Sacrococcygeal chordoma in a 9-year-old boy

Noronha, Lúcia de; Werner, Betina; Mendonça, Carmem Maria C.; Nomura, Luiz; Bleggi-Torres, Luiz Fernando

doi:10.1590/S0004-282X1995000400018

Abstracts

A case of sacrococcygeal chordoma in a 9-year-old boy is presented. The symptoms at presentation were pain in both legs and sacrococcygeal region for the last two years that increased in the last four weeks irradiating mainly to the left leg. X-ray and CT scan examinations of the lumbar region revealed an expansive process in the coccygeal region with multiple calcifications and a partially eroded coccyx. There was no invasion of the retroperitoneum and regional lymph nodes. A biopsy was performed and showed cords and nests of cells with large cytoplasm, sometimes vacuolated, nuclei with moderate pleomorphism and clumped chromatin. Immunohistochemistry with avidin-biotin peroxidase technique showed positivity for CK, S-100 protein, CEA, vimentin and to EMA. Chordomas are a distinctly uncommon neoplasm in the first two decades of life, specially in the sacrococcygeal region. They have an aggressive behavior. Treatment of choice is complete resection.

chordoma; immunohistochemistry; notochord; bone tumors

Os autores apresentam um caso de cordoma sacroccígeo em um menino de 9 anos de idade. O paciente foi admitido no hospital com história de dor na região sacral e nos membros inferiores com dois anos de evolução, piorando nas últimas quatro semanas. O exame físico revelou atrofia muscular moderada em ambos os membros inferiores, diminuição do reflexo patelar e presença do sinal de Lasègue à esquerda. Os exames de imagem da região lombar mostraram um processo expansivo na região sacrococcígea com erosão parcial do coccix e focos de calcificação, sem evidência de metástases para linfonodos regionais. Foi realizada biópsia diagnóstica que mostrou neoplasia formada por cordões e ninhos de células de citoplasma amplo, por vezes vacuolado, com núcleos moderadamente pleomórficos com cromatina grumosa. O estudo imuno-histoquímico revelou positividade para CK, proteína S-100, CEA, vimentina e EMA. Cordomas são tumores raros que representam em torno de 2% de todas as neoplasias malignas do osso. Os locais de maior acometimento são as extremidades da coluna espinhal e são incomuns nas primeiras duas décadas de vida, especialmente na região sacrococcígea.

cordoma; imuno-histoquímica; notocorda; tumores ósseos

Sacrococcygeal chordoma in a 9-year-old boy

Cordoma sacrococígeo em um menino de 9 anos de idade

Lúcia de Noronha^I; Betina Werner^I; Carmem Maria C. Mendonça^II; Luiz Nomura ^III; Luiz Fernando Bleggi-Torres^I

^ISection of Neuropathology and Electron Microscopy

^IIDepartment of Pediatrics, Hospital de Clínicas, University of Paraná

^IIIHospital Nossa Senhora das Graças, Curitiba

SUMMARY

A case of sacrococcygeal chordoma in a 9-year-old boy is presented. The symptoms at presentation were pain in both legs and sacrococcygeal region for the last two years that increased in the last four weeks irradiating mainly to the left leg. X-ray and CT scan examinations of the lumbar region revealed an expansive process in the coccygeal region with multiple calcifications and a partially eroded coccyx. There was no invasion of the retroperitoneum and regional lymph nodes. A biopsy was performed and showed cords and nests of cells with large cytoplasm, sometimes vacuolated, nuclei with moderate pleomorphism and clumped chromatin. Immunohistochemistry with avidin-biotin peroxidase technique showed positivity for CK, S-100 protein, CEA, vimentin and to EMA. Chordomas are a distinctly uncommon neoplasm in the first two decades of life, specially in the sacrococcygeal region. They have an aggressive behavior. Treatment of choice is complete resection.

Key words:chordoma, immunohistochemistry, notochord, bone tumors.

RESUMO

Os autores apresentam um caso de cordoma sacroccígeo em um menino de 9 anos de idade. O paciente foi admitido no hospital com história de dor na região sacral e nos membros inferiores com dois anos de evolução, piorando nas últimas quatro semanas. O exame físico revelou atrofia muscular moderada em ambos os membros inferiores, diminuição do reflexo patelar e presença do sinal de Lasègue à esquerda. Os exames de imagem da região lombar mostraram um processo expansivo na região sacrococcígea com erosão parcial do coccix e focos de calcificação, sem evidência de metástases para linfonodos regionais. Foi realizada biópsia diagnóstica que mostrou neoplasia formada por cordões e ninhos de células de citoplasma amplo, por vezes vacuolado, com núcleos moderadamente pleomórficos com cromatina grumosa. O estudo imuno-histoquímico revelou positividade para CK, proteína S-100, CEA, vimentina e EMA. Cordomas são tumores raros que representam em torno de 2% de todas as neoplasias malignas do osso. Os locais de maior acometimento são as extremidades da coluna espinhal e são incomuns nas primeiras duas décadas de vida, especialmente na região sacrococcígea.

Palavras-chave:cordoma, imuno-histoquímica, notocorda, tumores ósseos.

Texto completo disponível apenas em PDF.

Full text available only in PDF format.

Aceite: 5-maio-1995.

Luiz F. Bleggi Torres, M.D., Ph.D. - Seção de Neuropatologia e Microscopia Eletrônica, Departamento de Patologia, Hospital das Clínicas - Rua General Carneiro 181 - 80060-900 Curitiba PR - Brasil.

1. Abenoza P, Sibley RK. Chordoma: an immunohistologic study. Hum Pathol 1986; 17:744-747.
2. Bjornsson J, Wold LE, Ebersold MJ, Laws ER. Chordoma of the mobile spine: a clinicopathologic analysis of 40 patients. Cancer 1993; 71:735-740.
3. Coffin CM, Swanson PE, Wick MR, Dehner LP. Chordoma in childhood and adolescence: a clinicopathologic analysis of 12 cases. Arch Pathol Lab Med 1993; 117:927-933.
4. Hruban RH, Traganos F, Reuter VE, Huvos AG. Chordomas with malignant cell components: a DNA flow cytometric and immunohistochemical study with histogenetic implications. Am J Pathol 1990; 137:435-447.
5. Kaiser TE, Pritchard DJ, Unni KK. Clinicopathologic study of sacrococcygeal chordoma. Cancer 1984; 54:2574-2578.
6. Kaneko Y, Sato Y, Iwaki T, Shin RW, Tateishi J, Fukui M. Chordoma in early childhood: a clinicopathological study. Neurosurgery 1991; 29:442-446.
7. Keisch ME, Garcia DM, Shibuya RB. Retrospective long-term follow-up analysis in 21 patients with chordomas of various sites treated at a single institution. JNeurosurg 1991; 75:374-377.
8. Miettinen M. Chordoma: antibodies to epithelial membrane antigen and carcinoembryonic antigen in differential diagnosis. Arch Pathol Lab Med 1984; 108:891-895.
9. Rich TA, Schiller A, Suit HD, Mankin MJ. Clinical and pathological review of 48 cases of chordoma. Cancer 1985; 56:182-187.
10. Salisbury JR. The patology of the human notochord. J Pathol 1993; 171:253-255.
11. Salisbury JR, Deverell MH, Cookson MJ, Whimster WF. Three-dimensional reconstruction of human embryonic notochords: clue to the pathogenesis of chordoma. J Pathol 1993; 171:59-62.
12. Walaas L, Kindblom LG. Fine-needle aspiration biopsy in the preoperative diagnosis of chordoma: a study of 17 cases with application of electron microscopic, histochemical and immunocytochemical examination. Hum Pathol 1991; 22:22-28.
13. Wold LE, Laws ER. Cranial chordomas in children and young adults. J Neurosurg 1983; 59:1043-1047.

Publication Dates

Publication in this collection
20 Dec 2010
Date of issue
Sept 1995

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Abenoza P, Sibley RK. Chordoma: an immunohistologic study. Hum Pathol 1986; 17:744-747.

[2] 2. Bjornsson J, Wold LE, Ebersold MJ, Laws ER. Chordoma of the mobile spine: a clinicopathologic analysis of 40 patients. Cancer 1993; 71:735-740.

[3] 3. Coffin CM, Swanson PE, Wick MR, Dehner LP. Chordoma in childhood and adolescence: a clinicopathologic analysis of 12 cases. Arch Pathol Lab Med 1993; 117:927-933.

[4] 4. Hruban RH, Traganos F, Reuter VE, Huvos AG. Chordomas with malignant cell components: a DNA flow cytometric and immunohistochemical study with histogenetic implications. Am J Pathol 1990; 137:435-447.

[5] 5. Kaiser TE, Pritchard DJ, Unni KK. Clinicopathologic study of sacrococcygeal chordoma. Cancer 1984; 54:2574-2578.

[6] 6. Kaneko Y, Sato Y, Iwaki T, Shin RW, Tateishi J, Fukui M. Chordoma in early childhood: a clinicopathological study. Neurosurgery 1991; 29:442-446.

[7] 7. Keisch ME, Garcia DM, Shibuya RB. Retrospective long-term follow-up analysis in 21 patients with chordomas of various sites treated at a single institution. JNeurosurg 1991; 75:374-377.

[8] 8. Miettinen M. Chordoma: antibodies to epithelial membrane antigen and carcinoembryonic antigen in differential diagnosis. Arch Pathol Lab Med 1984; 108:891-895.

[9] 9. Rich TA, Schiller A, Suit HD, Mankin MJ. Clinical and pathological review of 48 cases of chordoma. Cancer 1985; 56:182-187.

[10] 10. Salisbury JR. The patology of the human notochord. J Pathol 1993; 171:253-255.

[11] 11. Salisbury JR, Deverell MH, Cookson MJ, Whimster WF. Three-dimensional reconstruction of human embryonic notochords: clue to the pathogenesis of chordoma. J Pathol 1993; 171:59-62.

[12] 12. Walaas L, Kindblom LG. Fine-needle aspiration biopsy in the preoperative diagnosis of chordoma: a study of 17 cases with application of electron microscopic, histochemical and immunocytochemical examination. Hum Pathol 1991; 22:22-28.

[13] 13. Wold LE, Laws ER. Cranial chordomas in children and young adults. J Neurosurg 1983; 59:1043-1047.