nelson's club repro lecture

Free Powerpoint Templates

Gynecologic Problems of Childhood

Nelson’s Club

April 2011


Neoplasms

• Most common gynecologic neoplasm found in children

of ovarian originusually presents as an abdominal mass• Ovarian neoplasmsconstitute 1% of all childhood malignancies8% of all malignant and abdominal tumors

in children


Neoplasms

• Ovarian neoplasms10-30% of the ovarian neoplasms

operated on during childhood or adolescence are malignant

• Other common neoplasms:Paraovarian tumorsUterine neoplasms


Neoplasms

• Vagina or vulva may also be the site of a benign or malignant lesion in children

• Cervical dysplasia may occur in adolescents

• Chemotherapy, especially with alkylating agents is associated with germ cell damage in the postpubertal ovary


Neoplasms

• Prepubertal ovary, on the other hand, is markedly resistant to chemotherapeutic damage of germ cells

• Survivors of childhood cancer who previously underwent abdominal or gonadal irradiation have an increased rate of spontaneous abortions


Ovaries

• Neonatal and Pediatric Ovarian cystso most often clinical manifestations of

ovarian tumors are abdominal pain, mass, or both

• Teratoma – the most common neoplasm in adolescents; are usually benign

-Calcification on abdominal roentgenogram is often a hallmark of a benign teratoma


Ovaries

• Ovarian adenomas are the second most common benign tumor

• Ovarian cancers are extremely uncommon in children, but are responsible for 1:500 ovarian malignancies; affect only 4% of women


Ovaries

Dysgerminoma

- The most common germ cell tumor

- Other germ cell tumors are malignant teratomas, endodermal sinus tumors, embryonal carcinomas, mixed cell neoplasms, and gonadoblastomas


Ovaries

Immature teratomas and endodermal sinus tumors

- more aggressive malignancies than dysgerminomas

- occur in a significantly higher proportion of younger girls (<10 yr of age)


Ovaries10-yr survival rates:

73% for epithelial carcinomas

44% for sex cord stromal tumors

73% for dysgerminomas

33% for malignant teratomas

39% for endodermal sinus tumors

25% for embryonal carcinomas

30% for other germ cell neoplasms

100% for gonadoblastomas


OvariesDysgerminomas usually are associated

with XY gonadal dysgenesisGerm cell tumors - associated with

positive alpha-fetoprotein, human chorionic gonadotropin (HCG), and chorioembryonic antigen


OvariesTreatment:

surgical excision followed by postoperative chemotherapy; radiotherapy


Ovaries

Embryonal Carcinoma

-accounts for 6% of pediatric ovarian neoplasms and 8% of all germ cell tumors

-presents with progressive increase in abdominal pain and distention

-highly malignant with an average age of detection between 13-15 yr

-60% of patients have endocrinologic signs and symptoms


Ovaries

Embryonal Carcinoma

-60% of patients have endocrinologic signs and symptoms (precocious pseudopuberty, abnormal vaginal bleeding, and hirsutism)

-produce HXG and alpha-fetoprotein

-Treatment involves unilateral salpingo-oophorectomy for stage I disease


Ovaries

Endodermal Sinus Tumor

-are the most lethal of all ovarian cancers

-median age of presentation is 19 yr

-alpha-fetoprotein as a useful tumor marker

-tumors are radiosensitive

-may be treated surgically if they are stage I, unilateral salpingo-oophorectomy is performed


Ovaries

Choriocarcinoma

-Primary choriocarcinoma of the ovary is extremely rare and often fatal

-0.6% of germ cell tumors are choriocarcinomas

Mixed Germ Cell Tumor

-4% of all ovarian neoplasms in children


OvariesSex Cord Stromal Tumor- 5% of ovarian neoplasms- granulosa cell tumor is the most common- Isosexual precocity and occasionally

virilization may be observed in the juvenile variety

- characteristic histologic features: modular architecture, follicle formation, microcysts, cell necrosis, and increased mitotic activity


OvariesSertoli-Leydig Cell Tumor

- also a sex cord stromal tumor; accounts for 2% of pediatric ovarian tumors

- rare in prepubertal girls

- presents with evidence of virilization

- Treatment:unilateral salpingo-oophorectomy for stage I disease


OvariesOvarian Follicular Cysts

- occur from birth to puberty and usually disappear spontaneously

- UTZ:cyst usually presents as a nonechogenic area, frequently larger than 20 mm at its greatest diameter; diffuse swelling of the ovarian parenchyma and follicular enlargement of the cortical zone are also noted


OvariesPolycystic Ovarian Syndrome (PCOS)

- presents with menstrual dysfunction and signs and symptoms of androgen excess often at the time of puberty

- menstrual irregularity can range from amenorrhea to oligomenorrhea


OvariesFunctional and Hemorrhagic Ovarian Cysts- cysts are an integral part of follicular

development during the menstrual cycle- Following ovulation, it is termed a corpus

hemorrhagicum- are often symptomatic and best evaluated by

ultrasonography- Monophasic oral contraceptives can facilitate

suppression of functional cysts


OvariesOvarian Torsion- a complication that should always be

considered in the differential diagnosis of ovarian tumors or abdominal pain in a female patient

- often presents with intermittent sharp abdominal pain, radiates down the ipsilateral extremity


OvariesOvarian Torsion- when unilateral torsion is diagnosed,

oophoropexy (plication) of the contralateral adnexa may be indicated

- Incidence in one series was 25% (16 of 63 benign ovarian masses)

- often be evaluated and managed with the use of Doppler flow studies and laparoscopy


OvariesOvarian Torsion

-underlying cause: associated ovarian cyst or neoplasm

-managed conservatively


OvariesAutoamputation of the Ovary

- presents as a small, calcified, free-floating mass associated with absent adnexa

- may be asymptomatic

- hypothesized that antenatal or subclinical ovarian torsion leads to necrosis, calcification, and separation of the adnexa from its blood supply


OvariesJuvenile Granulosa Cell Tumors of the Ovary

(JGCT)- 1-2% of all ovarian tumors- median age of presentation is 7.6 yr, with a range of

6 mo-17.5 yr- Majority presents with abdominal distention and

sexual precocity- better prognosis with multidrug chemotherapy- neurotoxicity, especially ototoxicity, and bone

marrow depression are serious complications


Cervix• prevalence of dysplasia and carcinoma in situ is

18.8/1,000 for those 15-19 yr of age• biopsy-proven cases of all grades of cervical

intraepithelial neoplasia (CIN) in the teenage population have a prevalence of 13.3/1,000

• Bethesda Classification System with the Papanicolaou (Pap) smear is widely used for diagnosis

• overall frequency of abnormal Pap smear results in the adolescent population is 3.8%


Cervix• 1% of all abnormal Pap smears are associated

with CIN• Papillomavirus infectionand altered vaginal flora

are consistent findings in patients with CIN• Colposcopic examination is essential when CIN

is diagnosed in an adolescent• Other pathologic abnormalities:cervical polyps

and mixed mesodermal tumors


UTERUS (BENIGN AND MALIGNANT TUMORS OF THE UTERINE CORPUS)

Adenocarcinoma of the corpus - are in children and adolescents- frequent presenting sign: vaginal bleeding not

associated with sexual precocity- Treatment: hysterectomy, with removal of the

ovaries, followed by adjunctive radiotherapy or chemotherapy or both, depending on the operative findings


UTERUS (BENIGN AND MALIGNANT TUMORS OF THE UTERINE CORPUS)

Mixed mesodermal tumors and leiomyomas of the uterus

-included in the differential diagnosis of a pelvic mass in an adolescent

Leiomyosarcoma

-extremely rare, has also been noted in an adolescent

-abnormal vaginal bleeding usually is present


Vagina

Gartner duct (mesonephric) cyst

- a common vaginal wall abnormality

- usually is an incidental finding; requires no specific therapy

- excision may be necessary if there is associated dyspareunia


Vagina

Paramesonephric (müllerian) duct cysts- often become symptomatic at menarche when the

cavity fills with menstrual blood- Women exposed to diethylstilbestrol (DES) in

utero have a high incidence of adenosis of the vagina and cervix

- potential reproductive abnormalities:infertility, habitual abortion, and tubal and uterine cavity abnormalities


Vagina

Clear cell adenocarcinoma of the vagina and cervix

-rare sequela of DES exposure in uteroSarcoma botryoides

- vaginal carcinoma that occurs primarily in pediatric patients

- best treated by surgical excision


Vulvovaginal and Müllerian Anomalies

Imperforate Hymen

- presents with primary amenorrhea; secondary sex characteristics are normal

- requires surgical interventionCongenital Absence of the Vagina (Mayer-

Rokitansky-Küster-Hauser Syndrome)

-incidence: 1/4,000 to 1/5,000 births

-etiology is unknown



Congenital Absence of the Vagina (Mayer-Rokitansky-Küster-Hauser Syndrome)

-characterized by primary amenorrhea, normal vulva, duplication anomaly of the uterus, attenuated fallopian tubes, normal ovaries, normal female karyotype and phenotype

-associated anomalies: renal and skeletal



Congenital Absence of the Vagina (Mayer-Rokitansky-Küster-Hauser Syndrome)

- treatment: delayed until the patient is ready to be sexually active; frank dilators, combined laparoscopic vulvar procedure, McIndoe procedure involving a split-thickness skin graft placed in the vagina


Incomplete Vertical Fusion of the Vagina

-Transverse and longitudinal vaginal septa represent failure of complete canalization of the vagina

-presents with amenorrhea and cyclical pain, which is a result of cryptomenorrhea

-usually asymptomatic until puberty, hydrometrocolpos may occur in children

-most common site of the septum is between the middle and upper thirds of the vagina

-patients have a functional uterus, although their fertility is often compromised


Incomplete Vertical Fusion of the Vagina

-prognosis is worse for higher obstructions

-increased incidence of endometriosis secondary to retrograde menstruation

-treatment:surgical resection of the obstruction from below

-anastomosis of the upper and lower segments should be attempted to prevent stenosis


DISORDERS OF LATERAL FUSION

• Include anatomic variations of nonobstructive longitudinal septum

• obstructed hemivagina- associated with a didelphic uterus and often with a pelvic mass


UTERINE ABNORMALITIES

• Incidence ranges from 1/100 to 1/1,000• may present with primary amenorrhea or with

irregular or even regular menses• pregnancy wastage and infertility may cause the

first suspicion of a uterine anomaly• diagnosis should include a pelvic ultrasound

examination, renal tract assessment, and skeletal inspection for anomalies


CONGENITAL ATRESIA OF THE UTERINE CERVIX

• extremely rare anomaly often presents at puberty with cryptomenorrhea, amenorrhea, and pelvic pain

• associated with significant renal anomalies in 5–10% of patients

• complete absence of a cervix but a palpable uterus is found


COMPLETE VULVAR DUPLICATION

• rare congenital anomaly presents in infancy

• consists of 2 vulvas, 2 vaginas, and 2 bladders, a didelphic uterus, a single rectum and anus, and 2 renal systems


LABIAL HYPERTROPHY

• surgical revision may be indicated if there are symptoms of discomfort or bulging is noted when wearing tight-fitting garments


CLITORAL ABNORMALITIES

• Agenesis of the clitoris is rare

• Clitoral duplication- associated with pelvic organ abnormalities, including agenesis of other genital tract structures and bladder exstrophy


HYMENAL ABNORMALITIES

• often associated with mucocolpos

• Other abnormalities include cribriform or stenotic hymen


CLOACAL ANOMALIES

• represent a common urogenital sinus into which both urethral and anal orifices exit

• the single opening (cloaca) requires surgical correction


Special Gynecologic Needs

• Mentally Handicapped Children

-face several gynecologic issues including varying levels of understanding of reproduction, sexuality, and contraception

-perineal hygiene is also of concern

-cyclical behavioral changes (crying, self-abusive behavior, and tantrums) prior to menstruation are common



• Mentally Handicapped Children

-changes are presumed to result from pain or cramps as 65% of the patients responded to nonsteroidal anti-inflammatory drugs (NSAIDs)

-treatment with NSAIDs unsuccessful: birth control pills and depo-medroxyprogesterone acetate improved behavior in 40–66% of patients

-SSRIs are also effective



• Mentally Handicapped children

-sexual abuse is a major concern

-as many as 25% of mentally impaired women have been sexually assaulted


Gynecologic Imaging

• Ultrasonography is a key screening tool, enabling appropriate diagnosis in patients presenting with ambiguous genitalia, ovarian or uterine masses, primary amenorrhea, and abdominal or pelvic pain

• Ovarian cysts and hydrocolpos or hydrometrocolpos are the most common abnormalities noted in neonates


Gynecologic Imaging

• Hydrocolpos- dilatation of the vagina, which usually is associated with accumulation of serous fluid or urine (if there is a urogenital sinus)

• Hydrometrocolpos- dilatation of both the uterus and the vagina; may also be associated with vaginal or cervical atresia, stenosis, or an imperforate hymen


Gynecologic ImagingTABLE 556-1 -- Normal Ovarian and Uterine Dimensions

OVARY• Birth• 15 mm long, 3 mm wide, 2.5 mm thick Ovarian volume 0.7 cm3[*]• Postpuberty• 22.5–50.0 mm in length 1.5–3.0 cm in width; 0.6–1.5 cm in

thickness Ovarian volume 1.8–5.7 cm3[*]• UTERUS• Neonate• Length 2.3–4.6 cm Anteroposterior diameter 0.8–2.2 cm• Infant to 7 yr of age• Length 2.5–3.3 cm Anteroposterior diameter 0.4–1.0 cm• Postpuberty

• Length 6 cm

nelson's club repro lecture

Documents

neoplasms ovarian neoplasms

common neoplasms

ovarian malignancies

abdominal tumors

neoplasms vagina

mixed cell neoplasms

germ cell tumors

neoplasms prepubertal