Primary intracranial Parachordoma: An unusual tumor in brain.

BACKGROUND: Parachordomas are rare soft tissue tumors commonly occurring in limbs, chest, Abdomen, and back. The World Health Organization (WHO) classification includes parachordomas in the same group as mixed tumors and myoepitheliomas. Exact histogenesis of this tumor is unclear. CASE DESCRIPTION: A 52-year-old male presented with headache and blurring of vision since one month. Preoperative computed tomography (CT) scan of brain revealed left parieto-occipital tumor extending up to the trigone. Total excision of the tumor was done. Histopathologically, the tumor was composed of relatively uniform cells with eosinophilic cytoplasm in a myxoid stroma and with cartilaginous and osseous metaplasia. The tumoral cells were immunoreactive for cytokeratin, epithelial membrane antigen (EMA), S-100, and vimentin. The constellation of findings revealed the tumor to be parachordoma. Magnetic resonance imaging (MRI) brain during follow-up at one year showed no recurrent tumor. No adjuvant therapy was given to this patient. CONCLUSION: This is the first reported case of primary intracranial parachordoma. It is difficult to diagnose the lesion preoperatively by imaging alone. Long-term follow-up is necessary in view of few reports in literature of recurrence and metastasis, of parachordomas in other anatomical locations.

A rare case of an extra-axial cavernous angioma in the cerebellopontine angle.

Intracranial extra-axial cavernous angiomas are rare lesions. We report a rare case of extra-axial cavernous angioma in the cerebellopontine angle (CPA) in a 50-year-old male, who presented with lower cranial nerve palsy and gait ataxia. Computed tomography (CT) scan of the brain showed a hyperdense lesion in the left cerebellopontine angle. The lesion was totally excised by the retrosigmoid approach and a pathological examination confirmed the lesion to be a cavernous angioma. Following surgery, the lower cranial nerve palsy recovered significantly.

Hashimoto's thyroiditis associated Evans syndrome: A rare case report on the clustered autoimmune disease triad.

Evans syndrome is a rare combination of autoimmune hemolytic anemia and immune thrombocytopenia. Their association with autoimmune thyroid diseases has been reported by few authors; however, a sequential development of the Evans syndrome in cases of Hashimoto's thyroiditis is extremely rare. The clustering of these autoimmune diseases might share a common pathogenic pathway. We present the fourth such case in world literature, of a 34-year-old female diagnosed with Hashimoto's thyroiditis in 2006, who has been taking synthetic thyroid hormone since then. Her condition is now clinically complicated with the development of the Evans syndrome.

Encephalocraniocutaneous Lipomatosis (Haberl and syndrome): A case report and review of literature.

Encephalocraniocutaneous lipomatosis (ECCL) is a rare sporadic neurocutaneous syndrome characterized by presence of central nervous system, ocular and cutaneous anomalies. The exact pathogenesis is still not known. We present the third case from the Indian subcontinent, who is a five year old girl with history of right sided seizures. Dermatological examination showed alopecia on right side of the scalp and ipsilateral limbal dermoid and nodular skin tags over the upper eyelid. The computerized tomography scan of the brain revealed porencephalic cyst, cerebral calcifications and atrophy of right brain. The histopathology of the skin lesions showed lipomatous hamartoma and features of non scarring alopecia. The constellation of these findings and in adherence to the diagnostic criteria of ECCL proposed in 2009, we consider this report as a definite case of ECCL.

Intracranial metastasis from primary spinal primitive neuroectodermal tumor.

Primary spinal primitive neuroectodermal tumors (PNET) are rare tumors, with only 94 cases reported till date. Metastasis to brain from a spinal PNET is even rarer. In the present report, we evaluate the pathology and treatment of solitary intracranial metastasis from spinal PNET in a 22-year-old female who presented with headache and left hemiparesis and was diagnosed to have right parietal parasagittal tumor. She has been previously diagnosed to have cervicothoracic primary spinal PNET, and was treated by surgery, radiotherapy, and chemotherapy seven years back. The intracranial tumor has been removed and pathological examination confirmed as PNET. She received radiotherapy and chemotherapy with ifosfamide and etoposide, following surgery for the right parietal PNET. At 20 months follow-up, patient is stable and has no recurrence of the disease. Critical review of reported cases of primary spinal PNET metastsising to brain was done.

Spinal epidural angiolipoma: A rare cause of spinal cord compression.

Spinal epidural angiolipomas are rare, benign tumors composed of mature lipocytes admixed with abnormal blood vessels. Only 128 cases of spinal epidural angiolipomas have been reported in literature till now. Spinal angiolipomas are predominantly located in the mid-thoracic region. We report a case of dorsal epidural angiolipoma in a 56-year-old male who presented with paraparesis and was diagnosed to have D4-5 epidural angiolipoma. Total surgical excision of the epidural angiolipoma was done and his paraparesis gradually improved.

Surgical excision of hypothalamic hamartoma in a twenty months old boy with precocious puberty.

A twenty months old boy presented to our department with true precocious puberty due to hypothalamic hamartoma. Total surgical excision of pedunculated hypothalamic hamartoma was done successfully by the pterional trans-sylvian approach as he could not afford medical management. Patient had uneventful post-operative course with normalization of serum testosterone levels and regression of secondary sexual characters.

Mantle cell lymphoma--a clinicopathological study of 13 cases

Mantle cell lymphoma is an uncommon non-Hodgkin's lymphoma. In a period of four years, 13 cases of mantle cell lymphoma were diagnosed in our department, comprising 3.1% of all non-Hodgkin's lymphoma diagnosed. The mean age of presentation was 52 years with a slight male preponderance. The disease was nodal in twelve and extra-nodal in tonsil in one. Five patients had bone marrow involvement. Five cases showed a nodular pattern on lymph node biopsy while the remaining eight had a diffuse pattern. Immunophenotyping showed positivity for CD20 and cyclin D1. Despite certain morphological similarity to other low-grade lymphomas, mantle cell lymphoma has a characteristic appearance of its own. It is more aggressive than other low-grade lymphomas and hence needs to be accurately diagnosed.