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TUMORI RARI DEL DISTRETTO EPATO-BILIARE

TUMORI RARI DEL DISTRETTO EPATO-BILIARE. http://www.raretumours.it. http://www.siapec.it. Tumori primitivi rari dei dotti biliari. Cistoadenoma Adenocarcinoma papillare Carcinoma spinocellulare Carcinoma misto adenosquamoso Carcinoma mucoepidermoide Carcinoma a piccole cellule

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TUMORI RARI DEL DISTRETTO EPATO-BILIARE

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  1. TUMORI RARI DEL DISTRETTO EPATO-BILIARE

  2. http://www.raretumours.it http://www.siapec.it

  3. Tumori primitivi rari dei dotti biliari • Cistoadenoma • Adenocarcinoma papillare • Carcinoma spinocellulare • Carcinoma misto adenosquamoso • Carcinoma mucoepidermoide • Carcinoma a piccole cellule • Carcinosarcoma • Carcinoide • Sarcomi

  4. Cistadenoma biliare • 5% of all hepatic solitary cyst • Middle aged women • Strong tendency to recur and a potential of malignant transformation. • Cyst wall with a single layer of cuboidal – to -columnar epithelium • A moderately cellular stroma and an outer dense layer of collagenous connective tissue • Biliary adenofibroma (<5 reported cases) (AJSP 2003;27:693) Manouras A et al World J Gastroenterol 2008 July 14; 14(26): 4257-4259

  5. Adenocarcinoma papillare • 5% of all malignant tumors of the biliary tract • The tumor is soft, less invasive to adjacent tissues and tends to grow into the lumen • Papillary histology is the most significant determinant of ultimate survival and cure • Intraductal papillary mucinous adenocarcinoma of the bile ducts (Gastrointest Endosc. 2006;64(3):438-9) • Biliary papillomatosis; papillary adenomatous proliferation with a high chance of malignant degeneration (Ned Tijdschr Geneeskd. 2003;5;147(27):1323-7) Prognosis and treatment of bile duct carcinoma. Am Surg. 1998 Oct;64(10):921-5.

  6. Carcinoma spinocellulare 20 cases have been reported, but only 6 cases were well documented Primary squamous cell carcinoma of the bile duct diagnosed by transpapillary cholangioscopy: case report and review. Gastrointest Endosc. 2004 Aug;60(2):300-4.

  7. Carcinoma mucoepidermoide • 11 primary mucoepidermoid carcinomas of the bile ducts • 9 intrahepatic; 3 right lobe and 6 left lobe. • 2 extrahepatic; common hepatic ducts. • 6 men and 5 women • Range 44-78 years old; mean age was 60 years • Size 1.5-18 cm; average 8.4 cm. • The tumor metastasized frequently to the regional lymph nodes. Invasion of the portal vein and hepatic artery has also occurred. • The prognosis is poor Primary mucoepidermoid carcinoma of the intrahepatic bile duct: a case report with review of literature. J Med Assoc Thai. 2000;83(2):197-203.

  8. Carcinoma misto adenosquamoso • A rare histologic subtype of extrahepatic bile duct carcinoma (3.0～4.7%). • Histologically, composed of squamous and glandular components which mixed in an intermingled pattern • High local aggressiveness mainly due to the squamous component. • Patients with adenosquamous carcinomas had worse survival (median 11 months) than those with adenocarcinoma (median 32 months). Adenosquamous carcinoma of extrahepatic bile duct: clinicopathologic study of 12 cases. Int J Clin Exp Pathol. 2008 Jan 1;1(2):147-56.

  9. Spindle cell-type undifferentiated carcinoma arising from the extrahepatic bile duct is extremely rare Nakanishi Y, et al. Spindle cell-type undifferentiated carcinoma of the common bile duct of the hepatic hilus: report of a case. Surg Today. 2007;37(8):708-12. Oikawa H, et al. Spindle and giant cell type undifferentiated carcinoma arising in the common bile duct: a case report. Pathol Res Pract. 2007;203(3):179-84 Albores-Saavedra J, et al. Giant cell tumor of the extrahepatic biliary tree: a clinicopathologic study of 4 cases and comparison with anaplastic spindle and giant cell carcinoma with osteoclast-like giant cells. Am J Surg Pathol. 2006 Apr;30(4):495-500. Dowaki S, et al. Undifferentiated spindle and giant cell carcinoma of the common bile duct. Tokai J Exp Clin Med. 2003 Oct;28(3):127-30 Shinoda M, et al. Spindle cell carcinoma of the intrahepatic bile duct in a patient with primary sclerosing cholangitis. J Gastroenterol. 2003;38(11):1091-6 Ng CS, et al. Spindle cell carcinoma of bile duct origin. Histopathology. 1988 Mar;12(3):329-31 Carcinoma a cellule fusate

  10. Carcinoma a piccole cellule • 10 reported cases of small cell carcinoma of the bile duct • A preponderance of elderly males (mean age 63.7, male to female ratio 9:1) • Pathologically classified as: • pure type (small cell carcinoma) • combined type (small cell carcinoma and adenocarcinoma) Small cell carcinoma of the extrahepatic bile duct diagnosed with EUS-FNA and effectively treated with chemoradiation. Intern Med. 2008;47(7):621-5.

  11. Carcinoide • 50 cases reported • Sites: • the common biliary duct (58%) • perihilar region (28%) • cystic duct (11%) • common hepatic duct (3%). • Women, fifth decade of life • Aggressive local invasion by the primary tumor is rare, and metastases occur in less than one-third of patients. • Non-functional in the majority of cases • Association with Von Hippel-Lindau syndrome (VHL) Carcinoid tumor of the common bile duct: a rare complication of von Hippel-Lindau syndrome. World J Gastroenterol. 2008 Feb 28;14(8):1299-301Am Surg 2008; 74(1):87-90 (review)

  12. Carcinosarcoma Only 3 cases of the extrahepatic bile duct have been reported in the world literature

  13. Sarcomi dei dotti biliari • A patient with synovial cell sarcoma primary to the gallbladder and common bile duct. J Gastrointest Surg. 2008 Mar;12(3):609-11. • Common bile duct schwannoma: a case report and review of literature. World J Gastroenterol. 2007 Feb 28;13(8):1275-8. • Myeloid sarcoma of the extrahepatic bile ducts presenting as obstructive jaundice. APMIS. 2006 Sep;114(9):666-8. • Nonleukemic granulocytic sarcoma in the bile duct: a case report. J Korean Med Sci. 2006 Aug;21(4):745-8 • Myeloid sarcoma of the main biliary duct mimicking a bile duct carcinoma: report of one case Gastroenterol Clin Biol. 2006 Jan;30(1):159-60 • Cholestasis caused by biliary botryoid sarcoma. Eur J Pediatr Surg. 1991 Aug;1(4):242-3 • Sarcoma botryoides of the common bile duct: preoperative diagnosis by coronal CT and PTC. Pediatr Radiol. 1991;21(2):152-3 • Malignant schwannoma in siblings with neurofibromatosis. Cancer. 1987 Feb 15;59(4):829-35 • Rhabdomyosarcoma of the biliary tree: the case for aggressive surgery. J Pediatr Surg. 1982 Oct;17(5):508-11 • Sarcoma botryoides of the common bile duct. A case report. Indian J Cancer. 1982 Jul-Aug;19(3):170-2 • Sarcoma botryoides of the extrahepatic bile ducts. A light- and electron microscopic study of a case. Pathol Res Pract. 1980 Dec;170(1-3):258-66 • Ultrastructure of botryoid sarcoma of the common bile duct. Gastroenterol Jpn. 1977;12(4):305-10 • Sarcoma botryoides arising from the biliary tract of children. A case report with review of the literature. Acta Pathol Jpn. 1976 Nov;26(6):709-18 • Embryonal rhabdomyosarcoma (sarcoma botryoides) of the common bile duct: a case report. Hum Pathol. 1976 Nov;7(6):705-8 • Sarcoma botryoides of the common bile duct. Report of a case and review of the literature. J Pediatr Surg. 1966 Dec;1(6):571-8

  14. Sarcoma granulocitico • Microscopic finding of bile duct shows a monomorphous and discohesive cellular infiltration along the bile duct wall having medium to large nuclei with one or more distinct but not prominent nucleoli and a dispersed chromatin. • Tumor cells are positive for myeloperoxidase

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