阑尾粘液囊肿影像学诊断

Luise Pernar, HMS IV Gillian Lieberman, MD Appendiceal Mucoceles Luise Pernar, Harvard Medical School IV Gillian Lieberman, MD September 2006 Luise Pernar, HMS IV Gillian Lieberman, MD 2 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 IV Gillian Lieberman, MD 3 Patient 1: JH - CT PACS, BIDMC - Courtesy of Karen Lee, MD Cecum Terminal Ileum Cyst (27 HU) Focal wall calcification Luise Pernar, HMS IV Gillian Lieberman, MD 4 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 IV Gillian Lieberman, MD 5 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 IV Gillian Lieberman, MD 6 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 IV Gillian Lieberman, MD 7 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 IV Gillian Lieberman, MD 8 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 IV Gillian Lieberman, MD 9 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 Luise Pernar, HMS IV Gillian Lieberman, MD 10 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 IV Gillian Lieberman, MD 11 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 IV Gillian Lieberman, MD 12 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 IV Gillian Lieberman, MD 13 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 IV Gillian Lieberman, MD 14 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 IV Gillian Lieberman, MD 15 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 IV Gillian Lieberman, MD 16 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 IV Gillian Lieberman, MD 17 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 IV Gillian Lieberman, MD 18 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 IV Gillian Lieberman, MD 19 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 IV Gillian Lieberman, MD 20 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 IV Gillian Lieberman, MD 21 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 IV Gillian Lieberman, MD 22 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 IV Gillian Lieberman, MD 23 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 IV Gillian Lieberman, MD 24 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 Luise Pernar, HMS IV Gillian Lieberman, MD 25 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 IV Gillian Lieberman, MD 26 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 IV Gillian Lieberman, MD 27 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 IV Gillian Lieberman, MD 28 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 IV Gillian Lieberman, MD 29 • 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 IV Gillian Lieberman, MD 30 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 nodes are 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 IV Gillian Lieberman, MD 31 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 Luise Pernar, HMS IV Gillian Lieberman, MD 32 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 IV Gillian Lieberman, MD 33 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. Luise Pernar, HMS IV Gillian Lieberman, MD 34 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 Appendiceal Mucoceles Patient 1: JH Patient 1: JH - CT Patient 1: JH - CT Patient 2: LC Patient 2: LC - CT Patient 2: LC - CT Appendiceal Mucocele - Definition Appendiceal Mucocele - Definition Incidence and Diagnosis Differential Diagnosis Imaging of Mucoceles Mucocele on US – Companion Patient 1 Mucocele on US – Companion Patient 2 Mucocele on US – Companion Patient 3 Mucocele on X-ray – Companion Patient 4 Mucocele on X-ray – Companion Patient 5 Mucocele on Barium Enema – Companion Patient 6 Mucocele on Barium Enema – Companion Patient 7 Mucocele on Colonoscopy – Companion Patient 6 Mucocele on Colonoscopy – Companion Patient 8 Mucocele on CT – Companion Patient 6 Mucocele on CT – Companion Patient 9 Imaging of Mucoceles – �Summary of Findings Why Pre-op Diagnosis? Pseudomyxoma Peritonei on US – Companion Patient 10 Pseudomyxoma Peritonei on CT – Companion Patient 11 Pseudomyxoma Peritonei on CT – Companion Case 12 Imaging of Pseudomyxoma Peritonei – �Summary of Findings Treatment Outcomes Summary Bibliography Acknowledgement