Evaluation of surgically treated primary spinal cord tumors in a single Indian institution: A case series study of 178 patients.

Introduction: Primary spinal cord tumors are rare tumors that are usually heterogeneous having variable histopathological subtypes. Rapidly, growing space-occupying lesions in the spinal canal cause severe loss of function. This study aimed to analyze all adult patients diagnosed with primary spinal cord tumors and to describe their location, symptoms, histopathological types, surgical outcome, complications, recurrence in a single institution, and prognostic factors. Materials and Methods: The study was a retrospective study of 178 patients with spinal tumor who underwent surgical intervention at the department of neurosurgery at a tertiary care institute from 2014 to 2021. Patient demographic characteristics, symptoms, radiological features, spinal level of tumor, spinal compartment, preoperative and postoperative McCormick grade, and complications were recorded, and a comparison of outcomes after surgical intervention was done. Results: In our study, 97 patients were males and the rest of the 81 patients were females. The mean age at the time of surgery was 43.5 years. The thoracic region of the spinal canal was the most involved with 67 (37.64%) cases, whereas intradural extramedullary was the most common (65.73%). Schwannoma was the most common histopathological subtype (46.06%). Total excision was achieved in 154 cases. A total of 37 patients were in Grades 4 and 5 of modified McCormick's grade preoperatively. However, postoperatively, there were only 17 patients in this group. Two patients with cervical intramedullary tumor expired in the postoperative period. Conclusion: Early surgical intervention with sound microneurosurgical skills gives good outcomes independent of the type and location of tumor. Better outcomes are seen in extradural tumors, early surgical intervention, tumors better amenable to total resection, and in patients with better preoperative neurological status.

Clival Tuberculosis: A Case Report.

A 39-year-old female presented with complaints of occipital headaches, diplopia, numbness over left half of face and deviation of face to the right. On examination she had hypoesthesia over left half of face, associated with bilateral abductor and left facial palsy. Neuroradiology showed a well-defined lytic lesion involving the clivus and adjacent sphenoid sinus and sella. The patient underwent an endoscopic transnasal decompression of the clival lesion. Intraoperative squash preparation was reported to show tuberculous granulation, which was confirmed on postoperative histology. The patient was advised anti-tubercular therapy. At 12 months follow up neuroradiology showed a near total resolution of the clival lesion. The patient had completely recovered from her cranial nerve deficits. Tuberculous involvement of spheno-clival region is rare and the authors' literature search has yielded only three previous similar case reports. A surgical decompression followed by anti-tubercular therapy is the recommended approach for management of clival tuberculosis. The relevant literature on the subject is presented.

Intracanalicular epidermoid cyst. A case report.

We describe the presentation and management of a rare case of an epidermoid cyst located within canal of the left internal acoustic meatus, lying entirely within the petrous part of the temporal bone. The patient underwent surgery by an extended middle fossa approach and a total excision of a cyst containing cheesy, pearly white, avascular material was achieved. Neuroradiology provides a diagnosis with reasonable accuracy and an appropriate surgical approach can be planned for the excision of the tumour.

Retrospective analysis of peripheral nerve sheath tumors of the second cervical nerve root in 60 surgically treated patients.

OBJECT: This study is a retrospective analysis of 60 surgically treated patients with 64 peripheral nerve sheath tumors (PNSTs) at the second cervical (C-2) nerve root. The anatomical subtleties of these tumors and their implications for surgical strategy when compared with other spinal PNSTs and other tumors in the foramen magnum region are reviewed. METHODS: Sixty patients with C-2 PNSTs treated surgically in the Department of Neurosurgery at King Edward VII Memorial Hospital and Seth Gordhandas Sunderdas Medical College between 1992 and 2006 were studied. All patients underwent magnetic resonance imaging. Tumors were divided into 3 groups depending on their anatomical location identified during surgery. Those tumors located within or extending into the spinal dural tube were called Type A, those located within the dural tube of the C-2 ganglion were labeled as Type B, and tumors extending laterally into the paraspinal region were labeled as Type C. Follow-up durations ranged from 6 months to 15 years (mean 64 months). RESULTS: There were 38 male and 22 female patients in the study, who ranged in age from 6 to 62 years (mean 28 years). Nine patients had clinical features indicative of neurofibromatosis (NF). The mean duration of symptoms at the time of presentation was 27 months (range 4 days-5 years). Two patients had no specific symptoms related to the C-2 PNST, 6 patients had only local symptoms such as neck pain or stiffness, and 52 patients had symptoms of varying degrees of myelopathy. There were 5 solely Type A tumors, 7 Type A + B tumors, 31 Type B tumors, and 21 Type B + C tumors. All Type A, A + B, and B tumors were totally resected. Seven of 21 Type B + C tumors were partially resected, and the remainder were completely resected. All patients postoperatively reported varying improvement in their preoperative symptoms. Except for patients with NF who were disabled by other tumors, the rest of the patients resumed their normal life style. There have been no cases of symptomatic tumor recurrence. CONCLUSIONS: The majority of PNSTs located at the C-2 level in these patients probably arose from the large C-2 ganglion and are limited within the dural confines or are interdural in location. In contrast to other spinal PNSTs, the location of C-2 PNSTs is in most cases posterior to the lateral mass of the atlas and axis and the atlantoaxial joint and is exposed to the posterior without any bone cover. Radical tumor resection is safe, resolution of clinical symptoms is rapid, and recurrence rates are extremely low. In a selected number of cases, bone work for tumor exposure and resection can be entirely avoided.

Primary Ewing's sarcoma of the petroclival bone.

A 17-year-old male presented with signs of raised intracranial tension and multiple cranial nerve dysfunction. Radiological imaging revealed an extradural calcified tumor involving the right petrous bone and adjacent clivus. The patient underwent extensive surgical debulking of the mass. Histopathological study revealed that the tumor was Ewing's sarcoma. Post-operative radio- and chemotherapy were administered. The patient's cranial nerve dysfunctions had recovered to some extend at 12 months' follow-up. Ewing's sarcoma arising from the skull base is an extremely rare lesion. The relevant literature on the subject is reviewed.

Transthoracic approach for ventrally situated paraspinal extradural hydatid cyst of the dorsal spine.

A 40-year-old female presented with back pain without any neurological deficits. Radiological investigations revealed a ventrally situated paraspinal lesion causing widening of the right D4 neural foramen. She underwent a right posterolateral thoracotomy and excision of the lesion in toto, which was reported as a hydatid cyst on histology. Ventrally situated paraspinal extradural hydatid cysts are rare. A Transthoracic surgical exposure offers a direct approach for complete excision of the lesion, minimizing the chances of rupture and spillage.

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.

Severe intracranial hypertension in slit ventricle syndrome managed using a cisterna magna-ventricle-peritoneum shunt.

OBJECT: Severely increased intracranial pressure (ICP) can be life threatening in patients who had previously undergone shunt treatment but who do not experience ventricular enlargement. The authors analyzed the utility of placing shunts into the cisterna magna concurrently with ventricular shunts in patients who were not candidates for lumboperitoneal (LP) shunt placement. METHODS: Ten patients treated with cisterna magna-ventricle-peritoneum (CMVP) shunts for complex problems of shunt function were reviewed retrospectively. All patients had documented increases in ICP and ventricles that did not expand despite life-threatening increases (> 80 mm Hg in one case) in ICP. Between 1995 and 2003, 10 patients (four males and six females, age range 4-32 years) were identified as having life-threatening increases in ICP despite small or slit-like ventricles on imaging studies. Each episode was documented with intraparenchymal pressure monitoring. All patients had documented ventricular catheter failures at the time of the intervention, and all had undergone at least one previous attempt to treat the condition with a valve upgrade and replacement of the ventricular catheter. Three patients had achondroplasia, four had spina bifida, and three had a preexisting Chiari malformation Type I. All patients improved after the procedure, and none suffered permanent complications. For at least 48 hours after surgery, all patients underwent intraparenchymal monitoring of ICP (an intraparenchymal monitor was used that documented normal ICP). CONCLUSIONS: The CMVP shunts are an excellent option for patients who are not candidates for LP shunts but who have high ICP and ventricles that do not enlarge at shunt failure. The ability to access the spinal fluid in the cortical subarachnoid space presumably accounts for this success.

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.

Primary intramedullary primitive neuroectodermal tumor of the cervical spinal cord. Case report.

Primary intramedullary primitive neuroectodermal tumors (PNETs) of the spinal cord are rare. Only six cases have previously been reported, all involving tumors in the thoracic or lumbar spine. The authors report the case of a 54-year-old woman who presented with quadriplegia and bladder and bowel dysfunction. The patient had suffered symptoms of neck pain for 1 month and left shoulder weakness for 10 days. Magnetic resonance imaging of the cervical spine revealed an intramedullary mass extending from C-2 to C-5 with an exophytic component in the adjacent left subarachnoid space. Multiple biopsy specimens were obtained, and a partial excision was performed. Histological examination revealed nodular growth and neuronal differentiation, with a striking resemblance to desmoplastic medulloblastoma. A positron emission tomography scan did not reveal uptake at any site. These findings confirmed the diagnosis of a primary intramedullary PNET. Postoperatively, the patient was given craniospinal radiotherapy with a radiation boost to the tumor bed.

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.

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.

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.

Radiologic Evaluation of Basilar Invagination Without Obvious Atlantoaxial Instability (Group B Basilar Invagination): Analysis Based on a Study of 75 Patients.

BACKGROUND: We evaluated the radiologic features of 75 patients with group B basilar invagination who exhibited no evidence of atlantoaxial instability based on the conventional parameter of an abnormal increase in the atlantodental interval. We specifically studied the variability and possible significance of the presence of cerebrospinal fluid (CSF) within and outside the confines of neural tissues. MATERIALS AND METHODS: During the period January 2008-May 2015, we encountered 75 cases with group B basilar invagination. These patients were divided into 4 groups depending on cervical spinal imaging that showed the presence of syringomyelia (group B1), increased CSF volume in the extramedullary space or external syrinx (group B2), the presence of both syringomyelia and external syrinx (group B3), and no abnormality of CSF cavitation in the spinal canal (group B4). RESULTS: Our cohort comprised 39 group B1 cases, 10 group B2 cases, 20 group B3 cases, and 6 group B4 cases. The neck size and posterior fossa height were simultaneously reduced, by 15.89% and 15%, respectively, but the length of the neural structures remained within the normal range. Excessive amounts of CSF were present within or outside the confines of neural structures, including the spinal cord, brainstem, and cerebellum. CONCLUSIONS: In cases of basilar invagination, various musculoskeletal and neural alterations seem to have a common functional role in protecting the craniocervical cord and delaying or stalling neurologic dysfunction.

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.

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.

Spontaneous resolution of residual pituitary adenoma. Case report.

A 34-year-old man presented with progressive diminution of vision in the left eye for 7 years. He had suffered left hemicranial headache associated with left retro-orbital pain and diplopia for 3 months. Magnetic resonance (MR) imaging revealed a pituitary tumor located in the sella and extending into the right cavernous sinus. After transsphenoidal surgery, the vision improved drastically but the diplopia persisted. Postoperative MR imaging showed residual tumor in the right cavernous sinus. Follow-up examination after 3 years showed the diplopia had completely recovered and the residual tumor in the cavernous sinus had disappeared. Spontaneous resolution of a large intracavernous sinus residue of a pituitary adenoma may occur due to tumor necrosis.