British Journal of Radiology (2004) 77, 71-73
© 2004 British Institute of Radiology
doi: 10.1259/bjr/35139284
Extensive peritoneal and omental lymphomatosis with raised CA 125 mimicking carcinomatosis: CT and intraoperative findings
M Horger, MD1,
M Müller-Schimpfle, MD1,
I Yirkin, MD2,
M Wehrmann, MD3 and
C D Claussen, MD1
Departments of 1 Diagnostic Radiology, 2 General Surgery and 3 Pathology, Eberhard-Karls-Universität, Hoppe-Seyler-Str.3, 72076 Tübingen, Germany
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Abstract
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Diffuse peritoneal and omental seeding are well-known forms of dissemination of metastatic carcinoma. A wide variety of primary neoplasms may cause peritoneal and omental carcinomatosis, most commonly carcinomas of the ovary, gastrointestinal tract and breast. Extensive involvement of the peritoneal cavity with lymphoma is, however, rare. The association of peritoneal lymphoma with a raised CA 125, a tumour marker which is commonly raised in ovarian carcinoma, is a highly challenging clinical situation, which to our knowledge has not been published before in the medical literature. Not being aware of the possibility of this unusual combination of clinical, laboratory and imaging findings can lead to an erroneous diagnosis, as in our case.
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Introduction
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Omental and peritoneal lymphomatosis are rare manifestations of aggressive histological subtypes of high grade lymphomas. The differentiation between lymphomatosis and carcinomatosis, as well as the uncommon form of peritoneal tuberculosis or other pathological entities with cellular spread into the peritoneal cavity, is difficult on the basis of CT or MR imaging alone. Usually biopsy is required, unless a combined clinical, laboratory and radiological diagnosis is conclusive.
Raised CA 125 antigen is present in the serum of patients with non-mucinous epithelial ovarian carcinoma. Serum levels of CA 125 correlate directly with tumour bulk, therefore elevation of this tumour marker strongly suggests the presence of ovarian carcinoma, leading sometimes, as in our case to an incorrect diagnosis. There are also other primary malignancies, e.g. primary peritoneal or endometrial carcinoma, as well as inflammatory conditions, e.g. endometriosis, which can be associated with an elevated CA 125.
We report an extensive form of peritoneal and omental lymphomatosis occurring shortly postpartum without further typical evidence of lymphoma, accompanied by lymphomatous involvement of the right ovary, infiltration of the pelvic segment of the ipsilateral ureter with secondary hydronephrosis and raised CA 125 tumour marker, mimicking ovarian carcinoma with diffuse peritoneal tumour infiltration.
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Case report
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A 32-year-old woman was admitted to the emergency department of a neighbouring, rural hospital, 5 weeks after giving birth, complaining of acute abdominal pain and rapidly increasing abdominal girth. Her symptoms had started a few days earlier, increasing rapidly. Abdominal ultrasound performed in the emergency unit revealed massive ascites, enlargement of both adnexa and bilateral hydronephrosis, the amount of these changes being less distinct on the left side. On clinical examination, dyspnoea and peripheral oedema were found.
Laboratory data showed a slight increase of gamma-glutamyltransferase and rapidly raising lactate dehydrogenase with a level of 1800 U l-1 (units per litre). The peripheral blood count was unremarkable. Total plasma protein level was slightly lowered. The tumour marker CA 125 level was considerably elevated to 276 U ml-1 (normal value <35 U ml-1).
Chest radiograph showed no pathological changes apart from bilateral raised diaphragms. An abdominal CT scan was performed on a Somatom Plus 4 CT-scanner (Siemens Medical Engineering, Forchheim, Germany). It revealed diffuse thickening of the parietal and visceral peritoneum with contrast enhancement accompanied by a large amount of ascites (Figure 1a
). The omentum was markedly thickened, the retroperitoneal and iliac nodes were normal. There was right adnexal enlargement (Figure 1b). Circumferential thickening of the terminal ileum without stenosis was erroneously interpreted as a further manifestation of carcinomatosis of the visceral peritoneum (Figure 1c). The pelvic segments of both ureters were encased by tumour with dilatation predominantly of the right ureter with secondary hydronephrosis.
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Figure 1. (a) 32-year-old woman with a peritoneal and omental lymphomatosis by Burkitt-like high grade lymphoma. Contrast-enhanced CT: peritoneal and omental lymphomatosis mimicking carcinomatosis. Note also right-sided hydronephrosis. (b) Enlarged right adnexa, as well as, thickening of the parietal peritoneum and ascites. (c) The terminal ileum shows a markedly thickened wall and slight aneurysmal dilatation. (d) Intraoperative image showing peritoneal lymphoma implants along the visceral peritoneum.
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Exploratory laparotomy was performed with right-sided oophorectomy and omentectomy with the assumption of ovarian carcinoma with extensive pelvic and peritoneal infiltration. Intraoperatively, diffuse tumour infiltration of the adnexa as well as numerous micronodules and macronodules were found, simulating peritoneal and omental carcinoma (Figure 1d
). Some of the larger tumour implants, the right ovary and the thickened ileal segment revealed haemorrhagic change. No signs of hepatic or splenic infiltration were detected. Histopathology surprisingly diagnosed a high grade lymphoblastic Burkitt-like, B-cell lymphoma of the terminal ileum with omental and peritoneal lymphomatosis.
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Discussion
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Peritoneal and omental lymphomatosis is a rare condition in malignant lymphoma mimicking metastatic dissemination of carcinoma, so-called carcinomatosis. Patterns of tumour involvement of mesentery, omentum and peritoneum seen in peritoneal lymphomatosis are mostly indistinguishable from those seen in peritoneal carcinomatosis [1] therefore requiring a biopsy or fluid sample to establish the diagnosis. Peritoneal lymphomatosis is usually associated with high grade lymphomas with aggressive histological subtypes such as: small-cell, large-cell, mixed large and small-cell, non-cleaved lymphoma or lymphoblastic Burkitt-like lymphoma as in our case [2–5]. In contrast to peritoneal carcinomatosis, peritoneal lymphomatosis is curable without surgery.
The differentiation from other pathological entities with similar imaging features such as tuberculous peritonitis, mesothelioma, infiltrating fibromatosis, desmoid, or round cell desmoplastic tumour of the mesentery is, however, difficult because of considerable overlap [6]. Pre-operative needle biopsy enables the diagnosis to be made without surgery. Image-guided biopsy with standard haematoxylin-eosin analysis of the biopsy cores, supplemented by immunohistiochemical markers to CA 125, carcinoembryonic antigen, cytokeratin 7 and cytokeratin 20, is a quick and safe diagnostic tool in the differentiation between ovarian carcinoma and for instance lymphoma, and is able to replace laparotomy in the majority of cases [7].
CT is the imaging method of choice in the staging and subsequent follow up of patients with diffuse or focal peritoneal diseases. Our own experience of over 20 cases of peritoneal lymphomatosis in high grade lymphoma patients, agrees with that of other authors [8] who concluded that some imaging findings such as lymphadenopathy, aneurysmal dilatation of a gut segment with wall thickening and poor delineation at the mesenteric border, as well as, liver or splenic enlargement with or without macroscopic signs of infiltration, especially in younger patients, should suggest the diagnosis of lymphoma, most of them originating in the gastrointestinal tract. However, these signs are not always present in this patient group, so that the exclusion of other pathologies cannot be done reliably. On the other hand, the involvement of the ovary with encasement of the pelvic ureteric segment and hydronephrosis, raised CA 125 tumour marker as well as the absence of enlarged retroperitoneal, mesenteric or parailiacal lymph nodes, as in our case, can lead to the false presumptive diagnosis of an ovarian carcinoma with peritoneal carcinomatosis. Even intraoperatively the differentiation of these conditions may not be made as in our case, leading to resection of the right adnexa and great omentum performed with the aim of achieving cytoreduction. Primary ovarian lymphoma should theoretically also be considered as a potential cause of ovarian enlargement and lymphomatosis, but it is a rare entity. It was the raised CA 125, a strong indicator of an ovarian aetiology of the peritoneal and omental disease, which misled us in the diagnosis of this patient, even if its level was not adequate to the amount of peritoneal disease.
We found this case unusual because of its unusual occurrence shortly postpartum with inconclusive imaging, clinical and laboratory (raised CA 125 tumour marker) features leading initially to a false presumptive diagnosis and requiring explorative laparotomy.
Being aware of the similarities, between the imaging features of peritoneal lymphomomatosis and other pathological entities, especially of the much more frequent carcinomatosis, due mostly to ovarian, pancreatic, breast or gastrointestinal malignancies, one should consider the utility of image-guided needle biopsy in order to avoid laparotomy or even erroneous tumour debulking, as in our case.
Received for publication August 27, 2002.
Revision received April 2, 2003.
Accepted for publication April 24, 2003.
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References
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- Runyon BA, Hoefs JC. Peritoneal lymphomatosis with ascites a characterization. Arch Intern Med 1986;146:887–8.[Abstract/Free Full Text]
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Abstract
Introduction
