The patient is a 39-year-old gentleman with a history of Maffucci's syndrome who presents with a painless neck mass he has had for the past several months. The patient noticed the mass has enlarged and denies any neurovascular deficits.
On physical examination, patient has an obvious deformity of his right neck. A 5 x 5-cm mass is located just below and posterior to the right ear. The mass is firm, non-mobile, and non-tender. He has full active range of motion and is neurovascularly intact.
No treatments or imaging has been performed on this mass.
Past Surgical Treatments
Multiple orthopedic surgeries but, of note, a large right-shoulder resection due to chondrosarcoma and left below elbow amputation for chondrosarcoma as well.
Radiographs demonstrate multiple enchondromas and subcutanenous lesions consistent with hemangiomas. The right lateral neck mass is seen. Specifically, the AP radiograph (Figure 1A) demonstrates a chondroid matrix mass. The lateral radiograph (Figure 1B) demonstrates a lytic lesion as well.
Magnetic resonance imaging demonstrates an exophytic, lobulated bony mass extending from the right aspect of the C2 vertebral body posteriorly measuring 7.1 x 5.0-cm (Figure 2A). The lesion has a heterogenous signal and demonstrates extensive displacement of the surrounding soft tissues (Figure 2B).
Computerized tomography demonstrates a right-sided upper neck mass with mixed bone and soft tissue showing rim enhancement. The mass has a chondroid matrix appearance. (Figures 3A, 3B)
Preoperative angiography revealed that at the level of the mass, the right vertebral artery was non-patent. The majority of the mass was avascular as well. The brain stem was supplied by collaterals and the left vertebral artery.
Discussion of Treatment Options and Recommendation
4. En Bloc Resection with Reconstruction
Maffucci's syndrome is characterized by soft tissue angiomatosis and bony enchondromatosis. Furthermore, the potential for malignant degeneration of the angiomatosis to angiosarcoma, or the enchondroma to chondrosarcoma, is high, probably greater than 50%.
Despite the high incidence of malignant degeneration, the prevalence of Maffucci's is low. Primary chondrosarcoma is also a rare condition. Because of the rarity of this malignancy, few reports beyond case reports exist. In a review of patients at M.D. Anderson over a 43-year period, only 21 cases were identified. Furthermore, only 37 patients, who presented at the Mayo Clinic from 1916 to 1981, had chondrosarcomas located in the spine.
Radiographically, the typical chondrosarcoma has a malignant appearance consisting of poor margination, cortical destruction, and soft-tissue extension. Calcified matrix is present in about two-third of patients. The treatment advocated by both groups is en bloc resection, if possible. Local recurrence was found to be diminished with en bloc resection. Because surgical ablation is often difficult if sufficient margins are not obtainable, postoperative radiation has been advocated; however, the efficacy of radiation has been challenged. No chemotherapeutic agents have definitively proved to be of benefit in the treatment of chondrosarcoma.
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In this case, a C1-C4 fusion after a posterior hemilaminectomy of C2 was performed because of the extensive involvement of the upper cervical spine. Occipitocervical fusion was not deemed necessary as there was no involvement of the C1 arch or occiput.
Through a posterior approach, stability was achieved first with left C2 pedicle screw and C1, C3, and C4 lateral mass screw insertion and instrumentation. To supplement the instrumentation, the Gallie wiring technique was utilized from C1-C3 with tricortical iliac crest bone graft. Next, resection of the right C2 lamina, lateral mass, and facet joint was performed first to reveal the C2 nerve root and vertebral artery. Both the C2 nerve root and vertebral artery were divided without entering the tumor mass. The anterior decompression was then addressed by a partial right anterior corpectomy of C2 with the insertion of a tricortical iliac crest allograft between C1-C3.
With the help of an otolaryngologist, an anterior approach was performed through a high submandibular incision connecting the proximal posterior incision transversely so that a skin flap exposed the sternocleidomastoid muscle and spinal accessory nerve. The sternocleidomastoid muscle was detached and the tumor mass dissected and mobilized with sufficient soft tissue margins. An osteotome and power burr was used to resect the C2 vertebral body with negative bony margins, and the entire tumor mass was removed in one piece. Because of press fit of the allograft, anterior instrumentation was not needed.
Figure 4 (below)is a postoperative radiograph demonstrating C1-C4 posterior instrumented fusion on the left-side supplemented with Gallie wiring. An anterior C2 hemicorpectomy with iliac crest allograft was also performed.
Postoperatively, the patient was intubated for two-days. Unfortunately, patient had difficulty swallowing temporarily and was unable to tolerate an oral diet. The patient failed a swallow study; therefore, tube feedings began through a nasogastric tube. The patient failed a second swallow study during hospitalization, so a gastric tube was placed for feeding. The patient was discharged from the hospital on postoperative day 21.
At most recent follow-up, 10-months, the patient is alive and does not exhibit evidence, clinically or radiographically, of local recurrence of his chondrosarcoma. He does not endorse any neurological complaints and is now on an oral diet without the use of a gastric tube.
Doctors Park and An present a difficult case of a malignant chondrosarcoma involving the right lateral portion of C2 and C3. The surgery included placement of instrumentation and iliac bone graft on the left from C1 to C4. An en bloc tumor resection was performed by first resecting the C2 root and vertebral artery posteriorly and then dissecting and removing the tumor from an anterior approach and placing an allograft. The patient is doing well 10-months out from surgery.
I suspect the authors performed endovascular testing to help make certain that vertebral artery sacrifice would not be associated with an infarct. Often, this can be done a few days before surgery and the vessel taken with coils at the same time. This allows the cerebrovascular system to adjust for a period of time before being tested with all of the physiologic changes associated with a prolonged procedure and anesthesia. If any deficits develop, the patient can be given some time to recover before proceeding with the tumor resection.
This was a relatively aggressive approach for a man with a limited life expectancy. While an en bloc resection is preferable, such a management strategy needs to be carefully considered. Some tumor cases can be managed more safely with internal decompression and subsequent removal of the peripheral portion. This is more attractive in those patients in which there is concern for a limited life expectancy.