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Mucinous Appendiceal Neoplasms: Preoperative MR Staging and Classification Compared with Surgical and Histopathologic Findings

Russell N. Low1,2, Robert M. Barone3, Jonathan M. Gurney1,2 and Wayne D. Muller4

1 Sharp and Children's MRI Center, Sharp Memorial Hospital, 7901 Frost St., San Diego, CA 92123.
2 Department of Radiology, Sharp Memorial Hospital, San Diego, CA.
3 Department of Surgical Oncology, Sharp Memorial Hospital, San Diego, CA.
4 Department of Pathology, Sharp Memorial Hospital, San Diego, CA.


Figure 1
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Fig. 1A 67-year-old woman with mucinous appendiceal neoplasm. Delayed gadolinium-enhanced image shows marked enhancement (level 4) of bulky tumor encasing stomach, spleen, and splenic flexure (long white arrows). Enhancing right subphrenic tumor (short white arrows) and tumor in superior recess of lesser sac (black arrow) are also noted. MRI findings indicate peritoneal mucinous carcinomatosis tumor. Delayed enhancement of cellular tumor on gadolinium-enhanced MR images markedly improves tumor conspicuity in pseudomyxoma peritonei syndrome and distinguishes tumor from nonenhancing mucin and ascites.

 

Figure 2
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Fig. 1B 67-year-old woman with mucinous appendiceal neoplasm. Coronal gadolinium-enhanced image shows confluent bulky omental and left paracolic tumor (long white arrows) surrounding splenic flexure and descending colon. Diffuse enhancing mesenteric and small-bowel serosal tumor (short thin white arrows) is present predicting incomplete surgical resection. Right subphrenic tumor (thick white arrows) and tumor encasing gastric antrum (black arrow) are also noted. Subsequent surgical cytoreduction was incomplete.

 

Figure 3
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Fig. 1C 67-year-old woman with mucinous appendiceal neoplasm. High-power microscopic view of mucin contains large aggregates of well-differentiated adenocarcinoma. Specimen showed many tumor cells. Note cytologic atypia (arrow). Findings are that of peritoneal mucinous carcinomatosis tumor.

 

Figure 4
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Fig. 2A 81-year-old man with mucinous appendiceal neoplasm. Fat-suppressed T2-weighted MR image shows right paracolic high-signal-intensity mucin (arrow). T1-weighted image (not shown) depicted similar right paracolic collection.

 

Figure 5
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Fig. 2B 81-year-old man with mucinous appendiceal neoplasm. Delayed gadolinium-enhanced gradient-echo image depicts minimal (level 2) peripheral enhancement (arrow) of mucinous epithelium. Central portion of collection shows no enhancement. MRI findings correlate with disseminated peritoneal adenomucinosis–type mucinous appendiceal neoplasm composed almost entirely of mucin. No involvement of small-bowel mesentery or small-bowel serosa was present.

 

Figure 6
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Fig. 2C 81-year-old man with mucinous appendiceal neoplasm. Medium-power view shows columnar glandular epithelial cells with basally oriented nuclei and mucinous cytoplasm (arrow). Neither cytologic atypia nor mitotic activity is present. Adjacent abundant pink-staining mucin and collagen are also noted. Findings are that of disseminated peritoneal adenomucinosis tumor.

 

Figure 7
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Fig. 3A 77-year-old woman with mucinous appendiceal neoplasm. T1-weighted MR image shows extensive complex mass (arrow) that is interposed between stomach, liver, and spleen. Additional right subphrenic material surrounds liver.

 

Figure 8
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Fig. 3B 77-year-old woman with mucinous appendiceal neoplasm. T2-weighted image shows extensive complex mass (long arrows) interposed between stomach, liver, and spleen. Additional right subphrenic material (short arrow) surrounds liver.

 

Figure 9
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Fig. 3C 77-year-old woman with mucinous appendiceal neoplasm. Axial delayed gadolinium-enhanced gradient-echo image through middle abdomen shows bulky mesenteric mass (arrow) encasing mesenteric vessels.

 

Figure 10
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Fig. 3D 77-year-old woman with mucinous appendiceal neoplasm. Coronal gadolinium-enhanced gradient-echo image shows confluent upper and large middle abdominal mesenteric mass (long arrows). Right subphrenic tumor (short arrow) is also noted. Note that degree of tumor enhancement is less than that of liver parenchyma. Both observers recorded level 2 enhancement (less than liver parenchyma), which indicates disseminated peritoneal adenomucinosis or intermediate-type tumor. Extensive mesenteric tumor predicts incomplete surgical resection. At surgery, bulky diffuse abdominal tumor was found encasing transverse colon, mesentery, and small bowel. Cytoreduction was incomplete.

 

Figure 11
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Fig. 3E 77-year-old woman with mucinous appendiceal neoplasm. Low-power microscopic view shows abundant central mucin (M) with surrounding collagen and single layer of mucinous epithelial cells (arrow).

 

Figure 12
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Fig. 3F 77-year-old woman with mucinous appendiceal neoplasm. Although most specimens had low-grade appearance, high-power microscopic view shows focal area of adenocarcinoma (arrow) invading smooth muscle (SM) of stomach wall. Findings are that of intermediate-type tumor.

 

Figure 13
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Fig. 4A 61-year-old woman with mucinous appendiceal neoplasm. Unenhanced T1-weighted MR image shows 4-cm right paracolic mass (arrow). Solid tumor and mucin cannot be distinguished on unenhanced MRI.

 

Figure 14
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Fig. 4B 61-year-old woman with mucinous appendiceal neoplasm. Unenhanced T2-weighted MR image shows 4-cm right paracolic mass (arrow). Solid tumor and mucin cannot be distinguished on unenhanced MR images.

 

Figure 15
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Fig. 4C 61-year-old woman with mucinous appendiceal neoplasm. Axial delayed gadolinium-enhanced image shows marked enhancement of solid component of tumor (long arrow); 4-cm mass (short arrow) contains nonenhancing mucin centrally and rim of enhancing solid tumor.

 

Figure 16
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Fig. 4D 61-year-old woman with mucinous appendiceal neoplasm. High-power microscopic view shows complex group of neoplastic cells with marked cytologic atypia diagnostic of adenocarcinoma. Arrow is pointing at an abnormal cell with enlarged nucleus having macronucleolus and high nuclear-tocytoplasmic ratio. Findings are that of peritoneal mucinous carcinomatosis tumor.

 

Figure 17
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Fig. 5 64-year-old woman with mucinous appendiceal tumor. Delayed gadolinium-enhanced spoiled gradient-echo MR image shows thin rim of peritoneal tumor (arrow) adjacent to inferior right hepatic lobe. Small-volume small-bowel serosal tumor found at laparotomy was not identified on MR image by either observer. Surgical cytoreduction was complete.

 

Figure 18
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Fig. 6 Quantitative analysis of peritoneal tumor enhancement on delayed gadolinium-enhanced MR images in 21 patients with mucinous appendiceal neoplasm. Patients 1–10 have disseminated peritoneal adenomucinosis or intermediate-grade tumors and patients 11–21 have peritoneal mucinous carcinomatosis tumors. Scatterplot graph shows ratios of tumor-to-liver signal ({diamondsuit}) and tumor-to-intravascular gadolinium signal ({square}). Measurements of mean signal intensity were made on workstation by placing region of interest over enhancing tumor, liver parenchyma, and abdominal aorta or inferior vena cava.

 

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