phacomatosis
Biology

phacomatosis


Term: phacomatosis (often called neurocutaneous syndromes)

Literally meaning: ?tumor of lenses?

Origin: Anc Greek
?????/phacos(=lens, spot) >????/phaos(=light)
+(-???)/-oma (suffix denoting ?mass growth? or a ?complete set of?)

Coined/History
     The term was coined in 1920 by the Dutch opthalmologist Jan Van der Hoeve (
1878 ? 1952), who initially had included retinal hamartomas and later the tuberous sclerosis, the neurofibromatosis, and the cerebroretinal angiomatosis (von Hippel-Lindau disease).  The term ?phakomatosis? became inappropriate when Van der Hoeve included encephalotrigeminal angiomatosis (Sturge-Weber syndrome) which is not characterized by phakomas or hamartomas. Etymologically, this term also is inadequate to encompass this entire group because it does not include the nervous system. In any case, its use should be reserved to those conditions which manifest retinal hamartomas (?phakomas?), as in the original description.

Definition
A generic term for of hereditary diseases characterized by the development of benign tumors consisting of a disorganized tissue mass of surrounding tissues (hamartomas) in the central nervous system that additionally result in lesions on the skin and the eye.




- Angiomyolipoma
Term: angiomyolipomaLiterally meaning: ?growth of lipoid vessel?Origin: Anc Greek??????/aggio (=vessel, receptacle) > ?????/aggos(=tube)+(-???)/(-oma)=suffix denoting state or condition of mass growthCoined/HistoryGrawitz coined the term angio-myo-lipoma...

- Angiosarcoma
Term: angiosarcomaLiterally meaning: ?growth of vessel?s flesh?Origin: Anc Greek??????/aggio (=vessel, receptacle) > ?????/aggos(=tube)+ ????/sarx(=flesh) >genitive form ??????/sarcos+(-???)/(-oma)=suffix denoting state or condition of mass growth...

- Phacoid
Term: phacoidLiterally meaning: ?lens shaped?Origin: Anc Greek?????/phacos(=lens, spot) >????/phaos(=light)Coined/HistoryDefinitionPhacoid (adj): lens shaped ...

- Poliosis
Term: poliosisLiterally meaning: ?old or gray state or whitening?Origin: Anc Greek??????/polios(=old, gray, venerable) >???????/palaios(=old, as in Paleontology)+(-????/(-osis)(=suffix denoting process or state)Coined/History(?)DefinitionPoliosis...

- Hamartoma
Term: hamartoma Origin: Anc Greek???????/hamartia(=sin, quilt, crime, pity)-???/oma(=complete set of, or a condition.)The Greek word "hamartia" is translated in medical terminology as a ?failure? meaning a tumor formed from malformed rather than truly...



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