ABSTRACT. We report the first case of extensive involvement of isolated intracranial Rosai-Dorfman's disease (RDD) in a child. Our case is unique because it presents with involvement of the middle cranial fossa, cavernous sinus, pituitary gland, orbit, ethmoid and sphenoid sinuses. Previous cases of intracranial RDD in children have reported separate involvement of cavernous sinus, suprasellar region, and frontal and petroclival regions. Involvement of the pituitary gland has so far not been reported. A 14-year-old male presented with a medical history of loss of vision, raised erythrocyte sedimentation rate (ESR), and abnormal prolactin and cortisol levels. Radiologically the diagnosis was meningioma. The histopathological diagnosis was RDD with emperipolesis and S-100 positivity. RDD is a histiocytic proliferation of unknown aetiology, which commonly affects lymph nodes. Uncommonly it involves the extranodal sites and rarely the central nervous system (CNS). 80 cases of RDD involving CNS have been reported in the literature, and only 5 were in children. Although the definitive diagnosis of RDD disease is based on the histopathology report, it should be included in the differentials of a lesion mimicking meningioma especially in children.

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Spontaneous pneumorrhachis, non-traumatic, non-iatrogenic air within the spinal canal, is a very rare occurrence. We report a case of spontaneous pneumorrhachis, multiple air pockets in the epidural space, with vacuum discs and spndylolisthesis. Probably this is the first report of such case.

The occurrence of neoplastic and nonneoplastic dural based masses that mimic meningiomas is infrequent and may not be considered during radiological and intraoperative analysis. We describe single institute study of 20 such rare cases. This study included total of 20 cases of meningioma mimics. The clinical, radiological and histopathological findings were evaluated. Tissue fixed in 10% formalin was routinely processed and 5 μ thick sections were cut and stained with hematoxylin & eosin. Histochemistry and immunohistochemistry using avidin-biotin complex immunoperoxidase method was done wherever indicated. In the present study group, 15 were male and 5 female with a male: female ratio of 3:1. The age ranged from 14 to 78 years. Radiologically all these lesions were extra-axial in location, predominantly hypointense on T2W, isointense on T1W images and showed intense homogenous enhancement on contrast administration. Four cases were in pediatric age group with histopathological diagnosis of Rosai Dorfman disease, medulloblastoma, hemangiopericytoma and malignant melanoma. In the adult population, the histopathological diagnoses were hemangiopericytoma, undifferentiated sarcoma, extraskeletal osteosarcoma, Rosai Dorfman disease, medulloblastoma, and metastases from systemic malignancies. Of the total 6 cases of metastases 1 was nonseminomatous germ cell tumor from a primary in testis, 1 was adenocarcinoma from an unknown primary, and 4 were adenocarcinoma from lung. There was a single case of dural based frontal lobe malignant melanoma with congenital hairy nevi on anterior chest wall, scalp, anterior abdominal wall and inguinal region. As the management and biologic behaviour of many of the MM are different, it is essential to familiarize ourselves to them.

Sexual differences in the basioccipital portion of the skull of dogs have been described and an index is presented which reliably predicts the sex of the skull. 92 dolichocephalic skulls (44 male, 48 female) from mongrel dogs were used. In the basioccipital region of the male skulls, a triangular area, which extends from the basion to a line joining the medial-most points of the two jugular foramina, appears narrow and elevated. The pharyngeal tubercle is also prominent. In female skulls the rostral angle of this triangle is wider and the triangular area seems smoother and flat. The anterior half of the female basioccipital portion is wider and smoother than in the male skull. In order to quantify the observed differences, four indices were calculated. All of the indices were statistically significant. However, only index IV could be reliably used in predicting the sex of an unknown skull: breadth IV × 100 Index IV = length II was derived from breadth IV (distance between the two temporo-occipital fissures at their lateralmost points) and length II (distance between the basion and a line drawn between the two medialmost points of the jugular foramina in the midline). Values of less than 123 for male and greater than 136 for female placed the skulls in their proper sex group. Skulls that had intermediate values could be placed in correct sex grouping only in conjunction with strong observational criteria. It is suggested that in absence of such strong observational criteria these skulls may belong to immature or castrate animals.

We describe a case report where the diagnosis of sporadic Creutzfeldt-Jakob disease(CJD) was suspected by the magnetic resonance imaging (MRI) radiologist in a 75-year-old lady who presented with rapid cognitive decline. MRI revealed cortical T2 and FLAIR(fluid attenuation and inversion recovery) hyperintensities in bilateral fronto-parietal and temporo-occipital locations and showed significant restriction on diffusion-weighted images(DWI). In this case report, we discuss the role of MRI to suspect the diagnosis of CJD in appropriate clinical settings.

Intradiploic epidermoid cysts are congenital benign lesions. They commonly occur intracranially. Intradiploic epidermoid cyst of the calvarium is very rare and few cases have been reported to date. We describe a giant intradiploic epidermoid cyst of the occipital bone with CT and MRI characteristics including DW imaging and ADC values.

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.