胰腺实性假乳头状瘤课件

胰腺实性假乳头状瘤 Fudan Cancer Center 陈其文 Solid-Pseudopapillary Tumor of Pancreas(SPTP) Introduction 1.Franz described this tumor in 1959 as a “papillary tumor of the pancreas, benign or malignant” 2.1996,WHO in its classification of exocrine pancreatic tumors, recognized them as “solid pseudopapillary tumors” of the pancreas. 3. Low potentially malignant, about 10% have malignant behavior 4. Capsule intact or not has a relation to the malignant behavior and recurrence after surgery Franz VK. Tumors of the pancreas. In: Atlas of tumor pathology: fasc 2728, ser 7. Washington, DC: Armed Forces Institute of Pathology, 1959:3233 Papavramidis T, Papavramidis S. Solid pseudopapillary tumors of the pancreas: review of 718 patients reported in English literature. J Am Coll Surg, 2005,200(6):965-972 1.Unusual primary tumor of the pancreas account for 9% of the cystic tumor of pancreas and 1%- 2.25% of the exocrine pancreatic tumors 2.With a low potential for malignancy 3. Unknown cell origin 4.Seen mostly in young women, may be accelerated by pregnancy the male to female ratio is 1:9.5 . Klimstra DS, Wenig BM, Heffess CS. Solidpseudopapillary tumor of the pancreas: a typically cystic carcinoma of low malignant potential. Semin Diagn Pathol 2000; 17:6680 Features Age distribution of the patients with SPTP Distribution of Patients According to Tumor Diameter TUMOR SITES Pancreatic Head :39.8% Tail :24.1% Body and Tail :19.5% Body :11.2% Neck :3.6% Extrapancreatic SPT was found in 1.8% patients Metastasis and Invasion Metastasis: Liver metastasis is seen in a few cases, and rare cases of lymph node metastasis, peritoneal spread, and multiplicity have been reported Extracapsular Invasion: SPTP infrequently invades a capsule and the surrounding structures, mainly the spleen, portal vein, and duodenum. Of 553 patients, 45 (8.1%) were diagnosed as malignant SPTP Involved Organs: Portal Vein/Spleenic Vein/Superior Mesenteric Vein:11 Omentum or Peritoneum:7 Duodenum:7 Liver :6 Spleen:4 Stomach:3 Colon:2 Diaphragmatic Muscle:1 Left Kidney :1 Peng FY, Zhen HH, Xin BW, et al. Solid pseudopapillary tumor od the pancreas: A review of 553 cases in Chinese literature. World Journal of Gastroenterology. 2010,16(10):1209- 1214. Typical and Atypical SPTP Typical SPTP: characterized by a well-encapsulated mass with varying amounts of intratumoral hemorrhage Atypical SPTP: metastasis ductal obstruction parenchymal and extracapsular invasion intratumoral calcification occurrence in a male patient. Symptoms of SPTP Patients in Chinese Literature in English Literature Pathology Gross appearance Pathologic examination reveals that solid pseudopapillary tumor is usually a large, encapsulated mass composed of a mixture of cystic, solid, and hemorrhagic components. Histologic Appearance Immunohistochemical parameters of SPTP Tumor markers AFP/CEA/CA199/CA125/CA242 1.Mostly within the normal level 2.Very few increase, just slightly 3.The increased level of tumor makers does not mean the tumor is malignant or bad prognosis Ultrasound Ultrasonographically, the tumors are well-defined hypoechoic solid masses, solid masses containing cystic areas, cystic mass, and semicircularly calcified lesions. Features on CT 1.Both a capsule and intratumoral hemorrhage are important clues to the diagnosis because these features are rarely found in other pancreatic neoplasms 2.Majority are round or oval shaped.The lesion can be totally solid, solid cystic and totally cystic.And solid cystic consists the most of the lesion Cantisani V, Mortele KJ, Levy A, et al. MR imaging features of solid pseudopapillary tumor of the pancreas in adult and pediatric patients. AJR 2003;181:395401 Features on CT 3. Smaller solid pseudopapillary tumors are less sharply circumscribed and often appear unencapsulated. Cantisani V, Mortele KJ, Levy A, et al. MR imaging features of solid pseudopapillary tumor of the pancreas in adult and pediatric patients. AJR 2003;181:395401 44-year-old woman with pancreatic mass found on sonography. A, Radiograph shows curvilinear calcification (arrow) in left upper quadrant of abdomen. B, Transverse sonogram shows dense peripheral rim calcification with posterior acoustic shadowing (arrow). The classic CT features of solid pseudopapillary tumor are a large well-encapsulated mass with varying solid and cystic components caused by hemorrhagic degeneration 19-year-old woman with palpable abdominal mass of solid pseudopapillary tumor. Contrast-enhanced CT scan shows wellencapsulated heterogeneous mass(arrow) in tail of pancreas. Metastasis occurs in a small number of cases, the most common site being the liver. Choi BI, Kim KW, Han MC, Kim YI, Kim CW. Solid and papillary epithelial neoplasms of the pancreas:CT findings. Radiology 1988; 166:413416 B, CT scan shows cystic mass (arrow) with focal solid portion in liver. Several features, such as old age, invasion into a capsule or adjacent normal pancreatic parenchyma, and both vascular and perineural invasion, are associated with an increase in potential for Malignancy 42-year-old woman with pancreatic mass found incidentally on imaging. A, Axial CT scan of pancreas shows encapsulated mass with peripheral calcification and focal extracapsular extension (arrow). B, T1-weighted gradient-echo image of pancreas shows heterogeneous mass (arrow). 42-year-old woman with pancreatic mass found incidentally on imaging. Photograph of gross pathologic specimen shows focal pericapsular extension (arrow). 58-year-old woman with pancreatic mass on sonography. Coronal reformatted CT scan shows dense, thick, peripheral calcification (arrow). 44-year-old woman with pancreatic mass found on sonography. Unenhanced CT scan shows peripheral curvilinear calcification (thin arrow) and internal hemorrhage (wide arrow). Smaller solid pseudopapillary tumors are less sharply circumscribed and often appear unencapsulated. Fine Needle Aspiration 1.Percutaneous fine-needle cytology of the cystic wall can be useful and can influence subsequent management. 2. Endoscopic ultrasonography scan with fine- needle aspiration biopsy becomes more popular, and can be useful in identifying the tumor. 赵玉沛，等. 胰腺实性假乳头状瘤的诊断和治疗. 中华消化外科杂志，2008，7（6）： 411. Treatment Surgery is the only curative way to treat SPTP. 97.3% of the patients can receive surgery. 96.4% patients have disease free survival after surgery Patients of Metastasis or Invasion may NOT avoid surgery 1.The presence of metastasis in the SPT patients is NOT a contraindication for surgery。In these cases with metastasis, complete resection of both primary tumor and metastasis lesions, if possible, is proposed. 2. Invasion to the portal vein or superior mesenteric artery should NOT be included as a criterion for nonresectability of these pancreatic neoplasms. Principles of Surgery 1.Extensive lymphatic dissection or more radical local approaches are not indicated. 2.For the metastases, there is also general consensus that surgical debulking should be performed. 3.Specifically, when liver metastases were present, operative excisions were used in most patients Shimizu M, Matsumoto T, Hirokawa M, et al. Solidpseudopapillary carcinoma of the pancreas. Pathol Int 1999;49:231234. Matsunou H, Konishi F. Papillary-cystic neoplasm of the pancreas: a clinicopathologic study concerning the tumor aging andmalignancy of nine cases. Cancer 1990;65:283291. Operations Used in Management of SPTP Treatments for “nonsurgical” candidates 1.Chemotherapy based on Gemcitabine is successful in some patients 2.TACE in patients with multiple hepatic metastases seems to be of value 3.Radiotherapy has been suggested in cases of unresectable SPTs because they are radiosensitive. Maffuz A, Bustamante Fde T, Silva JA, et al. Preoperative gemcitabine for unresectable, solid pseudopapillary tmor of the pancreas. Lancet Oncol, 2005,6(3):185-186. Fried P, Cooper J, Balthazar E, et al. A role for radiotherapy in the treatment of solid and papillary neoplasms of the pancreas. Cancer, 1985,56(12):2783-2785 Prognosis 1 As reported so far, there have been 4 articles regarding spontaneous regression of SPTP and the patients were all pediatric patients. 1. Nakahara K, Kobayashi G, Fujita N, Noda Y, Ito K, Horaguchi J, et al. Solid-pseudopapillary tumor of the pancreas showing a remarkable reduction in size over the 10-year follow-up period. Intern Med. 2008;47:13351339. 2. Suzuki M, Shimizu T, Minowa K, Ikuse T, Baba Y, Ohtsuka Y. Spontaneous shrinkage of a solid pseudopapillary tumor of the pancreas: CT findings. Pediatr Int. 2010;52:335336. 3. Hachiya M, Hachiya Y, Mitsui K, Tsukimoto I, Watanabe K, Fujisawa T. Solid, cystic and vanishing tumors of the pancreas. Clin Imaging. 2003;27:106108. 4.Hyun Jung Yoon,Jae Hoon Lim. Solid Pseudopapillary Tumor of the Pancreas with Hepatic Metastasis: Spontaneous Regression Over 10-Year Follow-