Dr. Frederick Lang
Sections
Cranial
About the section
Our neurosurgeons perform more complex surgeries on brain tumor patients than any hospital in the nation. The Cranial section manages all brain tumor types including gliomas (glioblastomas, astrocytomas, oligodendrogliomas), intraventricular tumors, pineal region tumors, thalamic and brainstem region tumors, hemangioblastomas, ependymomas and meningiomas. Additionally, our neurosurgeons treat patients with brain metastases, who require advanced treatment approaches provided through a dedicated Brain Metastasis Clinic. Brain tumor treatment involves a multidisciplinary approach which includes surgery, radiation therapy, chemotherapy and targeted therapy. Our team uses cutting-edge technology, including Gamma Knife Radiosurgery, the iMRI Suite, laser interstitial thermal therapy (LITT), 5-ALA fluorescent technique to maximize tumor resections, specialized brain mapping including awake craniotomy and electrocorticography techniques carefully planned with patient's lifestyle in mind. All these interventions have resulted in superior oncological outcomes and improved survival for the patients. Our surgeons are also actively involved in recruiting patients to clinical trials to provide comprehensive surgical care that fits their specific tumor type and location.
Cranial cases (including skull base) by fiscal year
- 2018: 1,447
- 2019: 1,636
- 2020: 1,275
- 2021: 1,362
- 2022: 1,289
Faculty
Frederick Lang, M.D., FACS
Professor and Chairman
Director, Brain Tumor Center Executive Committee
Surgery for primary and metastatic brain tumors, surgery for insular brain tumors, surgery for tumors in eloquent brain regions, viral therapies for brain tumors
Ian E. McCutcheon, M.D., FRCS(C)
Professor
Surgery of pituitary neoplasms, spinal and brain tumors, neurofibromatosis, Gamma Knife Radiosurgery
Sherise D. Ferguson, M.D.
Associate Professor | Center Medical Director
Surgery for primary and metastatic brain tumors, Gamma Knife Radiosurgery
Christopher A. Alvarez-Breckenridge, M.D., Ph.D.
Assistant Professor
Surgery for primary and metastatic brain tumors, immunotherapy, Gamma Knife Radiosurgery
Robert North, M.D., Ph.D.
Assistant Professor
Surgery for primary and metastatic brain tumors, Gamma Knife Radiosurgery, laser interstitial thermal therapy
Jeffrey S. Weinberg, M.D.
Professor and Deputy Chairman
Vice Chair of Clinical Operations
Primary and metastatic brain tumors, pediatric brain tumors, computer-assisted surgery for biopsy and tumor resection, surgery for tumors in eloquent brain regions, Gamma Knife Radiosurgery
Sujit Prabhu, M.D., FRCS (Ed)
Professor
Surgery for primary and metastatic brain tumors, surgery for tumors in eloquent brain regions, brain mapping, laser interstitial thermal therapy, Gamma Knife Radiosurgery
Betty Kim, M.D., Ph.D.
Professor
Primary and metastatic brain tumors, nanomedicine and immunotherapy for brain tumors, Gamma Knife Radiosurgery
Chibawanye I. Ene, M.D., Ph.D.
Assistant Professor
Surgery for primary and metastatic brain tumors in eloquent brain regions, Laser interstitial thermal therapy, Gamma Knife Radiosurgery, viral and macrophage therapies for brain tumors.
Featured publication
Frederick Lang, M.D.
Al-Holou WN, Suki D, Hodges TR, Everson RG, Freeman J, Ferguson SD, McCutcheon IE, Prabhu SS, Weinberg JS, Sawaya R, Lang FF. Circumferential sulcus-guided resection technique for improved outcomes of low-grade gliomas. J Neurosurg. 2022 Jan 7:1-11. doi: 10.3171/2021.9.JNS21718. Epub ahead of print. PMID: 34996044.
Low grade gliomas (LGGs) can infiltrate the functional brain, which make resection of these tumors technically challenging. While pre-operative and intraoperative imaging and awake mapping techniques have facilitated improved resection, the ideal surgical technique for resecting LGGs is not defined.
While most neurosurgeons use an intralesional or piecemeal approach (PMR) to resect LGGs, our neurosurgeons at MD Anderson have increasingly used a circumferential or sulcus-guided resection (SGR) technique. In PMR, the resection remains within the tumor, whereas in the SGR, the edges of the tumor are defined using the surrounding sulci as a guide and the tumor is resected circumferentially at its interface with the brain, without entering the core of the tumor. In this article, a detailed description of SGR is provided. It also includes the results of a study that evaluated the extent of resection and associated surgical complications with SGR and PMR for LGGs in 519 patients, with 208 in the SGR group and 311 in the PMR group. Both univariate and multivariate analysis demonstrated that SGR resulted in greater extent of resection compared with PMR, and that smaller tumor volumes were associated with a greater extent of resection.
The results of this study demonstrated that SGR was at least as safe as PMR with no significant differences between the groups in terms of neurological complications. While a larger study with more extensive analysis is needed to evaluate survival benefit after SGR, this study supports the idea that SGR should be considered for LGGs, including in eloquent brain regions, because it is associated with a higher rate of complete resection.
Related Media
Featured Case
Awake Craniotomy of a Low Grade Glioma in the Motor Cortex
This patient presented with cramping of the right hand and a generalized tonic-clonic seizure. The patient also reported decreased appetite, fatigue, intermittent visual disturbance in the right eye, occasional headaches, difficulty with concentration and loss of balance. An MRI of the brain demonstrated a mass in the posterior left frontal lobe, in the motor cortex, just medial to the hand-knob which was suspected to be a low-grade glioma.
The patient’s work involved using his hands on a key-board and was right side dominant. It was very important that we preserve function of his dominant right hand including small muscles of his hand. He plays the drums and was happy to use a drum kit during surgery to test his hand function and his motor skills.
The patient consented to awake craniotomy and tumor resection, and prior to surgery, the patient had functional MRI and navigated transcranial magnetic stimulation for motor mapping and augmented reality (AR) study to further characterize functional activity.
On the surface of the brain, we placed a High-definition (HD) grid to characterize the motor and sensory areas and also identify parts of the motor gyrus (hand-knob) which controls fine movements of his right hand. Additionally, the patient was able to use his hands and wrists on a drum pad to generate a rhythm. This was used primarily to test fine motor coordination in his hand.
Using additional intraoperative technologies including intraoperative navigation and ultrasound, the tumor margins were identified. Tumor resection was then carried out in a piecemeal manner using a sulcal approach with the aid of an operating microscope, resulting in significant tumor debulking. Subsequent analysis of the tumor revealed that it was a WHO grade 2 astrocytoma. During the resection, the patient intermittently played the drums and continued to execute finger movements which allowed the surgeon to complete a safe and complete resection of the tumor.
The patient was ambulatory and discharged 3 days after the procedure. The patient was back to his normal function when seen 4 weeks after surgery. He was able to use the keyboard and mouse of his computer in a regular manner and continues to enjoy playing drums.
BrainSuite
Preoperative and Postoperative Brain MRI
An MRI of the brain demonstrated a mass in the posterior left frontal lobe, in the motor cortex, just medial to the hand-knob. Resection resulted in considerable tumor debulking.
Augmented Reality View of Tumor (left) and nTMS generated CST (right)
Augmented reality study of the brain was performed to characterize functional activity. The augmented reality view of the tumor (in blue) is shown with overlaid nTMS points. Functional MRI and navigated transcranial magnetic stimulation (nTMS) were performed for motor mapping. The nTMS generated corticospinal tract (CST) is shown, the tumor is visible between the fiber tracts.
High Definition Grid
A High-definition grid was applied to the surface of the brain to characterize the motor and sensory areas, and the parts of the motor gyrus that control fine movements of the patient's right hand.
Intraoperative images before (left) and after (right) resection
Cranial Cases Fiscal Year 2022
1,289
Total Cases
392
Stereotactical Radiosurgery Cases
Skull Base
About the section
Our skull base team is widely recognized for its extensive experience and surgical expertise. Our surgeons have been at the forefront of surgical advancements in open and endoscopic skull base surgery. For every patient, the team considers whether an open, endoscopic or combination approach will be most effective based on the specific tumor type and location. Providing expertly performed surgery, often as part of a carefully constructed, personalized multimodal care plan, is critical in optimizing our patients' outcomes.
The skull base section manages complex tumors of the skull base, including meningiomas, acoustic neuromas (also known as vestibular schwannomas), trigeminal schwannomas, jugular schwannomas, paragangliomas, glomus tumors, craniopharyngiomas, pituitary tumors, and several types of malignant skull base tumors including chordomas, chondrosarcomas, and sinonasal cancers. The most common skull base tumor locations include the intratemporal fossa, jugular foramen, clivus, foramen magnum, sella turcica, anterior cranial fossa, petrous apex, middle cranial fossa, temporal bone, posterior fossa, sinonasal tract, nasopharynx, orbit, parapharyngeal space, cavernous sinus, cerebellopontine angle and cranial nerves.
Our team utilizes several advanced technologies, including Gamma Knife Radiosurgery, virtual reality surgical planning, proton therapy, a hybrid operating room with intraoperative angiography and intraoperative MRI, endoscopic surgical suites, exoscopic technology and molecular diagnostics.
Skull base tumor cases by fiscal year
- 2018: 187
- 2019: 208
- 2020: 174
- 2021: 195
- 2022: 184
Faculty
Franco DeMonte, M.D., FRCS(C), FACS
Professor
Surgery of benign and malignant skull base tumors, meningioma, acoustic neuroma, Gamma Knife Radiosurgery
Shaan
Raza, M.D.
Associate Professor
Open and endoscopic skull base surgery, surgery of benign and
malignant skull base tumors, surgery of pituitary neoplasms
Featured publication
Franco DeMonte, M.D.
Shaan Raza, M.D.
Mehta GU, Passer JZ, Raza SM, Kim BYS, Su SY, Kupferman ME, Hanna EY, DeMonte F.
J Neurosurg. 2021 Oct 8:1-9. doi: 10.3171/2021.5.JNS21772. Epub ahead of print. PMID: 34624857
Tumors that arise in the nasal cavity and the paranasal sinuses often involve the antior skull base and may extend intracranially. These malignancies have been managed using a variety of surgical approaches, with a more recent shift towards the incorporation of endoscopic surgical techniques.
The long-term clinical outcomes and risks associated with surgical management of sionasal tumors extending to the anterior skull base have not been extensively studied. In this study led by Dr. DeMonte, the authors evaluated the clinical outcomes in 225 patients who were treated at MD Anderson over a period of 28 years for these types of tumors.
To account for differences in the surgical techniquest and adjuvant multimodal therapies used over time, patients were stratified by the time period (decade) during which they were treated. Interestingly, while the frequency of higher-stage disease at the time of surgery increased over time, median overall survival remained stable. The rate of major complications significantly decreased from the first to the third decade studied. The use of a lumbar drain was a significant predictor of complications.
Overall, this study found that the surgical management of sinonasal malignancies with anterior skull base involvement was safe, and that the safety of surgery has improved over time most likely due to the increased use of endoscopic surgical techniques and the avoidance of the routine use of lumbar spinal drains.
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Surveillance and Survivorship Clinic
The Neurosurgery Surveillance and Survivorship Clinic is a unique program that follows and provides support for patients who have benign tumors of the skull base. These patients have completed surgery or other treatment and have had stable disease for 2 years post-treatment. These patients are long-term survivors, who typically do not succumb to their disease, but may have long-lasting side effects which may appear months or even years after treatment. These side effects may include problems or changes with thinking, memory, concentration, behavior, speech, swallowing, vision, hearing, gait imbalance, fatigue, mood changes, hormone changes, and weight gain or loss. Our APP team members serve as providers for this clinic, evaluating patients using an algorithm for follow-up and maintaining close communication with the attending physicians.
Surveillance and Survivorship Clinic Team
Franco DeMonte, M.D., FRCS(C), FACS
Professor
Surgery of benign and malignant skull base tumors, meningioma, acoustic neuroma, Gamma Knife Radiosurgery
Susan Boutte, APRN, FNP-BC
Manager, advanced practice providers
Supporting: Franco DeMonte, M.D.
Shaan
Raza, M.D.
Associate Professor
Open and endoscopic skull base surgery, surgery of benign and
malignant skull base tumors, surgery of pituitary neoplasms
Cheryl Martin, APN, FNP-C
New Patient APP Navigator &
APP for Skull Base Surveillance Clinic
Featured Case
Olfactory Neuroblastoma
Brief History:
This patient is a 44-year-old, right-handed female without significant past medical history who began experiencing intermittent epistaxis (nosebleeds). Management was with moisturization and the use of a humidifier. She then developed a loss of the sense of smell about 18 months later. Nasal endoscopy at that time identified a sinonasal mass. Biopsy was consistent with olfactory neuroblastoma. A subsequent comprehensive evaluation revealed areas of suspicion in the nodes of the neck and in the lung. Biopsies of these areas were without evidence of tumor. Evaluation of the sinonasal biopsy at MD Anderson agreed with the diagnosis of olfactory neuroblastoma, Hyams grade 2.
MRI of the skull base without and with contrast revealed an avidly enhancing tumor located in the ethmoid sinuses with transcranial extension into the anterior cranial fossa and invasion of the left frontal lobe. Dural enhancement extending over the roof of the left orbit was suspicious for, but not conclusive of, tumor extension. Radiographic staging of this tumor was Kadish C, and PET/CT identified a tumor of high radionucleotide uptake consistent with malignancy.
Procedure Details
With the patient under general anesthesia, a bicoronal incision was made ensuring preservation of a robust vascularized pericranial graft. A bifrontal craniotomy extending inferiorly to the frontonasal suture was elevated. The posterior wall of the frontal sinuses was removed, as was the roof of the ethmoid sinuses bilaterally. The anterior ethmoidal arteries were identified, coagulated and divided.
Under the operating microscope, the dura was incised over both frontal lobes, and the superior sagittal sinus was suture-ligated and divided. The division was brought through the anterior falx cerebri superior to the tumor. Intraoperative frozen section pathology confirmed a tumor-free margin. The tumor invading the underside of the left frontal lobe was then removed. The right olfactory nerve was resected and was free of disease. The intradural aspect of the cribriform plate region was exposed. Incisions were then made circumferentially around the tumor-infiltrated dura. Intraoperative pathology again confirmed tumor-free margins. The bone of the skull base was exposed. Osteotomies were placed into the anterior and posterior ethmoidal sinuses bilaterally and into the sphenoid sinus posteriorly. The posterior ethmoidal arteries were coagulated and divided. A commercially available dural patch was used to repair the defect in the dura. This patch was sewn in, creating a water-tight dural seal.
Using an endoscopic approach, the sinonasal component of the tumor was accessed and removed, having been freed from the brain, dura and bony skull base during the transcranial approach. Multiple tissue margins were assessed, and all were free of disease.
The vascularized pericranial graft was then rotated intracranially to seal off the floor of the anterior cranial fossa. This robust reconstruction was sutured to the bone of the base of skull under the operating microscope. The bone was then returned to its proper position and held in place with titanium plates, and the scalp was repaired.
The patient was monitored in the NPCU for 24 hours and transferred to the floor. She was neurologically intact and made an uneventful recovery. Post-operative MRI obtained within 24 hours after surgery revealed a complete resection of both the intracranial and intra-sinonasal portions of the tumor. Pathology determined the tumor to be a Hyams grade 2 olfactory neuroblastoma. All margins were microscopically determined to be negative for tumor. The patient completed the prescribed postoperative radiation treatments and is without evidence of disease.
Discussion
Olfactory neuroblastoma is one of the family of neuroendocrine tumors that can arise in the nasal cavity and paranasal sinuses. It is a malignant tumor that is classified by its location (Kadish staging system) and by its histopathological features (Hyams grading system).
Lower-grade tumors (Hyams grade 1 or 2) are usually managed by surgical resection and postoperative irradiation. Higher-grade tumors are usually treated with neo-adjuvant chemotherapy. Most patients then go on to surgical removal and postoperative chemoradiation. Outcomes of treatment are good, with five-year disease specific survivals of over 90 percent.
Preoperative MRI
Preoperative PET/CT (left) and Cranio-endoscopic Surgical Approach (right)
Postoperative MRI
Previous Featured Cases
Skull Base Fiscal Year 2022
184
Total Skull Base Cases
17
Stereotactic Radiosurgery Cases
Skull Base: Pituitary Tumor Program
About the Program
We provide comprehensive surgical care for the whole range of neuroendocrine tumors and cysts growing from the pituitary gland and for tumors growing from structures next to the pituitary that can affect it secondarily. These include pituitary adenomas (those that secrete hormones and those that do not), craniopharyngiomas, pituitary carcinomas, metastases to the pituitary from cancers elsewhere in the body, meningiomas, chordomas and Rathke’s cleft cysts. We aim to control and cure these tumors to improve patients’ hormone function, protect their vision, and thus enhance their quality of life safely and with compassion.
Our surgeons have 40 years of experience between them and have operated on more than 2,000 patients with pituitary pathology during their careers. We believe in providing safe, effective and expert care for our patients with neuroendocrine tumors, and work hard to maintain good communication with referring physicians about their patients. Our excellent clinical outcomes and extensive record of publications on pituitary disease are foundational to maintaining MD Anderson as a center of excellence for pituitary tumor care.
Pituitary tumor cases by fiscal year
- 2018: 85
- 2019: 72
- 2020: 58
- 2021: 91
- 2022: 81
Faculty
Ian
E. McCutcheon, M.D., FRCS(C)
Professor | Director, Pituitary Tumor Program
Surgery of pituitary neoplasms, spinal and brain tumors, neurofibromatosis, Gamma Knife Radiosurgery
Shaan
Raza, M.D.
Associate Professor
Open and endoscopic skull base surgery, surgery of benign and
malignant skull base tumors, surgery of pituitary neoplasms
Featured Publication
Ian McCutcheon, M.D.
Santos-Pinheiro F, Penas-Prado M, Kamiya-Matsuoka C, Waguespack SG, Mahajan A, Brown PD, Shah KB, Fuller GN, McCutcheon IE. Treatment and long-term outcomes in pituitary carcinoma: a cohort study. Eur J Endocrinol. 2019 Oct;181(4):397-407. doi:10.1530/EJE-18-0795. PMID: 31349217.
Pituitary carcinoma is a rare neuro-endocrine tumor which has historically been difficult to manage. Typically, surgery, radiation therapy or a combination of both are recommended and chemotherapy is used if surgery or radiation is not possible. Unfortunately, response to therapy is not long-term and the disease recurs in many patients, with poor prognosis.
In this article, the authors conducted a retrospective review of 17 adult patients with pituitary carcinoma who were seen over a 22-year period at The University of Texas MD Anderson Cancer Center. These patients had received multiple types of treatment for their pituitary carcinoma including surgery, radiation and chemotherapy.
The study evaluated the time that it took for the patients to have a recurrence or progression of their tumor after each type of treatment. In this group of patients, the median time between diagnosis and first recurrence of the disease was longer in patients who were treated with the chemotherapeutic agent temolozolomide (TMZ) up front.
Additionally, based on their analysis, combination therapy with surgery, radiation and chemotherapy (particularly TMZ) may result in prolonged survival. The authors conclude that management of pituitary carcinoma with multidisciplinary care and multimodality therapy, particularly therapy including TMZ, is beneficial.
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Spine
About the Section
Our spine surgeons are widely recognized for their extensive experience and expertise, performing more spine tumor surgeries than any other hospital in the nation. Known for our innovation, we utilize advanced technologies including intraoperative imaging, spinal navigation, laser interstitial thermal therapy (LITT), and minimally invasive techniques to optimize outcomes for each individual patient. Many of these advances have been developed and optimized at M.D. Anderson. Moreover, we collaborate extensively with colleagues in plastic surgery, vascular surgery, colorectal surgery, orthopedic surgery, and head and neck surgery to bring multidisciplinary surgical expertise to our complex patient population.
Beyond the operating room, we work hand-in-hand with our radiation oncologists, medical oncologists, pain management specialists, and physiatrists. We recognize that expertly performed surgery is only one part of the carefully designed, personalized, multimodal plan of care needed to offer the best results for our patients.
The spine section treats patients with primary and metastatic tumors of the spinal column, spinal cord, and peripheral nerves. Primary bone tumors include hemangioma, aneurysmal bone cyst, osteoblastoma, giant cell tumor, chordoma, chondrosarcoma, osteosarcoma, Ewing’s sarcoma, and soft tissue sarcomas. We specialize in techniques of en bloc resection (removing the tumor in one piece) needed to provide patients with malignant bone tumors the best chance of cure. Tumors of the intradural space (including the spinal cord) include meningioma, schwannoma, neurofibroma, ependymoma, astrocytoma, and hemangioblastoma. We also have a world-class program for treating spinal metastases (tumors that have spread to the spine from other organs including the breast, kidney, lung, colon, prostate, etc.).
In collaboration with our radiation oncologists, we have built one of the busiest spinal stereotactic radiosurgery programs in the world. This technology allows us to deliver high-dose, focused, conformal radiation to spine tumors, improving our ability to treat symptoms and protect neurologic function. Used in conjunction with our minimally invasive stabilization techniques, laser interstitial thermal therapy, and vertebral augmentation, we are able to treat spinal metastases with fewer complications and shorter hospital stays, allowing our patients to return to an active life.
Spine tumor cases by fiscal year
- 2018: 253
- 2019: 264
- 2020: 233
- 2021: 263
- 2022: 207
Faculty
Laurence D. Rhines,
M.D.
Professor | Director, Spine Tumor Program
Surgery for primary and metastatic spinal tumors, spine stereotactic radiosurgery
Robert North, M.D., Ph.D.
Assistant Professor
Surgery for tumors of the spine and spinal cord
Claudio Tatsui, M.D.
Professor
Surgery for spine and spine tumors, application of local delivery technologies to the treatment of brain and spinal tumors, laser interstitial thermal therapy for spine
Christopher A. Alvarez-Breckenridge, M.D., Ph.D.
Assistant Professor
Spinal tumors, metastatic disease involving the brain and spine, immunotherapy
Featured Publication
Laurence Rhines, M.D.
Outcomes of Surgery for Sacral Chordoma and Impact of Complications: A Report of 50 Consecutive Patients With Long-Term Follow-Up. Zuckerman SL, Lee SH, Chang GJ, Walsh GL, Mehran RJ, Gokaslan ZL, Rao G, Tatsui CE, Rhines LD Global Spine J. 2021 Jun;11(5):740-750. doi: 10.1177/21925682211011444. PMID: 34047643; PMCID: PMC8165918.
Sacral chordomas are primary bone tumors found along the axial skeleton, most commonly arising in the sacrococcygeal region.
While en bloc resection is the preferred treatment for sacral chordomas due to the limited response of these tumors to radiation and chemotherapy, the large size of many sacral chordomas at diagnosis and complex anatomy of the sacropelvic region often make this approach difficult and increase the risk of surgical complications. As such, it is important to identify whether perioperative factors, such as surgical complications, predict postsurgical outcomes in these patients.
A series of 50 patients who underwent en bloc resection of a sacral chordoma at MD Anderson Cancer Center from January 1995 to June 2016, was reviewed. After a median of 5.3 years of follow-up, our team, led by Laurence Rhines, M.D., found that while negative margin resection was associated with a decreased risk of local recurrence, major complications and reoperation did not significantly impact overall survival, local recurrence, or functional outcome.
Therefore, it appears that the inherently high surgical morbidity associated with these invasive operations does not adversely alter the trajectory of survival and recurrence.
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Featured Case
Brief History
This patient is a 36-year-old female who has a history of metastatic catecholamine-producing sympathetic paraganglioma with widespread skeletal metastasis including the spine. She had previously undergone multiple surgeries for skeletal metastasis including in the thoracic spine, shoulders, hips, and long bones of the extremities.
She presented to the MD Anderson Brain and Spine Center with symptoms of worsening upper and lower back pain as well as imaging findings of multi-site progressive spinal metastatic disease. More specifically, she had pathological fractures of T2 and L2 vertebra, high-grade spinal cord compression from metastatic tumor at two separate sites (T2 and T12), and thoracic radiculopathy (a compressed nerve in the thoracic region).
We recommended a surgical plan to treat all the sites in a single stage surgical procedure with pre-operative embolization to reduce intraoperative blood loss. Specifically, we recommended partial T2 vertebrectomy with instrumented spinal stabilization/fusion, T11-T12 laminectomy with tumor resection and instrumented spinal stabilization/fusion, and a L2 vertebroplasty. Radiolucent carbon-fiber reinforced PEEK spinal instrumentation was utilized to facilitate adjuvant radiosurgery planning and improve surveillance imaging.
Surgery and Post-surgical Recovery
The procedures were completed not only on the same day, but in the same operating session and without complication. After surgery our patient remained neurologically intact and was able to be discharged home from the hospital after a brief, uneventful convalescence. She was able to go on to receive adjuvant stereotactic radiosurgery to progressive tumors to facilitate local control.
After her initial recovery the patient was able to resume normal activities. At three months follow up, an MRI of the spine revealed stability of the lesions. A year later, treated lesions remained stable. MD Anderson’s team of surgeons and physicians, stereotactic radiosurgery program, and world-class intraoperative imaging abilities allowed the best outcome for this patient.
Discussion
Paragangliomas are rare tumors. These tumors can occur on the head or neck, but sites of origin may include the spine or elsewhere in the body. Most paragangliomas are benign, but some become cancerous and spread throughout the body. More specifically, a paraganglioma is a type of neuroendocrine tumor (NET). Neuroendocrine cells are nerve cells that produce hormones. Neuroendocrine tumors (NET) are tumors of these cells.
Paraganglioma usually appears in adults aged 30 – 50. People with paraganglioma may experience symptoms like rapid heartbeat, high blood pressure, shakiness, headache, and sweating. Surgery is usually required to treat paraganglioma. Treatment and management of paraganglioma requires specialized equipment and multidisciplinary expertise. Causes of paraganglioma are unknown. Scientists believe that paragangliomas come from mutations of chromaffin cells – nervous system cells. A paraganglioma that occurs in the adrenal gland is called pheochromocytoma.
Preoperative and Postoperative MRI of the Spine
Brain and Spine Center
Spine Cases Fiscal Year 2022
207
Total Spine Cases
Peripheral Nerve Tumor Program
About the Program
We provide safe, compassionate, careful and effective surgical care for patients with all varieties of tumors arising in peripheral nerves. This includes benign nerve sheath tumors such as neurofibromas and schwannomas that are associated with the genetic syndromes neurofibromatosis and schwannomatosis, more aggressive, malignant nerve sheath tumors such as malignant peripheral nerve sheath tumors (MPNST), perineuromas, ganglioneuromas, desmoid tumors, neuromuscular choristomas, intraneural ganglion cysts and neuromas. We aim to control and cure these tumors to reduce patients’ symptoms, including pain, weakness and numbness, and in so doing, enhance their quality of life.
Our two surgeons specializing in peripheral nerve surgery serve a clinic that follows 1,600 patients, the largest in North America. Many of these patients require surgical care on multiple occasions. We operate in all areas of the body to remove nerve tumors while sparing the nerve of origin and employ intraoperative nerve monitoring. When needed, we are adept at restorative surgery (including nerve grafts, nerve transfers and regenerative peripheral nerve interface). Our excellent clinical outcomes, wide-ranging publications on nerve tumors and collaborative care involving a neurologist who coordinates medical care for these patients make this a center of excellence without peer in this country.
We are leaders in education on surgical techniques for treating peripheral nerve tumors, done both didactically and through directed participation in clinical care. We participate actively in postgraduate education through lectures in national and international forums and our involvement in hands-on courses and workshops. Our innovative clinical trial protocols and basic research in this area make us a unique venue to learn the principles and nuances of serving patients with simple and complex nerve tumors, whether benign or malignant in character.
Peripheral nerve tumor cases by fiscal year
- 2018: 29
- 2019: 28
- 2020: 22
- 2021: 21
- 2022: 40
Faculty
Ian
E. McCutcheon, M.D., FRCS(C)
Professor | Director, Peripheral Nerve Tumor Program
Surgery of peripheral nerve tumors, neurofibromatosis, pituitary neoplasms, spinal and brain tumors, Gamma Knife Radiosurgery
Robert North, M.D., Ph.D.
Assistant Professor
Brachial plexus and peripheral nerve tumors, nerve reconstruction, nerve transfers for functional restoration, nerve entrapments including carpal tunnel syndrome, cubital tunnel syndrome, and peroneal neuropathy
Featured Publication
Dr. Ian McCutcheon
Landry JP, Schertz KL, Chiang YJ, Bhalla AD, Yi M, Keung EZ, Scally CP, Feig BW, Hunt KK, Roland CL, Guadagnolo A, Bishop AJ, Lazar AJ, Slopis JM, McCutcheon IE, Torres KE. Comparison of Cancer Prevalence in Patients With Neurofibromatosis Type 1 at an Academic Cancer Center vs in the General Population From 1985 to 2020. JAMA Netw Open. 2021 Mar 1;4(3):e210945. doi: 10.1001/jamanetworkopen.2021.0945. PMID: 33734413; PMCID: PMC7974640.
Neurofibromatosis type 1 (NF1) is a complex genetic disorder which can result in the development of benign and malignant tumors. Although it is known that NF1 is associated with the development of tumors, the risk of developing specific types of tumors in those patients with NF1 is not known.
This large study of patients diagnosed with NF1 between 1985 and 2020 was conducted to determine the prevalence of specific tumor types in NF1 patients, collect information on how their disease was treated or managed and what their clinical outcomes were.
This study found that patients with NF1 develop tumors more frequently and at a younger age than the general population, and the most common tumor types were low grade gliomas, malignant peripheral nerve sheath tumors (MPNST), breast cancer, pheochromocytoma, gastrointestinal stromal tumors (GIST) and melanoma. NF1 patients with undifferentiated pleomorphic sarcoma, high grade gliomas, MPNST, ovarian carcinoma and melanoma had significantly lower disease specific survival rates when compared with patients who developed other types of tumors.
The findings from this study may be useful for follow up and counseling of NF-1 patients and supports a multidisciplinary approach to NF1 patient care.
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Featured Case
Brief History
This patient is a 30-year-old woman with a pilocytic astrocytoma of the upper cervical spinal cord. She was initially diagnosed and treated as a child.
Prior to coming to MD Anderson, she had undergone several surgeries attempting to remove the tumor. She had also undergone radiation therapy and surgery to correct spinal deformity, including screws and rods surgically inserted to fuse and stabilize her spine.
Despite surgical resection and radiotherapy, the tumor continued to grow very slowly. The combination of tumor growth and effects from multiple surgeries and radiation therapy lead to chronic, severe weakness in both her legs and left arm.
When the patient came to us, she had started to develop progressive weakness in her right arm, which she relied on for most of her activities. The weakness was most pronounced in movements of the shoulder and elbow. The weakness in elbow flexion (or bending her elbow) was preventing her from performing many daily tasks of living.
Our clinical examination, electromyography (EMG), and nerve conduction study (NCS) indicated that radiation-induced lower motor neuron disease and cervical spinal cord dysfunction (cervical myelopathy) was the reason for worsening symptoms.
We felt that attempts at resecting the tumor or further correction of the spinal deformity would risk devastating complications, canceling out any possible benefits. Therefore, we chose a more isolated goal: to improve elbow flexion strength.
To that end, we recommended a rerouting of the nerves in her arm, also known as a nerve transfer. In a nerve transfer, a healthy nerve is connected to a damaged nerve. The healthy nerve is the donor nerve. The damaged nerve is the recipient nerve.
In this case, we recommended using a small portion of the ulnar nerve to be rerouted to the brachialis branch of the musculocutaneous nerve. The ulnar nerve primarily helps with hand movements; the musculocutaneous nerve helps with elbow flexion.
Key to the transfer was to only take a small portion of the ulnar nerve so as not to cause problems with hand movements. Still, we had to take enough to provide nerve signals to the muscles for flexing the elbow. A similar nerve transfer of the ulnar nerve to the biceps branch of the musculocutaneous nerve is more commonly performed. However, we chose to transfer to the brachialis branch because the patient’s biceps muscle was stronger to begin with, and we didn’t want to risk damaging its function.
Procedure Details
During surgery, the patient was under general anesthesia with her arm outstretched. Following incision, we identified the relevant nerves. We carefully isolated several branches of the musculocutaneous nerve. We cut one nerve branch to the brachialis muscle proximally along its origin to the musculocutaneous nerve.
We then isolated a portion of the ulnar nerve using internal neurolysis. We confirmed it to have motor function and that the remaining ulnar nerve would still carry signals to control the hand. This isolated portion served as the donor nerve.
We cut the donor nerve and transferred it toward the isolated nerve to brachialis, tunneling underneath the surrounding nerves and soft tissue. We connected the nerve ends using a a surgical microscope, very small sutures (with a thickness of only 0.040 mm) and a biocompatible glue.
After seven months, the patient had improved functioning of her right arm. In fact, she could completely actively flex that arm. She could also perform essential activities of daily life: feeding herself with the right hand, washing her face, combing her hair and even writing. She continued physical therapy, and her functioning improved. She was able to work full time.
Discussion
The Peripheral Nerve section at the MD Anderson Neurosurgery department has experience treating many types of nerve disorders. This case is a good example of how our team treats conditions beyond just the primary cancer; we also treat problems associated with cancer treatments.
While this patient did have a history of cancer, further tumor resection was not an option. It was also not possible to repair the damage induced by radiation. Instead, we focused on restoring function and improving quality of life through a unique and novel nerve transfer procedure.
This case involved a novel nerve transfer procedure that was uniquely tailored for our patient and is not part of the standard treatment options. Instead of transferring ulnar fascicle to the biceps branch of the musculocutaneous nerve, we transferred it to the brachialis branch.
The result was the same: successful restoration of elbow flexion and improved quality of life. This case study is also described in a published article by Larkin et al.
