Recurrent intermediate grade orbital melanocytomas are rarely encountered and have to be distinguished from other topographically similar primary melanotic tumors. They have to be managed more aggressively if intracranial extension is present due to its close relation to the visual pathways.
Primary melanoma is an extremely rare tumour of the spinal cord. We are reporting a case of primary melanoma of the spinal cord in a 55-years-old male patient. Magnetic resonance imaging showed an extradural intraspinal lesion opposite the L4 vertebral body. The lesion was completely resected and a microscopic diagnosis of melanoma was made. Thirty eight months later, the patient is alive, with no evidence of any tumour recurrence. INTRODUCTION CASE REPORTA 55-year-old male patient with a history of back pain, a weakness in the lower extremities and with urinary and anal incontinences, was admitted. His symptoms had progressively worsened to paraplegia. The neurological signs were consistent with lower spinal cord compression. A careful general physical examination did not reveal any lymphadenopathy, organomegaly or cutaneous lesions. A Magnetic Resonance Imaging (MRI) scan revealed an extradural intraspinal lesion opposite the L4 vertebral body, which was widening the intervertebral foramen. A provisional diagnosis of a neurofibroma was made. Intraoperatively, a bluish, engorged, extradural, intraspinal lesion which measured 1 cm was seen to arise in relation to the right L4 root. The lesion was completely resected and it was sent for a histopathological examination. Pathological FindingsGrossly, multiple black friable tissue bits were received. The histological examination showed a spinal cord tissue with a tumour which was composed of proliferating polygonal to spindle shaped cells which had an abundant intra-cytoplasmic brown to black pigment [Table /Fig-1A, B]. The tumour cells had vesicular nuclei with prominent nucleoli [Table /Fig-1C]. The cells were arranged in tight clusters and sheets. Numerous mitotic figures along with an atypical mitosis were found [Table/ Fig-1C]. Bizarre multinucleated giant cells were seen focally. Areas of haemorrhage and necrosis were also present. Masson's Fontana stain for the melanin pigment was positive [Table/ Fig-1D]. A melanin bleach confirmed that the intracytoplasmic pigment was melanin and it highlighted the Pathology Section Ruchi Sinha, TauSeef huSain Rizvi, ShRijeeT chaKRaboRTi, chandRa KumaR ballal, anuP KumaR nuclear features. The Perl's Prussian Blue stain was negative and it excluded the haemosiderin pigment [Table/ Fig-1E]. DISCUSSIONThe present World Health Organiztion classification classifies primary melanocytic lesions of the CNS into diffuse melanocytosis, melanocytoma, meningeal melanomatosis and malignant melanoma. Among these lesions, primary malignant melanoma of the CNS accounts for only 1% of all melanoma cases [1]. The incidence of primary spinal melanoma is even rarer. According to the Hayward classification [3], the diagnosis of primary spinal cord melanoma is based on the absence of a melanoma outside the CNS, the absence of this lesion at any other site in the CNS and a histologic confirmation of melanoma.[ Table/
Esthesioneuroblastoma, also known as olfactory neuroblastoma, is an uncommon malignant neoplasm arising from the olfactory epithelium in the roof of the nasal cavity. There are very few case reports published worldwide. The common presenting symptoms of Esthesioneuroblastoma are unilateral nasal obstruction (70%), epistaxis (50%), anosmia, rhinorrhoea, facial pain, headache, excessive lacrimation and rarely proptosis and visual disturbance. Apart from being locally aggressive, it metastasizes by haematogenous and lymphatic routes. We report an extremely rare case of esthesioneuroblastoma in a 20-year-old man with orbital involvement presenting as dystopia. This rare tumour should be considered in the differential diagnosis for young patients presenting to ophthalmic outpatient department with dystopia.
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Paraganglioma is a rare tumor of the cerebellopontine angle. We report a case of a 55-year-old female presented with otalgia and decreased hearing in the left ear, hoarseness of voice, difficulty in swallowing, nasal regurgitation, and tinnitus. The tumor was excised by left retromastoid and posterior fossa craniectomy approach. Intra-operative crush smear and histopathological examination revealed a paraganglioma. Post-surgery the patient was treated with fractionated radiotherapy.
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