Langerhans cell histiocytosis (LCH) with skull lesions and lymphocytic hypophysitis

Case contributed by Dr Mohammad A. ElBeialy


Loss of appetite, polydipsia and polyuria. Physician diagnosis is central diabetes insipidus.

Patient Data

Age: 2 years
Gender: Male

There is a well-defined smoothly osteolytic mass lesion within the right frontal bone's both tables measuring 14.3 X 7 mm, having small intra-cranial extra-axial and subtle extra-cranial subgalial extension. There are similar left posterior high parital as well as clival and anterrior inferior frontal enhancing bony lesions. 

There is bilateral globus pallidus rather symmetrical increased T2 signal intensity that is inconspicuous in T1 and shows no post-contrast enhancement.

The pituitary stalk is a bit prominent and homogeneously enhancing with loss of the normal bright T1 signal of its posterior lobe. The pineal gland is prominent and intensely enhancing.

There is opacification of the right mastoid air cells and middle ear cavity by low T1 and high T2 signals with post-contrast enhancement. This could represent otomastoiditis versus a LCH lesion.

No other significnat abnormaity noted.

Case Discussion

A case known to have signs of central diabetes insipidus, showing loss of the normal T1 high signal of the posterior hypophysis. The association of right frontal, left posterior parietal as well as the clival and anterior inferior frontal calvarial lesions in such age, highly suggests Langerhans cell histiocytosis (LCH) as the etiology with lymphocytic hypophysitis in the given clinical scenario.

The bilateral globus pallidus signal alteration may represent sequel to electrolyte imbalance rather than LCH lesions.

The less likely differential diagnosis of bony lytic lesions would be metastatic deposits (e.g. from a neuroblastoma) in the absence of a known primary tumor.

The images are courtesy of Dr. Ahmad Yousry, MD.

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Case information

rID: 78482
Published: 4th Jun 2020
Last edited: 26th Nov 2020
Inclusion in quiz mode: Included

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