http://www.raretumours.it
http://www.siapec.it
Tumori primitivi epatici rari
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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): 798–803
Cancer 2006;106:1331–8
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 mucinproducing 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
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
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Raro; meno di 20 casi
riportati in letteratura
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Numerosi sinonimi
(carcinoma a cellule fusate,
pseudosarcoma, carcinoma
polipoide, carcinoma
sarcomatoide, variante
fusocellulare di carcinoma,
ecc.)
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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
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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):90
Primary 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
Hepatobiliary cystadenoma. An area
with abundant mesenchymal stroma.
A multilocular cystic neoplasm lined
with papillary adenocarcinoma.
Sarcomi primitivi del fegato
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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 JanFeb;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
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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)
Liposarcoma
Kuo 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):811-3
Gastroenterol Hepatol 2008; 31 (1):12-7 (review)
Altri sarcomi primitivi del fegato
• Rhabdomyosarcomas of
liver typically appear in
children
Mt Sinai J Med 2005; 72(1):50–2.
• Osteosarcoma: less than
10 case reports
European Journal of Radiology Extra
Vol 50, Issue 1, April 2004, Pages 31-36
• Primary hepatic
schwannoma reported
typically associated with
von Recklinghausen’s
disease
• Alveolar soft-part
sarcoma
Am 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, ckit/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
Linfoma primitivo del fegato
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~ 100 cases reported
75% men
Median 55 years, but all ages
Diffuse sinusoidal infiltration
Lymphomas involving the liver
include, with decreasing
frequency:
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diffuse large B-cell lymphoma
small lymphocytic lymphoma
Hodgkin's lymphoma
peripheral T-cell lymphoma
follicular lymphoma
extranodal marginal zone Bcell lymphoma.
Archives 2001; 125:695
Pathologe. 2006 Jul;27(4):263-72.
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.