Malignant peripheral nerve sheath tumor

Malignant peripheral nerve sheath tumor of the superior cervical sympathetic ganglia

October 8, 2012     Juliette Thariat, MD, PhD; Pierre-Yves Marcy, MD; Isabelle Peyrottes, MD; Alexandre Bozec, MD, PhD; Nicolas Venissac, MD, PhD
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Abstract

The prevalence of malignant peripheral nerve sheath tumor (MPNST) among the general population is only 0.001%. Most cases originate in peripheral nerve root trunks in the extremities; MPNST rarely occurs in the head and neck. We describe the case of a 61-year-old man who presented with Horner syndrome, dysphonia, and enlarged neck nodes. Imaging detected a paraspinal mass of the deep superior cervical chain next to the internal carotid artery. The patient underwent an upfront modified radical neck dissection of the right-sided lymph nodes from levels II through V. Postoperative irradiation was performed. Two years after the initial diagnosis, the patient was found to have a pulmonary metastasis of the MPNST. A wedge resection was performed, and at 6 months of follow-up, the patient was alive without disease. However, he subsequently developed soft-tissue metastases in his buttocks, and he died 2 years later of brain metastases. In our case, the clinical symptoms suggested the anatomic location of the primary tumor, and imaging findings suggested the diagnosis, which was confirmed on histopathology. As far as we know, no case of MPNST of the superior ganglia of the sympathetic chain has been previously described in the literature.

Report of a case of malignant peripheral nerve sheath tumor of the neck with an early local recurrence

October 26, 2011     Deniz Tuna Edizer, MD, Ahmet Özdoğan, MD, Emin Karaman, MD, and Hüseyin Işıldak, MD
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Abstract

Malignant peripheral nerve sheath tumor (MPNST), which originates in Schwann cells, is uncommon in the head and neck. These highly aggressive sarcomas are characterized by local recurrence and distant metastasis. Many are associated with neurofibromatosis type 1; the survival rate in such cases is poorer than the rate associated with tumors that arise de novo. Hypercellularity and pleomorphism are cardinal microscopic features. Immunohistochemical staining is widely used to determine S-100 positivity and a Ki-67 score, either of which may establish the prognosis. We present a case of MPNST of the neck that arose in a 17-year-old girl, who was also found to have a parapharyngeal neurofibroma. The cervical mass was excised (intraoperative complications precluded removal of the parapharyngeal mass) and radiotherapy was administered, but the patient experienced a recurrence within 2 months. Another surgery was performed, and this time both tumors were removed. At 1 year of follow-up, the patient was without disease, but in postoperative year 2, she died from complications of a cervical recurrence and mediastinal metastasis.

Cervicothoracic malignant peripheral nerve sheath tumor

June 13, 2011     Harrison W. Lin, MD, David D. Tieu, MD, Karen Ferrer, MD, Urjeet Patel, MD, and Brian K. Reilly, MD
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Malignant peripheral nerve sheath tumor of the infratemporal fossa with intracranial extension

December 17, 2010     Martin Tamarit, MD, Rosario Navarro, MD, and Lucía Alcázar, MD
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Abstract

Malignant peripheral nerve sheath tumor (MPNST) is an uncommon neoplasm that rarely involves the head and neck region. We report the case of a 66-year-old man who presented with symptoms compatible with uncinate seizures. Imaging studies detected a left infratemporal fossa tumor with bone tissue destruction and extension into the intracranial compartment, which exerted a mass effect on the temporal lobe. Histologic studies identified the tumor as an MPNST. Complete removal of the lesion was not possible, so the patient was treated with adjuvant radiotherapy. He died 6 months later. This rare case of MPNST with intracranial involvement illustrates the dismal prognosis for patients with such a lesion. Survival is limited because of the difficulty of performing radical surgery with free margins in such a location.

Malignant peripheral nerve sheath tumors of the head and neck: Two cases and a review of the literature

May 31, 2006     Pablo Martinez Devesa, FRCS (Edin); Timothy E. Mitchell, MA, FRCS (ORL-HNS); Ian Scott, FRCPath; David A. Moffat, BSc, FRCS
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Abstract
Malignant peripheral nerve sheath tumors are uncommon lesions that occasionally affect the head and neck. We describe 2 new cases of head and neck pathology. One tumor involved the parotid gland and resulted in erosion of the temporal bone, and the other affected the lower lip. A rapid diagnosis has significant implications for management because of the tumor's potential for aggressive behavior and its high rate of recurrence. To the best of our knowledge, lip involvement is rare and temporal bone involvement has not been previously described.

Malignant peripheral nerve sheath tumor of the paranasal sinuses

September 30, 2004     Farhan Ahsan, MRCS; Maggie K. Lee, MBChB; Kim W. Ah-See, MD, FRCS; Andrea D. Chapman, MRCPath
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Abstract
Malignant peripheral nerve sheath tumors of the nose and paranasal sinuses are extremely uncommon. We report the case of a 65-year-old woman who presented with a rapid emergence of "nasal polyps" that completely obstructed her nasal passages. Six years earlier, she had been assessed elsewhere for nasal polyps. At presentation, the patient exhibited gross polyposis, with lesions protruding from both nostrils. Histology confirmed a diagnosis of a malignant peripheral nerve sheath tumor. This case demonstrates that symptoms of nasal obstruction are not always secondary to simple causes. We discuss the clinical picture of nasal and paranasal malignant peripheral nerve sheath tumor, its pathology, and its treatment.