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Tumori primitivi epatici rari
Tumori primitivi epatici rari

  • Carcinoma fibrolamellare

  • Carcinoma misto epato-colangiocellulare

  • Epatoblastoma

  • Carcinosarcoma

  • Carcinoide primitivo epatico

  • Cistoadenoma e il cistoadenocarcinoma

  • Sarcomi

  • Linfomi

Carcinoma fibrolamellare
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): 798–803

Cancer 2006;106:1331–8

Carcinoma misto epato colangiocellulare
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

Type I

Type II

Cancer 2002; 94(7):2040–6

J Med Invest 2008; 55:161-5 (review)

Type III


  • 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.


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)


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


  • 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.

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

Carcinoide primitivo epatico
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
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 cistoadenocarcinoma1
Cistoadenoma e cistoadenocarcinoma

A multilocular cystic neoplasm lined with papillary adenocarcinoma.

Hepatobiliary cystadenoma. An area with abundant mesenchymal stroma.

Sarcomi primitivi del fegato
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.


  • 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.

Large, hyperchromatic, atypical endothelial cells in hepatic sinusoids.

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

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


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
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.


  • 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
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)


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

Sarcoma embrionale
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
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


  • < 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
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

Linfoma primitivo del fegato
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.

Archives 2001; 125:695

Pathologe. 2006 Jul;27(4):263-72.

Primary extramedullary plasmacytoma of the liver
Primary extramedullary plasmacytoma of the liver

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

(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.