A rare case of solitary fibrous tumour of the pre-sacral space: morphological and immunohistochemical features. (49/166)

A 28-year-old woman presented with abdominal pain. Ultrasonograhic examination showed a pre-sacral mass, with complex structure and well delimitated cystss with thick walls. The resected specimen was 7.5 x 6 x 4 cm in size, well circumscribed and yellow in colour, with cysstic change containing mucoid-like material. Histologically, the lesion was composed of spindle cells with high cellularity and rich vascularization with a haemangiopericytoma-like pattern. The diagnosis of solitary fibrous tumour (SFT) was made. The differential diagnosis for SFT of the pre-sacral spaace involves haemangiopericytoma, GIST, malignant mesothelioma, synovial sarcoma, leiomyomatous tumours and granulosa cell tumour. Immunohistochemical studies revealed reactivity for CD34, CD99 and Bcl-2, but no staining for desmin, inhibin, c-kit, EMA, CK, SMA, S-100 and CD31, confirming a diagnosis of SFT. Although SFT is usually associated with a favourable prognosis, close follow-up is recommended because of the limited information on its long-term behaviour.  (+info)

Ganglioneuroma : primary tumor or maturation of a suspected neuroblastoma? (50/166)

Ganglioneuroma is a benign neurogenic tumor. These tumors are originating from neuroepithelium along sympathetic ganglia. Main localization is the mediastinum in children older than 10 years. An association with malignant neuroblastoma is rarely observed and it still remains a topic under current discussion. We describe the clinical course of a 17 year-old female patient with a large presacral mass causing amenorrhoea and weight loss. Eleven years before presentation, an incidental urine test showed an elevation of vanillylmandelic acid (VMA) and homovanillic acid (HMA) and a neuroblastoma was suspected. However, further investigations showed no tumor and the test results turned out to be normal within 1 year. Now, a malignant neurogenic tumor was again suspected, but a CT-guided biopsy revealed a benign tumor. The mass was originating from the left sacral nerve roots. A tumor resection via a dorsal approach was performed. Final histology showed a differentiated ganglioneuroma. This is, to our knowledge, the first report describing a patient with elevated VMA/HMA and suspected neuroblastoma who developed a ganglioneuroma 11 years later. The association of ganglioneuroma and neuroblastoma and the abnormal urine tests pointing toward a neuroblastoma 11 years ago remains unclear and the possible answers are discussed in our report.  (+info)

Radiofrequency sinus excision: better alternative to marsupialization technique in sacrococcygeal pilonidal sinus disease. (51/166)

BACKGROUND: A number of techniques have been described for treatment of patients with sacrococcygeal pilonidal sinus disease. The author reports his experience in the surgical management of pilonidal sinus using radiofrequency sinus excision technique. The results are compared with those obtained after excision and marsupialization procedure. MATERIALS AND METHODS: Seventeen patients were operated by radiofrequency sinus excision and 18 with excision and marsupialization. An Ellman radiofrequency device was used. The two groups were matched for age, gender and presentation symptoms. The median follow-up was two years. The patient's satisfaction on the outcome of the procedure was also evaluated. RESULTS: In the radiofrequency sinus excision group, we found on average a shorter operation time (10 vs. 36 minutes, p < 0.001), shorter hospitalization (nine vs. 30 hours, p < 0.001), significantly less postoperative pain, fewer cumulatve requests for analgesia by the patients (14 vs. 25 tablets, p < 0.001), and earlier return to work (six vs. 16 days, p < 0.001) The wounds with marsupialization did heal faster than those with sinus excision; however, the difference was not significant. At two year follow-up, one patient from each group had a recurrence. CONCLUSION: This pilot study shows that this new technique can be performed as a day care surgery. With reduced postoperative pain and early resumption to work, patient satisfaction is better in comparison to excision and marsupialization technique.  (+info)

A carcinoembryonic antigen-secreting adenocarcinoma arising in tailgut cyst: clinical implications of carcinoembryonic antigen. (52/166)

Tailgut cysts (TGCs) are rare congenital cysts that occur in the retrorectal or presacral spaces. Although most tailgut cysts have been reported as benign, there have been at least 9 cases associated with malignant change. We report herein on an unusual case of a 40-year-old woman with a carcinoembryonic antigen (CEA)-producing adenocarcinoma arising within a TGC who underwent surgical resection and local radiation therapy. Despite the complete resection, metastatic adenocarcinoma developed five months after surgery. CEA-producing adenocarcinoma from a TGC is extremely rare and only two cases, including this case, have been reported in the English medical literature. Besides CEA, the serum levels of CA 19-9 became markedly elevated in this patient. Given that the serum CEA level decreased to the normal range after complete resection of tumor and that the tumor recurrence was associated with a rebound of the CEA serum level, our case shows that serial measurements of serum CEA can be used for treatment planning and for assessing the patient's treatment response for this rare disease.  (+info)

The instant axis of rotation influences facet forces at L5/S1 during flexion/extension and lateral bending. (53/166)

Because the disc and facets work together to constrain spinal kinematics, changes in the instant axis of rotation associated with disc degeneration or disc replacement may adversely influence risk for facet overloading and arthritis. The relationships between L5/S1 segmental kinematics and facet forces are not well defined, since previous studies have separated investigations of spinal motion and facet force. The goal of this cadaveric biomechanical study was to report and correlate a measure of intervertebral kinematics (the centrode, or the path of the instant axis of rotation) and the facet forces at the L5/S1 motion segment while under a physiologic combination of compression and anterior shear loading. Twelve fresh-frozen human cadaveric L5/S1 joints (age range 50-64 years) were tested biomechanically under semi-constrained conditions by applying compression plus shear forces in several postures: neutral, and 3 degrees and 6 degrees of flexion, extension and lateral bending. The experimental boundary conditions imposed compression and shear representative of in vivo conditions during upright stance. The 3-D instantaneous axis of rotation (IAR) was calculated between two consecutive postures. The facet joint force was simultaneously measured using thin-film sensors placed between both facet surfaces. Variations of IAR location and facet force during motion were analyzed. During flexion and extension, the IAR was oriented laterally. The IAR intersection with the mid-sagittal plane moved cephalad relative to S1 endplate during flexion (P=0.010), and posterior during extension (P=0.001). The facet force did not correlate with posture (P=0.844). However, changes in the facet force between postures did correlate with IAR position: higher IAR's during flexion correlated with lower facet forces and vice versa (P=0.04). During lateral bending, the IAR was oblique relative to the main plane of motion and translated parallel to S1 endplate, toward the side of the bending. Overall, the facet force was increased on the ipsilateral side of bending (P=0.002). The IAR positions demonstrate that the L5 vertebral body primarily rotates forward during flexion (IAR close to vertebral body center) and rotates/translates backward during extension (IAR at or below the L5/S1 intervertebral disc). In lateral bending, the IAR obliquity demonstrated coupling with axial torsion due to resistance of the ipsilateral facet.  (+info)

When is Onuf's nucleus involved in multiple system atrophy? A sphincter electromyography study. (54/166)

BACKGROUND: External anal sphincter (EAS) electromyography (EMG) abnormalities can distinguish multiple system atrophy (MSA) from Parkinson's disease in the first five years after disease onset. However, the prevalence of the abnormalities in the early stages of MSA is unknown. OBJECTIVES: To present EAS-EMG data in the various stages of MSA. METHODS: 84 patients with "probable" MSA were recruited (42 men, 42 women; mean age 62 years (range 47 to 78); mean disease duration 3.2 years (0.5 to 8.0; <1 year in 25%); 50 cerebellar form (MSA-C), 34 parkinsonian form (MSA-P)). EAS motor unit potential (MUP) analysis and EMG cystometry were carried out in all patients. RESULTS: The overall prevalence of neurogenic change of the EAS MUP was 62%-52% in the first year after disease onset, increasing to 83% by the fifth year (p<0.05); it also increased with severity of gait disturbance (p<0.05), storage and voiding disorders, and detrusor sphincter dyssynergy (NS). The neurogenic change was not correlated with sex, age, MSA-P/C, postural hypotension, constipation, erectile dysfunction in men, underactive or acontractile detrusor, or detrusor overactivity. In 17 incontinent patients without detrusor overactivity or low compliance, urinary incontinence was more severe in those with neurogenic change than in those without (p<0.05). CONCLUSIONS: Involvement of Onuf's nucleus in MSA is time dependent. Before the fifth year of illness, the prevalence of neurogenic change does not seem to be high, so a negative result cannot exclude the diagnosis of MSA.  (+info)

Sacral neuromodulation in the treatment of severe anal incontinence. Forty consecutive cases treated in one institution. (55/166)

INTRODUCTION: Sacral neuromodulation is a recognized therapeutic option in severe anal incontinence from neurogenic origins, when medical treatment has failed. METHODS: We report the results of this procedure applied in 40 consecutive patients operated on by a single surgeon from August 2001 to June 2004. Mean duration of incontinence was 5 years. There were 33 women and 7 men of mean age 59 (range 29-89). All patients had had medical treatment, 26 had had physiotherapy and 9 had been previously operated on for that problem. Neuromodulation consisted in a temporary electrical stimulation test followed by implantation of a stimulator in case of efficacy. RESULTS: Twenty nine patients had a positive test and were implanted. Ten had a negative test and one is waiting for implantation. From the 29 patients, 23 had uneventful postoperative course. Incontinence score varied from 17 before neuromodulation to 6 after in the 24 patients who were improved. Mean resting pressure, mean maximum squeeze pressure and mean duration of squeeze pressure did not change from pre to postoperative period. CONCLUSION: Sacral neuromodulation is a safe and efficacious procedure in properly selected anal incontinent patients. However, we observed no correlation between clinical and manometric data.  (+info)

Spontaneous rupture of sacrococcygeal teratoma associated with acute fetal anemia. (56/166)

Sacrococcygeal teratoma (SCT) is the most common congenital tumor, with affected fetuses having a high risk of perinatal complications and death. We report a case of a fetus with an SCT that developed acute anemia due to spontaneous rupture of the tumor in utero. The fetus was referred at 25 weeks' gestation for evaluation of a large solid and cystic mass in the sacral region. There were no signs of hydrops or placentomegaly. At 33 weeks' gestation, loss of variability in the fetal heart rate pattern was recorded. Doppler ultrasonography showed increased middle cerebral artery peak systolic velocity, suggesting fetal anemia. Markedly bloody amniotic fluid, with 82% hemoglobin F, was observed on amniocentesis, suggesting rupture of the SCT with active fetal bleeding. An emergency Cesarean section was performed. At delivery, the tumor was bleeding actively and the neonatal hemoglobin concentration was 3.1 g/dL. There were no findings of hemorrhage or necrosis within the tumor. The neonate received a blood transfusion, and surgical resection of the tumor was carried out on the first day after delivery. Postoperatively, the baby did well. We suggest that fetal SCTs run the risk of inducing acute fetal anemia due to intrauterine hemorrhage of the tumor, a finding which could lead to an earlier and more appropriate management of this condition.  (+info)