Ann Hepatobiliary Pancreat Surg.  2022 Nov;26(4):407-411. 10.14701/ahbps.22-022.

Progression of a persisting mesenchymal hamartoma to intrahepatic cholangiocarcinoma 24 years after the initial diagnosis: A case report

• Gang S
• Choi Y
• Lee S
• Hong Sy
• Suh S
• Han ES
• Hong SK
• Yi NJ
• Lee KW
• Suh KS

• KMID: 2536392
• DOI: http://doi.org/10.14701/ahbps.22-022

Abstract

Mesenchymal hamartoma of the liver (MHL) is a rare benign tumor that often presents in early childhood, and it rarely occurs in adulthood. Aberrant development of the portal tract is a known cause of MHL. Although limited information is available on the natural course of MHL, malignant transformation has been reported in a few cases. Here, we report a case of a 26-year-old female with intrahepatic cholangiocarcinoma secondary to unresected MHL. The patient underwent resection of the hepatic mass, which was diagnosed as MHL at 2 years of age, due to an increase in mass size and a suspicion of malignant transformation during work-up. Histopathology confirmed intrahepatic adenosquamous carcinoma in the background of MHL, with a T2N0M0 pathological stage (stage II). The surgical margin was free from tumor cells. The patient fully recovered postoperatively and started receiving adjuvant chemotherapy. Previous case reports have only reported about the development of undifferentiated embryonal sarcoma or angiosarcoma as malignant transformation of MHL. Cases of other malignancies have not been published; however, it is difficult to rule out the occurrence of various malignancies related to the portal tract when considering the pathogenesis of the disease. To the best of our knowledge, this is the first case report of adenocarcinoma of bile duct origin secondary to MHL. This case report suggests that aggressive surgical management should be considered after the initial diagnosis of MHL.

Keyword

Hamartoma; Cholangiocarcinoma; Liver; Hepatectomy

Figure

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  Fig. 1 Right hepatic mass in imaging findings (A) Magnetic resonance imaging (MRI) at the initial diagnosis (at 16 months of age). On coronal view, a cystic mass measuring 9 cm × 8 cm is observed in the right lobe of the liver. (B) Follow-up ultrasonography (at 5 years old). Although the limitation of the test precludes an accurate evaluation of size, continuous monitoring shows that the mass with multiple cysts does not exhibit a rapid increase in size. (C) Computed tomography before the operation. A complex solid, cystic mass with thick wall and calcification in the liver segment 6 is observed along with an increase in size (12.9 cm × 8.3 cm). In the portal phase, a peripheral enhancing lesion measuring 6 cm is seen in the posterior portion. This indicates the possibility of abscess formation or malignant transformation to undifferentiated embryonal sarcoma. (D) MRI before the operation. In the T2 enhanced image, the lesion is observed with a solid component and peripheral rim-like enhancement along the posteroinferior portion of the mass. (E) F-18-fluorodeoxyglucose positron emission tomography (PET). The rim-like enhancement on MRI shows intense hypermetabolism on PET.

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  Fig. 2 Diagnosis of intrahepatic cholangiocarcinoma (iCCA) in the background of mesenchymal hamartoma of the liver (MHL). On gross pathology, a mass (size: 12.0 cm × 11.0 cm × 8.0 cm) with a necrotic portion (10%) and calcification is observed. Histopathology reveals iCCA in the background of mesenchymal hamartoma and indicates the presence of adenosquamous carcinoma with intrahepatic vascular invasion. Lymphatic and venous invasions are confirmed microscopically, although no perineural invasion is observed. Immunohistochemistry shows expression of cytokeratin (CK) 19 and p40 and loss of p53. This finding suggests a cancer with squamous differentiation. (A) Left ×2: Gross photos. Middle: H&E stain ×40. Right: H&E stain ×40. (B) CK19 statin (×40). (C) p40 stain (×40). (D) p53 stain (×40).

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  Fig. 3 Changes in the serum levels of tumor markers after treatment. Shortly after surgery, the levels of tumor markers were normalized. CEA, carcinoembryonic antigen; CA19-9, carbohydrate antigen 19-9; OP, operation.

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