Principles of Surgery, Companion Handbook - page 41

ANATOMY

External Genitalia (Vulva) The vulva is bounded by the symphysis pubis anteriorly, the anal sphincter posteriorly, and the ischial tuberosities laterally. The labia majora form the cutaneous boundaries of the lateral vulva and represent the female homologue of the male scrotum. Adjacent and medial to the labia majora are the labia minora, smaller folds of connective tissue covered laterally by non-hair- bearing skin and medially by vaginal mucosa. The anterior fusion of the labia minora forms the prepuce of the clitoris; posteriorly, the labia minora fuse in the fossa navicularis, or posterior fourchette.

Musculature of the Pelvic Floor The levator ani muscles form the muscular floor of the pelvis. These muscles include, from anterior to posterior, bilaterally, the pubococcygeus, puborectalis, iliococcygeus, and coccygeus muscles. Distal or caudad to the levator ani muscles, or levator sling, are the superficial muscles that constitute the urogenital diaphragm. The lateral-most components are the ischiocavernosus muscles. The bulbocavernosus muscles arise in the inferoposterior border of the symphysis pubis and around the distal vagina before inserting into the perineal body. The transverse perinei muscles arise from the inferior rami of the symphysis just anterior to the pubic tuberosities and insert medially into the perineal body, lending muscle fibers to this structure as well.

Internal Genitalia The uterus and cervix are suspended by the cardinal, or Mackenrodt's, ligaments, which insert into the paracervical fascia and into the muscular sidewalls of the pelvis laterally. Posteriorly, the uterosacral ligaments support for the vagina and cervix and insert into the paracervical or endopelvic fascia. The fallopian tubes arise from the cornua of the uterus. Each widens in the distal third, or ampulla. The ovaries are attached to the cornu by the ovarian ligaments. These fibrous bands are analogous to the gubernaculum testis in the male and continue from the uterus as the round ligaments. These structures exit the pelvis through the internal inguinal ring and course through the inguinal canal and external inguinal ring to the subcutaneous tissue of the mons veneris. The ovaries are suspended from the lateral pelvis by their vascular pedicles, the infundibulopelvic ligaments. The peritoneum enfolding the adnexa is referred to as the broad ligament.

The peritoneal recesses in the pelvis anterior and posterior to the uterus are referred to as the anterior and posterior cul de sacs. The latter is also called the pouch or cul de sac of Douglas.

Several avascular and therefore important surgical planes can be identified. These include the lateral paravesical and pararectal spaces and the prevesical space of Retzius and presacral spaces.

The muscles of the pelvic sidewall include the iliacus, the psoas, and the obturator internus. The blood supply arises from the internal iliac arteries, except for the middle sacral artery, which originates at the aortic bifucation. The hypogastric arteries divide into anterior and posterior branches. The latter supply lumbar and gluteal branches and give rise to the pudendal arteries. The nerve supply to the pelvis is composed of the sciatic, obturator, and femoral nerves. The ureters enter the pelvis as they cross the distal common iliac arteries laterally and then course inferior to the ovarian arteries and veins until they cross under the uterine arteries just lateral to the cervix.

DIAGNOSIS

Gynecologic History The gynecologic history should include the patient's age, date of her last menstrual period (LMP), the number of pregnancies, the number of deliveries, and the number of abortions. Gravidity, parity, and abortions are frequently indicated as G-P-A. The examiner should inquire as to when the patient's last cervical cytology was obtained and, in patients over age 35, the date of the patient's last mammogram.

Physical Examination The initial evaluation should include a general physical examination and a description of the patient's height, weight, nutritional status, blood pressure, head and neck, including thyroid , heart, lungs, and lymph nodes. The gynecologic portion of the examination should document an examination of the breasts, the abdomen, and the pelvis.

Diagnostic Procedures

Cervical Cytology Cervical cytology (Pap smear) should be performed beginning at 18 years of age or sooner if the patient is sexually active. Most women should have a cervical cytologic evaluation yearly at the time of their annual pelvic examination. After total hysterectomy, the Pap smear should be obtained annually in patients treated for cervical neoplasia. After hysterectomy for conditions that did not include cervical neoplasia, the vaginal apex may be screened cytologically every 3–5 years . The practitioner should expect a report from the laboratory in the format of the Bethesda classification (Table 39-1) for cervical cytologic reporting. All cytologic reports must be studied carefully to determine whether further evaluation or treatment is indicated. Atypical smears or smears with severe inflammation should be repeated generally in 3 months. Persistent atypical smears should be evaluated with colposcopic examination. All smears that indicate dysplasia or neoplasia should be investigated with colposcopy. Colposcopy is a specialized technique that allows evaluation of the cervix under magnification to do directed biopsies. The colposcopic examination following abnormal cervical cytology will preempt cone biopsy and allow office treatment of cervical dysplasia in most patients. When an endocervical lesion is found, the biopsy indicates a lesser lesion than cytologic report, or the biopsy is indicative of microinvasion of the cervix, a cone biopsy is indicated (Fig. 39-1).

TABLE 39-1 THE BETHESDA CLASSIFICATION FOR THE CLASSIFICATION OF PAP SMEAR ABNORMALITIES

FIGURE 39-1 The management of abnormal cytologic findings.

Office Tissue Biopsy Biopsy of suspicious lesions of the vulva, vagina, cervix, and uterus should be obtained in the office. Vulvar biopsy is obtained by infiltrating the biopsy site with a small amount of 1% lidocaine. Adequate biopsies can be obtained using a dermatologic skin punch. Biopsy of the ectocervix does not require anesthesia. Specialized cervical biopsy punches, such as the Kevorkian or Tischler type, are used. The endocervical canal should be sampled with an endocervical curette such as the Kevorkian. Biopsy of the endometrial cavity is an office procedure. It is essential to be assured that the patient is not pregnant before performing this procedure.

Vaginal Discharge The patient's complaint of abnormal vaginal discharge should be investigated. The pH of the vagina, which is normally between 3.8 and 4.4, may be an aid to diagnosis. A vaginal pH of 4.9 or more indicates either a bacterial or protozoal infection. Vaginal fluid is collected for study. The wet mount is prepared by placing a small amount of the saline suspension on a microscopic slide with a cover slip and examining it under magnification. The examiner may note motile trichomonads, indicative of Trichomonas vaginalis; characteristic clue cells, indicative of bacterial vaginosis; or pus cells , which may be indicative of a variety of vaginal, cervical, and uterine problems such as gonorrhea, chlamydia, or other bacterial infections. A drop of 10% potassium hydroxide is placed on the specimen, and the vaginal material is again evaluated. Potassium hydroxide has the ability to lyse cellular material to appreciate the presence of mycelia characteristic of Candida vaginitis.

Cultures Vaginal and cervical cultures are most useful for the detection of sexually transmitted disease. Gonorrhea is cultured on a chocolate agar plate. Cultures are most conveniently collected on a Thayer-Martin medium. Chlamydial infection is suggested by the finding of a characteristic thick yellow mucus (mucopus). Mucopus should be collected with a calcium alginate–tipped swab in transport medium specifically designated for Chlamydia.

Pregnancy Tests A number of pregnancy tests are available for use in the office. These tests measure increased amounts of the beta subunit of human chorionic gonadotropin (hCG) in urine. These urine tests are very sensitive and specific, measuring hCG as low as 50 mIU/mL. Serum tests are even more accurate and sensitive, and they have the advantage that they can be quantitated to give an hCG level.

Abnormal Bleeding

After the first menstrual period (menarche), cyclic bleeding is considered the norm. Menstrual interval varies from 21–45 days. Menstrual duration varies from 1–7 days. Abnormal genital bleeding falls into six categories.

Bleeding Associated with Pregnancy The availability of extremely sensitive pregnancy tests has made it possible to confirm pregnancy in the early days of gestation. Although bleeding can occur in up to 25 percent of all normally pregnant women, this symptom must be considered a threatened abortion until the bleeding is otherwise clarified. In the presence of threatened abortion, the pregnancy test is positive, the cervix is closed, and the uterus is generally consistent with the history of gestation. A threatened abortion is considered inevitable when the cervix is dilated and fetal tissue appears at the cervical os. Abortion is incomplete after a portion of the products of conception has been expelled; it is considered complete after all the products of conception have been expelled.

Ectopic pregnancy must be considered in any patient with a positive pregnancy test, pelvic pain, and abnormal uterine bleeding. Approximately 20 percent of patients with ectopic pregnancy have no bleeding. Gestational trophoblastic disease also causes abnormal bleeding associated with a positive pregnancy test. Molar pregnancy is suggested when the uterus is larger than would be expected from the history of gestation, vaginal bleeding, and the passage of grapelike tissue from the vagina. Gestational trophoblastic disease must be differentiated from normal pregnancy. Ultrasound examinations are helpful in diagnosis.

Dysfunctional Uterine Bleeding This type of bleeding abnormality is characterized by irregular menses with occasional extended intervals of amenorrhea. Evaluation of these patients should include a pregnancy test, which should be negative. Endometrial sampling reveals a nonsecretory or proliferative endometrium. In most instances the condition can be managed with cyclic estrogen/progesterone treatment.

Trauma The bleeding associated with genital trauma may be secondary to rape or genital injury . In the premenarchial female, the vaginal canal should be examined carefully for foreign bodies. Repair in the operating room under anesthesia may be necessary.

Bleeding Secondary to Neoplasm Tumors, both benign and malignant, involving the genital tract from the vulva to the ovary can produce abnormal bleeding. The most common cause of abnormal bleeding in the reproductive age group is leiomyomas (fibroids). Leiomyomas are almost always benign and are a common cause of menometrorrhagia.

Bleeding from Infection Bleeding is an uncommon symptom of pelvic inflammation.

Bleeding of Nongenital Etiology Genital bleeding can be associated with coagulopathy, systemic anticoagulants, clotting disorders, or blood dyscrasias.

Pain

Pelvic and abdominal pain is a common gynecologic complaint. Pain associated with menses is the most common office complaint. Cyclic pain limited to that period is referred to as dysmenorrhea. Pain occurring without a demonstrable pathologic lesion is referred to as primary dysmenorrhea. Secondary dysmenorrhea is commonly associated with endometriosis, cervical stenosis, and pelvic inflammation. Acute pain may have its origin in abnormal pregnancy, benign or malignant neoplasia, or a variety of nongynecologic diseases. Pregnancy disorders include threatened abortion, inevitable abortion, incomplete abortion, and ectopic pregnancy.

Neoplasms cause acute pain through degeneration of a myoma or torsion of a myoma or ovarian neoplasm. The spontaneous rupture of an ovarian cyst can produce severe pelvic pain. Acute pain may be caused by salpingitis or endometriosis. Pain secondary to inflammatory conditions is associated with fever and other evidence of infection in most cases. Pelvic infection secondary to Chlamydia trachomatis is the exception to this rule. The possibility of a nongynecologic condition as the cause of pain always must be considered, e.g., appendicitis or urinary problems such as renal and ureteral stones. In women in the reproductive age group, a differential diagnosis commonly involves appendicitis, ectopic pregnancy, and salpingitis.

Bilateral low abdominal pain increased by movement of the cervix most often indicates acute pelvic inflammatory disease. Right abdominal pain with a history of gastrointestinal symptoms will indicate appendicitis. In many cases it may not be possible to make a definitive diagnosis. Direct visualization of the pelvis can be carried out with a laparoscope.

Pelvic Mass

The finding of a pelvic tumor is a common event in reproductive-age women. Pregnancy should be considered in all cases of uterine enlargement in reproductive-age women. In addition to a carefully performed pelvic examination, abdominal and vaginal ultrasonography is a useful tool. No imaging method will distinguish between benign and malignant disease, however.

INFECTIONS

Vulvar and Vaginal Infections

Vulvar, perineal, and perianal itching and burning are symptoms that may indicate an inflammatory condition.

Mycotic Infection The most common cause of vulvar pruritus is candidal vulvovaginitis. This is common in patients who are diabetic, pregnant, or on antibiotics. The majority of cases are caused by Candida albicans. Diagnosis is confirmed by characteristic pseudomycelia. The condition is treated by topical application of any one of a number of imidazole preparations . Systemic treatment is possible through the oral use of fluconazole.

Parasitic Infections Pin worms ( Enterobius vermicularis ), which are common in young girls , cause vulvitis. Diagnosis is made by finding the adult worms or recognizing the ova. Mebendazole therapy is indicated. Trichomonas vaginalis causes primarily a vaginal infection. The patient complains of heavy, foul-smelling discharge. Diagnosis is made by microscopic examination. Treatment consists of metronidazole 250 mg given three times daily for 7 days. The vulvar skin is a frequent site for infestation by Phthirus pubis (crab lice) and Sarcoptes scabiei (scabies, itch mites). Treatment consists of lindane, available for medical use as Kwell.

Bacterial Infections Many bacteria attack the vulvovaginal region. The streptococci and staphylococci are the most common offenders. Gardnerella vaginalis is the most common bacterial pathogen. The patient complains of a foul, fishy, or dead mouse odor. Diagnosis is made by microscopic study to identify characteristic clue cells. The condition is treated with metronidazole 500 mg orally every 12 h for 1 week.

Viral Infections A number of viral infections affect the vulva and vagina, the most common of these being condyloma acuminatum. The causative organism is the human papillomavirus. Treatment depends on the destruction of the lesions with caustic agents , cryocautery, laser ablation, or electrocautery.

Herpes simplex infection causes painful vesicles followed by ulceration of the vulva, vagina, or cervix. Culture is confirmatory for herpes infection. There is a tendency for the lesions to recur at various intervals for the life of the patient. The attacks may be aborted and the interval between attacks lengthened through the use of acyclovir 200 mg orally five times daily. Cesarean section is recommended in patients in labor with vulvar or vaginal ulceration as a result of herpes simplex infection.

Molluscum contagiosum causes groups of small pruritic nodules with an umbilicated center. The lesions are treated by ablation.

Pelvic Inflammatory Disease

While pelvic inflammatory disease is basically a medical problem, it has profound surgical implications. This condition could be responsible for over a hundred thousand surgical procedures annually. The condition might produce infertility in 10 percent of the cases that occur; 3 percent or more of patients will have ectopic pregnancy, and chronic pain is a problem in many others. Pelvic inflammatory disease is largely limited to sexually active females. Pelvic inflammatory disease is classified as acute or chronic. The most common organisms that produce the condition are Neisseria gonorrhoeae and Chlamydia, but numerous other organisms have been incriminated. Diagnosis of pelvic inflammatory disease is based on clinical findings. The classic signs include fever, lower abdominal pain with pelvic tenderness, and purulent vaginal discharge. In patients requiring further study, laparoscopy, pelvic ultrasonography, and pelvic CT scanning may be helpful in confirming a diagnosis.

Treatment Patients with evidence of peritonitis, high fever, or suspected tuboovarian abscess should be admitted to the hospital for observation and intravenous antibiotics. Some specialists believe that all women with pelvic inflammatory disease should be admitted to the hospital for more intensive care, which might preserve their fertility. Recommendations include one of the following outpatient therapy combinations: cefoxitin 2.0 g intramuscularly with oral probenecid or ceftriaxone 250 mg intramuscularly or equivalent cephalosporin plus doxycycline 100 mg orally two times daily for 10–14 days.

Follow-up of patients treated on an ambulatory basis should be carried out within 48–72 h. If there is no improvement in the patient, she should be admitted for intravenous antibiotics. Recommendations from the Centers for Disease Control and Prevention for inpatient treatment include cefoxitin 2.0 g intravenously every 6 h plus a loading dose of gentamicin 2.0 mg/kg intravenously, followed by a maintenance dose of 1.5 mg/kg intravenously every 8 h. Doxycycline 100 mg orally twice daily is given after the patient is discharged from the hospital to complete a total of 10–14 days of therapy. An alternative regimen is clindamycin 900 mg intravenously every 8 h plus a loading dose of gentamicin 2.0 mg/kg intravenously, followed by a maintenance dose of 1.5 mg/kg intravenously every 8 h. Some patients may require surgery for persistent abscess or chronic pelvic pain.

Surgical Therapy Surgery becomes necessary under the following conditions: (1) the intraperitoneal rupture of a tuboovarian abscess, (2) the persistence of a pelvic abscess despite antibiotic therapy, and (3) chronic pelvic pain. At one time, total abdominal hysterectomy with bilateral salpingo-oophorectomy was considered the procedure of choice. Good antibiotics and a better understanding of the pathophysiology of the disease allow less radical surgery. In the presence of unilateral disease, a unilateral salpingo-oophorectomy may be more appropriate. The rupture of a tuboovarian abscess is a true surgical emergency. A shocklike state commonly accompanies rupture. This problem was common, and mortality approached 100 percent. With prompt surgical intervention and intensive medical management, the mortality rate today is less than 5 percent. The patient with a ruptured abscess must be explored. Hysterectomy and oophorectomy are commonly indicated.

ENDOMETRIOSIS

Endometriosis is one of the most common conditions encountered ; it is found in approximately 20 percent of all laparotomies in women in the reproductive age group. It is found most often in the third and fourth decades. Endometriosis persists into the postreproductive years. The cause of endometriosis is unknown, but the most common theory is retrograde menstruation. Endometriosis can be recognized as bluish or black lesions giving them a gunpowder burn appearance. It is found most commonly on the ovary. Other involved organs can include the uterosacral ligaments, the peritoneal surfaces of the deep pelvis, the fallopian tubes, the rectosigmoid, and a number of distant sites.

Many patients are asymptomatic even with widespread endometriosis; others have severe pain, particularly dysmenorrhea, and dyspareunia. Infertility and abnormal bleeding are common problems.

The finding of a pelvic mass and tender nodularity of the uterosacral ligament strongly suggests endometriosis. The mass usually represents an ovarian endometrioma, often referred to as a chocolate cyst. Endometriomas are found in approximately a third of women with endometriosis and are often bilateral. Although endometriosis may be suspected on the basis of clinical findings, definitive diagnosis is made laparoscopically. Medical management of this condition should not be started without a confirmed diagnosis.

Treatment Choices of treatment include expectant management only, medical management, and surgery. Asymptomatic patients can be cared for through simple observation and management with cyclic oral contraceptives and simple analgesia. Pseudomenopause is currently the most common medical treatment for endometriosis. The most common medications used today for this purpose are the gonadotropin-releasing hormone agonists (GnRH-a). They can be given by depot injection or daily nasal spray. Because bone loss is also a result of hypoestrogenism, it is recommended that the treatment not be continued for more than 6 months.

Conservative surgical therapy for endometriosis has become much more common with the advancement of laparoscopic surgery. Superficial endometrial implants can be ablated with electrocautery or laser, and ovarian endometriomas can be removed. Ovarian endometriomas deserve special consideration. These chocolate cysts cannot be treated effectively medically. Even large endometriomas can be drained and the cyst lining removed laparoscopically. Extirpative surgery is the only permanent treatment for symptomatic endometriosis. In younger patients, a normal ovary may be spared in some cases. If total hysterectomy with bilateral salpingo-oophorectomy is required, replacement hormone therapy is indicated, and recurrence is uncommon. To minimize the risk of recurrent endometriosis, it is recommended that replacement hormones include daily estrogen combined with a progestin such as medroxyprogesterone acetate 2.5 mg given orally.

ECTOPIC PREGNANCY

Women in the reproductive age group have an increased risk of ectopic pregnancy as they age. A history of salpingitis is common in women with ectopic pregnancy. Sterilization protects against ectopic pregnancy, but when sterilization methods fail, the risk of tubal implantation is increased.The most common complaint of patients with ectopic pregnancy is pain, frequently associated with irregular vaginal bleeding. Approximately 80 percent of affected women will recall a missed menstrual period. Physical findings include abdominal tenderness on cervical motion. An adnexal mass may be palpated in approximately 50 percent of patients.

The most helpful laboratory examination is measurement of the beta subunit of hCG ( b -hCG). Pelvic ultrasonography, particularly when performed with a vaginal transducer, is proving important in differentiating uterine gestations from ectopic gestations. Vaginal probe enables the clinician to determine whether the developing pregnancy is in the uterus or in the tube at a time when the hCG levels are barely more than 1000 mIU/mL. Significant intraperitoneal hemorrhage also can be visualized. In those patients who do not desire to continue the pregnancy, curettage of the uterus with examination of the tissue can be diagnostic. A diagnostic laparoscopy is usually required in the symptomatic patient for definitive diagnosis. In the presence of hemodynamic instability, immediate laparotomy is indicated.

Treatment Laparoscopic Procedures The laparoscope has been an important diagnostic tool for the last several decades, but only recently has it become the standard approach for treatment. Linear salpingostomy is the treatment of choice for ectopic pregnancies less than 4 cm in diameter that occur in the distal third (ampullary) segment of the tube. Closing the tube is not necessary because the tube closes spontaneously. Partial or total salpingectomy is indicated when the pregnancy is located in the isthmic portion of the tube. Larger ectopic pregnancies are managed by total salpingectomy because adequate hemostasis is difficult to achieve without extensive tubal damage.

Medical Therapy A relatively new approach to ectopic pregnancy is the use of methotrexate. Conservative criteria for treatment of ectopic pregnancy with methotrexate include serum b -hCG levels less than 3500 IU/L and vaginal ultrasound that reveals the tubal pregnancy to be less that 3.5 cm in diameter with no visible fetal cardiac motion and no sign of hemoperitoneum. Intramuscular methotrexate will result in complete resolution of the ectopic pregnancy in 96 percent of patients.

PELVIC SUPPORT DEFECTS

Pelvic support defects include uterine prolapse, cystocele, rectocele, enterocele, urethral detachment, and posthysterectomy vaginal prolapse. Pelvic support defects may be produced by obstetric injury, conditions that increase abdominal pressure, obesity, decreased estrogen levels, and inherent tissue weakness secondary to genetic or nutritional factors.

Uterine Prolapse Uterine prolapse is abnormal descent of the uterus relative to the bony pelvis and vagina. If the entire uterus prolapses through the introitus, the condition is considered a total prolapse; otherwise, it is partial.

Cystocele and Rectocele These conditions are due to herniation of the bladder and the rectum into the vaginal canal, generally through a widened vaginal introitus.

Enterocele An enterocele, herniation of intraperitoneal organs generally at the vaginal apex, most often follows hysterectomy. The hernia sac is lined by peritoneum. Enteroceles are frequently misdiagnosed as rectoceles.

Urethral Detachment At one time, urethral detachment was called urethrocele. In most cases, the urethrocele coexists with a cystocele.

Stress Urinary Incontinence Urinary incontinence affects almost 40 percent of all women over 60 years of age and is a common problem for younger women. Before considering operation, the patient should be evaluated with a cystometrogram.

Therapeutic Considerations Minor asymptomatic support defects may be treated expectantly or by pubococcygeal exercises. Pubococcygeal exercises involve contracting and relaxing the levator muscle repetitively several times daily. Symptoms that may require surgery include urinary stress incontinence; symptomatic prolapse of the uterus, bladder, or rectum; urinary retention; vaginal ulceration due to prolapse; and constipation secondary to rectal sacculation.

BENIGN TUMORS

Ovarian Tumors

NONNEOPLASTIC CYSTS

By definition, a cystic enlargement of the ovary should be at least 2.5 cm in diameter to be termed a cyst.

Follicular Cysts These are unruptured, enlarged graafian follicles.

Corpus Luteum Cysts These cysts become as large as 10–11 cm. They can rupture and lead to severe hemorrhage and occasionally vascular collapse.

Endometriomas These account for most chocolate cysts and are cystic forms of endometriosis of the ovary.

Wolffian Duct Remnants These are not ovarian cysts. They are small, unilocular cysts. In most instances, they are incidental findings.

NONFUNCTIONING TUMORS

Cystadenomas Serous cystadenomas appear as cysts within translucent walls containing clear fluid and lined by simple ciliated epithelium. They are adequately treated by simple salpingo-oophorectomy. Some cystadenomas are classified as borderline tumors or adenocarcinomas of low malignant potential. These (grade 0) carcinomas usually are associated with an excellent prognosis and, if they are unilateral, may be treated by unilateral adnexectomy for women in their reproductive years. Occasionally, a condition known as pseudomyxoma peritoneii is encountered; this is a locally infiltrating tumor composed of multiple cysts containing thick mucin. These tumors arise either from ovarian mucinous cystadenomas or from mucoceles of the appendix, both of which commonly coexist.

Mature Teratoma These germ cell tumors are thought to arise from the totipotential germ cells of the ovary. The tumors often contain calcified masses, and occasionally either teeth or pieces of bone can be seen on abdominal radiographs. If a teratoma (dermoid) is encountered in a young woman , it is preferable to shell it out from the ovarian stroma, preserving functioning tissue in the affected ovary. These cysts contain ectodermal, mesodermal, and endodermal tissues in addition to a thick, greasy, fatty material. If spilled during surgery, a chemical peritonitis may result; therefore, it is important to remove these tumors intact. In approximately 12 percent of patients, these tumors are bilateral.

Brenner Tumor These are rare epithelial tumors that usually do not secrete hormones. Histologically, the epithelial elements are similar to Walthard rests and are believed to arise from these. Simple oophorectomy is usually sufficient therapy.

Meig's Syndrome This pertains to ascites with hydrothorax, seen in association with benign ovarian tumors with fibrous elements, usually fibromas. Meig's syndrome can be cured by excising the fibroma.

FUNCTIONING TUMORS

Granulosa Cell–Theca Cell Tumor Pure theca cell tumors (thecomas) are benign, but those with granulosa cell elements may be malignant. Usually, granulosa cell tumors elaborate estrogen; these tumors have no hormone production. In young girls they are characteristically manifested by isosexual precocity, and in elderly women they are sometimes associated with endometrial carcinoma . If the tumor is discovered in the reproductive years and confined to one ovary without signs of surface spread or dissemination , a simple oophorectomy may be sufficient therapy. If it is discovered in later life, removal of both ovaries with the uterus is indicated.

Sertoli–Leydig Cell Tumors (Arrhenoblastomas) These rare but potentially malignant tumors are associated with androgen output and masculinization. They usually occur in the reproductive age group and appear to contain tubular structures as well as Leydig-type cells. In young patients with a single involved ovary, unilateral oophorectomy is adequate therapy, provided there is no extension of the tumor. For older patients or for those with bilateral involvement, total hysterectomy and bilateral salpingo-oophorectomy are performed.

Struma Ovarii This refers to the presence of grossly detectable thyroid tissue in the ovary, usually as the predominant element in dermoid cysts. This tissue occasionally may produce the clinical picture of hyperthyroidism.

UTERINE TUMORS

Leiomyomas Uterine leiomyomas are the most common benign tumor in the female pelvis. It is estimated that up to 50 percent of all women at some time in their life have one or more of these uterine tumors. Many leiomyomas are asymptomatic; when they do produce symptoms, they cause pain, abnormal uterine bleeding, infertility, ureteral obstruction, bladder distortion, and pressure symptoms secondary to the enlarged uterus. Uterine leiomyomas are subject to a number of degenerative changes, including calcification, necrosis, and fatty degeneration. Malignant degeneration occurs in less than 1 percent of all tumors.

Treatment Most symptomatic tumors can be managed expectantly. When symptoms indicate surgical treatment, surgery should be fitted to the needs and desires of the patient. Therapeutic options might include myomectomy, total abdominal hysterectomy, or transvaginal hysterectomy.

Adenomyosis Adenomyosis is a growth of endometrial tissue in the myometrium of the uterus and is sometimes referred to as endometriosis of the uterine corpus. The condition occurs during reproductive years and leads to a thickening of the myometrial wall.

Polyps A polyp is a local hyperplastic growth of endometrial tissue that usually causes postmenstrual or postmenopausal bleeding or staining, which is cured by polyp removal or curettage. The polyps are usually benign, but cases of adenocarcinoma of the endometrium arising in a polyp have been reported .

Cervical Lesions Cervical polyps cause the same symptoms as endometrial polyps. They often can be removed as an outpatient procedure followed by cauterization of the base of the polyp. Nabothian cysts are mucous inclusion cysts of the cervix. They are harmless, usually asymptomatic, and generally do not require surgery.

VULVAR LESIONS

The term leukoplakia is often used for any white patch of the vulva. These alterations may precede the development of malignant changes. Lichen sclerosus is a pruritic lesion that does not appear to be premalignant. Hyperplastic lesions termed hypertrophic dystrophies are found that may be benign (epithelial hyperplasia) or may show atypia, in which case dysplastic changes can be observed . The pruritic symptoms can be helped by topical application of corticosteroids or testosterone . Noninvasive malignant change of the surface squamous epithelium of the vulva occurs in the same way as that described for the cervix. Carcinoma in situ of the vulva both histologically and clinically behaves like carcinoma in situ of the cervix. Bowen's disease and usually Paget's disease are considered part of the carcinoma in situ complex of the vulva; they are adequately treated by wide local surgical excision (simple vulvectomy). The laser also is used to treat these lesions locally.

Malignant Tumors

OVARIAN TUMORS

Ovarian Carcinoma Ovarian carcinomas are divided histologically into epithelial, germ cell, and stromal malignancies. The majority of the 26,700 or more cases of ovarian cancer diagnosed annually in the United States are of the epithelial type. The median age at diagnosis for epithelial ovarian cancer is 61 years, and the overall 5-year survival rate for epithelial cancers is 37 percent.

Although the etiology of ovarian cancer is uncertain , approximately 5 percent of patients with epithelial tumors come from families where one or more first-degree relatives also have the disease. In such families, prophylactic oophorectomy may be considered at the completion of childbearing, especially if specific BRCA1 mutations are identified. Primary peritoneal carcinomatosis has been reported in women who have undergone prophylactic surgery, however.

The FIGO (International Federation of Gynecology and Obstetrics) staging system for ovarian cancer is outlined in Table 39-2. Efforts to establish other cost-effective screening programs using serum markers such as CA-125 and vaginal ultrasound examination are being developed. Vaginal ultrasound is a promising technology that is not presently cost-effective in screening programs. Currently, the majority of women with epithelial cancers have stage III tumors at the time of diagnosis.

TABLE 39-2 FIGO (1986) STAGING SYSTEM FOR OVARIAN CANCER

Treatment Therapy for epithelial ovarian cancer consists of surgical resection and appropriate staging followed by chemotherapy. Women with low-grade early-stage (IA or IB) cancers may be treated with surgery without adjuvant therapy. In all other patients (stage IA, grade 3, and stage IB and above), appropriate initial surgery includes bilateral salpingo-oophorectomy, abdominal hysterectomy if the uterus has not been removed on a prior occasion, appropriate staging, and tumor resection.

Staging Staging indicates surgical resection or biopsy of all potential areas of tumor spread. Among patients whose cancer is confined to one or both ovaries at the time of gross inspection, occult metastases can be identified by careful surgical staging in one-third. Epithelial ovarian cancers disseminate along peritoneal surfaces and by lymphatic channels. The omentum is a common site for metastases, as are both the paraaortic and pelvic lymph nodes.

The terms debulking and cytoreduction have been introduced to indicate aggressive surgical removal of ovarian cancer. When disease remaining after surgical resection consists of nodules or plaques less than 1–2 cm in diameter, the surgical effort is termed optimal, and when a larger volume of residual disease remains, the surgical removal is termed suboptimal. Because of the survival advantage, every effort should be made to resect as much disease at the time of diagnostic laparotomy as is possible.

Second Look Operations Second Look Laparotomy Ovarian cancer often defies diagnosis because it does not produce symptoms and is detectable neither radiographically nor serologically even in relatively advanced stages. CA-125 is more sensitive than radiographic or magnetic resonance scanning but is also associated with a number of false-positive results and may not be elevated in patients with mucinous tumors. In addition, approximately half of patients with advanced ovarian cancer whose CA-125 levels normalize during chemotherapy harbor viable and clinically undetectable disease.

Second look surgery is currently used primarily as a research tool. New treatment regimens can be evaluated quickly by performing a second look operation. Second look laparotomy is performed through a midline abdominal incision. Peritoneal washings are obtained from both abdominal gutters, the diaphragms, and the pelvis. Since persistent cancer is most likely to be identified in sites where there was tumor at the conclusion of the primary operation, these areas are explored first.

Other Secondary Operations Surgical resection of tumor after chemotherapy or at the time of relapse is termed secondary cytoreduction. In the occasional patient who undergoes diagnostic biopsy only before the administration of chemotherapy, early reexploration may be termed interval cytoreduction. In patients with a massive tumor burden , this approach not only may be safer but also might result in a more successful tumor resection before the completion of chemotherapy.

Palliative Surgery In most cases of advanced ovarian cancer, death is associated with bowel dysfunction or frank obstruction. When bowel obstruction occurs early on in the clinical course of ovarian cancer, and particularly if it occurs before the administration of chemotherapy, surgical intervention is warranted and should be aggressive. When bowel obstruction occurs after chemotherapy, the prognosis is unfavorable. Surgery is often difficult to perform because of extensive tumor. Laparotomy may be complicated by enteric injury or fistula. Often the best approach in these patients is the use of a percutaneous or endoscopically positioned gastrostomy tube and intravenous fluids or conservative nutritional support.

Laparoscopy in Ovarian Cancer At present, our ability to resect large ovarian cancers successfully using laparoscopic equipment is limited. With the advent of new equipment and techniques, the role of laparoscopy in the staging and treatment of ovarian malignancies is expanding. Several investigators have developed successful methods of performing both pelvic and paraaortic lymphadenectomies using endoscopic equipment.

Tumors of Low Malignant Potential These are epithelial tumors of malignant potential intermediate between benign lesions and frank malignancies. Most are of the serous type. They are distinguished from invasive cancers microscopically by the lack of stromal invasion. The median age of diagnosis is approximately 10 years younger than that of patients with epithelial cancers. The vast majority occur in stage I. Surgery should include abdominal hysterectomy and bilateral salpingo-oophorectomy unless fertility is to be preserved in patients with unilateral lesions. These patients may undergo unilateral salpingo-oophorectomy. Patients with stage III and IV lesions have 5-year survival rates that approach 85 percent after complete surgical resection. There is little evidence that either chemotherapy or radiotherapy improves survival.

Germ Cell Tumors These tumors occur in women in the first three decades of life and typically grow rapidly . Most are unilateral, and all have a tendency to spread to the paraaortic lymph nodes. Dysgerminoma, the female equivalent of testicular seminoma, is composed of pure, undifferentiated germ cells. It is bilateral in 10 percent of patients and is occasionally associated with elevated levels of hCG or lactate dehydrogenase (LDH). It is the most common ovarian malignancy diagnosed during pregnancy. Patients should undergo appropriate staging at the time of the primary resection but need not undergo hysterectomy (if fertility is to be preserved) or removal of the opposite ovary if it is normal in appearance. Adjuvant therapy is unnecessary unless there is evidence of extraovarian spread. This tumor is exquisitely sensitive to chemotherapy or radiation.

The other germ cell tumors in order of frequency are immature teratoma, endodermal sinus or yolk sac tumor, mixed tumors, embryonal carcinomas, and choriocarcinomas. The first may be associated with elevated levels of alpha-fetoprotein (AFP). Elevated AFP levels are found in all patients with endodermal sinus tumors and mixed tumors that contain this component. Embryonal carcinomas are associated with abnormal levels of both AFP and hCG, and choriocarcinomas secrete hCG. These tumors are invariably unilateral. Except for those with completely resected stage I, grade 1 immature teratomas and those with stage I dysgerminoma, all patients with germ cell tumors require systemic chemotherapy. Three courses of a platinum and etoposide-containing combination suffice in those patients whose tumors are completely resected. Cure rates in these patients approach 90 percent.

CARCINOMA OF THE CERVIX

Carcinoma of the cervix accounts for about 16,000 cases and 5000 deaths annually in the United States. Risk factors include multiple sexual partners , early age at first intercourse, and early first pregnancy. DNA related to that found in the human papillomavirus has been identified in cervical dysplasia and carcinoma in situ, both precursor lesions, as well as in invasive cancers and lymph node metastases. In no cancer has widespread screening had as profound an impact on mortality as it has in carcinoma of the cervix. Georges Papanicolaou devised the cytologic smear that bears his name in 1943. Since that time, screening programs have dramatically reduced the rate of invasive cervical cancer. The Pap smear has shifted the frequency of cervical abnormalities toward the premalignant intraepithelial diseases, dysplasia, and carcinoma in situ. All intraepithelial lesions are noninvasive and can be treated successfully using conservative methods. Eighty percent of all cervical cancers are squamous cell in type and arise from the squamocolumnar junction of the cervix. The remainder of cervical malignancies arise in the endocervical canal and are either adenocarcinomas or adenosquamous carcinomas. Other rare histologic varieties associated with poor prognosis are neuroendocrine small cell carcinomas and clear cell cancers. The latter are frequently associated with maternal exposure to diethylstilbestrol.

Staging Cervical cancers spread predominantly by lymphatic channels. The first lymph nodes involved are the paracervical or parametrial area. The supraclavicular lymph nodes are the most common site of distant nodal metastases. FIGO staging for cervical cancer is based on clinical examination, intravenous pyelography, and chest radiography. The FIGO staging system is illustrated in Table 39-3. All patients with stage IIB cancer and above are treated primarily with radiotherapy in the United States.

TABLE 39-3 FIGO STAGING SYSTEM FOR CERVICAL CANCER

Treatment Intraepithelial or Preinvasive Disease Abnormal Pap smears must be evaluated by colposcopy and biopsy. Colposcopy is examination of the cervix with a low-power (10–50) microscope after application of dilute acetic acid to the cervix. The acid solution is mucolytic and serves to desiccate the epithelium, a process that brings out subtle epithelial patterns referred to as white epithelium, punctation, mosaicism, and abnormal vasculature. Cervical intraepithelial neoplasia is treated in a number of ways. In general, the larger the lesion and the higher the grade of dysplasia, the greater is the failure rate. The most definitive treatment for cervical intraepithelial neoplasia is vaginal or abdominal hysterectomy. Cervical cone biopsy is curative in most cases of cervical intraepithelial neoplasia. In patients in whom the surgical margins of the cone specimen are uninvolved, the risk of recurrence is less than 5 percent. If the surgical margins are involved, half of such patients will develop recurrent disease.

More conservative methods of treating cervical intraepithelial neoplasia include wire loop excision, laser vaporization, and cryosurgery. Loop excision can be done under local anesthesia (paracervical block) in the outpatient setting.

Microinvasive Cervical Cancer Simple hysterectomy is adequate therapy. In some, cone biopsy and excision may be used, provided close surveillance is possible.

Early Invasive Cervical Cancer (Stage IB and IIA) Stage IB and IIA tumors are associated with a risk of pelvic lymph node metastases of 10–15 percent and a risk of spread to the paraaortic nodes of about 5 percent. Radical hysterectomy with pelvic lymphadenectomy or definitive radiotherapy is effective treatment in this stage cancer. Women with stage IB ₂ cervical cancers ( exceeding 4 cm in diameter), especially those endocervical primaries that distend the cervix circumferentially, may require a combination of radiotherapy and surgery. These large endocervical tumors are referred to as barrel lesions and are refractory to surgery or radiotherapy alone.

Stage IB ₁ lesions and early stage IIA cancers may be treated successfully with radical hysterectomy and pelvic lymphadenectomy. Because early cervical cancer so rarely spreads to the ovaries, radical hysterectomy need not include oophorectomy. Ovarian preservation is one of the strongest arguments for the use of surgery over radiotherapy.

Locally Advanced Carcinoma of the Cervix (Stages IIB to IVA) These cancers are treated primarily with radiotherapy. Treatment consists of a combination of external therapy to the pelvis (teletherapy) from a high-energy source such as a linear accelerator and a local dose delivered to the cervix and parametrial tissue (brachytherapy) using a cesium applicator such as a Fletcher-Suite tandem and ovoids.

The finding of metastases in the common iliac or paraaortic chain indicates the need for extended-field radiotherapy encompassing these areas in addition to the pelvis. Even with such therapy, 5-year survival rates are low, seldom exceeding 20 percent.

Recurrent Cervical Cancer As a rule, patients who develop local recurrences after preliminary surgical therapy are treated most effectively with external- and internal-beam radiotherapy. Although those with lymph node failures may not be curable in this setting, those with vaginal recurrences often can be saved with such an approach. Women who develop recurrent cancer following primary radiotherapy are generally not candidates for curative therapy. If, however, the recurrent lesion is small, the interval to failure is a year or more, and the lesion is unaccompanied by symptoms such as back or leg pain or edema, surgical resection may be possible. Most gynecologic oncologists prefer to perform pelvic exenteration in such circumstances. Often, an anterior exenteration with en bloc removal of the bladder, cervix, uterus, and upper vagina is feasible . The preferred method of diversion in these patients is the creation of a sigmoid urostomy or transverse colon conduit. Other surgical options include a Koch pouch or the Indiana reservoir, both of which provide a means of urinary continence without an external applicance.

In general, about half the patients thought to be candidates for pelvic exenteration are found to have intraperitoneal spread or nodal metastases at the time of exploratory laparotomy and, in most centers, do not undergo resection. Of the remaining patients in whom surgery is possible, 30–50 percent will develop a second, nearly always fatal recurrence after surgery.

ENDOMETRIAL CANCER

Endometrial cancer is the most common female genital malignancy, accounting for 34,000 cases annually in the United States. It is a highly treatable cancer, and only 6000 deaths are reported each year.

Risk factors for endometrial cancer include obesity, diabetes mellitus, hypertension, low parity, early menarche, and late menopause. Excessive exposure to estrogens is implicated in the genesis of endometrial cancer and its precursor, endometrial hyperplasia. Women who take estrogens in the menopausal years are known to have a sixfold increase in the risk of endometrial cancer if progestational agents are not taken as well. There is also an increase in the incidence of endometrial lesions in women with a history of chronic anovulation (Stein-Leventhal syndrome) and in those with estrogen-producing ovarian stromal neoplasms such as granulosa cell tumors. Endometrial hyperplasia may be divided into simple and complex, depending on the microscopic architecture, and into those with or without atypia. Atypical complex hyperplasias are most likely to give rise to frank adenocarcinomas. Simple hysterectomy is the preferred method of treatment for the hyperplasias. In women with underlying health problems that preclude surgical therapy, therapy with progestational agents such as megestrol or medroxyprogesterone acetate may be used with success.

Treatment Endometrial cancer is staged according to the FIGO criteria detailed in Table 39-4. Pelvic lymph node metastases occur in about 12 percent of patients with endometrial cancer apparently confined to the uterus.

TABLE 39-4 FIGO (1988) STAGING SYSTEM FOR ENDOMETRIAL CANCER

Risk factors associated with lymph node spread include high histologic grade (grade 2 or 3), low levels of progesterone receptor, deep myometrial or lymphatic channel invasion, spread to the adnexa, endocervical extension, and unusual histologic variants such as papillary serous or clear cell carcinomas.

Vaginal hysterectomy is occasionally useful in patients with early endometrial cancer when lymph node metastases are thought to be unlikely . This operation is particularly well suited for massively obese parous patients. It is critical to remove the ovaries in women undergoing surgery for endometrial cancer because 5 percent harbor occult metastases. Radiotherapy alone may be the treatment of choice in patients at excessive risk for operative intervention.

VULVAR CANCER

Vulvar cancer accounts for about 5 percent of all gynecologic cancers. Although uncommon histologic types such as malignant melanoma and adenocarcinoma of the Bartholin's gland occur, over 90 percent of vulvar malignancies are squamous carcinomas. Epidemiologic risk factors include older age, smoking, previous intraepithelial or invasive squamous cancer of the cervix or vagina, chronic vulvar dystrophy, and immunocompromise. Human papillomavirus-like DNA has been identified in both preinvasive and invasive squamous carcinomas of the vulva. It is likely that the human papillomavirus plays an important role. Spread of squamous carcinoma of the vulva is primarily via the lymphatics of the vulva.

The 1988 FIGO staging system for vulvar cancer (Table 39-5) is currently accepted. This system requires surgical evaluation of the inguinal lymph nodes and provides a schema in which prognosis and therapy are closely linked with stage.