ABSTRACT
A unique case of eyelid metastasis from nasopharyngeal chondroid chordoma in a 63-year-old woman was reported. Chordomas are rare tumors of the bone deriving from remnants of the embryonic notochord. Histologically, the tumor showed lobulated structure and concludes two types of cells: liquid drop cell and small round/cubic cell. Immunohistochemically, AE1/AE3, epithelial membrane antigene (EMA), and S100 showed a uniform and strong positivity. It has a great capacity for recurrence and malignant transformation, despite their slow-growing nature. The most common sites of metastases are liver, lungs, and bones. The eyelid metastasis from chordoma is an extremely rare finding, which may suggest a poor prognosis for the patient. Its significant clinicopathological characteristic could prompt us to take it into consideration when assessing the patient's prognosis.
ABSTRACT
Chordomas are uncommon tumors arising from the embryonic remnants of the fetal notochord. The spheno-occipital region accounts for 36 percent of chordomas. The authors are presenting four cases of intracranial chordomas with clinical manifestation was analyzed. All patients were men and their mean age was 37 years old. The presenting complaints were diplopia in 2 patients and decreased visual acuity in the order two. Radiologic studies demonstrated extensive bony destruction of the skull base and soft mass with punctate calcification in all 4 cases. Treatment consisted of subtotal removal of tumor and subsequent radiotherapy. Histopathologically, three of the four patients had typical chordomas and the remaining case was confirmed as chondroid chordoma. The three typical chordomas recurred after initial treatment. 2 of these three were reoperated but died in the 10 months and 33 months, respectively after initial diagnosis. The patient with chondroid chordoma remained well for 46 months after initial treatment without significant neurological deficits. In conclusion, therapeutic outcome of intracranial chordomas are grim because of their aggressive that, frequent recurrence and inability to remove tumors completely. With the exception, chondroid chordoma had good prognosis and long term survival.
Subject(s)
Adult , Humans , Male , Chordoma , Diagnosis , Diplopia , Notochord , Prognosis , Radiotherapy , Recurrence , Skull Base , Visual AcuityABSTRACT
An unusual case of suprasellar chondroid chordoma in childhood is reported. The patient, a 14-year old boy, presented with total blindness of the left eye which was initially diagnosed as primary optic nerve atrophy. The frontotemporal craniotomy was performed and irradiated postoperatively. Intracranial chordomas in children are rare especially in chondroid varient of the classical chordoma. We reviewed the literatures and stressed this rare case in aspects of the treatment, prognosis and pathological characteristics.
Subject(s)
Adolescent , Child , Humans , Male , Atrophy , Blindness , Chordoma , Craniotomy , Optic Nerve , PrognosisABSTRACT
The authors report an unusual case of a primary chondroid chordoma arising from the base of the petrous temporal bone. A 43-year-old female presented with left sided facial weakness and left sided jugular foramen syndrome. The CT findings of a tumor arising from the base of left petrous temporal bone and it involved left cerebello-pontine angle, and extended through left jugular foramen to parapharyngeal space. The clinical and histopathologic differences between classical(typical) chordoma and its variant are highlighted. The rarity of primary chordomas at this site is stressed.
Subject(s)
Adult , Female , Humans , Chordoma , Temporal BoneABSTRACT
The authors present a case of occipital chondroid chordoma in a 12 year-old boy who presented with occipital mass amd mild tenderness but no neurologic symptoms or signs. On plain skull X-rays, there was an irregular calcific mass at the left occipital area. The CT scan revealed irregular extracranial calcific mass with intracranial extradural lentiform low density lesion at the left occipital area. Also, the occipital bone was thickened. Angiography showed hypervascular mass feeded by occipital artery of left ECA.