A Multi-Disciplinary Approach to a Rare Pediatric Extra-Axial Chordoma

Thursday, September 15, 2022

3:00 PM – 3:10 PM East Coast USA Time

Location: JW Grand Ballroom 5-6

Reference 1: Evans S, Khan Z, Jeys L, Grimer R. Extra-axial chordomas. Ann R Coll Surg Engl. 2016 May;98(5):324-8. doi: 10.1308/rcsann.2016.0138. PMID: 27087325; PMCID: PMC5227051.
Reference 2: Das, P, Soni, P, Jones, J, et al. Descriptive epidemiology of chordomas in the United States. J Neurooncol. 2020;148:173–178.
Reference 3: Beccaria K., Sainte-Rose C., Zerah M., Puget S.: Paediatric chordomas. Orphanet J Rare Dis 2015; 10: pp. 116.

Primary Presenter(s)

TC

Tara Chandran, DO

General surgery resident PGY4
Henry Ford Macomb

Disclosure(s): No financial relationships to disclose

Author(s)

EW

Elisabeth Wynne, MD

Dayton Children's Hospital

Disclosure information not submitted.
KK

Kambiz Kamian, MD

Dayton Children's Hospital

Disclosure information not submitted.

Disclosure(s):

Tara Chandran, DO: No financial relationships to disclose

Introduction/Purpose: Extra-axial chordomas are extremely rare malignant tumors with an incidence of less than one per million. Of those, less than five percent of cases are in the pediatric population. There are no standardized guidelines due to the rare nature of the tumor. Several case reports suggest wide resection as the mainstay of treatment given its locally aggressive nature and relative chemoresistance and radioresistance. This case report will review a pediatric extra-axial chordoma of the lower extremity treated with wide local excision in conjunction with neurosurgery for peripheral nerve preservation.

Methods or Case Description:
The patient is a 10 year old female who presented with right lower extremity mass that was first noticed one month prior to arrival. The lesion was flesh-colored with occasional purple discoloration over the top that was not painful and waxed and waned in size. She had no history of trauma, no systemic B cell symptoms, no weight loss and no prior nodules or neurofibromatosis. Her primary care physician ordered an MRI which demonstrated an ovoid mass of 1.4 x 1.9 x 2.4 cm in the subcutaneous fat concerning for a nerve sheath tumor, possibly associated with the peroneal nerve. She underwent a core needle biopsy that suggested extra-axial chordoma or schwannoma. After multidisciplinary tumor board discussion, she was taken to the operating room for wide local excision with neurosurgery to assist for peripheral nerve identification and preservation. The mass was well encapsulated, confined to the subcutaneous tissues and it was associated with a cutaneous branch of the sural nerve. A wide local excision was complete with gross total resection. The main trunk of the sural nerve was identified and preserved. Nerve monitoring was used throughout the case.

Outcomes: Post-operatively, patient recovered well. She was discharged the day of surgery and returned to clinic without postoperative complications. Pathology returned as extra-axial chordoma with granulation change and positive resection margins. The case was represented to a multidisciplinary tumor board that included oncology, radiation oncology, radiology and surgery. The decision was made for watchful waiting secondary to the low rate of metastasis associated with this tumor.

Conclusion: Due to the indolent nature of extra-axial chordoma and its likelihood of local recurrence rather than metastasis, we elected to monitor with close follow-up every 3 months including physical exams and ultrasound. For rare tumors without standardized guidelines, it is essential to discuss treatment and follow up with a multidisciplinary tumor board.
Learning Objectives:
Describe the embryological origins of the extra-axial chordomas
Explain why radiation is not recovered in treatment of extra-axial chordomas
Define the mosty common place where chordomas are found