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DISEASES-OF-THE-VULVA-AND-VAGINA-OUTLINE

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					                        DISEASES OF THE VULVA AND VAGINA



Vulvar disorders fall into the following main categories:

       Malformations
       Infectious
       inflammatory
       Immunologic
       Neoplastic etiologies


Many inflammatory dermatologic diseases that affect skin elsewhere on the body may also occur
on the vulva:

       Psoriasis
       Eczema
       Allergic dermatitis


BARTHOLIN CYST AND ABSCESS
Bartholin glands are located at the entrance to a woman's vagina, one on each side
The duct is lined with transitional epithelium. The peripheral acini are single-layered columnar
cells with clear cytoplasm.

Bartholin cyst




Bartholin cysts form when the ostium of the duct becomes obstructed, leading to distention of the
gland or duct with fluid

Usually secondary to nonspecific inflammation or trauma

lined by the metaplastic squamous epithelium
Bartholin abscess




Result from a primary gland infection or infected cyst
Patients = acute, rapidly progressive vulvar pain

Abscesses are polymicrobial and rarely attributable to sexually transmitted pathogens

most common = are opportunistic bacteria such as Staphylococcus species, Streptococcus
species, and most commonly, Escherichia coli.

LEUKOPLAKIA.
Definition: lesions of the vulva presents as a whitish patch or plaque-like mucosal thickening
that may produce itching (pruritus) and scaling

Represent a variety of benign, premalignant, or malignant:

   1. Lichen simplex chronicus (squamous cell hyperplasia): An idiopathic condition in
      which lichenification of the vulva is caused by persistent itching and scratching




       thickening of the skin, leathery or bark-like appearance of the skin

       Clinical presentation: erythematous, firm, rough plaques with exaggerated skin lines
       (lichenified plaques). Hyperpigmentation of lichenified plaques
           a person senses pruritus in a specific area of skin (with or without underlying
           pathology) and causes mechanical trauma to the point of lichenification

           Occurs in the genital and perianal regions, as well as the posterior neck, forearms
           and pretibial areas

   Etiology unknown but patients with underlying atopic dermatitis appear to be at increased
   risk

   A relationship is suspected between the central nervous system and inflammatory cell
   products in the perception of itch and ensuing skin changes in lichen simplex chronicus.
   Emotional tensions in predisposed subjects may play a key role in inducing a pruritic
   sensation, leading to scratching that can become self-perpetuating.

   Micro findings:

           Repetitive scratching or rubbing from irritants can result in squamous cell
           hyperplasia. Squamous cell hyperplasia is the hallmark of lichen simplex
           chronicum

           Epithelial thickening, expansion of the stratum granulosum, and significant
           surface hyperkeratosis

           Increased mitotic activity

           Leukocytic infiltration of the dermis is sometimes pronounced. The hyperplastic
           epithelial changes show no atypia




Recent years: Some cases of lichen simplex chronicus in elderly women have been published
associated with Human papillomavirus-negative invasive squamous carcinoma of the vulva
in the adjacent skin

2. Lichen sclerosus: the skin = thin, whitened, wrinkled, and can cause itching and pain;
   most commonly occurs in the vulva, and in the anal region
postmenopausal women

Etiology:

       Genetic factors: families are genetically predisposed after experiencing trauma,
       injury, or sexual abuse.


       Autoimmune diseases: possibly extracellular matrix protein-1 (ECM-1) as
       antibodies to this protein; Other autoimmune conditions such as thyroid disease
       (about 20% of patients), pernicious anemia, vitiligo, and psoriasis are reported

       Clinical presentation: most common = vulvar itching; anal itching possible

       experience dyspareunia, dysuria, painful defecation and anal fissures

Gross: the skin appears white, with no pigmentation, and very thin and wrinkled,
classically referred to as a “cigarette paper” (or “parchment”) appearance




       It may progress and distort the appearance of the genital area as the labia minora
       become atrophic, fuse, disappear and bury the clitoris

       The vagina can become narrowed, and cracks, fissures, and thickened, scarred
       skin in the genital and anal area

Morphology: thinning of the epidermis and disappearance of rete ridges, hydropic
degeneration of the basal cells, superficial hyperkeratosis, and dermal fibrosis with a
scant perivascular, mononuclear inflammatory cell infiltrate
Lichen sclerosus and cancer  increased risk for developing squamous cell carcinoma of the
vulva

   1. Vulvar intraepithelial neoplasia and vulvar carcinoma (VIN)  precancerous
      lesions
      5% of all female genital tract malignancies and occurs most frequently in women
      between the ages of 65 and 75; vast majority (approximately 90%) are squamous cell
      carcinomas

       other histologic lesions including melanomas, adenocarcinomas, basal cell carcinomas,
       and sarcomas

       Classification: two groups
       A. Warty carcinoma , Basaloid carcinoma




          high oncogenic risk HPVs (type 16)

          Basaloid carcinoma shows an infiltrating tumor characterized by nests and cords of
          small, tightly packed malignant squamous cells lacking maturation that resemble
          immature cells from the basal layer of the normal epithelium. The tumor may
          have foci of central necrosis




          Verrucous carcinoma is characterized by exophytic, papillary (warty) architecture and
          prominent koilocytic atypia (i.e. cellular features of HPV infection). Verrucous
          carcinoma is locally invasive but rarely metastasizes
       B. keratinizing squamous cell carcinoma

           not related to HPV infection.


Vulvar carcinomas develop from a precancerous in situ lesion called vulvar intraepithelial
neoplasia (VIN).

       1. Classic VIN is associated with HPV infections, most often HPV type 16, and
          typically occurs in women between 30 and 40 with a history of multiple sexual
          partners.


              proliferation of small immature cells, nuclear atypia and increased mitoses,
              involving full thickness of the epidermis

       2. simplex VIN

              postmenopausal age range

              not associated with HPV

              Simplex VIN has a stronger association with lichen sclerosus and lichen simplex
              chronicus

       Micro: marked atypia of the basal layer of the squamous epithelium with apparently
       normal epithelial maturation and differentiation in the superfical layers, thus the
       designation “differentiated VIN.”

              Presents as red and velvety, or white and elevated plaque-like lesion that usually
              involves labia majora.
      A. HPV-associated vulvar squamous cell carcinomas (classic)

         long-standing lichen sclerosus or lichen simplex chronicus. The mean age of the
         patients is 76 years

         simplex VIN  initial lesion characterized this way

      Histologic examination reveals infiltrating nests and tongues of malignant squamous
      epithelium with prominent central keratin pearls

             1. Verrucous carcinoma (histo)

                 well-differentiated, low grade tumor

                 exophytic mass resembling condyloma acuminatum. Its prognosis is
                 excellent; slow regrowth may be a problem

                 tumor has pushing borders into the underlying dermis, but does not show
                 frank destructive invasion

                 Shows verrucous architecture

Papillary hidradenoma

      Vulva may contain tissue closely resembling breast (“ectopic breast”) and develop two
      tumors with counterparts in the breast:

      Papillary hidradenoma

      Extramammary Paget disease: characterized by infiltration of the squamous mucosa or
      adnexa by mucin-producing neoplastic cells; similar in its manifestations to Paget
      disease of the breast.

             Multipotential cells of epidermal basal layer that differentiate along glandular
             (sweat gland) lines
Gross:
         Papillary hidradenoma in general is a sharply circumscribed nodule, most
         commonly on the labia majora or interlabial folds, and may be confused
         clinically with carcinoma because of its tendency to ulcerate.

Histo:
         Identical in appearance to intraductal papillomas of the breast and consists of
         papillary projections covered with two layers of cells: the top columnar, secretory
         cells and an underlying layer of flattened myoepithelial cells; myoepi =
         characteristic of sweat glands and sweat gland tumors




Extrammamary Pagent disease of the Vulva

         Elevated, scaling, pruritic, and red eczematous-appearing area, occurring usually
         on the labia majora.

         Micro: Epidermis contains large pale tumor cells (Paget cells) that form small
         solid nests or a continuous layer along the epidermal basement membrane

         Stains with periodic acid–Schiff (PAS), Alcian blue, or mucicarmine stains.

         Vulvar lesions are most frequently confined to the epidermis of the skin and
         adjacent hair follicles and sweat glands
              Prog: Paget disease is treated with wide local excision and shows a high
              recurrence rate.

              Frequently present beyond the margins of surgical excision

               Intraepidermal Paget disease may persist for many years, even decades, without
              invasion or metastases

Malignant melanoma: Tend to have the same biologic and histologic characteristics as
melanomas occurring elsewhere in the skin and are capable of widespread metastatic
dissemination.

       Prognosis is linked principally to depth of invasion, with greater than 60% mortality for
       lesions invading deeper than 1 mm



Diseases of the Vagina

   A. DEVELOPMENTAL ANOMALIES

   1. Imperforate hymen  the most frequent obstructive anomaly of the female genital
      tract; usually asymptomatic until a child reaches menarche; repaired with a simple
      incision
   2. Vaginal agenesis  absence of the uterus, proximal vagina, and, in some cases, the
      fallopian tubes. This anomaly has been recently termed Müllerian aplasia
   3. Vaginal atresia  the urogenital sinus fails to contribute to the inferior portion of the
      vagina; müllerian structures are usually normal but fibrous tissue completely replaces the
      inferior segment of the vagina
   4. Double vagina  failure of total fusion of the müllerian ducts and accompanies a double
      uterus (uterus didelphys); vaginal duplication and lack of absorption of the wall between
      the two ducts will leave a residual vaginal septum
   5. Vaginal adenosis  remnant of columnar, endocervical-type epithelium that during
      embryonal development extends from the endocervix and covers the ectocervix as well as
      the upper vagina and is subsequently replaced by the squamous epithelium advancing
      upwardly from the urogenital sinus. Small patches of unreplaced glandular epithelium
      may persist focally into adult life.

              It has been reported in 35% to 90% of women exposed to diethylstilbestrol
              (DES) in utero  DES was shown to cause a rare vaginal tumor (clear cell
              adenocarcinoma of the vagina) arising in DES-related adenosis in girls and young
              women who had been exposed to this drug in utero
             red, granular areas contrasting with the normal pale-pink vaginal mucosa


             Micro: columnar mucinous epithelium indistinguishable from endocervical
             epithelium




   6. Gartner duct cysts  lesions found along the lateral walls of the vagina and derived
      from wolffian (mesonephric) duct rests



VAGINAL INTRAEPITHELIAL NEOPLASIA AND SQUAMOUS CELL CARCINOMA

Primary carcinoma  an extremely uncommon cancer

      Almost all of these tumors are squamous cell carcinomas associated with high risk
      HPVs. The greatest risk factor is a previous carcinoma of the cervix or vulva

      Squamous cell carcinoma of the vagina arises from a premalignant lesion, vaginal
      intraepithelial neoplasia, analogous to cervical squamous intraepithelial lesions

              affects the upper posterior vagina, particularly along the posterior wall at the
             junction with the ectocervix.

             lesions in the lower two thirds of the vagina metastasize to the inguinal nodes,
             whereas upper lesions tend to involve the regional iliac nodes

   A. CLEAR CELL ADENOCARCINOMA
      upper vagina of children and young adults.

      history of intrauterine exposure to diethylstilbestrol (DES)  2/3rd of patients
B. EMBRYONAL RHABDOMYOSARCOMA (Also called sarcoma botryoides)
   Infants and in children younger than 5 years of age and consists predominantly of
   malignant embryonal rhabdomyoblasts

   Divided into several histological subsets
   Embryonal  most frequently observed

   Embryonal botryoid  arise under the mucosal surface of body cavities such as the
   urinary bladder or vagina. Vaginal bleeding and discharge are the most common
   presenting symptoms.

   Spindle cell subtypes
   Alveolar
   Anaplastic
   Undifferentiated

   On physical exam polypoid mass resembling a bunch of grapes fills the vagina

   The tumor cells are crowded beneath the vaginal epithelium, in a so-called cambium
   layer, but in the deep regions they lie within a loose fibromyxomatous stroma that is
   edematous and may contain many inflammatory cells

   Can be mistaken for benign inflammatory polyps

   These tumors tend to invade locally and cause death by penetration into the peritoneal
   cavity or by obstruction of the urinary tract. Conservative surgery, coupled with
   chemotherapy, seems to offer the best results in cases diagnosed sufficiently early.

				
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posted:11/26/2011
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