
Neurosurgeon Dr. Donato R. Pacione, in collaboration with a multidisciplinary team of experts, deciphered a patients severe symptoms to identifyand safely removea rare pituitary tumor.
Photo: 做厙TV Langone Staff
When a patient presented with a series of increasingly severe idiopathic symptoms, a multidisciplinary team of endocrinologists, neurosurgeons, and radiologists pursued an elusive diagnosis to identify her conditions root cause: a rare pituitary tumor. Supported by advanced diagnostics and intraoperative imaging, the team collaborated to plan a minimally invasive surgical resectionand halt the patients decline.
Accurate Diagnosis Requires Extensive Diagnostic Sleuthing
The 38-year-old patient had a complex history, with a range of comorbidities including prior stroke, hypertension, cardiomyopathy, and treatment-resistant type 2 diabetes mellitus with hyperglycemia. Despite several hospitalizations at other centers, her symptoms continued to elude a definitive diagnosis. She consulted with a multidisciplinary team at 做厙TV Langone, including endocrinologist Eliud Sifonte, MD, that suspected and confirmed hypercortisolism and a right-sided adrenal adenoma thought to be benign and unrelated to the hypercortisolism. Indications pointed to Cushings syndrome. However, the source of the hormonal irregularity remained unconfirmed, and the patients symptoms persisted, threatening her life and demanding swift diagnosis.
With a case like this, its critical to really nail down the diagnosis and determine what is essentially giving this 38-year-old patient the clinical profile of a sick 75-year-old, says Nidhi Agrawal, MD, clinical assistant professor in the and director of pituitary diseases at the Pituitary Center. And that diagnosis necessitates far more than a single blood test in isolationyou need a combination of clinical suspicion and the right advanced tools to confirm it.
With a case like this, its critical to really nail down the diagnosis and determine what is essentially giving this 38-year-old patient the clinical profile of a sick 75-year-old.Nidhi Agrawal, MD
In this case, clinical suspicion focused on the patients pituitary gland. Physicians used a combination of analytical tools including the highly sensitive adrenocorticotropic hormone (ACTH) assay, which is offered at few centers outside 做厙TV Langone. This assay is critical to diagnostic specificity.
With diagnostic indicators pointing almost certainly toward pituitary involvement, Donato R. Pacione, MD, assistant professor in the , collaborated with Eytan Raz, MD, PhD, assistant professor in the , to perform inferior petrosal sinus sampling, which would confirm the diagnosis. Response from the pituitary to microcatheter-injected hormone confirmed it was the source of the patients symptoms, and imaging subsequently revealed a pituitary microadenoma, a rare type of pituitary tumor.
Resecting the Root Cause, Carefully
Diagnosis in hand, the surgical planning began. After the long road to diagnosis, surgery should be the simpler partexcept that youre working with something thats 6 millimeters in size, and if you dont resect every trace of the tumor, it still secretes hormone, notes Dr. Pacione.
Understanding the historically high recurrence rate of such pituitary tumors, Dr. Pacione recommended an endoscopic endonasal approach for tumor resection, a minimally invasive approach that would enable precise navigation. Dr. Pacione worked closely with Elcin Zan, MD, assistant professor in the Department of Radiology, to develop a detailed anatomical map that would inform his surgical target.
During the procedure, the approach was performed by Seth M. Lieberman, MD, assistant professor in the . The gland was identified and elevated to reveal inferiorly the tumor tissue, which Dr. Pacione dissected and removed. He also observed two other firm nodules anterior to the gland, in an area Dr. Zan had identified as a potential site of additional abnormal tissue, and removed them. When an intraoperative MRI demonstrated no evidence of residual tumor, the patient returned to the operating room (OR), where Dr. Lieberman performed the closure, and then moved to recovery.
Along with the careful multidisciplinary planning, advanced intraoperative imaging was critical to confirming full resection of the tumor. The intraoperative MRI, stereotactic navigation, and an advanced intraoperative histology systemwhich quickly and accurately identifies residual tumor while sparing healthy tissueworked in tandem in a surgery that demanded extreme precision to achieve a positive outcome.
Real-time, intraoperative feedback is the game changerit enables us to both make faster surgical decisions and leave with a higher degree of confidence that the tumor is completely out, observes Dr. Pacione.
Expertise and Access Change Clinical Trajectory
Within a day of surgery, the patients hormone level normalized, and she lost 30 pounds in 4 weeks. For this patient, multidisciplinary coordination of care, access to leading-edge diagnostic tools and imaging, and a collaborative pursuit of differential diagnosis reversed a precipitous decline in health toward a better outcome.
This patient was already in heart failure and probably would have died if we hadnt pinpointed and corrected her root issue, notes Dr. Pacione. Its the difference between care that simply solves whatever is immediately broken on a given day and finding the underlying cause. Thats what changes the course and saves a patient.