Ashis Patnaik

Primary intraosseous malignant peripheral nerve sheath tumor of spine with a giant paraspinal and retrospinal subcutaneous extension

Background: According to World Health Organization (WHO) classification of tumors, malignant peripheral nerve sheath tumors (MPNST) encompass the tumors, which were previously termed as malignant schwannoma, neurogenic sarcoma, and neurofibrosarcoma. These are rare tumors constituting only 5% of all malignant soft tissue tumors. As per their name, they arise from the malignant proliferation of cells forming sheath of a nerve root. They cause spinal cord compression, secondary changes in the surrounding bone with variable amount of tumor tissue going into the paraspinal space. However, purely intraosseous origin of the MPNST with no visible connection with a nerve root or dura is rare and few cases have been described in the literature. Case Description: We present a primary intraosseous MPNST arising from the body of a thoracic spine with a minimal intraspinal component. However, there was a huge tumor part occupying the paraspinal and retrospinal region. The latter component was so large that it extended to lie just beneath the skin. The intraspinal component was confined to only one level. The giant extraspinal part was spanning multiple corresponding spinal level. We could not find such presentation in the literature. Conclusion: Gross total removal (GTR) followed by adjuvant chemo-radiotherapy is the optimal treatment for MPNST of spine. In case of multiple laminectomy or gross spinal instability, spinal instrumentation makes the treatment protocol complete.

A rare case of primary extranodal, extradural histiocytic sarcoma of the thoracic spine and review of literature

Background: Histiocytic sarcoma (HS) is a rare aggressive malignant neoplasm of hematopoietic cell origin showing features of fever, weight loss, adenopathy, hepatosplenomegaly, and pancytopenia. Primary histiocytic sarcoma of spine without involvement of lymph nodes, bone marrow or systemic features is very rare. Due to highly malignant, aggressive behavior and frequent local and distant metastases, prognosis of histocytic sarcoma is poor. Case description: We report a case of primary extranodal variety of histiocytic sarcoma without involvement of bone marrow at extradural location of thoracic spine. Immunohistochemistry panel ruled out other more common lesions in this location. We could not find such presentation of extradural histiocytic sarcoma at the thoracic region in the literature. Conclusion: Primary involvement of extradural space by histiocytic sarcoma could arise from the proliferation of multipotent hematopoietic stem cells which are left in this space embroyologically.

Complex forms of spinal dysraphism.

IntroductionSpinal dysraphisms are a heterogeneous group of congenital malformations involving the bony component of vertebrae or spinal cord or both. Simple forms include the contiguous, solitary malformations such as myelomeningocele and diastematomyelia, and can be either aperta or occulta type.MaterialComplex forms include various types of spinal malformations occurring in combination, one type of malformations occurring at multiple levels in noncontiguous manner or spinal dysraphisms with other organ anomalies. Anomalous development in gastrulation, primary neurulation, and secondary neurulation stage in variable proportions give rise to these types of complex malformations. These cases can be diagnosed postnatally in early infancy by the strong clinical suspicion, supplemented by whole spinal axis and cranial magnetic resonance imaging (MRI) screening, to detect any occult spinal dysraphisms like split cord malformations, neurenteric cyst, lipoma, or cranial anomalies like Chiari malformations and hydrocephalus. Computed tomography and MRI are the gold standard to detect bony anomalies and cord malformations, respectively. Complex spinal dysraphisms, to our mind, should always be tried to be corrected in single operative stage, however, in case of ruptured defects, correction through multiple operations can be considered.DiscussionOverall, prognosis of complex forms of spinal dysraphism is poor in comparison to their simple forms and depends on preoperative neurological status and the nature of complex dyraphism. We discuss the classification, embryogenesis, investigation, and treatment protocol with review of literature of such complex anomalies.

Review of spinal neuroectodermal tumor

Abstract Primary spinal primitive neuroectodermal tumors (PNETs) are rare tumors. Most of these tumors occur in children and young adults. To date, 47 cases of primary spinal PNET have been reported in the literature. These tumors are highly aggressive with rapid growth. Review of the literature shows that the overall prognosis of PNETs of spinal cord is very poor even with adequate surgery, radiotherapy and chemotherapy. All the cases reported to date are reviewed in terms of surgical treatment, adjuvant therapy and outcome and the experience with two of these cases are described. Both cases were thoracic extradural ones with intrathoracic extension through intervertebral foramina resembling neurofibroma. Both cases underwent gross total removal of intraspinal and thoracic component. Post-operatively both underwent cranio-spinal radiotherapy. One patient died after a post-operative period of 4 months and the other one is still alive 8 months after surgery, radiotherapy and chemotherapy.

Intradiploic ossified giant cavernous hemangioma of skull with a dural tail sign mimicking primary calvarial meningioma.

250 Neurology India | Mar-Apr 2012 | Vol 60 | Issue 2 The case reported by Yilmazer et al.,[1] demonstrated a fusiform aneurysm of the left transverse sinus with bony erosion accompanied by internal jugular vein aneurysm. The patient was not treated and was followed up. Marco et al.,[2] described a case with a wide-necked venous aneurysm of the left transverse sigmoid sinus, and they treated it by endovascularly stent placement across the aneurysm neck and embolization of the aneurysm with Guglielmi detachable coils. Gologorsky et al.,[3] reported a widenecked venous aneurysm of the right transverse sigmoid sinus, and they treated it by surgically coagulation of aneurysmal neck and reconstruction of transverse sigmoid junction with a series of U-clips. In our patient both surgical and endovascular treatments were rather contraindicated, so palliative third ventriculostomy was done for obstructive hydrocephalus. This procedure helped the patient in attaining weight and growth. Perhaps use of fl ow diverters would also be useful in such cases. However, because we had little experience with the use of fl ow diverters in infants and as we also felt that the infant might grow out of it, we did not use this method.

Pan-brachial plexus neuropraxia following lightning: A rare case report.

Background: Neurological complications following lightning are rare and occur in form of temporary neurological deficits of central origin. Involvement of peripheral nervous system is extremely rare and only a few cases have been described in the literature. Isolated unilateral pan-brachial plexus neuropraxia has never been reported in the literature. Steroids have long been used for treatment of neuropraxia. However, their use in lightning neural injury is unique and requires special mention. Case Description: We report a rare case of lightning-induced unilateral complete flaccid paralysis along with sensory loss in a young patient. Lightning typically causes central nervous involvement in various types of motor and sensory deficit. Surprisingly, the nerve conduction study showed the involvement of peripheral nervous system involvement. Steroids were administered and there was significant improvement in neurological functions within a short span of days. Patients’ functions in the affected limb were normal in one month. Conclusion: Our case was interesting since it is the first such case in the literature where lightning has caused such a rare instance of unilateral pan-brachial plexus lesion. Such cases when seen, raises the possibility of more common central nervous system pathology rather than peripheral involvement. However, such lesions can be purely benign forms of peripheral nerve neuropraxia, which can be managed by steroid treatment without leaving any long-term neurological deficits.

Management of Chronic Subdural Haematoma in a Case of Idiopathic Thrombocytopenic Purpura

Intracranial hemorrhage is a rare devastating complication of idiopathic thrombocytopenic purpura (ITP), mostly presenting as intraparenchymal or subarachnoid haemorrhage. Isolated chronic subdural haematoma (SDH) is still very rare and the optimal management is unsettled. Spontaneous resolution of chronic SDH in patients with idiopathic thrombocytopenic purpura is possible. We report a case of spontaneous chronic SDH in a patient with ITP with hemiparesis where the haematoma significantly decreased in size with complete resolution of hemiparesis as a result of platelet transfusions and continuing therapy with steroids. Surgical treatment of subdural haematoma with consequent bleeding complications can be avoided in this scenario.

Spontaneous extradural and subgaleal hematoma: A rare neurosurgical crisis of sickle cell disease

Extradural hematoma (EDH) in absence of trauma is a rare entity with only few cases reported in literature. The various causes reported include: Vascular malformation of dura, coagulopathies, sinus infection, middle ear or orbital infection, and tumor. Occurrence of spontaneous EDH as a complication of sickle cell disease is even much rarer. We report a case with sickle cell disease who presented with spontaneous extradural and subgaleal hematomas following an episode of vaso-oclusive crisis. He was managed successfully with surgery. The association of epidural hematomas in sickling hemoglobinopathies is reviewed. In all cases, we noticed one episode of sickle cell crisis just before the occurrence of spontaneous EDH. Perhaps this crisis puts an extra demand over the hematopoietic skull tissue disrupting inner and outer skull margins leading to spontaneous EDH and subgaleal hematoma.

Spontaneous leaking craniopharyngioma causing preoperative chemical meningitis.

Sir, Spontaneous chemical meningitis in craniopharyngioma is a rare phenomenon. The cause of this meningitis is due to rupture of craniopharyngioma and release of chemical contents particularly cholesterol crystals. We report an interesting case of chemical meningitis due to a leaking craniopharyngioma rather than a ruptured one. Very few cases of leaking craniopharyngioma have been reported in the literature.[2,5] A 13-year-old girl presented with an intermittent headache for last 1 year. She had complete loss of vision on the left side eye and progressively diminishing vision on the right one. The computed tomography (CT) scan showed a hypodense lesion in pituitary fossa with extension to the suprasellar region. There were areas of calcifications on the peripheral part of the lesion [Figure 1]. Magnetic resonance imaging showed the lesion to be predominantly cystic with a small solid part in inferior and posterior part of the lesion [Figure ​[Figure2a2a–f]. Patients’ hormonal status was normal. While she was waiting for the transphenoidal excision of the lesion, she developed fever, altered sensorium with neck stiffness. Vision status remained same as before. Lumbar puncture was done, and the cerebrospinal fluid (CSF) study revealed increased cell count (90) with predominant mononuclear cells (>90%). The protein level was grossly elevated to 8 g%. Cholesterol level in CSF was unusually high at 7 mg%. The culture of the CSF was negative. Repeat CT scan demonstrated the lesion to be of the same size without any reduction [Figure 3]. The patient was treated with intravenous steroid and antibiotics and she recovered from meningitis within 1 week. She later underwent transphenoidal complete excision of the lesion [Figure 4]. The lesion was mostly cystic containing a muddy brownish fluid with cholesterol crystals in it. The solid part was chalky white in color and removed in piecemeal. The patient normally recovered in the postoperative period with no features of meningitis, CSF leak or hypopituitarism. Histopathology was suggestive craniopharyngioma [Figure ​[Figure5a5a–c]. In the follow-up, 2 months after the operative procedure, patients’ vision improved in the right eye but there was no improvement on the left side. Figure 1 Plain computed tomography scan showing a globular, well-defined hypodense lesion in sellar and suprasellar area with wall calcification Figure 2 Magnetic resonance imaging pictures of the lesion. (a) T1-weighted sagittal image showing the lesion containing fat containing T1 hyperintense antero-superior and hypointense solid part in postero-inferior part. (b) T2-weighted coronal image of the lesion. ... Figure 3 Computed tomography images of the lesion after meningitis showing the lesion to be of same size as before Figure 4 Complete excision of the lesion except a small part of its wall attached to the surrounding vessels Figure 5 (a) Low magnification showing the epithelial lining with keratin. (b) High magnification showing the squamous epithelial lining. (c) Wet keratin flecks secreted by the epithelial lining Aseptic or chemical meningitis is a rare complication in craniopharyngiomas particularly cystic ones, due to rupture and spillage of its contents containing cholesterol into the subarachnoid space, secreted by its squamous epilthelial lining. The occurrence of meningitis following rupture of the cyst is directly related to the cholesterol contents as its absence makes the rupture asymptomatic. Takahashi et al.,[8] reported two cases of spontaneous rupture of craniopharyngiomas without any meningitic symptoms. In both of these cases, the cysts did not contain cholesterol crystals. The rupture of the cyst also leads partial decompression leading improvement of the symptoms. In our case, the meningitis followed the leakage of its contents rather than frank rupture as evidenced by no change in cyst size in the repeat scan. There was no change in the vision as the rupture would have resulted in partly decompression on the visual pathway leading to vision improvement. Suprasellar cystic tumors such as craniopharyngioma, dermoid, and epidermoid, Rathkes cleft cyst can rupture spontaneously with remission of symptoms particularly those caused by a compressive effect like vision symptoms. Cerebral infarction due to vasospasm following craniopharyngioma cyst rupture has also been reported in the literature.[7] Few cases of spontaneous rupture of craniopharyngioma have been reported until now.[1,3,4,6,7,8,9] This rupture can result in chemical meningitis depending upon the cholesterol content of the cyst fluid. The exact mechanism of cyst rupture in these tumors is not clearly known, but it may be induced by the progressive enlargement of the cyst causing weakness of the cyst wall and finally rupture. The leaking variety, as in the present case, can occur if the rent in the cyst wall is small enough to prevent the significant escape of cystic fluid into the subarachnoid space. This also explains the nonreduction in the size of the cyst as well as no relief of compressive effect of the cyst on surrounding structures. Our case was exclusive in that the meningitis episode occurred preoperatively without any reduction in the cyst size raising possibility of a bacterial cause. However, the CSF study established the chemical nature of the lesion. Neurosurgeons should be aware of such spontaneous leakage or rupture of the cyst in preoperative stage causing chemical meningitis in a cystic tumor. CSF and radiological examination must be done to establish the exact cause. Treatment is directed toward immediate management of meningitis followed by removal of the cause. Financial Support and Sponsorship Nil. Conflicts of Interest There are no conflicts of interest.

Primary hydatid cyst of brain: Two cases report

Brain involvement with hydatid disease occurs in 1-2% of all Echinococcus granulosus infections. Isolated cerebral hydatid disease is a rare manifestation of it. In this report, we analyze two cases of isolated cerebral hydatid cysts and discuss their mode of presentation, radiological features, operative procedure and outcome. In our two cases, radiological features and surgical approaches were different from one another. The literature concerning isolated cerebral hydatid disease is reviewed.