appendiceal mucoceles - lieberman's...
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Luise Pernar, HMS IVGillian Lieberman, MD
Appendiceal Mucoceles
Luise Pernar, Harvard Medical School IV
Gillian Lieberman, MD
September 2006
Luise Pernar, HMS IVGillian Lieberman, MD
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Patient 1: JH
• 68 yo woman with history of myelodysplastic syndrome
• Admitted to BIDMC for induction of chemotherapy for acute myelogenous leukemia
• During course of induction patient developed fever and neutropenia
• CT scan was performed to search for possible site of infection
Luise Pernar, HMS IVGillian Lieberman, MD
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Patient 1: JH - CT
PACS, BIDMC - Courtesy of Karen Lee, MD
Cecum
Terminal Ileum
Cyst
(27 HU)
Focal wall calcification
Luise Pernar, HMS IVGillian Lieberman, MD
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Patient 1: JH - CT
• Findings summary:– Cystic structure adjacent to cecum near
ileocecal junction; appendix not seen separately
– Density ~ 27HU– Suggestion of rim-enhancement of cystic wall
with focal calcification– No surrounding stranding suggestive of
inflammation Officially read as: ‘likely an appendiceal
mucocele’
Luise Pernar, HMS IVGillian Lieberman, MD
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Patient 2: LC
• 54 yo woman with history of breast cancer diagnosed in 2000; treated with L mastectomy, chest wall radiation therapy, tamoxifen
• Presented with complaints of abdominal distention and mild abdominal pain
• Paracentesis yielded 1.5L of ascites fluid with malignant cells
• CT scan was performed to determine possible source
Luise Pernar, HMS IVGillian Lieberman, MD
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Patient 2: LC - CT
PACS, BIDMC - Courtesy of Karen Lee, MD
Cecum
Terminal Ileum
Cyst (34 HU)
Rim-enhancement
Ascites fluid
Omental caking
Luise Pernar, HMS IVGillian Lieberman, MD
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Patient 2: LC - CT
• Findings summary:– Cystic structure adjacent to cecum and ileum;
appendix not seen separately – Density ~ 34HU– Suggestion of rim-enhancement of cystic wall– No surrounding stranding suggestive of
inflammation– Omental caking and ascites Officially read as: ‘could represent an
appendix mucocele’
Luise Pernar, HMS IVGillian Lieberman, MD
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Appendiceal Mucocele - Definition
• Appendiceal lesion characterized by – Appendiceal lumen dilation– Mucosal lining alteration– Hypersecretion of mucus– Potential for extension outside the appendix
This definition is problematic since it is descriptive and does not convey information about the primary underlying disease
Higa et.al. Cancer 1973
Luise Pernar, HMS IVGillian Lieberman, MD
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Appendiceal Mucocele - Definition• Histologically mucoceles can be divided into
– Mucosal hyperplasia (25%)– Mucinous cystadenoma (63%)– Mucinous cystadenocarcinoma (12%)– Retention cysts have also been described
• Malignancy of mucoceles has been variably defined by – Histologic type of epithelial cells in resected specimen– Dissection of the appendiceal wall by mucin– Presence of epithelial cells in mucin if there has been
egress into the peritoneal cavity
Lo and Sarr Hepatogastroenterology 2003; Higa et.al. Cancer 1973
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Incidence and Diagnosis• Frequency: 0.1% - 0.4% of all appendectomy specimens
show findings consistent with mucocele• F:M = 1.2-4:1• Age at diagnosis 50’s-60’s• 49% symptomatic
– Malignant appendiceal mucoceles more frequently become symptomatic
– Common presenting complaints are • abdominal pain (27%)• palpable abdominal mass (16-25%)• abdominal distention (14%)• weight loss (10%)
• Laboratory analysis may show elevated CEA, WBC, and ESR
Stocchi et.al. Arch Surg 2003; Lo and Sarr Hepatogastroenterology 2003; Blair et.al. Am J Surg 1993; Landen et.al. Surg Gynecol Obstet 1992
Luise Pernar, HMS IVGillian Lieberman, MD
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Differential Diagnosis• Intraperitoneal
– Other appendiceal neoplasm (lipoma, fibroma, neuroma, carcinoid, lymphoma)
– Appendicitis– Cyst (ovarian, mesenteric, omental)– Mesenteric hematoma or tumor– Abdominal abscess– Hydrosalpinx
• Retroperitoneal– Inflammation– Tumor– Hemorrhage
Horgan et.al. AJR 1984
Luise Pernar, HMS IVGillian Lieberman, MD
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Imaging of Mucoceles• Correct preoperative diagnosis is key for
appropriate surgical intervention (more on this later)
• Diagnostic imaging modalities used in preoperative diagnosis include– US– X-ray– Barium enema– Endoscopy– CT
Luise Pernar, HMS IVGillian Lieberman, MD
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Mucocele on US – Companion Patient 1
Trans-abdominal US showing:
• Elongated, unilocular cystic structure with internal echos
• Enhanced through- transmission suggested cyst is fluid-filled
• Indistinct cystic wall
Pickhardt et.al. RadioGraphics 2003
Luise Pernar, HMS IVGillian Lieberman, MD
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Mucocele on US – Companion Patient 2
Trans-abdominal US showing:
• Elongated, unilocular cystic mass (M) with internal echos
• No distinct cyst wall• No posterior or lateral
shadowing
Sasaki et.al. Abdom Imaging 2003
Luise Pernar, HMS IVGillian Lieberman, MD
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Mucocele on US – Companion Patient 3
Trans-abdominal US showing:
• Cystic mass with echogenic layers
‘onion-skin’ sign
Caspi et.al. J Ultrasound Med 2004
Luise Pernar, HMS IVGillian Lieberman, MD
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Mucocele on X-ray – Companion Patient 4
Coned-down plain film of RLQ showing:
• Round mass (Arrowheads)
• Curvy-linear calcifications (White arrows)
Pickhardt et.al. RadioGraphics 2003
Luise Pernar, HMS IVGillian Lieberman, MD
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Mucocele on X-ray – Companion Patient 5
Plain film of RLQ showing:
• Rounded mass suggested by wall cacifications (Arrows)
Higa et.al. Cancer 1973
Luise Pernar, HMS IVGillian Lieberman, MD
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Mucocele on Barium Enema – Companion Patient 6
Single contrast barium enema showing:
• Smooth, broad-based filling defect (Arrowhead) in the medial cecum adjacent to the ileocecal valve
Pickhardt et.al. RadioGraphics 2003
Luise Pernar, HMS IVGillian Lieberman, MD
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Mucocele on Barium Enema – Companion Patient 7
Air-barium double contrast enema showing:
• Smooth, submucosal filling defect (M) in the medial cecum
Pickhardt et.al. RadioGraphics 2003
Luise Pernar, HMS IVGillian Lieberman, MD
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Mucocele on Colonoscopy – Companion Patient 6
Colonoscopy, performed on patient 6 seen previously, showing:
• Bulbous, smooth submucosal lesion (M) protruding into the cecum
• Mass’s movement with respiration is thought classic for a mucocele
Pickhardt et.al. RadioGraphics 2003
Luise Pernar, HMS IVGillian Lieberman, MD
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Mucocele on Colonoscopy – Companion Patient 8
Colonoscopy showing:• Bulbous, smooth
submucosal lesion protruding into the cecum at the site of the appendiceal orfice
• Appendiceal orfice seen at the center of the mound is the ‘volcano’ sign considered classic for a mucocele
Zanati et.al. Gastrointest Endosc 2005
Luise Pernar, HMS IVGillian Lieberman, MD
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Mucocele on CT – Companion Patient 6
CT scan, of patient 6 seen previously, showing:• Cystic lesion (Arrowhead) adjacent to cecum extending into the
peritoneal cavity (Arrow)• Density range for mucoceles seen on CT ~ 10-45HU• Note absence of peri-appendiceal inflammation or abscess
Pickhardt et.al. RadioGraphics 2003
Luise Pernar, HMS IVGillian Lieberman, MD
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Mucocele on CT – Companion Patient 9
CT scan showing:• Low-density, well-
capsulated mass (Arrow) adjacent to the cecum in the expected location of the appendix
Sasaki et.al. Abdom Imaging 2003
Luise Pernar, HMS IVGillian Lieberman, MD
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Imaging of Mucoceles – Summary of Findings
• US – Elongated, unilocular cyst-like mass with internal echos; indistinct wall; ‘onion-skin’ sign may be pathognomonic
• X-ray – RLQ rounded mass with curvilinear calcification• Barium enema – Smooth, broad-based filling defect in
the cecum• Endoscopy – Bulbous, smooth, submucosal lesion
protruding into the cecum near site of the appendiceal orfice; ‘volcano sign’ and movement of the mass with respirations are considered classic for appendiceal mucocele
• CT – RLQ mass adjacent to the cecum with low- attenuating content (0-45HU) and wall calcification
Zanati et.al. Gastrointest Endosc 2005; Caspi et.al. J Ultrasound Med 2004; Pickhardt et.al. RadioGraphics 2003; Sasaki et.al. Abdom Imaging 2003; Higa et.al. Cancer 1973
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Why Pre-op Diagnosis?• Feared complication of appendiceal mucocele,
due to any cause, is PSEUDOMYXOMA PERITONEI
– Diffuse, gelatinous, cellular ascites– Origin thought to be
• Dissemination of mucinous cells from appendiceal mucocele due to rupture of appendix or metastatic spread OR
• Neoplastic transformation of peritoneum following mucinous metaplasia of mesothelium
– Often fatal without treatment as it causes intestinal obstruction
Hinson and Ambrose Br J Surg 1998; Prayson et.al. Am J Surg Pathol 1994
Luise Pernar, HMS IVGillian Lieberman, MD
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Pseudomyxoma Peritonei on US – Companion Patient 10
Rectal ultrasound showing:
• Thick, gelatinous fluid (F) in the pouch of Douglas
Khan et.al. Ultrasound Obstet Gynecol 2002
Luise Pernar, HMS IVGillian Lieberman, MD
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Pseudomyxoma Peritonei on CT – Companion Patient 11
CT scan showing:• Diffuse intraperitoneal
locules with mass effect on adjacent bowel
• Bowels do not float centrally
• Omental caking is present
Pickhardt et.al. RadioGraphics 2003
Luise Pernar, HMS IVGillian Lieberman, MD
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Pseudomyxoma Peritonei on CT – Companion Case 12
CT scan showing:• Scalopping of solid
organs by mucinous implants
• Septal calcifications in mucinous fluid (Arrowheads)
Pickhardt et.al. RadioGraphics 2003
Luise Pernar, HMS IVGillian Lieberman, MD
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• US – Thick gelatinous fluid; usually not mobile with maneuvers; fluid septations may be seen
• CT – Increased abdominal girth; diffuse intraperitoneal locules; mass-effect and distortion of bowel; scalloping of surfaces of solid organs
Imaging of Pseudomyxoma Peritonei – Summary of Findings
Pickhardt et.al. RadioGraphics 2003; Khan et.al. Ultrasound Obstet Gynecol 2002; Dachman et.al. AJR 1985
Luise Pernar, HMS IVGillian Lieberman, MD
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Treatment
Dhage-Ivatury and Sugarbaker J Am Coll Surg 2006; Witkamp et.al. Br J Surg 2001
Mucocele Diagnosed Pre-operatively?
Perform laparotomy•To prevent mucocele rupture•To allow thorough examination of peritoneal cavity for mucinous fluid
If employing laparoscopic approach, convert to laparotomy
• Appendectomy typically sufficient• Proceed to right hemicolectomy if
•Appendiceal or ileocecal lymph nodesare positive
•Resection margin is positive
• If no fluid is present workup is complete• If fluid is present
• Harvest all mucinous fluid• Submit to pathology for examination
for epithelial cells
If epithelial cells are present• Diagnose pseudomyxoma peritonei• Refer patient for
•debulking surgery (complete resection of gelatinous masses, greater omentum, major viscera, as appropriate)
•Intraperitoneal chemotherapy (mitomycin C +/- 5-fluorouracil)
Yes No
Luise Pernar, HMS IVGillian Lieberman, MD
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Outcomes• 91-100% survival after resection of mucocele
due to mucosal hyperplasia, mucinous cystadenoma, and mucinous cystadenocarcinoma if not complicated by pseudomyxoma peritonei
• 25-33% survival in presence of pseudomyxoma peritonei
• Debulking surgery with intraperitoneal chemotherapy yields 3 year survival between 61-86% with an associated 35% risk of morbidity including bowel perforation, fistula formation and anastomotic leak
Dhage-Ivatury and Sugarbaker J Am Coll Surg 2006; Witkamp et.al. Br J Surg 2001; Sugarbaker and Jablonski Ann Surg 1995; Landen et.al. Surg Gynecol Obstet 1992; Higa et.al. Cancer 1973
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Summary
• Appendiceal mucoceles are rare and are often found incidentally; incorrect intraoperative handling may lead to major complications
• Suggestive and characteristic imaging findings can help establish the pre-operative diagnosis of mucoceles highlighting the role radiologists play in pre-operative planning and in ensuring good patient outcomes
Luise Pernar, HMS IVGillian Lieberman, MD
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Bibliography• Blair NP; Bugis SP; Turner LJ; MacLeod MM. Review of the pathologic diagnoses of 2,216 appendectomy
specimens. Am J Surg (1993) 165: 618-620.• Caspi B; Cassif E; Auslender R; Herman A; Hagay Z; Appelman Z. The onion skin sign; a specific sonographic
marker of appendiceal mucocele. J Ultrasound Med (2003) 23: 117-121.• Dachman AH; Lichtenstein JE; Friedman AC. Mucocele of the appenidx and pseudomyxoma peritonei. AJR
(1985) 144: 923-929.• Dhage-Ivatury S; Sugarbaker PH. Update on the surgical approach to mucocele of the appendix. J AM Coll Surg
(2006) 202: 680-684.• Higa E; Rosai J; Pizzimbono CA; Wise L. Mucosal hyperplasia, mucinous cystadenoma, and mucinous
cystadenocarcinoma of the appendix; a re-evaluation of the appendiceal mucocele. Cancer (1973) 32: 1525-1541.• Hinson FL; Ambrose NS. Pseudomyxoma peritonei. Br J Surg (1998) 85: 1332-1339.• Horgan JG; Chow PP; Richter JO; Rosenfield AT; Taylor KJW. CT and sonography in the recognition of mucocele
of the appendix. AJR (1984) 143: 959-962.• Khan S; Patel AG; Jurkovic D. Incidental ultrasound diagnosis of pseudomyxoma peritonei in an asymptomatic
woman. Ultrasound Obstet Gynecol (2002) 19: 410-412.• Landen S; Bertrand C; Maddern GJ; Herman D; Pourbaix A; deNeve A; Schmitz A. Appendiceal mucolceles and
pseudomyxoma peritonei. Surg Gynecol Obstet (1992) 175: 401-404.• Lo NS; Sarr MG. Mucinous cystadenocarcinoma of the appendix; the controversy persists: a review.
Hepatogastroenterology (2003) 50: 432-437.• Pickhardt PJ; Levy AD; Rohrmann CA; Kende AI. Primary neoplasms of the appendix: radiologic spectrum of
disease with pathologic correlation. RadioGraphics (2003) 23: 645-662.• Prayson RA; Hart WR; Petras RE. Pseudomyxoma peritonei; a clinicopathologic stduy of 19 cases with emphasis
on site of origin and nature of associated ovarian tumors. Am J Surg Pathol (1994) 18: 591-603.• Sasaki K; Komatsuda T; Suzuki T; Konno K; Ohtaka M; Sato M; Ishida J; Sakai T; Watanabe S. Appendiceal
mucocele: sonographic findings. Abdom Imaging (2003) 28: 15-18. • Stocchi L; Wolff BR; Larson DR; Harrington JR. Surgical treatment of appendiceal mucocele. Arch Surg (2003)
138: 585-590.• Sugarbaker PH; Jablonski KA. Prognostic features of 51 colorectal and 130 appendiceal cancer patients with
peritoneal carcinomatosis treated by cytoreductive surgery and intraperitoneal chemotherapy. Ann Surg (1995) 221: 124-132.
• Witkamp AJ; de Bree E; Kaar MM; van Slooten GW; van Doevorden F; Zoetmulder FAN. Extensive surgical cytoreduction and intraoperative hyperthermic intraperitoneal chemotherpay in patients with pseudomyxoma peritonei. Br J Surg (2001) 88: 458-463.
• Zanati SA; Martin JA; Baker JP; Streutker CJ; Marcon NE. Colonoscopic diagnosis of the mucocele of the appendix. Gastrointest Endosc (2005) 62: 452-456.
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Acknowledgement
Thank you:• Karen Lee, MD – for giving me great
cases• Gillian Lieberman, MD – for directing and
teaching a rotation I wish I had taken earlier
• Pamela Lebkowski – for outstanding support and organization
• Larry Barbaras