The Surgical Management of Symptomatic Benign Peripheral Nerve Sheath Tumors of the Neck and Extremities: An Experience of 442 Cases.

BACKGROUND: The occurrence of benign peripheral nerve sheath tumors (PNSTs) is not uncommon. The surgical excision of symptomatic benign PNSTs along with preservation of the affected nerve and its function is an ideal treatment option. OBJECTIVE: To analyze the outcome with respect to morbidity, extent of resection, and recurrence, and to review and compare our results with those reported in literature. METHODS: A retrospective review of clinical and radiological findings of 442 patients with benign PNSTs involving the neck and extremities treated surgically from 2000 to 2014 was performed. RESULTS: In our series, benign PNSTs involved the extremities in 290 (65.6%) patients and the brachial plexus in 146 (33%) patients, and 6 (1.4%) patients had tumors of the extracranial portion of the vagus and hypoglossal nerves in the neck. The mean age of patients was 38 yr. The presenting features were painful mass and paresthesia. Preoperative motor weakness in the extremity was noted in 15.6% of patients. The common nerves involved by the tumors were the ulnar nerve (15.8%), sciatic nerve (12.7%), and upper cervical roots (11.5%). The excision was total in 81.2%, gross total (>90%) in 17.9%, and subtotal (>50%) in 0.9% patients. In 17.6% of patients, there was severe postoperative neurogenic pain. In 28 (6.3%) patients, a new motor deficit was noted following surgery. Recurrence was seen in 2 patients in our series. The mean follow-up was 30.2 mo. CONCLUSION: Benign PNSTs have excellent clinical outcome, and the goal for surgical treatment is total to gross total excision of the tumor with neural preservation.

Outcome Analysis in Cases of Spinal Conus Cauda Ependymoma.

INTRODUCTION: One half of all central nervous system ependymomas, arise within the spinal canal and about 40% of these arise from filum terminale. The myxopapillary variant of spinal ependymoma almost exclusively occurs in the lumbosacral region and they are histologically designated as Grade I. Long term control is best achieved by gross total removal at the initial operation. There is as yet no consensus on the management of incompletely excised tumour. Opinions regarding radiotherapy are controversial and the indications are empirical. AIM: In the present study, we investigated the clinical characteristics and long-term outcomes in patients with conus cauda ependymoma that were managed at our center with baseline comparison of our findings with those reported in literature. MATERIALS AND METHODS: A retrospective analysis of 44 cases of conus cauda ependymoma tumours treated at the Department of Neurosurgery at a tertiary care centre from January 2001 to December 2015 was done. Detailed scrutiny and analysis of the patient's data with respect to the demographic features, clinical findings, investigative procedures, extent of surgical resection, intra and postoperative complications, efficacy of adjuvant therapy, postoperative results and long term follow-up were done. RESULTS: The analysis was done in 44 patients with conus cauda ependymoma over a period of 15 years. The mean age of presentation was 31 years. Incidence of male predominance was noted. Average duration of presenting features was 10 months. Back pain and motor weakness in the lower limbs were the commonest clinical findings. Total excision of the tumour was possible in 89% cases. Myxopapillary ependymoma was the commonest variant. Radiotherapy was only given in patients with near total to subtotal excision of tumour. Back pain and motor weakness improved in majority of patients after surgery. There is limited role of radiotherapy in cases with total tumour excision. CONCLUSION: Conus cauda ependymomas are relatively benign tumours. The long term prognosis is excellent with respect to recurrence and functional outcome in cases with complete tumour excision. Early diagnosis and surgery will prevent occurrence of permanent neurological deficits. Radiotherapy can be given in cases of subtotal excision but there is limited role of radiotherapy in cases with total tumour excision.

Primary benign brachial plexus tumors: an experience of 115 operated cases.

Primary benign brachial plexus tumors are rare. They pose a great challenge to the neurosurgeon, because the majority of patients present with minimal or no neurological deficits. Radical to complete excision of the tumor with preservation of neurological function of the involved nerve is an ideal surgical treatment option with benign primary brachial plexus tumor surgery. We present a review article of our 10-year experience with primary benign brachial plexus tumors surgically treated at King Edward Memorial Hospital and P.D. Hinduja National Hospital from 2000 to 2009. The clinical presentations, radiological features, surgical strategies, and the eventual outcome following surgery are analyzed, discussed, and compared with available series in the world literature. Various difficulties and problems faced in the management of primary benign brachial plexus tumors are analyzed. Irrespective of the tumor size, the indications for surgical intervention are also discussed. The goal of our study was to optimize the treatment of patients with benign brachial plexus tumors with minimal neurological deficits. It is of paramount importance that brachial plexus tumors be managed by a peripheral nerve surgeon with expertise and experience in this field to minimize the neurological insult following surgery.

Cranial migration of complete ventriculo-peritoneal shunt assembly.

A 10-month-old male child with severe congenital hydrocephalus due to aqueduct stenosis presented with cranial migration of the entire ventriculo-peritoneal (VP) shunt. The complete shunt assembly, including the shunt chamber, was noted in the dilated ventricles. The migrated shunt was left in situ. A VP shunt was performed on the opposite side. The complete intraventricular migration of a VP shunt is a rare complication. This complication may be avoided by firm anchoring of the connector sites to the periosteum, and avoidance of large burr holes and dural openings. The possible mechanisms of such an event and the relevant literature are discussed.

Dandy-Walker malformation associated with polycystic kidneys: Goldston syndrome revisited.

A 32-year-old woman was diagnosed with chronic bilateral frontoparietal and interhemispheric subdural haematomas (SDH). Abdominal ultrasonography revealed chronic renal failure due to bilateral large cystic kidneys. Brain MRI demonstrated Dandy-Walker malformation associated with hypoplastic vermis, in addition to the chronic subdural haematomas. This association of polycystic kidneys with Dandy-Walker malformation has been referred to as Goldston or cerebrorenal syndrome. The case is rare and is the first to be reported in an adult patient. The relevant literature is reviewed.

Hemangioblastoma of the filum terminale.

A 52-year-old man presented with low backache, paraesthesiae and spasticity of both lower limbs. He had urinary retention and constipation. Investigations revealed a vascular intradural cauda equina-conus tumor. MRI scan demonstrated an enhancing mass at the second and third lumbar vertebral levels. There were multiple dilated and tortuous veins draining from both poles of the tumor. Digital subtraction spinal angiogram showed the tumor to be supplied mainly by the radiculo-medullary artery from first lumbar artery and dural branches of the second and third lumbar arteries. At surgery, after pre-operative embolization, a well-defined tumor with an orange hue and fleshy consistency was encountered arising from the filum terminale. The tumor was excised en bloc. A sporadic hemangioblastoma arising from the filum terminale should be considered as a rare cause of back pain and sciatica. Total excision of the tumor offers cure.

Spontaneous cerebrospinal fluid rhinorrhea following excision of a massive torcular meningioma.

A massive supratentorial torcular meningioma was completely resected in a 50-year-old woman. On the tenth postoperative day she presented with spontaneous cerebrospinal fluid (CSF) rhinorrhea. The site of the CSF leak was through the region of the cribriform plate, remote from the site of surgery. The possible cause of the leak and the relationship with the massive meningioma are analyzed.

Pineal epidermoid cysts: a study of 24 cases.

BACKGROUND: A retrospective analysis of clinical features and management strategy in 24 cases of epidermoids in the pineal region is done. Long-term outcome of radical surgical resection is analyzed. METHODS: Twenty-four patients having epidermoids in the pineal region were surgically treated in our department during an 11-year period (1992 up to 2003). These cases were retrospectively analyzed. The average duration of follow-up was 5.2 years. RESULTS: The age at the time of presentation ranged from 13 to 51 years (mean, 29.2 years). There were 15 male and 9 female patients. The average duration of complaints was 7 months. Headache and ataxia were the commonest symptoms. The infratentorial-supracerebellar approach and the interhemispheric posterior parietooccipital route were used to excise the epidermoids in 20 and 3 patients, respectively. In one patient, the tumor was removed in 2 stages, first by the infratentorial-supracerebellar approach and subsequently by the interhemispheric posterior parietooccipital surgical approach. Total excision of the solid part of the tumor and the capsule was achieved in 6 patients. In 13 cases, the solid part of the tumor was completely resected, but at least, a part of the tumor capsule was left behind. In 5 cases, a part of the solid portion of the tumor was either deliberately or inadvertently left behind. Incomplete tumor resection was primarily due to adhesions of the capsule to large veins in the region, dissection around which was considered hazardous, and extensive and invasive nature of extension of the tumor. During the period of follow-up, one patient had asymptomatic growth of the residual tumor after 6 years of surgery. One patient with residual tumor continued to get generalized seizures. All patients are leading active lives. CONCLUSION: Radical surgery for pineal region epidermoid tumors is associated with an excellent immediate postoperative and long-term outcome.

Cerebellar oligodendroglioma in a young adult.

A rare case of an oligodendroglioma of the cerebellum in an 18-year-old male is presented. Radical surgical excision and craniospinal radiotherapy appear to be the treatment options for these potentially malignant lesions.

Ossified ligamentum flavum of the atlantoaxial region.

A 30-year-old male presented complaining of a six-month history of progressive weakness and paraesthesia in all four limbs. Symptoms occurred following moderately severe neck trauma. Investigations revealed ossification of the ligamentum flavum (OLF) between the atlas and axis, with marked cord compression. The patient showed remarkable neurological recovery following excision of the OLF. OLF causing cord compression is rare and has never been reported in the atlantoaxial region.

Segmental costovertebral malformation associated with lipomyelomeningocoele.

We describe 2 patients with segmental costovertebral malformation, a form of spondylocostal dysostosis, associated with tethering of the conus to a lipomyelomeningocoele. Such an association is rare. In both these patients the defects occurred sporadically. The relevant literature is reviewed.

Intracranial optic nerve germinoma.

A 11-year-old boy presented with complaints of diminution of vision in the left eye for three months. On examination, the vision in this eye was reduced to only perception of light. Magnetic resonance imaging revealed a thickened left intracranial optic nerve. The lesion was explored by a pterional craniotomy. At surgery, the left optic nerve was diffusely thickened. The biopsy of a fleshy mass along the optic nerve showed that it was a germinoma on histopathology. Isolated optic nerve germinomas are extremely rare. Exclusive primary involvement of the intracranial portion of the optic nerve by a germinoma has not been reported in the literature. This rare case is discussed and the relevant literature has been reviewed.

Tuberculomas of the hypophysis cerebri: report of five cases.

Tuberculomas involving the hypophysis cerebri are extremely rare lesions, and these may occur even in the absence of systemic tuberculosis. This report presents the clinical data of 5 patients harbouring tuberculomas of the pituitary gland. Sellar tuberculomas commonly mimic pituitary adenoma. Histological diagnosis of a sellar lesion is mandatory irrespective of the clinical presentation and radiological findings. A history of extracranial tuberculosis in the past associated with radiological findings like leptomeningeal enhancement, parenchymatous brain tuberculomas or a thickened pituitary stalk on contrast MRI, are indicative of the possibility of a sellar tuberculoma. The aim of surgery is tissue diagnosis and tumour debulking. The response to long-term antituberculous chemotherapy is excellent. There is often a complete resolution of the granuloma and a satisfactory recovery of visual and endocrinological function.

Atlantoaxial fixation using plate and screw method: a report of 160 treated patients.

OBJECTIVE: We review our experience with the use of the plate and screw method of fixation in the treatment of 160 patients with atlantoaxial instability during a 14-year period at our center. We previously described this method of fixation in 1994. METHODS: Between 1988 and 2001, 160 patients with atlantoaxial instability were treated with the use of a plate and screw method of fixation at the Department of Neurosurgery at King Edward Memorial Hospital in Bombay, India. The study group was composed of 91 males and 69 females (mean age, 23 yr; age range, 18 mo-79 yr). Atlantoaxial instability was a result of congenital abnormality in 132 patients (83%) and occurred after trauma in 28 patients (17%). All patients had mobile, completely reducible atlantoaxial dislocation. For 3 months postoperatively, a hard cervical collar was used. The mean follow-up period was 42 months (range, 4 mo-14 yr). RESULTS: Three patients died in the postoperative phase. Successful stabilization of the atlantoaxial region was documented with dynamic radiography in the other 157 patients. There was no incidence of implant rejection. In one patient, one screw was found to be broken 18 months after surgery; however, firm bony fusion was documented in this patient. There were no neurological, vascular, or infective complications. CONCLUSION: The plate and screw method of fixation with the use of intra-articular bone grafts in patients with atlantoaxial instability yielded a 100% fusion rate with a low incidence of complications.

Tuberculum sellae meningioma: a report on management on the basis of a surgical experience with 70 patients.

OBJECTIVE: The surgical strategies and outcomes for 70 patients operated on for tuberculum sellae meningioma were analyzed retrospectively. The analysis was based on factors that probably determined the difficulties encountered during surgery, assisted in predicting the extent of resectability, and helped in predicting the surgical outcome. METHODS: Seventy patients with tuberculum sellae meningiomas were operated on in the neurosurgery department at King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Bombay, India, between 1991 and May 2001. The patients were analyzed retrospectively on the basis of clinical and radiological factors that appeared to affect the outcome. Each factor was given points, and for each tumor, the points were added to obtain a score. On the basis of the score, the tumors were then divided into three grades. The grades determined the difficulties that could be anticipated during surgery. The majority of patients were operated on by use of unifrontal craniotomy on the side of worse vision by standard dissection techniques. RESULTS: Total tumor resection was achieved in 59 patients, and subtotal tumor resection with less than 7% of tumor left behind was achieved in 11 patients. All patients with subtotal resection had higher-grade tumors. Preoperative visual status had a bearing on the visual outcome after surgery. Four patients were misdiagnosed as having a pituitary tumor. Two patients died in the immediate postoperative period. The average follow-up was 46 months (range, 6 mo to 9 yr). There was symptomatic recurrence in one patient in whom a subtotal resection had been performed. CONCLUSION: The extent and duration of visual symptoms, encasement of the anterior cerebral artery complex, and size of the tumor were the more important factors that affected the surgery. The grading system gave a reasonable assessment concerning the possible surgical problems.

Surgical management of colloid cyst of the third ventricle--a study of 105 cases.

BACKGROUND: Colloid cyst of the third ventricle is a relatively rare intracranial tumor. It generates tremendous interest for the neurosurgeon because of its benign nature, deep location, and an excellent prognosis when diagnosed early and excised. METHOD: A retrospective analysis of 105 cases of third ventricle colloid cyst treated between 1967 to 1998 was conducted. The clinical presentation, radiological findings, different surgical approaches, and outcome were analyzed. The transcallosal and transcortical-transventricular approaches were predominantly used. Memory and psychological assessment were carried out both pre- and postoperatively. A computerized tomography (CT) scan was performed during follow-up. RESULTS: The male to female ratio was 1.5:1. The age of the patients ranged from 10 to 68 years. Headache was the most common symptom. Papilledema and short-term memory disturbances were the most common signs. In 5 patients the colloid cyst was detected incidentally. Surgery for colloid cyst was performed in 93 patients. Transcallosal and transcortical-transventricular approaches were performed in 62 and 30 patients, respectively. In 1 patient the cyst was excised through the subfrontal lamina terminalis approach. Total excision was achieved in 90 patients, while partial cyst excision was done in three patients. Moderate to severe lateral ventricular enlargement was found in 76 patients at presentation. A ventriculoperitoneal shunt was the only surgical procedure performed in 7 patients. In 16 patients colloid cyst excision was conducted after cerebrospinal fluid (CSF) diversion via a shunt. No surgical treatment of any kind was performed in 5 patients. Five patients died. Eighty-six patients came for follow-up, with a range from 1 month to 25 years (average 3 years and 8 months). Postoperatively, transient recent memory deficits occurred in 14 patients, while a permanent recent memory loss was noted in 2 patients. There was no incidence of postoperative disconnection syndrome or behavioral disturbance. A CT scan was performed in 44 patients during follow-up. Recurrence was detected in 1 patient in whom the cyst had been partially excised. CONCLUSIONS: Colloid cyst, although a benign tumor, is surgically challenging because of its deep midline location. Early detection and total excision of the colloid cyst carries an excellent prognosis.