Term
| what characterizes the incidence of ectopic mammary tissue in the vulva? |
|
Definition
| ectopic mammary tissue in the vulva occurs along the *primitive milk line and is subject to physiologic/pathologic changes (including swelling/secretion of milk during pregnancy). *fibroadenomas, such as those seen in the breast may appear and are composed of well circumscribed, firm fibrous tissue/glandular tissue. *phylloides tumors may also appear and are more aggressive. there may be various *fibrocystic changes (cyst formation) and *breast CA may also occur. |
|
|
Term
| what are some inflammatory diseases which may affect the vulva? |
|
Definition
| syphilis, granuloma inguinale, lymphogranuloma venereum, crohn's, behcet's (vascular), necrotizing fasciitis, and vulvar vestibulitis |
|
|
Term
| how does syphilis affect the vulva? |
|
Definition
| syphilis causes (painless) chancres to form on the vulva which are composed of *plasma cells*, lymphocytes and histiocytes. these chancres are covered by a *zone of ulceration infiltrated by neutrophils and necrotic debris as well as *endarteritis* (inflammation of small blood vessels w/*plasmacytic infiltration). |
|
|
Term
| what characterizes granuloma inguinale in the vulva? microscopic/gross presentation? |
|
Definition
| granuloma inguinale is a chronic infection caused by calymmatobacterium granulomatis which begins as a soft, non-specific elevated granulomatous area, which enlarges slowly by peripheral extension and ulcerates. histologically, there is dense stromal infiltrate composed of histiocytes and plasma cells (ddx for syphilis) and *donovan's bodies - which are small round encapsulated bodies w/in the cytoplasm of the histiocytes. grossly, granuloma inguinale appears as *scattered, small abscesses which may spread to the the retroperitoneum (mimics a soft tissue neoplasm). |
|
|
Term
| what characterizes a lymphogranuloma venereum infection in the vulva? |
|
Definition
| lymphogranuloma venereum is due to a *chlamydia infection of the vulva which affects the *lymph vessels and lymphoid tissue. lymphogranuloma venereum is characterized by *swelling of the inguinal nodes w/*stellate abscesses surrounded by pale epitheloid cells (significant cytoplasm)*. there may be a small *ulcer early on in the infection and scarring with *fistulas/strictures in the vagina/urethra/rectum may occur (may cause dysuria/constipation/bleeding). |
|
|
Term
| what characterizes the effet of crohn's on the vulva? |
|
Definition
| crohn's disease is associated with *perineal disease and fistula formation*. *erythematous areas w/ulceration are a common presentation and there may also be *noncaseating granulomas. |
|
|
Term
| what characterizes the effect of behcet's disease in the vulva? |
|
Definition
| behcet's is an *autoimmune disease which rarely affects the vulva. however, if it does, it usually will present as *vasculitis/nonspecific ulceration. |
|
|
Term
| what characterizes the effect of necrotizing fasciitis in the vulva? tx? |
|
Definition
| necrotizing fasciitis in the vulva may occur more commonly in *DM pts and is associated with a high mortality rate (can lead to ascending infections/voiding problems). tx: *wide excision. |
|
|
Term
| what are examples of reactive lesions affecting the vulva? |
|
Definition
| bartholin cysts, vulvar vestibulitis, lichen sclerosis, lichen simplex chronicus, hidradenoma papilliferum, and condyloma acuminata |
|
|
Term
| what are bartholin cysts? |
|
Definition
| relatively common *acute inflammation of the bartholin gland due to infection, often associated with *gonorrhea. *abscess formation may occur. as bartholin cysts are true cysts, they are lined by *epithelium (transitional/squamous metaplasia) and may produce pain/discomfort. CA may occur and is usually SCC (arises from metaplasia). bartholin cysts can be excised. |
|
|
Term
| what is vulvar vestibulitis? |
|
Definition
| vulvar vestibulitis involves small glands in the vestibule and can produce severe *pinpoint pain*. it is due to a *chronic inflammatory infiltration (lymphocytes) involving the lamina propria and periglandular connective tissue of the vestibular region. |
|
|
Term
| how does lichen sclerosis present histologically? |
|
Definition
| lichen sclerosis appears a *atrophy of the epidermis w/elimination of rete pegs (the normal protrusions of the epidermis down). there is hydropic change of the basal cells, *replacement of the dermis by dense collagen infiltration*, and bandlike lymphocytic infiltrate. |
|
|
Term
| how does lichen sclerosis present grossly? |
|
Definition
| white, dry, parchment-like patches. lichen sclerosis can *mimic healed scar tissue due to trauma - important to consider sexual assault, particularly in *older age groups (peri/post-menopausal). |
|
|
Term
| is lichen sclerosis considered pre-malignant? |
|
Definition
| no, but it is associated with a greater than expected risk of CA when associated w/genetic alterations. lichen sclerosis is considered to be auto-immune related. |
|
|
Term
| how do lichen sclerosis and squamous hyperplasia compare? |
|
Definition
| both have hyperkeratosis, but lichen sclerosis has a thinned epidermis over a *glassy collagen infiltrate and squamous hyperplasia has a thickened epidermis where the rete pegs are still present. lichen sclerosis has sclerosis of the dermis w/atrophy of the adnexa and a *band of lymphocyes while squamous hyperplasia has a dermis w/mild chronic inflammatory infiltrate (diffuse lymphocytes). a small % of lichen sclerosis will lead to CA, while squamous hyperplasia is not likely to at all. |
|
|
Term
| what is lichen simplex chronicus? what should be noted on bx? |
|
Definition
| lichen simplex chronicus is caused by *rubbing due to pruritus and presents as *acanthosis/hyperkeratosis of the vulvar epithelium. it may show increased mitotic activity (some association w/CA), variable leukocytic infiltration of the dermis (may be due to infection/chemical exposure). on bx, *r/o cellular differentiation and nuclear atypia*. (can mimic hyperplasia - but not a sclerosis b/c there is not thinning of epidermis). |
|
|
Term
| what is hidradenoma papilliferum? how does it appear microscopically? |
|
Definition
| hidradenoma papilliferum is one of the lesions which may arise from *ectopic breast tissue (identical to intraductal papillomas of the breast). it appears as a well circumscribed nodule covered w/normal skin (benign). hidradenoma papilliferum may *ulcerate and mimic CA and may have some degree of pleomorphism. microscopically: a complex *papillary structure w/a *myoepithelial layer (indication of benignity). |
|
|
Term
| what are condyloma acuminata? |
|
Definition
| sexually transmitted, benign lesions which are usually multiple and may coalesce. histologically, they appear as *branchlike projections of squamous epithelium w/a *fibrous stroma. |
|
|
Term
| what are condyloma acuminata caused by? associated with? |
|
Definition
| condyloma acuminata is caused by HPV 6 and 11 and is associated with acanthosis, parakeratosis, hyperkeratosis, and *koilocytosis (cells affected by HPV that shrink and have perinuclear halos)*. condyloma acuminata is *not precancerous but is a marker for STD. |
|
|
Term
| what characterizes classic type vulvar intraepithelial neoplasm (VIN)? |
|
Definition
| VIN is associated w/high risk HPV (16,18) and is characterized by nuclear atypia, increased mitoses, lack of surface differentiation, multicentricity, and often co-existence with poorly differentiated CA (VIN can precedes then coexist w/CA). *however VIN is considered pre-malignant (1/3 epith. involved – least severe, 2/3 – intermediate, 3/3 – CA in situ).* grossly: white or pigmented plaques (may mimic lichen sclerosis). |
|
|
Term
| what characterizes differentiated variant of vulvar intraepithelial neoplasm (VIN)? |
|
Definition
| the differentiated variant of VIN is associated with squamous cell hyperplasia and lichen sclerosis (not HPV). the differentiated variant of VIN has *p53 over-expression and CA may develop quickly as a nodule in a background of inflammation. the invasive form has keratinization. |
|
|
Term
| what generally characterizes vulvar CA? |
|
Definition
| most vulvar CA is *SCC* and presents in pts 60+ |
|
|
Term
| what are the risk factors for vulvar CA? |
|
Definition
| increased number of lifetime sexual partners (increases exposure to HPV), cigarette smoking, and immunodeficiency (check HIV status if quick onset). |
|
|
Term
| what are the 2 main types of vulvar CA? |
|
Definition
| *classic type: younger women, associated w/HPV - often w/a warty histology (poorly differentiated). *differentiated: older women, not associated w/HPV, and keratinizing (due to better differentiation). these 2 types can be mixed. |
|
|
Term
| where is vulvar CA mostly likely to be seen? how does this affect metastasis? |
|
Definition
| vulvar CA is seen mainly in the *labia majora and the tumors can then metastasize from there to the lymph nodes. tumors of the clitoris metastasize directly to the deep lymph nodes. |
|
|
Term
| what is the prognosis for vulvar CA? |
|
Definition
| 5 yr survival: 50-70%. depends on tumor diameter, LN status, and depth of invasion. |
|
|
Term
| what is microinvasive vulvar CA? |
|
Definition
| CA which only penetrates < 5 mm, which therefore has a low incidence of LN metastasis. eosinophils in VIN may be a clue to early invasion (this mechanism is not quite understood). |
|
|
Term
|
Definition
| a type of SCC which may be large, exophytic, and locally infiltrating. *mets are almost nonexistent. verrucous CA can mimic condyloma acuminata and conventional SCC - however verrucous CA *does not have cytologic atypia and/or a clearly infiltrative pattern of growth*, as seen in conventional SCC. |
|
|
Term
| what is extramammary paget's disease? |
|
Definition
| paget's disease of the breast can also involve the vulva, usually the *labia majora and presents as pruritic, crusted, *sharply demarcated areas (submucosal thickening may be apparent on palpation). *paget cells arise from primitive epithelium and have a clear halo (larger, more rounded nuclei than what you would see in koilocytosis).* extramammary paget's disease may persist for years w/o invasion. |
|
|
Term
| what is an aggressive angiomyxoma? |
|
Definition
| aggressive angiomyxoma presents as a grossly *edematous ill-defined mass which may mimic a bartholin gland cyst (if it enlarges). most pts are 20-30 and recurrence is common (hard to completely excise). histologically: overgrowth of hypocellular stroma w/little atypia or mitotic activity w/large blood vessels (not malignant, but can be locally invasive). |
|
|
Term
| what is the second most common malignant tumor of the vulva (2ndary to SCC)? what characterizes its incidence? prognosis? |
|
Definition
| melanoma, which occurs esp in the *labia majora in pts over 50. most lesions are advanced by the time of dx, but LN status/depth of penetration/ulceration determine prognosis (5 yr survival: 35%). grossly: metastasis may appear as sarcomas, CAs, etc. microscopically, melanoma can mimic paget's w/halos - but they are not perinuclear in melanoma. |
|
|
