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Survival After Resection for Epidermoid Cyst-originated Squamous Cell Carcinoma of the Liver: Case Report and Review of the Literature

Published:August 30, 2022DOI:https://doi.org/10.1016/j.jceh.2022.08.006
      Epidermoid cyst is a rare type of congenital hepatic cyst. Reports on squamous cell carcinoma (SCC) of the liver are very limited and mostly have poor outcomes. We present a 45-year-old woman who came to our hospital due to obstructive jaundice caused by an epidermoid cyst-originated SCC. She underwent radical resection and lived for two years without signs of recurrence. The presentation, treatment, pathological results, as well as literature review, are provided below.

      Keywords

      Abbreviations:

      EC (Epidermoid cyst), SCC (Squamous cell carcinoma)
      Epidermoid cysts (EC) are congenital solitary cysts that develop during the fetal period. Their walls are filled with fibrous and stratified squamous epithelium.
      • Lombardo F.P.
      • Hertford D.E.
      • Tan L.K.
      • et al.
      Epidermoid cyst of the liver complicated by microscopic squamous cell carcinoma: CT, ultrasound, and pathology.
      The symptoms are ambiguous, most of the cases are found incidentally during laparotomy. Due to its rarity, the exact incidence in the population is unknown.
      Primary squamous cell carcinoma (SCC) is an uncommon type of hepatic neoplasm, and EC-originated SCC is even scarcer. Though EC is considered a benign cyst, evidence has demonstrated that malignant transformation has occurred over six months, showing its aggressiveness.
      • Ödemiş B.
      • Küksal A.Ş.
      • Yöksel O.
      • Kacar S.
      • Turhan N.
      Squamous Cell Cancer of the Liver Arising from an Epidermoid Cyst: Case Report and Review of the Literature.
      Overall survival for patients with primary SCC is poor, most patients die after less than one year.
      • Zhao R.
      • Zhu K.
      • Wang R.
      • et al.
      Primary squamous cell carcinoma of the liver: a case report and review of the literature.
      Treatment standards have not yet been published, but radical resection is considered the only curative option.
      • Ödemiş B.
      • Küksal A.Ş.
      • Yöksel O.
      • Kacar S.
      • Turhan N.
      Squamous Cell Cancer of the Liver Arising from an Epidermoid Cyst: Case Report and Review of the Literature.
      We report a 45-year-old woman who is still alive with no recurrence after two years of right trisegmentectomy for primary SCC from an epidermoid cyst.

      Presentation of case

      A 45-year-old woman with no previous medical history came to our hospital after two weeks of obstructive jaundice. She had no sign of infection or weight loss. On examination, she was in stable condition with no ascites and no tenderness. Her bilirubin level was 316.8 μmol/l; AST/ALT levels were 253/310 U/L, respectively. CA 19–9 was 938 U/ml. Ultrasound image showed a 64∗87 mm mass with mixed echogenicity and an ill-defined border, suggestive of gallbladder carcinoma. MRI also demonstrated a large mass that developed into segments IV and V, which was continuous with the gallbladder (Figure 1). The mass invaded the hilum, which explained the jaundice situation. A CT scan was also performed, however, revealed a non-enhancing, hypodense, and well-defined intrahepatic cystic tumor. A needle biopsy was conducted afterward but could not find evidence of malignancy. The bilirubin level was reduced to 141 mol/l following one week of percutaneous biliary drainage (Figure 2). Volumetry and left lateral segment hypertrophy were used to illustrate the viability of right hepatectomy/trisegmentectomy.
      Figure 2
      Figure 2Imaging of the percutaneous biliary drainage.
      Following a laparotomy, it was determined that the lesion was a cystic tumor in segments IV, V, and VIII that was compressing the hilum and causing intrahepatic biliary dilatation. The tumor was not discovered in any other organs. The cyst was filled with a dark brown mucinous fluid (Figure 3). Cholecystectomy, right trisegmentectomy, and lymph node dissection were performed, with all margin resection confirmed as negative by frozen section (Figure 4). Bilioenteric anastomosis for bile duct segments II and III was performed (Figure 5). According to the results of the histological and immunohistochemical analyses, the cystic tumor was a primary SCC that originated from an epidermoid cyst and invaded the portal tract and portal branch . The cyst had a sclerosing wall and was filled with squamous epithelium, secreting cells, and high-differentiation cancer cells with no biliary tract cells (Figure 6). R0 resection was achieved. None of the ten lymph nodes removed were positive. Overall staging is T2N0Mx (based on TNM 8th 2017 classification). Postoperative course was uneventful. The patient was discharged after 14 days. The patient was routinely followed, she has lived for 24 months without sign of recurrence.
      Figure 4
      Figure 4Negative resection margin after hepatectomy.
      Figure 5
      Figure 5Bilioenteric anastomosis of the bile duct.
      Figure 6
      Figure 6The histopathological imaging of the tumor.
      Table 1Review of Literature About Epidermoid Cyst-related Squamous Cell Carcinoma.
      NoAge/GenderTypeSiteTreatmentSurvival (months)References
      1.59 FECRight lobeRight hepatectomy16, alive
      • Banbury J.
      • Conlon K.C.
      • Ghossein R.
      • Brennan M.F.
      Primary squamous cell carcinoma within a solitary nonparasitic hepatic cyst.
      2.18 MECSegment VIII; vascular, biliary invasionRight hepatectomy, Whipple's procedure7
      • Ödemiş B.
      • Küksal A.Ş.
      • Yöksel O.
      • Kacar S.
      • Turhan N.
      Squamous Cell Cancer of the Liver Arising from an Epidermoid Cyst: Case Report and Review of the Literature.
      3.59 FECRight lobeRight hepatectomy8, alive
      • Lombardo F.P.
      • Hertford D.E.
      • Tan L.K.
      • et al.
      Epidermoid cyst of the liver complicated by microscopic squamous cell carcinoma: CT, ultrasound, and pathology.
      4.21 MECSegment V, VIRight hepatectomy, right hemicolectomy, antrectomy3, alive
      • Caratozzolo E.
      • Massani M.
      • Recordare A.
      • et al.
      Squamous cell liver cancer arising from an epidermoid cyst.
      5.30 MECRight lobeExtended right hepatic lobectomy4
      • Bloustein P.A.
      • Silverberg S.G.
      Squamous cell carcinoma originating in an hepatic cyst. Case report with a review of the hepatic cyst-carcinoma association.
      6.63 MECLeft lobeCeliotomy, subtotal hepatic cystectomy6
      • Lynch M.J.
      • McLeod M.K.
      • Weatherbee L.
      • Gilsdorf J.R.
      • Guice K.S.
      • Eckhauser F.E.
      Squamous cell cancer of the liver arising from a solitary benign nonparasitic hepatic cyst.
      7.37 MECBoth lobeLaparotomy2
      • Greenwood N.
      • Orr W.M.
      Primary squamous-cell carcinoma arising in a solitary non-parasitic cyst of the liver.
      8.78 MECSegment IV, V, VIIIPreservation2
      • Gresham G.A.
      • Rue 3rd, L.W.
      Squamous cell carcinoma of the liver.
      9.62 FECRight lobe with multiple organ invasionLaparotomy5
      • Nieweg O.
      • Slooff M.J.
      • Grond J.
      A case of primary squamous cell carcinoma of the liver arising in a solitary cyst.
      10.74 FECSegment VI, VII, VIIIRight hepatectomy54, alive
      • Weimann A.
      • Klempnauer J.
      • Gebel M.
      • et al.
      Squamous cell carcinoma of the liver originating from a solitary non-parasitic cyst case report and review of the literature.
      11.71 FECRight lobeCystostomy1
      • Monteagudo M.
      • Vidal G.
      • Moreno M.
      • et al.
      Squamous cell carcinoma and infection in a solitary hepatic cyst.
      12.42 MECBoth lobePreservation8 (180 since the cyst found)
      • Yagi H.
      • Ueda M.
      • Kawachi S.
      • et al.
      Squamous cell carcinoma of the liver originating from non-parasitic cysts after a 15 year follow-up.
      13.45 FECSegment IV, V, VIIIRight trisegmentectomy23, aliveOur case
      EC, Epidermoid cysts.

      Discussion

      Epidermoid cysts are rare and limitedly reported. It should be noted that EC is different from other non-paracystic hepatic cysts. While major nonparasitic solitary cysts arise from von Meyemberg complexes, epidermoid cysts grow from the hepatic diverticulum or adjacent embryonic gut.
      • Homer L.W.
      • White H.J.
      • Read R.C.
      Neoplastic transformation of v. Meyenburg complexes of the liver.
      ,
      • Caratozzolo E.
      • Massani M.
      • Recordare A.
      • et al.
      Squamous cell liver cancer arising from an epidermoid cyst.
      EC is characterized by pseudostratified cylindrical epithelium along with squamous metaplasia. They had four layers: the epithelium with mucin-secreting cells, connective tissue, a smooth muscle layer, and the outer layer of fibrous capsule.
      • De J.
      • Rossman L.
      • Kott M.M.
      • Deavers M.T.
      Cytologic diagnosis of ciliated hepatic foregut cyst.
      We concluded that it is a primary SCC originating from epidermoid cyst based on three main findings. Firstly, the inner layer was all covered by squamous cells not cylindrical cells, suggesting a congenital cyst. Secondly, it was not totally covered by SCC, and no sign of a second tumor was found, which excluded the chance of metastasis SCC. And lastly, if it is a cystadenoma or cystadenocarcinoma, then it is more likely that the ovarian-like stroma should be presented, but no specimens showed this evidence.
      SCC arising from EC is very rare. In our review of literature, 13 cases with SCC have been published. Ten of them (55.5%) are male, the youngest patient was 21 years old, while the oldest was 78 years old (Table 1). As imaging could not exactly determine these cysts, all of them were identified via laparotomy or surgical resection. 55.5% survived less than one year. For other lived cases, as the follow-up time is short, it is hard to determine the overall survival. In one case published by Yagi (2004), the patient had ciliated hepatic foregut cyst for 15 years, but then suddenly turned into malignancy in a short amount of time. Only two cases (including ours) showed a survival time of more than two years. Thus, the prognosis of epidermoid cyst-related SCC is poor.
      As the number of cases and evidence regarding systemic chemotherapy for SCC is limited, complete resection seems to be the only curative treatment.
      • Boscolo G.
      • Jirillo A.
      • Da Pian P.
      Complete remission of poorly differentiated squamous liver carcinoma after systemic chemotherapy and surgery. A case report.
      • Zhang X.-F.
      • Du Z.-Q.
      • Liu X.-M.
      • Lv Y.
      Primary squamous cell carcinoma of liver: case series and review of literatures.
      • Kaji R.
      • Sasaki N.
      • Tateishi I.
      • et al.
      A case report of primary hepatic squamous cell carcinoma that remarkably responded to low dose arterial injection of anti-cancer drugs.
      We chose to perform trisegmentectomy due to tumor infiltration to the hilum and difficulty in dissecting the triad. Right hepatectomy would be inadequate, while central hepatectomy is not technically feasible. Our case showed a good outcome after radical resection for an epidermoid cyst. Due to its aggressiveness, we thought that surgery should be considered the first option. Then, adjuvant chemotherapy should be further studied to optimize the treatment outcomes.
      Our case demonstrated the benefits of radical surgery in EC-based SCC over drainage, marsupialization, or somehow chemotherapy. However, it is a case report, and a definite conclusion would be insufficient. Further studies with a larger population, with other types of treatment, and longer follow-up times should be conducted for a better understanding of this condition.
      Epidermoid cyst-originated SCC of the liver is a very rare condition. Our case has shown that the benefit of radical surgery is encouraging. Higher quality evidence is needed to provide a comprehensive knowledge of this condition.

      CREDIT AUTHORSHIP CONTRIBUTION STATEMENT

      Khai Ninh Viet: Perform the operation, conceptualization, investigation, writing
      Dang Do Hai: Writing, data curation
      Tuan Hoang, Phuong Tran Ha: Data curation
      Lanh Nguyen Sy: Investigation, writing
      Nghia Nguyen Quang: Supervision, Review & Editing

      Conflicts of interest

      The authors have none to declare

      Funding

      None.

      Consent

      Verbal informed consent was obtained from the patients and her husband for the report publication.

      Provenance and peer review

      Not commissioned, externally peer-reviewed.

      References

        • Lombardo F.P.
        • Hertford D.E.
        • Tan L.K.
        • et al.
        Epidermoid cyst of the liver complicated by microscopic squamous cell carcinoma: CT, ultrasound, and pathology.
        J Comput Assist Tomogr. 1995; 19: 131-134
        • Ödemiş B.
        • Küksal A.Ş.
        • Yöksel O.
        • Kacar S.
        • Turhan N.
        Squamous Cell Cancer of the Liver Arising from an Epidermoid Cyst: Case Report and Review of the Literature.
        Digestive Diseases and Sciences. 2006; 51: 1278-1284
        • Zhao R.
        • Zhu K.
        • Wang R.
        • et al.
        Primary squamous cell carcinoma of the liver: a case report and review of the literature.
        Oncol Lett. 2012; 4: 1163-1166
        • Homer L.W.
        • White H.J.
        • Read R.C.
        Neoplastic transformation of v. Meyenburg complexes of the liver.
        J Pathol Bacteriol. 1968; 96: 499-502
        • Caratozzolo E.
        • Massani M.
        • Recordare A.
        • et al.
        Squamous cell liver cancer arising from an epidermoid cyst.
        J Hepatobiliary Pancreat Surg. 2001; 8: 490-493
        • De J.
        • Rossman L.
        • Kott M.M.
        • Deavers M.T.
        Cytologic diagnosis of ciliated hepatic foregut cyst.
        Diagn Cytopathol. 2006; 34: 846-849
        • Banbury J.
        • Conlon K.C.
        • Ghossein R.
        • Brennan M.F.
        Primary squamous cell carcinoma within a solitary nonparasitic hepatic cyst.
        J Surg Oncol. 1994; 57: 210-212
        • Bloustein P.A.
        • Silverberg S.G.
        Squamous cell carcinoma originating in an hepatic cyst. Case report with a review of the hepatic cyst-carcinoma association.
        Cancer. 1976; 38: 2002-2005
        • Lynch M.J.
        • McLeod M.K.
        • Weatherbee L.
        • Gilsdorf J.R.
        • Guice K.S.
        • Eckhauser F.E.
        Squamous cell cancer of the liver arising from a solitary benign nonparasitic hepatic cyst.
        Am J Gastroenterol. 1988; 83: 426-431
        • Greenwood N.
        • Orr W.M.
        Primary squamous-cell carcinoma arising in a solitary non-parasitic cyst of the liver.
        J Pathol. 1972; 107: 145-148
        • Gresham G.A.
        • Rue 3rd, L.W.
        Squamous cell carcinoma of the liver.
        Hum Pathol. 1985; 16: 413-416
        • Nieweg O.
        • Slooff M.J.
        • Grond J.
        A case of primary squamous cell carcinoma of the liver arising in a solitary cyst.
        HPB surgery: a world journal of hepatic, Pancreatic and biliary surgery. 1992; 5: 203-208
        • Weimann A.
        • Klempnauer J.
        • Gebel M.
        • et al.
        Squamous cell carcinoma of the liver originating from a solitary non-parasitic cyst case report and review of the literature.
        HPB Surg. 1996; 10: 45-49
        • Monteagudo M.
        • Vidal G.
        • Moreno M.
        • et al.
        Squamous cell carcinoma and infection in a solitary hepatic cyst.
        Eur J Gastroenterol Hepatol. 1998; 10: 1051-1053
        • Yagi H.
        • Ueda M.
        • Kawachi S.
        • et al.
        Squamous cell carcinoma of the liver originating from non-parasitic cysts after a 15 year follow-up.
        Eur J Gastroenterol Hepatol. 2004; 16: 1051-1056
        • Boscolo G.
        • Jirillo A.
        • Da Pian P.
        Complete remission of poorly differentiated squamous liver carcinoma after systemic chemotherapy and surgery. A case report.
        Tumori. 2005; 91: 71-72
        • Zhang X.-F.
        • Du Z.-Q.
        • Liu X.-M.
        • Lv Y.
        Primary squamous cell carcinoma of liver: case series and review of literatures.
        Medicine. 2015; 94
        • Kaji R.
        • Sasaki N.
        • Tateishi I.
        • et al.
        A case report of primary hepatic squamous cell carcinoma that remarkably responded to low dose arterial injection of anti-cancer drugs.
        Kurume Med J. 2003; 50: 71-75