CT appearance of disseminated peritoneal adenomucinosis

Abstract

Disseminated peritoneal adenomucinosis (DPAM) is a relatively rare cause of pseudomyxoma peritonei, marked by peritoneal lesions with abundant extracellular mucin and little cytologic atypia among exfoliated tumor cells, and usually associated with appendiceal adenomas. Peritoneal mucinous carcinomatosis (PMCA) also causes diffuse peritoneal tumor but is marked by neoplastic mucinous epithelium with cytologic features of carcinoma and associated with appendiceal, colon, gastric, or small bowel carcinoma. Compared with PMCA, DPAM has a different distribution of disease and a significantly better prognosis. DPAM is characterized by the lack of lymph node involvement, with primarily superficial peritoneal involvement, and a relatively benign, relapsing course over many years. Dominant primary masses may not be evident in DPAM. Despite considerable overlap in the two main causes of pseudomyxoma peritonei, there are features of the two that may help differentiate between them. We present the computed tomographic appearance of DPAM in a series of seven cases and emphasize characteristics of the disease.

Clinical Presentation of Pseudomyxoma Peritonei

“…Of the 23 patients diagnosed preoperatively with PMP or who underwent laparotomy with a preoperative suspicion of PMP, 18 (78%) were investigated with computed tomography preceding initial surgery. All those 18 patients produced typical radiological findings for PMP, as reported elsewhere (15), although in 11 cases the CT scan was not the first investigation to suggest such a diagnosis. Suspected ovarian tumour was the most common cause for surgery, overall comprising 26 of 82 initial surgeries (32%).…”

Secondary Tumors and Tumorlike Lesions of the Peritoneal Cavity: Imaging Features with Pathologic Correlation

“…The volume of mucin tends to be greater in true pseudomyxoma peritonei compared with that caused by mucinous peritoneal carcinomatosis (38). Mucin within the peritoneum is usually low in CT attenuation, but areas of soft-tissue attenuation may be present that represent solid tumor elements, fibrosis, or compression of the mesentery (35).…”

“…Mucinous carcinomatosis tends to involve the chest more frequently with effusions or pleural masses and may also be accompanied by mesenteric or retroperitoneal lymphadenopathy, omental caking, and invasion into parenchymal organs (38). In contrast, pseudomyxoma peritonei typically does not invade visceral organs or spread by lymphatic or hematogenous routes (25).…”

Malignant Glomus Tumor of the Peritoneum: Case Report

“…Imaging differentiation of the classic pseudomyxoma peritonei from mucinous carcinomatosis is difficult. Mucinous carcinomatosis tends to invade into parenchymal organs, more frequently involves the chest and omentum and may also be accompanied by metastatic lymphadenopathy (19). …”