Uterine clear cell adenocarcinoma with adenomyosis: a case report with review of literature

ABSTRACT

Corpus cancer is the most frequently occurring female genital cancer. Clear cell carcinoma is a rare form of endometrial cancer accounting for 1-1.5% of all endometrial carcinomas and is often associated with an aggressive clinical behaviour and poor clinical outcome. The molecular pathways involved in development of clear cell carcinoma are still unclear with the present presumption that it arises from atrophic endometrium. Patients with clear cell carcinoma often experience relapse in pelvis, in para-aortic nodes and at distant sites. Total abdominal hysterectomy and bilateral salphingo-oopharectomy with comprehensive surgical staging is the standard surgical treatment of patients with clear cell carcinoma of endometrium, whereas pelvic irradiation, para-aortic irradiation, whole abdominal irradiation and chemotherapy have been widely employed as post operative therapy. This is a case report of a rare case of clear cell carcinoma of endometrium with adenomyosis. A 65 years menopausal lady presented with bleeding per vagina for the past 6 months. She was married and nulliparous. She was a hypertensive patient on irregular treatment. Physical examination was unremarkable. She was evaluated for her post menopausal bleeding. She had an unsatisfactory colposcopy. MRI showed a large polypoidal endometrial mass lesion with an impression of neoplastic endometrial growth. With a provisional diagnosis of endometrial polyp/ carcinoma endometrium she was planned for total abdominal hysterectomy and bilateral salphing-oopharectomy surgery. Following a difficult surgery for a suspected uterine malignancy she had a good post operative recovery. Histopathological report revealed a high grade endometrial clear cell carcinoma with focal papillary serous differentiation with adenomyosis and leiomyoma. In view of high grade invasive carcinoma of endometrium she was referred to a radiotherapy unit for post operative radiotherapy. This case was analyzed with review of other reported case. Review re-emphasised the need of accurate histopathological diagnosis in addition to clinical staging especially in rare variants of endometrial carcinomas like clear cell carcinoma and squamous carcinoma in optimizing the treatment. However the importance of myometrial invasion cannot be overlooked especially when adenomyosis co-exists with adenocarcinoma. It is important to have a clear distinction between a true myometrial invasion by adenocarcinoma and involment of adenomyotic foci by malignant cells without myometrail invasion in determining the staging and prognosis. In view of non-availability of commonly accepted guidelines for the management of patients with clear cell carcinoma of endometrium, and adequate molecular, characterization of clear cell carcinoma is strongly warranted in order to identify new biological prognostic variables of the disease and to develop novel molecular targeted therapies