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PubMed1986

Nasopharyngeal carcinoma metastatic to the cauda equina.

A case of nasopharyngeal carcinoma metastatic to the cauda equina is reported. The patient, treated 3 years earlier for his primary disease, presented with bilateral weakness of his legs. Myelographic studies showed only patchy thickening of certain lumbosacral nerve roots. A biopsy from a root in the cauda equina was made through a T-12 to L-2 laminectomy revealing carcinomatous involvement. Careful examination of the specimen was necessary to distinguish the tumor from a lymphoma. Review of the literature has revealed no other cases of this type of neoplastic metastasis.

Adult↗
PubMed1994

Cytogenetic analysis of soft tissue sarcomas. Recurrent chromosome abnormalities in malignant peripheral nerve sheath tumors (MPNST).

Malignant peripheral nerve sheath tumors (MPNST) are known to develop in patients with neurofibromatosis 1 (NF1), thus providing an excellent model for the study of multistep carcinogenesis in genetically predisposed individuals. To determine the sites of gene(s) involved in such a process, we have performed cytogenetic analysis on 10 tumors. The patients were five males and five females ranging in age from 15 to 77 years. Nine patients had NF1. Karyotypic analysis of these tumors exhibited complex clonal abnormalities of several chromosomes. Recurrent abnormalities (numerical as well as structural) of chromosomes 1, 11, 12, 14, 17, and 22 occurred in a substantial proportion of tumors studied. Although abnormalities of these chromosomes have been seen in a variety of other tumors, the aberrations of chromosomes 17 and 22 are of particular interest; chromosomes 17 and 22 carry the genes for NF1 and NF2, respectively. In addition to other clonal aberrations, six tumors had abnormalities of both chromosomes 17 and 22, while three tumors only had an abnormality of chromosome 17. In eight tumors a structural abnormality of chromosome 17 included deletion or a relative deficiency of 17p; in four of the tumors there was also either deletion or rearrangement of the NF1 locus at the cytogenetic level. One tumor had monosomy of chromosome 17. The abnormality of chromosome 22 was deletion of 22q11.2-->qter. This study suggests that the germline mutation in one of the copies accompanied by loss or inactivation of the second copy of the NF1 gene and tumor suppressor gene(s) on 17p and 22q may be associated with the neoplastic transformation; abnormalities of other chromosomes may be related to progression of MPNST. Although the role of the p53 gene in carcinogenesis is well documented in several tumor types, the role of the NF2 gene or other unidentified tumor suppressor gene(s) on chromosomes 22q, 1p, 11, 12, 14 remains to be seen.

Adolescent↗
PubMed1993

Cutaneous malignant peripheral nerve sheath tumour (MPNST) of the hand: a review of current literature.

Malignant peripheral nerve sheath tumours (MPNST) are spindle cell sarcomas normally situated in the deep soft tissues. Cutaneous MPNST is an uncommon variant, usually occurring in the head and neck. When it arises in the upper limb this tumour may pose a considerable diagnostic and therapeutic problem. We present a case of cutaneous MPNST arising in the hand. Tumour extension was exclusively perineural along three major nerve trunks from an interdigital origin. Current knowledge of the clinical behaviour of cutaneous MPNST is reviewed.

Amputation, Surgical↗
PubMed2006

Intraneural perineurioma of the oral mucosa.

A 16-year-old boy presented with an intraneural perineurioma arising from a small nerve in the buccal mucosa. Histologically, the tumour comprised a tortuous proliferation of spindle cells arranged like an onion bulb. To our knowledge this is the third example of an intraneural perineurioma that did not present in a major nerve.

Adolescent↗
PubMed2006

Preoperative diagnosis of extrapancreatic neural invasion in pancreatic cancer.

BACKGROUND & AIMS: Pancreatic cancer recurs in most patients after resection with curative intent. Recurrence is particularly common in patients with extrapancreatic neural invasion (EPNI), the presence of which correlates with poor prognosis. Macroscopic EPNI may be detected with conventional noninvasive imaging and endoscopic ultrasound (EUS) imaging, but microscopic EPNI has required postoperative pathologic examination of surgical specimens. We report the preoperative diagnosis of cancer infiltration into celiac ganglia. We hypothesized that microscopic pancreatic cancer metastasis to neural ganglia can be detected by EUS-guided biopsy examination. METHODS: We performed a retrospective review of patients with pancreatic cancer undergoing EUS in whom celiac ganglia were sampled to exclude malignant infiltration. RESULTS: Six patients with pancreatic cancer underwent EUS-guided fine-needle aspiration or trucut biopsy examination of presumed celiac ganglia. Metastatic cancer was found in ganglia of 2 patients. Specimen review identified adenocarcinoma and neural tissue in the absence of lymphocytes. At laparoscopy, 1 of the 2 patients with positive celiac biopsy specimens also had several unexpected peritoneal metastatic deposits. The other patient was considered to have locally advanced unresectable disease. Both patients are receiving supportive care. CONCLUSIONS: EPNI may be shown preoperatively in patients with pancreatic cancer using EUS-guided sampling of celiac ganglia. A preoperative diagnosis of EPNI has the potential to improve staging accuracy and patient outcomes.

Adenocarcinoma↗
PubMed2006

Causes of lumbosacral plexopathy.

The lumbosacral plexus represents the nerve supply to the lower back, pelvis and legs. This review will focus on diseases and disorders affecting the pathway as demonstrated by magnetic resonance imaging (MRI) and computed tomography (CT). We stress the need to review the lumbosacral plexus in patients with non-specific symptoms such as back, hip, pelvic pain, and in those who present with sciatica unaccompanied by demonstrable intervertebral disc prolapse. We illustrate that the imaging appearances may be non-specific and re-inforce the importance of the clinical history and the use of tissue sampling to achieve an accurate diagnosis.

Abscess↗
PubMed2005

Intraneural perineurioma of the radial nerve in a child.

An intraneural perineurioma is an uncommon solitary neoplasm of major nerve trunks composed of perineurial cells from the peripheral nerve sheath. The typical course of intraneural perineurioma is indolent, with gradual-onset motor loss and presentation for evaluation months to years after onset of symptoms. We report a 9-year-old girl with an intraneural perineurioma of the radial nerve. Diagnosis was confirmed by histology and immunohistochemistry. Resection and nerve graft reconstruction were performed to prevent progression of motor compromise and to allow recovery of motor function. An algorithm for the management of focal intraneural tumors is shown.

Child↗
PubMed2005

Intraneural perineurioma in a child with Beckwith-Wiedemann syndrome.

To the authors' knowledge, this is the first report of an intraneural perineurioma in a child with Beckwith-Wiedemann syndrome. Intraneural perineurioma, previously known as localized hypertrophic neuropathy, is a rare benign peripheral nerve sheath tumor arising from perineurium. This report adds a new entity in the spectrum of tumor formation in Beckwith-Wiedemann syndrome.

Beckwith-Wiedemann Syndrome↗
PubMed2006

Malignant peripheral nerve sheath tumor arising from the colon in a newborn: report of a case and review of the literatures.

A malignant peripheral nerve sheath tumor (MPNST) is a rare neoplasm arising from peripheral nerve sheath. Here, we report the first case of MPNST arising in the colon and also the youngest case of MPNST in the gastrointestinal tract. The patient was a 2-day-old neonate with symptoms and signs of intestinal obstruction. The patient had no family history or stigmata of neurofibromatosis type 1. A computed tomographic scan revealed a 5-cm-sized mass in ascending colon causing intestinal obstruction, and emergent right hemicolectomy was performed. The microscopic examination showed atypical spindle cells with hyperchromatic nuclei and high mitotic activity. The results of immunohistochemical staining, which showed positivity for S-100 and vimentin as well as negativity for smooth muscle actin, CD34, and c-Kit, supported the final diagnosis of MPNST. Genetic analysis of the patient revealed no abnormalities. After surgery, the patient recovered uneventfully and has been free of the disease for 17 months.

Colon↗
PubMed2005

Orbital invasion by periocular basal cell carcinoma.

OBJECTIVES: To present a large series of patients with orbital invasion by periocular basal cell carcinoma (BCC). DESIGN: Retrospective, noncomparative, interventional case series. PATIENTS: All cases diagnosed with orbital invasion by periocular BCC between January 1985 and July 2004 in 3 Orbital Units in Australia. METHODS: The clinical records of all patients were reviewed. MAIN OUTCOME MEASURES: Patients' demographics, clinical presentation, histologic subtypes, treatment modalities, recurrence rate, and tumor-related death. RESULTS: There were 64 patients (49 males) with a mean age of 70+/-13 years. Most tumors (84.4%) were recurrent or previously incompletely excised, and the medial canthus was most frequently involved (56.2%). Signs suggestive of orbital involvement included a mass with bone fixation (35.7%), limitation of ocular motility (30.4%), and globe displacement (17.6%). There were no signs suggestive of orbital invasion in 35.7%. Most patients (51.6%) had infiltrative histologic findings, and perineural invasion was present in 19.3%. Treatment modalities were mainly exenteration alone or combined with radiotherapy. During a mean follow-up period of 3.6 years, 3 cases of recurrence (4.7%) were diagnosed. Only 1 patient (1.6%) died from tumor-related causes. CONCLUSIONS: Orbital invasion by periocular BCC is an uncommon event that may be associated with significant ocular morbidity and, rarely, death. Because orbital invasion may often be clinically silent, clinicians need to be alert to the possibility in high-risk tumors and consider appropriate imaging. Surgical treatment with exenteration or excision, with or without radiotherapy, results in a low recurrence and mortality rate.

Adult↗
PubMed1985

Soft tissue sarcomas in childhood.

The authors discuss the epidemiology, pathology, clinical presentation, and treatment of rhabdomyosarcoma as well as some of the less common sarcomas of childhood and adolescence. Special focus is placed on making careful histopathologic distinction between entities and on clearly recognizing distinct clinical syndromes.

Adolescent↗
PubMed1999

Peripheral nerve tumors of the orbit.

Neurofibromas and schwannomas are orbital neoplasms with similar clinical and radiologic features. Solitary or circumscribed neurofibromas and schwannomas demonstrate slow progressive growth with ocular displacement and are amenable to surgical resection. Plexiform and diffuse neurofibromas are diffusely infiltrative in the orbit and periocular region, they are difficult to resect, and they have a high rate of recurrence. Malignant peripheral nerve tumors are rare, but they have a very high rate of recurrence and a low 5-year survival rate because of extension through the superior orbital fissure to the midbrain region. This article describes the clinical and radiologic features of peripheral nerve sheath tumors.

Disease Progression↗
PubMed1997

Development of a malignant peripheral nerve sheath tumor following treatment for testicular seminoma.

A novel case is reported in which an S2 nerve root malignant peripheral nerve sheath tumor was diagnosed approximately 8 years after treatment for Stage I testicular seminoma. This patient underwent right orchiectomy and subsequent irradiation therapy to the periaortic region, including the sacrum. Postoperative radiation therapy likely played a role in the development of this second malignancy.

Adult↗
PubMed1992

Malignant peripheral nerve sheath tumor of the orbit in a 15-month-old child. Nine-year survival after local excision.

BACKGROUND: Malignant peripheral nerve sheath tumors are extremely rare orbital tumors that carry a poor prognosis despite wide excision with disfiguring surgery, chemotherapy, or radiotherapy. The authors present the youngest reported case, a 15-month-old boy who underwent an orbitotomy to excise a bilobed tumor from the right orbit. FINDINGS: Histologic examination revealed a malignant peripheral nerve sheath tumor; standard treatment options, including orbital exenteration, chemotherapy, and radiotherapy, were denied and instead the patient was followed with serial computed tomography scans and magnetic resonance imaging. RESULTS: Nine years later, the patient remains without evidence of recurrent tumor and visual acuity is 20/20. CONCLUSION: This patient's course suggests that orbital malignant peripheral nerve sheath tumors, if believed to be completely excised at the time of surgery, might be followed with careful neuroimaging studies for signs of recurrence.

Follow-Up Studies↗
PubMed1997

Perineural spread of cutaneous squamous cell carcinoma via the orbit. Clinical features and outcome in 21 cases.

OBJECTIVE: To describe the clinical features, treatment, and outcome of a series of patients with perineural spread (PNS) of cutaneous squamous cell carcinoma (SCC) via the orbit. DESIGN: A cohort study. PARTICIPANTS: Twenty-one patients identified with PNS of cutaneous SCC via the orbit. INTERVENTION: Patients were treated with various combinations of conservative or radical surgery, external beam radiation therapy, and chemotherapy. RESULTS: Of 21 cases, 17 (81%) were male. Age at presentation with PNS ranged from 38 to 82 years (median, 66 years). The forehead and eyebrow were the most common site of the primary lesion. All but one had altered or decreased sensation, but only nine presented with pain. Fourteen (67%) had ophthalmoplegia at presentation, and 14 (67%) had evidence of PNS involving branches of the facial nerve. Despite combinations of radical surgery, conservative surgery, and radiation therapy, no method of treatment appeared more effective, and 14 patients died from 9 months to 5 years after presentation with PNS (median, 3 years), usually from local and intracranial disease. Two survived to 14 and 18 years, one is alive at 3 years with recurrent local and distant disease, and four are alive without evidence of disease at 2, 3, 4 and 12 months after radiation therapy. CONCLUSIONS: Perineural spread of cutaneous SCC via the orbit carries a poor prognosis. Early radiation therapy may offer the best form of palliation. The role of radical surgery probably is limited once orbital involvement is apparent, as the cavernous sinus and facial nerve branches usually are involved.

Adult↗
PubMed1986

Olfactory neuroblastoma with spinal metastasis--a problem in diagnosis.

The olfactory neuroblastoma or esthesioneuroblastoma is a rare neuroectodermal tumor originating from the olfactory neuroepithelium, which can metastasis via cerebrospinal fluid pathways. In the present case of an extensive nasal malignancy with cervical lymph node metastases in a 75-year old woman, its difficult histology alternatively led to a diagnosis of anaplastic carcinoma and non-Hodgkin lymphoma. The patient died from complications following spread of the tumor to the spinal cord and cauda equina. Review of the literature shows that this tumor is notorious for its chameleonic character. In view of its clearly demonstrated malignancy an aggressive therapeutic approach is advocated.

Aged↗
PubMed1997

Lesions of the cauda equina: a clinical and pathology review from the Armed Forces Institute of Pathology.

We reviewed the clinical records of 149 patients with pathologically proved cauda equina lesions in order to define the relative frequency and clinical presentations of the various diagnoses. The most common pathology was ependymomas (47 patients) followed in frequency by nerve sheath tumors (35 patients), metastases (27 patients), nonependymal glial neoplasms (six patients), meningiomas (six patients), lipomas (five patients), paragangliomas (five patients) and various other diagnoses (19 patients). Mean patient age at presentation for the various lesions included: metastases (51.5 years), nerve sheath tumors (49.7 years), nonependymal glial tumors (46.5 years), paragangliomas (41.2 years), ependymomas (38.3 years), meningiomas (34.7 years), and lipomas (18.4 years). ANOVA showed that the relationship between age and diagnosis for these groups to be statistically significant at a high level (P = 0.002). Low back pain was the most common symptom and occurred in 44 patients. Other symptoms included unilateral lower extremity pain or tenderness (24 patients), bilateral lower extremity pain or tenderness (16 patients), and bilateral lower extremity weakness (16 patients). No relationship between pathologic diagnosis and specific symptoms was found.

Academies and Institutes↗