Young child with Hurler disease shows a J-shaped sella tursi | Figure 1

CincyKidsRad

Cincinnati Children's Radiology

Young child with Hurler disease shows a J-shaped sella tursica (arrow). The sella has this configuration due to enlargement of the pituitary. The differential diagnosis includes normal anatomic variation, mucopolysaccharidoses, neurofibromatosis, and a pituitary mass.


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Axial T2-W image in a child with neurofibromatosis type 1, shows a large plexiform neurofibroma of the pelvis. The plexiform neurofibroma is composed of multiple lesions, each with a characteristic target appearance. The target appearance occurs because the neurofibroma contains a central area of dense collagen. The target sign is not always present in plexiform neurofibromas. However, if a tumor loses the sign, this is considered to be a finding of degeneration to a malignant peripheral nerve sheath tumor. Other findings of malignant degeneration include heterogeneous enhancement, lobulation of the tumor, and rapid change in size, and irregular margins.\


35 year old male patient, as per him, he was treated for keloids 3 times but no effect. he has extensive keloids on bilateral shoulders, anterior chest, abdomen, lower back and anterior shoulders. He has multiple nevus which according to him has been present since birth. i think they are cafe au lait spots and keloids could be due to correction of neurofibromatosis lesions in past. what would be your differential and how would you approach this case?


Sagittal T1-W post-contrast (image 1) and T1-W precontrast (image 2) MRI shows nodular thickening (arrow) of the pituitary infundibulum and absence of the posterior pituitary bright spot. The differential diagnosis for infundibular masses in an adolescent include Langerhans cell histiocytosis, germinoma, and other inflammatory conditions such as sarcoid, or lymphocytic hypophysitis. This patient is thought to have a germinoma. Germinomas are usually diagnosed in adolescence. They are most common in the pineal gland but also can occur in the suprasellar region or in the paraventricular basal ganglia. When they involve the infundibulum, as in this patient, they are associated with diabetes insipidus. #NeuroWednesday


Sagittal T1 weighted MRI shows a hyperintense mass (arrow) extending between the sella and suprasellar region. The differential diagnosis for this lesion includes craniopharyngioma, Rathke's cleft cyst, and arachnoid cyst. Craniopharyngioma is the most common pituitary mass and child. They most commonly occur between five and 10 years of age. #FridayQuizDay

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