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.
Systemic chemotherapy started with cisplatin (60 mg/m2), 5-fluorourasil (5-FU) (600 mg/m2), vincristine (1.5 mg/m2) and total 3 cycles of chemotherapy were done every 4 week.
Entecavir 0.5 mg was also started for chemoprophylaxis of CHB.
After the 3rd chemotherapy cycle, follow-up abdominal CT and PET scan showed progression of multiple intraperitoneal metastasis with large amount of intraperitoneal fluid (Fig.6A, B) and follow-up AFP was further increased to 254 ng/mL.
So chemotherapy regimen was changed to carboplatin (350 mg/m2) with doxorubicin (30 mg/m2) every 3 weeks.
The patient experienced neutropenia after the new regimen, but recovered shortly after treatment with granulocyte colony-stimulating factor (G-CSF).
After second carboplatin with doxorubicin chemotherapy, follow-up AFP was increased 1510.19 ng/mL, but abdominal CT scan showed that amount of ascites was decreased (Fig.7).
Because of radiologic improvements, third and fourth chemotherapy were performed.
After 4th chemotherapy, follow-up abdominal CT revealed progression of multiple intraperitoneal metastatic masses, newly developed hepatic metastasis and large amount of intraperitoneal fluid (Fig.8).
A diagnostic paracentesis was performed and showed white blood cell 310/mm3 (poly 20%, lymph 80%), protein 3.6 g/dL and albumin 2.3 g/dL on ascitic fluid analysis.
The serum albumin was 3.1 g/dL and serum-ascites albumin gradient (SAAG) was 0.8 g/dL.
The AFB stain and culture were all negative in ascitic fluid.
The AFP was 6881.93 ng/mL on serum.
Regarded as peritoneal carcinomatosis, the patient underwent conservative treatments including the use of repeated therapeutic paracentesis for 1 month before death.