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At least 523 records · Page 29Linked to original sources
PubMed1999

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.

Antigens, CD34↗
PubMed2002

Malignant granular cell tumor of the lateral femoral cutaneous nerve: report of a case with cytogenetic analysis.

Malignant granular cell tumors (MGCTs) are rare neoplasms of uncertain histogenesis. We report a case of MGCT involving a peripheral nerve with peritoneal and omental dissemination in which cytogenetic findings are available. Our results show that MGCTs share some cytogenetic abnormalities with malignant peripheral nerve sheath tumors (MPNSTs), supporting the hypothesis that they may represent histogenetically related lesions.

Adult↗
PubMed1996

Pretreatment with [131I] metaiodobenzylguanidine and surgical resection of advanced neuroblastoma.

Pretreatment with [131I] metaiodobenzylguanidine (MIBG) followed by surgical resection in advanced neuroblastoma (stage 3 and 4) has been studied in relation to resectability, morbidity and mortality, survival rate after two years, control of distant metastasis and serum levels of LDH as prognostic factors. Twenty-one patients with advanced neuroblastoma were primarily treated with MIBG radiotherapy, followed by surgical resection. Sixteen patients had stage 4 disease. Between 2 and 6 courses of MIBG treatment were given per patient. In 17 patients gross complete resection was achieved. Two patients developed complications directly related to the operation, one died as a result of this. The overall mortality was 38%. MIBG therapy resulted in partial response in 13 patients and in stable disease in 8 patients. Two years survival in the group with partial response was 86% and in the group with stable disease 28%. Because of the resulting excellent general condition of the patients the interval between pretreatment with MIBG and surgery could be very short. Follow-up till December 1994 showed that 13 children were alive for 3 to 47 months. Seven had no evidence of disease. Preoperative MIBG de novo treatment in advanced neuroblastoma is equal to induction chemotherapy, but less toxic.

3-Iodobenzylguanidine↗
PubMed1984

Myxopapillary ependymoma of the cauda equina with extension to the skin of the sacrococcygeal region.

Cutaneous involvement by myxopapillary ependymoma of the cauda equina is described clinically and morphologically. The patient, a young woman, was 12 years old at the time that the neoplasm was diagnosed. Skin involvement developed 12 1/2 years following the initial diagnosis of the primary neoplasm and after the third attempt at surgical extirpation. Involvement of the skin preceded the death of the patient by 11 months.

Adolescent↗
PubMed1984

Malignant peripheral nerve sheath tumors arising from ganglioneuromas.

Two typical malignant peripheral nerve sheath tumors (PNST) arising in preexisting ganglioneuromas are described. To the best of our knowledge, this association of tumors has not been reported in detail previously. Neither patient had the stigmata nor family history of Von Recklinghausen's neurofibromatosis. In both cases, the ganglioneuromas evolved from more primitive neuroectodermal tumors (one neuroblastoma, one ganglioneuroblastoma) and both patients developed their malignant PNST at previously irradiated sites. Both patients died within 2 years of the diagnosis of their malignant PNST. The origin of these malignant PNSTs from Schwann cells is supported by an ultrastructural analysis of 27 neuroblastomas, ganglioneuroblastomas, and ganglioneuromas.

Adolescent↗
PubMed1991

Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma).

Twenty-six cases of malignant peripheral nerve sheath tumor with a predominant epithelioid pattern were studied to determine the range of its histologic patterns, immunophenotype, and biologic behavior. The tumor presented as an asymptomatic mass either in superficial (16 cases) or in deep soft tissue (10 cases) of the extremity. Characteristically, those in deep soft tissue were composed of vague nodules of varying cellularity made up of cords or strands of rounded epithelioid cells with prominent nucleoli. Those in superficial soft tissue were uninodular masses composed of tight clusters of cells showing cell-to-cell molding but possessing the same prominence of nuclei and mitotic activity as those in deep soft tissue. Several were associated with a preexisting benign nerve sheath tumor. A number of cases deviated from the above description, including cases that resembled a clear cell carcinoma, a malignant rhabdoid tumor, and a pleomorphic sarcoma. The majority of cases (80%) strongly expressed S-100 protein and neuron-specific enolase, but all lacked a melanoma-associated antigen (as defined by HMB-45) and cytokeratin. Stains for type IV collagen defined linear immunoreactivity around single cells and groups of cells. This pattern did not differ substantially from that of melanomas and therefore did not serve as a reliable discriminant. Follow-up information indicated a more favorable course for those in superficial soft tissue compared with those in deep sites. Two of 16 patients in the former group developed metastatic disease compared with three of 10 in the latter group. Tumors in superficial soft tissue may be eminently treatable and curable, depending on size.

Adolescent↗
PubMed1999

Cutaneous sclerosing perineurioma with cryptic NF2 gene deletion.

Sclerosing perineurioma is a recently described variant of perineurioma that characteristically occurs in the fingers and palms of young adults. We report a cutaneous sclerosing perineurioma with preservation of the axons and Schwann cells in the center of the whorls of perineurial cells, a feature that previously was reported to be typically absent in these lesions. Additionally, clonal chromosomal abnormalities of chromosome 10 and a cryptic deletion of the 5'BCR and NF2 loci on chromosome 22 were present. These findings further support the hypothesis that a gene on chromosome 22 may play a role in the pathogenesis of perineurioma. The NF2 gene is a logical candidate because of its involvement in other nerve sheath tumors.

Adolescent↗
PubMed2005

Neurotropic melanoma of the head and neck with clinical perineural invasion.

OBJECTIVE: The purpose of this article is to report our experience with neurotropic melanoma, a rare malignancy that sometimes produces neurologic symptoms because of a direct extension of the primary tumor. METHODS: We report 3 consecutive patients with neurotropic melanoma of the head and neck who presented with clinical perineural invasion. RESULTS: Two patients had incompletely resectable tumors and were treated with definitive radiotherapy (RT), and 1 patient received surgery and postoperative RT. One patient experienced recurrence in a regional lymph node 30 months after RT and underwent salvage surgery; he is disease-free at 45 months after initial treatment. The remaining 2 patients are disease-free 34 months and 14 months after treatment. CONCLUSIONS: Radiotherapy alone or combined with surgery may provide relatively long-term local control in patients who have neurotropic melanoma with clinical perineural invasion.

Adult↗
PubMed2004

Cervical sympathetic chain schwannoma.

OBJECTIVES: Schwannomas are benign, slow-growing tumors that arise from nerves. Those originating from the sympathetic cervical chain are rare. We describe our experience with the clinical presentation, surgical management, and outcomes of patients with this pathology. STUDY DESIGN: Retrospective chart review of a case series in a tertiary referral center. METHODS: Four cases of cervical sympathetic chain schwannomas were reviewed. Patients presented with either an asymptomatic neck mass discovered on routine physical examination (1 patient), an enlarging neck mass (2), or an acute onset of a Horner's syndrome (1). All patients underwent preoperative imaging (magnetic resonance imaging, computed tomography, or both). RESULTS: The location and soft-tissue characteristics of the mass, along with displacement of the carotid sheath vessels, were typical of a cervical sympathetic chain schwannoma. All patients underwent surgical excision of the mass. Postoperative Horner's syndrome was encountered in all patients. First bite syndrome was encountered in two patients. CONCLUSIONS: Cervical sympathetic chain schwannomas are rare tumors. Preoperative imaging characteristics facilitate the diagnosis. First bite syndrome can occur and may be debilitating postoperatively. Long-term prognosis is excellent.

Aged↗
PubMed2006

Soft tissue perineurioma in a patient with neurofibromatosis type 2: a tumor not previously associated with the NF2 syndrome.

Neoplasms that commonly affect patients with neurofibromatosis type 2 (NF2) include schwannomas, meningiomas, astrocytomas, ependymomas, and neurofibromas. Perineuriomas are rare tumors of the peripheral nerve sheath that share some characteristics with meningioma. As in both NF2-associated and sporadic cases of schwannoma and meningioma, perineuriomas often harbor mutations or deletions of the NF2 gene. However, perineuriomas have not previously been reported in the clinical setting of NF2. A 30-year-old man with a history of bilateral vestibular schwannomas, a parasagittal meningioma, an intraspinal ependymoma, and multiple other neoplasms involving both cranial and peripheral nerves (thereby fulfilling the diagnostic criteria for NF2) presented with an enlarging thigh mass. The diagnosis of cellular soft tissue perineurioma was confirmed by both immunohistochemical and ultrastructural analysis. This case represents the first report of a soft tissue perineurioma arising in the setting of NF2.

Adult↗
PubMed2004

Retroperitoneal malignant peripheral nerve sheath tumor: evaluation with serial FDG-PET.

Retroperitoneal malignant peripheral nerve sheath tumor (MPNST), a rare type of neurogenic tumor, was diagnosed in a 14-year-old girl with a history of neurofibromatosis type 1 (NF1). Immunochemistry demonstrated spindle cells positive for S-100 protein. The patient had multiple tumor recurrences and she was evaluated with serial F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET). A tumor in the right iliac wing showed increased FDG uptake on PET. FDG-PET played an important role in therapy planning and subsequent follow up. This case emphasizes the important role FDG-PET could play in the staging, restaging, and posttherapy follow up of MPNST.

Adolescent↗