DOI: 10.1148/rg.256055015
RadioGraphics 2005;25:1689-1692
© RSNA, 2005
Best Cases from the AFIP
Borderline Papillary Serous Tumor of the Right Ovary1
Kimberly J. Burkholz, MD,
Beverly P. Wood, MD and
Craig Zuppan, MD
1 From the Departments of Radiology (K.J.B., B.P.W.) and Pathology (C.Z.), Loma Linda University Medical Center, 11234 Anderson St, Loma Linda, CA 92354. Received January 31, 2005; revision requested March 1 and received April 4; accepted April 5. All authors have no financial relationships to disclose.
Address correspondence to K.J.B. (e-mail: kimberlyburkholz{at}yahoo.com).
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History
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A 14-year-old previously healthy girl complained of 6 weeks of increasing abdominal pain that began in the periumbilical region and then localized to the right lower quadrant. She had gained weight despite a decreased appetite. An earlier medical visit resulted in treatment with antacids and laxatives, without subsequent resolution of, or decrease in, her pain. Physical examination showed an abdominal fluid wave and tenderness in the right lower quadrant. Results of laboratory studies showed a complete blood cell count of 11.1 x 109/L (normal range, 4.8–11.8 x 109/L) and a slightly elevated erythrocyte sedimentation rate of 30 mm/h (normal range, 0–20 mm/h). The results of a serum CA-125 assay, which were received 3 days after admission, showed a markedly elevated level of cancer antigen, at 351 U/mL (normal range, 0–34 U/mL).
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Imaging Findings
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Imaging evaluation with contrast material–enhanced computed tomography (CT) of the abdomen and pelvis showed a large, heterogeneously enhancing soft-tissue mass with multiple frondlike projections located in both sides of the pelvis. The dimensions of the mass were 10 x 4 x 8 cm. Extensive ascites was present, with multiple soft-tissue nodules scattered along the omentum, the peritoneum, and the peritoneal surface of the liver and spleen (Fig 1). Multiple mesenteric and retroperitoneal lymph nodes with a diameter of less than 1 cm also were observed.
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Figure 1. Contrast-enhanced CT scan of the pelvis shows a complex right adnexal mass with multiple papillary projections (white arrows) that extends into the cul-de-sac and left adnexa and that is associated with ascites and a peritoneal lesion (black arrow). Neither ovary is discernible as a discrete structure. The right ureter (arrowhead) is opacified.
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Transabdominal pelvic images obtained with Doppler ultrasonography (US) showed a large mass that was predominantly associated with the right adnexa and that extended into the posterior cul-de-sac and left adnexa. Multiple cystic areas, some with a central area of intermediate echogenicity and with prominent vascularity, were the dominant US features (Fig 2).
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Figure 2a. (a) Transverse Doppler US image of the right ovary shows a partly cystic (arrowheads) and partly solid right adnexal mass anterior to the iliac vessels (arrow). There is prominent vascularity in the solid portion of the tumor, anterior to the cystic region. The fluid-filled bladder (B) is adjacent to the mass, and ascites (A) is visible laterally. (b) Sagittal US image of the left ovary shows the cystic mass (arrowheads) with a central area of echogenicity (small *) and a solid portion (large *) of tumor adherent to the left ovary. The uterus (arrow) and bladder (B) also are identifiable. At laparoscopic biopsy, histologic findings in the left and right ovarian masses were identical.
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Figure 2b. (a) Transverse Doppler US image of the right ovary shows a partly cystic (arrowheads) and partly solid right adnexal mass anterior to the iliac vessels (arrow). There is prominent vascularity in the solid portion of the tumor, anterior to the cystic region. The fluid-filled bladder (B) is adjacent to the mass, and ascites (A) is visible laterally. (b) Sagittal US image of the left ovary shows the cystic mass (arrowheads) with a central area of echogenicity (small *) and a solid portion (large *) of tumor adherent to the left ovary. The uterus (arrow) and bladder (B) also are identifiable. At laparoscopic biopsy, histologic findings in the left and right ovarian masses were identical.
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Pathologic Evaluation
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At exploratory laparotomy, massive ascites was found. There was a large cauliflower-shaped mass adherent to the right ovary and a smaller but similar mass on the surface of the left ovary. Multiple peritoneal and omental tumor implants were present. Evaluation of a rapid frozen histopathologic section of a peritoneal implant showed an ovarian epithelial neoplasm. A right salpingo-oophorectomy, subtotal left oophorectomy, omentectomy, resection of multiple peritoneal implants, and retroperitoneal lymph node assessment were performed. The right ovary contained a 12 x 8 x 5-cm 234-g friable grayish-tan granular papillary tumor mass with a solid white connective tissue core. A 3-cm central cystic region encased multiple granular papillary projections (Fig 3). The resected right ovarian mass consisted largely of a floridly exophytic papillary serous tumor with fairly extensive micropapillae, or tufts, that indicated borderline malignant potential (Fig 4). Analysis of tissue specimens obtained from subtotal resection of the left ovary and from multiple serosal tumor implants showed similar histologic components. No stromal invasion (as in full-fledged carcinoma) was present.
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Figure 4. Photomicrograph (original magnification, x200; hematoxylin-eosin stain) shows exophytic papillary serous tumor with extensive micropapillae, or tufts, which are characteristic of a tumor with low malignant potential.
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Discussion
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Borderline papillary serous tumors of the ovary, also known as ovarian tumors of low malignant potential, were first described in 1929 and were designated for separate classification in the early 1970s by the World Health Organization. Like most epithelial ovarian tumors, borderline papillary tumors are derived from serous or mucinous cell types. These tumors are a subset of ovarian epithelial neoplasms that are associated with extraovarian disease but have an indolent course and a more favorable prognosis than that typical of their malignant counterparts (1–4). The borderline category comprises approximately 4%–14% of all epithelial ovarian neoplasms. The mean age of women affected by borderline tumors is approximately 10 years younger at manifestation than the mean age of women affected by malignant ovarian epithelial tumors. Borderline tumors primarily occur in teens and young women, as in the case reported here (5,6). The tumors are clinically silent until they achieve an advanced size or stage. In one study, the most frequent initial manifestations were abdominal pain, increasing abdominal girth or distension, and abdominal masses. Sixteen percent of patients were asymptomatic at diagnosis (7).
Ovarian epithelial tumors, whether benign, of low malignant potential, or malignant, are primarily cystic masses (either unilocular or multilocular) (8). In general, the amount of solid tissue correlates with the likelihood of malignancy (9,10). Borderline tumors tend to develop in an exophytic growth pattern, on the surface of the ovary, without invading the underlying stroma. Papillary projections are characteristic of and are often abundant in epithelial tumors with low malignant potential, whereas they are not typically seen, or are insignificant, in benign cystadenomas. Papillary projections also are present in many malignant ovarian epithelial tumors, although solid elements are often the dominant feature of such tumors (10–12). In one study, papillary projections were observed in 13% of benign neoplasms, 67% of low-malignant-potential neoplasms, and 38% of malignant neoplasms on CT scans or magnetic resonance (MR) images (11). The diagnosis of borderline tumor should be considered when an ovarian tumor with an abundance of papillary projections is seen in young patients. Since imaging specificity does not allow confident differentiation of a borderline tumor from a malignant serous ovarian neoplasm, both types of tumor require exploratory laparotomy for surgical and pathologic staging (13).
The reported incidence of retroperitoneal lymphatic involvement detected at surgical staging in patients with borderline ovarian tumors was 21% in one study; although the nodal status of patients did not significantly affect survival, patients with localized intraperitoneal disease and nodal involvement had a higher rate of recurrence (14).
The rate of detection of intratumoral blood flow at Doppler US in borderline tumors is similar to that in malignant neoplasms: Flow is depicted in 90% of borderline tumors and 92% of malignant neoplasms. The resistance and pulsatility indexes are also significantly reduced in carcinoma and borderline ovarian tumors, compared with those in benign tumors (15). The combined use of color Doppler US and serum CA-125 testing has been advocated for differentiation of benign from malignant ovarian neoplasms (16). Serum CA-125 levels are elevated in patients with borderline serous tumors in approximately 90% of cases (reported median, 66 U/mL; reported range, 5–272 U/mL; normal range, 0–34 U/mL) (17) and are higher in patients with borderline and malignant tumors than in those with benign cystic neoplasms (18).
A unique feature of borderline tumors is the noninvasive behavior of extraovarian tumor implants in the advanced stages of the disease. Tumor implants to the contralateral ovary, omentum, and peritoneal surface are present in the advanced stages, although they behave in a benign fashion and remain located on the surface of the underlying tissues, in contradistinction to invasive tumor implants from malignant ovarian carcinoma. A minority of borderline tumors are associated with invasive peritoneal implants, which may recur or progress and behave as do low-grade carcinomas (19–21). Careful examination of the contralateral ovary and peritoneum should be performed at follow-up imaging in all patients treated for advanced-stage disease, particularly in those in whom disease is associated with invasive tumor implants. CT and MR imaging are as useful for detecting extraovarian disease (8) as they are for detecting malignant ovarian neoplasms.
Tumor invasion may be established with laparotomy, which is essential for staging (9). The prognosis is excellent when disease is confined to the ovary (stage I disease); the survival rate is 99.5%. Surgical excision is considered curative for stage I disease. For patients with stage II–IV disease, the most reliable prognostic indicator is the type of peritoneal tumor implant (invasive or noninvasive). In one review article, survival for patients with advanced-stage tumors and noninvasive tumor implants was reported to be 95.3%, compared with 66% survival for patients with invasive implants (20). Treatment of advanced-stage disease often involves a combination of surgery and chemotherapy, although no prospective clinical trials have yet proved the effectiveness of adjuvant therapy (19). Since many patients with borderline tumors of the ovary are in their reproductive years, treatment with fertility-sparing surgery is important; however, this option is generally reserved for patients with stage I disease. The results of one study showed a higher recurrence rate in patients who underwent cystectomy (with or without contralateral oophorectomy) than in patients who underwent oophorectomy of the ovary with the primary tumor (58% and 23%, respectively); however, mortality rates were low in both patient groups (22).
Surgical treatment in the case reported here consisted of right salpingo-oophorectomy, subtotal left oophorectomy, and tumor debulking. No adjuvant chemotherapy was given. Because the patient and her family were reluctant to proceed with immediate total hysterectomy and bilateral salpingo-oophorectomy, close follow-up is necessary to monitor the patient for recurrence, as elements of disease may remain in the left ovary.
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Footnotes
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Editor’s Note.—Everyone who has taken the course in radiologic pathology at the Armed Forces Institute of Pathology (AFIP) remembers bringing beautifully illustrated cases for accession to the Institute. In recent years, the staff of the Department of Radiologic Pathology has judged the "best cases" by organ system, and recognition is given to the winners on the last day of the class. With each issue of RadioGraphics, one or more of these cases are published, written by the winning resident. Radiologic-pathologic correlation is emphasized, and the causes of the imaging signs of various diseases are illustrated.
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References
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- International Federation of Gynecology and Obstetrics. Classification and staging of malignant tumours in the female pelvis. Acta Obstet Gynecol Scand 1971;50:1–7.[Medline]
- Serov SF, Scully RE, Sobin LH. Histologic typing of ovarian tumors. In: World Health Organization. International histological classification and staging of tumors. No. 9. Geneva, Switzerland: World Health Organization, 1973.
- Eltabbakh GH, Natarajan N, Piver MS. Epidemiologic differences between women with borderline ovarian tumors and women with epithelial ovarian cancer. Gynecol Oncol 1999;74(1):103–107.[CrossRef][Medline]
- Barakat RR. Borderline tumors of the ovary. Obstet Gynecol Clin North Am 1994;21:93–105.[Medline]
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History
Imaging Findings
