1naresh
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No. Age (yr)/Sex Symptoms Areas of Involvement Biopsy Sites CNS Imaging Findings 1 37/F Diffuse bone pain,extremity numbness,gait instability,diabetes insipidus Pituitary/hypothalamus,brain stem, calvarium/skull base, spine, appendicular skeleton, lungs Brain stem lesion, lung, left clavicle Enlarged pituitary stalk, FLAIR hyperintensity hypothalamus, FLAIR hyperintensity/enhancement pons, lytic calvarial and mandibular lesions, spinal and sacral lesions (Fig 5) 2 69/M Hydro-nephrosis No CNS disease demonstrated, retroperitoneum, interstitial lung disease Retroperitoneum, lung NA 3 47/M Hydro-nephrosis, diabetes insipidus Pituitary, mediastinum, lung, retroperitoneum, appendicular skeleton Renal Enlarged pituitary stalk 4 66/M Diabetes insipidus Brain stem, sinuses, orbit, appendicular skeleton, retroperitoneum, renal Sphenoid sinus, orbit, retroperitoneum, renal T2 hyperintensity pons, sphenoid sinus mass, intraconal orbital mass, enlarged pituitary stalk 5 69/M Diabetes insipidus No CNS disease demonstrated, appendicular skeleton, retroperitoneum, chest Femur, lung NA 6 62/M Numbness head and neck, gait instability Brain stem, appendicular skeleton, perinephric infiltration Tibia FLAIR hyperintensity/enhancement pons and cerebellum (Fig 3) 7 61/F Leg pain No CNS disease demonstrated, appendicular skeleton, skin Extremity soft tissues, tibia NA 8 49/M Fatigue, diabetes insipidus Orbits, sella/cavernous sinuses, appendicular skeleton, retroperitoneum Orbit Intra-/extraonal orbital mass (Fig 4), sellar/cavernous sinus mass (Fig 4), enlarged pituitary stalk 9 60/M Ascites Calvarium/skull base, mesentery, retroperitoneum Bone marrow Enhancing calvarial foci 10 48/F Fatigue, mental status change Intracranial extra-axial, cerebellum, orbit, skull base NA IC extra-axial lesions (Fig 1), dentate nucleus hyperintensity, intraconal retrobulbar exophthalmos, sclerotic focus skull base 11 64/M Abdominal pain, diabetes insipidus, extremity weakness Intracranial, intra-axial, brain stem, cerebellum, orbit, mediastinum, retroperitoneum, appendicular skeleton Pararenal Ependymal enhancement to lentiform nucleus (Fig 2), FLAIR hyperintensity/enhancement, pons and cerebellum, intraconal orbital mass
Note:—NA indicates not applicable; IC, intracranial.