Hepatic neurofibromatosis, malignant schwannoma, and angiosarcoma in von Recklinghausen's disease

Lederman, Seth; Martin, Eric C.; Laffey, Karen T.; Lefkowitch, Jay H.

doi:10.1016/0016-5085(87)90866-3

Cited by 78 publications

(34 citation statements)

References 19 publications

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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%

“…The biochemical investigations did not always demonstrate altered liver function (3)(4)(5)(6)(7)(8)(9)(10)(11)(12). CT demonstrated a heterogeneous rapidly enlarging liver mass 7-30 cm in size (3)(4)(5)(6)(7)(8)(9)(10)(11)(12). The inability to perform a surgical resection was associated with a poor prognosis.…”

Section: Discussionmentioning

confidence: 97%

“…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%

“…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). CT demonstrated a heterogeneous rapidly enlarging liver mass 7-30 cm in size (3)(4)(5)(6)(7)(8)(9)(10)(11)(12).…”

Section: Discussionmentioning

confidence: 99%

“…Most cases of MPNST are accompanied by neurofibromatosis 1 (NF-1, von Recklinghausen's disease). MPNSTs arising from the liver are extremely rare, with only 10 cases have reported in the literature (3)(4)(5)(6)(7)(8)(9)(10)(11)(12). Hepatic MPNST without NF-1 is even rarer (10).…”

Section: Introductionmentioning

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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Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

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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Resection of Primary Schwannoma of the Liver Not Associated With Neurofibromatosis

Heffron

Coventry²,

Bedendo³

et al. 1993

Arch Surg

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A 38-year-old woman developed right upper quadrant pain due to a mass in the left lobe of the liver. The tumor was resected along with segment 3 of the left lobe. Histologic examination and immunochemistry supported a diagnosis of benign schwannoma. No metastatic disease was present, and the patient has been well for more than 18 months after surgery without recurrence. This is the first reported case of successful resection of a schwannoma of the liver in a patient without von Recklinghausen's disease.

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Comparison of Cancer Prevalence in Patients With Neurofibromatosis Type 1 at an Academic Cancer Center vs in the General Population From 1985 to 2020

et al. 2021

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IMPORTANCE Neurofibromatosis type 1 (NF1) is a complex genetic disorder that is associated with not only neurofibromas, but also an increased susceptibility to other neoplasms. OBJECTIVE To evaluate the prevalence of neoplasia and outcomes among patients with NF1. DESIGN, SETTING, AND PARTICIPANTS This cohort study was conducted among patients with NF1 at a single academic cancer center from 1985 to 2020 with median (range) follow-up of 2.9 years (36 days to 30.5 years). Of 2427 patients evaluated for NF1, 1607 patients who met the National Institutes of Health consensus criteria for NF1 were included. This group was compared with estimates from Surveillance, Epidemiology, and End Results (SEER) Cancer Statistics Review 1975 to 2015 and SEER participants database unless otherwise specified. Data were analyzed from August 2018 to March 2020. MAIN OUTCOMES AND MEASURES Disease-specific survival (DSS) was measured from diagnosis date to date of neoplasm-specific death or censorship and calculated using the Kaplan-Meier method. Survival curves were compared using the log-rank test. Deaths from disease were considered a DSS end point; other deaths were considered censored observations. Secondary outcome measures were comparisons of (1) overall survival of patients with NF1 with neurofibroma neoplasms vs those without nonneurofibroma neoplasms, (2) neoplasm prevalence in the NF1 group vs general population estimates, and (3) age at diagnosis in the NF1 group vs general population estimates for the most common neoplasms in the NF1 group. RESULTS Among 1607 patients with NF1, the median (range) age at initial visit was 19 years (1 month to 83 years) and 840 (52.3%) were female patients. Among 666 patients who developed other neoplasms in addition to neurofibromas (41.4%), 295 patients (18.4%) developed glioma and 243 patients (15.1%) developed malignant peripheral nerve sheath tumor (MPNST), the most common neoplasms. Patients with NF1, compared with the general population, developed several neoplasms at a younger mean (SD) age (low-grade glioma: 12.98 [11.

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Hepatic neurofibromatosis, malignant schwannoma, and angiosarcoma in von Recklinghausen's disease

Cited by 78 publications

References 19 publications

Malignant Peripheral Nerve Sheath Tumor of the Liver

Malignant Peripheral Nerve Sheath Tumor of the Liver

Resection of Primary Schwannoma of the Liver Not Associated With Neurofibromatosis

Comparison of Cancer Prevalence in Patients With Neurofibromatosis Type 1 at an Academic Cancer Center vs in the General Population From 1985 to 2020

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