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Journal of Clinical Oncology, Vol 25, No 7 (March 1), 2007: pp. 897-898 © 2007 American Society of Clinical Oncology. DOI: 10.1200/JCO.2006.09.6537
Hepatic Pneumatosis As a Complication of an Abdominal Desmoid TumorDepartments of Medical Oncology and Radiology, Vall d'Hebron University Hospital, Barcelona, Spain A 36-year-old man was admitted to our emergency department with severe diffuse abdominal pain, nausea, and fever that had begun 48 hours earlier. He denied having diarrhea or weight loss. He had previously been diagnosed with familial adenomatous polyposis (FAP), and a prophylactic colectomy had been performed 5 years earlier. In addition, 1 year before he was admitted to the emergency department, he was diagnosed with an asymptomatic 7 x 8 cm desmoid tumor of the anterior abdominal wall, and he had refused any treatment so far. His vital signs showed temperature 39.3°C, blood pressure 100/64 mmHg, pulse 110 beats per minute, and respiratory rate 20 breaths per minute. His abdomen was mildly distended without peritoneal signs. The remainder of the physical examination was normal. A complete blood count revealed a white blood cell count of 22.000/mm3 (80% neutrophils), hemoglobin of 13.2 mg/dL, and platelet count of 90.000/mm3. Serum chemistries, liver function tests, and a urinalysis were unremarkable. An abdominal computed tomography (CT) scan revealed an intra-abdominal abscess at the mesenteric base of the desmoid tumor (Fig 1, arrow) with diffuse portal pneumatosis (Fig 2). Percutaneous CT scan–guided drainage of the collection was performed. Clostridium perfringens was cultured from blood and drained samples. Follow-up abdominal magnetic resonance revealed neither sign of residual portal venous gas nor signs of infection. The patient's condition gradually improved after supportive treatment, and he was discharged with an uneventful recovery.
Desmoid tumors or aggressive fibromatosis occur at a rate of 10% to 15% in patients with FAP, an autosomal inherited disease caused by germline mutations in the tumor suppressor gene for adenomatous polyposis coli, located on band 5q21.1 Although desmoids are histologically benign, they tend to form large infiltrative masses that, if not widely excised, recur repeatedly (Fig 3; CT scan without contrast enhancement showing large desmoid tumor originating from the right rectus muscle with extensive mesenteric fibromatosis). The synonym aggressive fibromatosis describes the marked cellularity and aggressive local behavior. In patients with FAP, desmoid tumors are usually intra-abdominal, arising from the mesenterium. Primary surgery with negative surgical margins is the most successful primary treatment modality. The role of adjuvant radiotherapy and chemotherapy in the treatment of desmoid tumors still remains unclear. Responses to single-agent doxorubicin chemotherapy have been reported,2 as well as responses to combination chemotherapy at either standard or relatively low doses.3,4 Despite these different treatment modality approaches, desmoid tumors represent the major cause of death in patients with FAP undergoing prophylactic colectomy.5 Intra-abdominal desmoid tumors remain asymptomatic for long periods, and the initial symptoms are usually due to intestinal, vascular, or ureteric compression and obstruction. Exceptionally, there are a few cases in the literature with invasion of desmoid tumor into the bowel wall, resulting in fistula formation and suspected translocation of intestinal bacteria to the tumor with abscess formation.6,7 Percutaneous drainage has been the initial method of treatment in each case. Furthermore, intra-abdominal abscesses and other nonischemic abdominal conditions have been rarely associated with hepatic portal venous gas (HPVG).8 The mechanism for the appearance of gas in the portal vein is not well understood. In contrast to mesenteric ischemia, these cases are generally associated with a favorable outcome without surgery. To our knowledge, HPVG associated with intra-abdominal desmoid tumors has not been reported. We report a rare complication of an abdominal wall desmoid tumor with intratumor abscess formation and secondary massive hepatic pneumatosis. Complications such as intra-abdominal infections should be taken into consideration in patients with abdominal desmoid tumors.
AUTHORS' DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The authors indicated no potential conflicts of interest.
REFERENCES 1. Klemmer S, Pascoe L, DeCosse J: Occurrence of desmoids in patients with familial adenomatous polyposis of the colon. Am J Med Genet 28:385-392, 1987[CrossRef][Medline] 2. Seiter K, Kemeny N: Successful treatment of a desmoid tumor with doxorubicin. Cancer 71:2242-2244, 1993[CrossRef][Medline] 3. Patel S, Evans H, Benjamin R: Combination chemotherapy in adult desmoid tumors. Cancer 72:3244-3247, 1993[CrossRef][Medline] 4. Weiss A, Horowitz S, Lackman R: Therapy of desmoid tumors and fibromatosis using vinorelbine. Am J Clin Oncol 22:193-195, 1999[CrossRef][Medline] 5. Arvanitis ML, Jagelman DG, Fazio VW, et al: Mortality in patients with familial adenomatous polyposis. Dis Colon Rectum 33:639-642, 1990[CrossRef][Medline] 6. Cholongitas E, Koulenti D, Panetsos G, et al: Desmoid tumor presenting as intra-abdominal abscess. Dig Dis Sci 51:68-69, 2006[CrossRef][Medline] 7. Maldjian C, Mitty H, Garten A, et al: Abscess formation in desmoid tumors of Gardner's syndrome and percutaneous drainage: A report of three cases. Cardiovasc Intervent Radiol 18:168-171, 1995[Medline] 8. Sebastia C, Quiroga S, Espin E, et al: Portomesenteric vein gas: pathologic mechanisms, CT findings, and prognosis. Radiographics 20:1213-1224, 2000
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Copyright © 2007 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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