Squamous cell carcinoma of the liver originating from non-parasitic cysts after a 15 year follow-up

Abstract

Squamous cell carcinoma is an extremely rare primary liver tumour. A 42-year-old man presented at our hospital on 19 February 1986, with pain in the right upper quadrant of the abdomen and general fatigue, and reported an 8 year history of this complaint. Ultrasonography showed four cystic masses in the liver with a maximum diameter of 15 cm, one of which contained a solid component. A computed tomography (CT) scan confirmed a huge, predominantly cystic, mass in the liver with a small solid component and irregular wall. Calcifications were seen in the solid components. On 22 April 1986, a laparotomy was performed but the masses were too large to be removed. During 15 years of follow-up after the laparotomy, there had been no change seen in his abdominal CT scan. He subsequently arrived at our hospital again on 10 July 2001 with loss of appetite and of body weight. A CT scan showed a cyst in the liver of 25 cm in diameter with calcification that had a large solid part invading the liver. A post-mortem pathological dissection showed multiple cysts, the largest of which was 25 cm in diameter. They had large solid parts with calcification invading the liver. There were widespread metastatic lesions. Microscopic examination showed the tumour to be a well differentiated squamous cell carcinoma. To the best of our knowledge, this is the first report of a squamous cell carcinoma arising from 15 multiple non-parasitic hepatic cysts after a 15 year follow-up. Furthermore, 23 years had passed since the patient's symptoms appeared for the first time.

Primary Squamous Cell Carcinoma of the Liver Associated with Caroli’s Disease: A Case Report

“…The carcinogenesis process is still unknown, but a secondary squamous metaplasia due to chronic inflammation of the biliary lined cyst or ducts and subsequentneoplastictransformationisthemostpredictable mechanism. The positivity staining reported for biliary-relatedcytokeratinsinthepathologicalfindingsstrengthensthe abovehypothesis [21,22].Inourcase,thechronicinflammationrelatedtothehepatolithiasisprobablytriggeredthemetaplasia/carcinogenesisinthebiliaryducts,andtheCK7expres-sion at the pre-operative liver biopsy led to suspicion of a biliaryorigin.Thebiologicalbehaviorofthistumorishighly aggressive,asconfirmedbythesizeofthemassreported,in mostcasesover10cmindiameter [5,[7][8][9]11,19,20],thelow rate of resectability, the presence of necrotic areas in the pathologicalspecimen,andtherateofsurvival(table1).Consideringthewholegroupofpatients,only7outof24(26%) werestillaliveattheendoffollow-up(medianfollow-up:35 months;range8-84months).Themediansurvivalamongthe deceasedpatientswas6months(range13daysto15years).…”

“…Primarysquamouscellcarcinoma(SCC)arisingintheliveris a very rare occurrence, usually originating from a solitary benign nonparasitic hepatic cyst (SBNHC) [1][2][3][4][5][6][7][8][9][10][11], hepatic teratomas [12],andseldom,ifever,fromhepatolithiasis [13] orcirrhosis [14,15].Althoughthecarcinogenesismechanism isstillunknown,itseemstoberelatedtothechronicinflammation of the biliary epithelium or the metaplastic and subsequentneoplastictransformationofpre-existingcystsofthe liver [13].Ascommonlyhappenswithararedisease,thereis no univocally accepted management and the therapeutic strategy ranges from systemic chemotherapy [2] or transarterial chemoembolization (TACE) [11,[14][15][16] to major hepatic resection [7,8,10,13,[17][18][19][20][21][22]. The prognosis has a dichotomized presentation with a majority of cases not exceeding1yearofsurvival,butinsomecasesgoingbeyond 5yearsofsurvivalwithoutanysignofrecurrence(table1).…”

“…In4cases,thetumorwastreatedwithTACE,withencouraging responses in delaying the disease progression: Yagi et al [11] reported a case of a patient treated with TACE (4 cycles of cisplatin), who then died as a result of systemic metastasisafter15yearsoffollow-up.…”

Primary squamous cell carcinoma of the liver: A case report and review of the literature

“…Previous studies have reported that potentially related diseases, including congenital cyst of the liver (7,8), calculus of the intrahepatic duct (9), hepatic cirrhosis (11), Caroli's disease (12) and hepatic teratoma (1), are also rare.…”

“…The pathogenesis of the disease is undetermined. Previous studies have reported that potentially related diseases, including congenital cyst of the liver (2)(3)(4)(5)(6)(7)(8), calculus of the intrahepatic duct (9), hepatic cirrhosis (10,11), Caroli's disease (12) and hepatic teratoma (1), are rare. However, hepatic primary squamous carcinoma without latent hepatic injury has also been reported (13).…”