A rare case of intramedullary solitary juvenile xanthogranuloma of the lumbar spine in the adult: a case report.

Solitary juvenile xanthogranuloma (SJX) of the spine is an extremely rare proliferative histiocytic disorder with only few cases reported in literature. We present the first case of intramedullary spinal SJX. A 22-year-old male presented with a nine-month history of progressively worsening sphincteric disturbances and saddle hypoesthesia. Magnetic resonance imaging showed an intra-axial lesion located in the conus medullaris; T1 hypointense, T2 iso-hyperintense and uniformly enhancing after contrast administration. The lesion was removed through a T12-L1 laminectomy and a median myelotomy with neurophysiological monitoring. Histological examination and immunohistochemical testing confirmed the diagnosis of SJX. Due to the intramedullary localization and the absence of a clear cleavage plane, radical removal was not possible. The tumor subsequently recurred and new surgical procedures were necessary followed by adjuvant radiotherapy. Patient made good neurological recovery. Three years after the latest treatment, MRI showed no recurrence. In accordance with the literature, the treatment of choice for SJX its radical removal, or subtotal removal followed by adjuvant radiotherapy.

Pleomorphic xanthoastrocytoma: long-term results of surgical treatment and analysis of prognostic factors.

BACKGROUND: Pleomorphic Xanthoastrocytoma (PXA) is a rare brain tumour, most commonly affecting children and young adults. To date, only few data regarding the long-term follow-up of these patients after surgery are available. The aim of this study is to describe our single-institution experience in the surgical management of this particular glioma over a period of over 18 years. METHODS: We performed a retrospective review of all cases of PXA (40 patients) operated upon at the Department of Neurosurgery of Verona, Italy, between 1990 and 2008. The impact of clinical, radiological, surgical and histological factors on overall survival (OS) and progression-free survival (PFS) was analysed by means of univariate and multivariate models. FINDINGS: We achieved a gross total resection (GTR) in 65% of patients. Histological diagnosis was of grade II in 80%; anaplastic features were present in the remaining 20%. Adjuvant treatment, radiotherapy or chemo-radiotherapy, was administered in 40% of the cases. Median follow-up was 74 months. OS at 5- and 10 years was 76.32% and 68.24%, respectively. PFS at 5- and 10 years was 71% and 58%, respectively. In the multivariate model, histological grade, extent of resection and age at diagnosis (≤ 30 years vs > 30 years) were the only independent prognostic factors for both OS and PFS. CONCLUSIONS: Our retrospective long-term study confirms the relatively favourable prognosis associated with PXA. Young patients with a low-grade tumour (WHO grade II) who underwent GTR carry the longest OS and PFS.

Cerebral metastasis from a previously undiagnosed appendiceal adenocarcinoma.

Brain metastases arise in 10%-40% of all cancer patients. Up to one third of the patients do not have previous cancer history. We report a case of a 67-years-old male patient who presented with confusion, tremor, and apraxia. A brain MRI revealed an isolated right temporal lobe lesion. A thorax-abdomen-pelvis CT scan showed no primary lesion. The patient underwent a craniotomy with gross-total resection. Histopathology revealed an intestinal-type adenocarcinoma. A colonoscopy found no primary lesion, but a PET-CT scan showed elevated FDG uptake in the appendiceal nodule. A right hemicolectomy was performed, and the specimen showed a moderately differentiated mucinous appendiceal adenocarcinoma. Whole brain radiotherapy was administrated. A subsequent thorax-abdomen CT scan revealed multiple lung and hepatic metastasis. Seven months later, the patient died of disease progression. In cases of undiagnosed primary lesions, patients present in better general condition, but overall survival does not change. Eventual identification of the primary tumor does not affect survival. PET/CT might be a helpful tool in detecting lesions of the appendiceal region. To the best of our knowledge, such a case was never reported in the literature, and an appendiceal malignancy should be suspected in patients with brain metastasis from an undiagnosed primary tumor.

Conservative treatment of an atlantoaxial degenerative articular cyst: case report.

BACKGROUND: Atlantoaxial degenerative articular cysts are rare lesions that can cause extradural compression of the cervicomedullary junction. When symptomatic, they usually require surgical treatment. We report an unusual case of spontaneous regression of an atlantoaxial degenerative articular cyst after conservative treatment with an external cervical brace along with a systemic therapy with nonsteroidal anti-inflammatory drugs (NSAIDs) and steroids. We also discuss the potential pathogenetic mechanisms involved. PURPOSE: To describe a case of significant volume reduction of an atlantoaxial articular degenerative cyst in a patient treated with a Philadelphia collar and anti-inflammatory drugs. STUDY DESIGN: Case report with analysis of the literature. METHODS: A 80-year-old patient was admitted to our institution with a history of progressive tetraparesis, ataxic gait, and cervical pain. A cervical spine magnetic resonance imaging (MRI) scan showed an extradural mass lesion behind the dens of C2 causing significant compression of the cervicomedullary junction, suggesting the diagnosis of atlantoaxial degenerative articular cyst. The patient refused surgery in favour of a conservative treatment with a Philadelphia collar and a short-term course of NSAIDs and corticosteroids. RESULTS: After 6 weeks, the patient's neurological condition improved, and a 6-month follow-up cervical spine MRI scan revealed an almost complete regression of the atlantoaxial cystic lesion. At a 1-year follow-up, his clinical condition was further improved. CONCLUSIONS: Atlantoaxial articular degenerative cysts are rare lesions that should be included in the differential diagnosis of those extradural lesions that can cause a ventral or ventrolateral compression of the cervicomedullary junction. They most commonly occur in elderly female patients affected by diffuse arthrosic degeneration of the cervical spine, with or without clear radiological signs of atlantoaxial instability, and have a typical appearance on MRI imaging. Surgery, with direct excision of the cyst and/or a C1-C2 fusion, is the first treatment of choice. Nevertheless, our report points out the possibility of a significant spontaneous regression of these lesions following a simple conservative strategy based on the use of an external cervical brace together with a systemic anti-inflammatory therapy.

Intraneural cavernous malformation of the cauda equina.

Spinal cavernous angiomas are rare vascular malformations occurring mainly in the vertebral body with or without an extradural extension. Only 3-5% of these lesions are entirely located in the spinal canal where they can occupy an extradural, intradural-extramedullary or intramedullary position. We present a 75-year-old woman with signs and symptoms of multiple lumbar radiculopathy. The lumbosacral MRI showed an intradural cauda equina lesion with heterogeneous contrast enhancement that was subsequently radically removed through an L3-L4 laminectomy. The microscopic appearance was suggestive of cavernous angioma with intraneural growth. Clinical, radiological, and surgical features of this unusual lesion (to date, only 12 cases are reported) are discussed.