TUMORI RARI DEL DISTRETTO EPATO-BILIARE

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Tumori primitivi rari dei dotti biliari

• Cistoadenoma • Adenocarcinoma papillare • Carcinoma spinocellulare • Carcinoma misto adenosquamoso • Carcinoma mucoepidermoide • Carcinoma a piccole cellule • Carcinosarcoma • Carcinoide • Sarcomi

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

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.

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.

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 .

Carcinoma misto adenosquamoso

• A rare histologic subtype of extrahepatic bile duct carcinoma (3.0

component. 32 months). ～ 4.7%).

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

Carcinoma a cellule fusate

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 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.

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):1299-301 Am Surg 2008; 74(1):87-90 (review)

Carcinosarcoma

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

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

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

Pathol Res Pract. 1980 Dec;170(1-3):258-66 • Ultrastructure of of the extrahepatic bile ducts. A light- and electron microscopic study of a case.

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)

Pathol. 1976 Nov;7(6):705-8 of the common bile duct: a case report. Hum

Sarcoma botryoides

of the biliary tree: the case for aggressive surgery. J Pediatr Surg. 1982 of the common bile duct. Report of a case and review of the literature. J Pediatr Surg. 1966 Dec;1(6):571-8

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