Tumori rari del distretto epato-biliare

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http//www.raretumours.it
http//www.siapec.it
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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

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Carcinoma fibrolamellare

• 1-5 of HCC
• Younger Caucasian patients (20-40 years) 30-40
  lt 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) 798803 Cancer
200610613318
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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)20406 J Med Invest 2008
55161-5 (review)
Type III
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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.

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

• 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 dellWHO 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
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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.
2009272(1)90Primary hepatic carcinoid tumor
case report and review of 53 cases. Med Sci
Monit. 20017(4)746-50
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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 Jun17(2)203-6.
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Cistoadenoma e cistoadenocarcinoma
A multilocular cystic neoplasm lined with
papillary adenocarcinoma.
Hepatobiliary cystadenoma. An area with abundant
mesenchymal stroma.
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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
200710913916.
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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.

Large, hyperchromatic, atypical endothelial cells
in hepatic sinusoids.
World J urg Oncol 2008 306104 (review) World J
Gastroenterol 2006 7 12(5)804-808
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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-appearin
g tumour cells, mimicking carcinoma. In contrast
to epithelioid haemangioendothelioma, there is no
stroma.
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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) 56282.
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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-Feb32(1)25-8) total 3 cases
  reported

Am J Gastroenterol 1995 90(4) 64952 Korean J
Gastroenterol 200851194-198
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Istiocitoma fibroso maligno

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

Mt Sinai J Med 2005 72(1)502. Am J Surg Pathol
2008 32 1144-58 (review)
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Liposarcoma
Kuo LM et al.World J Gastroenterol 2006 February
21 12(7)1157-1159
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Sarcoma embrionale
The tumour is gelatinous and fish-flesh in
consistency with areas of necrosis. Undifferentia
ted tumour cells in a myxoid matrix. Some tumour
cells contain eosinophilic globules.
Indian J Pathol Microbiol 2007 Oct50(4)811-3Gas
troenterol Hepatol 2008 31 (1)12-7 (review)
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Altri sarcomi primitivi del fegato

• Rhabdomyosarcomas of liver typically appear in
  childrenMt Sinai J Med 2005 72(1)502.
• 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 Recklinghausens disease
• Alveolar soft-part sarcomaAm Surg 2008
  74(1)43-6

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Angiomiolipoma

• lt 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 26493 (c-kit
staining) Archives 2002 12649 (melanoma
markers) AJSP 1999 2334 (rewiew)
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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
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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 125695 Pathologe. 2006
Jul27(4)263-72.
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Primary extramedullary plasmacytoma of the liver
Demirhan B. et al J Clin Pathol 19975074-76
(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.