Abstracts

Management of primary spinal chondrosarcoma: report of two cases causing cord compression

Manejo dos condrosarcomas espinhais primários: relato de dois casos causando compressão medular

Daniel Monte-Serrat Prevedello^I; Joacir Graciolli Cordeiro^I; Andrei Koerbel^II; Léo Fernando da Silva Ditzel^II; João Cândido Araújo^III

Department of Neurosurgery, Hospital Nossa Senhora das Graças, Curitiba PR, Brazil:

^IResident in Neurosurgery

^IINeurosurgeon

^IIINeurosurgeon Coordinator of the residency program

ABSTRACT

Chondrosarcomas are malignant tumors that rarely grow inside the spinal canal. Prognosis depends on histological features, patient's age and surgical margins free from tumor. Response to radio and chemotherapy is poor. Ideal treatment consists of total " en-block" resection, not always achievable due to limitation of location, compromise of stability and risk of inducing neurological deficits. Two cases of spinal chondrosarcoma causing cord compression are reported, located in the cervical and thoracic spine. Microsurgical technique consisted of initial debulking followed by removal of margins until limits free from tumor were obtained. Total resection was accomplished and neurological function improved in both cases. Follow-up has been seven and one year respectively, with no evidence of recurrence and preserved neurological functions. Association between chondrosarcoma and estrogen-dependent tumor has been confirmed in this report. Although " en-block" resection of a chondrosarcoma should be tried whenever possible, tumor fragmentation should be considered in difficult cases, as in the present report, in which a long period free from recurrence with good quality of life can be obtained.

Key words: chondrosarcoma, spinal neoplasms, spinal cord compression, microsurgery.

RESUMO

Os condrosarcomas são tumores malignos, raramente localizados no interior do canal espinhal, com prognóstico dependente do grau histológico do tumor, idade do paciente e margens cirúrgicas livres. Esses tumores apresentam pouca resposta à radio e quimioterapia. O tratamento ideal consiste em ressecção tumoral em bloco, condição particularmente difícil em se tratando de tumores causando compressão medular, devido à localização da lesão, comprometimento da estabilidade axial e necessidade da manutenção ou recuperação da integridade da função neurológica do paciente. Relatamos dois casos de condrossarcomas causando compressão medular, um na coluna cervical e outro na torácica, submetidos a cirurgia com esvaziamento tumoral seguido de remoção das margens, atingindo-se ressecção total e melhora das funções neurológicas em ambos os casos. Um paciente se encontra há 7 anos e outro há 1 ano livre de recorrências, ambos com funções neurológicas preservadas. Associação entre condrosarcoma e tumor dependente de estrógeno é confirmada clinicamente pelo presente estudo. Embora uma ressecção em bloco dos condrossarcomas deva ser tentada quando possível, deve-se considerar uma ressecção com fragmentação tumoral em casos específicos, podendo-se atingir, como demonstrado nos presentes casos, períodos livres de recorrência e longo tempo de sobrevida.

Palavras-chave: condrossarcoma, neoplasias medulares, compressão da medula espinhal, microcirurgia.

Chondrosarcomas are malignant lesions, which represent the second most common tumor of the skeleton. However, presentation as primary tumor of the spine is extremely rare. These tumors can originate from healthy bone or develop from a cartilaginous lesion with sarcomatous degeneration¹. Primary malignant tumors should be resected with wide safety margins. However, limitations to total resection may involve risk of causing spinal instability and/or inflicting new deficits. Isolated cases of spinal chondrosarcomas have been reported^2-18 as well as a few series with limited number of patients^19-22.

Specific surgical strategies are delineated in the present report emphasizing the necessity of total removal, particularly considering tumor resistance to adjuvant therapy^19,20.

CASES

Case 1. An 80 year-old woman was admitted with progressive weakness in her lower limbs, which resulted in inability to walk, one week before admission. Neurological examination showed spastic paraparesis. Hypoesthesia with partial loss of pain and temperature below T4 level and preserved proprioception and vibration sense were also demonstrated. She was submitted to a cervical and thoracic spinal magnetic resonance study (MRI), which showed a posterior and lateral extradural tumor from C5 to T1, mainly on the left side, inside the spinal canal, causing significant cord compression. Laminectomies from C5 to T1 were carried out exposing an extradural mass of great proportions on the left side of the spinal canal affecting the posterior elements of C6 to T1 and causing a compressive effect on the spinal cord. The mass was completely resected in a piece-meal fashion until margins free of tumor were obtained. Post-operative period was uneventful with gradual reversal of her neurological deficits. Pathology specimen was compatible with chondrosarcoma and no adjuvant therapy was used.

Four years after the spinal surgery the patient underwent a histerectomy due to a endometrium carcinoma. After two years, she was submitted to another procedure, a radical mastectomy, due to a breast cancer.

Seven years have elapsed since the spinal operation with no signs of tumor recurrence.

Case 2. A 25 year-old man was admitted with progressive right intercostal pain irradiated from the back, at a mid thoracic level, of one-year duration. Six months prior to admission he started with gait difficulties and frequent falls, progressing to a severe paraparesis and inability to walk.

His neurological examination demonstrated bilateral normal strength in upper extremities, and only minor proximal muscle function in his lower limbs. Partial loss of pain and temperature and diminished proprioception and vibration were seen below the T6-T7 level, in addition to severe spasticity. Thoracic spine MRI demonstrated a large extradural mass lesion, at T6-T7 level, 3 cm in diameter, compressing the spinal cord, growing through an enlarged right intervertebral foramen (Fig 1). The patient underwent a T6-T7 laminectomy with removal of the respective right facet, which was compromised by a whitish dense extradural tumor. The spinal cord was compressed anteriorly and shifted to the left as tumor advanced through the T6-T7 right foramen. The lesion was completely resected, in a piece-meal fashion until margins free of tumor were obtained. The T6-T7 foramen was left wide opened and the nerve root free of compression. MRI in the first post-operative day confirmed total resection. Neurological deficits gradually improved. Pathology specimen was compatible with chondrosarcoma and no adjuvant therapy was used.

One year has elapsed since the operation and the patient remains asymptomatic. A new MRI was recently obtained confirming no signs of tumor recurrence (Fig 2).

DISCUSSION

Surgical treatment of spinal chondrosarcoma is particularly difficult. Prognosis depends mainly on tumor removal, whereas the peculiar anatomical features of the spine practically avoids an ideal " en-block" resection. When all locations are considered, survival from chondrosarcoma is relatively high, reaching more than 87% of patients at 5 years; however, it is considerably lower when only pelvic, sacral and spinal locations are considered, varying between 25 and 54%¹⁹.

There are only a few series in the literature concerning spinal chondrosarcomas^19-22. Thoracic spine is the most common location and the male sex is predominant^20-21. According to Bergh et al. factors associated with worse prognosis are high histological grade, advanced age, primary surgery out of a referral center, incisional biopsy only and inadequate surgical margins¹⁹. York et al. evaluated 21 patients with spinal chondrosarcoma, during a period of 43 years, demonstrating a longer disease free interval in patients with total rather than subtotal resection. The tumor recurred in 64% of the patients and the median disease free interval was 16 months without adjuvant therapy²⁰. Shives et al. studied 20 patients with chondrosarcoma of the spine and found 100% of recurrence in patients submitted to tumor debulking only, half of the cases recurring among the six patients with inappropriate surgical margins and no recurrences in two patients with radical resection²¹.

Even though rare cases with an " en-block" resection of the lesions have been reported, some with spinal instrumentation, and the prognosis in the long run has not been clearly defined^23-26. Patients submitted to radiotherapy did not demonstrate significant increase in survival in relation to those treated by surgery only^19,20. There are no studies in the literature able to demonstrate effectiveness of radiotherapy, as well as of chemotherapy, in spinal chondrosarcoma.

Case 2 patient presented with enlargement of the intervertebral foramen, raising the suspicion of neurofibroma as first possible diagnosis^27,28. Only one case of foraminal chondrosarcoma has been reported byYünten et al., probably originating from a previous osteochondroma²⁸.

Association between chondrosarcoma and adenocarcinoma of breast , without previous radiotherapy, has been reported by Mummaneni and Rosenberg²⁹. Chondrosarcomas may suffer hormonal influence, even in the absence of detectable estrogen receptors. High estrogen levels seem to increase their growth³⁰.

Case 1 patient developed subsequently both breast and endometrial carcinoma, representing the only case reported in the literature with such an association.

Total surgical resection is the best therapeutic option for chondrosarcomas, considering its resistance to radiotherapy and chemotherapy. Piece-meal removal is recommended when " en-block" resection is not feasible, but assurance of margins free from tumor must be obtained^31,32.

When complete resection is obtained prognosis may be good, even in the presence of adverse factors, such as advanced age, as in case 1. Association between chondrosarcoma and estrogen-dependent tumors has been confirmed in this report.

Received 6 January 2004, received in final form 26 March 2004. Accepted 18 May 2004

Dr. Daniel Monte Serrat Prevedello - Rua Alcides Munhoz 433 - 80810-040 Curitiba PR - Brazil. E-mail: prevedello@hotmail.com

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