; and neuroendocrine carcinomas. Box 7.1 Glandular and Neuroendocrine Carcinomas of the Vulva Vulvar neoplasms with glandular differentiation Adenosquamous carcinoma Paget disease Ectopic breast carcinoma Adnexal carcinomas Microcystic carcinoma Adenocystic…

Squamous Neoplasia of the Vulva

Introduction This chapter focuses on pure squamous lesions of the vulva and focuses on three general categories, including benign common human papillomavirus (HPV) associated lesions (condylomata), two accepted and one rather newly appreciated category of preinvasive squamous neoplasia, and the range of squamous carcinomas ( Figs. 6.1 and 6.2 ). This chapter emphasizes the differential diagnosis of each of these and their clinical significance and highlights…

Benign Cysts, Rests, and Adnexal Tumors of the Vulva

Introduction The clinician and pathologist must recognize benign cysts and adnexal lesions of the vulva to exclude more serious conditions and avoid misclassifying them as malignancies ( Table 5.1 ). They typically do not pose a diagnostic problem with the exception of (1) ectopic breast tissue, particularly when containing fibroadenomas; (2) endometriosis, particularly if it is gland-poor (stromatosis); and (3) hidradenoma papilliferum (HP), which may be…

Infectious Disorders of the Lower Genital Tract

Introduction A variety of organisms can infect the female genital tract, accounting for considerable suffering and morbidity ( Table 4.1 ). Some, such as candidal infections, trichomoniasis, and bacterial vaginosis, are extremely common and may cause significant discomfort, but with no serious sequelae. Others, such as gonorrhea and chlamydial infection, are major causes of female infertility. Viruses, principally human papillomavirus (HPV), are involved in the pathogenesis…

Localized Vulvodynia

Introduction Vulvodynia is defined as a chronic discomfort or pain, consisting of burning, stinging, irritation, and rawness on the vulva. It was once believed to be a rare condition, but is now known to affect millions of women. It can be a chronic pain disorder, at times associated with other chronic comorbid pain conditions. The prevalence of vulvodynia is 8.3%. The prevalence remains stable through age…

Noninfectious Inflammatory Disorders of the Vulva

Introduction Diagnostic dermatopathology of the vulva is a field that may be practiced on a multitude of levels. Patients may be seen by gynecologists, family practitioners, internists, or dermatologists. The biopsy may be read by a dermatopathologist, gynecologic pathologist, or general pathologist. Thus, any chapter addressing diagnostic vulvar dermatopathology must serve a multitude of audiences. This chapter is designed to provide an organized approach to inflammatory…

Female Genital Tract Development and Disorders of Childhood

Overview of Reproductive Tract Development The female genital tract is formed by a complex series of events beginning in the fourth week of development. This process involves the formation of the gonads following germ cell migration from the yolk sac to the dorsal mesentery, formation and fusion of the müllerian ducts to create the uterine corpus and tubes, induction of squamous mucosa in the vagina and…

Miscellaneous Tumors of Uncertain Differentiation

Benign tumors Superficial Angiomyxoma Definition An uncommon distinct type of myxoma characterized by the presence of thin-walled blood vessels This is likely to represent the same entity as cutaneous myxoma, including the lesions seen in Carney complex Clinical features Epidemiology Presents during adulthood and shows slight male predilection Commonly affects the head and neck and trunk areas Presentation Usually solitary Size: 1 to 5 cm Prognosis and…

Tumors of neuroectodermal origin

Reactive lesions Traumatic Neuroma Definition A reactive/reparative process characterized by proliferation of axons, Schwann cells, and perineurial fibroblasts in a disorganized pattern in the background of collagenous stroma (scar) at the proximal end of an injured/severed peripheral, cranial, or autonomic nerve Causally related to trauma to a nerve as a result of diverse etiologies, most commonly surgical procedure Peripheral sensory nerve(s) most commonly affected, followed by…

Bone- and cartilage-forming tumors and tumors of joints

Benign tumors and reactive proliferations Osteoma Cutis Definition Osteoma cutis represents a reactive, metaplastic, lamellar bone formation in the dermis and/or subcutis Primary and secondary forms of osteoma cutis can be separated on the basis of the absence or presence of an underlying skin disorder Primary osteoma cutis develops in normal skin or in the absence of an identifiable skin disorder (idiopathic) Secondary osteoma cutis always…

Tumors of vascular origin

Benign tumors including reactive vascular proliferations and ectasias Intravascular Papillary Endothelial Hyperplasia (Masson tumor) Definition A nonneoplastic, reactive, endothelial proliferation representing an organizing thrombus, characterized by formation of arborizing and interconnecting intravascular papillary cores lined by endothelial cells Clinical features Epidemiology Middle-aged adults Slight female predominance Presentation Predilection for the head and neck, extremities (particularly fingers), and trunk Occasionally on mucosal surfaces Slowly growing, solitary reddish,…

Skeletal Muscle Tumors

Benign tumors Rhabdomyomatous Mesenchymal Hamartoma Definition A rare dermal or subcutaneous benign lesion composed of disordered mature elements, including skeletal muscle, adipose tissue, nerve bundles, blood vessels, adnexal tissue, and collagen Initially reported as striated muscle hamartoma Additional terms used in the past include congenital midline hamartoma and hamartoma of cutaneous adnexa and mesenchyme Could reflect abnormalities in the migration of mesoderm during embryogenesis Clinical features…

Pericytic (perivascular) tumors

Benign tumors Glomus Tumor Definition A benign tumor composed of modified smooth muscle cells (e.g., glomus cells) Subclassified into three groups based on the proportions of glomus cells, blood vessels, and (perivascular) smooth muscle cells into: Classical or solid glomus tumor Predominance of glomus cells Blood vessels small and nondistinctive Glomangioma Blood vessels are prominent and dilated Represents the most common variant Glomangiomyoma Prominent smooth muscle…

Cutaneous smooth muscle tumors

Benign tumors and hamartomas Smooth Muscle Hamartoma Definition A hamartomatous lesion composed of a dermal proliferation of mature smooth muscle cells growing in haphazardly arranged bundles Clinical features Epidemiology Slight male predominance Prevalence of 1 in about 2700 live births The majority of the lesions are congenital Familial occurrence exceptional, with autosomal-dominant inheritance reported in some families Presentation Skin-colored indurated patch, plaque, follicular papules, or variably…

Fibrohistiocytic tumors

Benign tumors Fibrous Papule Definition A benign proliferation in the superficial dermis, composed of fibroblasts, blood vessels, and sparse inflammatory cell infiltrate in the background of a sclerotic dermal collagen Multiple lesions can be associated with tuberous sclerosis and, less commonly, with Birt–Hogg–Dube syndrome Similar lesions on the penis have been designated as pearly penile papules Clinical features Epidemiology Solitary lesions most common in middle-aged adults…

Fibroblastic and myofibroblastic tumors of the skin

Benign fibroblastic and myofibroblastic tumors Hypertrophic Scar Definition A reactive proliferation of fibroblasts/myofibroblasts confined to the boundaries of the wound forming short fascicles, whorls, or nodules, typically lacking keloidal collagen Generally associated with previous local trauma or burns Clinical features Epidemiology Most commonly occurs in the second decade of life Incidence after surgery from 40% to 70%, after burns from 30% to 90% Lesions developing in…

Adipocytic tumors

Benign tumors and reactive conditions Lipoma Definition A benign dermal or subcutaneous mesenchymal tumor composed of mature adult-type adipose tissue Clinical features Epidemiology The most commonly encountered mesenchymal tumor Majority of patients are adults No sex predilection Presentation Most cases are asymptomatic Occasionally painful when compressing nerves Slowly growing, but size is variable Usually solitary Multiple lipomas may be associated with neurofibromatosis, multiple endocrine neoplasia syndromes,…

External agents and artifacts

1 Electrocautery Keratinocytes show marked parallel vertical elongation Homogenization of the collagen 2 Gelfoam Blue-purple, arabesque, netlike pattern with surrounding granulomatous reaction 3 Aluminum chloride Epidermal effacement and horizontal fibrosis consistent with scar and underlying light gray-blue granules, often in histiocytes, with focal calcification 4 Monsel's solution (ferric subsulfate) Epidermal effacement and horizontal fibrosis with collagen necrosis and chunky golden, refractile pigment in macrophages Perls' iron…

Skin ultrastructure

1 Desmosome A classic desmosome showing the following features: (1) uniform gap of 20–30 nm between the apposed trilaminar plasma membranes with an intermediate line (arrow) in this gap; and (2) sharply delineated dense plaques into which tonofibrils (F) converge. 2 Langerhans cell (with Birbeck granules) This electronmicrograph shows characteristic racket-shaped profiles of the granules in the cytoplasm (inset with higher magnification of the Birbeck granule). You’re…