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Abstract
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the second frequent cause of primary amenorrhea. There have been several reports concerning gynecologic disease in MRKH syndrome, but there has been no case about a colon cancer in a patient with this condition. A 43 years old woman, who reported primary amenorrhea with sexual infantilism, was evaluated in our department because of lower abdominal pain: she presented with a suprapubic mass. To evaluate the suprapubic abdominal mass and primary amenorrhea, abdominal MRI, colonoscopy and endocrine tests were done. She had no gynecologic organs, and she finally was diagnosed as having colon cancer with the atypical form of MRKH syndrome (bilateral gonadal agenesis 46 XX). To the best of our knowledge this is the first case concerning primary colon cancer in a patient with MRKH syndrome.
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Fig. 3
Post-operation gross finding. A, Gross section finding of a Lt. colon cancer. The mucosal surface shows an ulcerofungating mass (dark arrow), measuring 10.5 × 5 cm across. The mass invaded to the pericolic fat and mesentery of small intestine; B, Gross section finding of a Rt. colon cancer. The mucosal surface shows a well demarcated fungating mass (dark arrow) and a large pedunculated polypoid mass (white arrow) in the ascending colon, measuring 4 × 3 cm across in the former and 4 × 3 cm across in the latter.
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