Chpt 48 Vulvar and Vaginal CA – Flashcards
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What percentage of gynecologic malignancies originate on the vulva?
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3% to 5%.
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What is the average delay in diagnosis of vulvar cancer?
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12 months (6 months patient delay, 6 month physician delay).
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What are the borders of the superficial inguinal nodes?
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Inguinal ligament superior. Border of sartorius muscle laterally. Border of adductor longus medially.
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What is the name of the fascia that is located above the deep inguinal nodes?
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The cribriform fascia that makes up the covering of the femoral sheath.
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Observational association of what risk factors have been linked with vulvar cancer?
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(1) Advancing postmenopausal age. (2) Hypertension. (3) Diabetes. (4) Obesity. (5) Smoking
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What link does the human papilloma virus (HPV) have, if any, with vulvar cancer?
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HPV DNA can be identified in about 70% to 80% of intraepithelial lesions, but is seen in only 10% to 50% of invasive lesions. HPV type 16 seems to be most common, but types 6 and 33 have also been identified.
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True or False: Lichen sclerosis has been proven to be a precursor of and leads to invasive vulvar cancer.
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False.
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What is the definition of a stage IA vulvar cancer using the latest International Federation of Gynecologists and Obstetricians (FIGO) staging (2009)?
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Tumor confined to vulva or perineum; 2 cm or less in greatest dimension; no nodal metastasis with stromal invasion ≤1.0 mm.
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Name the three mechanisms of the spread of vulvar cancer.
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(1) Local growth and extension. (2) Embolization to regional lymph nodes in the groin. (3) Hematogenous dissemination to distant sites.
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Name the three characteristics that describe the growth pattern of vulvar cancer as these growth patterns influence the rate of lymph node metastasis and survival.
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(1) Confluent. (2) Compact (3) Fingerlike (or spray).
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What is the name and location of the last node of the deep femoral nodal group?
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The Cloquet node, or node of Rosenmüller, is located just beneath the Poupart ligament.
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Name, in order, the five most common histologic subtypes of vulvar neoplasms.
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(1) Epidermoid (squamous cell). (2) Melanoma. (3) Sarcoma. (4) Basal cell. (5) Bartholin gland.
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True or False: The deep pelvic nodes are essentially never involved with metastatic disease when the more superficial inguinal nodes are uninvolved, even with a clitoral lesion.
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True.
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Which vulvar lesion has the classic "cake-icing effect" appearance secondary to hyperemic areas associated with a superficial white coating?
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Paget disease of the vulva.
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What underlying malignancy must be ruled out when Paget disease of the vulva is diagnosed?
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Adenocarcinoma of the vulva.
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What is the treatment of Paget disease without an underlying adenocarcinoma?
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This is a true intraepithelial neoplasia and can be treated as such with wide local excision.
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What is the most frequent histologic subtype seen in Bartholin gland cancer?
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Adenocarcinoma and squamous cell carcinoma occur with equal frequency and comprise 80% of all primary malignant tumors at this site.
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How does the lymph node spread pattern of Bartholin gland cancer differ from typical squamous cell vulvar carcinoma?
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The lesion can have a tendency to spread into the ischiorectal fossa and can spread posteriorly directly to the deep pelvic nodes in addition to the typical inguinal lymph node spread pattern.
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What is the reported lymph node metastasis rate of stage I squamous carcinoma of the vulva with a thickness of 5 mm or more?
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At least 15%.
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Have squamous cell vulvar tumors with a depth of ≤1 mm shown any significant risk of lymph node metastasis?
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No. Tumors of this depth or less carry little or no risk of lymph node metastasis.
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What is the name of the vulvar tumor that is a neuroendocrine tumor of the skin, morphologically resembles small-cell carcinomas of neuroendocrine type in other body sites and is associated with frequent lymph node metastasis and a poor prognosis?
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Merkel cell tumor.
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What HPV subtype has been associated with verrucous carcinomas of the vulva?
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HPV type 6
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Where is the most common site on the vulva to find an adenoid cystic carcinoma?
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The Bartholin gland. It comprises 15% of all Bartholin gland carcinomas.
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Name the most frequent primary vulvar sarcoma identified and its usual location.
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Leiomyosarcoma. It commonly arises in the labium majus or the Bartholin gland area.
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What is the single most important prognostic factor in women with vulvar cancer?
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Lymph node metastasis. The presence of inguinal node metastasis routinely results in a 50% reduction in long-term survival.
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What is the incidence of positive lymph node involvement in T1 and T2 lesions?
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The incidence of positive inguinal and pelvic lymph nodes varies considerably; however, in the largest study to date it was found that 20% of T1 lesions and 45% of T2 lesions had positive lymph node involvement (higher if adjuvant radiation therapy is administered).
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What are the two most common complications associated with radical vulvectomy?
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Wound breakdown occurs in approximately 50% of patients in most series and lymphedema following surgery has been reported in up to 70% of patients.
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What is the 5-year survival rate by stage in vulvar cancer?
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Stage I—91%. Stage II—81%. Stage III—48%. Stage IV—15%.
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What is the effect of lymph node involvement on survival?
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If lymph node involvement is negative overall, survival is 90% regardless of stage; however, survival rate drops precipitously even if only one lymph node is positive for metastasis (57%).
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What is the survival rate with positive deep pelvic nodes in vulvar cancer regardless of stage?
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20%.
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How does the International Society for the Study of Vulvar Disease (ISSVD) define microinvasive carcinoma of the vulva?
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A squamous carcinoma having diameter of 2 cm or less, with depth of invasion ≤1 mm. The presence of vascular space involvement would exclude the lesion from this category.
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What parameters should be addressed in the pathology report in early superficial vulvar cancer?
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(1) Tumor thickness. (2) Vascular invasion. (3) Depth of invasion. (4) Confluence of invasive neoplastic tongues. (5) Grade of cell differentiation. (6) Host response.
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What has the term "giant condyloma of Buschke-Lowenstein" been used to describe?
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Verrucous carcinoma of the cervix.
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What have postoperative spindle cell nodules on the vulva been confused with?
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Leiomyosarcomas.
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What are the most common metastatic tumors to the vulva?
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Squamous cell cancer of the cervix and adenocarcinomas of the endometrium. Other primary sites include the vagina, ovary, urethra, kidney, breast, melanoma, choriocarcinoma, rectum, and lung.
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Prior to the treatment of Paget disease of the vulva, what screening should be performed?
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Because of the high incidence of associated carcinomas of the breast and genitalia, a thorough search for such tumors should be performed prior to any consideration of therapy. This should involve breast examination, mammography, cytologic and colposcopic evaluation of cervix, vagina and vulva, and sigmoidoscopy/colonoscopy.
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What is the stage of a 3 cm vulvar cancer confined to the vulva with unilateral regional lymph node metastasis of 3 mm?
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Stage III—AT 2 N1A M0.
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What is the stage of vulvar cancer that is 1 cm in size with adjacent spread to the lower urethra, nodes negative?
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Stage II—T 2 N0 M0.
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What alternatives to radical surgery are available for women with a locally advanced vulvar carcinoma?
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Preoperative chemoradiation has been utilized to reduce the size of many tumors that may be initially invading structures such as the bladder and anus. This treatment plan may allow for limited surgical resection.
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Which vulvar cancer has a predilection for hematogenous spread?
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Vulvar sarcomas. In one series 50% had pulmonary metastases.
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What is the name of the vaginal tumor that presents as a mass of grapelike nodules most commonly in the first 2 years of life?
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Embryonal rhabdomyosarcoma (sarcoma botryoides).
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What is the current acceptable conservative surgical treatment of a vulvar cancer confined to one labia with no central involvement?
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Wide local excision or vulvectomy with ipsilateral groin node dissection that should include all nodes. No attempt should be made to distinguish between superficial and deep inguinal lymph nodes. Groin node dissection cannot be totally dispensed of unless invasion is less than 1 mm.
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What is the overall rate of recurrence in treated vulvar cancer, and where does it recur?
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Approximately 25% of patients will recur, and 80% of these recurrences are in the first 2 years. Most recurrences are on the vulva, with a few in the groin.
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What is the treatment of node-positive vulvar cancer?
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Two factors appear to be important in the management of regional disease. Radiation therapy can have a significant impact on controlling or eradicating small volume nodal disease, and surgical resection of bulky nodal disease also improves regional control and probably enhances the curative potential of radiation. Patients with positive nodes, particularly more than one positive node, are likely to benefit from postoperative irradiation to the groin and pelvis.
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Is surgical debulking of positive pelvic nodes in vulvar cancer superior to radiation for treatment?
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No. Radiation therapy has been found to be superior in the management of patients with positive pelvic nodes.
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True or False: Primary cancer of the vagina is one of the rarest of the malignant processes in the human body.
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True.
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What is the most common type of vaginal cancer?
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Squamous cell carcinoma.
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If a malignant neoplasm involves both the cervix and the vagina and is histologically compatible with origin in either organ, is it classified vaginal or cervical?
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Cervical cancer.
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What is the spread pattern of vaginal cancer?
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If it occurs in the upper half of vagina, extension is similar to cervical cancer; if it occurs in the lower part of the vagina, extension is similar to carcinoma of the vulva.
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What is the cause of most vaginal tumors/cancer seen?
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Secondary carcinoma from extension of a cervical cancer; primary probably account for the greatest number of so-called vaginal cancers.
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What is the histologic distribution of primary vaginal cancers?
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Squamous - 85% Adenocarcinoma - 6% Melanoma - 3% Sarcoma - 3% Misc - 3%
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Where is the most frequent location of a primary vaginal carcinoma lesion?
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The predominance of lesions is in the upper third and posterior wall of the vagina.
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Have causes of chronic irritation of the vaginal wall, that is, use of a vaginal pessary, prolapse of the vaginal wall, syphilis, leukoplakia, been proven to be a cause of vaginal cancer?
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No. The cause of squamous cell carcinoma of the vagina is unknown.
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What is the most frequent presenting symptom of vaginal cancer?
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Vaginal discharge, often bloody, is the most frequent symptom in most series. The signs and symptoms of invasive vaginal cancer are similar to that of cervical cancer.
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Are the course and destination of lymphatic channels from different areas in the vagina predictable and consistent?
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No. All lymph nodes in the pelvis may at one time or another serve as a primary site or regional drainage for vaginal lymph and its contents.
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How is vaginal cancer staged?
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Clinically similar to cervical cancer. All patients should have a physical examination, chest film, IVP, cystoscopy, and proctoscopy. Optional studies include lymph angiogram and barium enema.
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When should a barium enema be definitely included in patients with vaginal cancer?
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In patients with a history of recurrent diverticulitis since it may be important in planning radiation therapy.
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What is the primary mode of therapy for vaginal cancer?
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Radiation therapy.
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What is the typical radiation treatment plan for larger stage I vaginal cancers and above?
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4000 to 5000 cGy whole pelvis external radiation with an interstitial implant delivery approximately 3000cGy locally.
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What is the stage of a vaginal cancer that has extended onto the pelvic sidewall?
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Stage III.
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What is typical treatment of a bulky stage I or II vaginal cancer?
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External radiation 4000 to 5000 cGy followed by (in some centers) vaginal ovoids and an intrauterine tandem (Fletcher-Suite). These are used to deliver a surface dose of up to 6000 cGy in 72 hours or 8000 cGy in two applications of 48 hours each separated by 2 weeks, depending on initial thickness and regression of the lesion. Many centers now administer high-dose radiation (HDR) brachytherapy on an outpatient schedule.
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In addition to standard radiation therapy, which additional treatments should be considered for a vaginal tumor occurring in the distal third of the vagina?
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Since these tumors frequently metastasize to the inguinal nodes, these nodes are best treated by radical inguinal dissection before radiation therapy.
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In clear cell adenocarcinoma of the vagina, what is the precursor lesion found?
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Adenosis.
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What has clear cell carcinoma of the vagina and cervix been thought to be associated with?
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Diethylstilbestrol (DES) exposure in utero. Sixty-five percent of clear cell carcinomas of the vagina and cervix have evidence of in utero exposure to DES; however, data does not substantiate that DES intrauterine exposure is a carcinogenic event. It has been shown to be teratogenic with increased adenosis and other uterine anomalies.
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What is the treatment of clear cell adenocarcinoma confined to the upper vagina and/or cervix?
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Radical hysterectomy with upper vaginectomy and pelvic lymphadenectomy with retention of the ovaries.
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What is the overall survival rate of clear cell adenocarcinoma of the vagina/cervix?
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80%. This is better than 65% crude survival rate for squamous cell cancer of the cervix and much higher than 35% to 40% survival rate reported for squamous cell cancer of the vagina.
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Can primary adenocarcinoma of the vagina occur without intrauterine exposure to DES?
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Yes. In both pre- and postmenopausal women.
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What is the treatment of malignant melanoma of the vagina?
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Surgical excision (radical excision with nodal dissection). Radiation and chemotherapy have not been found to be effective in the upper two-third of the vagina. An exenterative procedure must be used.
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What is the overall survival rate of patients with vaginal melanomas?
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15%.
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What is the peak age at presentation of a DES exposure-related clear cell adenocarcinoma of the vagina or cervix?
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19 years old.
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What is the histologic finding associated with clear cell adenocarcinomas?
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Hobnails.
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Where are clear cell adenocarcinomas of the genital tract in the female most commonly located?
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These tumors appear to arise equally in the ectocervix and upper anterior wall of the vagina.
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What is the treatment of sarcoma botryoides in a young child?
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Surgery and adjuvant chemotherapy consisting of a combination of vincristine, actinomycin, and cyclophosphamide that can be used up front permitting more conservative surgery.
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What association has been described between the risk of developing vaginal cancer and the time of first exposure in utero to DES?
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The risk was greatest for those exposed in the first 16 weeks in utero and declined for those whose exposure began in the 17th week or later.
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What is the incidence of clear cell adenocarcinoma in women prenatally exposed to DES?
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0.14 to 1.4 per 1000.
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What is the frequency of recurrence in vaginal cancer by stage?
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Stage I—10% to 20% pelvic recurrence. Stage II—35% pelvic recurrence/22% distant metastasis. Stage III—35% pelvic recurrence/23% distant metastasis. Stage IV—58% pelvic recurrence/30% distant metastasis.
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What is the classical gross appearance of adenosis of the vagina?
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Red, velvety grapelike clusters in the vagina.
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Name the different types of vaginal cancers
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Epithelial - squamous cell verrucous - small cell Malignant melanoma - Malignant lymphoma Smooth muscle tumors - Rhabdomyosarcoma Clear cell adenocarcinoma
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Has chemotherapy been proven to be a useful adjuvant therapy in vaginal cancer?
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No. It has been used only as a salvage agent with poor results.
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What is the survival rate with locally recurrent vulvar cancer?
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Recurrence-free survival can be obtained in up to 75% of cases when the recurrence is local and limited to the vulva and can be resected with a gross clinical margin.
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Does recurrence of vulvar cancer in the groin have a good prognosis?
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No. Unanticipated recurrence in the groin is almost universally fatal.
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What are the major prognostic factors in vulvar cancer?
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Tumor size, depth of tumor invasion, nodal spread, and distant metastasis.
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When compared in a randomized prospective study, was radiation to the groin and deep pelvic nodes found to be superior compared with surgical debulking of the deep pelvic nodes in patients with clinically positive inguinal nodes in vulvar cancer?
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Yes. The 2-year survival rates were 59% compared with 31%.
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With a malignant melanoma, when can lymphadenectomy be avoided and is not necessary to complete?
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Superficial melanomas (Clark level I-II) as risk of metastatic disease are minimal. A poor prognosis is associated with Clark level IV-V, thickness >2 mm, or mitotic count >10/mm2.
