Non-hotspot PIK3CA mutations are more frequent in CLOVES than in common or combined lymphatic malformations

Table 3 Comparison of the mutation types between the different cohorts

	Number of patients	Pathology	Hotspot	Non-hotspot	Fisher's Exact Test	p value	Adjusted p value	Significance
This study	n = 87	C&C LMs	83	4
	n = 21	PROS	9	12	C&C LMs vs PROS	1.329e-07	1.5948e-06	***
	12	CLOVES	5	7	C&C LMs vs CLOVES	1.441e-05	5.764e-05	***
	4	KTS	3	1	C&C LMs vs KTS	0.2055	0.274
	5	Uncl PROS	1	4	C&C LMs vs Uncl PROS	0.0001207	0.0002896	***
Literature	n = 107	C&C LMs	100	7
	n = 98	PROS	85	13	C&C LMs vs PROS	0.1562	0.2343
	47	CLOVES	35	12	C&C LMs vs CLOVES	0.002331	0.004662	**
	20	KTS	20	0	C&C LMs vs KTS	0.5955	0.7146
	31	Uncl PROS	30	1	C&C LMs vs Uncl PROS	0.6832	0.7453
Combined	n = 194	C&C LMs	183	11
	n = 119	PROS	94	25	C&C LMs vs PROS	7.742e-05	2.232e-04	***
	59	CLOVES	40	19	C&C LMs vs CLOVES	5.575e-07	3.345e-06	***
	24	KTS	23	1	C&C LMs vs KTS	1	1
	36	Uncl PROS	31	5	C&C LMs vs Uncl PROS	0.1434	0.2343

C&C (common and combined) LMs: LM, LVM, CLVM. PROS: includes CLOVES, KTS and unclassified (Uncl) PROS. Detailed values of PROS subgroups are given in italic. Literature: from references [6,7,8, 10, 13, 14]. Combined: this study and the literature. **p value < 0.01; ***p value < 0.001. The distributions between hotspot and non-hotspot are shown in Fig. 3

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