DOI: 10.1148/radiol.2403040110
(Radiology 2006;240:905-909.)
© RSNA, 2006
Case 98: Primary Serous Papillary Carcinoma of the Peritoneum1
Mustafa Kemal Demir, MD,
Fugen Vardar Aker, MD and
Neset Koksal, MD
1 From the Departments of Radiology (M.K.D.), Pathology (F.V.A.), and Surgery (N.K.), Haydarpasa Numune Education and Research Hospital, Istanbul, Turkey. Received January 25, 2004; revision requested April 2; revision received April 9; accepted May 24; final version accepted June 23.
Correspondence: Address correspondence to M.K.D., 11 kisim, Yasemin Apt, D blok, Daire 35 Ataköy, Istanbul, Turkey 34158 (e-mail: demirkemal{at}superonline.com).
 |
HISTORY
|
|---|
A previously healthy 69-year-old woman presented with fatigue, intermittent poorly localized abdominal pain, and discomfort of several weeks duration associated with an 8-kg weight loss. She was afebrile. Physical examination revealed abdominal distention and a palpable mass in the left lower quadrant of the abdomen. Laboratory tests did not reveal any abnormalities, with the exception of a serum CA-125 level of 560 U/mL (normal level, <35 U/mL). Gastroscopy and colonoscopy revealed normal findings. Findings of chest radiographic and recent annual mammographic examinations also were within normal limits. Computed tomography (CT) and pelvic magnetic resonance (MR) imaging were performed. Other abdominal structures—such as the pancreas, kidneys, and bladder—appeared normal on CT and MR images (not shown).
|
IMAGING FINDINGS
|
|---|
Contrast material–enhanced helical CT of the abdomen and pelvis (Fig 1) revealed nodular implants and irregular thickening of the peritoneum. Most of the nodules coalesced to form large omental plaques (omental cakes). The largest plaque was located in the left lower quadrant of the abdomen; it extended to the pelvis but did not involve the ovary. There was no calcification within the masses. The size of the ovaries was normal, but the left ovary had distinct small implants. A moderate amount of ascites was present in the pelvis. MR imaging of the pelvis confirmed the CT findings. The left ovarian implants appeared as surface nodules and had signal intensities that were similar to signal intensities of other pelvic nodules and peritoneal omental cakes (Fig 2). All of the masses were enhanced with contrast material. The mesentery and uterus were classified as normal. No abdominal or pelvic lymph node enlargement was detected.
View larger version (143K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 1a: Transverse CT images of the (a, b) lower abdomen and (c) pelvis obtained after oral administration of 5% ioxithalamate meglumin (Telebrix Gastro; Guerbet, Aulnay-sous-bois, France) and intravenous administration of 100 mL of iohexol (300 mg of iodine per milliliter, Omnipaque; Nycomed, Oslo, Norway). In a, the omental cake (arrow) is seen in the left lower quadrant of the abdomen. It displaces adjacent loops of the large and small intestines and replaces the omentum. The mesentery is normal and without lymphadenopathy. Ascites is not seen at this level. In b (obtained at a lower level than a), lacelike omental infiltration (large arrow) is seen with irregular nodular thickening of the peritoneum (small arrow). Mesenteric fat is normal, and no enlarged lymph nodes or ascites is seen. In c, the omental cake (small arrow) and a moderate amount of ascitic fluid (*) are seen. A small left adnexal implant (large arrow) can be distinguished from the omental cake.
|
|
View larger version (138K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 1b: Transverse CT images of the (a, b) lower abdomen and (c) pelvis obtained after oral administration of 5% ioxithalamate meglumin (Telebrix Gastro; Guerbet, Aulnay-sous-bois, France) and intravenous administration of 100 mL of iohexol (300 mg of iodine per milliliter, Omnipaque; Nycomed, Oslo, Norway). In a, the omental cake (arrow) is seen in the left lower quadrant of the abdomen. It displaces adjacent loops of the large and small intestines and replaces the omentum. The mesentery is normal and without lymphadenopathy. Ascites is not seen at this level. In b (obtained at a lower level than a), lacelike omental infiltration (large arrow) is seen with irregular nodular thickening of the peritoneum (small arrow). Mesenteric fat is normal, and no enlarged lymph nodes or ascites is seen. In c, the omental cake (small arrow) and a moderate amount of ascitic fluid (*) are seen. A small left adnexal implant (large arrow) can be distinguished from the omental cake.
|
|
View larger version (126K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 1c: Transverse CT images of the (a, b) lower abdomen and (c) pelvis obtained after oral administration of 5% ioxithalamate meglumin (Telebrix Gastro; Guerbet, Aulnay-sous-bois, France) and intravenous administration of 100 mL of iohexol (300 mg of iodine per milliliter, Omnipaque; Nycomed, Oslo, Norway). In a, the omental cake (arrow) is seen in the left lower quadrant of the abdomen. It displaces adjacent loops of the large and small intestines and replaces the omentum. The mesentery is normal and without lymphadenopathy. Ascites is not seen at this level. In b (obtained at a lower level than a), lacelike omental infiltration (large arrow) is seen with irregular nodular thickening of the peritoneum (small arrow). Mesenteric fat is normal, and no enlarged lymph nodes or ascites is seen. In c, the omental cake (small arrow) and a moderate amount of ascitic fluid (*) are seen. A small left adnexal implant (large arrow) can be distinguished from the omental cake.
|
|
View larger version (146K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2a: (a, b) Transverse T2-weighted fast spin-echo MR images of the pelvis (repetition time msec/echo time msec, 3500/90) reveal peritoneal soft-tissue masses that appear as multiple nodules forming a large omental cake (arrowhead) in the left side and pelvic ascites (*). Left ovarian implants appear as a surface nodularity distinct from the omental cake, with normal-sized ovaries (arrows). The left ovarian implants have signal intensities similar to those of the pelvic peritoneal omental cakes. (c) Transverse T1-weighted gadolinium-enhanced fast spin-echo MR image of the pelvis (550/14) shows peritoneal soft-tissue masses (arrows).
|
|
View larger version (144K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2b: (a, b) Transverse T2-weighted fast spin-echo MR images of the pelvis (repetition time msec/echo time msec, 3500/90) reveal peritoneal soft-tissue masses that appear as multiple nodules forming a large omental cake (arrowhead) in the left side and pelvic ascites (*). Left ovarian implants appear as a surface nodularity distinct from the omental cake, with normal-sized ovaries (arrows). The left ovarian implants have signal intensities similar to those of the pelvic peritoneal omental cakes. (c) Transverse T1-weighted gadolinium-enhanced fast spin-echo MR image of the pelvis (550/14) shows peritoneal soft-tissue masses (arrows).
|
|
View larger version (127K):
[in this window]
[in a new window]
[Download PPT slide]
|
Figure 2c: (a, b) Transverse T2-weighted fast spin-echo MR images of the pelvis (repetition time msec/echo time msec, 3500/90) reveal peritoneal soft-tissue masses that appear as multiple nodules forming a large omental cake (arrowhead) in the left side and pelvic ascites (*). Left ovarian implants appear as a surface nodularity distinct from the omental cake, with normal-sized ovaries (arrows). The left ovarian implants have signal intensities similar to those of the pelvic peritoneal omental cakes. (c) Transverse T1-weighted gadolinium-enhanced fast spin-echo MR image of the pelvis (550/14) shows peritoneal soft-tissue masses (arrows).
|
|
|
DISCUSSION
|
|---|
The main differential diagnostic considerations of diffuse peritoneal involvement associated with ascites include infectious processes (mainly tuberculosis) and malignant neoplastic conditions.
Peritoneal involvement is a rare form of abdominal tuberculosis. Peritoneal tuberculosis occurs predominantly in patients aged 20–40 years. Drug abuse, alcoholism, acquired immunodeficiency syndrome, cirrhosis, and steroid therapy are the usual risk factors for peritoneal involvement in patients with tuberculosis (1). Peritoneal infection can appear as the wet type, with ascites or pockets of loculated fluid; as the dry type, with bulky mesenteric thickening and lymphadenopathy; as the fibroadhesive type, with mass formation due to omental thickening; or as a combination of types (2,3). It is usually seen in association with widespread abdominal disease that includes lymphadenopathy, organomegaly, ascites, or bowel involvement. Ascites is seen in massive amounts or as loculated areas that have high attenuation on CT scans or are somewhat hyperintense on T1-weighted MR images because of the exudative content. The mesentery generally thickens and increases in attenuation (the so-called misty mesentery) and may include necrotic or calcified lymphadenopathy. The peritoneal thickening associated with tuberculosis is usually mild and smooth; however, nodular irregular thickening with large omental cakes was seen in this patient.
Tumors that involve the peritoneum and cause ascites are usually malignant, and metastatic peritoneal carcinomatosis is considerably more common than primary tumors. Ovarian carcinoma is the most frequent cause of metastatic disease of the omentum. The features of primary malignant ovarian epithelial tumors include a diameter of more than 4 cm, varying proportions of a solid component with necrosis, a thick irregular wall, thick septae, papillary projections, and the presence of ascites and invasive characteristics, such as peritoneal disease or lymphadenopathy (4,5). In this case, the ovaries were normal in size but showed surface nodularities similar to the pelvic peritoneal omental cakes on CT scans and MR images; this finding was indicative of metastasis in the left ovary and made a diagnosis of primary ovarian carcinoma less likely.
Other tumors that frequently spread to the peritoneum include primary tumors arising from the stomach, colon, breast, pancreas, kidney, bladder, or uterus. No identifiable primary tumor was detected in this patient.
The differential diagnosis of malignant primary tumors of the peritoneum with omental involvement and a variable amount of ascites includes malignant peritoneal mesothelioma, peritoneal lymphomatosis, and primary serous papillary carcinoma of the peritoneum.
Malignant peritoneal mesothelioma is a tumor that arises from mesothelial cells lining the peritoneal cavity. The peritoneal form represents approximately 30% of all mesotheliomas (6). Previous exposure to asbestos is an important risk factor, but about half of the patients with this abnormality do not have a history of asbestos exposure (7). This tumor has a clear male predominance, with a 7:1 male-to-female ratio. Imaging features include (a) diffuse or nodular thickening of the peritoneum, (b) peritoneal or omental masses mainly in the upper abdomen, (c) local invasion of adjacent abdominal organs, (d) thickened mesentery and serosal ligaments, and (e) ascites. The organs most commonly involved are the colon and liver (6). Pleural effusion and pleural plaques are also sometimes observed (8). Given the imaging studies performed in this patient, malignant peritoneal mesothelioma was not the most likely diagnosis.
Primary non-Hodgkin lymphoma may rarely involve the peritoneum, omentum, and mesentery (9,10). Although the involvement pattern of omentum and ascites without any loculation or septations matches the findings in this patient, the absence of enlarged lymph nodes in the retroperitoneum and mesentery without primary gastrointestinal lesions argues against a diagnosis of peritoneal lymphomatosis.
Pseudomyxoma peritonei can also be considered in the differential diagnosis. This disorder represents a form of intraperitoneal spread of mucin-secreting tumors. This condition usually arises from tumors in the appendix (adenoma or adenocarcinoma) and ovaries (benign or malignant mucinous tumors). Typical imaging features include widespread heterogeneous peritoneal fluid collections that displace and distort the hollow viscera or produce a scalloping effect on solid organs (11). Mucinous implants on the peritoneal surfaces and omentum may contain linear or septal calcifications. The absence of a primary tumor and mucinous ascites with the mucinous involvement of peritoneal surfaces, omentum, and bowel loops made a diagnosis of pseudomyxoma peritonei unlikely.
Primary serous papillary carcinoma of the peritoneum (PSPCP) is a rare primary peritoneal tumor. It is believed to arise from the secondary müllerian system, which comprises the pelvic and lower abdominal mesothelial lining. The mesothelium of the peritoneum and the germinal epithelium of the ovary arise from the same embryologic origin; therefore, the peritoneum may retain the multipotentiality of the müllerian system and allow a primary carcinoma to develop. The clinical and histologic appearances of PSPCP are similar to those of papillary serous ovarian carcinoma. PSPCP may occur even in the absence of ovarian tissue. Women who have undergone bilateral oophorectomy can still develop PSPCP (12–14), and PSPCP has been reported in men (15,16).
The imaging features of PSPCP, much like the imaging features of papillary serous ovarian carcinoma, include ascites and focal or diffuse peritoneal nodules. However, the size of ovaries is usually normal, even though implants may occur on the surface of the ovaries in patients with PSPCP. Diffuse omental involvement ranged from lacelike infiltrations to large masses and irregular nodular peritoneal thickening of the lower abdominal cavity that was caused by pelvic ascites in this elderly postmenopausal woman with normal-sized ovaries; these findings were most compatible with a diagnosis of PSPCP (14,17). Moreover, a surface nodularity on the left ovary represented metastasis due to the superficial spread of the tumor in the absence of an identifiable primary site.
A preoperative evaluation often reveals an elevated serum CA-125 level, which is consistent with findings in this patient; however, this finding is not diagnostic. CA-125 is an antigen determinant of a high-molecular-weight glycoprotein: It is not a tumor-specific antigen, as it is also elevated in approximately 1% of healthy control subjects; in patients in the first trimester of pregnancy; in patients with liver cirrhosis, endometriosis, or infectious processes—such as tuberculosis or pancreatitis; and in 40% of patients with advanced intraabdominal nonovarian malignancy (18–20). Calcifications of the omental masses and peritoneum, lymphadenopathy, focal bowel wall thickening, and large adnexal masses are the other reported imaging findings associated with PSPCP (21,22); however, none of these findings were present in this patient.
Exploratory laparotomy confirmed the presence of diffuse peritoneal carcinomatosis in association with bilateral ovarian metastatic implants and without an identifiable primary tumor. PSPCP was diagnosed on the basis of histologic evaluation of a surgical specimen.
This patient met the PSPCP criteria identified by the Gynecologic Oncology Group. The following findings are necessary for PSPCP to be diagnosed at macroscopic examination: normal-sized ovaries, tumor bulk in the peritoneum, and greater tumor involvement at one or more extraovarian sites than on the surface of either ovary. In addition, the following microscopic criteria are required for a diagnosis of PSPCP: no tumor or tumor confined to the surface epithelium in the ovary; tumor smaller than 5 mm in diameter involving the ovarian surface and the underlying cortical stroma; or tumor smaller than 5 x 5 mm within the ovarian substance, with or without surface involvement (23). Differential diagnosis of malignant mesothelioma was based, apart from morphologic criteria, on the immunoreactivity of the tumor to epithelial specific antigen ab-9 (clone Ber-EP4) and carcinoembryonic antigen, and on the lack of immunostaining for calretinin and mesothelial cell antigen (clone HMBE-1).
|
FOOTNOTES
|
|---|
| Part one of this case appeared 4 months previously and may contain larger images.
|
|
References
|
|---|
- Hulnick DH, Megibow AJ, Naidich DP, Hilton S, Cho KC, Balthazar EJ. Abdominal tuberculosis: CT evaluation. Radiology 1985;157:199–204.[Abstract/Free Full Text]
- Leder RA, Low VH. Tuberculosis of the abdomen. Radiol Clin North Am 1995;33:691–705.[Medline]
- Ha HK, Jung JI, Lee MS, et al. CT differentiation of tuberculous peritonitis and peritoneal carcinomatosis. AJR Am J Roentgenol 1996;167:743–748.[Abstract/Free Full Text]
- Jung SE, Lee JM, Rha SE, Byun JY, Jung JI, Hahn ST. CT and MR imaging of ovarian tumors with emphasis on differential diagnosis. RadioGraphics 2002;22(6):1305–1325.[Abstract/Free Full Text]
- Occhipinti KA. Computed tomography and magnetic resonance imaging of the ovary. In: Anderson JC, ed. Gynecologic imaging. London, England: Churchill Livingstone, 1999; 345–359.
- Raptopoulos V. Peritoneal mesothelioma. Crit Rev Diagn Imaging 1985;24:293–328.[Medline]
- Busch JM, Kruskal JB, Wu B; and Armed Forces Institute of Pathology. Best cases from the AFIP: malignant peritoneal mesothelioma. RadioGraphics 2002;22(6):1511–1515.[Free Full Text]
- Smith TR. Malignant peritoneal mesothelioma: marked variability of CT findings. Abdom Imaging 1994;19(1):27–29.[CrossRef][Medline]
- Runyon BA, Hoefs JC. Peritoneal lymphomatosis with ascites: a characterization. Arch Intern Med 1986;146:887–888.[Abstract]
- Kim Y, Cho O, Song S, Lee H, Rhim H, Koh B. Peritoneal lymphomatosis: CT findings. Abdom Imaging 1998;23(1):87–90.[CrossRef][Medline]
- Walensky RP, Venbrux AC, Prescott CA, Osterman FA Jr. Pseudomyxoma peritonei. AJR Am J Roentgenol 1996;167:471–474.[Free Full Text]
- Muto MG, Welch WR, Mok SC, et al. Evidence for a multifocal origin of papillary serous carcinoma of the peritoneum. Cancer Res 1995;55:490–492.[Abstract/Free Full Text]
- Piver MS, Jishi MF, Tsukada Y, Nava G. Primary peritoneal carcinoma after prophylactic oophorectomy in women with family history of ovarian cancer: a report of the Gilda Radner Familial Ovarian Cancer Registry. Cancer 1993;71:2751–2755.[CrossRef][Medline]
- Zissin R, Hertz M, Shapiro-Feinberg M, Bernheim J, Altaras M, Fishman A. Primary serous papillary carcinoma of the peritoneum: CT findings. Clin Radiol 2001;56(9):740–745.[CrossRef][Medline]
- Shmueli E, Leider-Trejo L, Schwartz I, Aderka D, Inbar M. Primary papillary serous carcinoma of the peritoneum in a man. Ann Oncol 2001;12(4):563–567.[Abstract/Free Full Text]
- Shah IA, Jayram L, Gani OS, Fox IS, Stanley TM. Papillary serous carcinoma of the peritoneum in a man: a case report. Cancer 1998;82(5):860–866.[CrossRef][Medline]
- Chopra S, Laurie LR, Chintapalli KN, Valente PT, Dodd GD 3rd. Primary papillary serous carcinoma of the peritoneum: CT-pathologic correlation. J Comput Assist Tomogr 2000;24(3):395–399.[CrossRef][Medline]
- Zanaboni F, Vergadoro F, Presti M, Gallotti P, Lombardi F, Bolis G. Tumor antigen Ca 125 as a marker of ovarian epithelial carcinoma. Gynecol Oncol 1987;28:61–67.[CrossRef][Medline]
- Barutcu O, Erel HE, Saygili E, Yildirim T, Torun D. Abdominopelvic tuberculosis simulating disseminated ovarian carcinoma with elevated Ca-125 level: report of two cases. Abdom Imaging 2002;27(4):465–470.[CrossRef][Medline]
- Kebapci M, Vardareli E, Adapinar B, Acikalin M. CT findings and serum Ca-125 levels in malignant peritoneal mesothelioma: report of 11 new cases and review of the literature. Eur Radiol 2003;13(12):2620–2626.[CrossRef][Medline]
- Stafford-Johnson DB, Bree RL, Fracis IR, Korobkin M. CT appearance of primary papillary serous carcinoma of the peritoneum. AJR Am J Roentgenol 1998;171:687–689.[Abstract/Free Full Text]
- Furukawa T, Ueda J, Takahashi S, et al. Peritoneal serous papillary carcinoma: radiological apperance. Abdom Imaging 1999;24:78–81.[CrossRef][Medline]
- Bloss JD, Liao SY, Buller RE, et al. Extraovarian peritoneal serous papillary carcinoma: a case-control retrospective comparison to papillary adenocarcinoma of the ovary. Gynecol Oncol 1993;50:347–351.[CrossRef][Medline]
Congratulations to the 62 individuals and five resident groups that submitted the most likely diagnosis (primary serous papillary carcinoma of the peritoneum) for Diagnosis Please, Case 98. The names and locations of the individuals and resident groups, as submitted, are as follows:
Individual responses
- Canan Altay, MD, Balcova, Izmir, Turkey
- Amalin Aucar, Capital Federal, Argentina
- Eric L. Bressler, MD, Minnetonka, Minn
- Douglas C. Brown, MD, Virginia Beach, Va
- Dr Andrea Bruscagnin, Venice, Italy
- Manuela Certo, MD, Santo Tirso, Portugal
- Neal R. Conti, MD, Seattle, Wash
- Manoel de Souza Rocha, São Paulo, Brazil
- J. F. K. de Villiers, Gisborne, New Zealand
- Thaworn Dendumrongsup, MD, Hat, Yai, Songkla, Thailand
- Mark T. DiMarcangelo, DO, MS, FACR, Cherry Hill, NJ
- Juliet H. Fallah, MD, Chicago, Ill
- Ángeles Franco, Madrid, Spain
- Akira Fujikawa, Tokyo, Japan
- Ann S. Fulcher, MD, Richmond, Va
- Douglas Gardner, MD, Windsor, Ontario, Canada
- Gilles Genin, MD, Annecy, France
- Paulo Gil Agostinho, Coimbra, Portugal
- Thomas Grant, DO, Chicago, Ill
- Horacio Gutierrez, MD, Longmont, Colo
- Srinivasan Harish, Hamilton, Ontario, Canada
- Maureen Heldmann, MD, Shreveport, La
- Kiriakos Kalampoukas, MD, Athens, Greece
- Koki Kato, Tochigi, Japan
- Myeong-Jin Kim, MD, PhD, Seoul, Korea
- Glenn Krinsky, MD, Ridgewood, NJ
- Stefanos Lachanis, MD, Athens, Greece
- Mario Laguna, West Allis, Wis
- Douglas R. Lake, Charleston, SC
- Eugene Lin, MD, Seattle, Wash
- Kao-Lang Liu, MD, Taipei, Taiwan
- Patricia Lowry, MD, Richmond, Va
- Chikara Maeda, Wakayamashi, Japan
- Frank McKowne, MD, Vancouver, Wash
- Manabu Minami, MD, Tsukuba, Ibaraki, Japan
- Sankar Ranjan Mondal, MD, Nassau, Bahamas
- Dr Thomas Moser, Strasbourg, France
- Hiroshi Nobusawa, MD, Tokyo, Japan
- Marcos Nogueira Chagas, MD, Brasilia, Brazil
- Carlos Ovejero Vela, MD, Barcelona, Spain
- Neeraj J. Panchal, MD, San Diego, Calif
- David M. Panicek, MD, New York, NY
- Narendrakumar P. Patel, MD, Newburgh, NY
- Juan Carlos Pernas, MD, Barcelona, Spain
- Francesco Pierazzoli, Padua, Italy
- Ivan Pilate, MD, Mechelen, Belgium
- Hilton Pittman, MD, Pensacola, Fla
- Prashant Raghavan, MD, Charlottesville, Va
- Anuradha T. Rao, Houston, Tex
- Bharat Raval, MD, Houston, Tex
- Steven Schepers, Herent, Belgium
- Kouichi Sugiyama, Numazu, Japan
- Weawdao Tachawattanakul, MD, Chiengrai, Thailand
- Norio Takahashi, MD, Fukui, Japan
- Eliko Tanaka, Yokohama, Japan
- Yumiko Oishi Tanaka, MD, Tsukuba, Ibaraki, Japan
- Kazuma Terauchi, Shizuoka, Japan
- Hiroyuki Ueda, Kyoto, Japan
- Shigeaki Umeoka, MD, Wakayama, Japan
- Dr Bonny Varghese, Abu Dhabi, United Arab Emirates
- Dr Silvio Vollmer, Cipolleti, Rio Negro, Argentina
- Stanko Yovichevich, MD, Sydney, Australia
Resident group responses
- Kyoto City Hospital Radiology Residents, Kyoto, Japan
- Maine Medical Center Radiology Residents, Portland, Me
- Prince of Songkla University Radiology Residents, Hat Yai, Songlka, Thailand
- Trakya University School of Medicine Radiology Residents, Edirne, Turkey
- University of Pennsylvania Radiology Residents, Philadelphia, Pa
TOP
HISTORY
IMAGING FINDINGS
