At Penn Medicine, neurosurgeons, in close collaboration with orthopedic oncologists, colon and rectal surgeons and plastic surgeons, are performing en bloc resection for spinal chordomas with the goal of achieving negative margins to prevent recurrence and ensure patient quality of life. Penn Medicine is a regional leader in the treatment of chordomas and other sarcoma-family tumors.
Defined clinically as sarcomas, chordomas are exceptionally rare notochordal tumors of the skull base, spine, and sacrum. The tumors are indolent and infiltrative, and because they invade adjacent neurovascular structures and seed resection cavities, have a high rate of local post-surgical recurrence. Sacral chordomas (the topic of discussion herein*) present as lower back pain refractory to non-steroidal anti-inflammatory drugs, and are often misdiagnosed until an advanced stage of the disease, with generally poor long-term prognosis.
*For a review of cervical chordoma therapy at Penn Medicine, see the En Bloc Spondylectomy and TransOral Robotic Surgery for Cervical Chordoma case study and its accompanying video.
Treatment
Given the infrequency with which chordomas occur (1 per million persons in the United States) and the complications that may ensue with treatment, they are best treated at medical centers possessing a multidisciplinary surgical faculty with an advanced skillset and access to innovative technologies, instrumentation and operative spaces.
Surgeons at Penn Medicine, for example, have developed technologies and techniques to minimize the invasiveness of chordoma surgery and thus allay the potential for significant morbidity in these procedures. This approach, coupled with the engagement of specialists across the surgical spectrum at Penn, offers the best chance for postoperative quality of life.
Because chordomas are relatively resistant to radiation therapy and chemotherapy (alone or in combination), surgery is the preferred approach for first-line treatment. In recent decades, chordoma surgery has evolved from partial excision to en bloc resection with wide margins, which aims to remove the tumor and as much of its residua as possible. The achievement of negative surgical margins in en bloc surgery has been shown to produce a significantly longer disease-free interval and the greatest likelihood of disease-free survival. Proton therapy is available to chordoma patients at Penn Medicine , as well, in keeping with limiting the effects of treatment on sensitive neurovascular structures.
Case Study
Figure 1: Large sacral chordoma involving the sacrum and coccyx.
Mr. V, a 41 year old patient was referred to the Emergency Room at the Hospital of the University of Pennsylvania (HUP) after a CT scan at his community hospital revealed a large mass at his sacrum (Figure 1). In the three years prior to this event, he had experienced progressive pain at his sacrum and coccyx, with a significant effect on his daily life.
Following additional imaging at HUP, Mr. V had a biopsy in the Department of Neuroradiology that was consistent with chordoma. Mr. V’s case was then presented at the Penn Multidisciplinary Sarcoma Tumor Board, a panel comprised of neurosurgeons, orthopedic oncologists, radiation oncologists, medical oncologists, radiologists, and pathologists. On the basis of this discussion, a plan was developed for en bloc surgical resection to be followed by proton therapy. Given the size and the complex nature of the tumor, several surgical teams were involved in the planning and execution of the surgery.
Figure 2: Postoperative CT scan following en bloc resection of this tumor (left). Resected chordoma (right) measuring ~8 inches in diameter.
The initial resection was carried out by Penn neurosurgeons and orthopaedic oncologists using image guidance and navigation to make precise bone cuts in the sacrum and stay outside the boundaries of the tumor. The tumor was removed en bloc (Figure 2); the final pathology showed clean surgical margins.
Because the tumor involved the nerves controlling bowel and bladder function, surgeons in the Division of Colon and Rectal Surgery performed a temporary diversion for Mr. V (he would subsequently have a permanent colostomy).
Surgeons from Penn Plastic Surgery then performed a planned flap reconstruction to address the wound. After his hospital recovery, Mr. V was discharged to a rehabilitation facility and thereafter to home. In the weeks following surgery, he had proton therapy without issue. To date, his follow-up imaging shows no evidence of disease.
About Treatment of Sacral Chordoma at Penn Medicine
The treatment of sacral chordoma tumors at Penn Medicine involves the concerted effort of a variety of disciplines, including surgeons, oncologists, and radiologists. Patients are reviewed in a multi-disciplinary conference to determine the surgical approach and goals of resection, as well as to minimize risk to neurovascular structures and optimize postoperative radiation therapy, most often proton therapy. Surgery is typically minimally invasive with advanced techniques to prevent complications involving spinal fluid leaks, nerve injury and other issues. Continued follow up care, generally with imaging, is needed for years afterwards because of risk of recurrence, both locally as well as the potential for metastasis.
Performing En Bloc Surgery for Sacral Chordoma at Penn Medicine
Neurosurgery
James M. Schuster, MD, PhD
Neil R. Malhotra, MD
Plastic Surgery
Stephen J. Kovach, III, MD
Colon and Rectal Surgery
Najjia N. Mahmoud, MD
Orthopaedic Oncology
Cara A. Cipriano, MD, MSc
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