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OP13.04: McCune Albright syndrome: a diagnosis to be kept in mind
Author(s) -
Barros L.,
Sousa F.,
Bernardo M. D.
Publication year - 2011
Publication title -
ultrasound in obstetrics and gynecology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.202
H-Index - 141
eISSN - 1469-0705
pISSN - 0960-7692
DOI - 10.1002/uog.9381
Subject(s) - medicine , mccune–albright syndrome , polyostotic fibrous dysplasia , precocious puberty , secondary sex characteristic , breast development , adrenarche , bone age , pediatrics , gynecology , hormone , fibrous dysplasia , surgery
A 64-year old woman presenting with symptoms of abdominal pressure and bloody discharge was found to have a pelvic mass above the vaginal apex on ultrasound scan. She had undergone hysterectomy and bilateral salpingo-oophorectomy and pelvic lymphadenectomy, 13 years previously, for cervical adenocarcinoma with early invasion. There were no lymph node metastases, and at that time all other staging procedures showed normal findings. Now, both transvaginal and transabdominal ultrasound showed a 6 cm nonhomogenous solid and richly vascularized pelvic mass immediately affecting the bladder wall, but it was not possible to determine whether the tumor originated from or infiltrated the bladder. She had another large tumor of similar structure in the right iliac fossa as well as severe hydronephrosis of the right kidney. The suspected diagnosis was that of a recurrence of the cervical carcinoma or a tumor of the urinary bladder. A CT scan confirmed the findings of the pelvic masses, one engulfing the right ureter and the other engulfing the external iliac vessels, but did not add valuable information regarding tumor origin, except to exclude invasion of the bladder. Again the suspected diagnosis was a recurrence or the possibility of a lymphogenic tumor. She underwent surgery, which included dissection of the tumor from the bladder with partial resection of the bladder, resection of the affected segment of the ureter, reconstruction of the bladder and direct reimplantation of the ureter into the bladder. Histopathology supplemented by immunohistochemistry concluded with the diagnosis of an atypically proliferating endometrioid papillary cystadenoma (borderline tumor) with extensive mucinous metaplasia that originated from cystic endometriosis.