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International Journal of Surgery Case Reports
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INTERNATIONAL JOURNAL OF SURGERY CASE REPORTS
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Case report
Adrenocortical carcinoma mimicking hepatocellular carcinoma: A case report
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Takahiro Ikeda ª,”, Amane Kitasato ª, Tatsuya Okamoto ª, Momoko Soedab, Shiro Miura b, Tamotsu Kuroki ª
a Department of Surgery, National Hospital Organization Nagasaki Medical Center, Japan
b Department of Diagnostic Pathology, National Hospital Organization Nagasaki Medical Center, Japan
ARTICLE INFO
Keywords:
Adrenocortical carcinoma Hepatocellular carcinoma Imaging findings Histologic findings ACC HCC
ABSTRACT
Introduction and importance: Adrenocortical carcinoma (ACC) is a relatively rare tumor arising in the adrenal cortex. Its imaging and histopathologic findings are not well known to be similar to those of hepatocellular carcinoma (HCC). We report here a case of ACC with hepatic resection in the preoperative diagnosis of HCC. Case presentation: A 46-year-old woman was noted to have a tumor 45 mm in size in the segment 7 of the liver on CT during a medical checkup. The tumor had consistent imaging findings as HCC on Ultrasound, CT, and MRI examinations, and the result of the liver tumor biopsy was a diagnosis of intermediate differentiated HCC. We considered the tumor to be HCC and performed a posterior segmentectomy with combined resection of the right adrenal gland, which had adhesions suspected to direct invasion. The pathology of the resected specimen confirmed the diagnosis of ACC with direct invasion into the liver.
Clinical discussion: ACC may show a contrast pattern similar to that of HCC on imaging, and histopathology may show atypical cells with eosinophilic sporulation, similar to that of HCC. Our case serves to alert physicians that ACC should be considered a differential diagnosis in patients with suspected HCC in the posterior segment. Conclusion: Tumors suspected of HCC in the dorsal posterior segment of the liver should be considered as possible ACC.
1. Introduction
Adrenocortical carcinoma (ACC) is a relatively rare tumor arising in the adrenal cortex with a reported annual incidence of 0.5-2 per million population [1]. It is not well known that the imaging and histopatho- logic findings of ACC similar to those of hepatocellular carcinoma (HCC). We experienced a case of ACC that underwent hepatectomy with preoperative diagnosis of HCC, and present this case as a challenge to clinicians and pathologists. This case has been reported in line with the SCARE 2020 criteria [2].
2. Presentation of case
A 46-year-old woman was referred to our hospital after a CT scan of her medical examination revealed a 45 mm tumor on the segment 7 of the liver. She had underlying hypertension. She was not a habitual drinker and was negative for both hepatitis B virus and hepatitis C virus.
The biochemical examination showed normal coagulation function, liver enzymes, and tumor markers. Tumor markers were measured for carcinoembryonic antigen (CEA) at 3.71 ng/ml, carbohydrate antigen 19-9 (CA19-9) at 2.07 U/ml, «-fetoprotein (AFP) at 6 ng/ml, and protein induced by vitamin K absence or antagonist-II (PIVKA-II) at 27 mAU/ml. Ultrasonography showed a well-defined hypoechoic mass on the dorsal aspect of hepatic segment 7 (Fig. 1). Abdominal CT scan showed arterial enhancement (Fig. 2A) and delayed washout lesions (Fig. 2B) with mosaic structure and capsules, consistent with simple nodular HCC. Magnetic resonance imaging (MRI) showed high signal in diffusion- weighted images (Fig. 3A) and decreased uptake in the hepatocellular contrast phase (Fig. 3B). CT and MRI showed the right adrenal gland, and the liver tumor was thought to be in contact with the right adrenal gland. Histopathological examination of tumor biopsy showed hyper- cellularity of eosinophilic cells with a high nuclear/cytoplasmic ratio, consistent with a moderately differentiated HCC (Fig. 4A). Preoperative diagnosis of HCC led to a posterior segmentectomy with
* Corresponding author at: Department of Surgery, National Hospital Organization Nagasaki Medical Center, 2-1001-1 Kubara, Ohmura-shi, Nagasaki 856-8562, Japan.
https://doi.org/10.1016/j.ijscr.2023.108122
H
cholecystectomy. Intraoperatively, the right adrenal gland, which had adhesions suspected to direct invasion, underwent a combined resection (Fig. 4B). We considered it a right adrenal invasion of HCC. The patient had a good postoperative course and was discharged on the 8th post- operative day. Grossly, the tumor showed a yellowish-white, well- defined, single nodular lesion covered by a fibrous capsule. It was also suggested that the tumor may have grown from the adrenal side into the liver (Fig. 4C). As with the preoperative biopsy tissue, the hematoxylin- eosin (HE) staining of tumor tissue showed atypical cells with eosino- philic endoplasmic reticulum, similar to HCC (Fig. 4D). Tumor tissue showed positive immunohistochemical staining for Melan A (Fig. 4E) and negative staining for Hepatocyte (Fig. 4F). The pathology diagnosis was direct invasion of the liver by ACC. It was Stage IV according to the World Health Organization (WHO) classification, with a resection status
of R0, and Ki67 was found to occur in 20 % of hot spots. The patient is currently undergoing chemotherapy and has been recurrence-free for 17 months postoperatively.
3. Discussion
ACC is a relatively rare tumor of the adrenal cortex. ACC can occur at any age, but there is a bimodal age distribution, with the disease reaching its peak before the age of five and the second peak appearing in the 40s and 50s of life [3]. Most nonfunctioning ACC are asymptomatic and are often discovered incidentally. The present case was also detected incidentally on CT during a medical examination. ACC is generally re- fractory to chemotherapy and radiation therapy and has a poor prog- nosis, but complete resection is expected to improve the prognosis [4]. Imaging findings of adrenocortical tumors, including ACC, have been reported to show heterogeneous contrast on CT and contrast patterns similar to HCC [5-10]. Histopathologically, ACC also resembles HCC with atypical cell proliferation with eosinophilic hydatid cytoplasm [11]. Generally, preoperative biopsy for liver tumors is not aggressively recommended because of the slight risk of seeding. In the present case, since the background liver was normal, a preoperative biopsy was per- formed using a 21G biopsy needle under ultrasonography guidance to aid in the diagnosis. ACC itself is a rare disease, and there are few reports of cases showing that it was difficult to differentiate from HCC. The following reports show clinical features of ACC misdiagnosed as HCC [7,11,12] (Table.1). Case 1 had a history of heavy alcohol consumption and also a history of eating raw freshwater fish, and Case 3 had a history of chronic hepatitis C, making the diagnosis of ACC more difficult to differentiate from HCC. Case 2, Case 3, and our case were misdiagnosed as HCC, although biopsy was even performed. In the present case, because of the suspicion of a primary adrenal tumor on gross findings, Melan A, Calretinin, and Hepatocyte were added as immunohisto- chemical stains to the resected tumor tissue. For hepatocellular tumors, Melan A and Calretinin stain negative, while Hepatocyte stains positive. Ghorab Z, et al. reported that Melan A is expressed in ACC but not in HCC and renal cell carcinoma, and may be useful in differentiating them [13]. We discussed the histopathologic findings of ACC with the pathologist. According to them, it is a histopathologic finding consistent with HCC without immunohistochemical staining. We consider that the presence of a gross image contiguous with the adrenal gland and immunohistochemical staining would be useful in differentiating the diagnosis.
Standard chemotherapy for ACC has not been established. According to the WHO classification, this patient was Stage IV and was considered a high-risk case for recurrence. There is a report that adjuvant mitotane therapy may prolong recurrence-free survival [14], and after consulta- tion with the patient, our hospital initiated adjuvant mitotane therapy.
(A)
(B)
Arterial phase
Portal phase
(A)
Diffusion weighted image
(B)
Hepatocellular contrast phase
(A)
(B)
(C)
adrenal grand
10
20
25
30
Biopsy
(D)
(E)
(F)
HE
Melan A
Hepatocyte
Clinicians should be aware of the difficulty in differentiating ACC from HCC on imaging and histopathologic findings. Our case serves to alert physicians that ACC should be considered a differential diagnosis in patients with suspected HCC in the dorsal posterior hepatic segment. We make several recommendations to differential diagnosis between pos- terior segment non functioning ACC and HCC. Preoperative recom- mendation is to check closely for continuity with the right adrenal gland by CT or MRI. The intraoperative recommendation is to check for con- tinuity with the right adrenal gland. Pathologic recommendations are to consult with a pathologist and perform additional immunohistochem- ical staining.
4. Conclusion
Tumors suspicious for HCC in the dorsal posterior hepatic segment should be considered for possible ACC, and additional immunohisto- chemical staining should be performed in consultation with the pathologist.
Informed consent
Written informed consent was obtained from the patient for publi- cation of this case report and accompanying images. A copy of the
| Case no. | Sex/ age | Diameter location | Findings that highly mimic HCC | Liver status | Immunohistochemistry | Reference |
|---|---|---|---|---|---|---|
| 1 | M/75 | 6.0 cm segment 6 and 7 | CT | Alcohol | Melan A, alpha-inhibin, epithelial membrane antigen, S-100 protein, Chromogranin, HSA | [7] |
| 2 | M/73 | 7.1 cm segment 6 and 7 | biopsy | Non-B, non-C normal | Unknown | [12] |
| 3 | F/38 | 5.5 cm segment 7 | CT, MRI, biopsy | HCV-Ab(+) chronic hepatitis | Unknown | [11] |
| Our case | F/46 | 4.5 cm segment 7 | CT, MRI, biopsy | Non-B, non-C normal | Melan A, Calretinin, Hepatocyte, alpha-inhibin |
written consent is available for review by the Editor-in-Chief of this journal on request.
Ethical approval
Ethical approval was waived by the authors institution.
Funding
This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Guarantor
Takahiro Ikeda.
Research registration number
N/A.
CRediT authorship contribution statement
TI and AK: study concept
TI, AK, MS, SM: data collection, writing the paper TO, MS, SM, TK: reading and correcting the paper All authors: approving the final manuscript.
Declaration of competing interest
The authors have no conflicts of interest to declare.
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