In transnasal surgery (TNS), a surgeon goes through the nose using an endoscope to remove a pituitary tumor. The approach is the first-choice treatment for children with Cushing’s disease due to ACTH-secreting adenomas — or tumors — in the pituitary gland.
In Memory of those we have lost. Janice died Tuesday, September 4, 2001. The very first Cushing's Awareness Ribbons were made for, and worn at, Janice's funeral.
Ryan passed away peacefully and is free from illness and pain....Also mourning his loss are aunts, uncles, cousins, many life-long friends and friends through Cushing's Disease Awareness.
Can someone please help me? Over the past few years I have gained over 40 lbs, suffer from extreme fatigue, muscle cramps, headaches, just to name a few. I also have a hump between my shoulders. I have gone to see my primary care physician and asked about Cushing’s syndrome and she said that there’s just no way I could have this because it is so rare. […]
A time will be arranged for your interview. On that day, please call the guest call-in number about 5-10 minutes before the interview is scheduled. You can chat informally with the MaryO before the chat begins...
A plasma adrenocorticotropic hormone suppression test performed shortly after surgical adenomectomy may accurately predict both short- and long-term remission of Cushing’s disease
Functional remission did not occur in most patients with Cushing syndrome who were considered to be in biochemical and clinical remission, according to a study published in Endocrine. This was evidenced by their quality of life, which remained impaired in all domains.
This observational, longitudinal study evaluated the effects of the 11β -hydroxylase inhibitor metyrapone on adult patients with Cushing syndrome. Urinary-free cortisol and late-night salivary cortisol levels were evaluated in 31 patients who were already treated with metyrapone to monitor cortisol normalization and rhythm.
In healthy individuals, the levels of cortisol — a steroid hormone secreted by the adrenal glands — go through changes over a 24-hour period, with the lowest levels normally detected at night. But this circadian rhythm is disrupted in certain diseases such as Cushing’s syndrome, where night cortisol levels can be used as a diagnostic tool. Among the tests th […]
Alexis, can you tell me if your workup has revealed any answers? Someone I love is experiencing the same symptoms. With weight gain, rounded face, resting HR high, heat intolerance, nausea, vomiting, fainting..
I have very complex issues also with the pcos and all how it basically attached to new system. It’s very very hard to treat for the many unknowns. I have found odd dr whom thinks outside box typically works best. ....
Danielle had suddenly gained more than 20kg, found herself losing hair, constantly breaking bones and struggling to sleep. Making matters worse, the young mother became severely depressed and noticed an unusual-looking ‘hump’ on her back.
Surgical removal of tumors in the pituitary gland of the brain remains the gold standard for Cushing’s disease treatment. Transsphenoidal surgery (TSS) usually leads to good remission rates ranging from 68-95%, depending on the location and type of tumor, the neurosurgeon’s expertise, follow-up period, and the definition of remission. Today, TSS consists of […]
The UCSF website in an obituary from Kate Vidinsky reads “He took a particular interest in pituitary disorders, those affecting the pea-sized ‘master gland’ at the base of the brain responsible for controlling the body’s hormone levels. He was a pioneer of transsphenoidal surgery – the endonasal approach for removing pituitary tumors – and performed more than 3,300 of these procedures at UCSF Medical Center.”
The New York Times in an obituary published yesterday described him as “a pioneering and virtuosic San Francisco neurosurgeon”.
Endocrine Today, October 2017 Brooke Swearingen, MD; Stephanie L. Lee, MD, PhD, ECNU
A 63-year-old man was referred to the Massachusetts General Hospital Neuroendocrine & Pituitary Tumor Clinical Center for management of a pituitary macroadenoma. He experienced increasingly severe retro-orbital headaches in the past year. He reported no double vision, fatigue, orthostatic dizziness, change in beard growth or reduction in libido. An outside head CT scan showed an enlarged pituitary gland.
Imaging and laboratory tests
A pituitary MRI with magnified pituitary slices and gadolinium contrast was ordered. A well-circumscribed “snowman-shaped” sellar mass was identified, measuring 2.6 cm x 2 cm x 1.8 cm (anteroposterior x transverse x craniocaudal) with suprasellar extension (Figure 1). The lesion was heterogeneous on T1-weighted scans after enhancement with IV gadolinium contrast. An area of hypointensity in the superior margin was consistent with a small area of cystic or hemorrhagic degeneration.
Although the mass did not extend laterally into the cavernous sinus, the sellar mass extended upward into the suprasellar cistern through a hole in the dural, the diaphragma sellae, to compress the optic chiasm. The restriction of adenoma growth by the diaphragma sellae results in the snowman shape of the macroadenoma. The optic chiasm and infundibulum (pituitary stalk) could not be identified on coronal or sagittal images (Figure 1). Visual field on confrontation suggested lateral field deficits (bilateral lateral hemianopsia) that were confirmed on formal Goldmann kinetic perimetry visual fields.
Figure 1. Preoperative MRI scan. A large “snowman-shaped” pituitary adenoma (green arrow) has heterogeneous enhancement after gadolinium contrast administration. A small hypodense area in the adenoma likely represented hemorrhage/cystic degeneration (yellow arrow). The tumor does not surround the carotid siphon, an S-shaped portion of the internal carotid artery (red arrows) within the cavernous sinus located laterally from the sella turcica where the pituitary gland resides. (A) Coronal image. (B) Sagittal image. Abbreviation: SS = spenoid sinus.
Source: Stephanie L. Lee, MD, PhD, ECNU. Reprinted with permission.
Initial hormonal evaluation was normal and included morning adrenocorticotropic hormone 18 pg/mL, cortisol 13.64 µg/dL, thyroid-stimulating hormone 2.14 uIU/mL, free thyroxine 1.2 ng/dL and prolactin 12.6 ng/mL. The patient’s morning testosterone level was normal at 324 ng/dL, with follicle-stimulating hormone 2.4 mIU/mL and luteinizing hormone 1.6 mIU/mL. His insulin-like growth factor I level was normal at 124 ng/mL.
Tumor resection
The patient was treated preoperatively with stress-dose hydrocortisone 50 mg. He then underwent transsphenoidal pituitary tumor resection. After the surgeon believed there was an adequate excision of the tumor, the extent of tumor resection was confirmed by an intraoperative MRI (Figure 2 on page 8).
Figure 2. Intraoperative MRI scan. The large macroadenoma is not seen after transsphenoidal surgery. The optic chiasm (yellow arrow) can be seen after removal of the tumor. (A) Coronal image. (B) Sagittal image. Abbreviation: SS = spenoid sinus.
The operation was concluded after the imaging confirmed the complete resection of the pituitary adenoma. The patient’s postoperative course was uneventful. Imaging 4 weeks after the resection confirmed complete resection of the suprasellar mass with residual enhancement of the resection bed and sphenoid sinuses (Figure 3 on page 8). The postoperative MRI revealed a normal optical chiasm and a downward tending of the infundibulum to the residual pituitary gland located inferiorly along the sella turcica (pituitary fossa) of the sphenoid bone. Pathology confirmed a pituitary adenoma. His anterior and posterior pituitary function were normal 6 weeks postoperatively, and his visual field deficit improved.
Intraoperative MRI
Imaging like that used in this case occurs in a specially designed operating room that allows MRI scans during surgery without moving the patient from the surgical table. The MRI is kept in a shielded enclosure during the procedure and then moved along a track into the operating room for imaging. Clinical indications for the use of intraoperative MRI in neurosurgery include resection of pituitary macroadenomas. In the past, these tumors underwent transsphenoidal resection, and the postoperative MRI was performed after 1 or more days after the procedure to check for complete removal. If residual tumor was found, the patients underwent watchful waiting, external radiation or repeat surgery.
The strategic advantage of an intraoperative MRI is that the imaging is performed during the operative procedure, and if there is any residual tumor, surgery can be resumed after the MRI is moved back into the shielded enclosure.
Figure 3. Four-week postoperative MRI scan. The large macroadenoma is not seen after the transsphenoidal survey. The optic chiasm and infundibulum (pituitary stalk) can be seen after resection of the tumor. The pituitary stalk is deviated to the left of the sella where the residual normal thyroid is locate along the sella turcica. The floor of the sella enhances with gadolinium infusion after surgery due to postoperative inflammation. (A) Coronal image. (B) Sagittal image. Abbreviation: SS = spenoid sinus.
It has been reported that the use of intraoperative MRI does not increase complication rates compared with conventional transsphenoidal surgery. Reports on the improvement of gross tumor resection using intraoperative MRI are variable, perhaps due to the expertise of the surgeon. Several reports suggest the use of intraoperative MRI allowed additional resection of noninvasive macroadenomas in 67% to 83% of the patients with a gross tumor resection. These results suggest that a substantial volume reduction and increased gross tumor resection of pituitary macroadenomas occurs with the use of intraoperative MRI compared with standard surgery. One study demonstrated that the gross tumor resection rates of invasive tumors was also improved with the use of intraoperative MRI compared with usual preoperative imaging and surgery (25% vs. 7%).
The use of intraoperative MRI, especially with transsphenoidal reoperations for invasive and noninvasive pituitary macroadenomas, leads to significantly higher “gross tumor resection” rates. This method prevents additional operations or treatment, such as radiation, because it reduces the number of patients with residual adenoma after surgery. This technology is usually found in specialized tertiary care hospitals but should be considered for reoperation for large pituitary macroadenomas or initial operation for large invasive pituitary macroadenomas.
References:
Boellis A, et al. J Magn Reson Imaging. 2014;doi:10.1002/jmri.24414.
Fomekong E, et al. Clin Neurol Neurosurg. 2014;doi:10.1016/j.clineuro.2014.09.001.
Tandon V, et al. J Clin Neurosci. 2017;doi:10.1016/j.jocn.2016.10.044.
For more information:
Stephanie L. Lee, MD, PhD, ECNU, is an Endocrine Today Editorial Board Member. She is associate professor of medicine and director of thyroid health in the Section of Endocrinology, Diabetes and Nutrition at Boston Medical Center. She can be reached at Boston Medical Center, 88 E. Newton St., Boston, MA 02118; email: stephanie.lee@bmc.org.
Brooke Swearingen, MD, is associate professor of surgery (neurosurgery) at Harvard Medical School and co-director of the neurological ICU at Massachusetts General Hospital. He can be reached at bswearingen@partners.org.
Disclosures: Lee and Swearingen report no relevant financial disclosures.
Ed started as Senior Staff Fellow in the Surgical Neurology Branch at the NIH (1981). After 5 years, Ed would become the Chief of the Surgical Neurology Branch. He would stay on as Branch Chief and lead the neurosurgical effort at the NIH for the next 21 years. During his tenure, he developed clinical, research, and training programs in epilepsy, congenital malformations, syringomyelia, nervous system neoplasia, drug delivery, and vascular malformations. The strength of these programs was his leadership and their multidisciplinary nature, which incorporated physicians and scientists across the basic, translational, and clinical arenas. Research investigation was always targeted at defined clinical problems. Under his direction, these programs shaped understanding of the studied neurological disorders, as well as improving patient care.
Please let us know your experiences with this new program!
Hallmark Health and Tufts Medical Center have established a new neurosurgery program at Melrose-Wakefield Hospital to bring advanced care and services to the community. Fellowship-trained neurosurgeon Mina G. Safain, MD, has been jointly hired by Hallmark Health and Tufts Medical Center to lead the new program. He will provide care at both Melrose-Wakefield Hospital and Tufts Medical Center.
The neurosurgery program is an example of clinical integration of services between Hallmark Health and Tufts Medical Center since Hallmark Health joined Wellforce as a third founding member this past January. At that time, leaders from the organizations discussed finding ways to bring specialized care traditionally performed at academic medical centers into the community hospital setting for the benefit and convenience of patients.
“Offering neurosurgery provides a service for our patients that few community hospitals can offer,” said Steven Sbardella, MD, chief medical officer at Hallmark Health. “Our clinical relationship with Tufts Medical Center enables us to bring more highly specialized care options to our patients.”
“We are extremely excited to work with the physicians at Melrose-Wakefield Hospital and look forward to increasing the services available to care for patients with neurologic diseases,” said Carl Heilman, MD, neurosurgeon-in-chief at Tufts Medical Center. “Dr. Safain is an exceptionally talented and compassionate neurosurgeon and the perfect person to spearhead the launch of this new program.”
Dr. Safain’s clinical interests include all diseases affecting the brain, spine and peripheral nervous system. He has specific interests in minimal access procedures for degenerative, infectious and oncologic spine disorders, as well as minimally invasive treatments for brain tumors, including neuro-endoscopy.
“The opportunity to practice in the community is very important to me,” said Dr. Safain. “I look forward to working with the esteemed staff and providers at Melrose-Wakefield Hospital and Lawrence Memorial Hospital and treating the patients in the surrounding communities.”
“Welcoming such a highly-respected neurosurgeon as Mina Safain to our team is a tremendous benefit for our communities and patients across our system including Lawrence Memorial Hospital in Medford and Melrose-Wakefield Hospital,” said Dr. Sbardella.
Dr. Safain, together with Ran Ku, PA, a neurosurgery physician assistant with more than 12 years of experience, will provide neurosurgery coverage and expertise five days a week.
Dr. Safain received his medical degree from Yale University School of Medicine. He completed his neurosurgery residency at Tufts Medical Center serving as chief resident during his final year. Dr. Safain also completed fellowship training in pituitary and neuro-endoscopic surgery at Brigham and Women’s Hospital.
Dr. Safain has published and presented nationally on a range of topics related to neurosurgical diseases and minimally invasive treatments for brain tumors.
The Cushing/Whitney Medical Library is pleased to announce the completion of a grant funded to catalog 2,600 glass plate negatives from the Cushing Brain Tumor Registry. The grant proposal, “Rethinking Early Neurosurgery: The Harvey Cushing Collection,” was funded through a National Network of Libraries of Medicine-New England Region Knowledge/Data Management Award. From mid-February through April 30th 2017, a team of graduate and undergraduate students carefully inputted information on over 3,000 glass plate negatives into the Cushing Center database, exceeding the estimated amount in the grant. The negatives depict Dr. Harvey Cushing’s patients, including histology.
Harvey Cushing, the pioneer and father of neurosurgery, was born on April 8, 1869 in Cleveland, Ohio. He graduated from Yale University in 1891, studied medicine at Harvard Medical School and received his medical degree in 1895. In 1896, he moved to Johns Hopkins Hospital where he trained to become a surgeon under the watchful eye of William S. Halstead, the father of American surgery. By 1899 Cushing became interested in surgery of the nervous system and began his career in neurosurgery. During his tenure at Johns Hopkins, there were countless discoveries in the field of neuroscience.
In 1913, Cushing relocated to Harvard as the surgeon-in-chief at the new Peter Bent Brigham Hospital. Cushing continued to operate on several hundred patients a year with remarkable results. In addition he was relentless in his recording of patient histories and continued his careful attention to the details and documentation of each surgery.
In 1932 Harvey Cushing retired and in 1933 he agreed to join the staff at Yale University, his alma mater, as the Sterling Professor of Medicine in Neurology. Cushing died in 1939.
The negatives are undergoing rehousing and digitization, and will be made available for research through the Cushing Center database, which brings multiple parts of Harvey Cushing’s work together in one place. The database, still in development, will allow researchers to explore Cushing’s medical work and patients. Please contact Terry Dagradi, Cushing Center Coordinator, for details.
