Unusual Cause of a Massive Abdominal Cystic Tumor

Abstract

Question: A 74-year-old Chinese woman presented to the gastroenterology unit with a 3-day history of abdominal distention. This was associated with intractable vomiting and an inability to tolerate food. There had been no bowel movement for the last 2 days. She had a history of total hysterectomy and bilateral salpingo-oopherectomy 19 years ago for ovarian granulosa theca cell tumor, but was otherwise healthy. Physical examination revealed a large, firm, immobile abdominal mass, with no hepatosplenomegaly. Computed tomography revealed a massive cystic lobulated and septated lesion measuring 21 cm in maximal diameter in the right peritoneal cavity (Figure A). Superiorly, it extended to the subhepatic space indenting the gallbladder. Laterally and anteriorly, it was inseparable from the anterior abdominal wall (Figure B). Blood investigations revealed anemia, with hemoglobin of 10.9 g/dL (normal, 11.7–14.7). Chest radiography was unremarkable. Tumor markers alpha-fetoprotein, CA19-9, CA12-5, CA15-3, and carcinoembryonic antigen levels were not elevated. The patient underwent resection of the tumor. Operative findings included a large, multiloculated, cystic tumor weighting 2.1 kg arising from the greater omentum, with no gross peritoneal disease. The tumor was removed, together with the greater omentum and the right-sided peritoneum. Figure C demonstrates hematoxylin and eosin-stained histologic section. What does the black arrow indicate? What is the diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. The patient had recurrence of adult granulosa cell ovarian tumor (GCT). Figure C demonstrates oval tumor cells with fine chromatin, single small nucleoli and an occasional longitudinal groove (black arrow), which is a characteristic feature of GCT. Figure D demonstrates uniform cytoplasmic staining with S-100. Of GCTs, 50% are positive for S-100 protein. Figure E shows patchy membranous staining for inhibin (white arrows) in tumor cells. The tumor cells were negative for AE 1/3, calretinin, CD117, CD34, and desmin. Our case illustrates the importance of considering late recurrence of GCT as a cause for an abdominal cystic mass. Accounting for 70% of ovarian sex cord-stromal tumors,1Schumer S. Cannistra S. Granulosa cell tumor of the ovary.J Clin Oncol. 2003; 21: 1180-1189Crossref PubMed Scopus (481) Google Scholar GCTs mainly presents in peri- or postmenopausal women with a median age at presentation of 50 years.2Fox H. Agrawal K. Langley F.A. A clinicopathologic study of 92 cases of granulosa cell tumor of the ovary with special reference to the factors influencing prognosis.Cancer. 1975; 35: 231-241Crossref PubMed Scopus (260) Google Scholar Infertility and treatments for infertility have been suggested as risk factors for the disease.3Unkila-Kallio L. Tiitinen A. Wahlstrom T. et al.Reproductive features in women developing ovarian granulosa cell tumour at a fertile age.Hum Reprod. 2000; 15: 589-593Crossref PubMed Scopus (53) Google Scholar GCTs are known to recur many years after the disease, with the greatest reported time from primary presentation to recurrence being 37 years.4East N. Alobaid A. Goffin F. et al.Granulosa cell tumour: a recurrence 40 years after initial diagnosis.J Obstet Gynaecol Can. 2005; 27: 363-364Abstract Full Text PDF PubMed Scopus (39) Google Scholar Prognostic factors predicting early recurrence include fewer Call-Exner bodies and higher mitotic rates on pathologic examination, as well as more advanced stage and larger tumor size. However, none of these is a significant predictor of late recurrence.5Auranen A. Sundström J. Ijäs J. et al.Prognostic factors of ovarian granulosa cell tumor: a study of 35 patients and review of the literature.Int J Gynecol Cancer. 2007; 17: 1011-1018Crossref PubMed Scopus (54) Google Scholar A typical radiologic appearance of GCT recurrence is discrete round or lobulated masses. Because the patient had undergone total hysterectomy and bilateral oophorectomy, nongynecologic differential diagnoses with similar radiologic appearances should also be considered, including cystic mesothelioma and peritoneal carcinoma. However, cystic mesothelioma tends to occur in a younger age group, and peritoneal carcinoma tends to be characterized by omental caking and calcification. Pseudomyxoma peritonei is also a consideration, but it tends to cause multiple complex masses with scalloping of the liver. Operative resection of GCT recurrence remains the treatment of choice, but several other treatment options have been tried, with responses for radiotherapy, chemotherapy, gonadotrophin-releasing hormone agonists, and aromatase inhibitors. Because the patient was well, she declined any adjuvant therapy, and has been recurrence free 1 year after the surgery.