Malignant peripheral nerve sheath tumors of the head and neck: Management of 10 cases and literature review

Minovi, Amir; Basten, O.; Hunter, Ben; Draf, Wolfgang; Bockmühl, Ulrike

doi:10.1002/hed.20537

Cited by 106 publications

(98 citation statements)

References 31 publications

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“…Pleomorphic and embryonal RMS like LMS are positive for desmin and SMA, but unlike LMS they are positive for myoglobin or myoD1 indicating a skeletal muscle origin [24]. Myofibroblastic sarcoma (or myofibrosarcoma) may be included in the differential diagnosis, but these lesions are generally of a low grade, exhibit fusiform rather than the cigarshaped nuclei seen in LMS, have a paler eosinophilic cytoplasm than LMS, and are only focally positive for SMA [24]. In addition, staining for h-caldesmon is generally negative in myofibroblastic sarcoma but positive in LMS [24].…”

Section: Discussionmentioning

confidence: 99%

“…FS generally presents with a more tapered cell nuclei and ''herring bone'' fascicular pattern than LMS and may show focal areas of staining for smooth muscle markers reflecting myofibroblastic differentiation but does not show the extent of diffuse positivity to smooth muscle markers as LMS [23]. MPNST has been reported rarely in the sinonasal tract and differs from LMS in showing a characteristic appearance of wavy or buckled cells and alternating zones of cellular and less cellular areas which may appear in a vaguely nodular pattern [22,24]. Spindled MPNST will be negative for smooth muscle actin and show focal S-100 positivity, whereas LMS is the opposite [22].…”

Section: Discussionmentioning

confidence: 99%

“…The spindle cell variant of embryonal RMS may also be seen in the sinonasal tract and may resemble LMS but generally occurs in young patients [26]. Pleomorphic and embryonal RMS like LMS are positive for desmin and SMA, but unlike LMS they are positive for myoglobin or myoD1 indicating a skeletal muscle origin [24]. Myofibroblastic sarcoma (or myofibrosarcoma) may be included in the differential diagnosis, but these lesions are generally of a low grade, exhibit fusiform rather than the cigarshaped nuclei seen in LMS, have a paler eosinophilic cytoplasm than LMS, and are only focally positive for SMA [24].…”

Section: Discussionmentioning

confidence: 99%

“…Myofibroblastic sarcoma (or myofibrosarcoma) may be included in the differential diagnosis, but these lesions are generally of a low grade, exhibit fusiform rather than the cigarshaped nuclei seen in LMS, have a paler eosinophilic cytoplasm than LMS, and are only focally positive for SMA [24]. In addition, staining for h-caldesmon is generally negative in myofibroblastic sarcoma but positive in LMS [24]. A final entity to consider is the monophasic fibrous variant of synovial sarcoma (SS).…”

Section: Discussionmentioning

confidence: 99%

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Nasal Sinus Leiomyosarcoma in a Patient with History of Non-Hereditary Unilateral Treated Retinoblastoma

Fitzpatrick

Woodworth

Monteiro

et al. 2010

Head and Neck Pathol

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Hereditary patients with a history of treated retinoblastoma (RB) have a greatly increased risk of a broad spectrum of secondary malignancies appearing many years later, with a high incidence in the head and neck region. Leiomyosarcomas (LMS) account for up to 58% of these tumors. LMS in the sinonasal region generally are uncommon and are associated with a locally aggressive course and have a poor prognosis. RB may occur in two forms. The hereditary form is generally bilateral but can present unilaterally with a positive family history and typically exhibits a germline mutation in the RB1 gene on chromosome 13. The non-hereditary form is usually unilateral but can show the same germline mutation in up to 10% of cases. Patients with hereditary RB have been shown to have a significantly higher cumulative risk of developing secondary malignancies than those with the non-hereditary form (28 vs. 1.44% respectively). Most reported cases of sinonasal LMS are in patients with a history of the bilateral hereditary form of treated RB. We report a case of LMS of the nasal sinus area in a 35-yearold African American male with a history of non-hereditary unilateral RB and radiation therapy. To the best of our knowledge, this is the first reported case of sinonasal LMS arising in a patient with a history of non-hereditary unilateral RB. The clinical history, radiology, and pathology are presented along with a brief discussion of the literature.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Nasal Sinus Leiomyosarcoma in a Patient with History of Non-Hereditary Unilateral Treated Retinoblastoma

Fitzpatrick

Woodworth

Monteiro

et al. 2010

Head and Neck Pathol

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“…1,5,9,12,22,31 When only MPNSTs of the head and neck are considered, the 5-year survival rate decreases to about 20%. 25 The limited nature of this analysis and poor consistency of follow-up makes it difficult to offer spe cific prognostic factors for trigeminal MPNSTs; however, it is clear that they tend to exhibit similar features of very rapid growth and extensive infiltration into surrounding tissues, which results in a generally grim prognosis. 4 Due to the aggressive natural history of these tumors and the more favorable outcomes seen with complete re section, 4,5,37 aggressive radical resection of trigeminal MPNSTs should be attempted, if safely possible.…”

Section: Prognosis and Treatmentmentioning

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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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 tumors of the head and neck: Management of 10 cases and literature review

Cited by 106 publications

References 31 publications

Nasal Sinus Leiomyosarcoma in a Patient with History of Non-Hereditary Unilateral Treated Retinoblastoma

Nasal Sinus Leiomyosarcoma in a Patient with History of Non-Hereditary Unilateral Treated Retinoblastoma

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

Malignant Tumors of Peripheral Nerves

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