Chondromyxoid Fibroma of the Lumbar Spine in a Pediatric Patient

Posted on November 11, 2008

By Nelson S. Saldua, MD; Anthony I. Riccio, MD; Jeffrey A. Cassidy, MD
ORTHOPEDICS 2008; 31:610

Chondromyxoid fibroma is a rare, nonmalignancy that comprises <1% of all bone tumors. It typically presents with pain, swelling, and tenderness to palpation. The lesion has a predilection for the metaphysis of long bones of the lower extremity, most commonly in the proximal tibial metaphysis. Patients often present in their second or third decade of life, although some reports have included a younger average age.1

Sporadic reports of chondromyxoid fibroma in the spine are found in the literature. This article presents the second case of a chondromyxoid fibroma of the lumbar spine in a pediatric patient, along with a literature review with emphasis on recurrence rates and malignant transformation.

Case Report

An 8-year-old boy initially presented to our facility in November of 2006 with mild lower back pain. He reported no fevers, chills, night sweats, or weight loss. Physical examination was remarkable for an obvious right-sided lumbar scoliotic curve on a forward bend test. He had normal sensation throughout all upper and lower extremity dermatomes. Motor strength was 5/5 throughout all upper and lower extremity muscle groups. Deep tendon reflexes were 2/4 throughout. There was no myoclonus and bilateral Babinski reflexes were downgoing.

Plain radiographs showed a right-sided lumbar curve that measured approximately 30° (Figure 1). Due to the patient’s pain level as well as the atypical appearance of his curve, magnetic resonance imaging (MRI) was obtained.

The MRI showed an expansile, lobulated mass in the posterior elements of L3 with heterogeneously bright T2 signal and several small fluid-fluid levels (Figure 2). Left posterolateral vertebral body extension was evident, and a soft tissue edema was noted that extended into the left paraspinal and psoas muscles. There was no spinal canal extension. A computed tomography (CT) scan demonstrated an expansile lytic lesion within the left L3 posterior elements with a thin, discontinuous cortical rim. Laboratory workup results were within normal limits. Bone scan showed no other areas of increased signal uptake concerning for lesions at other sites.

A CT-guided biopsy led to the diagnosis of chondromyxoid fibroma (Figure 3). En bloc resection and spinal fusion was planned without adjuvant chemotherapy or radiation.

Selective embolization of the mass via the left second and third lumbar arteries with 90 to 180 micron polyvinyl alcohol particles and 2-mm microcoils by interventional radiology was performed 24 hours preoperatively. A posterior approach was taken to expose the spinous process of L1 to L4. Intraoperative fluoroscopy confirmed the correct vertebral level. The thoracodorsal fascia was elevated off of the paraspinal fascia.

Because the preoperative MRI showed that the soft tissue component of the mass extended from the caudal aspect of the L1 vertebral body to the caudal aspect of the L4 vertebral body, transverse cuts were made in the paraspinal musculature at the proper levels. We resected the paraspinal muscles in 1 unit with grossly negative margins. The mass was then tethered only by its attachment into the L3 vertebral body.

A laminotomy was performed at the L2 level to assess the L2 nerve root. This nerve root had some adhesions to the mass but did not course through the mass itself. We removed the inferior facet of L3 and the superior facet of L4. A laminectomy of L3 was done, and the L3 nerve root was observed to be free of the lesion. Once the L2 and L3 nerve roots were identified and found to be free of the mass, the mass was transected near the pedicle and passed off of the table. With the mass removed, a rongeur and Kerrison punches were used to completely remove the L3 pedicle. A curette and rongeur were used the remove the posterolateral aspect of the L3 vertebral body.

Using a pedicle screw construct, a posterior spinal fusion was performed from L2 to L4 (Figure 4). To allow for comfort due to the amount of paraspinal musculature resected, the patient was placed into a thoracolumbosacral orthosis.

The patient has done well postoperatively, and at 13-month follow-up, postoperative CT scan and MRI were both been negative for recurrence.

Discussion

Originally described by Jaffe and Lichtenstein2 in 1948, chondromyxoid fibroma is considered the least common benign neoplasm of cartilage. It has a predilection for the metaphysis of the bones of the lower extremity, with the most common site being the proximal tibial metaphysis. When present in long bones, treatment typically consists of excision, curettage, and bone grafting. No cases of metastasis have been reported, however, this lesion has a tendency toward local recurrence.

Radiographically, chondromyxoid fibromas characteristically appear as well-defined radiolucency located in the metaphysis. A thin sclerotic rim often separates the lesion from the normal trabecular bone. Large lesions may completely penetrate the cortex without periosteal reaction; their eccentric position in long bones can also resemble an aneurysmal bone cyst or unicameral bone cyst. When found in short bones they can be more centrally located, causing a symmetric dilation of the cortex that can mimic the appearance of an enchondroma. Matrix calcification within a chondromyxoid fibroma is rare. On MRI, a multilobulated, high T2-signal mass has been emphasized.

Since the diagnosis of chondromyxoid fibroma depends on the histological analysis, an excisional biopsy is both diagnostic and therapeutic. The gross appearance of this lesion at the time of biopsy has been described as “a soft, pale gelatinous material,”3 “firm white almost avascular tissue”4 or a “mass of grayish-white or bluish-grey” color.5 The gross appearance of this patient’s tumor matched these descriptions in that it was a mass of pale white gelatinous material. Histological analysis shows the presence of all three types of tissue in the name of the tumor. The characteristic appearance of this lesion is that of a mixture of fibrous, myxoid and chondroid areas at different stages of maturity. This characteristic appearance is well represented in this patient’s histology (Figure 3). Focal areas of calcification, giant cells and pleomorphism can also be found.3 This lesion is considered to be benign, however, the pleomorphism and presence of giant cells can lead to the misdiagnosis of chondrosarcoma,2 however, recognition of this nonmalignant lesion is essential to avoid radical operative resections.6 This lesion has also been confused with the diagnosis of chondroblastoma.7,8

While the diagnosis of chondromyxoid fibroma is rare, the location of this lesion in the spine is more uncommon. A thorough review of the literature revealed few cases. Cabral et al9 describe 20 cases of chondromyxoid fibroma involving the spine, 4 of which involved the lumbar spine, with only 1 case reported in an adolescent. Wu et al10 reported a review of 278 cases in which 10 were localized to the vertebra; no mention of the ages or their affected vertebral level are made.

Of the 32 cases reported by Schajowicz and Gallardo,5 only 1 was found to be located in the spine. This case does not provide the patient’s age. Meredith11 reported a case of a 7-year-old girl who presented with an acute onset of lower extremity weakness who was found to have a chondromyxoid fibroma that involved the lamina of T2 causing extensive cord compression. Chondromyxoid fibromas in the spine have been reported in adults.12-15

In their original description of this tumor, Jaffe and Lichtenstein2 reported that “even with incomplete removal, spontaneous regression of the remnants followed.” This has not been found to be the case in the subsequently published literature. A wide variety of recurrence rates have been reported by various authors, ranging from 12% to 44%.3,6,16-19 Due to this high incidence of recurrence with curettage, some have recommended an en bloc excision with tumor free margins. In the case series of Gherlinzoni et al,19 the rate of recurrence was found to have drastically decreased with the addition of corticocancellous bone chips to curettage alone.

While metastasis of chondromyxoid fibroma has never been reported, reports of possible malignant transformation can be found. Iwata and Coley20 case series of 6 patients included a description of 1 tumor recurrence 1 month after curettage of a chondromyxoid fibroma located in the proximal fibula. A second resection was performed, however, 6 months later, the patient underwent amputation due to a second recurrence. Histological examination showed a low-grade chondrosarcoma.20 Of the 6 cases of recurrence in Gherlinzoni’s case series, 1 was due to an “alleged myxosarcomatous transformation.” This patient was treated with amputation 12 years after the initial curettage, but the histological slides were lost, and thus this malignant transformation cannot be verified

Our case report represents the second in the literature of a chondromyxoid fibroma in the lumbar spine in a child. This rare, nonmalignant bone tumor must be considered when dealing with a lytic lesion of the spine. Our patient was treated with an excision, curettage, and posterior spinal instrumentation and fusion. Given the high rates of local recurrence reported, as well as the possibility for malignant transformation, this patient must be and will be followed for tumor recurrence.

References

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  16. Desai SS, Jambhekar NA, Samanthray S, Merchant NH, Puri A, Agarwal M. Chondromyxoid fibromas: a study of 10 cases. J Surg Oncol. 2005; 89(1):28-31.
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Authors

Drs Saldua and Riccio are from Naval Medical Center San Diego, California; and Dr Cassidy is from Helen DeVos Children’s Hospital, Grand Rapids, Michigan.

Drs Saldua, Riccio, and Cassidy have no relevant financial relationships to disclose.

The views expressed in this article are those of the authors and do not reflect the official policy or position of the Department of the Navy, Department of Defense, or United States Government.

Correspondence should be addressed to: Nelson S. Saldua, MD, Department of Orthopedic Surgery, Naval Medical Center San Diego, 34800 Bob Wilson Dr, Ste 112, San Diego, CA 92134.

Posted in Oncology