|Year : 2013 | Volume
| Issue : 3 | Page : 514-516
Rare case of primary mucinous adenocarcinoma of vagina
Bharati Dhorepatil, Dipak Kumar Ladda, Aarti U Rapol
Department of Obstretics & Gynaecology, Shree Multispeciality Hospital, Pune-Nagar Road, Opposite Agakhan Palace, Pune, Consultant Pathologist, Shree Multispeciality Hospital, Pune- Nagar Road, Opposite Agakhan Palace, Pune, Assistant Gynaecologist, Department of Obstretics & Gynaecology, Shree Multispeciality Hospital, Pune-Nagar Road, Opposite Agakhan Palace, Pune, India
|Date of Web Publication||8-Oct-2013|
Department of Obstretics & Gynaecology Shree Multispeciality Hospital, Pune - Nagar Road, Opposite Agakhan Palace, Pune - 411006
Source of Support: None, Conflict of Interest: None
Here we present a rare case of primary mucinous adenocarcinoma of vagina treated successfully with wide local excision and post-operative radiotherapy. There was no recurrence even after four years.
Keywords: Mucinous, adenocarcinoma, primary, vagina, rare
|How to cite this article:|
Dhorepatil B, Ladda DK, Rapol AU. Rare case of primary mucinous adenocarcinoma of vagina. J Can Res Ther 2013;9:514-6
| > Introduction|| |
Carcinoma of the vagina is one of the rarest of malignancies comprising 1-2% of all gynecological malignancies.  Squamous cell vaginal cancers account for approximately 85% and Adenocarcinoma account for approximately 15% of the cases.  There are subtypes of adenocarcinoma such as papillary, mucinous adenocarcinoma and clear cell adenocarcinoma. Primary mucinous adenocarcinoma of vagina is one of the rarest subtypes.  Another variant of adenocarcinoma known as clear cell Adenocarcinoma is rare and occur mostly in patients below 30 years, who have a history of in utero exposure to diethylstilbestrol (DES).  Vagina is the most frequent for secondary to metastases from tumors of Cervix and Vulva. 
| > Case Report|| |
A 56 year old postmenopausal lady presented with chief complaint of per vaginal bleeding since one year which was on and off in nature. The patient was a known case of hypertension since ten years and diabetes since two years, well controlled by medications. On general examination the patient was obese and BP was 160/90 mm of Hg. Generalized lymphadenopathy was absent. On systemic examination, cardiovascular system and respiratory system were normal. Abdomen was soft with no palpable mass or organomegaly. On local examination labia majora and minora were normal. Per speculum examination showed an ulcer in the lower one third of vagina on the posterior wall, at the junction of vulva and vagina [Figure 1]. The ulcer measured approximately 3 × 2 cm in size. Both lateral walls of vagina were free. Cervix and the fornices looked normal. On per vaginal examination, uterus was normal in size. No mass felt in the fornices. Bimanual per rectal examination showed that rectum were free from the lesion. No palpable lymph nodes found. Biopsy of the ulcer was taken with a suspicion of malignancy and sent for histopathological examination [Figure 2].
Histopathology of ulcer
Sections of ulcer showed cells forming groups and glands, floating in pools of mucin. Nuclei were pleomorphic, hyperchromatic and vascular with prominent nuclei and mitoses. Some cells showed eccentric nuclei with vacuolated cytoplasm giving them signet ring appearance suggestive of mucinous adenocarcinoma.
Taking into consideration 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. Colposcopy, endocervical and endometrial biopsy were normal. All blood investigations including liver function tests, renal function tests, coagulation profile were normal. Ultrasonography of abdomen and pelvis revealed normal study. CT scan of abdomen and pelvis was normal. HRCT thorax was normal. Whole body PET scan did not show any evidence of metabolically active disease elsewhere in the body. There was no evidence of local or regional metastases.
Based on clinical findings and investigations, patient was diagnosed as a case of primary mucinous adenocarcinoma of vagina. Clinical staging was Stage I (FIGO classification).
Management: After obtaining fitness for surgery, patient was posted for surgery under general anesthesia. Wide local excision of the ulcer was done with primary closure of vaginal wall. Postoperative period was uneventful and patient was discharged on day four. Specimen was sent for Histopathological examination.
Histopathology of specimen
Sections showed tumor mass comprising of large collections of extracellular mucinous areas with significant number of signet ring cells with scattered gland formations. Cells revealed large hyperchromatic nuclei and scanty cytoplasm. Mucinous foci and signet ring cells were arranged in small, medium and large sized islands. 0-1 mitotic Figures were noted per high power field. Tumor mass was seen hugging up the basal portion of nonkeratinized stratified squamous epithelium and seen to be diffusely infiltrating lamina propria and partially invading and infiltrating muscle coat. Proximal surgical margin, left lateral surgical margin and base were seen to be free from tumor tissue invasion.
Impression: Mucinous adenocarcinoma with free proximal and lateral surgical margins.
[Figure 3]: Microscopic examination reveals abundant amount of extracellular mucin diffusely admixed with ovoid, oblong to spindly cells having large hyperchromatic nuclei and scant cytoplasm. There is marked pleomorphism with increased mitotic activity. The tumor mass is seen to be diffusely invading the muscle component.
|Figure 3: Microscopic examination reveals abundant amount of extracellular mucin diffusely|
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Based on this report surgical staging of the lesion was Stage II (FIGO classification) so that postoperative radiotherapy.
Follow-up: Up after four years did not show any local recurrence.
| > Discussion|| |
Primary mucinous adenocarcinoma is quite a rare entity. Commonest presentation of patient is painless vaginal bleeding in 65-80% of cases.  Since 80% of vaginal cancers are metastatic, primarily from the cervix or endometrium, primary site must be excluded beforehand.
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.  Malignant transformation of foci of adenosis can result in adenocarcinoma of vagina. 
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. , Embrahim S et al. commented that as primary mucinous adenocarcinoma of the vagina is rare tumor individualized treatment is justified until larger series have been published.  Primary mucinous adenocarcinoma of vagina is characterized by aggressiveness and poor prognosis because of its rapid growth and recurrence, its frequent distant metastases, and its relative resistance to conventional treatment modalities including surgery, radiotherapy and chemotherapy.  Diagnosis of primary clear cell adenocarcinoma of vagina should be based on normal cytopathologic examination of the cervix, endometrium, uterus, ovaries, kidneys and absence of detectable tumor in urinary tract. 
To conclude, primary mucinous 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.
| > Acknowledgement|| |
Dr. Shehbaaz Daruwala, Chief Embryologist, Pune Fertility Center, Pune, Dr. Harshal Pandve, Research Consultant and Dr. Sanjay Deshmukh, Onco-Surgeon.
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[Figure 1], [Figure 2], [Figure 3]