Paraganglioma of the cauda equina region

BACKGROUND CONTEXT: Cauda equina paragangliomas (CEPs) are rare neuroendocrine tumors. The difficulty in differential diagnosis with other tumors of this region may be misleading for surgical planning and prognostic expectations. PURPOSE: To report on a rare case of CEP and review the most current information regarding the diagnosis, treatment options, and outcomes. STUDY DESIGN: Case report and literature review. PATIENT SAMPLE: One patient affected by CEP. METHODS: We report on a 33-year-old woman with a 2-month history of worsening low back pain, aggravated by sitting, bending, and coughing. Neurological examination revealed normal power and muscular tone, no sensory or sphincter abnormality, and normal reflex. Magnetic resonance imaging of the lumbar spine demonstrated an intradural extramedullary lesion at L3, with homogeneous contrast enhancement and hypointense punctate foci. The patient underwent an L3 laminectomy and tumor removal. Relevant articles covering CEPs from 1970 to the present were reviewed. RESULTS: The histopathological examinations described paraganglioma features. The postoperative coursewas uneventful, and all the symptoms resolved, with no tumor recurrence after 3 years’ follow-up. CONCLUSIONS: Cauda equina paragangliomas are rare, benign, and slow-growing tumors. Except for its secreting tumor characteristics, preoperative CEP diagnosis is very difficult.Magnetic resonance imaging is important andmay suggest specific radiological features for these tumors; however, these are only relative, and it is rare that diagnosis ismade before surgery.Diagnosis is established by histological examination and electron microscopy, and immunohistochemical techniques must be used to achieve a correct diagnosis. Cauda equina paragangliomas arewell-encapsulated tumors that may be cured by surgery alone, whereas radiotherapy is reserved for incompletely resected tumors. Overall, prolonged postoperative observation is mandatory because of the slow tumor evolution and the possibility of tumor relapse even up to 30 years after surgery

Endocrinologic, neurologic, and visual morbidity after treatment for craniopharyngioma

Craniopharyngiomas are locally aggressive tumors which typically are focused in the sellar and suprasellar region near a number of critical neural and vascular structures mediating endocrinologic, behavioral, and visual functions. The present study aims to summarize and compare the published literature regarding morbidity resulting from treatment of craniopharyngioma. We performed a comprehensive search of the published English language literature to identify studies publishing outcome data of patients undergoing surgery for craniopharyngioma. Comparisons of the rates of endocrine, vascular, neurological, and visual complications were performed using Pearson’s chi-squared test, and covariates of interest were fitted into a multivariate logistic regression model. In our data set, 540 patients underwent surgical resection of their tumor. 138 patients received biopsy alone followed by some form of radiotherapy. Mean overall follow-up for all patients in these studies was 54 ± 1.8 months. The overall rate of new endocrinopathy for all patients undergoing surgical resection of their mass was 37% (95% CI = 33–41). Patients receiving GTR had over 2.5 times the rate of developing at least one endocrinopathy compared to patients receiving STR alone or STR + XRT (52 vs. 19 vs. 20%, χ2P < 0.00001). On multivariate analysis, GTR conferred a significant increase in the risk of endocrinopathy compared to STR + XRT (OR = 3.45, 95% CI = 2.05–5.81, P < 0.00001), after controlling for study size and the presence of significant hypothalamic involvement. There was a statistical trend towards worse visual outcomes in patients receiving XRT after STR compared to GTR or STR alone (GTR = 3.5% vs. STR 2.1% vs. STR + XRT 6.4%, P = 0.11). Given the difficulty in obtaining class 1 data regarding the treatment of this tumor, this study can serve as an estimate of expected outcomes for these patients, and guide decision making until these data are available

Supratentorial embryonal tumors in the elderly: Diagnostic pitfalls and clinical prognosis

Supratentorial embryonal tumors are very rare malignant tumors of neuroectodermal origin, characterized by an aggressive clinical behavior. They occur prevalently in children. They have been sporadically described in adults and represent an even rarer occurrence in elderly patients, raising many issues on the diagnostic pitfalls and their appropriate management. We present an unusual case of embryonal tumor in a 62-year-old man who presented with speech disorder, and partial deficit of the left 3rd and 5th cranial nerves secondary to a left temporo-insular embryonal tumor: the clinico-radiological features, histopathological insights, therapeutic options and results are discussed along with a review of the most relevant literature, addressing the specific issue of differential diagnosis and the expected results in the elderly population

Abrupt foramen magnum syndrome due to shunt malfunction in a previously asymptomatic chiari I malformation: the hidden predictable risk in long-lasting shunted patients

Cerebrospinal fluid overdrainage in long-lasting shunted hydrocephalus is a well recognized complication. It may lead to different clinical syndromes which may occur over time, namely cranioencephalic disproportion and hindbrain herniation. Among the latter, Chiari malformation type 1 classically refers to one of the mildest form. When symptomatic, the patients generally exhibit a chronic, slowly progressive disease course. Although well documented in Chiari type II malformation and hydrocephalus, ventriculo-peritoneal shunt malfunction causing acute neurological deterioration in acquired Chiari malformation type 1 has been described only in three cases. All these patients underwent urgent shunt revision and suboccipital decompression in two of them, achieving good clinical improvement. We report on a 20-year-old woman harboring a previously asymptomatic Chiari malformation type 1, who experienced an acute foramen magnum syndrome with ventriculo-peritoneal shunt malfunction. We performed an endoscopic third-ventriculo-cisternostomy and definite removal of the shunt, obtaining the complete resolution of symptoms and shunt independence at 2 years follow-up. The phisiopathogenetic mechanisms and surgical management are discussed

Multidisciplinary treatment of giant invasive prolactinomas in paediatric age: long-term follow-up in two children.

Abstract BACKGROUND: Invasive giant prolactinomas are rare tumours, still representing a therapeutic challenge due to their characteristics of invasiveness and variable clinical course. Giant prolactinomas in childhood are extremely rare. Only single case reports have been described in the literature. CASE PRESENTATION: We report on two children who presented a progressive visual deterioration. Magnetic resonance imaging showed extensive intra-suprasellar tumour invading the anterior skull base. The laboratory investigations detected markedly elevated prolactin levels. The patients were firstly started on dopamine agonist therapy with partial reduction in size of the tumours. The debulking of the residual lesions through a transsphenoidal approach was then performed in both patients, one of whom requiring a second surgical procedure for tumour regrowth. The complete shrinkage of the residual adenomas was achieved after the treatment with conventional radiotherapy, with a follow-up of 13 and 14 years, respectively. DISCUSSION: Multidisciplinary therapeutic approach for giant prolactinomas in paediatric patients can be an effective treatment; despite the invasiveness of these tumours, the efficacy of this combined treatment can reach a satisfactory control of the disease at long term, assuring a good quality of life as well

Cerebellopontine Angle Schwannomas: Surgery, Hypofractionated Stereotactic Radiotherapy, Combined Therapy. Strategy for Harmless Therapy

Objective: The pontocerebellar angle schwannomas represent a neurosurgical challenge because after radical removal important neurological deficits can result. Design: To evaluate the best therapy concerning control of tumoral evolution and functional outcome. Methods: A series of 13 vestibular schwannomas and 3 Gasserian ganglion schwannomas is presented. According to the size, three were defined as small (maximum extrameatal diameter 2 cm), eight tumors were classified as large (diameter 2.5 to 4 cm), and the last five tumors were defined as giant (diameter 4.5 to 6 cm). Thirteen patients underwent the retrosigmoid microsurgical approach, followed by hypofractionated stereotactic radiotherapy (HSRT) in 11 of the patients. Intraoperative brainstem-evoked potentials and monitoring of facial nerve were done. Two other tumors were submitted to HSRT alone. The last one was submitted to seriated controls. Results: Gross total removal (postoperative tumor remnant less than 1 cm of maximum diameter) was achieved in 10 patients; subtotal removal (postoperative remnant 1 to 2 cm) was performed in the other three patients. After surgery, worsening of a preoperative facial nerve paresis occurred in one patient operated on (gross total removal) for a giant tumor. After HSRT alone, temporary paucisymptomatic hydrocephalus occurred in one patient, treated by low-dose betamethasone and diuretics. Hearing function was preserved in all patients. Conclusions: Our results showed that for symptomatic schwannomas, gross total removal followed by HSRT is a valid therapeutic strategy, allowing improved symptoms and avoiding tumor recurrence with very a low rate of neurological deficit

Brain Granuloma: Rare Complication of a Retained Catheter

BACKGROUND: Ventriculoperitoneal (VP) shunt is the most frequent treatment for hydrocephalus. VP shunt malfunction is a very common problem in neurosurgical practice, often requiring shunt revision procedures. In some cases, complete removal of a VP shunt may present difficulties, leading the surgeon to leave the ventricular catheter in situ. This decision is often made to avoid possible adverse events, primarily risk of life-threatening hemorrhage. However, a nonfunctioning catheter left in the ventricular system may lead to further complications.CASE DESCRIPTION: We report the case of an adult man who presented with a tumor-like cystic lesion, with mural and nodular postcontrast enhancement, caused by a huge granuloma formed around the tip of a retained ventricular catheter.CONCLUSIONS: This occurrence is extremely rare, and this is the first reported case in the literature. Preoperative differential diagnosis was challenging and included infection, metastasis, dysembryogenetic lesions, and rare reversible porencephalic cysts. According to several physiopathogenetic theories recently reported in the literature, the granuloma may develop as the result of persistent chronic inflammatory reactions between the ventricular catheter left in situ and the brain parenchyma. Application of neuroendoscopic techniques is improving management of VP shunt revisions, allowing safe removal of catheters stuck or lost in the ventricular system. Neuroendoscopy may represent an additional option to avoid possible complications related to retained ventricular catheters