Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome

Table 1

Revised Proteus syndrome diagnostic criteria (Turner et al., 2004) [15].

To make a diagnosis of PS, one must have all the general criteria and various specific criteria
General criteria	All of the following:
	(i) Mosaic distribution of lesions
	(ii) Sporadic occurrence
	(iii) Progressive course
Specific criteria	Either
	(i) category A,
	(ii) two from category B, or; (iii) three from category C
Specific criteria categories	(A) Cerebriform connective tissue nevus
	(B) (1) Linear epidermal nevus
	(2) Asymmetric, disproportionate overgrowth
	One or more:
	(a) Limbs:
	Arms/legs
	Hands/feet/digits
	Extremities
	(b) Hyperostoses of the skull
	(c) External auditory meatus
	(d) Megaspondylodysplasia
	(e) Viscera: spleen/thymus
	(3) Specific tumors before 2nd decade
	One of the following:
	(a) Ovarian cystadenoma
	(b) Parotid monomorphic adenoma
	(C) (1) Dysregulated adipose tissue
	Either one:
	(a) Lipomas
	(b) Regional absence of fat
	(2) Vascular malformations
	One or more:
	(a) Capillary malformation
	(b) Venous malformation
	(c) Lymphatic malformation
	(3) Lung cysts
	(4) Facial phenotype
	All:
	(a) Dolichocephaly
	(b) Long face
	(c) Down slanting palpebral fissures and/or; minor ptosis
	(d) Low nasal bridge
	(e) Wide or anteverted nares
	(f) Open mouth at rest

Cerebriform connective tissue nevi are skin lesions characterized by deep grooves and gyrations as seen on the surface of the brain.
Asymmetric, disproportionate overgrowth should be carefully distinguished from asymmetric, proportionate overgrowth (see Discussion for recommended methods of distinction).
The facial phenotype has been found, to date, only in PS in patients who have mental deficiency and, in some cases, seizures and/or brain malformations.