Download case report primary papillary adenocarcinoma of vagina: a rare case

CASE REPORT
PRIMARY PAPILLARY ADENOCARCINOMA OF VAGINA: A RARE
CASE REPORT
Chandana C1, Shreedhar Venkatesh2
HOW TO CITE THIS ARTICLE:
Chandana C, Shreedhar Venkatesh. “Primary Papillary Adenocarcinoma of Vagina: A Rare Case Report”.
Journal of Evidence Based Medicine and Healthcare; Volume 1, Issue 8, October 15, 2014; Page: 842-845.
ABSTRACT: Primary vaginal adenocarcinoma is a rare malignant gynaecologic disease. A 56
year-old woman with post hysterectomy status presented with vaginal bleeding. She had a
proliferative growth in the posterior vaginal wall extending to the recto-vaginal space and lateral
pelvic wall. Vaginal growth biopsy revealed papillary adenocarcinoma and was diagnosed as FIGO
stage III primary papillary adenocarcinoma of vagina. As most of the adenocarcinoma of vagina is
metastatic, the possibility of primary papillary adenocarcinoma of vagina has to be kept in mind.
The present case was successfully treated with chemotherapy and radiotherapy, suggesting that
chemoradiation may be an option for the treatment of this type of tumor.
KEYWORDS: Papillary adenocarcinoma, chemotherapy, radiotherapy.
INTRODUCTION: Primary vaginal cancers are rare constitute 1-2% of gynaecological
malignancy. Primary adenocarcinoma of the vagina is a rare disease characterized by
aggressiveness and poor prognosis because of its rapid growth, recurrence and its frequent
distant metastases. We report a case of primary papillary adenocarcinoma of vagina and treated
successfully with external beam radiation concurrent with cisplatin followed by brachytherapy.
CASE REPORT: A 56-year-old woman with unconfirmed in utero diethylstilbestrol exposure
presented with vaginal bleeding of 3 months duration. She underwent total abdominal
hysterectomy with bilateral salphingo-ophorectomy for fibroid uterus 9yrs back. Histo pathalogy
of the uterus, cervix and both ovaries are unremarkable other than leiomyoma. On examination
she was moderately built and nourished with BMI 21, vitals were stable. Mild pallor was present
and no lymphadenopathy. Abdomen was soft, no organomegly and no mass felt. Per-speculum
examination revealed a proliferative growth of about 2cm x 3cm noted in the posterior vaginal
wall in the upper 1/3rd. On rectal examination, a hard mass of about 4cm noted involving the
recto vaginal space and parametrium extending up to pelvic wall. Vaginal biopsy revealed
papillary adenocarcinoma. MRI abdomen and pelvis shows ill- defined enhancing lesion about
5cm in the posterior vaginal wall involving recto vaginal fat planes and bilateral pelvic
lymphadenopathy. Metastatic workup included colonoscopy, upper GI endoscopy, chest X-ray and
mammography were normal. Patient was diagnosed with primary papillary adenocarcinoma of
vagina FIGO stage III. She was treated with external beam radiation concurrent with cisplatin
followed by brachytherapy.
DISCUSSION: Primary vaginal cancer is a rare tumor, representing only 1% to 2% of malignant
neoplasms of the female genital tract.1 Carcinoma of the vagina is defined as a primary carcinoma
J of Evidence Based Med & Hlthcare, pISSN- 2349-2562, eISSN- 2349-2570/ Vol. 1/ Issue 8 / Oct 15, 2014.
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CASE REPORT
arising in the vagina and not involving the external os of the cervix superiorly or the vulva
inferiorly. Incidence rates for all vaginal cancers combined were 0.18 per 100, 000 female
population for in situ cases and 0.69 for invasive cases.2 Squamous cell carcinomas (SCC) are the
most common form of vaginal cancer, occurring in 80% to 90% of cases, adenocarcinoma of the
vagina is rare, constituting 5-10% of primary tumors of the vagina.3,4 The most common
adenocarcinoma of the vagina are metastatic, which constitute the majority of vaginal cancers
(80% to 90%) originating from the colon, endometrium, ovary, or rarely from pancreas and
stomach. Hence primary adenocarcinoma of vagina is diagnosis of exclusion.
SCC and adenocarcinoma of vagina have different natural history of disease course. SCC
develops from vaginal intraepithelial lesion following exposure to HPV infection. Many of the
reported cases of primary adenocarcinoma of the vagina are clear cell adenocarcinomas in young
adolescents exposed to diethylstilbestrol (DES) in utero. The association between the clear cell
carcinomas and in utero exposure to DES was first reported in 1971.5 Vaginal adenosis is most
commonly found in young women who had in utero exposure to DES and may coexist or progress
to clear cell adenocarcinoma. However, some patients with primary adenocarcinoma of the vagina
have non-clear cell, non-DES-associated tumors. The natural history of such tumors has not been
clarified.
Glandular elements are occasionally found in the vagina which represents mesonephric or
paramesonephric remnants which may undergo malignant change to adenocarcinoma.
Adenocarcinomas may arise in foci of endometriosis.6 Adenocarcinoma of vaginal vault following
prolonged unopposed hormone replacement have been reported.7
When adjusted for age and prior cervical disease, the incidence of vaginal cancer is not
increased in women who have had hysterectomy for benign disease.8 So women who have had a
hysterectomy for benign disease and have no antecedent history of CIN, performance of Pap
testing is unnecessary. If the patient has a history of cervical dysplasia or cervical cancer, yearly
screening is recommended.
DES associated adenocarcinoma occurs in young girls means age at 19 years, non DES
associated adenocarcinoma occurs in older age group between 50-60 yrs. The most common
symptom of vaginal carcinoma is abnormal bleeding or discharge. With advanced tumors, pain or
urinary frequency occasionally occurs, especially in cases of anterior wall tumors. Constipation or
tenesmus has been seen with tumors involving the posterior vaginal wall. These tumors usually
are diagnosed by direct biopsy of the tumor mass, and abnormal cytologic findings often will lead
to diagnosis of a vaginal cancer.
Non–DES-associated adenocarcinomas generally have a worse prognosis than SCC
tumors, and DES-associated clear cell adenocarcinoma.
Therapeutic options depend on tumor stage, age and condition of the patient. Primary
therapy for locally invasive disease consists of external and intracavitary or interstitial irradiation,
except in selected early cases that may be treated surgically with local excision. Radical surgery,
which may involve partial or total pelvic exenteration, is generally reserved for pelvic recurrence
after irradiation and for patients with fistulas at diagnosis. Chemoradiation is the newer modality
of treatment for primary vaginal cancer.9 Our patient was treated with external beam radiation
concurrent with cisplatin followed by brachytherapy.
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CONCLUSION: In summary, primary papillary adenocarcinoma of the vagina is a rare disease
associated with a poor prognosis and with significantly worse outcomes than those seen in
patients with primary squamous cell carcinoma of the vagina and DES – associated
adenocarcinoma. Additional data about patients with this rare tumor should be collected and
analyzed in an attempt to elucidate its prognostic factors, characteristics, optimal treatment, and
outcome.
Fig. 1: Histopathology of vaginal growth
biopsy showing papillary adenocarcinoma
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oestrogen hormone replacement therapy. J Obstet Gynaecol. 2005; 25(2): 220-1.
8. Al-Kurdi M, Monaghan JM. Thirty-two years’ experience in management of primary tumours
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AUTHORS:
1. Chandana C.
2. Shreedhar Venkatesh
PARTICULARS OF CONTRIBUTORS:
1. Assistant Professor, Department of
Obstetrics and Gynaecology, Vydehi
Institute of Medical Sciences & Research
Centre, Bangalore.
2. Professor and HOD, Department of
Obstetrics and Gynaecology, Vydehi
Institute of Medical Sciences & Research
Centre, Bangalore.
NAME ADDRESS EMAIL ID OF THE
CORRESPONDING AUTHOR:
Dr. Chandana C,
# 868, 9th Main,
BTM Layout, 2nd Stage,
Bangalore – 76.
E-mail: [email protected]
Date
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Submission: 12/09/2014.
Peer Review: 15/09/2014.
Acceptance: 22/09/2014.
Publishing: 01/10/2014.
J of Evidence Based Med & Hlthcare, pISSN- 2349-2562, eISSN- 2349-2570/ Vol. 1/ Issue 8 / Oct 15, 2014.
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