Malignant Peripheral Nerve Sheath Tumor of the Liver

Kakizaki, Satoru; Horiguchi, Norio; Otsuka, Toshiyuki; Takizawa, Daichi; Yamazaki, Yuichi; Sato, Ken; Ohno, Yoshihiro; Kusano, Motoyasu; Yamada, Masanobu

doi:10.2169/internalmedicine.55.5456

Cited by 5 publications

(2 citation statements)

References 19 publications

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“…Primary hepatic MPNST is very rare with only 11 case reports in the literature. To our knowledge, our case is the 12th [234]. Nine of them are not associated with NF-1.…”

Section: Discussionmentioning

confidence: 92%

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Primary hepatic malignant peripheral nerve sheath tumor successfully treated with combination therapy: a case report and literature review

et al. 2016

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Primary malignant peripheral nerve sheath tumor (MPNST) in a young female patient, not associated with neurofibromatosis type-I is extremely rare in the liver. A 33-year-old female was admitted with a right flank pain for a weak. The CT scan showed 12.5-cm-sized mass located at the right hepatic lobe. At laparotomy, about 20.0-cm-sized mass was on the right hepatic lobe with attachment to right diaphragmatic pleura. Right hepatic lobe and adherent part of diaphragmatic pleura were resected. On histology and immunohistochemistry, it was diagnosed MPNST. Adjuvant radiotherapy for the right diaphragmatic pleura and adjuvant chemotherapy with adriamycin, ifosfamide and cisplatin were sequentially performed. The prognosis of MPNST is generally poor and it is associated with a highly aggressive course of recurrence, metastases, and death. Our case is probably a first report about combination therapy.

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“…Primary hepatic MPNST is very rare with only 11 case reports in the literature. To our knowledge, our case is the 12th [234]. Nine of them are not associated with NF-1.…”

Section: Discussionmentioning

confidence: 92%

“…They are rarely found on the abdominal organ or cavity [1]. More than half of them occur related to neurofibromatosis type-I (NF-1), so the primary hepatic MPNST cases without NF-1 have been reported rarely [234]. To date, only 11 cases have been reported in the literature [2].…”

Section: Introductionmentioning

confidence: 99%

Primary hepatic malignant peripheral nerve sheath tumor successfully treated with combination therapy: a case report and literature review

et al. 2016

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Neural and neurogenic tumours of the gastroenteropancreaticobiliary tract

2018

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Neural lesions occur uncommonly in the gastroenteropancreaticobiliary tract. However, due to the growing number of screening colonoscopy procedures, polypoid neural lesions of the colon are being recognised increasingly and range from benign tumours to high-grade malignant neoplasms. Morphological variability of neural tumours can be wide, although some entities share pathological features, and, as such, these lesions can be diagnostically challenging. We review the spectrum of pathology of neural tumours in the gastroenteropancreaticobiliary tract, with the goal of providing a practical approach for practising surgical pathologists.

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Case report of portal hepatic schwannoma: presentation of multimodality images

Amano

Takagi

et al. 2021

BMC Gastroenterol

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Background Portal hepatic schwannoma is a rare benign tumor and difficult to diagnose preoperatively because of its rarity and imaging manifestations that mimic malignancy. We present a case of portal hepatic schwannoma that showed moderate contrast enhancement on computed tomography (CT), extension along the bile duct on T2-weighted imaging and magnetic resonance cholangiopancreatography (MRCP), and uptake of 18F-fluorodeoxyglucose (FDG) on positron emission tomography. Case presentation Ultrasonography at an annual health checkup identified a hepatic mass in a 38-year-old woman. CT showed a well-defined portal hepatic tumor with mild contrast enhancement. T2-weighted imaging and MRCP showed a clavate tumor extending along the intrahepatic bile ducts but no dilatation of the ducts. The tumor exhibited increased FDG uptake, such as maximum standardized uptake values of 5.0 and 6.5 in the early and late phases, respectively. Neither dilatation of intrahepatic bile ducts nor lymphadenopathy was identified, and the multimodality imaging suggested hepatic portal lymphoma, gastrointestinal tumor, or IgG4-related disease rather than cholangiocarcinoma. A needle biopsy via endoscopic ultrasonography was performed, and immunohistology confirmed the tumor as a schwannoma. Conclusions The diagnosis of a portal hepatic schwannoma requires immunohistological examinations in addition to multimodality imaging studies to reflect fully the pathohistological characteristics of the tumor.

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Malignant Peripheral Nerve Sheath Tumor of the Liver

Cited by 5 publications

References 19 publications

Primary hepatic malignant peripheral nerve sheath tumor successfully treated with combination therapy: a case report and literature review

Primary hepatic malignant peripheral nerve sheath tumor successfully treated with combination therapy: a case report and literature review

Neural and neurogenic tumours of the gastroenteropancreaticobiliary tract

Case report of portal hepatic schwannoma: presentation of multimodality images

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