Malignant Peripheral Nerve Sheath Tumor of the Liver: A Case Report

Kopper, László; Nagy, Péter; Máthé, Z.; Hartmann, Erika; Doros, Attila; Paku, Sándor; Dezső, Katalin; Szepes, Zoltán

doi:10.1007/s12253-008-9078-0

Cited by 18 publications

(14 citation statements)

References 9 publications

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“…A leiomyoma would be negative for S-100 and positive for desmin or SMA (8). When the hepatic schwannoma presented with numerous sub-foci around or grown rapidly or associated with a highly-aggressive course of recurrence during follow-up, it almost certainly was the malignant type (9). In the present statistics, of the 9 malignant schwannoma cases, 1 was semi-malignant and 20 were benign.…”

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confidence: 53%

“…In the present statistics, of the 9 malignant schwannoma cases, 1 was semi-malignant and 20 were benign. Although the tumor in certain cases was reported to be S-100 negative, other characteristics of it proved the malignant nature (9). When the patient wants to take a conservative treatment, the pathology and immunohistochemistry of the liver tumor tissue must be performed to exclude the malignant transformation, since survival with observation alone for malignant schwannoma is poor (10).…”

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confidence: 99%

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Hepatic schwannoma: A case report and an updated 40-year review of the literature yielding 30 cases

Wan

Zhai

Ren

et al. 2016

Molecular and Clinical Oncology

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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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confidence: 53%

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confidence: 99%

Hepatic schwannoma: A case report and an updated 40-year review of the literature yielding 30 cases

Wan

Zhai

Ren

et al. 2016

Molecular and Clinical Oncology

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“…Distant metastases to the lung, lymph nodes, and liver have also been reported (2, 15, 16). However, primary hepatic MPNST is extremely rare (3)(4)(5)(6)(7)(8)(9)(10)(11)(12). To the best of our knowledge, only 11 cases (including our own case), of an MPNST arising from the liver have been reported (Table).…”

Section: Discussionmentioning

confidence: 91%

“…To the best of our knowledge, only 11 cases (including our own case), of an MPNST arising from the liver have been reported (Table). The median age at the onset of disease in the 11 cases was 63 years and there was a male predominance (8/11); the patients presented with a range of symptoms including right upper quadrant pain, liver mass, abdominal fullness, and jaundice (3)(4)(5)(6)(7)(8)(9)(10)(11)(12). The biochemical investigations did not always demonstrate altered liver function (3)(4)(5)(6)(7)(8)(9)(10)(11)(12).…”

Section: Discussionmentioning

confidence: 99%

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Malignant Peripheral Nerve Sheath Tumor of the Liver

Kakizaki

Horiguchi

Otsuka³

et al. 2016

Intern. Med.

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Malignant peripheral nerve sheath tumor (MPNST) of the liver is rare. Most cases of MPNST are accompanied by neurofibromatosis 1 (NF-1, von Recklinghausen's disease). We herein report an autopsy case of MPNST without NF-1 and review the pertinent literature. The tumor occupied the entire lobe of the liver, and was 18 cm in maximum diameter. The tumor revealed necrosis and cystic changes with hemorrhage and it had also metastasized to the peritoneum. Microscopically, the tumor was composed of pleomorphic spindle cells with hyperchromatic nuclei and mitogenic figures. The spindle cells stained positive for both S-100 and vimentin antibodies.

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Malignant Tumors of Peripheral Nerves

Kao

Parham

Fuller

2014

Pediatric Malignancies: Pathology and Imaging

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Malignant peripheral nerve sheath tumors (MPNST) represent a group of malignant spindle cell sarcomas with evidence of nerve sheath differentiation and/or arising from a peripheral nerve. Tumors showing origin from epineurial connective tissue or vascular structures are not considered to represent MPNST. Older/prior terminology for MPNST includes malignant neurilemmoma, malignant schwannoma, neurofi brosarcoma, and neurogenic sarcoma [ 1 ]. A number of histologic subtypes have been described. The majority are high grade aggressive sarcomas that show a strong tendency for local recurrence and metastases despite aggressive therapeutic measures [ 2 ]. Clinical Features and EpidemiologyThere is a strong association between MPNST and Neurofi bromatosis Type 1 (NF1), with 50 % of all MPNSTs arising within this patient population [ 3 ]. Approximately 10 % of individuals with NF1 will develop an MPNST over their lifetime, most of these tumors representing malignant degeneration occurring within preexisting plexiform neurofi bromas. One study of 476 NF1 patients documented a strong association between the presence of subcutaneous neurofi bromas and internal plexiform neurofi bromas, with an even stronger correlation between the presence of internal plexiform neurofi bromas and MPNST [ 4 ]. The remainder of MPNSTs will arise de novo, approximately 5-10 % representing radiation-induced sarcomas [ 1 ]. There is no particular gender predilection, and MPNSTs have been documented throughout a wide age range including children and the elderly. The majority however tend to present in adults with a median age in the mid 40s [ 5 ]. MPNSTs arising in the NF1 population tend to present up to a decade earlier, and these patients' tumors also tend to be larger [ 3 , 6 ].The majority of MPNST arise from larger peripheral nerves or within deep soft tissues, the most frequent sites including brachial plexus, sciatic, and paraspinal nerves, proximal upper and lower extremities, and buttock regions [ 5 ]. They have also been documented in a wide variety of locations throughout the body. Well over 50 cases of spinal MPNST have been reported [ 7 -13 ]. No particular spinal level is preferentially involved, and interestingly a significant proportion of spinal MPNST exhibit rhabdomyoblastic elements (Malignant Triton Tumor; see histologic description below) [ 14 -17 ]. MPNST represents one of the most aggressive tumors that may involve structures of the head and neck region [ 6 ]. It may arise from cranial nerves, particularly the vestibular [ 18 ], vagus [ 18 ], facial [ 18 , 19 ], and trigeminal [ 19 , 20 ]. Other sites include not only brachial plexus [ 21 ], but also scalp and bony structures (base of skull [ 22 -25 ], sinuses [ 26 , 27 ], and bones of the jaw) [ 28 , 29 ]

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Malignant Peripheral Nerve Sheath Tumor of the Liver: A Case Report

Cited by 18 publications

References 9 publications

Hepatic schwannoma: A case report and an updated 40-year review of the literature yielding 30 cases

Hepatic schwannoma: A case report and an updated 40-year review of the literature yielding 30 cases

Malignant Peripheral Nerve Sheath Tumor of the Liver

Malignant Tumors of Peripheral Nerves

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