BackgroundSchwannomas located in the periportal region are extremely rare. Only 14 cases have been reported in the medical literature worldwide. Cases of porta hepatic schwannomas reported in the literature worldwide were reviewed. As a result, it is very challenging for surgeons to make a preoperative diagnosis due to its rarity and nonspecific imaging manifestations.Case PresentationA 57-year-old Chinese female was admitted to our institution with complaint of upper abdominal distension and the abdominal CT in the local hospital revealed a hypodense mass in the porta hepatis. A fine needle aspiration (FNA) was made to confirm the diagnosis, but the result was just suggestive of spindle cell neoplasia. Eventually, the patient underwent surgery and postoperative pathology confirmed schwannoma in porta hepatis. The patient recovered uneventfully with no evidence of recurrence after a follow-up period of 41 months.ConclusionsIt is essential for the final diagnosis of porta hepatic schwannomas to combine histological examination with immunohistochemistry after surgery. The main treatment of porta hepatic schwannomas is complete excision with free margins and no lymph node dissection. In some cases, biliary reconstruction or the proper hepatic and the gastroduodenal artery resection was performed because the tumor was inseparably attached to the extrahepatic bile duct or the proper hepatic and the gastroduodenal artery. Malignant transformation of schwannomas is very rare and the overall prognosis is satisfactory.
Abstract. Hepatic schwannoma is a rare benign disease with a good prognosis. Early diagnosis is difficult due to the absence of specific clinical presentations and its rarity. The present study briefly described a 64-year-old female patient with hepatic schwannoma mimicking intrahepatic cholangiocarcinoma. Furthermore, the clinical data of 30 patients with hepatic schwannoma were also reviewed and analyzed. The mean age of the 30 patients was 51.7 years (range, 21-83 years) and ~2/3 were female. All patients in the benign group underwent surgical treatment and survived until the last follow-up, of whom 19 received complete resection and the remaining 1 underwent liver transplantation. However, in the malignant group, only three cases who underwent the surgical resection remained alive at last follow-up. Another seven cases were succumbed to mortality, 4 cases of whom had deteriorated to have no operation opportunity by the time they saw a doctor, and among the remaining three cases with hepatectomy, 1 died of liver dysfunction at 21 days postoperatively, 2 succumbed to recurrences at 18 and 23 months postoperatively. In conclusion, hepatic schwannoma is a rare benign disease with a good prognosis. However, once the malignant transformation occurs, the prognosis is not satisfied. Complete resection is the mainstay for cure and liver transplantation is often necessary.
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