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At least 73 records · Page 4Linked to original sources
PubMed1995

Cytogenetic findings in malignant peripheral nerve sheath tumors.

Clonal chromosome aberrations were detected in 8 short-term cultured malignant peripheral nerve sheath tumors (MPNST). Seven had a near-triploid chromosome number and I was in the hyperhaploid-hypodiploid range. No recurrent structural rearrangements were found; the bands most frequently involved (3 tumors) were 7p11, 12p13 and 14q11. The most common numerical changes were loss of a sex chromosome (all tumors) and loss of at least 1 copy of chromosomes 8, 16 and 22 (4 tumors). Pooling our data with those on the 20 previously published MPNST with abnormal karyotypes, we found that the chromosome number has often been in the triploid range (12 tumors), with stem line variation between 34 and 270. All chromosome arms, except 22p and the Y chromosome, were involved in recombinations. The most frequently rearranged bands were 7p22 (6 tumors) and 1p21, 7p11 and 14q11 (5 tumors each). Most numerical and unbalanced structural aberrations have led to loss of genetic material, in particular from Xq26-qter (13 tumors); 11q22-qter and 13p (12 tumors); 9p22-pter, 11p13-pter, 17p and 17q11-21 (11 tumors); 1p22-32 and 1p34-pter (10 tumors) and 6q25-qter and chromosome 16 (9 tumors).

Adolescent↗
PubMed1994

Malignant peripheral nerve sheath tumors of the head and neck: a clinicopathological study.

Twenty-seven cases of malignant peripheral nerve sheath tumors involving the head and neck region over a period of 7 years were reviewed. They were graded from 1-3 based on necrosis, mitosis, cellularity, and pleomorphism. Mean age of occurrence was 42 years, with a range of 12-70 years. Male preponderance was noted (M:F = 3.5:1). The most common site of involvement was the neck (44.6%). The main presenting symptom was an enlarging mass. The nerve of origin could be identified in 33% of patients. Treatment consisted of wide excision. The 5-year observed survival was 33%. Fifty-two percent of patients developed local recurrence of disease. Fifteen percent of patients died due to advanced local disease within 18 months of treatment. Distant metastasis was seen in 18.5% of patients. Lymph node metastasis was not seen. At the end of 5 years 15% of patients remained disease free. Large tumor size (> 5 cm) adversely affected the prognosis (P = < 0.1). No significant correlation was noted between the grade of tumor and survival.

Adolescent↗
PubMed2004

Bilateral intraneural perineurioma presenting as ulnar neuropathy at the elbow.

We describe a 36-year-old woman with progressive bilateral ulnar neuropathy. Sonographic and magnetic resonance imaging studies revealed extensive focal ulnar nerve enlargement at the elbow. Histological studies gave evidence of an intraneural perineurioma. Because intraneural perineurioma usually appears as a single mass lesion at sites other than typical entrapment sites, this mode of presentation is unusual. We discuss the nature of this benign tumor and the differential diagnosis of nerve enlargement. Knowledge of possible causes of nerve thickening is crucial when performing imaging in patients with neuropathies.

Action Potentials↗
PubMed1993

Malignant nerve sheath tumors of the head and neck: four case studies and review of the literature.

Malignant peripheral nerve sheath tumors (MPNST) are very uncommon neoplasms. While the incidence of these lesions is estimated to be 0.001% in a general population, they make up 5-15% of all soft tissue sarcomas in the head and neck region. We present four cases of MPNST in the head and neck. Since certain difficulties were encountered in diagnosis, the importance of clinical evaluation is emphasized. The prognosis for these tumor patients is poor in spite of improvements in diagnostic and therapeutic modalities.

Adult↗
PubMed2002

Epithelioid sarcoma of the sciatic nerve perineural sheath: a mimic of nerve sheath tumor.

We report herein a rare case of epithelioid sarcoma, in a 39-year old lady involving the sciatic nerve. Clinically and radiologically it stimulated a nerve sheath tumor. Involvement of a nerve by an epithelioid sarcoma is extremely uncommon. To the best of our knowledge, this is the first case of an epithelioid sarcoma involving the sciatic nerve and needs documentation.

Adult↗
PubMed2003

Soft tissue perineuriomas in children: report of three cases and review of the literature [corrected].

Perineuriomas (PN) are uncommon, slowly growing, usually benign tumors composed of well-differentiated perineural cells. Two variants are recognized: intraneural perineuriomas and soft tissue perineurioma, which includes a sclerosing subset of tumors. They are usually reported in the adult population. We present three cases of soft tissue perineuriomas in children. One was located in the deep soft tissue of the retroperitoneum in a 14-year-old girl, the second one in the left thumb of a 14-year-old boy, and the third one in the index finger of a 16-year-old boy. This report, which describes the clinicopathologic, immunohistochemical, and ultrastructural features of these tumors, should alert pathologists to the occurrence of perineuriomas in children. A review of the English language literature on perineuriomas in children is also included.

Adolescent↗
PubMedDate not supplied

Malignant peripheral nerve sheath tumor of bone in children and adolescents.

Malignant peripheral nerve sheath tumor (MPNST) of bone is a rare entity. We have examined three lesions that fit standard histopathologic criteria for MPNST of soft tissues but that arose in the skeleton of three children aged 6 to 13 years. None was affected by neurofibromatosis 1 (NF1). Histologic features typical of MPNST included spindle cells with comma-shaped nuclei, tactoid bodies, nuclear palisading, hyaline bands, and schwannoma-like and curlicue foci. Epithelioid foci were seen in two cases, and heterologous differentiation in one. Immunohistochemistry revealed positivity for S-100 (1 positive/3 tested), vimentin (3/3), glial fibrillary acidic protein (2/3), CD34 (1/1), and CD68 (1/2). Studies for CD99 (0/3), epithelial membrane antigen (0/3), cytokeratin (0/3), CD57 (0/3), and HMB-45 (0/2) were negative. Ultrastructural findings in one of two cases examined included interlacing, attenuated cytoplasmic processes, microtubules, and rare dense-core granules. We conclude that MPNST may arise as a primary bone neoplasm in children without NF1.

Adolescent↗
PubMed2003

Brain Tumors in Neurofibromatosis.

The neurofibromatoses are common neurocutaneous syndromes with multisystem involvement. These disorders place patients at increased risk for the development of malignancies. In particular, there is a predisposition to develop central nervous system and peripheral nervous system neoplasms. Distinct tumor types develop in association with neurofibromatosis type 1 (NF-1) different from those that are typically associated with neurofibromatosis type 2 (NF-2). In general, the tumors associated with NF-1 and NF-2 tend to demonstrate a more indolent course than similar tumors in patients without neurofibromatosis. An exception would be earlier presentation of tumors in each of these disorders. Management decisions are based on multiple factors. These include tumor location, presumed or known histology, and patient symptoms at time of diagnosis or evidence of progression either clinically or as demonstrated by neuroimaging. Once all of these factors have been weighed, therapeutic considerations include expectant observation, surgery, and radiation or chemotherapy. The overall philosophy of treatment is that of attempting to preserve neurologic function for as long as possible, because these are progressive disorders. At times, it may be preferable to keep interventions to a minimum in order to achieve this goal. A multidisciplinary approach is crucial in the care of these patients.

Journal Article↗
PubMed1976

Multiple endocrine adenomas in a patient with the Maffucci syndrome.

A patient with multiple cutaneous hemangiomas and skeletal dyschondroplasia (the Maffucci syndrome) was found to have a pituitary chromophobe adenoma, a parathyroid adenoma and two other neoplasms. The presence of two endocrine tumors suggested the syndrome of multiple endocrine adenomatosis, and raised the issue of an etiologic relationship between this disease and the Maffucci syndrome. Dyschondroplasia, however, has no known influence on the secretion of parathyroid hormone or any of the pituitary hormones. The Maffucci syndrome is associated with a high incidence of malignancy, but it involves primarily mesodermal derivatives whereas multiple endocrine adenomatosis affects tissues of ectodermal origin. The association of the two in our patient is probably fortuitous.

Adenoma, Chromophobe↗
PubMed1975

Optic nerve extension of intraocular neoplasms.

Histologic examination of ten intraocular melanomas (eight choroidal and two of the iris and ciliary body) with dispersion of tumor cells into the vitreous body showed optic nerve invasion ranging from only a few collections of tumor cells lying in the anterior portion of the nerve, to invasion of the entire nerve and possibly the chiasm. Nine of the ten melanomas were partially necrotic. Orbital recurrence developed in four cases in which the tumors extended to the surgically cut margin of the excised optic nerve. All of the eyes had no light perception and had angle-closure glaucoma. In a companion retrospective study of 38 endophytic retinoblastomas with optic nerve invasion, 29 had angle-closure glaucoma. The posterior dispersion of tumor cells, and possibly their optic nerve head invasion, may have been enhanced by the obstruction of the normal anterior segment aqueous outflow channels and the associated glaucoma.

Aged↗
PubMed1975

Computerized axial tomography in the detection of orbital space-occupying lesions.

Of 41 patients we examined for a suspected primary orbital tumor, 25 patients had evidence of a lesion either histologically, angiographically, or surgically. We examined nine of the 25 patients by means of an EMI-Scanner and 16 by a fine matrix addition (160 times 160 cells), and compared these results to those we received by axial tomography, orbital venography, and B-scan ultrasound. We also compared the absorption values of the recorded tumors. The EMI-Scanner had a diagnostic accuracy of 84%; orbital venography, 84%; axial hypocycloidal tomography, 71%; and ultrasound, 76%. The fine matrix scanner gave a preoperative diagnostic rate of 93% and defined optic nerve abnormalities accurately. However, EMI, scanning should complement existing noninvasive procedures and orbital venography.

Adolescent↗
PubMed1975

Retinal cryoprobe in orbital tumor management.

We used the retinal cryoprobe in the surgical management (biopsy and excision) of orbital and ocular adnexal tumors in four patients. The strong bond between the cryoprobe tip and the tumor allowed application of moderate traction to the tumors, facilitating their removal. The amount of traction on the tumor or cyst appeared to be much greater than that caused by forceps or clamps. In one case, however, marked traction with the cryoprobe caused rupture of the tumor capsule. In another case, in which a cyst wall was entered during dissection, we applied the cryoprobe to the opening, sealing the hole with an ice ball, to keep the cyst from collapsing. Histopathologic structure was not altered by cryoprobe applications.

Adult↗
PubMed1977

Congenital neurogenous sarcoma with rhabdomyosarcomatous differentiation.

A congenital malignant peripheral nerve sheath tumor contained small rhabdomyosarcomatous portions in both the retroperitoneal pelvic primary and in the subpleural lung metastasis. There was no family history of Von Recklinghausen's disease. Of the 10 patients with this tumor previously reported, the youngest is 14 yr old, and eight had familial neurofibromatosis.

Adult↗