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Tumori primitivi epatici rari

• Carcinoma fibrolamellare• Carcinoma misto epato-colangiocellulare• Epatoblastoma• Carcinosarcoma• Carcinoide primitivo epatico• Cistoadenoma e il cistoadenocarcinoma• Sarcomi• Linfomi

Carcinoma fibrolamellare

• 1-5% of HCC• Younger Caucasian patients

(20-40 years); 30-40% < 20 years old

• No gender preference• FC arises in an otherwise

normal liver, in contrast to most patients with HCC.

• A characteristic lamellar connective tissue is noted with macrohepatocytes.

• Better prognosis than classic HCC; 5 years survival 60%

Hepatology 2004; 39(3): 798–803Cancer 2006;106:1331–8

Type III

Type II

Carcinoma misto epato-colangiocellulare

• Less than 5% of primary hepatic carcinomas

• These tumors are divided into: –those coincidentally containing

HCC and cholangiocarcinoma in the same liver (type I)

–“transitional tumors” from HCC to cholangiocarcinoma (type II)

–“fibrolamellar tumors” which resemble FC but contain mucin-producing pseudoglands (type III)

• Classification problems• The majority of these tumors arise

in non-cirrhotic livers, with equal male/female distribution

• Aggressive, 5 year overall survival 18%, 24% after resection

Cancer 2002; 94(7):2040–6J Med Invest 2008; 55:161-5 (review)

Type I

Epatoblastoma

• Almost exclusively in children between 6 months and 3 years, with a male predominance

• 50% of liver mlignancies in children

• Derived from undifferentiated embryonal tissue and thought to develop from pluripotent hepatic stem cells.

• More commonly in families with familial adenomatous polyposis.

• Lesions are often a solitary mass in the right lobe of the liver.

Epatoblastoma

Tumour mass bulging from the surface with lobulations on cut section; fetal epithelial type: a characteristic light and dark appearance and abundant extramedullary haematopoiesis; embryonal epithelium (left) and fetal epithelium (right)

Epatoblastoma

Hepatoblastoma, mixed type: fetal epithelial component and an embryonal component mixed with primitive mesenchyme, with osteoid, partially calcified osteoid, and cartilage.

Carcinosarcoma

Hyeok Kwon J et al. Korean J Radiol 8(4), August 2007

• Raro; meno di 20 casi riportati in letteratura

• Numerosi sinonimi (carcinoma a cellule fusate, pseudosarcoma, carcinoma polipoide, carcinoma sarcomatoide, variante fusocellulare di carcinoma, ecc.)

• definizione dell’WHO “un tumore che contiene sia elementi carcinomatosi (epato o colangioca,) sia elementi sarcomatosi, includendo i tumori misti.

Carcinoide primitivo epatico

• 100 cases reported; less than 10 functional.

• These tumors may arise from a pluripotent stem cell

• Middle age (mean age = 48.2 years) and is more frequent in females (males/females = 20/33 cases)

• 80% and 84% of the cases are positive for Grimelius silver stain and immunohistochemically positive for chromogranin A, respectively

• 18% of recurrence rate and a 74% of a survival rate after 5 years

Primary hepatic carcinoid tumor: a case report and review of the literature. Cases J. 2009;27;2(1):90Primary hepatic carcinoid tumor: case report and review of 53 cases. Med Sci Monit. 2001;7(4):746-50

Cistoadenoma e cistoadenocarcinoma

• Nearly 200 cystadenomas and 100 cystadenocarcinomas have been reported.

• Cystadenomas are usually found in middle-aged women.

• The cause is unknown. • The typical appearance is of a

lobulated, multiloculated mass. • Histologically, most contain an

“ovarian-like” stroma.• CA 19-9 may be elevated.

Hepatobiliary cystadenoma: a report of two cases. J

Gastrointestin Liver Dis. 2008 Jun;17(2):203-6.

Cistoadenoma e cistoadenocarcinoma

A multilocular cystic neoplasm lined with papillary adenocarcinoma.

Hepatobiliary cystadenoma. An area with abundant mesenchymal stroma.

Sarcomi primitivi del fegato

• Less than 1% of all hepatic malignancies.

• Angiosarcoma, embryonal sarcoma, leiomyosarcoma, epithelioid hemangioendothelioma, fibrosarcoma, malignant fibrous histiocytoma, malignant solitary fibrous tumor, follicular dendritic cell sarcoma,

• Rhabdomyosarcoma in infants and children

• In immunocompromised individuals smooth muscle tumors of uncertain malignant potential have been described that are Epstein-Barr virus (EBV)-driven.

Only 30 primary hepatic sarcoma between 1981 and 2004, compared with 331 patients with liver metastases from sarcoma

Weitz J. et al Cancer 2007;109:1391–6.

Angiosarcoma

• Rare but the third most common primary malignant tumor of the liver.

• Predelection for elderly males.• The exposures to vinyl chloride,

arsenic, thorotrast and irradiation are associated with an increased risk (etiologic cofactors).

• Endothelial proliferation along the liver sinusoids, large necroses and cell atypias; solid and papillary.

• Immunohistochemical staining for CD31, CD34, MIB-1 confirme the diagnosis

• Poor prognosis.

World J urg Oncol 2008 30;6:104 (review)

World J Gastroenterol 2006 7; 12(5):804-808

Large, hyperchromatic, atypical endothelial cells in hepatic sinusoids.

Angiosarcoma

Atrophy of liver cell plates with sinusoids lined by malignant endothelial cells.Loss of hepatocytes, producing cavernous areas lined by malignant endothelial cells. Epithelioid-appearing tumour cells, mimicking carcinoma. In contrast to epithelioid haemangioendothelioma, there is no stroma.

Emangioendotelioma epitelioide

• More than 200 cases reported, with a link to oral contraceptives.

• Mean age 47 years, but occurs at any age

• Female predominance (60%)• Non specific symptoms; in over 40%

the tumor is found incidentally.• Histologically: dendritic and

epithelioid cells with immunohistochemistry positivity for at least one endothelial cell marker (FVIII-RAg,CD34,CD31)

• Indolent and slow growing; 5 year survival 43%

Am Surg 2008; 74 (1):64-8 (review)Cancer 1999; 85(3): 562–82.

Leiomiosarcoma

• Approximately 50 primary liver leiomyosarcomas are documented

• A mean age of 53• Equal male/female distribution.• No causative factors are known.• Primary hepatic myxoid

leiomyosarcoma: a case report and review of the literature (Ultrastruct Pathol. 2008 Jan-Feb;32(1):25-8) – total 3 cases reported

Am J Gastroenterol 1995; 90(4): 649–52

Korean J Gastroenterol 2008;51:194-198

Istiocitoma fibroso maligno

• Extremely rare• 29 cases reported• Mean age 51yrs• 16 men, 13 women

Mt Sinai J Med 2005; 72(1):50–2.Am J Surg Pathol 2008; 32: 1144-58 (review)

LiposarcomaKuo LM et al.World J Gastroenterol 2006 February 21; 12(7):1157-1159

Sarcoma embrionale

The tumour is gelatinous and fish-flesh in consistency with areas of necrosis.

Undifferentiated tumour cells in a myxoid matrix. Some tumour cells contain eosinophilic globules.

Indian J Pathol Microbiol 2007 Oct;50(4):811-3Gastroenterol Hepatol 2008; 31 (1):12-7 (review)

Altri sarcomi primitivi del fegato

• Rhabdomyosarcomas of liver typically appear in childrenMt Sinai J Med 2005; 72(1):50–2.

• Osteosarcoma: less than 10 case reports European Journal of Radiology ExtraVol 50, Issue 1, April 2004, Pages 31-36

• Primary hepatic schwannoma reported typically associated with von Recklinghausen’s disease

• Alveolar soft-part sarcomaAm Surg 2008; 74(1):43-6

Angiomiolipoma

• < 100 cases reported

• Often misdiagnosed

• Similar to renal angiomyolipoma

• Mean age 50 years (9-79)

• 80% women

• HMB45, MelanA/Mart1, MITF (50%), S100, actin (1A4), desmin, c-kit/CD117

Arch Pathol Lab Med 2008; 132 (10):1679-82 (review)AJSP 2002; 26:493 (c-kit staining)Archives 2002; 126:49 (melanoma markers)AJSP 1999; 23:34 (rewiew)

Schwannoma benigno

• 9 cases reported• Associated with neurofibromatosis

in about 50% of cases.• Malignant transformation is rare• They grow very slowly and are

well encapsulated in most cases. • Usually smaller than 5 cm at

diagnosis• Larger schwannomas have a

tendency to undergo secondary degeneration such as pseudocystic regression, hemorrhage and calcification.

Lee WH et al J Korean Med Sci 2008; 23: 727-30

Archives 2001; 125:695Pathologe. 2006 Jul;27(4):263-72.

Linfoma primitivo del fegato• ~ 100 cases reported• 75% men• Median 55 years, but all ages• Diffuse sinusoidal infiltration• Lymphomas involving the liver

include, with decreasing frequency:– diffuse large B-cell lymphoma– small lymphocytic lymphoma– Hodgkin's lymphoma– peripheral T-cell lymphoma– follicular lymphoma– extranodal marginal zone B-

cell lymphoma.

Primary extramedullary plasmacytoma of the liver

(A) Infiltrate of uniform mature plasma cells with mild atypia, invading the liver parenchyma (haematoxylin and eosin); (B) VS38 antibody positive cells (immunoperoxidase); (C) plasma cells showing K light chain restriction; (D) no i. light chain immunoreactivity is evident.

Demirhan B. et al J Clin Pathol 1997;50:74-76