[Rare differential diagnosis in a 40-year old patient with sleep apnea syndrome]. / Seltene Differenzialdiagnose bei einem 40-jährigen Patienten mit Schlafapnoe-Syndrom.

Respiratory disorders are common and important complications in acromegaly. The prevalence of sleep apnea syndrome (SAS) in this group of patients is high (20%-50%). Consequences of SAS are serious and associated with increased morbidity and mortality, mainly as a result of cardiovascular complications. The symptoms of sleep apnea are often reversible with treatment. We report on an acromegaly patient presenting with excessive snoring and severe headaches caused by sleep apnea.

Synovial sarcomas usually metastasize after >5 years: a multicenter retrospective analysis with minimum follow-up of 10 years for survivors.

BACKGROUND: Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years that evaluate long-term outcomes for survivors. PATIENTS AND METHODS: Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. Mean follow-up of living patients was 17.2 years and of dead patients 7.7 years. RESULTS: Mean age at diagnosis was 35.4 years (range 6-82 years). Overall survival was 38.7%. The 5-year survival was 74.2%; 10-year survival was 61.2%; and 15-year survival was 46.5%. Fifteen patients (24%) died of disease after 10 years of follow-up. Local recurrence occurred after a mean of 3.6 years (range 0.5-14.9 years) and metastases at a mean of 5.7 years (range 0.5-16.3 years). Only four patients were treated technically correctly with a planned biopsy followed by a wide resection or amputation. Factors associated with significantly worse prognosis included larger tumor size, metastases at the time of diagnosis, high-grade histology, trunk-related disease, and lack of wide resection as primary surgical treatment. CONCLUSIONS: In SS, metastases develop late with high mortality. Patients with SS should be followed for >10 years.

Late solitary bone metastasis of a primary pulmonary synovial sarcoma with SYT-SSX1 translocation type: case report with a long follow-up.

Primary synovial sarcoma outside its classical presentation in para-articular soft tissue of young patients is rare but regularly reported. One of the rarest primary locations is the lung. We describe a 73-year-old female patient who presented with a solitary malignant bone tumor 8 years after the resection of a lung neoplasm. The bone tumor was classified as an osteosarcoma and the lung tumor as an atypical carcinoid tumor at their first respective diagnostic work-ups. The resection of the affected humerus with allograft and endoprosthesis implantation followed. Reevaluation of the tumor samples at the time of the local recurrence of the bone tumor 6 years following the initial symptoms of the bone tumor lead to the reclassification of both specimens as synovial sarcomas. Both neoplasms contained the SYT-SSX1 type of the diagnostic translocation t(X;18) as detected by the reverse-transcription polymerase chain reaction analysis. The patient died 14 years after the resection of the primary synovial sarcoma of the lung and 6 years following the occurrence of the bone metastasis. This prolonged clinical course is uncommon for the SYT-SSX1 translocation, which, in other locations, is usually associated with an unfavorable prognosis.

High resolution susceptibility weighted MR-imaging of brain tumors during the application of a gaseous agent.

PURPOSE: To employ a high resolution blood oxygenation level dependent (BOLD) method called susceptibility weighted imaging (SWI) together with the breathing of carbogen to investigate the response of cerebral tumors to this breathing gas and to assess tumor anatomy at high resolution. METHODS: Five patients with cerebral tumors (four glioblastoma multiforme, one astrocytoma [WHO grade II]) were studied using a susceptibility weighted 3D gradient echo, first order velocity compensated sequence (TE = 45 ms, TR = 67 ms, alpha = 25 degrees , FOV = 256 x 192 x 64 mm(3), typical matrix = 512 x 192 x 64), on a 1.5 T MR scanner while they were breathing air and carbogen. Signal changes between the two breathing conditions were investigated. RESULTS: The glioblastomas showed strong but heterogeneous signal changes between carbogen and air breathing, with changes between + 22.4 +/- 4.9 % at the perimeter of the tumors and - 5.0 +/- 0.4 % in peritumoral areas that appeared hyperintense on T (2)-weighted images. The astrocytoma displayed a signal decrease during carbogen breathing (- 4.1 +/- 0.1 % to - 6.8 +/- 0.3 % in peritumoral areas that correspond to hyperintense regions on T (2)-weighted images, and - 3.1 +/- 0.1 % in the tumor-center). CONCLUSIONS: SWI provides high resolution images of cerebral anatomy and venous vascularization. Combined with hypercapnia it allows for regional assessment of tumor activity.

Residual lesions of cerebral fat embolism.

Necropsy findings of a case which survived for three months after massive cerebral fat embolism are presented. The lesions are characterized by numerous patchy necroses and areas of demyelination in the white matter of cerebral and cerebellar hemispheres and brain stem, accompanied by atrophy of white matter and ventricular enlargement. Attention is drawn to the significance of these lesions for posttraumatic coma or dementia.

Clinical experience with preoperative chemotherapy for osteosarcoma.

Since 1978 preoperative chemotherapy has been administered to 15 consecutive patients with osteosarcomas in Zurich. Preoperative chemotherapy was acceptably well tolerated and did not impair surgical procedures. Our retrospective analysis confirmed that the extent of necrosis after preoperative chemotherapy is of biological importance for the further course of the disease. Patients with extensive necrosis had better relapse-free survival and longer overall survival than those with little necrosis.

Acetylcholinesterase in lumbar and ventricular cerebrospinal fluid.

BACKGROUND: Soluble acetylcholinesterase (AChE, E.C. 3.1.1.7.) is released by neurons, glial and meningeal cells into the CSF. AChE activity in cerebrospinal fluid (CSF) is altered in various disorders of the nervous system. The objects of this study are to define a reference range for CSF AChE activity in human lumbar CSF, to prove that the enzyme activity does not depend on the blood/CSF barrier function, and to provide information about AChE in ventricular CSF. In addition, drugs used in neurosurgical care have been examined for their in vitro effects on CSF AChE activity to exclude interference with the test system. METHODS: We tested the AChE activity in 64 lumbar CSF samples collected from a clinically healthy population and in 169 ventricular CSF samples obtained from 90 neurosurgical patients. AChE activity was assayed with our inhibitor-free test procedure. RESULTS: The reference range determined for lumbar CSF AChE activity is 9.2-24.4 nmol/min per ml. Lumbar CSF AChE activity does not correlate with parameters characterising the status of the blood/CSF barrier. Ventricular puncture is only justified for underlying pathology making it impossible to provide reference data for ventricular CSF. Most measurements reveal ventricular enzyme activity below 4 nmol/min per ml. CONCLUSION: The results of this study suggest the utility of lumbar CSF AChE activity as a measure of specific secretory function in enzyme releasing cells of the nervous system.

Detailed analysis of intraoperative changes monitoring brain stem acoustic evoked potentials.

A series of 31 neurosurgical procedures in the posterior fossa monitored intraoperatively with ipsilateral brain stem acoustic evoked potentials (BAEPs) is analyzed for intraoperative potential changes. The evaluation of patients included pre- and postoperative BAEP recordings and pure tone audiometry. The series included 25 tumors, 4 neurovascular decompressions, 1 basilar artery aneurysm, and 1 arteriovenous malformation. Two intraoperative findings correlated significantly with a postoperative decrease in hearing: an amplitude reduction of more than 50% for Waves I to V and the loss of one of the waves, even if it was a wave that first appeared intraoperatively. We could attribute no significance to reversible or irreversible latency increases for all waves. The transient loss of one of the peaks followed by its reappearance was also insignificant with regard to postoperative hearing. A good prognostic sign was the intraoperative appearance of a peak undetectable on the preoperative recordings. These findings suggest that in intraoperative BAEP monitoring the observation of amplitude reduction is more important than that of latency increases. The surgeon should be informed when an amplitude reduction of more than 50% occurs before the peak is lost totally, as it is impossible to predict whether this peak will reappear intraoperatively.

Primary intracranial germ-cell tumors. A clinicopathological study of 14 cases.

Fourteen cases of primary intracranial germ-cell tumors are presented. Histologically, there were eight germinomas, three teratomas, and three germ-cell tumors of more than one histological type. Immunohistochemical studies revealed alpha-fetoprotein in yolk-sac tumor components in two cases and beta human choriogonadotropin in syncytiotrophoblastic giant cells in one case. One teratoma contained an unusual pleomorphic sarcomatous portion with features of early myoblastic differentiation. Comparison of intracranial with gonadal germ-cell tumors shows that the same subtypes are found in both locations with comparable incidence and similar biological behavior. The detailed World Health Organization classification of testicular germ-cell tumors should be applied to the histopathological classification of intracranial germ-cell tumors. Despite the critical location of intracranial germ-cell tumors, a good outcome can be achieved by optimal surgical excision. A primary microsurgical approach provides a histopathological diagnosis, which is indispensable for the proper choice of postoperative management.

Imaging the long head of biceps tendon--a pictorial essay emphasizing magnetic resonance.

Lesions of the long head of the bicepstendon (BT) are seen in association with tears of the rotator-cuff, particularly lesions of the subscapularis tendon and the rotator-interval. The frequency of positive MR-findings at the BT is approximately 25%. The pathologic alterations include complete medial luxation, subluxation and entrapment by the subscapularis tendon, tendinitis or tendovaginitis and lesions at the origin of the tendon at the superior labrum (superior labrum anterior to posterior (SLAP)--lesions). The imaging signs of BT pathology on MR include an abnormal course and position of the tendon, alterations in shape and changes in signal-intensity (SI), obliteration and thickening of the tendon-sheath. In long standing intra-articular BT rupture, neo-insertion of the tendon in the bicipital sulcus may ensue. The long BT needs to be visualized in transaxial, coronal oblique and sagittal oblique projections. Besides serving as stabilizer of the long head of biceps muscle, the BT is also an important stabilizer of the anterior joint capsule.

The use of CD31 and collagen IV as vascular markers. A study of 56 vascular lesions.

A monoclonal antibody against the endothelial cell adhesion molecule CD31 (endo-CAM, PECAM-1) was tested on wax embedded tissue of 56 cases of vascular lesions including benign and malignant vascular neoplasms. Additional preparations were stained with antibodies to type IV collagen and to von Willebrand factor/Factor VIII-RAG (vWf). Our results prove reliability of CD31 and its superiority to vWf in the labelling of endothelium and its subsets in such lesions, with the exception of lymphangiomas. As markers for basal lamina collagen and endothelium respectively, type IV collagen and CD31 together provide a powerful tool for 1) the diagnosis of endothelial neoplasms and 2) the definition of endothelial, mural and pericytic or perivascular tissue compartments in vascular lesions of complex architecture. Hence, we use CD31 and type IV collagen in cases of presumed vascular neoplasms, adding other markers to the panel in accordance with the differential diagnosis, as well as in the recognition of compromised endothelia, such as in vascular invasion by various malignant neoplasms.

Anatomy of the pelvic floor for translevatoric-transsphincteric operations.

The parasacral transsphincteric-translevatoric approach has proved of great interest in surgery of the lower rectum and urogenital organs. Due to this experience, anatomic specimens of the pelvic floor and pelvic organs were prepared in order to perfect the operative technique. The anatomic specimens show the pelvic floor and sphincter muscles and the blood supply by the pudendal vessels and nerve, as well as the topography of pelvic organs and fascias from the view of a surgeon performing parasacral surgery. The clinical experience with 118 cases of parasacral translevatoric-transsphincteric interventions at the Departments of Surgery of the University Hospital, Basel, and the Canton Hospital, Luzern, Switzerland, is presented. The results were mainly good. The operation technique is illustrated with selected cases of rectum resection, procedures for treatment of urethrorectal fistulas, reconstruction of pelvic floor for malformations, and reconstruction of injured urethra.

[Differentiating between tumor and implant material in the postoperative sella using MRT]. / Differenzierung zwischen Tumor und Implantatmaterial in der postoperativen Sella mit der MRT.

MRT criteria have been developed to distinguish between tumour and implant material following examination of 50 patients who had transsphenoidal hypophysectomies for tumours. Judgements were based on the postoperative hormonal status and the operation notes. Following contrast injection of Gd-DTPA and using T1 weighted spin-echo sequences, implant material appeared as sandwich-like, linear or circular structures. Residual recurrent tumour produced homogenous or non-homogenous aspects without marginal enhancement in 84% of cases. Postoperative displacement of the infundibulum to the opposite side was observed in 73% of patients with tumour remnants. Sensitivity of MRT was 70%, specificity 95%. There was a positive predictive value of 94% and a negative predictive value of 72% with an accuracy of 81%. This provides assistance in differentiating between tumour remnants and implant material. MRT is recommended as a method of examination for hypophyseal tumours to evaluate the success of surgery and where there is clinical doubt concerning residual or recurrent tumour.

[The value of MR tomography after transsphenoidal hypophyseal resection. A retrospective comparison between endocrinology, operation and MRT]. / Wertigkeit der MR-Tomographie nach transsphenoidaler Hypophysenresektion. Retrospektiver Vergleich zwischen Endokrinologie, Operation und MRT.

Postoperative magnetic resonance images of 40 patients after transsphenoidal surgery for pituitary adenoma were evaluated retrospectively. The signal intensities of packing material and residual tumour were analysed. T1-weighted spin-echo sequences were obtained in coronal and sagittal views with and without intravenous injection of contrast media. Residual tumour demonstrated homogeneous or inhomogeneous signal intensity on the native scan with homogeneous or inhomogeneous enhancement. Packing material demonstrated a homogeneous signal intensity on the images obtained without injection of contrast media with a peripheral enhancement or alternate layers of low and intermediate signal intensity with alternate enhancement after injection of contrast media. In 5 of 54 examinations different results were found in MRI, endocrinology and surgery (two false negative and one false positive MRI in correlation with endocrinology; two residual tumours on MRI where surgery was supposed to be complete). Application of intravenous contrast media facilitates the interpretation of postoperative examination of the pituitary gland as well as comparison with preoperative examinations and the knowledge of the intraoperative procedure.

Reduced vitamin A tolerance in a hyperlipidaemia patient with rapid destructive and hyperostotic osteoarthritis of the hip.

A 59-year-old patient suffering from hyperlipidaemia developed a chronic vitamin A intoxication syndrome after ingestion of 30000 IE retinol/daily over a period of six years. Functional disability of the right hip was caused by radiologically documented hyperostosis of the acetabular circumference. Finally, a prosthesis had to be implanted because of rapid destructive osteoarthritis of the right hip. Implications of vitamin A for rheumatological management are discussed.

Diagnostic accuracy of TMJ arthroscopy in correlation to histological findings.

From 1987-1993, 356 arthroscopic examinations of the temporomandibular joint were performed on 295 patients. During 69 examinations, biopsies were obtained to correlate arthroscopic findings with histology. In the overall group, histology confirmed in 78.9% the arthroscopic findings. Correlation was better in joints with degenerative changes (81.5%) than in those with a synovitic/hyperaemic appearance (61.5%). Chondroid metaplasia, detritus synovitis and synovial chondromatosis were additional diagnoses given by histological examination. In 11 joints, open arthrotomy was performed after arthroscopy with biopsy was carried out. The excised tissue was also investigated microscopically and correlates to the biopsy-result. Because no different pathological changes were found, it is concluded that biopsies performed during arthroscopy of the temporomandibular joint are representative for histological investigation. An additional perforation for introducing the biopsy forceps is not necessary, because results obtained with different techniques appeared to be equally accurate.

Loose bodies in the temporomandibular joint. The advantages of arthroscopy.

Loose bodies are a rare cause of temporomandibular joint symptoms. Their main source is synovial chondromatosis. We report on clinical findings, diagnostic methods, treatment choices and outcome following the removal of loose bodies in 10 patients. Seven patients were evaluated and treated by means of arthroscopy, while in three patients open arthrotomy was performed. In five patients, no diagnostic imaging technique had demonstrated the presence of loose bodies prior to arthroscopy. In six patients, histology revealed synovial chondromatosis. In four patients, osteochondral fragments alone were found. Until now, the recommended treatment of choice for the removal of all loose bodies and of affected synovial tissue required open arthrotomy. We conclude that the advantages of arthroscopy consist in locating loose bodies that are not detectable radiologically and in reducing operative trauma.

[Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation and glandular component]. / Tumeur maligne de la gaine des nerfs périphériques à différenciation rhabdomyoblastique et à composante glandulaire.

An exceptional case of malignant peripheral nerve sheath tumor with striated muscle differentiation and glandular component is reported, in a 52-year old man. This tumor measured 8 cm in diameter, and was localized in the chest wall, infiltrating the skeletal muscle. The mesenchymal portion of the tumor was composed mostly of spindle cells arranged in interlacing fascicles. Between these fascicles, there were large cells with abundant eosinophilic cytoplasm and clear elongated nucleus. Immunohistochemical study demonstrated cytokeratin, EMA and CEA expression in the glandular component and S100 protein expression in the major portion of the mesenchymal component. The large cells identified as rhabdomyoblasts, expressed desmin, myoglobin, alpha-SR actin and alpha-SM actin. The tumor recurred 5 years after its resection. Histological and immunohistochemical features were identical. We think that positivity of neoplastic striated muscle cells with alpha-SM actin reflects an early differentiation phase of these cells.

Hyperostotic and destructive osteoarthritis in a patient with vitamin A intoxication syndrome: a case report.

The authors present a case report of a 59-year-old female suffering from hyperlipidemia who developed chronic vitamin A intoxication syndrome after ingestion of 30,000 IU retinol/daily over a period of six years. The patient's main complaints included severe headaches, morning nausea, myalgias and disability around the hip, knee, and ankle joints. Radiologically, hyperostosis of the acetabular circumference and the spine was demonstrated. Because of rapidly increasing pain, total hip replacement was performed. Histology of cross sections from the femoral head revealed destructive osteoarthritis. Since no other causative reason was found, retinol may not only be responsible for hyperostotic bone and soft tissue formations but may perhaps also account for rapid progressing of degenerative joint disease. Despite the cessation of vitamin A intake the clinical symptoms persisted due to hyperlipidemia. The enlarged number of chylomicrons and the higher fraction of very low density lipoproteins may represent a second retinyl ester pool in case of overloaded fat storing Ito-cells in the liver. Therefore, rheumatological treatment reducing risk factors such as hyperlipidemia is mandatory.