Chronic Recurrent Subarachnoid Hemorrhage from a Trigeminal Nerve Malignant Peripheral Nerve Sheath Tumor: Case Report

Stark, Andreas M.; Buhl, Ralf; Hugo, Heinz-Hermann; Straube, Thorsten; Mehdorn, H. M.

doi:10.1227/01.neu.0000223498.83238.b2

Cited by 9 publications

(6 citation statements)

References 13 publications

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“…12,20-24 Also, we have retrieved one additional patient with a trigeminal nerve MPNST, a 59-year-old man, who exhibited subarachnoid hemorrhage as an initial symptom caused by intratumoral bleeding. 25 However, none of these reported patients had other identifiable cranial nerve tumors, unlike the present patient.…”

Section: Discussioncontrasting

confidence: 55%

“…According to a literature review by Gousias et al ., 10 patients with trigeminal nerve MPNSTs have been reported previously (6 male, 4 female: age 3–62 years, mean ± SD = 27.8 ± 19.8 years: 9 non‐associated and 1 associated with NF1: 7 dead and 3 alive at the time of writing) . Also, we have retrieved one additional patient with a trigeminal nerve MPNST, a 59‐year‐old man, who exhibited subarachnoid hemorrhage as an initial symptom caused by intratumoral bleeding . However, none of these reported patients had other identifiable cranial nerve tumors, unlike the present patient.…”

Section: Discussionmentioning

confidence: 99%

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Malignant peripheral nerve sheath tumor of the trigeminal nerve: Clinicopathologic features in a young adult patient

et al. 2012

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Malignant peripheral nerve sheath tumors (MPNSTs) arising from cranial nerves are rare and usually affect adults. Here we report the clinicopathologic features of a young adult patient with a trigeminal nerve MPNST, in whom another tumor involving the oculomotor nerve on the contralateral side was evident. The patient, an 18-year-old woman, had suffered recurrent paroxysmal sharp stabbing pain over her cheek and forehead on the right side for 1 month. A brain MRI study disclosed a mass, 35 mm in diameter, in the right Meckel's cave, and another mass, 10 mm in diameter, involving the intracranial portion of the left oculomotor nerve. Following gadolinium administration, the former and latter tumors exhibited strong and weak enhancement, respectively. The patient had no clinical stigmata characteristic of neurofibromatosis type 1. Following a tentative diagnosis of schwannoma, total resection of the trigeminal nerve tumor was performed. Histologically, the tumor consisted of highly cellular, spindle-shaped cells arranged in a fascicular pattern, with occasional mitotic figures, nuclear pleomorphism and necrosis. Immunohistochemically, the tumor cells showed variable intensities and frequencies of reactivity for S-100 protein, myelin basic protein, CD34, podoplanin and p53, but no reactivity for Smarcb1. Thus, the tumor exhibited features of MPNST. This case appears to provide information that is useful for accurate diagnosis and surgical planning in patients with bilateral or multiple cranial nerve tumors.

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Section: Discussioncontrasting

confidence: 55%

Section: Discussionmentioning

confidence: 99%

Malignant peripheral nerve sheath tumor of the trigeminal nerve: Clinicopathologic features in a young adult patient

et al. 2012

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“…These 29 articles provided 35 cases, to which we added 1 additional case from our institu tion. 1,2,[4][5][6][7][8]10,11,[14][15][16][17][18][19][20][21]24,[26][27][28][29][30][32][33][34][35][36][37] The complete results of our analysis of 36 cases are detailed in Table 1.…”

Section: Resultsmentioning

confidence: 99%

Malignant peripheral nerve sheath tumors of the trigeminal nerve: a systematic review of 36 cases

Schmidt¹,

Yick²,

Boghani³

et al. 2013

FOC

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Object Malignant peripheral nerve sheath tumors (MPNSTs) are a rare form of malignancy arising from the Schwann cells of peripheral nerves. MPNSTs of the trigeminal nerve are exceptionally rare, with only a handful of reports in the literature. These tumors are typically very aggressive, resulting in significant patient morbidity and a generally grim prognosis. Most current reports suggest that radical resection with radiation therapy offers the best benefit. In this study, the authors systematically reviewed the world English-language literature on MPNSTs of the trigeminal nerve to analyze the presentations, treatment options, and outcomes for patients with this disease. Methods A literature search for MPNSTs of the trigeminal nerve confined to nonanimal, English-language articles was conducted utilizing the PubMed database, with additional cases chosen from the references of selected articles. Only cases of confirmed MPNSTs of the trigeminal nerve or its peripheral branches, based upon surgical, pathological, or radiological analysis, were included. Results From the literature search, 29 articles discussing 35 cases of MPNSTs of the trigeminal nerve were chosen. With the addition of 1 case from their own institution, the authors analyzed 36 cases of trigeminal MPNSTs. The average age of onset was 44.6 years. These tumors were more commonly seen in male patients (77.1%). The gasserian ganglion was involved in 36.1% of the cases. Of the cases in which the nerve distribution was specified (n = 25), the mandibular branch was most commonly involved (72.0%), followed by the maxillary branch (60.0%) and the ophthalmic branch (32.0%), with 44.0% of patients exhibiting involvement of 2 or more branches. Altered facial sensation and facial pain were the 2 most commonly reported symptoms, found in 63.9% and 52.8% of patients, respectively. Mastication difficulty and diplopia were seen in 22.2% of patients, facial weakness was seen in 19.4%, and hearing loss was present in 16.7%. With regard to the primary treatment strategy, 80.6% underwent resection, 16.7% underwent radiation therapy, and 2.9% received chemotherapy alone. Patients treated with complete resection followed by postoperative radiation therapy had the most favorable outcomes, with no patients showing evidence of disease recurrence with a mean follow-up of 34.6 months. Patients treated with incomplete resection followed by postoperative radiation therapy had more favorable outcomes than patients treated with incomplete resection without radiation therapy or radiation therapy alone. Conclusions Trigeminal MPNSTs most commonly present as altered facial sensation or facial pain, although they exhibit a number of other clinical manifestations, including the involvement of other cranial nerves. While a variety of treatment options exist, due to their highly infiltrative nature, aggressive resection followed by radiation therapy appears to offer the greatest chance of recurrence-free survival.

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“…If the CSF is cytologically tumor free, biopsy of gadolinium-enhancing structures identified in MRI might be necessary. Notably, hydrocephalus in patients who underwent surgical intervention might have other causes: (A) Subarachnoid bleeding, either tumor-induced [ 10 ] or related to surgery and (B) bacterial meningitis.…”

Section: Discussionmentioning

confidence: 99%

Acute Hydrocephalus due to Secondary Leptomeningeal Dissemination of an Anaplastic Oligodendroglioma

Stark¹,

Hugo²,

Mehdorn³

et al. 2009

Case Reports in Medicine

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Secondary leptomeningeal dissemination of oligodendroglioma is very rare. We report the case of a 38-year-old Caucasian male who presented with acute hydrocephalus. 8 months before, the patient had undergone craniotomy for right frontal anaplastic oligodendroglioma, WHO grade III. By that time, there was no evidence of tumor dissemination. MRI now ruled out local tumor progression but revealed meningeal contrast enhancement along the medulla, the myelon, and the cauda equina. Repeated lumbar puncture revealed increased cerebro-spinal fluid (CSF) pressure and protein content. Malignant cells were not detectable. Surgical treatment consisted in (1) placement of an ommaya reservoir for daily CSF puncture, (2) Spinal dural biopsy confirming leptomeningeal oligodendroglioma metastasis, and (3) ventriculo-peritoneal shunt placement after CSF protein has decreased to 1500–2000 mg/l.

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Chronic Recurrent Subarachnoid Hemorrhage from a Trigeminal Nerve Malignant Peripheral Nerve Sheath Tumor: Case Report

Abstract: This is the first report regarding a malignant peripheral nerve sheath tumor of the trigeminal nerve that caused clinically significant subarachnoid hemorrhage caused by intratumoral bleeding.

Cited by 9 publications

References 13 publications

Malignant peripheral nerve sheath tumor of the trigeminal nerve: Clinicopathologic features in a young adult patient

Malignant peripheral nerve sheath tumor of the trigeminal nerve: Clinicopathologic features in a young adult patient

Malignant peripheral nerve sheath tumors of the trigeminal nerve: a systematic review of 36 cases

Acute Hydrocephalus due to Secondary Leptomeningeal Dissemination of an Anaplastic Oligodendroglioma

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