by Kimberly McGhee
Illustration by Emma Vought
The pineal gland, a fingertip-sized structure located deep in the center of the brain, is found in almost all vertebrates, and yet its function, if any, remains a mystery. Philosophers and mystics have sometimes magnified its importance, while physicians have tended to underestimate it. Seventeenth-century philosopher René Descartes thought it to be the seat of the soul, while nineteenth-century mystic Madame Blavatsky believed it to be an atrophied third eye. In contrast, medical science has long dismissed it as unimportant — a vestigial gland of little significance, the neurological counterpart of the appendix. For generations, neurosurgeons have been taught to discount imaging evidence of a pineal cyst as inconsequential, in part because resection of the gland in cases of pineal tumor has led to no functional loss.
This has left little clinical recourse to patients with pineal cysts who are experiencing symptoms such as headaches, double vision or nausea for which there is no other neurological explanation. Some patients are referred for psychiatric evaluation with the suspicion that the symptoms are psychosomatic. Others are left to seek relief for their symptoms one by one by seeing a variety of specialists.
Like most other neurosurgeons, Sunil J. Patel, M.D., chair of the Department of Neurosurgery, had been taught to dismiss pineal cysts as clinically inconsequential. Then, five years ago, a patient’s story and a resident’s question led him to re-examine that assumption. A patient presented with imaging evidence of a pineal cyst and a set of neurological symptoms that caused her to leave a very successful career and become virtually house-bound, unable to care for her children. Her symptoms were not consistent with a pineal tumor, and no other neurologic or metabolic cause for them could be found.
Having rounded on the patient, Patel was ready to hand down to a new generation of providers the long-held assumption that pineal cysts are clinically insignificant, when a resident raised the question whether it wasn’t possible, in the absence of all other explanations, that the pineal cyst was causing the patient’s problems. “It takes a young mind to question you,” said Patel. “It was a young resident who asked ‘Why not try removing the cyst/gland? How do you know that’s not causing her symptoms?’”
For the first time, after discussion with the patient, Patel opted to excise the cyst. The results shocked him. When the patient came in for a follow-up visit several weeks later, her symptoms had vanished. What’s more, the pathology report revealed that the excised mass was not a cyst at all but rather a benign tumor known as a pineocytoma.
Patel has since identified a constellation of symptoms — headaches, insomnia, episodic cognitive deficits such as speech or vision difficulties or lapses in short-term memory, and, rarely, numbness in limbs and problems with balance — that are associated with pineocytomas and that resolve upon their excision. He has also come to recognize certain changes on MRI that are indicative of pineocytomas. He highly suspects a pineocytoma when both characteristic symptoms and MRI changes are present. If the symptoms are interfering with a patient’s quality of life and all other causes have been ruled out, Patel recommends surgery to remove the suspected pineal lesion.
Patel, who is one of only a handful of surgeons in the world who resects pineal cysts, has now performed more than 40 of these surgeries. In most cases, the pineal cyst has turned out to be, as expected, a pineocytoma. Ninety percent of these patients have achieved complete symptom resolution, and all achieve at least partial resolution. In patients with comorbid conditions, such as migraine, the symptoms associated with the pineocytoma resolve, but those that are migraine-specific, for example, do not.
What does all of this mean for physicians? First, it’s worth taking a second look at MRI findings of a pineal cyst, because it could in fact be a pineocytoma. Even patients with small cysts who are not experiencing symptoms should undergo monitoring with MRI every year or so in case it turns out to be a cytoma and starts to grow. Second, patients already experiencing some of the telltale symptoms of the syndrome should be referred to a neurosurgeon with experience in intracranial masses for evaluation, because surgery could provide them complete or partial relief.
“Not all pineal gland cysts are asymptomatic and not all of them are nonneoplastic,” said Patel. “The majority of the symptomatic pineal cysts have turned out to be benign tumors, and removing them does provide relief of symptoms.”
Take the CME test for the article now (maximum of .50 AMA PRA Category 1 Credits™.)
Date of Release:
April 10, 2017
Date of Expiration:
April 10, 2019
You can complete the steps necessary to receive your AMA PRA Category 1 Credit(s)™ by visiting MUSC Health’s Physician Portal at MUSChealth.org/progressnotescme.
Physicians: The Medical University of South Carolina designates this enduring material for a maximum of .50 AMA PRA Category 1 Credits™. Physicians should claim only credit commensurate with the extent of their participation in the activity.
Nursing Credit: Most states accept CMEs that apply to a specific nursing specialty as nursing continuing education. Please check with your respective State Board of Nursing to ascertain the equivalent number of contact hours offered for .50 AMA PRA Category 1 Credits™.
All Participants: The Medical University of South Carolina will award .05 CEU (.50 contact hour) for reading the article and passing the posttest. (1 contact hour
equals .1 CEU)
The Medical University of South Carolina is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.
Disclosure Statement: In accordance with the ACCME Essentials and Standards, anyone involved in planning or presenting this educational activity is required to disclose any relevant financial relationships with commercial interests in the healthcare industry. Authors who incorporate information about off-label or investigational use of drugs or devices will be asked to disclose that information at the beginning
of the article.