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Pedunculated malignant peripheral nerve-sheath tumour of the diaphragm presenting as recurrent lung infection: ultrasound diagnosis.

We report a rare case of malignant peripheral nerve-sheath tumour of the diaphragm in a 12-year-old boy with neurofibromatosis presented clinically and radiographically as recurrent lung infection. Ultrasound revealed a pedunculated diaphragmatic tumour invading the posterior chest wall. This case highlights that this unusual tumour may limit diaphragmatic excursion leading to, and even being masked by, superimposed lung infection. In patients with neurofibromatosis, ultrasound is recommended when chest radiography shows a basal lung opacity.

Child↗

Metastasis of adenoid cystic carcinoma of the mandible to the Gasserian ganglion.

A case of adenoid cystic carcinoma of the mandible in a 47-year-old woman metastasized to the Gasserian ganglion probably through the perineural space of the inferior alveolar nerve. The patient was treated by irradiation after excision of the tumor from the ganglion. She is currently an outpatient in the oral and maxillofacial surgery department; two years and seven months after the first examination, there is no sign of recurrence or metastasis of adenoid cystic carcinoma.

Carcinoma, Adenoid Cystic↗

Neurofibroma with psammoma bodies.

Neurofibromas are benign tumours of the nerve sheath. Histologically they vary depending on their contents of cells, myxoid stroma and collagen. A 41-year old male with radicular pain had a tumour involving the posterior chest wall. Microscopically it resulted to be a neurofibroma with abundant psammoma bodies. Although these bodies are very frequent in some neoplasias, to our knowledge they have not been described in neurofibromas to date.

Adult↗

Malignant peripheral nerve sheath tumor of the pancreas with perineurial cell differentiation.

The authors report a malignant peripheral nerve sheath tumor (MPNST) that developed in the body of the pancreas of a 76-year-old woman. The well-demarcated tumor, 4.5 x 3.5 cm in size, was composed of spindle-shaped cells arranged in a storiform pattern or sweeping fascicles. Ultrastructurally, the tumor cells had elongated cytoplasmic processes with numerous pinocytotic vesicles, and were covered with discontinuous basal lamina-like substances. Immunohistochemically, tumor cells were diffusely positive for epithelial membrane antigen, vimentin, and CD34, but negative for S-100 protein and Leu 7. It was therefore concluded that the tumor was a MPNST with perineurial cell differentiation. Primary MPNST of the pancreas is extremely rare and the immunohistochemical and ultrastructural studies are necessary for its diagnosis.

Aged↗

Malignant peripheral nerve sheath tumors in the setting of segmental neurofibromatosis. Case report.

Approximately 100 cases of segmental neurofibromatosis (NF5) have been reported in the recent literature. Patients with NF5 present with café-au-lait macules, freckles, and/or neurofibromas limited to one or adjacent dermatomes. Neurofibromas arising in NF5 have been uniformly considered to be benign; patients were thought to have an excellent prognosis without the risk of developing malignant peripheral nerve sheath tumors (PNSTs), which are characteristic in patients with the generalized form of this disease, von Recklinghausen's NF. In this report the authors detail the first observations of malignant PNSTs in two patients with NF5. Indications for surgical removal of a neurofibroma in a patient with NF include pain. neurological impairment, compression of adjacent structures, cosmetic disfigurement, and rapid tumor growth suggestive of malignant degeneration. Surgical indications are similar for patients with NF5. All patients with neurofibromas should be considered at risk for malignant degeneration.

Adult↗

Surgical outcomes of 654 ulnar nerve lesions.

OBJECT: In this article the authors present a retrospective analysis of 654 surgical outcomes in patients with ulnar nerve entrapments, injuries, and tumors during a 30-year period. METHODS: Data were gathered between 1968 and 1998 at Louisiana State University Health Sciences Center. Mechanisms of injuries or lesions included 460 entrapments at the elbow level (70%), 76 lacerations (12%), 52 stretches/contusions (8%), 34 fractures/dislocations (5%), 12 gunshot wounds (2%), two injection-induced injuries (0.3%), and 13 nerve sheath tumors (2%). In cases of entrapment, direct operative recordings uniformly demonstrated a slowing of conduction at the elbow, even in cases in which preoperative noninvasive studies had been nondiagnostic. Intraoperative electrical "inching" studies also demonstrated significant conduction abnormalities that lie just proximal to and through the olecranon notch rather than distal, beneath the flexor carpi ulnaris muscle. There were only eight exceptions to this. Lesions not in continuity due to the injury required primary or secondary end-to-end sutures or graft repair. Aided by intraoperative nerve action potential recording, lesions in continuity received either external or internal neurolysis and split repair or resection followed by end-to-end suture or graft repair. Functional recoveries of Grade 3 or better were seen in 81 (92%) of 88 patients who underwent neurolysis, 42 (72%) of 58 patients who received suture repair, and 24 (67%) of 36 patients who received graft repair. Nevertheless, fewer Grade 4 or 5 recoveries were reached than those seen in patients with radial or median nerve injuries. Nerve sheath tumors were resected with preservation of preoperative function in five of seven patients. CONCLUSIONS: Although difficult to obtain, useful functional recovery can be achieved with proper surgical management of ulnar nerve entrapments and injuries.

Action Potentials↗

Outcomes of surgery in 1019 brachial plexus lesions treated at Louisiana State University Health Sciences Center.

OBJECT: Outcomes of 1019 brachial plexus lesions in patients who underwent surgery at Louisiana State University Health Sciences Center during a 30-year period are reviewed in this paper to provide management guidelines. METHODS: Causes of brachial plexus lesions included 509 stretches/contusions (50%), 161 plexus tumors (16%), 160 thoracic outlet syndromes (TOSs, 16%), 118 gunshot wounds (12%), and 71 lacerations (7%). Many features of clinical presentation, including prior treatment, patient's neurological status, results of electrophysiological studies, intraoperative findings, and postoperative level of function, were studied. The minimum follow-up period was 18 months and the mean follow-up period was 42 months. Repairs were best for injuries located at the C-5, C-6, and C-7 levels, the upper and middle trunk, the lateral cord to the musculocutaneous nerve, and the median and posterior cords to the axillary and radial nerves. Conversely, results were poor for injuries at the C-8 and T-1 levels, and for lower trunk and medial cord lesions, with the exception of injuries of the medial cord to the median nerve. Outcomes were most favorable when patients were carefully evaluated and selected for surgery, although variables such as lesion type, location, and severity, as well as time since injury also affected outcome. This was true also of TOSs and tumors arising from the plexus, especially if they had not been surgically treated previously. CONCLUSIONS: Surgical exploration and repair of brachial plexus lesions is technically feasible and favorable outcomes can be achieved if patients are thoroughly evaluated and appropriately selected.

Action Potentials↗

[Desmoid tumor of the chest wall infiltrating into the brachial plexus: report of a resected case].

We report a case of a desmoid tumor which developed in the apex of the chest wall. A 18-year-old woman was admitted with left shoulder pain. Chest X-ray showed a mass shadow in the left upper lung field. Chest MRI demonstrated the mass infiltrated into the left brachial plexus. A desmoid tumor was suspected on percutaneous needle biopsy. Resection of the tumor was performed. The mass was 13 x 9 x 5 cm in size and diagnosed pathologically as desmoid tumor. Adjunctive postoperative radiation therapy of 60 Gy was done. Postoperative course was uneventful except motor disturbances of the left fingers. At 15 months postoperatively, there was no evidence of recurrence.

Adolescent↗

Perineural invasion is not predictive of biochemical outcome following prostate brachytherapy.

PURPOSE: The purpose of this article is to evaluate whether the presence of perineural invasion (PNI) in the biopsy specimen is predictive of 5-year biochemical disease-free outcome after prostate brachytherapy. MATERIALS AND METHODS: Four hundred twenty-five patients underwent transperineal ultrasound-guided prostate brachytherapy using either 103Pd or 125I for clinical T1b/T3a NXMO (1997 American Joint Committee on Cancer) adenocarcinoma of the prostate gland from April 1995 to October 1999. No patient was lost to follow-up, and no patient underwent pathological lymph node staging. Two hundred twenty-one patients were implanted with 103Pd, and 204 patients were implanted with 125I. Of this cohort, 190 patients were implanted without supplemental beam radiation, and 235 received moderate-dose external-beam radiation therapy followed by a prostate brachytherapy boost. One hundred sixty-three patients received hormonal manipulation in conjunction with the brachytherapy implant (86 of these patients received moderate-dose external-beam radiation therapy before brachytherapy), and 262 patients were hormone naïve. Perineural invasion, defined as carcinoma tracking along or around a nerve within the perineural space, was identified in 105 patients (24.7% of the population). The median patient age was 68 years (range, 48-81 years). The mean follow-up was 37.1 +/- 15.2 months, and the median follow-up was 35.4 months (range, 6-74 months). Follow-up was calculated from the date of implantation. Biochemical disease-free survival was defined by the American Society of Therapeutic Radiation and Oncology (ASTRO) consensus definition. RESULTS: When Kaplan-Meier survival analysis was performed, the presence of PNI did not predict failure. When PNI was entered into a Cox regression analysis with evaluated clinical predictors of failure (age, clinical T stage, pretreatment prostate-specific antigen level, and Gleason score) or treatment parameters (use of neoadjuvant hormonal therapy, supplemental external-beam radiation therapy, and choice of isotope), PNI did not add to the predictive strength of these variables. The median disease-free prostate-specific antigen level was 0.1 ng/mL for the entire cohort. CONCLUSIONS: Our results indicate that actuarial 5-year biochemical outcomes with a prostate brachytherapy approach that utilizes generous periprostatic margins is not dependent on the presence of PNI. In addition, PNI should not be used as an independent prognosticator in determining the need for combined-modality therapy in patients undergoing prostate brachytherapy.

Adenocarcinoma↗