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Secondary chondrosarcoma of the lumbar spine in hereditary multiple exostoses
The Spine Journal 13 (2013) 1158–1159
Secondary chondrosarcoma of the lumbar spine in hereditary multiple exostoses We report a case of a 19-year-old woman with hereditary multiple exostoses, confirmed via EXT2 gene mutation, who presented to our clinic with a 4-month history of low back pain. On physical examination, she had a palpable mass on the right lower back and did not have any leg pain or weakness. Her surgical history is notable for osteochondromas removed from her right distal femur and proximal tibia. Radiographs demonstrated calcification in the right lumbar paraspinal soft tissue (Fig. 1, Top), and computed tomography demonstrated an exostoses arising from the L5 spinous process (Fig. 1, Bottom) with a large paraspinal mass containing calcifications. Magnetic resonance imaging (MRI) demonstrated a large lesion with high signal on T2 (Fig. 2, Top) that also enhanced on T1 with gadolinium. The diagnosis of low-grade chondrosarcoma was made based on biopsy. Using our previously published surgical planning algorithm [1], we preformed en bloc resection of the spinous processes of L3, L4, and L5 along with the right paraspinal muscles and portion of the right iliac crest (Fig. 2, Bottom). When managing patients with HME and back pain, one should have a low threshold to obtain imaging including radiograph and MRI to check for the presence of exostoses. A 27% rate of exostoses in the spinal canal have been reported, and some groups make it a routine practice to obtain a screening MRI of the spine in the growing years of children with HME [2]. Although malignant degeneration of exostoses in the spine is rare, one should keep it in their differential. References [1] Boriani S, Weinstein JN, Biagini R. Primary bone tumors of the spine: terminology and surgical staging. Spine 1997;22:1036–44. [2] Roach JW, Klatt JW, Faulkner ND. Involvement of the spine in patients with multiple hereditary exostoses. J Bone Joint Surg Am 2009;91:1942–8.
1529-9430/$ - see front matter Ó 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.spinee.2013.03.056
Fig. 1. (Top) Anteroposterior radiograph demonstrating calcification in the right lumbar paraspinal soft tissue. (Bottom) Axial computed tomography demonstrating an exostoses arising from the L5 spinous process.
A. Mesfin et al. / The Spine Journal 13 (2013) 1158–1159
1159
Addisu Mesfin, MDa Riccardo Ghermandi, MDb Emanuela Castiello, MDb Davide M. Donati, MDc Stefano Boriani, MDb a Division of Spine Surgery Department of Orthopaedic Surgery University of Rochester 601 Elmwood Ave., Box 665, Rochester NY 14642, USA b Department of Oncologic and Degenerative Spine Surgery Rizzoli Orthopaedic Institute Via G.C. Pupilli, 1-40136 Bologna, Italy c Department of Musculoskeletal Oncology Rizzoli Orthopaedic Institute Via G.C. Pupilli, 1-40136 Bologna, Italy FDA device/drug status: Not applicable. Author disclosures: AM: Nothing to disclose. RG: Nothing to disclose. EC: Nothing to disclose. DMD: Nothing to disclose. SB: Nothing to disclose.
Fig. 2. (Top) Axial magnetic resonance imaging demonstrating a large lesion with high signal on T2. (Bottom) Images of the specimen after the en bloc resection of the spinous processes of L3, L4, and L5 along with the right paraspinal muscles and a portion of the right iliac crest.
Secondary chondrosarcoma of the lumbar spine in hereditary multiple exostoses We report a case of a 19-year-old woman with hereditary multiple exostoses, confirmed via EXT2 gene mutation, who presented to our clinic with a 4-month history of low back pain. On physical examination, she had a palpable mass on the right lower back and did not have any leg pain or weakness. Her surgical history is notable for osteochondromas removed from her right distal femur and proximal tibia. Radiographs demonstrated calcification in the right lumbar paraspinal soft tissue (Fig. 1, Top), and computed tomography demonstrated an exostoses arising from the L5 spinous process (Fig. 1, Bottom) with a large paraspinal mass containing calcifications. Magnetic resonance imaging (MRI) demonstrated a large lesion with high signal on T2 (Fig. 2, Top) that also enhanced on T1 with gadolinium. The diagnosis of low-grade chondrosarcoma was made based on biopsy. Using our previously published surgical planning algorithm [1], we preformed en bloc resection of the spinous processes of L3, L4, and L5 along with the right paraspinal muscles and portion of the right iliac crest (Fig. 2, Bottom). When managing patients with HME and back pain, one should have a low threshold to obtain imaging including radiograph and MRI to check for the presence of exostoses. A 27% rate of exostoses in the spinal canal have been reported, and some groups make it a routine practice to obtain a screening MRI of the spine in the growing years of children with HME [2]. Although malignant degeneration of exostoses in the spine is rare, one should keep it in their differential. References [1] Boriani S, Weinstein JN, Biagini R. Primary bone tumors of the spine: terminology and surgical staging. Spine 1997;22:1036–44. [2] Roach JW, Klatt JW, Faulkner ND. Involvement of the spine in patients with multiple hereditary exostoses. J Bone Joint Surg Am 2009;91:1942–8.
1529-9430/$ - see front matter Ó 2013 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.spinee.2013.03.056
Fig. 1. (Top) Anteroposterior radiograph demonstrating calcification in the right lumbar paraspinal soft tissue. (Bottom) Axial computed tomography demonstrating an exostoses arising from the L5 spinous process.
A. Mesfin et al. / The Spine Journal 13 (2013) 1158–1159
1159
Addisu Mesfin, MDa Riccardo Ghermandi, MDb Emanuela Castiello, MDb Davide M. Donati, MDc Stefano Boriani, MDb a Division of Spine Surgery Department of Orthopaedic Surgery University of Rochester 601 Elmwood Ave., Box 665, Rochester NY 14642, USA b Department of Oncologic and Degenerative Spine Surgery Rizzoli Orthopaedic Institute Via G.C. Pupilli, 1-40136 Bologna, Italy c Department of Musculoskeletal Oncology Rizzoli Orthopaedic Institute Via G.C. Pupilli, 1-40136 Bologna, Italy FDA device/drug status: Not applicable. Author disclosures: AM: Nothing to disclose. RG: Nothing to disclose. EC: Nothing to disclose. DMD: Nothing to disclose. SB: Nothing to disclose.
Fig. 2. (Top) Axial magnetic resonance imaging demonstrating a large lesion with high signal on T2. (Bottom) Images of the specimen after the en bloc resection of the spinous processes of L3, L4, and L5 along with the right paraspinal muscles and a portion of the right iliac crest.