A man aged 38 years was admitted to hospital with fever and a soft mass in the right hypochondrium. Laboratory investigations revealed minor changes in liver enzymes, a normal plasma bilirubin and an elevated serum level of CA19.9. At the time of a barium meal radiograph, faint, tooth-like calcification was noted in the upper abdomen. A computed tomography (CT) scan revealed a cystic structure, approximately 8 cm in diameter, with a fat-fluid level and calcification in the wall (Fig. 1). Compression of the bile duct and gallbladder were noted during endoscopic retrograde cholangiography. At operation, the tumor was located in the hepatoduodenal ligament. The tumor was excised, a cholecystectomy was performed and the bile duct was reconstructed using a Roux-en-Y choledochojejunostomy. The tumor contained sebaceous material, hair and teeth (Fig. 2), and was identified histologically as a mature cystic teratoma.Teratomas are rare neoplasms that develop from the three embryonic germ layers and can potentially contain tissue of any type. They arise from totipotential cell lines and are usually located in the gonads. However, extragonadal teratomas do occur, particularly in midline structures such as the anterior mediastinum, retroperitoneum and sacrococcygeal region. To our knowledge, there is only one previous report of a teratoma in the hepatoduodenal ligament. The majority of extragonadal teratomas (60%) show calcification on plain abdominal radiographs, either calcification in the wall of the cyst or in structures such as teeth or bones. A CT scan is usually the most helpful investigation. Serum levels of alpha fetoprotein, carcinoembyronic antigen and CA19.9 are elevated in some patients but this does not appear to be helpful clinically. The most important aspect of management is careful surgical excision. Overall, approximately 25% of extragonadal teratomas are malignant but this frequency is higher in solid teratomas and lower in cystic teratomas.
Background
Although the primary treatment option for hilar cholangiocarcinoma (HC) has been surgical resection, most patients present with unresectable advanced tumors at the time of diagnosis. Particle therapy (PT) holds a great potential for HC, even though the anatomical proximity to the gastrointestinal tract prevents delivering a radical dose to the tumor. Space-making PT (SMPT), consisting of spacer placement surgery and subsequent PT, has been developed to minimize complications and maximize the therapeutic benefit of dose escalation for HC. This study aimed to conduct a dosimetric evaluation and examine the effectiveness of SMPT for the treatment of HC.
Methods
Between 2007 and 2018, 12 patients with unresectable HC treated with SMPT were enrolled. The treatment outcomes and effectiveness of spacer placement surgery were evaluated through analyses of pre- and post- surgical parameters of dose volume histograms.
Results
All patients completed the planned SMPT protocol. The median survival time was 29.6 months, and the 1- and 3-year overall survival rates were 82.5% and 45.8%, respectively. The mean V95% value (volume irradiated with 95% of the planned treatment dose) of the gross tumor volume and clinical target volume after spacer placement surgery improved to 98.5% and 96.6% from preoperative values of 85.6% and 78.1%, respectively (p = 0.0196 and p = 0.0053 respectively). Grade 3 or higher adverse events after SMPT were seen in 6 patients.
Conclusions
SMPT led to improvements in dosimetric parameters and showed good feasibility and excellent outcomes. SMPT can be a promising novel alternative for unresectable HC.
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