Sacrococcygeal chordoma in a 9-year-old boy

Abstract

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. Cordoma sacrococígeo em um menino de 9 anos de idadeRESUMO -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.Chordomas are rare slow-growing tumors that represent around 2% to 5% of all malignant tumors of bones 2 -5 . They derive from ectopic notochordal remnants along the axial skeleton at any location from the base of the skull to the sacrococcygeal region 7 . They have aggressive behavior with local invasion of bone and surrounding tissues 11 . Chordoma is distinctly uncommon in patients under 30 years of age and has a peak incidence around the sixth decade 13 . Less then 5% of cases are diagnosed before 20 years of age 3 . Although the sacrococcygeal region is the most frequent site in adults, tumors arising in the base of the skull appear to predominate in children 313 .

Solitary lymph node metastasis without local recurrence of primary chordoma

“…This tumor develops predominantly in the sacrococcygeal (50%), spheno-occipital (35%) and cervical (15%) region and is generally regarded as a locally aggressive tumor with slow progression growth rate and metastatic incidence ranging from 5 to 40% [5,20]. Its incidence is highest in the sixth decade, but it can also be found in younger population, even infancy (1%) [3,4,12,26]. The male-female ratio among patients is 1.8-2.1.…”