Does Preoperative Diagnosis Will Change the Treatment Plan of Clear Cell Carcinoma of Endometrium Masquerading as Desmoid Tumor of Anterior Abdominal Wall?: a Case Report

Clear cell carcinoma (CCC) of the female genital tract can arise in the ovary, endometrium, cervicovaginal region, in peritoneal and other extra pelvic sites. However, CCC involving anterior abdominal wall is a very rare entity. We are reporting a case of 55-year-old postmenopausal lady with ECOG PS-I with no comorbidity had history of cesarean section about 30 years back and presented with pain in abdomen at periumbilical region about 9 months back. She underwent evaluation at outside clinic with finding of an anterior abdominal wall swelling of size approximately 3 × 3 cm which was completely neglected by the patient. In the next period of 2 months, the swelling suddenly increased in size. CECT/PET CT showed a solid cystic mass involving anterior abdominal wall with possibility of desmoid tumor. We had decided to plan for surgical excision of the tumor. Intraoperatively we found that there was bicornuate uterus. The lesion was arising from one of the cornue of uterus and it was extended anteriorly to anterior abdominal wall with no ascites, no pelvic, or retroperitoneal lymphadenopathy. She underwent hysterectomy with bilateral salpingo-oophorectomy and defect was closed with bioabsorbable mesh. Final histopathology report was clear cell endometrial carcinoma mostly mullerian tract origin arising from endometriosis focus. Tumor cells were diffusely positive for PAX-8 and Napsin-A and negative for WT 1 with patchy positivity for P53 (wild type expression). She had received adjuvant chemotherapy and she is disease free after 2 years of completion of the treatment. Clear cell endometrial carcinoma arising from malignant transformation of an endometriosis focus to anterior abdominal wall is a very rare phenomenon and it is a difficult task to reach its final diagnosis.