Cases reported "Nerve Sheath Neoplasms"

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1/13. Benign glandular peripheral nerve sheath tumor of the seventh and eighth cranial nerve complex.

    A unique, deep-seated, benign, glandular peripheral nerve sheath tumor (PNST) of the cerebellopontine angle is described. The tumor arose from the seventh and eighth cranial nerve complex in a 15-year-old boy without neurofibromatosis. Histologically, benign glands were embedded in a bland spindle cell stroma. The epithelial cells were immunoreactive for CAM 5.2, and focally for chromogranin. The spindle cells were positive for S100P. The benign glandular PNST unassociated with neurofibromatosis is a controversial entity. The superficial location of most reported cases has made it difficult to exclude entrapped adnexae as a source for the glands. This tumor was separate from the internal auditory meatus on MRI scan, the most likely source of entrapped glands at this site. This case is the first report of a deep-seated, benign, glandular peripheral nerve sheath tumor. It suggests that glandular differentiation in PNSTs, while unusual, is not synonymous with neurofibromatosis or malignancy.
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2/13. Glandular malignant peripheral nerve sheath tumor: an unusual case showing histologically malignant glands.

    In this report, we describe a highly unusual case of glandular malignant peripheral nerve sheath tumor presenting as a neck mass in a previously healthy 29-year-old man. Grossly, the tumor was found to arise from a swollen peripheral nerve trunk. The tumor was largely composed of spindle cells that demonstrated marked nuclear pleomorphism and numerous abnormal mitotic figures. In addition, histologically malignant glandular structures lined by simple nonciliated columnar cells with goblet cells were found clustered in the center of the tumor. Examination of the swollen peripheral nerve trunk revealed the presence of a plexiform neurofibroma. The spindle cells were positive for S100. The glands were negative for S100 but positive for keratin, epithelial membrane antigen, and neuroendocrine markers (somatostatin, chromogranin, Leu-7, and calcitonin). This patient was subsequently diagnosed as having von Recklinghausen disease and died of tumor metastasis to the lungs 34 months after the presentation. To our knowledge, only 3 similar cases have been previously described in the literature.
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3/13. Non-neoplastic glandular structures in a benign peripheral nerve sheath tumor.

    It is a recognized fact that glandular structures sometimes occur in peripheral nerve sheath tumors (PNST). Reports indicate that epithelial potential could be expressed in malignant PNST, while the glands in most benign PNST could be trapped skin adnexa. We present a case of spindle cell tumor with glandular structures. The patient was a 55-year-old man who had a subcutaneous tumor excised. The spindle cell tumor had histological characteristics of neurofibroma. The glandular structures had a pattern of immunohistochemical staining that was similar to that of the secretory coils and excretory ducts of normal eccrine glands. Therefore, the glands were thought to be naturally existing eccrine glands. The glands were completely enclosed within the tumor and not connected to one another; a cluster formation was not observed. The upper portion of tumor included some glands of skin adnexa. The spindle cell tumor may have originated from the nerves distributed around the skin adnexa, and grown to the subcutaneous tissue. The glands may have been left behind rather than have been trapped in the tumor.
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4/13. Malignant peripheral nerve sheath tumor with divergent differentiation.

    Right posterior thigh malignant peripheral nerve sheath tumor with malignant glandular and rhabdomyoblastic components was diagnosed in a 24-year-old African American man. Malignant glands and rhabdomyoblastic cells were admixed with the spindle cells. immunohistochemistry demonstrated spindle cells positive for S100 and vimentin; the glandular component was positive for chromogranin, and rare cells were positive for CK20 but negative for CK7. Rhabdomyoblastic cells were positive for muscle-specific actin and desmin. A single pulmonary metastasis occurred 5 months after surgical resection of the tumor. death occurred 1 month later and was probably due to a pulmonary embolus.
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5/13. Malignant peripheral nerve sheath tumor of small round cell type with pleomorphic spindle cell sarcomatous areas.

    An unusual case of malignant peripheral nerve sheath tumor (MPNST) arising in the posterior mediastinum of a 59-year-old man is reported. Histopathologically, the tumor showed an admixture of a dense proliferation of small round cells resembling a primitive neuroectodermal tumor (PNET) and a pleomorphic spindle cell sarcomatous area. Abortive rosettes, primitive neural tube-like structures, and a few glandular structures were found in the small round cell area. Small round cells were immunoreactive for neural cell adhesion molecule and synaptophysin, but were not immunoreactive for MIC2 and neuron-specific enolase. Pleomorphic spindle cells were occasionally arranged in a storiform pattern and were diffusely immunoreactive for S-100 protein. The MPNST of small round cell type is distinguishable from PNET by its negative immunoreactivity for MIC2, and the present tumor is assumed to be derived from primitive neuroectodermal cells in the peripheral nerve capable of bidirectional (neuron and Schwann cell) differentiation.
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6/13. Malignant peripheral nerve sheath tumour metastasising to the parotid gland.

    Two rare cases of metastases from malignant peripheral nerve sheath tumours (MPNST) of the head and neck are described. The initial lesions were superficial, but despite early diagnosis and complete excision, they metastasised to the ipsilateral parotid gland. Both underwent further complete excision and neither metastatic lesion showed progression in grade. One patient is alive, however, the other died of widespread metastases. There are no other reported cases of these sarcomas in the head and neck metastasising to the parotid gland.
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7/13. Malignant peripheral nerve sheath tumor of the parotid gland.

    Malignant peripheral nerve sheath tumor (MPNST) has been defined as any malignant tumor arising from or differentiating toward cells of the peripheral nerve sheath. We treated a case of MPNST arising from the right parotid gland that showed a highly aggressive course. We reviewed the English-language literature published since 1990 and found 142 cases of head and neck MPNST reported within the past 13 years. The results of the review suggested that MPNSTs may arise from any organs of the head and neck. Immunohistochemical analysis of various neural markers plays a significant role in the evaluation of the histologic diagnosis. Curative treatment based on radical resection of MPNSTs of head and neck origin is more difficult than treatment of MPNSTs of other origins.
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8/13. Multiple brain metastases from malignant peripheral nerve sheath tumour (MPNST).

    Malignant peripheral nerve sheath tumours (MPNSTs) are rare soft tissue neoplasms arising from elements of the nerve sheath that often occur in the context of neurofibromatosis (NF) type 1. Their poor prognosis results from high local recurrence rate and distant dissemination. Nevertheless, the brain metastases are exceptional. We are presenting an unusual case of intrathoracic MPNST in a 33-year-old man with a five-year clinical course characterised by multiple times local recurrences of primary tumour and multiple remote metastases into the brain structures, thyroid and suprarenal gland. Moreover, the cerebellar metastasis regrew in spite of its total excision. Histologically, brain metastatic tumours were composed of spindle cells closely arranged in interlacing and woven fascicles. This highly cellular nerve tissue exhibited an advanced nuclear hyperchromasia and a high mitotic activity. The tumour exhibited rich delicate reticulin network. The schwannian nature of brain metastases has been confirmed by immunohistochemical findings showing S-100 protein and GFAP expression and ultrastructural evidences of the pericellular basal lamina.
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9/13. Ectopic hamartomatous thymoma: a case report with immunohistochemical study and review of the literature.

    Ectopic hamartomatous thymoma (EHT) is a rare benign tumor. We present a case of EHT, which was seen as subcutaneous mass on the left supraclavicular area in a 19-year-old man. The tumor consisted of spindle cells, epithelial cells, adipose cells, and a small amount of lymphocytes, as described previously. Immunohistochemically, spindle cells were positive for keratin, a-smooth muscle actin, CD34 and vimentin, but negative for desmin and S-100 protein. lymphocytes were positive for CD45RO but negative for CD20, CD1a, and CD99. Approximately, 5% of cells were positive for MIB-1 and no cells stained for p53 and bcl-2. Recognition of EHT is important and needs to be differentiated from high-grade sarcomas such as synovial sarcoma or glandular malignant peripheral nerve sheath tumor.
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10/13. Malignant peripheral nerve sheath tumors of the head and neck: two cases and a review of the literature.

    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.
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