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Vulvar Neoplasms
Correct! The most important step is to first biopsy the lesion. It would be inappropriate to
treat the lichen sclerosus first, as the lesion is suspicious for malignancy. Diagnostic studies
such as cultures and cytology of such a lesion are not appropriate given the exophytic,
nodular lesion seen on examination. A biopsy should be performed to make a definitive
diagnosis and rule out malignancy. It would also be inappropriate to treat the patient with
a vulvectomy and lymph node dissection before obtaining a clear diagnosis.
Correct! Given the findings of obvious, moderately differentiated carcinoma, definitive
treatment can be recommended with radical vulvectomy and groin node dissection. Only
microinvasive squamous cell carcinoma of the vulva can be treated by wide local excision,
but it is a diagnosis that is only made after pathology evaluation of a small (<2 cm), welldifferentiated lesion, with invasion <1.0 mm. Excisional biopsy is not indicated given the
larger lesion and confirmed finding of cancer. It would be inappropriate to laser a
malignant lesion. Squamous cell carcinoma is the most common vulvar malignancy and
may arise in the setting of chronic irritation from lichen sclerosus. Steroids would treat the
lichen sclerosus, but would only result in needless delay in treatment of the malignancy.
Cryotherapy is not an acceptable treatment for squamous cancer of the vulva.
Correct! Squamous cell carcinoma accounts for approximately 90% of vulvar cancers.
Patients commonly present with a lump and they commonly have a long-standing history
of pruritus. The chronic itch-scratch cycle of untreated lichen sclerosus, or any other
chronic pruritic vulvar disease, is thought to stimulate the development of squamous
carcinoma. The mean age of squamous cell carcinoma is 65 years and smoking is known to
increase the risk of development of vulvar cancer, especially in the setting of HPV infection.
With lichen sclerosus, the skin appears thin, inelastic and white, with a crinkled tissue
paper appearance. Pagets disease of the vulva is associated with white plaque-like lesions
and poorly demarcated erythema, not a discrete mass. Verrucous carcinoma has
cauliflower-like lesions. Melanoma typically presents as a pigmented lesion.
Correct! This lesion may be melanoma and a biopsy must be done to exclude this diagnosis.
The concerning features are the size and irregularity of the lesion. Melanoma represents
5% of vulvar cancer, which is not insignificant given the lack of sun exposure and the
relatively small surface area. There is no variability in the coloration, ulceration or
thickening of the lesion to suggest malignancy at this time. Squamous cell carcinoma is
typically not pigmented. High-grade vulvar intraepithelial neoplasia may be flat and
pigmented. Pagets disease is usually erythematous with a lacy white mottling of the
surface. Condyloma lesions have a characteristic verrucous appearance. With lichen
sclerosus, the skin appears thin, inelastic and white, with a crinkled tissue paper
appearance.
Correct! This presentation is classic for human papilloma virus (HPV) related VIN 3.
Melanoma would be unlikely to be multifocal and warts have a characteristic verrucous
appearance, although pigmentation can occur. Molluscum, a poxvirus, is characterized by
multiple shiny non-pigmented papules with a central umbilication. Pagets disease,
although multicentric, does not have brown pigmentation. Hidradenitis is a chronic,
unrelenting skin infection causing deep, painful scars and foul discharge.
Correct! The finding of a mass in the Bartholins gland is highly suspicious for malignancy
and requires excision/biopsy, especially in a post-menopausal women. The histology is
typically adenocarcinoma. This is unlikely to be a fibroma or lipoma given the recent onset
and fixed nature of the mass. A benign Bartholins gland cyst is also unlikely given the
patients age and rather abrupt onset, and any finding of a new Bartholins gland cyst in a
post-menopausal woman should be further investigated. This is not an abscess given the
absence of signs and symptoms of cellulitis or infection.
Correct! VIN III should be treated with local superficial excision. Even with complete
removal of all disease, the likelihood is very high for recurrence and the patient will need
close surveillance. It is inappropriate to do radical surgery in this setting as cancer has not
been diagnosed. Treatment with TCA and Aldara are reserved for condyloma, although
some studies have shown utility in the use of Aldara in treating low grade VIN. Cryotherapy
is primarily used to treat cervical dysplasia.
Correct! These lesions most likely represent an HPV-related condition such as condyloma
or vulvar dysplasia. Women who are on immunosuppressive therapy are at higher risk of
such conditions and require close surveillance. Although, her history of prior treatment for
warts suggests these could be condyloma again, their flat, subtle appearance raises a
concern that they may be dysplastic lesions, and further investigation with colposcopy and
biopsy are indicated. Treatment for presumed yeast infection is reasonable, given her
susceptibility, but would not be the sole treatment in this setting of new clinically evident
lesions especially in light of a negative wet prep. Wide excision is not indicated at this time
without a diagnosis.
Correct! Given the multifocality of the vulvar dysplasia (VIN 2), laser treatment is the best
choice. In order to adequately treat these lesions, a complete (skinning) vulvectomy would
be the other choice, but would be disfiguring and require removal of the clitoris which
would have detrimental effects on her sexual function. Treatment with TCA is
recommended for treatment of warts and not VIN. Smoking cessation is strongly
recommended regardless, but would not be the sole means of addressing these lesions.
Observation is not ideal, given her mild symptoms, moderate grade, and diffuse nature of
the lesions.
Correct! This is a typical description of Pagets disease of the vulva. Pagets is an in situ
carcinoma of the vulva. The association with breast cancer is significant, but not as high as
Pagets disease of the nipple. It would be unlikely for psoriasis to present this late in life.
Contact dermatitis is unlikely to last for years and this woman has had therapy for yeast.
Lichen sclerosus is possible and more common, but does not have the hyperkeratotic
overlay and would have more likely responded to steroid use.
Correct! The majority of risk factors for cervical cancer are related to HPV exposure
and include early-onset sexual activity, multiple sexual partners, a sexual partner
with multiple partners, history of HPV or other sexually transmitted diseases,
immunosuppression, smoking, low socioeconomic status and a lack of regular Pap
smears. In this patient with multiple risk factors, the presence of an HPV-related
condition (vaginal condyloma) already indicates infection with HPV. Although the
HPV type associated with condyloma is typically a low risk strain (e.g. types 6 and
11), she is also at risk of having been exposed to high-risk types that are typically
associated with high-grade dysplasia and cervical cancer (e.g. types 16 and 18).
Correct! The ASCCP (American Society of Colposcopy and Cervical Pathology)
recommends that management options for ASCUS include performing HPV DNA
testing or repeat cytology at 12 months following the abnormal Pap test result. If the
HPV testing is negative (as was reported in this case), then routine screening can be
resumed at three years. If HPV is positive, or if repeat cytology at 12 months reveals
ASCUS or higher, then colposcopy should be performed. For women ages 21 24, if
HPV is positive, then repeat cytology at 12 months is recommended with colposcopy
performed only if the repeat cytology reveals ASC-H (atypical squamous cell cannot
rule out high grade squamous intraepithelial lesion), AGC (atypical glandular cells)
or HSIL (high-grade squamous intraepithelial lesion). The presence of an underlying
infection does not affect the triage of an abnormal Pap smear but may explain the
presence of ASCUS. See ASCCP guidelines:
http://www.asccp.org/Portals/9/docs/Algorithms%207.30.13.pdf
Correct! This patient is at high-risk for cervical cancer. Her risk factors include
tobacco use and a poor screening history. The symptoms of postmenopausal and
postcoital bleeding should be taken seriously, and a cervical biopsy of the suspicious
cervical lesion performed. Her physical examination with fixation of the uterus and
thickening of the rectovaginal septum and back pain suggests involvement of the
parametria (Stage II) and possible extension to the sidewall (Stage III). A Pap smear
should not be used to exclude cervical cancer, as it is a screening test and not a
diagnostic test, and colposcopy would not be useful since a clinically visible lesion is
already present. Although a CT scan may ultimately be needed as part of the
evaluation of cervical cancer, a diagnosis must first be made by biopsy.
Ultrasonography may be helpful in the diagnostic evaluation of post-menopausal
bleeding, but not in the setting of an obvious cervical lesion.
Correct! A white plaque found on the cervix is called leukoplakia and should be biopsied
directly or under colposcopic guidance as soon as possible, regardless of Pap smear
outcome. Pap smears have a false-negative rate as high has 20-30%. If there is no evidence
of dysplasia and her Pap smear is normal, then routine screening can be resumed. A wet
mount would be indicated if there was evidence of white discharge. Although cervical
conization maybe necessary if high grade dysplasia is diagnosed, this is not the most
appropriate step in the management of this patient.
Incorrect! Correct answer is E. Punctations and mosaicism represent new blood
vessels on end and on their sides, respectively. Atypical vessels usually represent a
greater degree of angiogenesis and, thus, usually a more concerning lesion. An
ectropion is an area of columnar epithelium that has not yet undergone squamous
metaplasia. It appears as a reddish ring of tissue surrounding the external os.
Acetowhite epithelium can represent dysplasia but, in most cases, is less concerning
than the above vascular changes.
Correct! Because the entire lesion cannot be visualized, this colposcopy is
unsatisfactory. Severe dysplasia and even invasive cancer cannot be ruled out.
Endocervical curettage has a relatively low sensitivity (i.e. a high amount of false
negatives) and, therefore, you cannot rule out endocervical disease. The
endocervical canal must be histologically examined. A cervical conization should be
performed to obtain a pathologic specimen. Cryotherapy may serve to ablate part of
the canal, but will not provide a pathologic sample to assess for dysplasia or to rule
out cancer.
ncorrect! Correct answer is D. Indications for cervical conization include 1)
unsatisfactory colposcopy, including inability to visualize the entire transformation
zone, 2) positive endocervical curettage, 3) Pap smear indicating adenocarcinoma in
situ, 4) cervical biopsies that cannot rule out invasive cancer, and 5) a substantial
discrepancy between Pap smear and biopsy results. The other options are not
adequate evaluation. Since there was a significant discrepancy between the HSIL Pap
smear result and the normal reading on the biopsies and the ECC, the patient
requires a cervical conization such as LEEP or cold knife cone. Cotesting with
cytology and HPV can be repeated at 12 and 24 months with further management
based on these results. (See ASCCP guidelines:
http://www.asccp.org/Portals/9/docs/Algorithms%207.30.13.pdf)
Correct! Cervical dysplasia is graded based on extent of involvement of the epithelial
layer but does not extend below the basement membrane. Carcinoma in situ (CIS)
represents abnormal cells involving the entire epithelium to the basement
Women aged 21 29 years should have a Pap test every three years.
Women aged 30 65 years should have a Pap test and an HPV test
(co-testing) every five years (preferred). It is acceptable to have a Pap
test alone every three years.
Women who have a history of cervical cancer, are infected with HIV,
have a weakened immune system, or who were exposed to DES before
birth should not follow these routine guidelines.
ACOG recommends that women who have been vaccinated against HPV should follow
the same screening guidelines as unvaccinated women.
Uterine Leiomyomas
Correct! The major symptom associated with myomas is menorrhagia, thought to be
secondary to: 1) an increase in the uterine cavity size that leads to greater surface area for
endometrial sloughing; and/or 2) an obstructive effect on uterine vasculature that leads to
endometrial venule ectasia and proximal congestion in the myometrium/endometrium
resulting in hypermenorrhea. Other relatively frequent symptoms include pain and
pressure symptoms related to the size of the tumors filling the pelvic cavity, as well as
causing pressure against the bladder, bowel and pelvic floor.
Correct! Uterine fibroids are the most common solid pelvic tumors in women. On
postmortem examination, fibroids can be detected in as high as 80% of women. Most
uterine fibroids are asymptomatic and do not require any treatment. Pregnant patients
with fibroids usually are asymptomatic and do not have any complications related to the
fibroids. Fibroids may grow or become symptomatic in pregnancy due to hemorrhagic
changes associated with rapid growth, known as red or carneous degeneration. However,
this is uncommon for smaller fibroids. Uncommonly, fibroids can be located below the
fetus, in the lower uterine segment, or cervix, causing a soft tissue dystocia, necessitating
delivery by Cesarean section. Myomectomy (removal of the fibroid) during pregnancy is
contraindicated. Myomectomy at the time of Cesarean section should be avoided, if
possible, secondary to the risk for increased blood loss. It is not necessary to follow the
growth of fibroids during pregnancy, except for the rare cases when the fibroid is causing
symptoms (primarily pain) or appear to be located in a position likely to cause dystocia.
Correct! Leiomyomas are an infrequent cause of infertility, either by mechanical
obstruction or distortion (and interference with implantation). When a mechanical
obstruction of fallopian tubes, cervical canal or endometrial cavity is present and no other
pain and fibroid size, they are contraindicated in a patient who desires future fertility. The
failure rate is about 10-15%. A hysterectomy would obviously take care of her bleeding but
would not be performed if she desires future fertility.
Correct! Maximal response is usually achieved by three months of GnRH agonist treatment.
The reduction in size correlates with the estradiol level and with body weight. Hot flashes
are experienced by >75% of patients, usually in three to four weeks after start of treatment,
although they should not persist for longer than one to two months from end of treatment.
After cessation of treatment, menses return in four to ten weeks, and myoma and uterine
size return to pretreatment levels in three to four months. The regrowth is consistent with
the fact that reduction in size is not due to a cytotoxic effect. However, it is not true that
secondary to the GnRH agonist withdrawal they will grow at a more rapid rate.
Correct! The patients history and physical examination is typical for a perimenopausal
woman with probable uterine fibroids. Although it is possible that she could have
underlying endometrial hyperplasia, the most likely diagnosis is uterine fibroids. Uterine
leiomyosarcoma should be considered in a postmenopausal woman with bleeding, pelvic
pain coupled with uterine enlargement, and vaginal discharge, but it is exceedingly rare.
Endometrial hyperplasia is more common in perimenopausal women who do not ovulate
regularly and postmenopausal women. Endometrial carcinoma is typically a disease of
postmenopausal women, although 5-10% of cases occur in women who are menstruating
and 10-15% of cases occur in perimenopausal women. Adenomyosis may result in a
symmetrically enlarged boggy uterus, but usually presents with dysmenorrhea in
addition to menorrhagia.
Correct! Myomectomy is warranted in younger patients whose fertility is compromised by
the presence of fibroids that cause significant distortion of the uterine cavity. A
myomectomy may be indicated in infertility patients when the fibroids are of sufficient size
or location to be a probable cause of infertility and when no more likely explanation exists
for the failure to conceive. Hysteroscopy is a procedure that involves placing a scope
through the cervical os to assess the endometrial cavity. The patient has already been
diagnosed with uterine fibroids that are distorting her cavity and she has already had a
fluid contrast ultrasound, so it is unnecessary to perform hysteroscopy on this patient.
Treatment with GnRH agonists can be useful to shrink fibroids in anticipation of surgery, or
if menopause is expected soon. This patient desires future childbearing, therefore, its use
would not be an appropriate option. Uterine artery embolization can be recommended for
women who have completed child-bearing because of the unclear long-term effects on
fertility.
Endometrial Carcinoma
Correct! Endometrial cancer is a gynecologic malignancy that has easily identifiable risk
factors and typically presents with symptoms that lead to an early diagnosis. Risk factors
include nulliparity, obesity, late menopause, hypertension and exposure to unopposed
estrogens. Of these risk factors, obesity confers the greatest risk of developing endometrial
carcinoma, especially when the patient is more than 50 pounds over ideal body weight (10fold increase). However, in this case, the patients greatest risk for developing an
endometrial cancer is the presence of complex atypical hyperplasia (CAH) on endometrial
biopsy. If left untreated, this process has approximately a 28% chance of progressing to an
invasive cancer. More importantly, approximately 30% of women with a diagnosis of CAH
will be found to have an invasive endometrial cancer on final pathology. Most women who
develop endometrial cancer are postmenopausal, but this is less of an issue because of the
finding of CAH.
Incorrect! Correct answer is D. Less than 5% of women diagnosed with endometrial
cancer are asymptomatic. Approximately 80-90% of women with endometrial
carcinoma present with vaginal bleeding or discharge as their only presenting
symptom. Since this patient does not have any symptoms or risk factors for
endometrial cancer, she does not need to have any diagnostic testing. Risk factors for
endometrial cancer include late menopause, unopposed estrogen therapy,
nulliparity, obesity, Tamoxifen therapy and diabetes mellitus. Although sometimes
associated with Hereditary Non-polyposis Colorectal Cancer Syndrome (HNPCC, or
Lynch II), endometrial cancer is typically not a genetically-inherited malignancy, and
so genetic counseling for risk assessment would not be recommended unless a more
significant family history existed. Endometrial cancer ranks as the fourth most
common cancer detected in women in the US. In 2010, according to the American
Cancer Society, there will be an estimated 43,470 new endometrial cancer cases. It is
the most common gynecologic malignancy.
Top Five Cancers Detected in Women:
Breast 28%
Lung 14%
Colon 10%
Uterine 6%
Ovary 3%
Gynecologic Cancers:
Uterine 52%
Ovary 26%
Cervix 14%
Vulva 5%
Vagina 3%
Incorrect! Correct answer is A. Endometrial cancer is a disease that typically
presents with symptoms and clinical findings that lead to an early diagnosis. The
Ovarian Neoplasms
Correct! The events leading to the development of ovarian cancer are unknown.
Epidemiologic studies, however, have identified endocrine, environmental and
genetic factors as important in the carcinogenesis of ovarian cancer. The established
risk factors include nulliparity, family history, early menarche and late menopause,
white race, increasing age and residence in North America and Northern Europe.
Smoking has not been demonstrated to be associated with an increased risk of
ovarian cancer.
Correct! A womans risk for development of ovarian cancer during her lifetime is
approximately 1%. Factors associated with development of ovarian cancer include
low parity and delayed childbearing. Long-term suppression of ovulation appears to
be protective against the development of ovarian cancer. Oral contraceptives that
cause anovulation appear to provide protection against the development of ovarian
cancer. Five years cumulative use decreases the lifetime risk by one-half.
Prophylactic oophorectomy is not a practical choice for this patient with no family
history, even once she completes childbearing. This option might be considered for a
woman with a strong family history and the BRCA mutation.
Correct! BRCA1 and BRCA2 mutations are typically seen in cases of hereditary
ovarian cancers. Overall, it has been estimated that inherited BRCA1 and BRCA2
mutations account for 5 to 10 percent of breast cancers and 10 to 15 percent of
ovarian cancers among white women in the United States. Given this family history,
it is highly likely that a mutation is present, and the affected individual (proband)
should be tested if still alive. Because breast cancers are part of the BRCA mutation,
the affected mother should be tested. Routine screening for ovarian cancer has not
been established.
Correct! Functional ovarian cysts are a result of normal ovulation. They may present
as an asymptomatic adnexal mass or become symptomatic. Ultrasound
characteristics include a unilocular simple cyst without evidence of blood, soft tissue
elements or excrescences. An endometrioma is an isolated collection of
endometriosis involving an ovary. This would not classically appear as a simple cyst
on ultrasound. Serous cystadenomas are generally larger than functional cysts and
patients may present with increasing abdominal girth. Mucinous cystadenomas tend
to be multilocular and quite large. Dermoid tumors usually have solid components
or appear echogenic on ultrasound, as they may contain teeth, cartilage, bone, fat
and hair.
Correct! The most useful radiologic tool for evaluating the entire peritoneal cavity
and the retroperitoneum is computerized tomography. Specifically in this patient, it
would be important to look for significant involvement of the omentum. A chest xray provides adequate evaluation of the chest, unless it is abnormal. If there is a
suspicion for chest involvement on the chest film, then a chest CT is necessary. With
a normal colonoscopy and no symptoms suggestive of colonic obstruction, a barium
enema would not be useful. PET scan, to date, has not been shown to play a role in
the initial evaluation of women with a suspected ovarian malignancy. However, PET
scan may play a role in evaluating women with a known diagnosis of ovarian cancer
who have a suspected recurrence. An IVP would be useful if there was suspected
ureteral obstruction, but otherwise is quite limited in assessing the entire
abdominal/pelvic cavity.
Correct! Based on this patients initial evaluation, it seems highly likely that she has
an advanced stage ovarian carcinoma. Given her lack of significant medical comorbidities, and a gynecologic examination suggestive that the tumor would be
potentially resectable, it would be appropriate to offer her a surgical intervention.
The surgical staging of an apparent ovarian cancer should include a vertical skin
incision, sampling ascites for cytology, inspection of the entire peritoneal cavity,
total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy,
cytoreduction to microscopic disease or to residual disease <1 cm in maximum
diameter, and pelvic and para-aortic lymph node dissection if omental disease is <2
cm or if bulky, lymph node disease is present. Thoracentesis to rule out malignant
pleural effusion is part of the staging criteria, but may not be justified if the effusion
is too small, and would not impact the need to perform surgery. Neoadjuvant
chemotherapy is generally considered when a patient has unresectable disease or is
a poor surgical candidate, at which point paracentesis for cytologic confirmation
would be needed.
Correct! The five-year survival of patients with epithelial ovarian cancer is directly
correlated with the tumor stage. The volume of residual disease following
cytoreductive surgery is also directly correlated with survival. Patients who have
been optimally debulked (generally <2 cm or <1 cm maximal residual tumor
diameter) have a significant improvement in there median survival. Histologic grade
of tumor is important. Women with poorly differentiated tumors or clear-cell
carcinomas typically have a worse survival than those with well to moderately
differentiated tumors. This is especially important in early-stage disease. Tumor size,
bilaterality and ascites without cytologically positive cells, are not considered to be of
prognostic importance.
Correct! In all patients with advanced ovarian cancer, post-operative chemotherapy with a
combination of a taxane and platinum adjunct is considered standard of care in the United
States. Women who undergo surgical cytoreduction, followed by chemotherapy, have a
better overall survival rate than those who undergo surgery alone. The overall response
rate in women with advanced ovarian cancer following surgery and 4-6 cycles of
combination chemotherapy with a taxane and platinum adjunct is 60-80%. The overall
five-year survival for women with stage III and IV disease is approximately 30%. Second
look laparotomy is no longer considered standard of care.
Correct! The most common tumor found in women of all ages is the dermoid. The median
age of occurrence is 30 years, and 80% occur during the reproductive years. Dermoids may
contain differentiated tissue from all three embryonic germ layers. Dermoid tumors can
contain teeth, hair, sweat and sebaceous glands, cartilage, bone, and fat.