A rare case of plexiform neurofibroma of the liver in a patient without neurofibromatosis type 1
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CASE REPORT
A rare case of plexiform neurofibroma of the liver in a patient without neurofibromatosis type 1 Hiroko Nebiki1 · Shinsuke Hiramatsu1 · Yuhei Sakata1 · Takehisa Suekane1 · Tomoaki Yamasaki1 · Takashi Nakai2 · Yoichi Koda3 · Akishige Kanazawa4 · Takeshi Inoue5 Received: 15 July 2020 / Accepted: 27 August 2020 © Japanese Society of Gastroenterology 2020
Abstract Plexiform neurofibroma is mainly associated with neurofibromatosis type 1 and is seldom observed in the liver. Its occurrence in the liver without neurofibromatosis type 1 is even rarer. We report an extremely rare case of plexiform neurofibroma of the liver diagnosed by laparoscopic biopsy in a patient without neurofibromatosis type 1. The patient was a 35-year-old man who had neither clinical signs nor any family history of neurofibromatosis type 1. Abdominal ultrasonography, as part of a health screening, had detected a hepatic tumor. Subsequent contrast ultrasonography, computed tomography, and magnetic resonance imaging showed the tumor extending from the retroperitoneal space around the aorta to the hepatic hilum and distal portal branches in the right hepatic lobe, gallbladder, and left hepatic lobe. 18F-fluorodeoxyglucose positron emission tomography showed no abnormal accumulation. Histopathological examination of the tumor obtained laparoscopically led to a diagnosis of plexiform neurofibroma. Because the patient was asymptomatic with no features of malignancy, he was only monitored and managed. At follow-up 10 years later, computed tomography showed a decrease in tumor size. It is important to recognize that, while rare, plexiform neurofibroma can occur without neurofibromatosis type 1. We recommend follow-up instead of unreasonable surgery in such cases. Keywords Plexiform neurofibroma · Solitary neurofibroma · Hepatic tumor
Introduction
* Hiroko Nebiki [email protected] 1
Department of Gastroenterology, Osaka City General Hospital, 2‑13‑22, Miyakojima‑hondori, Miyakojima‑ku, Osaka 534‑0021, Japan
2
Department of Hepatology, Osaka City General Hospital, 2‑13‑22, Miyakojima‑hondori, Miyakojima‑ku, Osaka 534‑0021, Japan
3
Department of Radiology, Osaka City General Hospital, 2‑13‑22, Miyakojima‑hondori, Miyakojima‑ku, Osaka 534‑0021, Japan
4
Department of Hepato‑Biliary‑Pancreatic Surgery, Osaka City General Hospital, 2‑13‑22, Miyakojima‑hondori, Miyakojima‑ku, Osaka 534‑0021, Japan
5
Department of Pathology, Osaka City General Hospital, 2‑13‑22, Miyakojima‑hondori, Miyakojima‑ku, Osaka 534‑0021, Japan
Plexiform neurofibroma is a type of neurofibroma, mainly associated with neurofibromatosis type 1 (NF1) [1]. Plexiform neurofibroma is found in approximately 15–30% of patients with NF1 [2, 3] and is usually diagnosed in childhood. It is a benign tumor of the peripheral nerves, but can eventually transform into a malignant tumor in about 10% of the cases [4, 5]. It is usually found in the head and neck, extremities, or pelvis [1], but seldom observed in the liver [6–15]. Furthermore, plexiform neurofibro
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