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| Year : 2000 | Volume
: 48
| Issue : 4 | Page : 370-3 |
Infected Ewing's sarcoma of spine.
Kumar R, Prasad KK, Krishnani N, Chhabra DK
Departments of Neurosurgery and Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226014, India.
Correspondence Address:
Departments of Neurosurgery and Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226014, India.
An unusual case of extradural, intraspinal Ewing's sarcoma with significant extraspinal extension is reported. In view of associated constitutional symptoms, suggestive signs, leucocytosis, elevated ESR and operative findings, the initial diagnosis of Pott's spine was entertained. The lesion however turned out to be infected Ewing's sarcoma on histopathology and bacteriological examination.
How to cite this article:
Kumar R, Prasad K K, Krishnani N, Chhabra D K. Infected Ewing's sarcoma of spine. Neurol India 2000;48:370 |
Ewing's sarcoma is the most common primary round cell sarcoma of bones and accounts for 5 to 10% of primary bone tumours.[1] The tumour occurs most frequently in the second decade. There is no sex difference until the age of 13 years, following which, there is an increased incidence amongst males.[1] This tumour occurs most often in long bones, pelvic bones, ribs, mandible and clavicle,[2] but rarely may affect the spine.[1] The involvement of spinal cord is further rare. Among the 445 patients of Ewing's sarcoma none of the patients had spinal cord involvement.[3] We report a case of infected primary epidural Ewing's sarcoma.
A 12 year old female was admitted with three month's history of interscapular pain radiating to anterior chest wall and tingling in the lower limbs, right more than left. The weakness and numbness of both lower limbs rapidly progressed to cripple her completely in course of two and half months. She had constipation, loss of bladder sensation and intermittent high grade fever for the last two months. Her examination revealed marked pallor, toxicity, temperature of 103oF, spastic paraplegia, hypoaesthesia (90%) from D4 to D6 dermatome and 100% sensory loss below D7 dermatome, including the sacral area. Posterior column sensations were also impaired upto D4 level. Anal tone and reflex were diminished. There was significant tenderness at the level of mid dorsal region, but without any spinal deformity. Routine investigations showed a total leucocyte count of 1,10,500/mm3, ESR 42 mm in first hour, serum alkaline phosphatase 100/l, serum LDH 375 U/l, and normal skiagrams of chest. Skeletal survey (done after biopsy) remained normal. MRI dorsal spine showed a well defined, homogeneous, dorsally situated extradural mass at C7 to D4 vertebral level, without the involvement of vertebral bodies. The lesion was infiltrating and involved paravertebral and paraspinal muscles and ligaments at the level of D3 and D4 vertebrae with infiltration of corresponding posterior elements. The mass was homogeneously isointense to slightly hyperintense on T1W and homogeneously hyperintense on T2W and PD images [Figure - 1] and [Figure. 2]. Considering the common diagnosis of extradural spinal tuberculosis, she was put on antitubercular chemotherapy and decompression was planned. C7 to D4 cervicodorsal laminectomy was done. During surgery, at the level of muscle and spinous process, there was whitishyellow, creamy and jelly like thick fluid, mainly on left paraspinal muscle plane with a cavity extending further dorsally upto just short of subcutaneous plane. The involved muscle was however necrosed and dark coloured. In extradural plane, a pale vascular granulation like sheet was encountered, which could be separated easily from all around. Acid fast staining and culture of fluid remained negative but it yielded significant growth of Klebsiella, sensitive to amikacin and other antibiotics. Patient improved on antibiotics. Hypoaesthesia improved by 50%, tone improved, but power recovered only minimally at the time of discharge on 7th postoperative day. Histopathology revealed the sheets of round cells displaying perivascular arrangement in necrotic background at places [Figure. 3] and [Figure. 4]. The diagnosis of Ewing's sarcoma was established. The specimen also showed occasional infiltration of chronic inflammatory cells and pus cells at places. Postoperative irradiation was given. At 4 months follow-up, she was able to move her lower limbs on bed and had regained the bladder sensations. She was lost to followup.
Ewing's sarcoma is a highly malignant tumour of bone and was first described by Sir James Ewing in 1921.[1],[4] It is the commonest primary round cell sarcoma occurring in mid shaft of long and flat bones. Unlike other bone tumours, approximately 50% cases of Ewing's sarcoma involve the pelvis and sacrum, with only 5% involving the true veretbrae.[1] Extraosseous sarcoma was first described in 1969, as a small round cell tumour having the features of Ewing's sarcoma in the paravertebral region in four children.[4] Angervall and Enzinger described additional 39 cases of extraskeletal tumours similar to Ewing's sarcoma of bone. Twelve of these 39 cases were located in the paravertebral region, of which 7 were in the region of lumber spine and sacrum.[5] The case under discussion however had predominantly intraspinal extradural mass with significant extraspinal extension in muscle plane.
Majority of the patients present with pain or mass, with neurological deficit depending upon the site of involvement. Sacral tumour in children may be large resulting in neurogenic bladder. Systematic symptoms such as fever, leucocytosis or elevated ESR may be seen in the lesions of extremities.[1] The present case was also admitted with a history of local and radicular pain and profound, progressive neurological deficit. In addition, she had recurrent fever, leucocytosis and elevated ESR as well, which were most probably because of superadded infection of the mass, the cause of which remained unexplained.
The morphology and delineation study of 59 cases with Ewing's sarcoma on MRI by Haussler et al[6] showed that on T1WI 75% of extra osseous lesions were isointense and 92% of intra osseous lesions were hypointense. On T2W image, 100% of extra osseous lesions were hyperintense, and 93% of intra osseous lesions were hyperintense. Best delineation of intra osseous lesions was on T1W image. Differentiation between tumour and oedema was not possible in intra osseous lesions. Morphology of Ewing's sarcoma was uniform on MRI. [Signal] intensities of present case were similar as described here. It was a homogeneous sharply delineable mass in all the sequences.
The role of operative intervention in this neoplasm is controversial. A comparison of 40 patients having complete resection and the 221 having only biopsy showed a statistically significant improvement in survival of the former.[1] This data justifies the role of surgery in improving local control following systemic therapy. At present it is recommended that diagnostic biopsy be followed by induction chemotherapy to reduce the size of primary tumour, as well as to control systemic disease prior to any major attempt to resect the primary lesion.[1] In the reported case, the gross total excision of tumour was achieved, as there was a strong suspicion of tuberculosis, even on the operation table.
Hayes et al[7] reported a second complete remission rate of 84%. Five years survival in this group was 40%. They suggested that patients of Ewing's sarcoma relapse should be aggressively treated with multimodal and curative intent. In patients with localized disease,[5] 10 and 15 years disease free survival was found to be 51%, and 34% respectively.[8] This indicates that relapse may occur even after 15 years of continuous complete remission.
The disappearance of Ewing's sarcoma following treatment of bacterial infection has been reported.[9] The infected fistula developed following the biopsy of the tumour from pelvis. Patient had multiple spinal metastases and paraplegia, which resolved with the cure of infection. The disease had not responded earlier, despite initiation of chemotherapy. This patient was alive on follow up after 9 years.[9] The cause of infection in case under report is not clear. It seems that some subclinical systemic/contagious infection results in multiplication and colonification of bacteria at the site of tumour, on account of good availability of culture media of necrosed and vascular tumour tissue.
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| 6. | Haussler MD, Vestring T, Steinhoff B et al : The magnetic resonance tomography of Ewing's sarcoma : the morphology and tumour extension, Rofo Fortschr Geb Rontgenstr Neuen Bildgeb Verfahr (German) 1995; 163(4) : 316-320. |
| 7. | Hayes FA, Thompson EI, Kumar M et al : Long term survival in patients with Ewing's sarcoma relapsing after completing therapy. Med Pediatric Oncology 1987; 15 : 254-256. |
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