Melanotic progonoma of the brain: a case report and review.
So far, only 25 melanotic progonomas have been found in the central nervous system (male/female ratio 3.5), mostly located in the cerebellum. The average age is 8 years (range 3.5 months to 69 years) with 85% becoming clinically apparent in the first decade of life; 73.7% of the patients reported succumbed to their disease at a mean age of 2.8 years, with a postoperative survival time of just 9 months. Systemic metastases were reported in 9 cases and had mostly spread via cerebrospinal fluid. In contrast to peripheral melanotic progonomas usually found in the maxilla, cerebral progonomas have a much worse outcome and have to be regarded as malignant. We present the case of a 1-year-old boy suffering from a melanotic progonoma of the pineal gland, who died at the age of 22 months with extensive spinal and abdominal metastases 10 months after partial removal of the tumor.
Rickert
C H
CH
Institute of Neuropathology, Westfälische Wilhelms-Universität, Münster, Germany. rickchr@uni-muenster.de
Probst-Cousin
S
S
Blasius
S
S
Gullotta
F
F
D000008:Abdominal Neoplasms / Q000556:secondary; D014408:Biomarkers, Tumor / Q000032:analysis; D001932:Brain Neoplasms / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008264:Macrophages; D008297:Male; D008543:Melanins / Q000032:analysis; D008940:Mitotic Index; D017600:Neuroectodermal Tumor, Melanotic / Q000737:chemistry / Q000473:pathology*; D010870:Pineal Gland / Q000737:chemistry / Q000473:pathology*; D011379:Prognosis; D013125:Spinal Neoplasms / Q000556:secondary; D014057:Tomography, X-Ray Computed
D014408:Biomarkers, Tumor; D008543:Melanins