RESUMEN

BACKGROUND: Malignant melanotic nerve sheath tumors (MMNSTs) are rare tumors of presumed neural crest origin. Here, we present a 21-year-old female with a left L5/S1 MMNST along with a review of approximately 70 spinal cases reported in the literature, the majority of which were either local recurrences or metastases. CASE DESCRIPTION: A 21-year-old female presented with 3 months of severe left L5 distribution radicular leg pain and sensory loss. The MR revealed a dumbbell-shaped, heterogenously enhancing lesion centered on the left L5/S1 foramen; the intracanalicular component displaced the thecal sac to the right, while the extraforaminal portion of tumor extended anteriorly into the retroperitoneal space. Gross total resection was performed after a L5/S1 facetectomy. In the immediate postoperative period there were no complications, and the patient had full lower limb power. Four months later, the patient experienced generalized seizures, headache, and multiple cranial nerve palsies due to local and diffuse CNS dissemination. The MRI of the brain and whole spine revealed diffuse leptomeningeal enhancement along the full length of the spinal cord into the brainstem and cerebrum along with a focally recurrent epidural soft-tissue lesion located posterolaterally on the left at the L4/5 level (i.e., measuring 12 mm × 10 mm). An external ventricular drain and subsequent ventriculoperitoneal shunt were inserted, followed by craniospinal irradiation. She was discharged 3 months later with residual distal lower limb weakness. CONCLUSION: This case illustrates the rapid disease progression of MMNST despite gross total resection. Further such lesions should be aggressively treated locally, and followed by adjuvant radiotherapy and systemic chemotherapy/immunotherapy.

RESUMEN

BACKGROUND: Intracranial Ewing's sarcoma (ES) is a rare entity with <15 cases reported in the literature. It belongs to a family of round-cell neuroectodermally derived tumors bearing many similarities to peripheral primitive neuroectodermal tumor (pPNET). There is currently no established treatment protocol. Reported cases are treated with either surgery alone or surgery with adjuvant chemotherapy and radiation. CASE DESCRIPTION: We describe a case of intracranial left frontal ES in a 19-year-old patient who presented with change in behavior. Diagnosis was unclear based on radiological findings on MRI and CT alone. MRI brain with contrast demonstrated a large extra-axial ovoid heterogeneously enhancing left frontal convexity mass. The patient underwent gross total resection with adjuvant chemotherapy and radiation. No local or systemic recurrence was found at 12 months postoperatively. CONCLUSION: Intracranial ES/pPNET is rare tumor with nonspecific clinical presentation and radiological findings. They are locally invasive. Surgery with adjuvant chemoradiation is the mainstay treatment. Distinction of pPNET and cPNET is important for therapeutic and prognostic purposes.

RESUMEN

BACKGROUND: A bacterial cause of disc degeneration has evoked several controversies and, if true, would lead to a major shift in treatment paradigm. Earlier studies analyzing the relationship of bacterial disc infection within a degenerative cohort featured prolonged cultures susceptible to contamination. The degenerate-disc infection study with contaminant control (DISC) trial aims to investigate this theory further by examining infection rates using a non-degenerative control cohort in comparison to a degenerative internal control cohort and a sham cohort (sampling only sterile paraspinal tissue). To our knowledge, the current study is the largest evaluating the growth of organisms (or possible contamination rate) in paraspinal tissue if prolonged cultures are performed. Protocols on methodology have been previously published. PURPOSE: (1) To investigate the infection rates across cohorts (degenerative vs. nondegenerative control; paraspinal and/or disc controls vs. combined sampling cohorts) using stringent standardized aseptic surgical technique and laboratory processing. (2) To compare our findings to that of the literature and make a statement in support and/or against a possible contamination theory to positive cultures. STUDY DESIGN: Multicenter, multisurgeon case-control trial. PATIENT SAMPLE: In all, 812 surgical samples were retrieved across a 3.5-year period (2013-2016) including 25 trauma controls (nondegenerative), 550 "disc and paraspinal" samples (degenerative cases with internal control), 190 disc-only samples (degenerative cases without internal control), and 46 paraspinal only controls (sham group). OUTCOME MEASURES: Growth and/or Contamination rate (%) per cohort. Chi-square of growth in disc versus paraspinal samples as a means of examining the distribution of false positive and contaminant growth. The impact of previous injections and/or surgery on positive disc or paraspinal growth. Correlation of Modic changes with positive growth rates analyzed with the Kruskal-Wallis Test. The distribution of species in positive samples were also analyzed. METHODS: The DISC trial is registered under Australian and New Zealand clinical trials registry-ACTRN12616000541404. Institutional ethics review was obtained (HREC northern sector 13/218) at the primary center and further centers (n=6) were recruited. Patients undergoing spinal surgery with discectomy were eligible for trial entry with tissue specimens obtained using strict aseptic technique for microbiological examination. All specimens were handled with sterile instruments only and by a fresh instrument to a sterile pot that was closed immediately. Separate pots were used for the disc and paraspinal tissue respectively with similar stringent processing during microbiological assessment. A cohort of the degenerative cases at one single institution also underwent an additional histopathological examination. RESULTS: There was an expected significant difference in gender and age associated with the non-degenerative control group (due to trauma patients) compared with other cohorts. There was a higher percentage of positive-growth in the control group in comparison to the disc and paraspinal and disc only groups across positive disc growth (48% vs. 27% vs. 17%, p<.001). A similar infection rate was observed in the paraspinal samples across the equivalent controls (44% vs. 36% vs. 37%, p=.739). There was a significant difference in the proportions of positive growth with a large proportion of false positives (growth in both disc and paraspinal samples; p<.001). There was no difference in true positive growth between the case and control groups (16.0 vs. 7.7%, p=.112). These trends were preserved across all cohorts and when stratifying by spinal segment (cervical or lumbar). There was no correlation between Modic changes and positive disc culture growth (p=.398, n=144 samples). Cutibacterium (formerly Propionibacterium) acnes was the most dominant pathogen isolated, representing between 50% and 70% of positive disc and paraspinal specimens, followed by staphylococcal species. CONCLUSIONS: Our study failed to find a difference in true infection rates between the nondegenerative and degenerative disc populations. These findings are suggestive of a contamination theory and against a common infective etiology in the setting of discogenic back and neck pain. We believe the rationale for antibiotic therapy in the management of discogenic back pain warrants further evidence to establish efficacy.

Asunto(s)

RESUMEN

BACKGROUND: Multiple choroid plexus papillomas (CPPs) are rare. Usually, they correspond to villous hypertrophy or metastasis occurring during cerebrospinal dissemination. Multiple CPPs have rarely been reported as synchronous tumors. CASE REPORT: Three synchronous CPPs were resected in a 59-year-old female 6 years after their first imaging description. Pathology showed mucus-producing CPP in all 3, 1 of the 3 presenting some signs of atypia. No p53 or hSNF5/INI1 mutation, or signs of polyoma viruses infection were found. CONCLUSION: Although no clear cause for the multifocality was found, the simultaneous presence of the three tumors and their benign histology suggest that they were synchronous and not metastatic. The issue of differentiating synchronous CPPs from metastatic CPP is discussed.

Asunto(s)

RESUMEN

We describe the complete removal of a foramen magnum solitary fibrous tumour in a 36-year-old woman. It originated on a caudal vagus nerve rootlet, classically described as the 'cranial' accessory nerve root. This ninth case of immunohistologically confirmed cranial or spinal nerve SFT is the first of the vagus nerve.

Asunto(s)

RESUMEN

OBJECTIVE: We present a case of brachial plexus avulsion and reconstructive surgery with cerebrospinal fluid leak between the cervical subarachnoid space and the pleural cavity responsible for tonsillar herniation and syringomyelia. CLINICAL PRESENTATION: A 17-year-old man presented with headaches when he was positioned upright, simultaneously with a persistent right pleural effusion for about 4 months after reconstructive surgery for a right brachial plexus avulsion. In addition, the headaches had worsened considerably after two aspirations of the pleural effusion. Magnetic resonance imaging (MRI) demonstrated signs of chronic intracranial hypotension and tonsillar herniation with a presyrinx cavity from vertebral level C1 to C7. None of those abnormalities were seen on the MRI scan obtained a few days after the initial trauma 7 months previously. Plexus brachial MRI confirmed the presence of a cerebrospinal fluid leak between the avulsed root of C8 and the pulmonary apex. INTERVENTION: The leak was treated by surgical closure of the dural tear of the C8 root. Postoperatively, the patient's headaches immediately resolved, and MRI 4 months later showed resolution of cerebellar tonsil herniation and regression of the syrinx. CONCLUSION: Resolution of acquired tonsillar herniation and syringomyelia can be achieved by closure of the dural tear responsible of the cerebrospinal fluid leak.

Asunto(s)

RESUMEN

OBJECTIVE: The management of cerebrospinal fluid (CSF) fistulae after anterior cranial base fracture remains a surgical challenge. We reviewed our results in the repair of CSF fistulae complicating multiple anterior cranial base fractures via a combined intracranial extradural and intradural approach and describe a treatment algorithm derived from this experience. METHODS: We retrospectively reviewed the files of 209 patients with an anterior cranial base fracture complicated by a CSF fistula who were admitted between 1980 and 2003 to Liège State University Hospital. Among those patients, 109 had a persistent CSF leak or radiological signs of an unhealed dural tear. All underwent the same surgical procedure, with combined extradural and intradural closure of the dural tear. RESULTS: Of the 109 patients, 98 patients (90%) were cured after the first operation. Persistent postoperative CSF rhinorrhea occurred in 11 patients (10%), necessitating an early complementary surgery via a transsphenoidal approach (7 patients) or a second-look intracranial approach (4 patients). No postoperative neurological deterioration attributable to increasing frontocerebral edema occurred. During the mean follow-up period of 36 months, recurrence of CSF fistula was observed in five patients and required an additional surgical repair procedure. CONCLUSION: The closure of CSF fistulae after an anterior cranial base fracture via a combined intracranial extradural and intradural approach, which allows the visualization and repair of the entire anterior base, is safe and effective. It is essentially indicated for patients with extensive bone defects in the cranial base, multiple fractures of the ethmoid bone and the posterior wall of the frontal sinus, cranial nerve involvement, associated lesions necessitating surgery such as intracranial hematomas, and post-traumatic intracranial infection. Rhinorrhea caused by a precisely located small tear may be treated with endoscopy.

Asunto(s)

Asunto(s)

RESUMEN

We present a rare case of temporal bone angiosarcoma diagnosed in a 26-year-old female patient at 36 week of pregnancy. The patient was referred with a 2 months history of left otalgia and tinnitus with a tender swelling above the mastoid. Cranial imaging studies showed a 7 x 5 x 4 cm hypervascularized mass located in the left middle fossa with lysis of the temporal bone and extension to the subcutis. After the baby was delivered by caesarean section, the patient entered the oncology protocol. Selective embolization of the feeding vessels was followed by gross total surgical resection using a combined supra- and infra-tentorial approach. Pathological findings were those of a poorly differentiated, highly malignant sarcoma with a large epitheloid component and immunohistochemical evidence of endothelial differentiation (CD31, Factor VIII related antigen, CD34), consistent with an angiosarcoma with epitheloid features. No extra-cranial tumor was found after extensive staging. The patient received adjuvant radiotherapy followed by a course of chemotherapy consisting of 6 cycles of paclitaxel. At 15 months follow-up, she developed multiple distant metastasis to a left postauricular lymph node and to the lungs and ribs. The patient was given a second line chemotherapy using doxorubicine and ifosfamide. Despite an initial good response, she died with metastatic disease 26 months after diagnosis. We present a rare case of primary temporal bone angiosarcoma and report our experience with a multimode therapeutic approach combining surgery, radiotherapy and chemotherapy.

Asunto(s)