10 – Vulvular & Vaginal Pathology – Flashcards
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Vulvar pathology
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Ectopic mammary tissue Inflammatory diseases Bartholin cyst Non neoplastic epithelial disorders: lichen sclerosis and lichen simplex chronicus Benign exophytic lesions Squamous neoplastic lesions Glandular neoplastic lesions: hidradenoma papilliferum, Paget's disease
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Ectopic mammary tissue is found where:
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occurs along primitive milk line
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Ectopic mammary tissue in vulva: types
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Cysts Fibroadenomas - benign tumor comprised of fibrous tissue, encapsulated; glandular areas Phylloides tumor - similar as to what is seen in breast Carcinoma
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Ectopic mammary tissue: characteristics
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The tissue is subject to physiologic and pathologic changes Include swelling and secretion of milk during pregnancy
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Inflammatory diseases of vulva
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Syphilis Granuloma inquinale Lymphogranuloma venereum Crohn's disease Behcet's disease Necrotizing fasciitis Vulvar vestibulitis
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Syphilis
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- Chancre (hard ; painless lesion) composed of *plasma cells, lymphocytes and histiocytes* - Covered by a *zone of ulceration* infiltrated by neutrophils and necrotic debris - Endarteritis - infiltration of blood vessels - Can mimic many other disorders
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Granuloma inguinale: caused by
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Chronic infection caused by *Calymmatobacterium granulomatis *
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Granuloma inguinale: gross ; histologic features
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- Begins as a *soft elevated granulomatous* area which enlarges slowly by peripheral extension and ulcerates - *Dense stromal infiltrate* composed of *histiocytes and plasma cells * - Scattered small abscesses - *Donovan's bodies*: small round encapsulated bodies within the cytoplasm of histiocytes
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Donovan's bodies are found in what?
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Granuloma inguinale
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Granuloma inguinale: spread
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- May spread to the retroperitoneum and simulate a soft tissue neoplasm
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Lymphogranuloma venereum: caused by
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Chlamydia organisms
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Lymphogranuloma venereum: manifestations
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- Affects lymph vessels and lymphoid tissue - May have a *small ulcer* at the site early - Swelling of inguinal lymph nodes with *stellate abscesses* surrounded by *pale epitheliod cells* - Scarring with fistulas and strictures of the strictures of the vagina, urethra and rectum
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What disease causes *stellate abscesses* surrounded by *pale epitheliod cells?*
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Lymphogranuloma venereum
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Crohn's disease
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- Associated with perineal disease and fistula formation - Erythematous areas with ulceration - May have noncaseating granulomas
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Behcet's disease
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- Autoimmune disease - Vasculitis (may produce vulvar lesions) - Rare in vulva - May present as *nonspecific ulceration*
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Necrotizing fasciitis
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- May be seen in *diabetic women* - Associated with a high mortality rate - Wide excision is the treatment of choice
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Vulvar vestibulitis
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- Chronic inflammatory infiltrate - Involves the *lamina propria and periglandular connective tissue* of the vestibular region - Small glands in vestibule - can be inflamed - Can produce *severe pinpoint pain in vestibule*
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Bartholin Cyst: what is it?
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- Acute infection of Bartholin gland - Produces acute inflammation
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Bartholin Cyst: associated with
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Often associated with gonorrhea - relatively common
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Bartholin Cyst: gross/histologic features
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-May cause abscesses May become large 3-5 cm - Lined by *transitional epithelium or squamous metaplasia*
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Bartholin Cyst: symptoms, risk ; tx
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Produce pain and discomfort Can be excised - *Carcinoma can occur* and are mostly squamous cell carcinoma
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Vulva: Nonneoplastic epithelial disorders
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- Lichen sclerosis - Squamous cell hyperplasia AKA Lichen simplex chronicus
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Lichen Sclerosis: histologic manifestations
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- Atrophy of the epidermis (thin) with elimination of rete pegs - Hydropic change of basal cells - Replacement of dermis by *dense collagen*/Sclerosis of superficial dermis - *Bandlike lymphocytic infiltrate* - chronic inflammation of the deeper dermis
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Lichen Sclerosis: gross appearance
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Grossly presents as a white parchment-like patches
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Lichen Sclerosis: who gets it
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- Can occur at all ages - Most common in postmenopausal pts - May mimic previous sexual assault
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Lichen Sclerosis: causes/cancer risk?
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- Not recognized as premalignant - Associated with greater then expected risk of cancer when associated with genetic alterations - May be autoimmune in nature
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Squamous cell hyperplasia: caused by
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Caused by rubbing caused by pruritus May be caused by infections, chemical exposure
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Squamous cell hyperplasia: histologic features
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- *Acanthosis and hyperkeratosis* of vulvar epithelium - May show increased mitotic activity - THICKENED epidermis - Variable leukocytic infiltration of dermis
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Squamous cell hyperplasia: cancer risk?
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Sometimes associated with cancer Biopsy to look for cellular differentiation and nuclear atypia
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Vulva: Glandular neoplastic lesions
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Hidradenoma papilliferum Extramammary Paget Disease
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Hidradenoma Papilliferum: gross appearance
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- Benign tumor presents as a well circumscribed *nodule* covered with *normal skin* - Identical to intraductal papillomas of breast
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Hidradenoma Papilliferum: may arise from
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ectopic breast tissue
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Hidradenoma Papilliferum: histologic features
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- *May ulcerate* and mimic carcinoma - Microscopically has a *complex papillary structure with a myoepithelial layer* - May have some degree of pleomorphism
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Extramammary Paget Disease: typical location
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Usually on labia majora - May persist for years without invasion
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Extramammary Paget Disease: gross/histologic features
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- Pruritic, crusted sharply demarcated area - May have *palpable submucosal thickening* - *Paget cells*: arise from primitive epithelium, have a clear halo
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*Paget cells*
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- arise from primitive epithelium, have a clear halo - pale pink cytoplasm which contains mucopolysaccharide which stain with PAS , Alcian blue or mucicarmine - Paget cells display apocrine, eccrine and keratinocyte differentiation
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Vulva: Benign exophytic lesions
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Condyloma Acuminatum
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Condyloma Acuminata: gross/histologic features
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- Usually *multiple* and may coalesce - *Branchlike projections* of squamous epithelium with a *fibrous stroma* - Acanthosis, parakeratosis, hyperkeratosis and koilocytosis
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Condyloma Acuminata: caused by
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* HPV 6 and 11*, sexually transmitted
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Condyloma Acuminata: associations/cancer risk
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Benign Frequently regresses spontaneously Not a precancerous lesion Marker for STD
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Vulva: Squamous neoplastic lesions
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Vulvar intraepithelial neoplasia Vulvar carcinoma: invasive , microinvasive and verrucous carcinoma
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Vulvar intraepithelial neoplasm (classic type): associated with
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*high risk HPV *
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Vulvar intraepithelial neoplasm (classic type): characterized by
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*nuclear atypia, increased mitoses* - Lack of surface differentiation
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Vulvar intraepithelial neoplasm (classic type): gross presentation
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- *white or pigmented plaques Often multicentric*
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Vulvar intraepithelial neoplasm (classic type): cancer association
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Carcinoma often coexists or is preceded by VIN Cancers associated with it are often poorly differentiated
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90% of VIN contains
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HPV 16 and 18
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VIN (classic type) histologic features
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Nuclear enlargement Hyperchromasia Small immature basaloid cells extending to the epithelial surface
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Vulvar intraepithelial neoplasm(differentiated variant): associated with
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*with squamous cell hyperplasia and lichen sclerosis Not typically associated with HPV*
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Vulvar intraepithelial neoplasm(differentiated variant): genetics + cancer
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*P53 overexpression* Cancer may develop quickly as nodule in a background of inflammation Invasive form has keratinization
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Vulvar cancer - most common type
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Squamous cell ca makes up 95% of vulvar cancer
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Vulvar cancer - age at presentation
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60-74 years
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Vulvar cancer - risk factors
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Number of lifetime sexual partners, cigarette smoking, and immunodeficiency
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Vulvar cancer - characteristics of differentiated type
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older women, not associated with HPV , keratinizing
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Vulvar cancer - characteristics of classic type
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younger women, associated with HPV often with a warty histology
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Vulvar cancer: Tumors of labia metastasize to
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LN
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Vulvar cancer: Tumors of clitoris metastasize directly
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to deep lymph nodes
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Vulvar cancer Prognosis
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5 year survival 50-75%
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Vulvar cancer prognostics
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tumor diameter, LN status and depth of invasion
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Vulvar Microinvasive cancer
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Vulvar Ca with depth of penetration less than 5mm Often have low incidence of LN metastasis Presence of eosinophils in VIN may be a clue to early invasion
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Vulvar Verrucous carcinoma: gross feature ; mets
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- Type of squamous carcinoma - May be *large, exophytic and infiltrates locally* Mets are almost non existent
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Vulvar Verrucous carcinoma: mimics
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- Can mimic condyloma acuminata and conventional squamous cell carcinoma - *Does not have cytologic atypia and/or a clearly infiltrative pattern of growth* like that seen in conventional squamous cell carcinoma
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Vulvar: Aggressive angiomyxoma mimics
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Bartholin gland cyst
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Vulvar Aggressive angiomyxoma: most common in
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women in 2nd-3rd decade Recurrence is common
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Vulvar Aggressive angiomyxoma: gross/histologic features
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- Grossly edematous ill defined mass; Soft, Gelatinous, Encapsulated (not always) - *Hypocellular stroma with little atypia* or mitotic activity with *large blood vessels*
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Vulvar melanoma: where ; how prevalent
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Occurs especially in labia majora Second most common malignant tumor of vulva Most pts are over 50 years old
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Vulvar melanoma: prognosis
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Most lesions are advanced by time of diagnosis LN status, depth of penetration, and ulceration are prognostic factors 5 year survival about 35%
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Vulvar melanoma: histologic features
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Malignant melanocytes resemble Paget cells
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Vaginitis: causes
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Vaginal discharge Candida albicans: curdy white discharge Trichomonas vaginalis: copious gray green discharge
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Vagina: Benign epithelial tumors
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*Intramural papilloma Squamous papilloma Tubulovillous adenoma Benign mixed tumor*
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Vagina: *Intramural papilloma*
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branching configuration with a lining of a single layer of cuboidal cells
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Vagina: *Squamous papilloma*
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most are due to HPV
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Vagina: *Tubulovillous adenoma*
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similar to colorectal type
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Vagina: *Benign mixed tumor*
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made of *stromal-type spindle glands* mixed with mature squamous cells and *glands lined by mutinous epithelium* - spindle cells
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Vagina: Squamous cell carcinoma histologic features
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Well differentiated lesion Invasion of the superficial stroma
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Vaginal intraepithelial neoplasms and vaginal cancer- prevalence
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Uncommon, usually squamous cell CA Usually present in women over 60 Most arise from extension of cervical squamous cell CA but can arise as a primary tumor
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Vagina: Clear cell adenocarcinoma - occurs where?
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Usually occurs in the *anterior or lateral wall of the upper vagina*
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Vagina: Clear cell adenocarcinoma - affects what ages
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Average age of diagnosis is 17 years Second smaller peak at 70 years
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Vagina: Clear cell adenocarcinoma - caused by/risk factors
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- In 2/3s of patients there is a history of prenatal exposure to *diethylstilbesterol (DES)* or related nonsteroid estrogens - Risk of cancer in the exposed population is about 1 in 1000
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Vagina: Clear cell adenocarcinoma - symptoms
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*Vaginal bleeding* or discharge are common symptoms
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Vagina: Clear cell adenocarcinoma - histologic features:
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Tubules and cysts lined by clear cells Some solid areas
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Vagina: Mesenchymal tumors and tumorlike lesions
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Fibroepithelial polyps Leiomyoma Leiomyosarcoma Rhabdomyoma
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Vagina: *Fibroepithelial polyps*
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- may be seen in adult women or neonates, have a *fibrovascular core lined by squamous epithelium* - slightly thickened epithelium, Loose fibrovascular stroma
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Vagina: *Leiomyoma*
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- most common benign mesenchymal tumor of the vagina
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Vagina: *Leiomyosarcoma*
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- can be large and ulcerate, moderate to marked atypical cells
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Vagina: * Rhabdomyoma*
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- *polypoid mass*, seen in adults, haphazardly arranged *spindle cells* with few mitoses - Squamous epithelium - Skeletal muscle scattered in the stroma
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Vagina: Sarcoma Botryoides - gross ; histolgic features
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*Polypoid invasive tumor* arises from the *anterior vaginal wall* Soft polypoid masses resembling a bunch of grapes *Myxoid stroma with round or spindle cells* *Crowding of cells around blood vessels*
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Vagina: Sarcoma Botryoides - occurrence ; age
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RARE Most cases are in girls under 5 years old with many presenting during the first two years
