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Neurosarcoidosis

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Acquired hypogonadotropic hypogonadism.

Patient Data

Age: 30 years
Gender: Male

MRI pituitary and brain

mri

MRI of the pituitary and brain demonstrates abnormal contrast enhancement within the hypophyseal stalk, tuber cinereum and midline portion of hypothalamus as well as the subependymal portions of the third ventricle, particularly on the left. Contrast enhancement of soft tissue within the third ventricle at the level of the foramina of Monro appears more prolific than expected, suggesting the process may have also extended to this region with nodular enhancement also seen along veins running along the posterior aspect of the third ventricle. Faint parenchymal enhancement is also noted, particularly in the right parietal lobe.  The delayed sagittal images appear to show more vivid contrast enhancement of the capsule of the pituitary.

Annotated images

Annotated image

Abnormal contrast enhancement of the:

  • hypophyseal stalk, tuber cinereum and midline portion of hypothalamus as well as the subependymal portions of the third ventricle (blue arrows)
  • nodular enhancement also seen along veins running along the posterior aspect of the third ventricle (red arrows) 
  • faint parenchymal enhancement particularly in the right parietal lobe (yellow arrows)
  • soft tissue within the third ventricle at the level of the foramina of Monro (green arrow)

CT chest

ct

There are multiple enlarged mediastinal, hilar and axillary nodes. The largest subcarinal node measures 33.5 by 21.5 x 33.5 mm. The largest right paratracheal node measures 19 mm in short axis diameter and the largest left hilar node measures 17 mm in short axis diameter. 

Apart from minor dependent changes and a band in the right middle lobe, lungs and pleural cavities are clear with no evidence of a pulmonary infiltrate.

This patient went on to have a supraclavicular lymph node core biopsy.

Histology

MICROSCOPIC DESCRIPTION: The sections show cores of lymphoid parenchyma which are replaced by well formed non-necrotizing granulomas. The granulomas are composed of epithelioid histiocytes and scattered giant cells. Focal calcification is also noted. There is no evidence of a primary lymphoid neoplasm, malignancy, or organisms (including on ZN or FITE staining). 

CONCLUSION: Non-necrotizing granulomatous lymphadenitis. These findings are consistent with sarcoidosis. 

Case Discussion

The case demonstrates typical features of neurosarcoidosis, which are often subtle and require good quality contrast images to identify. 

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