1/33. Malignant peripheral nerve sheath tumor with perineurial cell differentiation (malignant perineurioma).A unique case of malignant peripheral nerve sheath tumor (MPNST) with perineurial cell differentiation occurring in a 63-year-old woman in a subcutis of the forearm is described. The tumor contained cellular and myxoid areas. The neoplastic cells were fusiform with distinct cell borders. They were arranged in storiform pattern and in wavy parallel cell cords in the cellular areas. Focally, a pleomorphism and mitotic activity (including atypical mitoses) similar to those of malignant fibrous histiocytoma were seen. The myxoid parts contained haphazardly oriented cells and scarce lipoblast-like multivacuolated cells mimicking a liposarcoma. In the differential diagnosis, myxoid liposarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma and low-grade fibromyxoid sarcoma were considered. Immunohistochemically, perineurial differentiation was indicated by the diffuse expression of epithelial membrane antigen and focal reactivity for CD34. The tumor was negative with antibodies to S-100 protein, Leu-7, CD68 (KP1), vimentin and cytokeratin AE1/AE3. Ultrastructure of tumor cells revealed features of MPNST. No recurrence occurred in the patient during 2 years follow up.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/33. Intraosseous epithelioid malignant peripheral nerve sheath tumor of the phalanx. Case report.We report the first case of intraosseous epithelioid malignant peripheral nerve sheath tumor (MPNST) occurring in the phalanx. The patient was a 50-year-old Japanese man with an intramedullary lytic lesion of the proximal phalanx. Microscopically, the tumor was composed of epithelioid cells or polygonal cells, forming large cell nests with central necrosis. Most tumor cells were diffusely and strongly immunopositive for S-100 protein and vimentin, and negative for cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, alpha-smooth muscle actin, and HMB-45. laminin-positive material was discontinuously demonstrated between the individual tumor cells. Electron microscopy showed prominent external lamina. Our case indicated that laminin is useful for differentiating epithelioid MPNST from metastatic carcinoma and malignant melanoma.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
3/33. Low-grade malignant perineurioma of the paravertebral column, transforming into a high-grade malignancy.A demarcated 6 x 5 cm right paravertebral tumor at the level of T6 in a 39-year-old male was removed surgically. Histologically, the tumor consisted of monomorphous benign-looking, low-cellular spindle cells embedded in desmoplastic stroma. Ten years later, the tumor recurred locally with metastasis to systemic organs, including the occipital skin. Malignancy was histologically evident by the increased cellularity, cellular atypia and mitotic activity. The patient died of respiratory failure at the age of 49. Retrospectively reviewed, the primary lesion was low-grade fibrosarcoma-like spindle cell tumor, with secondary transformation into a highly malignant form. The differential diagnoses included sclerosing epithelioid fibrosarcoma, low-grade fibromyxoid sarcoma and malignant peripheral nerve sheath tumor. Immunohistochemically, the spindle cells in the primary and recurrent tumors consistently expressed epithelial membrane antigen, vimentin, type 4 collagen and laminin. The tumor cells in the present case showed a differentiation toward perineurial cells, which are normally positive for these immunohistochemical markers. Hence, the appropriate diagnostic term should be 'malignant perineurioma', a subtype of malignant peripheral nerve sheath tumor.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
4/33. Extraneural retiform perineuriomas. A report of four cases.We present here four cases of extraneural perineurioma (EP), which occurred in 31, 32, 42 and 43 year old women in the left hand, right arm (2 cases) and right axillary regions, respectively. All tumors were well circumscribed, with a thin capsule. They were 2, 2, 1.5 and 1.5 cm in the largest diameter. Microscopically all tumors had a distinctive appearance composed of interconnected cords of cells which ran in a parallel fashion or formed a characteristically looking cellular lattice. These cords were composed of cells with tapered to stellate shaped nuclei having one or two inconspicuous nucleoli. The space between the cells of the lattice was edematous and empty looking, or it was composed of myxoid matrix, or it was filled with characteristically looking globular deposits of collagen extracellular matrix. These globular deposits of collagen extracellular matrix often indented the nuclei, giving them a stellar appearance. Immunohistochemically all tumors reacted positively with antibodies to epithelial membrane antigen. antibodies to smooth muscle actin, muscle-specific actin, S-100 protein, cytokeratin AE1/AE3, cytokeratin CAM 5.2, and HMB-45 gave uniformly negative immunohistochemical results.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/33. Intraneural perineurioma involving the median nerve.Intraneural perineurioma is a rare clinical entity, which tends to affect major nerve trunks in the upper extremities. On light microscopy, numerous pseudo-onion-bulb structures having a central clear area are surrounded by concentric layers of eosinophilic elongate cells having spindled nuclei. immunohistochemistry of concentric cells stains positive for epithelial membrane antigen but negative for S100 protein. Because of the small number of cases, no consensus has been made on proper treatment of this entity. Although none of the patients who have had excision of tumor with nerve grafting have had sensory nerve recovery, we believe each patient should be individualized until more data are available regarding this tumor.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
6/33. 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.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/33. Primary extracranial meningioma of the foot: a case report.We present a rare case of primary extracranial meningioma in a 36-year-old man, who had a solitary multinodular mass located in the plantar muscle of the foot. The histology of specimens from simple excision was typical of meningioma, showing bland spindle cell proliferation with a whorl pattern. Immunohistochemical analysis demonstrated that the tumor cells showed diffuse and strong positivity for epithelial membrane antigen as well as moderate reactivity for cytokeratin and vimentin. Ultrastructurally, the tumor cells were characterized by thin bipolar cytoplasmic processes and joined by multiple small desmosomes. There were frequent pinocytotic vesicles and a distinct external lamina on the cell surface. These findings suggest that this primary ectopic meningioma, arising in the soft tissue, may have been derived from perineurial cells of the peripheral nerve, but was morphologically distinguishable from perineurioma. Primary extracranial meningioma should be included in the differential diagnosis of soft-tissue spindle cell tumors, especially those of peripheral nerve origin.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/33. Subcutaneous malignant epithelioid schwannoma with cartilaginous differentiation.BACKGROUND: Malignant epithelioid schwannoma is a rare tumor. The aim of this study is to describe a case of subcutaneous malignant epithelioid schwannoma with cartilaginous differentiation. methods: Histological, immunohistochemical, and ultrastructural examinations were performed on a tumor that arose on the back of a 37-year-old woman. RESULTS: Histologically, the tumor predominantly consisted of small, round or polygonal cells arranged in cords or nests. Immunohistochemical study revealed that the tumors cells were positive for vimentin, S-100 protein, glial fibrillary acidic protein and neuron specific enolase. The cells were negative for HMB-45, keratin, epithelial membrane antigen, and CAM5.2. Electron microscopic study showed closely associated cells with undifferentiated features. The cells were devoid of external laminae and cellular junctions. CONCLUSION: Malignant epithelioid schwannoma should be pathologically differentiated from other epithelioid tumors with cartilaginous elements.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
9/33. Malignant peripheral nerve sheath tumor with perineurial differentiation: "malignant perineurioma".BACKGROUND: Although benign tumors derived from the nerve sheath perineurial cell have been described from a variety of anatomic sites and are known to be a component of a number of benign neoplasms, malignant nerve sheath tumors of perineurial origin are exceedingly uncommon. methods: We report an unusual case of a 70-year-old male who presented with a rapidly growing mass of the left arm, subsequently shown to be a malignant nerve sheath tumor with perineurial differentiation. A brief microscopic differential diagnosis and review of the literature are discussed. RESULTS: Histologic sections show a partially circumscribed tumor of atypical spindle cells arranged in sweeping fascicles embedded in a myxoid matrix with focal whorling. Nuclear pleomorphism was evident among scattered typical and atypical mitotic figures (mean mitotic index of 7/10 high-power fields). The immunophenotypic profile consisted of only vimentin and epithelial membrane antigen (EMA) positivity, while antibodies to S-100, CD34, smooth muscle actin, and pankeratins were negative. Ultrastructural features included spindle cells with long cytoplasmic processes invested by interrupted basal lamina and pinocytotic vesicles, consistent with perineurial differentiation. CONCLUSIONS: While the histogenic source of the benign perineurioma, the perineurial cell has only rarely been described in conjunction with malignant tumors. All cases to date have shown EMA-positive and S-100-negative atypical spindled cells arranged in fascicles embedded in a myxoid matrix. In addition to immunohistochemistry, ultrastructural examination may be necessary to support the diagnosis. The diagnostic differential includes melanoma, spindle cell squamous cell carcinoma, atypical fibroxanthoma, leiomyosarcoma, and conventional malignant peripheral nerve sheath tumor, most commonly of Schwannian differentiation. Recognizing perineurial differentiation is important since few cases have been reported to date and the biological potential of these neoplasms is not known.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
10/33. Benign peripheral nerve sheath tumor of the tongue.Peripheral nerve sheath tumors rarely occur in the oral cavity and include neurofibroma, schwannoma, and palisaded encapsulated neuroma. We report a rare case of benign peripheral nerve sheath tumor of the tongue. This tumor was a 0.8 X 0.5 cm sized, firm mass on the left lateral surface of the tongue. Histologically, this mass was composed of well- circumscribed variable sized nodules, which consisted of moderately cellular spindle cells with vague nuclear palisading and a small amount of fibrous tissue. Most of the tumor cells were strongly positive for S-100 protein, but negative for epithelial membrane antigen on immunohistochemistry. No axons were found by immunostaining for neurofilament and Bodian stains. In addition, the surrounding, compressed, fibrous tissue showed rare EMA- positive cells. The present case might be a rare case of neurofibroma arising in the tongue, although immunohistochemical and special stains did not support such a diagnosis.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
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