Fairleigh Dickinson University Maternal & Child Medicine Vuvla, Vagina, Endometrial, & Ovarian Neoplasms PDF

Christina Bottiglierie, MS PA-C PHAS 6320 Maternal & Child Medicine Vulvar, Vaginal, Uterine, Ovarian neoplasms (benign and malignant)  Topics List Vaginal / vulvar neoplasms Endometrial neoplasms Ovarian neoplasms  Learn...

Christina Bottiglierie, MS PA-C PHAS 6320 Maternal & Child Medicine Vulvar, Vaginal, Uterine, Ovarian neoplasms (benign and malignant)  Topics List Vaginal / vulvar neoplasms Endometrial neoplasms Ovarian neoplasms  Learning objectives Anatomy review Vagina/vulva Neoplasm Ovaries Ovarian cyst/Ruptured ovarian cyst Polycystic ovarian syndrome Ovarian cancer Ovarian torsion Uterus Endometrial Cancer Endometriosis Adenomyosis Leiomyoma...  Vagina Fibromuscular canal approximately 7–9 cm long Extends from the uterus to the vestibule of the external genitalia, where it opens to the exterior Blood supply Vaginal branch of the uterine artery Innervation Contains both sympathetic and parasympathetic fibers  Vulva Collective term for the external part of the female genitalia Consists of: Mons pubis - a hair-covered fat pad overlying the symphysis pubis Labia majora - rounded folds of adipose tissue forming the outer boundaries of the vagina Originating in the mons pubis and end at the perineum Labia minora – the thinner, inner folds of skin Extends anteriorly to form the prepuce Clitoris - homologue to the penis 2 cm in length  Bartholin’s Glands Primary function is the production of a mucoid secretion that aids in vaginal and vulvar lubrication The glands are located in the vulvar vestibule, at either side of the external orifice of the vagina They become active after menarche and are non- palpable. Each gland is oval-shaped and measures, on average, 0.5 cm. A two-centimeter-long efferent duct connects each gland to the posterolateral aspect of the vaginal orifice (between the hymen and the labia minora)  Vaginal Cancer Rare: 1% of gynecological malignancies and is usually secondary to other cancers (METS) Arising from adjacent gynecologic structures: endometrium, cervix, vulva, ovary and breast) Peak incidence at 60-65 years of age Squamous cell represents 95%, caused by HPV Adenocarcinoma caused by DES exposure Also can be from melanoma or sarcoma The most common location of vaginal carcinoma is the upper one-third of the posterior vaginal wall Cancer of the vagina is usually ASYMPTOMATIC and found by abnormal cytology Early sx: painless bleeding from ulcerated tumor Late sx: bleeding, pain, weight loss, swelling Most vaginal cancers are SQUAMOUS CELL  Vaginal Cancer RF Smoking HPV infection Multiple sex partners H/o lower genital tract neoplasia Clear cell variant is associated with in utero DES exposure Most common site for vaginal cancer is the upper vagina. Diagnosis Made by routine exam and confirmed with biopsy of the lesion via colposcopy DDX Benign tumor (uncommon) Ulcerative lesion from direct trauma or inflammatory reaction Granulomatous venereal disease Endometriosis which penetrates the cul-de-sac of Douglas into upper vagina  Vaginal Cancer Treatment Surgery (local excision) for Stage 1 lesions with consideration of radical hysterectomy, upper vaginectomy, and pelvic lymphadenectomy Primary radiotherapy with brachytherapy for small superficial lesions External beam radiotherapy for larger lesions Prognosis Size and stage of disease at time of diagnosis are most important prognostic indicators in squamous cell cancers Vaginal tumors are staged clinically based on findings from physical and pelvic examination, cystoscopy, proctoscopy, and chest and skeletal radiography 5-year survival rate Stage 1=77% Stage 2=45% Stage 3=31% Stage 4=18%  Vaginal Cancer FIGO Nomenclature Stage I The carcinoma is limited to the vaginal wall. Stage II The carcinoma has involved the subvaginal tissue but has not extended to the pelvic wall. Stage III The carcinoma has extended to the pelvic wall. Stage IV The carcinoma has extended beyond the true pelvis or has involved the mucosa of the bladder or rectum; bullous edemas as such does not permit a case to be allotted to stage IV. IVa - Tumor invades bladder and/or rectal mucosa and/or direct extension beyond the true pelvis. IVb - Spread to distant organs. FIGO = Fédération Internationale de Gynécologie et d’Obstétrique.  Vulvar Cancer Peak incidence is at 50 years old (ave age 68) Vaginal pruritis the most common presentation (70%) Histologic types: Squamous cell *Common Melanoma, basal cell carcinoma, Bartholin gland adenocarcinoma, sarcoma, and Paget disease 90% are squamous cell cancers and melanoma Cancer of the vulva is uncommon (4%) Risks factors: HPV subtypes 16, 18, and 31 – pruritic black lesions Cigarette smoking (most frequent association) Immunodeficiency syndromes H/o cervical cancer or dysplasia Chronic h/o vulvar irritation / pruritis (vulvar lichen sclerosis) A family history of melanoma and dysplastic nevi anywhere on the body may increase the risk of vulvar cancer Exam findings Early lesions: similar to chronic vulvar dermatitis. Late lesions: large cauliflower appearance or hard ulcerated area of the vulva  Vulvar Cancer Diagnosis Application of acetic acid or staining with toluidine blue may help direct optimal biopsy location DDX Epidermal inclusion cysts Seborrheic dermatitis Lichen sclerosis Condyloma Granulomatous venereal disease Pyogenic infections Benign tumor (granular cell myoblastoma) Treatment Prognosis depends of presence or absence of lymph node metastasis, size and location of lesion and histologic type. Lymph node status is most important prognostic variable. Surgery for staging and treatment. Complete surgical removal all tumor when possible. Common procedure is wide radical local excision with inguinal lymph node dissection Adjuvant radiation therapy if cancer has spread to lymph nodes  Vulvar Cancer Follow up Post op period: examine q 3 months x 2 years and q 6 months thereafter to detect recurrence. Nearly 80% of recurrence occurs within the first 2 years. Prevention The HPV vaccine can prevent the strains of HPV responsible for most vaginal, vulvar, and cervical cancers  Vulvar Cancer Squamous cell carcinoma(most common) Grade 1: well differentiated, forming keratin pearls Grade 2: moderately well differentiated Grade 3: poorly differentiated Keratinizing type is assoc with chronic vulvar irritation and older women Warty type is assoc with HPV and immunodeficiency seen in younger women. Verrucous carcinoma is a SCC variant. It resembles a mature condylomatous growth. Histopathology of base shows papillary fronds with no central core. Treatment is wide vulvectomy  Vulvar Cancer Carcinoma of Bartholin’s Gland: rare, but most common site for vulvar adenocarcinoma. Basal Cell Carcinoma: small elevated lesions with ulcerated center and rolled edges. “Rodent ulcers”. Most arise from skin of labia major. Wide local excision necessary to prevent recurrence Malignant Melanoma: 2nd most common vulvar cancer. Darkly pigmented, raised lesion is characteristic. Arises from labia minor and clitoris. May also be non pigmented or amelanotic. Excisional biopsy with wide margins for removal  Ovary Anatomy & Function Small, oval-shaped glands Located on either side of your uterus Produces an ovum Secrete hormones Control menstrual cycle and fertility  Ovarian Cyst Ovarian cyst - sac of fluid that form on or in an ovary Usually 1 to 2 inches wide, but they can be bigger Common in females of reproductive age Physiologic cysts (more common) Follicular cyst, corpus luteal cyst Pathologic cysts (less common) Endometriomas, benign adult teratomas, cystadenomas, malignant neoplasms Histology Epithelial (most common) Common causes: Ovulation or pregnancy Dermoid cysts Polycystic ovary syndrome (PCOS) Endometriosis Cancer Diagnosis - ultrasound  Ovarian Cyst Treatment (general) Treat the underlying condition Watchful waiting – repeat ultrasound every couple of months to reevaluate the size of the cyst OCP Limits new cysts from growing Surgery to remove a cyst or the whole ovary  Ruptured Ovarian Cyst Presentation Cyst rupture may be asymptomatic or symptomatic (pain) Associated with mid-cycle pain (mittelschmerz) If Symptomatic: characterized by the sudden onset of unilateral, lower abdominal pain Often following strenuous physical activity (sexual intercourse, exercise) Bleeding Concerns: The release of cyst contents into peritoneal cavity causing irritation Serous fluid, blood, sebaceous material Diagnosis Ultrasound findings of an ovarian cyst plus blood or a large amount of serous fluid in the pelvis Differential diagnosis Ectopic pregnancy, adnexal torsion, appendicitis, and tubo-ovarian abscess  Ruptured Ovarian Cyst Management Uncomplicated (most cases) – observation Complicated cases (ie, hemodynamic instability, large or ongoing blood loss, signs of an infection process, findings suggestive of malignancy) may require inpatient management and/or surgery Surgery Laparoscopy In a premenopausal patient with a benign ovarian cyst (physiologic or nonphysiologic), preservation of ovarian tissue via cystectomy is generally preferable to complete oophorectomy In a postmenopausal patient, unilateral oophorectomy is generally performed; bilateral salpingo-oophorectomy is only indicated if malignancy is suspected Polycystic Ovarian Syndrome (PCOS) Polycystic Ovarian Syndrome (PCOS)  Polycystic Ovarian Syndrome (PCOS) Endocrine disorder affecting 5-10% of women of reproductive age Polycystic ovarian syndrome (PCOS) may be diagnosed when two of the following three criteria are met: Oligomenorrhea Hyperandrogenism Polycystic ovaries on ultrasound Patients may present with menstrual irregularities, infertility, hirsutism, acne, obesity, ovarian enlargement, and acanthosis nigricans. Increased risk of diabetes mellitus, cardiovascular disease and metabolic syndrome, nonalcoholic fatty liver disease NASH now called MASH), endometrial cancer The pathophysiology of the disorder is poorly understood, but it is thought to be associated with hypothalamic pituitary dysfunction and insulin resistance. Polycystic Ovarian Syndrome (PCOS) Polycystic Ovarian Syndrome (PCOS)  Polycystic Ovarian Syndrome (PCOS) Polycystic ovaries — Polycystic ovary syndrome (PCOS) Results in enlarged ovaries from multiple small follicular cysts Clinical Features 1 Menstrual dysfunction Menarche may be delayed Oligomenorrhea Fewer than nine menstrual periods in a year Amenorrhea (less often) No menstrual periods for three or more consecutive months Women with PCOS often experience more regular cycles after age 40 years 2 Hyperandrogenism Hirsutism, acne, male-pattern hair loss Elevated serum androgen concentrations (hyperandrogenemia) Virilization - Signs of more severe androgen excess Deepening of the voice and clitoromegaly (rare)  Polycystic Ovarian Syndrome (PCOS) 3 Hirsutism Excess body hair in a male distribution Above the upper lip, chin, periareolar area, in the midsternum, and along the linea alba of the lower abdomen Diagnosis Transvaginal ultrasound (TVUS) Polycystic ovaries Management Polycystic ovaries themselves do not require treatment Treat clinical manifestations Manage abnormal uterine bleeding, infertility, insulin resistance, obesity, and hirsutism Other clinical manifestations related to PCOS Metabolic issues/cardiovascular risks, sleep apnea  Polycystic Ovarian Syndrome (PCOS) Ultrasonography may demonstrate a characteristic “string of pearls” appearance within the ovaries. Diet and exercise are recommended for all patients Metformin and GLP 1’s can help with weight loss as well as regulating menstruation. Oral contraceptives are effective in treating hirsutism and acne. Infertility is typically treated with metformin or clomiphene citrate; in rare cases ovarian cautery and laser vaporization are used. Polycystic Ovarian Syndrome (PCOS)  Hirsutism in PCOS Excess growth of dark and coarse hair over areas of the body where it ordinarily wouldn't grow, such as the face and back Polycystic Ovarian Syndrome (PCOS) Polycystic Ovarian Syndrome (PCOS)  Test your knowledge 16-year-old female with irregular menses, fatigue, and hirsutism. Vital signs normal BMI 32.8 Exam shows obese female with acne. Which of the following are the best tests for an initial evaluation? A Glucose, insulin, c peptide, Hgb A1c B TSH, insulin, free testosterone C DHEAS and morning cortisol D Prolactin, FSH, LH  Ovarian Cancer What is the strongest known risk factor? Family history: BRCA1 and BRCA2 mutations  Ovarian Cancer Second most type of GYN cancer in women (the first is endometrial cancer) The most common cause of gynecologic cancer death in the US Population: 40-60 years of age, ascites, abdominal pain ⇒ 75% diagnosed at an advanced stage Average age at diagnosis of ovarian cancer in the US is 63 years old If a woman has ascites ovarian cancer is the most likely tumor to be found Protective factors for the risk of ovarian cancer include multiparity, OCP use, breast-feeding IUD and tubal ligation Use of oral contraceptives: 5 years of use decreases risk by 20%; 15 years by 50% Risk factors: Null gravidity (or infertility), increasing age, PCOS, early menarche, late menopause, endometriosis, smoking, BRCA1, BRCA2 90% are epithelial tumors ⇒ germ cell tumors are more common in patients < 10 years old  Ovarian Cancer Symptoms EARLY STAGE: vague symptoms, not severe enough to seek medical attention LATE STAGE: more than 70% of all diagnoses. Increased abdominal girth (ascites or tumor mass) Abdominal or pelvic pain, bloating Urinary frequency or urgency Early satiety Exam Pelvic exam will reveal fixed, solid, irreg adnexal mass. May show presence of ascites or upper abdominal mass. Abdominal distention is common. Differential dx: Unilateral cystic mass 80%  Uterine / Endometrial Cancer 4 Major Categories 1. Leiomyosarcoma (LMSs)-arise from myometrial smooth muscle or cell lining vessels in myometrium. Make up 35-40% of all uterine sarcomas. MOST COMMON symptom is AUB! Followed by pelvic/abdominal pain Difficult to dx with D&C due to deeply situated intramural position of tumor Usually dx by pathologic analysis of hysterectomy specimen  Uterine / Endometrial Cancer 2. Endometrial sarcomas: Endometrial stromal sarcoma (ESS) 8% of all sarcomas. Most common sx are bleeding and lower abdominal pain. Dx usually made by D&C. Can be low grade, endometrial stroll nodules, or high grade, undifferentiated. Carcinosarcoma (MMMT, malignant mixed mesodermal tumors) 50% of all sarcomas. Most occur in postmenopausal women. Etiology is unknown. Presents with bleeding and abdominal pain. May have large, bulky mass filling uterine cavity and prolapsing through the cervix. Can be diagnosed by D&C as most are endometrial in origin.  Uterine / Endometrial Cancer 3. Adenosarcomas: 1-2% of uterine sarcomas. Usually occur in post menopause but can be found in adolescents and women of reproductive age. Arises from endometrium and most common symptom isbleeding. Associated with prior Tamoxifen or radiation thx. 4. Other uterine sarcomas: Embryonal rhabdomyosarcoma of the cervix: occurs in infants and children. Fibrosarcoma, hemangiosarcoma, reticulum cell sarcoma, hemangiopericytoma are rare.  Uterine / Endometrial Cancer What are the most common sites for early metastasis of uterine sarcoma? Abdomen Liver Lung (coin lesions on CXR are characteristic) Disorders of the Gynecological System  Pelvic Pain Should be categorized as being acute or chronic; cyclic or non-cyclic. Causes include: Primary Dysmenorrhea: Menstrual pain associated with cycles in the absence of pathology 75% of women are affected and 5-6% have incapacitating pain Treat with NSAIDs, heat therapy, oral contraceptives or IUD Endometriosis Aberrant growth of endometrium outside of the uterus Symptoms: chronic pain, dyspareunia, and infertility Exam may show tender nodules in the cul-de-sac or rectovaginal septum, uterine tenderness or adnexal mass or tenderness; “chocolate cysts” seen on laparoscopy Cause: retrograde menstruation, prevalence of 6-10% Treatment: oral contraceptives, IUD, laparoscopic ablation Common conditions assoc. with chronic pelvic pain Endometriosis  Endometriosis Definition Presence of both endometrial glands & stroma outside the uterine cavity and musculature Epidemiology Mostly women of reproductive age All races Prevalence: 7-10% Strong family association Risk factors: nulliparity, prolonged exposure to estrogen, exposure to DES in utero, & lower BMI  Endometriosis Pathogenesis Direct implantation of endometrial cells Vascular & lymphatic dissemination of endometrial cells Cells in peritoneal cavity develop into endometrial tissue Pathology Present as hemorrhagic petechiae, white plaques, chocolate cysts, rust colored spots Occur on ovaries, pelvic structures, sigmoid colon  Endometriosis Symptoms Pelvic pain Infertility Dysmenorrhea Dyspareunia Abnormal bleeding Signs Fixed uterus Tenderness Endometriomas on ovary None! ** Location of endometriosis can affect the symptoms the patient  Endometriosis Differential diagnoses Appendicitis, Chlamydia, UTI, Cystitis, Diverticulitis, Ectopic Pregnancy, Gonorrhea, Ovarian Cysts, Ovarian Torsion, Pelvic Inflammatory Disease Diagnosis Direct visualization & biopsy by laparoscopy Treatment Oral contraceptives Oral & IM progestins Danazol – suppresses LH & FSHGnRH agonist injections (Lupron) Surgery  Endometriosis Hormonal contraceptives decrease fertility by preventing the monthly growth and buildup of endometrial tissue Reduces the pain The medroxyprogesterone (Depo-Provera) injection is also effective in stopping menstruation Stops the growth of endometrial implants  Endometriosis Conservative surgery Women who want to get pregnant Hormonal treatments are not effective The goal of conservative surgery is to remove or destroy endometrial growths without damaging the reproductive organs Laparoscopy (minimally invasive surgery) Used to both visualize and diagnose endometriosis Also used to remove the endometrial tissue Non-conservative Total hysterectomy  Endometriosis Complications Endometriosis is associated with an increased risk of epithelial ovarian cancer (EOC) Prognosis Prognosis for reproductive function in early or moderately advanced endometriosis appears to be good with conservative therapy Patient education It is important to stress to patients the importance of continuing medical therapy for the full 6 months Pt’s should return for recurrent symptoms  Adenomyosis Definition: Ectopic placement of endometrial tissue in myometrium Most likely results from microtrauma to the myometrium and subsequent deposition of endometrial implants into the damaged area Clinical Findings: Asymptomatic, pelvic pain / pressure, dyspareunia, dysmenorrhea, infertility, menorrhagia, chronic pelvic pain  Adenomyosis Clinical Findings: Asymptomatic, pelvic pain / pressure, dyspareunia, dysmenorrhea, infertility, menorrhagia, chronic pelvic pain Diagnosis TVUS, MRI – may see diffusely enlarged uterus, increased junctional zone width between endometrium and myometrium, myometrial cysts Treatment GnRH agonists, oral contraceptives, or hysterectomy  Leiomyoma of the Uterus (Fibroids) Most common benign neoplasm of the female genital tract; Irregular enlargement of the uterus- may be asymptomatic Subgroups: intramural, subserosal, submucosal Symptoms may include heavy or irregular menstrual bleeding, pelvic pain, dysmenorrhea, pressure May enlarge during pregnancy or OCP use Smokers less susceptible due to lower estrogen Diagnosis is confirmed by ultrasonography Treatment: NSAIDs, tranexamic acid and/or oral hormones or intrauterine devices (IUD) for severe bleeding; radiofrequency ablation, uterine artery embolization, surgical myomectomy or hysterectomy for more severe cases Fibroid locations in the uterus. Questions?