Giant cell angiofibroma of gingiva in tuberous sclerosis complex: a case report and literature review

Diagnostic Pathology

Table 1 Updated clinical diagnostic criteria for tuberous sclerosis complex

Clinical diagnostic criteria	Clinical findings	This case
Major features	1. Hypomelanotic macules (≥ 3, at least 5-mm diameter)	+
	2. Angiofibromas (≥ 3) or fibrous cephalic plaque	+
	3. Ungual fibromas (≥ 2)	+
	4. Shagreen patch	+
	5. Multiple retinal hamartomas	-
	6. Cortical dysplasias*	-
	7. Subependymal nodules	-
	8. Subependymal giant cell astrocytoma	-
	9. Cardiac rhabdomyoma	-
	10. Lymphangioleiomyomatosis (LAM)†	-
	11. Angiomyolipomas (≥ 2)†	-
Minor features	1. “Confetti” skin lesions	+
	2. Dental enamel pits (> 3)	+
	3. Intraoral fibromas (≥ 2)	+
	4. Retinal achromic patch	-
	5. Multiple renal cysts	-
	6. Nonrenal hamartomas	+

Definite diagnosis: Two major features or one major feature with ≥ 2 minor features
Possible diagnosis: One major feature or ≥ 2 minor features
*Includes tubers and cerebral white matter radial migration lines
†A combination of the two major clinical features (LAM and angiomyolipomas) without other features does not meet the criteria for a definite diagnosis

ISSN: 1746-1596