Death may result with development of a locally aggressive pituitary tumor. However, does not commonly occur with pituitary diseases. In the rare case, ACTH-secreting tumors can become malignant. Morbidity from the disease can occur due to pituitary tissue compression or replacement, and compression of structures that surround the pituitary fossa. The tumor can also compress the optic apparatus, disturb cerebrospinal fluid flow, meningitis, and testicular enlargement in rare cases. Research
Through multiple advancements within the medical field, caregivers have been able to stray away from utilizing bilateral adrenalectomy as the treatment for Cushings disease. This has decreased the risk of patients presenting with Nelsons syndrome. Alternative treatments for Nelsons syndrome have been discovered. The most utilized technique for Nelsons syndrome has been transsphenoidal surgery. In addition, pharmacotherapy, radiotherapy, and radiosurgery have been utilized accompanying a surgical procedure. Pharmacological drugs can also be given accompanying a transsphenoidal surgery including the following: pasireotide, temozolomide and octreotide. Within rats/mice, rosiglitazone has been an effective measure, however this has not been discovered in humans yet. References


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There is a reported association between CNL and multiple myeloma, so the bone marrow biopsy may show evidence of a plasma cell dyscrasia with increased numbers of atypical plasma cells. Spleen
Splenic infiltrates are typically found only in the red pulp. Liver
Hepatic infiltrates can be found in either the sinusoids, portal triad regions, or both. Immunophenotype
No distinct immunophenotype abnormality for CNL has been described. See OHSU 2013 findings of gene CSF3R, mutation p. T6181

Epidemiology
This is a rare disease, with less than 100 cases reported. Of these cases, an equal male:female ratio was observed,
with cases typically seen in older adults. References


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