Comprehensive Gynecology 12th Ed: Benign Gynecologic Lesion

Diagnostic Ultrasound Most common and least expensive of pelvic

imaging techniques

VULVA

URETHRAL CARUNCLE Small, fleshy outgrowth of the distal edge of the

urethra Tissue of the caruncle is soft, smooth, friable,

and bright red Initially appears as an eversion of the urethra Generally small, single, and sessile, but they may

be pedunculated and grow to be 1-2 cm in diameter

Occur most frequently in postmenopausal women

Must be differentiated from urethral carcinomas. Believed to arise from an ectropion of the

posterior urethral wall Associated with retraction and atrophy of the

postmenopausal vagina Growth of the caruncle is secondary to chronic

irritation or infection. Histologically

Composed of transitional and stratified squamous epithelium with a loose connective tissue

Submucosal layer contains relatively large dilated veins

Frequently subdivided by their histologic appearancea) Papillomatousb) Granulomatousc) Angiomatous Varieties

Often secondarily infected, producing ulceration and bleeding.

If the diagnosis is entertained in a child, most likely the correct diagnosis is Urethral Prolapse

Symptoms associated are variable Many women are asymptomatic, whereas

others experience dysuria, frequency, and urgency. Sometimes produces point tenderness

after contact with undergarments or during intercourse.

Ulcerative lesions usually produce spotting on contact more commonly than hematuria.

Differential diagnosis includes Primary carcinoma of the urethra Prolapse of the urethral mucosa

Urethral caruncles are not a precursor for Urethral Carcinoma, grossly the two are often confused. Urethral Carcinoma is primarily a disease

of elderly women Majority are of squamous cell origin. Most of these rare carcinomas arise from the

distal urethra Symptoms include bleeding, urinary

frequency and dysuria Signs include a mass protruding from the

urethra, tenderness and induration of the urethra.

Diagnosis is established by biopsy under local anesthesia.

Initial therapy is oral or topical Estrogen and avoidance of irritation.

If the caruncle does not regress or is symptomatic, it may be destroyed by cryosurgery, laser therapy, fulguration, or operative excision. Following operative destruction, a Foley

catheter should be left in place for 48-72 hrs. Follow-up is necessary to ensure that the

patient does not develop urethral stenosis. Often may recur.

Small, asymptomatic urethral caruncles do not need treatment.

Urethral Prolapse Predominantly a disease of the

premenarcheal female, although it does occur in postmenopausal women.

Patients may have dysuria; however, most are asymptomatic.

Annular rosette of friable, edematous Prolapsed mucosa does not have the bright

red color of a caruncle Not as circumscribed in gross configuration. May be ulcerated with necrosis or grossly

edematous. Therapy

Hot sitz baths

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Antibiotics to reduce inflammation and infection.

Topical Estrogen cream is sometimes an effective treatment.

In rare cases it may be necessary to excise the redundant mucosa.

CYSTS Most common large cyst of the vulva is a cystic

dilation of an obstructed Bartholin’s duct (Bartholin Duct Cyst) 2% of new gynecologic patients present with

an asymptomatic Bartholin’s duct cyst. Treatment is not necessary in women

younger than 40 unless the cyst becomes infected or enlarges enough to produce symptoms.

Glands of Vestibule Cyst Ducts of mucous glands of the vestibule are

occluded Cysts may be clear, yellow, or blue. Similar small mucous cysts occur in the

periurethral region. Wolffian Duct Cysts or Mesonephric Cysts

Rare Found near the clitoris and lateral to the

hymeneal ring Cysts have thin walls and contain clear

serous fluid. Skene’s Duct Cysts

Rare, usually small May present with symptoms of discomfort

or be found on routine examination. Cysts arise secondary to infection and scarring of

the small ducts Differential includes Urethral Diverticula

Physical compression of the cyst, unlike compression of a urethral diverticula, should not produce fluid from the urethral meatus

Imaging studies may assist in establishing the diagnosis.

Asymptomatic cysts in premenopausal women may be managed conservatively.

Treatment is excision with careful dissection to avoid urethral injury.

Most common small vulvar cysts are Epidermal Inclusion Cysts or Sebaceous Cysts Cannot be differentiated grossly Epithelial Cysts are discovered much more

frequently than Sebaceous Cysts. Epithelial Cysts

Located immediately beneath the epidermis. Most commonly they are discovered on the

anterior half of the labia majora Cysts are usually multiple, freely movable,

round, slow growing, and non-tender Firm to shotty in consistency

Contents are usually under pressure. Grossly, they are white or yellow and the

contents are caseous, like a thick cheese. Local scarring of the adjacent skin

sometimes occurs when rupturenof the contents of the cyst produces an inflammatory reaction in the subcutaneous tissue.

May develop following trauma when an in folding of squamous epithelium has occurred beneath the epidermis in the site of an episiotomy or obstetric laceration.

Most Inclusion Cysts of the Vagina are directly related to previous trauma

Most Inclusion Cysts of the Vulva are not related to trauma. Alternative theories of histogenesis

include Embryonic remnants Occlusion of pilosebaceous ducts

of sweat glands Histology:

Characterized by an epithelial lining of keratinized, stratified squamous epithelium

Center of cellular debris that grossly resembles sebaceous material.

Most vulvar epidermal cysts do not have sebaceous cells or sebaceous material identified on microscopic examination.

Usually are multiple cysts, with the majority being > 1 cm in diameter

Asymptomatic unless they are secondarily infected.

Large epidermal cysts may be confused with Fibromas, Lipomas, and Hidradenomas.

Most require no treatment. If the cyst becomes infected, treatment

consists of heat applied locally and incision and drainage.

Cysts that become recurrently infected or produce pain should be excised when the acute inflammation has subsided.

NEVUS Commonly referred to as a Mole Localized nest or cluster of melanocytes

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Undifferentiated cells arise from the embryonic neural crest

Present from birth. Many are not recognized until they become

pigmented at the time of puberty.

Vulvar Nevi One of the most common benign neoplasms in

females. Exhibit a wide range in depth of color, from blue

to dark brown to black and some may be amelanotic.

Diameter ranges from a few millimeters to 2 cm. Grossly: may be flat, elevated, or pedunculated. Differential diagnosis includes Hemangiomas,

Endometriosis, Malignant Melanoma, Vulvar Intraepithelial Neoplasia, and Seborrheic Keratosis.

Generally asymptomatic. Histologically lesions are subdivided into three

major groups: a) Junctionalb) Compound,c) Intradermal Nevi

5-10% of all malignant melanomas in women arise from this region Biologic reasons for this discrepancy are

unknown. Hypothesis that junctional activity is

common in vulvar nevi Many irritants to which vulvar skin is

exposed may lead to malignancy. 50% of malignant melanomas arise from a

preexisting nevus Majority of women who develop melanomas are

in their 50s. Family history of melanoma is one of the

strongest risk factors for the disease. Ideally, all vulvar nevi should be excised and

examined histologically. Special emphasis should be directed toward

the Flat Junctional Nevus and the Dysplastic Nevus, for they have the greatest potential for malignant transformation

Lifetime risk developing melanoma from Congenital Junctional Nevus that

measures >2 cm in diameter is approximately 10%.

Dysplastic Nevi is 15 times that of the general population

Dysplastic Nevus 5 mm in

diameter

With irregular borders and patches of variegated pigment.

Removal may be accomplished with local anesthesia or coincidentally with obstetric delivery or gynecologic surgery.

Proper excisional biopsy should be three-dimensional adequate in width and depth. Approximately 5-10 mm of normal skin

surrounding the nevus should be included Biopsy should include the underlying dermis

as well Nevi that are raised or contain hair rarely

undergo malignant change If they are frequently irritated or bleed

spontaneously, they should be removed. Recent changes in growth or color, ulceration,

bleeding, pain, or the development of satellite lesions mandate biopsy

Characteristic clinical features of an early Malignant Melanoma ABCD: Asymmetry, Border Irregularity,

Color variegation, and Diameter usually > 6 mm.

HEMANGIOMA Rare malformations of blood vessels rather than

true neoplasms. Vulvar hemangiomas frequently are discovered

initially during childhood Usually single 1-2 cm in diameter Flat, and soft Range in color from brown to red or purple

histologically Multiple channels of hemangiomas are

predominantly thin-walled capillaries arranged randomly and separated by thin connective tissue septa.

Tumors change in size with compression and are not encapsulated.

Most are asymptomatic; occasionally they may become ulcerated and bleed.

At least five different types of vulvar hemangiomas Strawberry and Cavernous Hemangiomas

are congenital defects discovered in young children

Strawberry Hemangioma Usually bright red to dark red Elevated Rarely increases in size after age 2

Approximately 60% of vulvar hemangiomas discovered during the first years of life spontaneously regress in size by the time the child goes to school.

Cavernous Hemangiomas

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Purple in color Vary in size, with the larger lesions

extending deeply into the subcutaneous tissue

Initially appear during the first few months of life

May increase in size until age 2 Spontaneous resolution generally occurs

before age 6 Senile or Cherry Angiomas

Common small lesions that arise on the labia majora

Usually in postmenopausal women Most often < 3 mm in diameter Multiple Red-brown to dark blue

Angiokeratomas Approximately twice the size of Cherry

Angiomas purple or dark red Occur in women between the ages of 30 and

50. Noted for their rapid growth and tendency to

bleed during strenuous exercise Differential diagnosis are Kaposi’s sarcoma

and Angiosarcoma Pyogenic Granulomas

Overgrowth of inflamed granulation tissue lesions grow under the hormonal influence

of pregnancy With similarities to lesions in the oral cavity. Approximately 1 cm in diameter May be mistaken clinically For Malignant

Melanomas, Basal Cell Carcinomas, Vulvar Condylomas or Nevi.

Treatment involves wide and deep excision to prevent recurrence.

Diagnosis is usually established by gross inspection of the vascular lesion.

Asymptomatic hemangiomas and hemangiomas in children rarely require therapy.

In adults, initial treatment of large symptomatic hemangiomas that are bleeding or infected may require subtotal resection.

When the differential diagnosis is questionable, excisional biopsy should be performed

Hemangioma that is associated with troublesome bleeding may be destroyed by cryosurgery or use of an argon laser. Cryosurgical treatment usually involves a

single freeze/thaw cycle repeated three times at monthly intervals

If the histologic diagnosis is questionable, any bleeding vulvar mass should be treated by excisional biopsy so that the definitive pathologic diagnosis can be established.

Surgical removal of a large, Cavernous Hemangioma may be technically quite difficult.

Lymphangiomas of the vulva do exist but are extremely rare.

Vulvar Venous Malformation Lesions may become symptomatic at any

age relatively prone to thrombosis Different from Vulvar Varicosities

Exacerbated with pregnancy Tend to regress postpartum. Marrocco-Trischitta and coworkers:

Reported on the successful use of sclerotherapy for the malformations, after venography and Doppler ultrasound verified the diagnosis.

FIBROMA Most common

benign solid tumors of the vulva

More frequent than lipomas, the other common

Benign tumors of mesenchymal origin

Occur in all age groups

Most commonly are found in the labia majora

Actually arise from deeper connective tissue Should be considered as Dermatofibromas Grow slowly Majority are between 1 and 10 cm in diameter Smaller fibromas are discovered as

subcutaneous nodules. As they increase in size and weight, they become

pedunculated. Smaller fibromas are firm Larger tumors often become cystic after

undergoing myxomatous degeneration. Sometimes the vulvar skin over a fibroma is

compromised by pressure and ulcerates. Have a smooth surface and a distinct contour. On cut surface the tissue is gray-white. Microscopically fat or muscle cells may be

associated with the interlacing fibroblasts Low-grade potential for becoming malignant Smaller fibromas are asymptomatic

Larger ones may produce chronic pressure symptoms or acute pain when they degenerate.

Treatment is operative removal if the fibromas are symptomatic or continue to grow. Occasionally they are removed for cosmetic

reasons.

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LIPOMA Benign, slow-

growing, circumscribed tumors of fat cells arising from the subcutaneous tissue of the vulva

Similar to lipomas of other parts of the body

Softer and usually larger than fibromas

Majority of lipomas are < 3 cm in diameter Second most frequent benign vulvar

mesenchymal tumor. Because of the fat distribution of the vulva, most

are discovered in the labia majora Superficial in location

Malignant potential is extremely low. Substance is soft, yellow, and lobulated. Histologically:

More homogeneous than fibromas. Prominent areas of connective tissue

associated with the mature adipose cells of a true lipoma.

Unless extremely large, lipomas do not produce symptoms.

Excision is usually performed to establish the diagnosis

Smaller tumors may be followed conservatively.

HIDRADENOMArare, small, benign vulvar tumor that originatesfrom apocrine sweat glands of the inner surface of thelabia majora and nearby perineum (Fig. 18-7). Occasionally,they may originate from eccrine sweat glands. For unknownreasons, they are discovered exclusively in white women betweenthe ages of 30 and 70, most commonly in the fourth decade oflife. These tumors have not been reported prior to puberty.Hidradenomas may be cystic or solid. In a review by Woodworthand colleagues 55% were cystic. Whereas 38% originated fromthe labia majora, 26% arose from the labia minora. Approximately50% of hidradenomas are less than 1 cm in diameter.

These tumors are well defined and usually sessile, pinkishgraynodules not larger than 2 cm in diameter. In most casesthe surface epithelium is white, but occasionally necrosis of acentral indented area occurs, with a protrusion of reddish-browngranulation tissue. These latter lesions may be confused withpyogenic granulomas.These tumors have well-defined capsules. These papillarytumors arise deep in the dermis. Histologically, because of itshyperplastic, adenomatous pattern, a hidradenoma may be mistakenat first glance for an adenocarcinoma. On close inspection,however, although there is glandular hyperplasia with numeroustubular ducts, there is a paucity of mitotic figures and a lack ofsignificant cellular and nuclear pleomorphism (Fig. 18-8).Hidradenomas are generally asymptomatic. However, theymay cause pruritus or bleeding if the tumor undergoes necrosis.Excisional biopsy is the treatment of choice.SYRINGOMAThe syringoma is a very rare, cystic, asymptomatic, benign tumorthat is an adenoma of the eccrine sweat glands. It appears assmall subcutaneous papules, less than 5 mm in diameter, that areeither skin colored or yellow and that may coalesce to form cordsof firm tissue. In the vulvar area, these asymptomatic papules areusually located in the labia majora. Identical tumors are oftenfound in the eccrine glands of the eyelids. This tumor is usually treated by excisional biopsy or cryosurgery. The most commondifferential diagnosis is Fox–Fordyce disease, a condition ofmultiple retention cysts of apocrine glands accompanied byinflammation of the skin. The latter disease often produces intensepruritus, while syringoma is generally asymptomatic.Fox–Fordyce disease is treated by oral or topical estrogens andtopical retinoic acid.

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ENDOMETRIOSISEndometriosis of the vulva is uncommon. Only 1 in 500 womenwith endometriosis will present with vulvar lesions. The firm,small nodule or nodules may be cystic or solid and vary froma few millimeters to several centimeters in diameter. The subcutaneouslesions are blue, red, or purple, depending on their size,activity, and closeness to the surface of the skin. The gross andmicroscopic pathologic picture of vulvar endometriosis is similarto endometriosis of the pelvis (see Chapter 19, Endometriosis).Vulvar adenosis may appear similar to endometriosis. Theformer condition occurs after laser therapy of condylomataacuminata.Endometriosis of the vulva is usually found at the site of anold, healed obstetric laceration, episiotomy site, an area of operativeremoval of a Bartholin’s duct cyst, or along the canal ofNuck. The pathophysiology of development of vulvar endometriosismay be secondary to metaplasia, retrograde lymphaticspread, or potential implantation of endometrial tissue duringoperation. Paull and Tedeschi documented 15 cases of vulvarendometriosis they believed were associated with prophylacticpostpartum curettage of the uterus to prevent postpartum bleeding.In their series, there was not a single case of vulvar endometriosisin 13,800 deliveries without curettage, but 15 cases ofvulvar endometriosis were associated with 2028 deliveries withprophylactic curettage. In general, symptoms do not appearfor many months following implantation.The most common symptoms of endometriosis of the vulvaare pain and introital dyspareunia. The classic history is cyclicdiscomfort and an enlargement of the mass associated with

menstrual periods. Treatment of vulvar endometriosis is bywide excision or laser vaporization depending on the size ofthe mass. Recurrences are common following inadequateoperative removal of all the involved area.GRANULAR CELL MYOBLASTOMAGranular cell myoblastoma is a rare, slow-growing, solid vulvartumor. The tumor originates from neural sheath (Schwann) cellsand is sometimes called a schwannoma. These tumors are foundin connective tissues throughout the body, most commonly inthe tongue, and occur in any age group. Approximately 7% ofsolitary granular cell myoblastomas are found in the subcutaneoustissue of the vulva. Twenty percent of multiple granular cellmyoblastomas are located in the vulva. The tumors are usuallylocated in the labia majora but occasionally involve the clitoris.These tumors are subcutaneous nodules, usually 1 to 5 cm indiameter. They are benign but characteristically infiltrate thesurrounding local tissue. The tumors are slow growing, but asthey grow, they may cause ulcerations in the skin. The overlyingskin often has hyperplastic changes that may look similar to invasivesquamous cell carcinoma. Grossly, these tumors are notencapsulated. The cut surface of the tumor is yellow. Histologically,there are irregularly arranged bundles of large, round cellswith indistinct borders and pink-staining cytoplasm. Initially thecell of origin was believed to be striated muscle; however, electronmicroscopic studies have demonstrated that this tumor isfrom cells of the neural sheath.The tumor nodules are painless. Treatment involves wide excisionto remove the filamentous projections into the surroundingtissue. If the initial excisional biopsy is not adequate and

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aggressive enough, these benign tumors tend to recur. Recurrenceoccurs in approximately one in five of these vulvar tumors.The appropriate therapy is a second operation with wider margins,as these tumors are not radiosensitive.VON RECKLINGHAUSEN’S DISEASEThe vulva is sometimes involved with the benign neural sheathtumors of von Recklinghausen’s disease (generalized neurofibromatousand cafe´-au-lait spots). The vulvar lesions of thisdisease are fleshy, brownish red, polypoid tumors. Approximately18% of women with von Recklinghausen’s diseasehave vulvar involvement. Excision is the treatment of choicefor symptomatic tumors.

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