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Journal of Clinical and Experimental Hepatology logoLink to Journal of Clinical and Experimental Hepatology
. 2022 Aug 31;13(1):182–186. doi: 10.1016/j.jceh.2022.08.006

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

Khai V Ninh ∗, Dang H Do ∗,∗, Tuan Hoang ∗, Phuong H Tran ∗, Lanh S Nguyen †, Nghia Q Nguyen ∗
PMCID: PMC9840083  PMID: 36647418

Abstract

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: squamous cell carcinoma, epidermoid cyst, hepatic cancer, right trisegmentectomy, case report

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.1 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.2 Overall survival for patients with primary SCC is poor, most patients die after less than one year.3 Treatment standards have not yet been published, but radical resection is considered the only curative option.2 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 1.

Figure 1

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Imaging of tumor in MRI.

Figure 2.

Figure 2

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Imaging 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 3.

Figure 3

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The gross image of the tumor.

Figure 4.

Figure 4

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Negative resection margin after hepatectomy.

Figure 5.

Figure 5

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Bilioenteric anastomosis of the bile duct.

Figure 6.

Figure 6

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The histopathological imaging of the tumor.

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.4,5 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.6

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.

Table 1.

Review of Literature About Epidermoid Cyst-related Squamous Cell Carcinoma.

No Age/Gender Type Site Treatment Survival (months) References
1. 59 F EC Right lobe Right hepatectomy 16, alive 7
2. 18 M EC Segment VIII; vascular, biliary invasion Right hepatectomy, Whipple's procedure 7 2
3. 59 F EC Right lobe Right hepatectomy 8, alive 1
4. 21 M EC Segment V, VI Right hepatectomy, right hemicolectomy, antrectomy 3, alive 5
5. 30 M EC Right lobe Extended right hepatic lobectomy 4 8
6. 63 M EC Left lobe Celiotomy, subtotal hepatic cystectomy 6 9
7. 37 M EC Both lobe Laparotomy 2 10
8. 78 M EC Segment IV, V, VIII Preservation 2 11
9. 62 F EC Right lobe with multiple organ invasion Laparotomy 5 12
10. 74 F EC Segment VI, VII, VIII Right hepatectomy 54, alive 13
11. 71 F EC Right lobe Cystostomy 1 14
12. 42 M EC Both lobe Preservation 8 (180 since the cyst found) 15
13. 45 F EC Segment IV, V, VIII Right trisegmentectomy 23, alive Our case
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Abbreviation: EC, Epidermoid cysts.

As the number of cases and evidence regarding systemic chemotherapy for SCC is limited, complete resection seems to be the only curative treatment.16, 17, 18 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.

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