Chordoma in an infant
Unusual presentation and clinical course
C. Strantzia-Michail, I. Bonou-Boukouvalea,
N. Arnoyannaki, I. Hager
Pathology Departments: “Aglaia Kyriakou“ Children’s Hospital,
Athens
and "Agios Savas“ Cancer Hospital, Athens,
Orthopedic Clinic of Children’s Hospital, Penteli, Attica
We describe a case of a typical Chordoma that presented in early
infancy with metastases in soft tissues. The tumor although left
untreated, led a slow protracted course for eleven years. A male
infant 2 months old presented with multiple subcutaneous nodules
of the upper and lower extremities. At the age of 11 months, paresis
of the extremities was superimposed. CTscan revealed a tumor of
the clivus with extension in the posterior fossa and upper spinal
canal. The tumor was characterized as unoperable. A biopsy of
the subcutaneous nodule disclosed histologically a Chordoma. The
family of the patient at that time refused any further therapeutic
procedures. At the age of 15 months a partial resection of the
basicranial tumor has been attempted and histological examinatrion
confirmed the diagnosis of a typical chordoma with small areas
of chondroid metaplasia. There was slight polymorphism but no
obvious mitoses. Immunohistochemical investigation showed that
tumor cells were positive for Cytokeretin, EMA, Vimentine and
S100. At the age of 11 years the patient succumbed because of
a local extension of the tumor in nasopharynx and spinal canal,
compressing the brain stem. Subcutaneous nodules were also increased
in size (7 cm).
Key words: Chordoma, Infancy, Basicranial.
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