Patient demographics and imaging findings
| 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.