阑尾巨细胞型粘液腺癌影像学表现

Dylan Carney, HMS III Gillian Lieberman, MD Giant Mucinous Adenocarcinoma of the Appendix with disseminated peritoneal adenomucinosis Dylan Carney, Harvard Medical School Year III Gillian Lieberman, MD January 2011 Dylan Carney, HMS III Gillian Lieberman, MD Overview • Case presentation – Patient presentation – Radiographic findings – Surgical procedure – Pathology findings • Neoplasms of the appendix – Histologic types – Menu of radiographic tests – Treatment and outcomes • Pseudomyxoma peritonei – Radiographic findings 2 Dylan Carney, HMS III Gillian Lieberman, MD Case Presentation • Our patient is a previously-healthy 48 y.o. male who presents with a 4-day history of focal, intermittent RLQ abdominal pain that is non-radiating • No nausea, vomiting, fever, chills, melena, hematochezia, hematemesis, association with PO intake • Physical notable for normal vital signs, normal bowel sounds, guarding in RLQ, tenderness to palpation with palpable mass in RLQ • Labs notable for leukocytosis of 13.9 3 Dylan Carney, HMS III Gillian Lieberman, MD The patient goes for urgent CT abdomen with and without contrast… 4 Dylan Carney, HMS III Gillian Lieberman, MD Our patient’s CT abdomen: what do you see? 5 PACS, BIDMC Coronal CT + C Dylan Carney, HMS III Gillian Lieberman, MD Out pt: appendiceal mass 6 Cecum is displaced  medially, superiorly,  and rotated Extension of fluid  into inguinal canal Large bowel is  constrast‐filled  throughout with air  distal to lesion, and is  of normal to mildly‐ dilated caliber Large, non‐enhancing  cystic mass with rim  of enhancement  extending from  appendix – indicating  pathology is likely  appendiceal in origin PACS, BIDMC Coronal CT + C Dylan Carney, HMS III Gillian Lieberman, MD What do you see in this coronal section? 7 PACS, BIDMC Coronal CT + C Dylan Carney, HMS III Gillian Lieberman, MD Out pt: mucinous neoplasm 8 Cecum adjacent to  cyst Large bowel with  contrast and air  throughout Cystic mass is 25HU,  denser than simple  fluid and less dense  than blood – consistent with  mucous. It is also  multi‐lobed PACS, BIDMC Coronal CT + C Dylan Carney, HMS III Gillian Lieberman, MD What do you see in this axial CT section? 9 PACS, BIDMC Axial CT + C Dylan Carney, HMS III Gillian Lieberman, MD Out pt: rim calcifications and free mucus 10 Cystic mass with rim  of enhancement and  calcifications. This  portion of the mass is  intraperitoneal,  anterior the the anterior perirenal space, medial to the  liver and lateral to  the ascending colon Free mucus in the  peritoneal cavity  consistent with  rupture Note the absence of  frank ascites or  psuedomyxoma peritonei “jelly  belly”, vasculature  non‐compressed PACS, BIDMC Axial CT + C Dylan Carney, HMS III Gillian Lieberman, MD What spaces is the pathology in? 11 PACS, BIDMC Axial CT + C Dylan Carney, HMS III Gillian Lieberman, MD Our pt: retroperitoneal extension 12 Free mucus in the  peritoneal cavity  consistent with  rupture Multi‐lobed cystic  mass with rim of  enhancement and  pockets of air, indicating rupture  into intestinal lumen  (v. emphysematous  infection). Note that  the posterior lobe of  the mass is in the  anterior perirenal space, adjacent to  the psoas. PACS, BIDMC Dylan Carney, HMS III Gillian Lieberman, MD How is the mass different on this lesion? 13 PACS, BIDMC Axial CT + C Dylan Carney, HMS III Gillian Lieberman, MD Our pt: heterogeneous mucocele 14 Mucocele displays  heterogeneity at this  level. The lobes here  are also fused and  invading the  retroperitoneum.  Thus, the mass is  singular and  continuous. The mucocele is now  directly abutting the  psoas. PACS, BIDMC Axial CT + C Dylan Carney, HMS III Gillian Lieberman, MD Differential Diagnosis • At this point it is narrow, and includes: – Appendiceal neoplasm with or without pseudomyxoma peritonei – Appendicitis with abscess 15 Dylan Carney, HMS III Gillian Lieberman, MD The patient is brought to the OR for tumor debulking. Let’s look at the intraoperative findings… 16 Dylan Carney, HMS III Gillian Lieberman, MD Our pt: visceral mucosal implants 17 Mucosal Implants Courtesy of Dr. James Hurst, Acute Care Surgery Dylan Carney, HMS III Gillian Lieberman, MD Our pt: resection cavity 18 Resection cavity Courtesy of Dr. James Hurst, Acute Care Surgery Dylan Carney, HMS III Gillian Lieberman, MD 19 Ascending Colon Mucocele Courtesy of Dr. James Hurst, Acute Care Surgery Gross Specimen: mucocele removed en bloc with ascending colon and transverse colon Dylan Carney, HMS III Gillian Lieberman, MD Mucocele, ileum, ascending, transverse colon and mucosal implants are sent to pathology. Let’s see the findings… 20 Dylan Carney, HMS III Gillian Lieberman, MD Histology: infiltrating glandular tissue Infiltrating glandular  tissue Courtesy of Dr. Robert Najarian and Dr. Shu‐Hsien Sheu, BIDMC Pathology 21 Dylan Carney, HMS III Gillian Lieberman, MD Histology: neoplastic epithelium Neoplastic epithelium with enlarged,  hyperchromatic nuclei, numerous mitotic  figures, abundant intraluminal mucin Courtesy of Dr. Robert Najarian and Dr. Shu‐Hsien Sheu, BIDMC Pathology 22 Dylan Carney, HMS III Gillian Lieberman, MD Histology: Pools of Mucin Pools of mucin outside lumen Courtesy of Dr. Robert Najarian and Dr. Shu‐Hsien Sheu, BIDMC Pathology 23 Dylan Carney, HMS III Gillian Lieberman, MD Pathology Report: Mucinous Adenocarcinoma • Well-differentiated mucinous adenocarcinoma of the appendix • 0 of 18 lymph nodes involved • Ileum through transverse colon normal with clear margins • Acellular mucosal studding along serosal surface consistent with disseminated peritoneal adenomucinosis 24BIDMC Pathology Dylan Carney, HMS III Gillian Lieberman, MD The patient recovered well post-operatively and was discharged on hospital day #6. He was seen in clinic 2 weeks later and reported resolution of symptoms with minimal residual pain. 25 Dylan Carney, HMS III Gillian Lieberman, MD Let’s learn more about neoplasms of the appendix… 26 Dylan Carney, HMS III Gillian Lieberman, MD Appendiceal Neoplasms • Common presentations: abdominal pain, appendicitis, ascites, obstruction • Incidental finding on 1% of appy specimens • Overall incidental 0.12 cases / 1M pt-years • Diagnosis – Contrast-enhanced CT (gold standard) – shows non-enhancing, mucous-filled, rim-enhancing lesion of appendix (because most patients present with appendicitis, this is usually the test of choice) – Plain films – may see air-fluid level if there is rupture, or mass effect on nearby organs – Barium enema – cecal impaction, non-filling of appendix – U/S – layering (“onion skin”), nodular mural thickening indicates malignancy (indicated in children, pregnant women) 27 Cerame MA. A 25‐year review of adenocarcinoma of the appendix. A frequently perforating carcinoma. Dis Colon Rectum 1988;31:145‐50. Connor SJ, Hanna GB, Frizelle FA. Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7,970 appendectomies. Dis Colon Rectum 1998;41:75‐80. Ito H, Osteen RT, Bleday R, Zinner MJ, Ashley SW, Whang EE. Appendiceal adenocarcinoma: long‐term outcomes after surgical therapy. Dis Colon Rectum 2004;47:474‐80.  Dylan Carney, HMS III Gillian Lieberman, MD CT Appearance of Appendiceal Mucoceles Compared to Acute Appendicitis • Findings in appendicitis and small appendiceal mucoceles often overlap overlap, making preoperative diagnosis of mucoceles tricky • CT features found to be associated with mucoceles – Cystic dilation of the appendix – Mural calcification – Luminal diameter > 1.3cm (71% sensitive and 95% specific) • Non-specific CT findings: appendicolith, fat stranding, periappendiceal fluid, lymphadenopathy 28Bennett GL, Tanpitukpongse TP, Macari M, Cho KC, Babb JS. CT diagnosis of mucocele of the appendix in patients with acute appendicitis. AJR Am J Roentgenol 2009;192:W103‐10.  Dylan Carney, HMS III Gillian Lieberman, MD CT Findings: Appendicitis with and without Mucocele 29Bennett GL, Tanpitukpongse TP, Macari M, Cho KC, Babb JS. CT diagnosis of mucocele of the appendix in patients with acute appendicitis. AJR Am J Roentgenol 2009;192:W103‐10.  Dylan Carney, HMS III Gillian Lieberman, MD Let’s see some other examples of appendiceal mucoceles 30 Dylan Carney, HMS III Gillian Lieberman, MD Companion Patient 1: Mucocele with Air and Mural Calcifications 31Bennett GL, Tanpitukpongse TP, Macari M, Cho KC, Babb JS. CT diagnosis of mucocele of the appendix in patients with acute appendicitis. AJR Am J Roentgenol 2009;192:W103‐10.  Air Calcifications Dylan Carney, HMS III Gillian Lieberman, MD Companion Patient 2: Mural Calcifications 32Bennett GL, Tanpitukpongse TP, Macari M, Cho KC, Babb JS. CT diagnosis of mucocele of the appendix in patients with acute appendicitis. AJR Am J Roentgenol 2009;192:W103‐10.  Calcifications Dylan Carney, HMS III Gillian Lieberman, MD Companion Patient 3: Mural Calcifications and Fat Stranding 33Bennett GL, Tanpitukpongse TP, Macari M, Cho KC, Babb JS. CT diagnosis of mucocele of the appendix in patients with acute appendicitis. AJR Am J Roentgenol 2009;192:W103‐10.  Fat Stranding Calcifications Dylan Carney, HMS III Gillian Lieberman, MD Companion Patient 4: Fat Stranding 34Bennett GL, Tanpitukpongse TP, Macari M, Cho KC, Babb JS. CT diagnosis of mucocele of the appendix in patients with acute appendicitis. AJR Am J Roentgenol 2009;192:W103‐10.  Fat Stranding Dylan Carney, HMS III Gillian Lieberman, MD Companion Patient 5: Fat Stranding 35Bennett GL, Tanpitukpongse TP, Macari M, Cho KC, Babb JS. CT diagnosis of mucocele of the appendix in patients with acute appendicitis. AJR Am J Roentgenol 2009;192:W103‐10.  Fat Stranding Dylan Carney, HMS III Gillian Lieberman, MD Bennett GL, Tanpitukpongse TP, Macari M, Cho KC, Babb JS. CT diagnosis of mucocele of the appendix in patients with acute appendicitis. AJR Am J Roentgenol 2009;192:W103‐10.  Companion Patient 5: Appendicolith 36 Appendicolith Dylan Carney, HMS III Gillian Lieberman, MD Bennett GL, Tanpitukpongse TP, Macari M, Cho KC, Babb JS. CT diagnosis of mucocele of the appendix in patients with acute appendicitis. AJR Am J Roentgenol 2009;192:W103‐10.  Companion Patients 5: Appendicitis! 37 These last two are actually examples of  acute appendicitis with appendicolith – there is no mucocele! Dylan Carney, HMS III Gillian Lieberman, MD Appendiceal Neoplasms: Histologic Types • Histologic types – Carcinoid – Epithelial (appendiceal mucoceles) • Mucosal hyperplasia (20%) • Simple cyst (18%) • Mucinous adenoma (10%) • Mucinous adenocarcinoma (52%) – Other (Signet Ring, lymphoma) 38 Higa E, Rosai J, Pizzimbono CA, Wise L. Mucosal hyperplasia, mucinous cystadenoma, and mucinous cystadenocarcinoma of the appendix. A re‐evaluation of appendiceal "mucocele". Cancer 1973;32:1525‐41. Isaacs KL, Warshauer DM. Mucocele of the appendix: computed tomographic, endoscopic, and pathologic correlation. Am J Gastroenterol. 1992;87(6):787‐9.  Landen S, Bertrand C, Maddern GJ, et al. Appendiceal mucoceles and pseudomyxoma peritonei. Surg Gynecol Obstet 1992;175:401‐4. Qizilbash AH. Mucoceles of the appendix. Their relationship to hyperplastic polyps, mucinous cystadenomas, and cystadenocarcinomas. Arch Pathol. 1975;99(10):548‐55.  Dylan Carney, HMS III Gillian Lieberman, MD Complications of Appendiceal Neoplasms • “Mucocele” encompasses any mucinous epithelial neoplasm of the appendix, from benign to malignant • Complications – Bowel obstruction – Perforation – Infection – Pseudomyxoma Peritonei (PMP) • Disseminated peritoneal mucinous tumors arising from perforation of true appendiceal neoplasms (adenoma or adenocarcinoma) and subsequent omental/peritoneal implantation of well-differentiated mucin-secreting epithelium • Implantation occurs in a gravity-dependent fashion: the pouch of Douglas, retrohepatic space, paracolic gutters, and the fossa created by ligament of Trietz 39 Sugarbaker PH, Ronnett BM, Archer A, Averbach AM, Bland R, Chang D, Dalton RR, Ettinghausen SE, Jacquet P, Jelinek J, Koslowe P, Kurman RJ, Shmookler B, Stephens AD, Steves MA, Stuart OA, White S, Zahn CM,  Zoetmulder FA. Pseudomyxoma peritonei syndrome. Adv Surg. 1996;30:233‐80. Dylan Carney, HMS III Gillian Lieberman, MD Appendiceal Mucocele: Treatment and Outcomes • Debate over appendectomy versus hemicolectomy, with trend towards hemicolectomy • Andersson et al, 1976 – Of 7 cases of mucinous adenocarcinoma, 6 were treated with appendectomy alone, 5 of whom survived beyond 5 years • Edmonds et al, 1984 – Of 10 cases, 5-year survival was significant longer with right hemicolectomy versus appendectomy (60% versus 20%) • Nitechki et al, 1994 – Of 94 cases, 5-year survival with right hemicolectomy was 68% versus 20% with appendectomy alone. – Secondary right hemicolectomy led to tumor downstaging and improved outcomes 40 Andersson A, Bergdahl L, Boquist L. Primary carcinoma of the appendix. Ann Surg 1976;183:53‐7. Edmonds P, Merino MJ, LiVolsi VA, Duray PH. Adenocarcinoid (mucinous carcinoid) of the appendix. Gastroenterology 1984;86:302‐9. Nitecki SS, Wolff BG, Schlinkert R, Sarr MG. The natural history of surgically treated primary adenocarcinoma of the appendix. Ann Surg 1994;219:51‐7.  Dylan Carney, HMS III Gillian Lieberman, MD What about pseudomyxoma peritonei? Let’s see some examples… 41 Dylan Carney, HMS III Gillian Lieberman, MD Companion Patient 6: Pseudomyxoma Peritonei • Non-contrast CT of the abdomen showing large cavity with an air fluid level (indicating frank rupture), compressing the small and large bowel 42Naidu LS, Jobling JC. Chronic colonic perforation in pseudomyxoma peritonei. Br J Radiol 2007;80:e94‐7.  Dylan Carney, HMS III Gillian Lieberman, MD Companion Patient 7: Pseudomyxoma Peritonei • Contrast-enhanced CT showing abundant intraperitoneal mucin with non-enhancing, fluid-filled cysts scalloping the liver and spleen 43Naidu LS, Jobling JC. Chronic colonic perforation in pseudomyxoma peritonei. Br J Radiol 2007;80:e94‐7.  Dylan Carney, HMS III Gillian Lieberman, MD Pseudomyxoma Peritonei - Treatment • Systemic and intraperitoneal chemotherapy • Debulking • Peritonectomy • Outcomes – typically poor – 25% 5-year survival – Mortality frequently secondary to intestinal or renal obstruction 44Landen S, Bertrand C, Maddern GJ, et al. Appendiceal mucoceles and pseudomyxoma peritonei. Surg Gynecol Obstet 1992;175:401‐4.   Dylan Carney, HMS III Gillian Lieberman, MD Summary • Appendiceal tumors are rare gastrointestinal neoplasms • Histological subtypes include carcinoid, epithelial (hyperplasia, cystic, mucinous adenoma and mucinous adenocarcinoma), signet ring and lymphomas • Patients frequently mimic acute appendicitis and tumors are frequently discovered on CT, intraoperatively, or via postoperative pathology • Complete debulking, usually with right hemicolectomy, should be performed with care to avoid rupture • Rupture of adenomas and adenocarcinoma can lead to peritoneal implants and subseuqent pseudomyxoma peritonei (PMP) • PMP is treated with debulking, intraperitoneal chemotherapy or peritonectomy, and overall 5-year prognosis is poor 45 Dylan Carney, HMS III Gillian Lieberman, MD Bibliography • Andersson A, Bergdahl L, Boquist L. Primary carcinoma of the appendix. Ann Surg 1976;183:53-7. • Bennett GL, Tanpitukpongse TP, Macari M, Cho KC, Babb JS. CT diagnosis of mucocele of the appendix in patients with acute appendicitis. AJR Am J Roentgenol 2009;192:W103-10. • Cerame MA. A 25-year review of adenocarcinoma of the appendix. A frequently perforating carcinoma. Dis Colon Rectum 1988;31:145-50. • Connor SJ, Hanna GB, Frizelle FA. Appendiceal tumors: retrospective clinicopathologic analysis of appendiceal tumors from 7,970 appendectomies. Dis Colon Rectum 1998;41:75-80. • Edmonds P, Merino MJ, LiVolsi VA, Duray PH. Adenocarcinoid (mucinous carcinoid) of the appendix. Gastroenterology 1984;86:302-9. • Higa E, Rosai J, Pizzimbono CA, Wise L. Mucosal hyperplasia, mucinous cystadenoma, and mucinous cystadenocarcinoma of the appendix. A re-evaluation of appendiceal "mucocele". Cancer 1973;32:1525-41. • Isaacs KL, Warshauer DM. Mucocele of the appendix: computed tomographic, endoscopic, and pathologic correlation. Am J Gastroenterol. 1992;87(6):787-9. • Ito H, Osteen RT, Bleday R, Zinner MJ, Ashley SW, Whang EE. Appendiceal adenocarcinoma: long-term outcomes after surgical therapy. Dis Colon Rectum 2004;47:474-80. • Landen S, Bertrand C, Maddern GJ, et al. Appendiceal mucoceles and pseudomyxoma peritonei. Surg Gynecol Obstet 1992;175:401-4. • McCusker ME, Cote TR, Clegg LX, Sobin LH. Primary malignant neoplasms of the appendix: a population-based study from the surveillance, epidemiology and end-results program, 1973-1998. Cancer 2002;94:3307-12. • Naidu LS, Jobling JC. Chronic colonic perforation in pseudomyxoma peritonei. Br J Radiol 2007;80:e94-7. • Nitecki SS, Wolff BG, Schlinkert R, Sarr MG. The natural history of surgically treated primary adenocarcinoma of the appendix. Ann Surg 1994;219:51-7. • Qizilbash AH. Mucoceles of the appendix. Their relationship to hyperplastic polyps, mucinous cystadenomas, and cystadenocarcinomas. Arch Pathol. 1975;99(10):548-55. • Sugarbaker PH, Ronnett BM, Archer A, Averbach AM, Bland R, Chang D, Dalton RR, Ettinghausen SE, Jacquet P, Jelinek J, Koslowe P, Kurman RJ, Shmookler B, Stephens AD, Steves MA, Stuart OA, White S, Zahn CM, Zoetmulder FA. Pseudomyxoma peritonei syndrome. Adv Surg. 1996;30:233-80. • Sugarbaker PH. The natural history, gross pathology, and histopathology of appendiceal epithelial neoplasms. Eur J Surg Oncol 2006;32:644-7. • Tsai CJ. Ultrasound features of disseminated adenomucinosis (pseudomyxoma). Br J Radiol 1998;71:564-6. 46 Dylan Carney, HMS III Gillian Lieberman, MD Acknowledgements 47 Giant Mucinous Adenocarcinoma of the Appendix�with disseminated peritoneal adenomucinosis Overview Case Presentation The patient goes for urgent CT abdomen with and without contrast… Our patient’s CT abdomen: what do you