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2 Tumori primitivi epatici rari
Carcinoma fibrolamellare Carcinoma misto epato-colangiocellulare Epatoblastoma Carcinosarcoma Carcinoide primitivo epatico Cistoadenoma e il cistoadenocarcinoma Sarcomi Linfomi

3 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

4 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

5 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.

6 Epatoblastoma 1. Hepatoblastoma. A lobectomy specimen, showing a tumour mass bulging from the surface with lobulations on cut section 2. Hepatoblastoma, fetal epithelial type. The tumour has a characteristic ‘light and dark’ appearance at low magnification. H&E. 3. Hepatoblastoma, fetal epithelial type with abundant extramedullary haematopoiesis. H&E. 4. Hepatoblastoma, epithelial type with embryonal epithelium (left) and fetal epithelium (right). H&E. 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)

7 Epatoblastoma 1 Hepatoblastoma, mixed type. There is a fetal epithelial component (right) and an embryonal component mixed with primitive mesenchyme (left). H&E. 2. Hepatoblastoma, mixed type, with osteoid. H&E. 3. Hepatoblastoma, mixed type. Partially calcified osteoid in the midst of a fetal epithelial component. H&E. 4. Hepatoblastoma, mixed type. An area with primitive mesenchyme and cartilage. H&E. Hepatoblastoma, mixed type: fetal epithelial component and an embryonal component mixed with primitive mesenchyme, with osteoid, partially calcified osteoid, and cartilage.

8 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 dell’WHO “un tumore che contiene sia elementi carcinomatosi (epato o colangioca,) sia elementi sarcomatosi, includendo i tumori misti. Raro; meno di 20 casi riportati in letteratura; numerosi sinonimi (carcinoma a cellule fusate, pseudosarcoma, carcinoma polipoide, carcinoma sarcomatoide, variante fusocellulera di carcinoma, ecc.; definizione dell’WHO “un tumorr che contiene sia elementi carcinomatosi (epato o colangiocarcinoma) sia elementi sarcomatosi, includendo i tumori misti. Hyeok Kwon J et al. Korean J Radiol 8(4), August 2007

9 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 Fig. 1. CT scan of the liver. The left image shows a large mass occupying the right lobe. Smaller lesions are seen in the left lobe of the liver. Right image: the tumor is highly vascular showing extensive peripheral enhancement with big central necrosis (arrows). Fig. 2. Fine needle aspiration cytology from the big mass in the right lobe of the liver shows a cellular lesion composed of small uniform cells lying scantly or forming loosely arranged clusters. The cytoplasm is scanty showing fi ne eosinophilic granules in some. The nuclei are uniform, small with granular chromatin. Papanicolaou. ! 400. Fig. 3. Positive immunohistochemical stains for chromogranin A, consistent with a neuroendocrine tumor (carcinoid). Chromogranin A. ! 400. 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

10 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 Jun;17(2):203-6.

11 Cistoadenoma e cistoadenocarcinoma
1. Hepatobiliary cystadenoma. An area with abundant mesenchymal stroma. H&E. 2. Hepatobiliary cystadenocarcinoma. A multilocular cystic neoplasm lined with papillary adenocarcinoma. H&E. Hepatobiliary cystadenoma. An area with abundant mesenchymal stroma. A multilocular cystic neoplasm lined with papillary adenocarcinoma.

12 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. The rarity of this disease is evident by the existence of only 30 patients with primary hepatic sarcoma managed at our institution between 1981 and 2004, compared with 331 patients with liver metastases from sarcoma admitted to our institution during a similar time period.21 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.

13 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 ;6:104 (review) World J Gastroenterol ; 12(5):

14 Angiosarcoma 1. Atrophy of liver cell plates with sinusoids lined by malignant endothelial cells. H&E 2. Loss of hepatocytes, producing cavernous areas lined by malignant endothelial cells. H&E. 3. Epithelioid-appearing tumour cells, mimicking carcinoma. In contrast to epithelioid haemangioendothelioma, there is no stroma. 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.

15 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% Epithelioid haemangioendothelioma. Multifocal tumour nodules are hard and white, mimicking metastatic carcinoma. 1. Epithelioid haemangioendothelioma. Individual and small groups of tumour cells in a mucopolysaccharide-rich stroma. Some tumour cells are epithelioid and some form capillary lumina. H&E. 2. Epithelioid haemangioendothelioma. Immunostain for CD34 demonstrates that the tumour cells are of endothelial origin 3. Epithelioid haemangioendothelioma. Pale, epithelioid tumour cells grow in sinusoids, and form papillary tufts, mimicking metastatic carcinoma. H&E. Epithelioid hemangioendothelioma is a distinctive vascular neoplasm that usually arises in liver, lung, or bone and can be regarded as a type of low-grade angiosarcoma. Am Surg 2008; 74 (1):64-8 (review) Cancer 1999; 85(3): 562–82.

16 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 Jan-Feb;32(1):25-8) – total 3 cases reported Leiomyosarcoma is a rare tumor of the liver. It usually arises from many other organs including uterus, gastrointestinal tract, retroperitoneum, and soft tissues. Primary hepatic leiomyosarcoma progresses very slowly and is not associated with chronic liver disease. When the tumor is detected early enough to be treated by operation, the prognosis is favorable. While several cases of primary hepatic leiomyosarcoma have been reported in Korea, there was no case associated with acute bleeding. We report a 80-year old male patient with huge primary hepatic leiomyosarcoma, who presented with acute bleeding and IVC obstruction. The patient was treated by embolization and IVC stenting. Am J Gastroenterol 1995; 90(4): 649–52 Korean J Gastroenterol 2008;51:

17 Istiocitoma fibroso maligno
Extremely rare 29 cases reported Mean age 51yrs 16 men, 13 women MFH of the liver is extremely rare, and only 28 cases have been reported to date.[6-14] In this report we present a case of primary hepatic MFH with multicystic presentations. Fig. 1. Abdominal CT showing a large multiseptated, multicystic lesion involving the terminus of the main hepatic vein. The tumor bulging from the diaphragmatic surface of the liver (A). The largest size of the tumor was 9.5×14.2 cm in dimension. The septae and focally thickened cystic wall were intensely enhanced (B) Occasionally, hepatic MFH with extensive necrosis may be manifested as a cystic tumor. Tumor necrosis in our case progressed to such an extent that it resembled a cystic mass or abscess on imaging studies. Cystic transformation of the entrapped small bile ducts and ductules was sometimes also demonstrated.[8] However, the absence of distinctly characteristic features makes a preoperative diagnosis of hepatic MFH extremely difficult. Since MFH was first described in 1964, many attempts have been made to define its pathogenesis and histogenesis.[21-24] According to its histopathologic features, MFH has been categorized into storiformpleomorphic, myxoid, inflammatory, giant cell, and angiomatoid variants. Among these types, the storiform-pleomorphic is the commonest. In our case, the histologic pleomorphism, the degree of vimentin staining, and the absence of smooth muscle marker desmin were consistent with the criteria for a diagnosis of MFH. However, the diagnosis of hepatic MFH is based on a process of exclusion.[25-27] Differential diagnosis firstly includes hepatocellular carcinoma.[28] A variety of poorly differentiated sarcomas such as angiosarcoma, the commonest type, and the infrequent liposarcomas, of rhabdomyosarcoma,[1] leiomyosarcoma,[29] fibrosarcoma, and embryonal sarcoma seen in children and young adults[30] hence should be included in the differential diagnosis. In addition, the presence of a large cystic cavity in the liver produces a clinical differential diagnosis that includes intrahepatic cholangiocarcinoma, benign liver cyst, liver abscess, and echinococcus alveolaris infection. Similar to MFH in the other sites of the body, surgery is the only effective method of treatment, but the prognosis is poor.[31-33] The overall 2-year survival rate is approximately 60% and 20% of the patients will die or suffer from local recurrence. Chemotherapy and radiotherapy have been attempted but failed.[34]. Fig. 2. Pathological examination showing a storiform architectural arrangement with fibroblast-like spindle cells (A). Multiple bizarre giant cells scattered between the spindle cells, and numerous mitotic figures identied (B) (HE, original magnification, A×60, B× 200). Fig. 3. Immunohistological staining for vimentin (A) and for CD68 (B) demonstrating positive reaction in the tumor cells (EnVision, original magnification×200). Mt Sinai J Med 2005; 72(1):50–2. Am J Surg Pathol 2008; 32: (review)

18 Kuo LM et al.World J Gastroenterol 2006 February 21; 12(7):1157-1159
Liposarcoma Kuo LM et al.World J Gastroenterol 2006 February 21; 12(7): Figure 1 A low-attenuation cystic mass located in segments 4, 5, and 8 with bilateral biliary tract compression in a non-cirrhotic liver by CT. Figure 2 A lobulated liver liposarcoma with intra-tumor hemorrhage Figure 3 Numerous lipoblasts and capillaries in the myxoid liposarcoma (hematoxylin-eosin staining, 400 ×). Figure 5 A high grade of undifferentiated liposarcoma composed of spindle cells with marked nuclear pleomorphism (hematoxylin-eosin staining, × 400 ).

19 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. 1. Embryonal sarcoma. The tumour is gelatinous and fish-flesh in consistency with areas of necrosis. 2. Embryonal sarcoma. Undifferentiated tumour cells in a myxoid matrix. Some tumour cells contain eosinophilic globules. H&E. 3. Embryonal sarcoma. Bizarre, trapped bile ducts and hepatocytes at the periphery of a tumour. H&E. Indian J Pathol Microbiol 2007 Oct;50(4):811-3 Gastroenterol Hepatol 2008; 31 (1):12-7 (review)

20 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 MFH of the liver is extremely rare, and only 28 cases have been reported to date.[6-14] In this report we present a case of primary hepatic MFH with multicystic presentations. Fig. 1. Abdominal CT showing a large multiseptated, multicystic lesion involving the terminus of the main hepatic vein. The tumor bulging from the diaphragmatic surface of the liver (A). The largest size of the tumor was 9.5×14.2 cm in dimension. The septae and focally thickened cystic wall were intensely enhanced (B).

21 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 1. Angiomyolipoma. A typical lesion with large, abnormal arteries, smooth muscle and adipose tissue. H&E. 2. Angiomyolipoma. A tumour with bizarre, epithelioid smooth muscle cells that might be mistaken for malignancy. H&E. 3. Angiomyolipoma. A tumour with a large amount of haematopoietic tissue in addition to the other components. H&E. Arch Pathol Lab Med 2008; 132 (10): (review) AJSP 2002; 26:493 (c-kit staining) Archives 2002; 126:49 (melanoma markers) AJSP 1999; 23:34 (rewiew)

22 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. Microscopic examination shows spindle cells like other stromal tumors. The histological diagnosis of a benign schwannoma is usually a simple procedure with standard Hematoxilin- Eosin staining. The distinction between a schwannoma and other spindle cell tumors or neurofibromas is based on the presence of a true capsule and Antoni A and Antoni B findings in the schwannoma (11). The type A area is dense and cellular, but the type B area is hypocellular. Immunohistochemical staining is diffusely and strongly positive for S-100 protein in a schwannoma consistent with the finding of a nerve sheath tumor. The tumor is also positive for the glial fibrillary acidic protein and CD57 (Leu 57) (15). Even though a few gastrointestinal stromal tumors are positive for S-100, they are also positive for either CD34 or CD117. However, a schwannoma is negative for both CD34 and CD117. A leiomyoma would be negative for S-100 and positive for desmin or smooth muscle actin Lee WH et al J Korean Med Sci 2008; 23:

23 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. 1. Diffuse large B cell lymphoma. The tumour causes irregular destruction of hepatic parenchyma. H&E. Archives 2001; 125:695 Pathologe Jul;27(4):

24 Primary extramedullary plasmacytoma of the liver
Demirhan B. et al J Clin Pathol 1997;50:74-76 Primary non-Hodgkin's lymphomas of the liver are rare. Many of these lymphomas are high grade, with only a minority of cases presenting as low grade tumours.l A proportion of primary low grade lymphomas of the liver would seem to be B cell lymphomas of mucosa associated lymphoid tissue (MALT) type.2 To our knowledge, only two cases of primary extramedullary plasmacytoma of the liver has been reported to date.3 (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.


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