Neurocutaneous spectrum of multiple endocrine neoplasia-1.

Multiple endocrine neoplasia type I or Wermer syndrome is characterized by primary hyperparathyroidism, enteropancreatic endocrine tumor, and a pituitary pathology. A 35-year-old male presented with visual field defects, hyperprolactinemia, and hypogonadism. He also had multiple infraumbilical skin-colored nodules. A syndromal association of Wermer syndrome was derived using the dermal, pituitary, parathyroid, and gastrointestinal hormonal manifestations of the tumor. The radiological and histological findings of lesion which underwent biopsy are discussed. The presence of collagenomas, lipomas, and hypopigmented macules in a patient with neuroendocrine symptoms should raise the suspicion of an underlying multiple endocrine neoplasia.

Recurrent posterior fossa anaplastic ependymoma with prominent chondroid metaplasia: A case report and review of literature.

We report an unusual case of a recurrent fourth ventricular anaplastic ependymoma with prominent chondroid metaplasia in a 16-year-old male. On initial presentation, the patient had a WHO Grade II tumor. However, at recurrence 1 year later, the tumor progressed to WHO Grade III tumor with more cellularity, necrosis and brisk mitotic activity. Chondroid metaplasia was present in both the initial and recurrent tumors.

Inflammatory myofibroblastic tumor parieto-occipital bone.

Inflammatory myofibroblastic tumor is a diverse group of lesions first described in lung and later on reported in various sites like stomach, bowel, spleen, bone. We report a case of inflammatory myofibroblastic tumor in a 30-year-old male who presented with a slowly progressive scalp swelling of two-year duration. Magnetic resonance imaging showed an intradiploic well enhancing lesion in parietal and occipital bone, isointense on T1 weighted images and hypo intense on T2 weighted images with dural enhancement. On histopathological examination, the lesion was composed of variable admixture of spindle cells with eosinophilic cytoplasm and inflammatory cells comprising of plasma cells and lymphocytes. The lesion was infiltrating the underlying dura. The spindle cells showed strong positivity for smooth muscle actin on immunohistochemistry. A final histopathologic diagnosis of inflammatory myofibroblastic tumor was rendered.

Chronic brain abscess secondary to a retained wooden foreign body: Diagnostic and management dilemmas.

A four-year-old child presented with recent onset generalized tonic clonic seizures. She was operated for a suspected intracranial tuberculoma and was found to harbor an intra-parenchymal retained wooden foreign body with a chronic abscess. The foreign body had entered the brain parenchyma after a minor head injury, sustained three years earlier. She was asymptomatic for the intervening three years. The initial diagnosis was missed by several physicians. A retained wooden fragment via a transtemporal apparently closed head injury is an extremely rare event. The present study reveals the diagnostic and therapeutic challenges and stresses the importance of high degree of suspicion to diagnose retained intracranial foreign bodies and the need for early surgical exploration, to avoid chronic and potentially life threatening neurological complications.

Isolated intracranial Rosai Dorfman disease masquerading as meningioma: a case report.

Isolated intracranial Rosai Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is rare. We present a 26-year-old male who presented with left focal motor seizures becoming secondarily generalized of one-year duration. Clinically and radiologically patient was diagnosed to have a right parietal convexity meningioma. However on histopathological examination a final diagnosis of intracranial Rosai Dorfman disease was rendered.

Fine needle aspiration cytology of epithelioid variant of schwannoma--a diagnostic dilemma.

Epithelioid variant of schwannoma is a rare type of benign nerve sheath tumor which has an aggressive clinical course in comparison to the conventional schwannoma. We report one such case in a 53 year old female with an aspirate from a swelling in the nape of the neck. It posed a diagnostic dilemma because of its cellularity numerous groups of plump epithelioid cells along with multinucleated cells. The cells were lying in a myxoid stroma, coming singly and in nests and cords thus mimicking an epithelial tumor. The initial cytologic diagnosis of possibly a skin adnexal tumor was rendered. This case highlights the diagnostic pitfall encountered in the aspiration cytology of neurogenic tumors.