Adenoid cystic carcinoma of the cervix in a young patient.
S Dixit, S Singhal, J Neema, R Soornarayan, HA Baboo
Dept of Radiotherapy, Gujarat Cancer and Research Institute, Asarwa Ahmedabad.
Dept of Radiotherapy, Gujarat Cancer and Research Institute, Asarwa Ahmedabad.
Adenoid cystic carcinoma of the cervix is a rare disease. It usually occurs in an elderly age group. A case who presented at a younger age (30 years) is being reported. The chief complaint was excessive vaginal bleeding for 2 months. Per vaginum examination revealed a growth. She was staged FIGO IIIb. She was treated with radical radiotherapy. Disease regressed slowly and complete local response appeared after six months of treatment. Presentation of adenoid cystic carcinoma in younger age group and effect of radiotherapy in advanced stage is reviewed.
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Dixit S, Singhal S, Neema J, Soornarayan R, Baboo H A. Adenoid cystic carcinoma of the cervix in a young patient. J Postgrad Med 1994;40:94-5
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Dixit S, Singhal S, Neema J, Soornarayan R, Baboo H A. Adenoid cystic carcinoma of the cervix in a young patient. J Postgrad Med [serial online] 1994 [cited 2023 Feb 2 ];40:94-5
Available from: https://www.jpgmonline.com/text.asp?1994/40/2/94/552
Adenoid cystic carcinoma (ACC) of the cervix was first reported by Paalman and Counseller. Generally it is manifested in elderly age group and is a disease of post-menopausal women. Reviewed literature shows that majority of cases present in early stage and surgery is considered as treatment of choice. Very few cases present in advanced stage with invariably poor results. We present a case diagnosed in younger age with advanced stage of cancer
A 30 years female (gravida: 5, para: 5) presented in October 1988 with a chief complaint of excessive bleeding per vaginum of two months duration. Her past medical history including menstrual history was normal. Except anaemia related finding nothing significant was found on her general examination. On per vaginum examination, an exophytic growth was found arising from cervix and occupying the upper two third of vaginal cavity, without involving its wall. Growth was involving both paramethum upto pelvic wall on either side. She was staged FIGO IIIb. Her haemolgobin level was 7.1 gm%. Other blood profile and biochemical investigations were within normal limits. Sonography revealed an echogenic mass in the region of cervix, paraaortic region, liver and kidney were normal. Lungs were clear radiologically. Biopsy turned out as adenoid cystic carcinoma.
Histologically (on hematoxylin and eosin staining) round to oval small polygonal tumour cells with hyperchromatic and monomorphic nuclei were observed. Cells were forming band like structures varying in size and having swiss cheese appearance. PAS and Alcian blue stains were positive. Focal cylindromatus structures were seen. In mucosal fragment dysplastic cells and intra-epithelial malignant squamous cells were also found.
External radiotherapy was delivered through CO60, using anterior - posterior pelvic portals covering main disease, whole length of vagina and regional lymph nodes. After a total tumour dose of 48 Gy in 20 tractions in tour weeks an intracavitary radiotherapy was planned to deliver 25 Gy to point A. She completed whole treatment upto December 1988. On follow-up examination in April 1989, there was a residual disease smaller than at the time of presentation. However at next follow-up in July 1989 there was complete regression of the disease. She remained disease free locally as well as at distant site at last follow-up in December 1992.
ACC of the cervix constitutes about 0.4 to 1.7% of all cervical carcinomas,,. Berhuck et al could trace total 85 cases of ACC of the cervix from the literature, Average age of most of the reported cases is found to be 20 years older than squamous cell carcinoma of the Cervix,. Average age of most of the reported 9 cases all were in the age of range of 52 to 99 years. Musa et al reported 17 cases having mean age of 68.8 years and youngest patient was of 44 years.
However King et al reported three cases who presented at the age of 24-38 years, all were in stage IB and were treated with radical surgery with or without post-operative cisplatin. Two of them found to be disease free at 12 and 64 months and one died with disease at 13 months. Only two cases reported in the literature were of age 31 and 33 years; both were treated with radical surgery including periaortic lymph node dissection and post-operative pelvic radiotherapy.. One remained disease free at 28 months while another died of metastases at 29 months. King et al could trace from the literature. 2 other cases with less than 40 years but one of them was of adenoid basal cell carcinoma while outcome could not be traced for the other. Including our report, among cases at less than 40 years 3 of 6 remained disease free at more than 28 month followup. Though radiotherapy and surgery both have been recommended in early staged adenoid cystic arcinoma of the cervix,,,, surgery should be preferred at an younger age in order to preserve ovarian function.
Excluding our case, all 5 patients younger than 40 years presented in stage IB. Of the 75 total cases of ACC reported in the English literature only 10 cases are of stage IIIB. Response of these patients to therapy is shown in [Table:1].
Our patient remained disease free for more than 40 months when treated with radical radiotherapy. Amongst other 9 reported cases of stage III B 3 cases received incomplete radiotherapy and in remaining 6 only one case remained disease free for 6 years, which too ultimately developed pelvic and lung recurrence. One more patient reported to be disease free at follow-up of 11 months.
Considering poor outcome of stage IIIB, high dose cisplatin may be tried as an adjuvant to radiotherapy. Its efficacy has been demonstrated in early stage adenoid cystic carcinoma of the cervix. However, non-responsiveness to various chemotherapeutic agents has also been reported in the literature,.
To summarize the case reported by us is an advanced stage of ACC at a young age. Though patient responded completely to radiotherapy, response was evident only after 6 months of therapy. Considering the late recurrence in other patients, there is a need for prolonged prolonged follow-up and may be supplementation of chemotherapy.
Paalman RJ, Counseller US. Cylindroma of cervix with procedentia. Am J Obstet Gynecol 1949; 58:184-187.|
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