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A case of hepatoblastoma misdiagnosed as combined hepatocellular carcinoma and cholangiocarcinoma in an adult.

Park KW, Seo CJ, Yun DY, Kim MK, Kim BS, Han YS, Oh HK, Lee CH - Clin Mol Hepatol (2015)

Bottom Line: Follow-up abdominal CT performed 3 months later showed a 5.5-cm metastatic mass in the left subphrenic area.Metastatic hepatoblastoma was found at multiple sites of the abdomen during follow-up, and so chemotherapy with cisplatin, 5-fluorouracil (5-FU), and vincristine was applied, followed by carboplatin and doxorubicin.Despite surgery and postoperative chemotherapy, she died 12 months after symptom onset.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Catholic University of Daegu School of Medicine, Daegu, Korea.

ABSTRACT
Hepatoblastoma usually occurs in children under the age of 2 years, with very few cases reported in adults. We experienced a case of adult hepatoblastoma in a 36-year-old female with chronic hepatitis B. She had experienced sudden onset abdominal pain. Her serum alpha-fetoprotein level was markedly elevated, and abdominal CT showed a 9-cm mass with internal hemorrhage in the right hepatic lobe with hemoperitoneum, so an emergency hepatic central bisectionectomy was performed. The initial histologic examination revealed that the mass mimicked combined hepatocellular carcinoma and cholangiocarcinoma with spindle-cell metaplasia of the cholangiocarcinoma element. Follow-up abdominal CT performed 3 months later showed a 5.5-cm metastatic mass in the left subphrenic area. Laparoscopic splenectomy with mass excision was performed, and hepatoblastoma was confirmed histologically. A histologic re-examination of previously obtained surgical specimens also confirmed the presence of hepatoblastoma. Metastatic hepatoblastoma was found at multiple sites of the abdomen during follow-up, and so chemotherapy with cisplatin, 5-fluorouracil (5-FU), and vincristine was applied, followed by carboplatin and doxorubicin. Despite surgery and postoperative chemotherapy, she died 12 months after symptom onset.

No MeSH data available.


Related in: MedlinePlus

Follow-up CT scans in the arterial (A) and portal (B) phases and PET (C) scans obtained 1 month after the second resection. (A, B) A newly developed 1.7-cm subtle enhancing soft-tissue mass was evident in the splenic bed. (C) Multiple areas of FDG uptake (maximum SUV > 3.80) were present in the left upper and lower quadrants of the abdomen and in the paralumbar area including the right subphrenic area.
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Figure 5: Follow-up CT scans in the arterial (A) and portal (B) phases and PET (C) scans obtained 1 month after the second resection. (A, B) A newly developed 1.7-cm subtle enhancing soft-tissue mass was evident in the splenic bed. (C) Multiple areas of FDG uptake (maximum SUV > 3.80) were present in the left upper and lower quadrants of the abdomen and in the paralumbar area including the right subphrenic area.

Mentions: A 36-year-old female presented at the emergency department with aggravating right upper abdominal pain for 2 hours. The patient was diagnosed hepatitis B virus (HBV) carrier for several years and non-alcoholics. No other specific personal and familial medical history was noted. Initial blood pressure was 100/60 mmHg, pulse rate 70/min, respiration rate 20/min, body temperature 37.5℃. The laboratory findings were white blood cell 12,000/mm3 (poly: 70%), hemoglobin 12.8 g/dL, platelet 198,000/mm3, prothrombin time 14.3 seconds, international normalized ratio 1.11, aspartate aminotransferase 22 IU/L, alanine aminotransferase 12 IU/L, total bilirubin 0.5 mg/dL, alkaline phosphatase 134 IU/L, lactate dehydrogenase 295 IU/L, gamma-glutamyl transpeptidase 26 IU/L, protein/albumin 6.4/4.0 g/dL, uric acid 5.0 mg/dL, blood urea nitrogen/creatinine 16.6/0.7 mg/dL. And serum viral markers were HBsAg (+), anti-HBs (-), anti-HBc (+), HBeAg (-), anti-HBe (+), HBV-DNA <20 IU/mL, anti-HCV (-) and anti-HIV (-). The patient's serum α-fetoprotein (AFP) was 676.5 ng/mL and carbohydrate antigen 19-9 (CA19-9) <0.6 U/mL. She took an abdominal CT scan, which showed a 9 cm sized necrotic mass with internal hemorrhage at the right hepatic lobe and ruptured to peritoneum (Fig. 1A), so an emergency hepatic central bisectionectomy was done (Fig. 2A). The initial histological diagnosis was cHCC-CC with spindle cell metaplasia of cholangiocarcinoma element (Fig. 2B). The serum AFP was decreased to 7.67 ng/mL at time of discharge. Regarding as cHCC-CC, postoperative adjuvant chemotherapy with tegafur/uracil (UFT) was administrated for 3 months. 3 months later, follow-up abdominal CT scanning showed previously unseen a 5.5 cm sized left subphrenic mass with mild enhancement in delayed image (Fig. 3A, B) and AFP was increased to 312.06 ng/mL. She underwent laparoscopic splenectomy with mass excision. On histologic examination, mesenchymal elements consisted of a proliferation of primitive-appearing mesenchymal spindle-shaped cells, intimately admixed with the epithelial elements in a highly cellular pattern. Cytoplasm was more abundant than that of mature fibroblasts, and the nucleus was elongated and plump. These cells blended progressively with areas of less intense cellular mesenchymal proliferation, and with relatively acellular, fibrous septa. Osteoid was present either within the primitive mesenchyme, near the fibrous septa or pseudocapsule, or admixed within the epithelial elements. Osteoid foci contained cells morphologically identical to osteoblasts (Fig. 4A). The immunohistochemistry stains showed expression of hepatocyte, β-HCG, AFP, vimentin, CK7, CK19, CD56 and β-catenin and negativity for CEA. (Fig. 4B, C, D, E, F, G). Metastatic hepatoblastoma was confirmed by histologic examination with immunohistochemistry stains, so immunohistochemistrically re-examination of previous surgical specimens was also confirmed as hepatoblastoma. Follow-up abdominal CT scan performed 1 month after reoperation showed a newly onset 1.7 cm sized subtle enhancing soft tissue mass in splenic bed, and positron emission tomography (PET) showed multiple FDG uptake(max SUV > 3.80) in left upper and lower quadrant area of abdomen, paralumbar area including right subphrenic area (Fig. 5A, B, C). Follow-up AFP was 162.69 ng/mL.


A case of hepatoblastoma misdiagnosed as combined hepatocellular carcinoma and cholangiocarcinoma in an adult.

Park KW, Seo CJ, Yun DY, Kim MK, Kim BS, Han YS, Oh HK, Lee CH - Clin Mol Hepatol (2015)

Follow-up CT scans in the arterial (A) and portal (B) phases and PET (C) scans obtained 1 month after the second resection. (A, B) A newly developed 1.7-cm subtle enhancing soft-tissue mass was evident in the splenic bed. (C) Multiple areas of FDG uptake (maximum SUV > 3.80) were present in the left upper and lower quadrants of the abdomen and in the paralumbar area including the right subphrenic area.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4612292&req=5

Figure 5: Follow-up CT scans in the arterial (A) and portal (B) phases and PET (C) scans obtained 1 month after the second resection. (A, B) A newly developed 1.7-cm subtle enhancing soft-tissue mass was evident in the splenic bed. (C) Multiple areas of FDG uptake (maximum SUV > 3.80) were present in the left upper and lower quadrants of the abdomen and in the paralumbar area including the right subphrenic area.
Mentions: A 36-year-old female presented at the emergency department with aggravating right upper abdominal pain for 2 hours. The patient was diagnosed hepatitis B virus (HBV) carrier for several years and non-alcoholics. No other specific personal and familial medical history was noted. Initial blood pressure was 100/60 mmHg, pulse rate 70/min, respiration rate 20/min, body temperature 37.5℃. The laboratory findings were white blood cell 12,000/mm3 (poly: 70%), hemoglobin 12.8 g/dL, platelet 198,000/mm3, prothrombin time 14.3 seconds, international normalized ratio 1.11, aspartate aminotransferase 22 IU/L, alanine aminotransferase 12 IU/L, total bilirubin 0.5 mg/dL, alkaline phosphatase 134 IU/L, lactate dehydrogenase 295 IU/L, gamma-glutamyl transpeptidase 26 IU/L, protein/albumin 6.4/4.0 g/dL, uric acid 5.0 mg/dL, blood urea nitrogen/creatinine 16.6/0.7 mg/dL. And serum viral markers were HBsAg (+), anti-HBs (-), anti-HBc (+), HBeAg (-), anti-HBe (+), HBV-DNA <20 IU/mL, anti-HCV (-) and anti-HIV (-). The patient's serum α-fetoprotein (AFP) was 676.5 ng/mL and carbohydrate antigen 19-9 (CA19-9) <0.6 U/mL. She took an abdominal CT scan, which showed a 9 cm sized necrotic mass with internal hemorrhage at the right hepatic lobe and ruptured to peritoneum (Fig. 1A), so an emergency hepatic central bisectionectomy was done (Fig. 2A). The initial histological diagnosis was cHCC-CC with spindle cell metaplasia of cholangiocarcinoma element (Fig. 2B). The serum AFP was decreased to 7.67 ng/mL at time of discharge. Regarding as cHCC-CC, postoperative adjuvant chemotherapy with tegafur/uracil (UFT) was administrated for 3 months. 3 months later, follow-up abdominal CT scanning showed previously unseen a 5.5 cm sized left subphrenic mass with mild enhancement in delayed image (Fig. 3A, B) and AFP was increased to 312.06 ng/mL. She underwent laparoscopic splenectomy with mass excision. On histologic examination, mesenchymal elements consisted of a proliferation of primitive-appearing mesenchymal spindle-shaped cells, intimately admixed with the epithelial elements in a highly cellular pattern. Cytoplasm was more abundant than that of mature fibroblasts, and the nucleus was elongated and plump. These cells blended progressively with areas of less intense cellular mesenchymal proliferation, and with relatively acellular, fibrous septa. Osteoid was present either within the primitive mesenchyme, near the fibrous septa or pseudocapsule, or admixed within the epithelial elements. Osteoid foci contained cells morphologically identical to osteoblasts (Fig. 4A). The immunohistochemistry stains showed expression of hepatocyte, β-HCG, AFP, vimentin, CK7, CK19, CD56 and β-catenin and negativity for CEA. (Fig. 4B, C, D, E, F, G). Metastatic hepatoblastoma was confirmed by histologic examination with immunohistochemistry stains, so immunohistochemistrically re-examination of previous surgical specimens was also confirmed as hepatoblastoma. Follow-up abdominal CT scan performed 1 month after reoperation showed a newly onset 1.7 cm sized subtle enhancing soft tissue mass in splenic bed, and positron emission tomography (PET) showed multiple FDG uptake(max SUV > 3.80) in left upper and lower quadrant area of abdomen, paralumbar area including right subphrenic area (Fig. 5A, B, C). Follow-up AFP was 162.69 ng/mL.

Bottom Line: Follow-up abdominal CT performed 3 months later showed a 5.5-cm metastatic mass in the left subphrenic area.Metastatic hepatoblastoma was found at multiple sites of the abdomen during follow-up, and so chemotherapy with cisplatin, 5-fluorouracil (5-FU), and vincristine was applied, followed by carboplatin and doxorubicin.Despite surgery and postoperative chemotherapy, she died 12 months after symptom onset.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Catholic University of Daegu School of Medicine, Daegu, Korea.

ABSTRACT
Hepatoblastoma usually occurs in children under the age of 2 years, with very few cases reported in adults. We experienced a case of adult hepatoblastoma in a 36-year-old female with chronic hepatitis B. She had experienced sudden onset abdominal pain. Her serum alpha-fetoprotein level was markedly elevated, and abdominal CT showed a 9-cm mass with internal hemorrhage in the right hepatic lobe with hemoperitoneum, so an emergency hepatic central bisectionectomy was performed. The initial histologic examination revealed that the mass mimicked combined hepatocellular carcinoma and cholangiocarcinoma with spindle-cell metaplasia of the cholangiocarcinoma element. Follow-up abdominal CT performed 3 months later showed a 5.5-cm metastatic mass in the left subphrenic area. Laparoscopic splenectomy with mass excision was performed, and hepatoblastoma was confirmed histologically. A histologic re-examination of previously obtained surgical specimens also confirmed the presence of hepatoblastoma. Metastatic hepatoblastoma was found at multiple sites of the abdomen during follow-up, and so chemotherapy with cisplatin, 5-fluorouracil (5-FU), and vincristine was applied, followed by carboplatin and doxorubicin. Despite surgery and postoperative chemotherapy, she died 12 months after symptom onset.

No MeSH data available.


Related in: MedlinePlus