CASE from the lesions. She was a

CASE
REPORT : A 61 year old hypertensive post menopausal woman
presented in our outpatient department on april 2017 with chief complaints of
white discharge  from vagina since 7 month,which
was watery in nature,associated with painless vaginal bleeding  since 2 months which was on and off in nature.

On examination she was moderately built and nourished,
vitals were stabled, pallor was present and generalized lymph-adenopathy was
absent. On systemic examination cardiovascular system, respiratory system &
per abdomen findings were normal.There was no palpable mass or organomegaly.On
local examination no abnormality was found on external genitalia.per speculum
examination revealed an ulceroproliferative growth on the lower one third of
vagina on the posterior wall, the ulcer measured approximately 5×4 cm in size
covered with white discharge.cervix os was not visualized due to obscured view
by large growth. On per vaginal examination there was large, irregular
proliferative growth  about 6x4cm size
felt in lower third of posterior vaginal wall. It was firm in
consistency.Bleeding was present on pv examination cervical os was smooth ,both
fornices were free. per rectal examination,rectum mucosa  was smooth &  free from the lesions. She was a multiparous
female with the first chid born at 20 yrs of age. There was no significant
personal,menstrual ,past or family history.

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Biopsy of the lesion
was advised with a suspicion of malignancy and sent for histopathological
examination that turned out primary papillary adenocarcinoma of vagina.

microscopic picture  shows bits of polypoidal mass entangled in
blood clots partially lined by papillary glandular epithelium. The papillary
fronds are showing evidence of stratification.nuclear atypia and presence of
abnormal mitotic figures. The tumor fragments 
are surrounded by dense & acute and chronic inflammatory infiltrate,
haemorrhage and necrosis. Taking in to consideration vaginal
adenocarcinoma is a rare entity,IHC confirmation was done. Immunohistochemistry
showed CK20, DUPAN 2 positive while  CA
125, serum CEA were within normal range.

 

 

The primary vaginal
adenocarcinoma is a rare malignancy,The chances of secondary from cervix,vulva
&endometrium is commonest .Taking into consideration of this thought that
commonest lesions in vagina are secondary arising from cervix, vulva and
endometrium, further evaluation of patient was done to rule out any other
primary focus of malignancy. Patient was evaluated and baseline
investigation were done.  complete hemogram,
chest x ray were normal.ultrasonography of abdomen & pelvis showed lower
one third of vaginal growth on posterior wall.CECT abdomen & pelvi
suggestive of large ill defined mass approximately 4.8 X2.7 cm size  seen in lower vagina with loss of fat plane
with rectum and urinary bladder. hepatomegaly with grade I fatty changes and
multiple cholelethiasis.cystoscopy & proctosigmoidoscopy revealed no
abnormal findings.

On the basis of
clinical examination & investigation. patient was diagnosed as a case of
primary papillary adenocarcinoma of vagina. Based on FIGO classification
patient  was staged as   FIGO stage III.

Management :Looking at
the size of the lesion patient hasbeen taken for neoadjuvant chemotherapy(
Nabpaclitaxel + cisplatin) planned for three cycles.we have observed that
lesion was progressive in spite of given two cycles of neoadjuvant
chemothery,then patient switched to EBRT with concurrent chemotherapy.AP – PA
portal was planned,2 Gy per fraction,total 60 Gy with weekly inj cisplatin 30
mg/iv was given.patient responded well to EBRT,there was 40 % growth regression
in first week of treatment.The treatment period was uneventfull .we have
achieved complete respons at the end of fifth week of treatment.she has also received
3 #  of 
( 6 Gy per # ) HDR brachytherapy.she was on regular followup with no
events of residual disease & recurrence clinically.

DISCUSSION-
Carcinoma of the vagina is one of the rarest malignancy comprising 1-2% of all
gynecological malignancies.1 Carcinoma of the vagina is defined as a
primary carcinoma arising in the vagina and not involving the external os of
the cervix superiorly or vulva inferiorly.  Squamous
cell vaginal cancers account for approximately 85% and Adenocarcinoma account
for approximately 15% of the cases.2 Histological patterns of
adenocarcinoma include clear cell carcinoma, adenosquamous, papillary &
undifferentiated. Other types are melanomas, sarcomas and unspecified types.

Risk factors includes increasing age, atypical
cells in the vagina called vaginal intraepithelial neoplasia (VAIN), exposure to
diethylstilbestrol (DES), multiple sexual partners,
early age at first intercourse, smoking, HIV infection.

Primary papillary
adenocarcinoma is quite a rare entity. Commonest presentation of patient is
painless vaginal bleeding in 65-80% of cases.3  But in our case it was watery discharge per
vagina followed by  painless vaginal
bleeding. The most common adenocarcinoma of the vagina are metastatic, which
constitute the majority of vaginal cancers (80%-90%) originating from the
colon, endometrium, ovary, or rarely from pancreas and stomach. Hence primary
adenocarcinoma of the vagina is diagnosis of exclusion. 

Prognostic factors are
stage of the disease at the time of diagnosis and type of the lesion. Survival
is reduced in patients who are 60 years and above, are symptomatic at the time
of diagnosis, have lesions of the middle and lower third of the vagina, or have
poorly differentiated tumours.4,5  Kucera H & Vavra N
studied in 434 patients reported  that
disease is primarily found in elderly as 78% were found to be older than 60
years of age. Younger patients had a 5-year survival of 50%; patients between
61 and 75 years of age, 41.2%; and those 76 years of age or older, 34.3%.

Squamous cell vaginal
cancers spread superficially within the vaginal wall and invade the paravaginal
tissues and parametria. Distant metastases occur most commonly in lungs and
liver. However, adenocarcinomas predominantly have pulmonary metastases and
supraclavicular and pelvic node involvement.6

 EBRT followed by ICRT remains the primary
treatment . Several studies have evaluated external beam radiotherapy for
vaginal adenocarcinoma. Frank et al.7 reported 26 patients with
primary vaginal adenocarcinoma treated with external beam radiotherapy and
brachytherapy with mostly grade 1-2 toxicities.

 The combination of external beam radiotherapy
and cisplatinum was described by Samant et al 8 He studied  on twelve patients that were treated with
concurrent weekly chemoradiotherapy and proved that  it is feasible to
deliver concurrent weekly Cis-platinum chemotherapy with high-dose radiation,
leading to excellent local control and an acceptable toxicity profile. In the
present case, concurrent cisplatin appeared to sensitize the tumor to radiation
since the tumor responded at a lower dose and at a faster rate than expected.
Concurrent chemotherapy with irradiation appears to significantly enhance
radiation effects on cancer and cycling epithelial cells, but it does not
appear to proportionately increase late effects in normal tissues.8

five year survival
rates for women with stage III  disease
ranges from 25 % to 58% 9 with local failure rates of 30 % to 75 %.10
Despite treatment with  EBRT and
brachytherapy ,only 20% to 30 %of patients with stage III & IV disease
achieve local control. Pelvic recurrences occur more often than distant
recurrences.11

CONCLUSION- To conclude, primary papillary adenocarcinoma is rare,  little is known about its etiology and
behavior. . Early diagnosis with high index of suspicion is
extremely important so that effective treatment can be done with less
recurrence with good quality of life.  This case reported here
for its rare presentation in all gynecological malignancies. 

REFERENCES

1.    
Cancers of
Vagina. As available from: http://www.altabatessummit.org/clinical/cancergynec.html.

2.    
Gallup DG,
Talledo OE, Shah KJ, Hayes C. Invasive squamous cell carcinoma of the vagina: A
14-year study. Obstet Gynecol 1987;69:782-5

3.    
Vaginal
Cance. As available from: http://emedicine.medscape.com/article/269188-overview#a010 Last accessed on 2012 Mar 28

4.    
Kucera H,
Vavra N. Radiation management of primary carcinoma of the vagina: Clinical and
histopathological variables associated with survival. Gynecol Oncol
1991;40:12-6. 

5.    
Eddy GL,
Marks RD Jr, Miller MC 3rd, Underwood PB Jr. Primary invasive vaginal
carcinoma. Am J Obstet Gynecol 1991;165:292-6.

6.    
Clement PB,
Benedet JL. Adenocarcinoma in situ of the vagina: A
case report. Cancer 1979;43:2479-85.

7.    
Frank SJ,
Deavers MT, Jhingran A, Bodurka DC, Eifel PJ. Primary adenocarcinoma of the
vagina not associated with diethylstilbestrol (DES) exposure . Gynecol Oncol. 2007;105:470–474.

8.    
Samant R,
Lau B, E C, Le T, Tam T. Primary vaginal cancer treated with concurrent
chemoradiation using Cis-platinum. Int J Radiat
Oncol Biol Phys. 2007;69:746–750.

9.    
Shah CA, et al. Factors affecting risk
of mortality in women with vaginal cancer.Obstet Gynecol 2009;113(5):1038–1045.

10.  Tran
PT, et al. Prognostic factors for outcomes and complications for primary
squamous cell carcinoma of the vagina treated with radiation. Gynecol Oncol 2007;105(3):641–649

11.  Frank
SJ, et al. Definitive radiation therapy for squamous cell carcinoma of the
vagina. Int J Radiat Oncol Biol Phys 2005;62(1):138–147.