Table 2.

Clinicopathological, histological and genetic review of female genital tract tumours of von Hippel Lindau disease

Source	Age	Site	Side & Size	Histology	LOH	VHL disease	Other	Treatment & behaviour
Gersell & King²	46	MS	Lt 3 cm	CCPC	D3S1110, D3S1038⁷	ReA, Cy (R-L-P), RA	–	LSO, benign
Funk & Heiken³	46	BL	Lt 3 cm	CCPC	Not analyzed	RCC, Cy (R-L-P)	Rt ovarian Cyst	LSO, benign
Korn et al.⁴	35	BL	Rt 5, Lt 2 cm	CCPC	Not analyzed	He (C).RCC, Cy (R-P)	–	TC, benign
Gaffey et al.⁵	20	PU	Rt 4 cm	CCPC	Not analyzed	RCC, He (C), Cy (R-P), MET	MET was locally invasive	TC, benign
Werness & Guccion⁶	38	BL	Lt 8 cm	ECAF	D3S1110, 104/105⁷	RA, Cy (R-L-P), He (C), Phaeocromocytoma	–	LSO Postop death
Aydin et al.⁷	32	MS	UL/NA	CCPC	Not analyzed	RCC	–	USO, Recurrence,
Case 1								5 years
Aydin et al.⁷ Case 2	NA	MS	UL/NA	CCPC	Not analyzed	Absent	–	USO, benign
Zanotelli et al.⁸	23	MS	Rt 3.5, Lt 0.8 cm	CCPC	Not analyzed	He (Im)	–	BSO, benign
Present series Case 1a	52	MS & OI	Lt 6, Omental implants 3–2 cm	CCPC	No Loss	Absent	Benign omental implants	TAH-BSO, plus omentectomy A&W 15 yrs
Present series Case 1b	52	MS	Lt 4 cm	ECAF	No Loss	Absent	–	TAH-BSO, A&W 15 years
Present series Case 2	35	BL	UL 3.5 cm	CCPC	Bad data	Massive Cy (R), He (C-Im)	Renal transplant	TC, A&W 7 years
Present series Case 3	34	MS	Rt 5.5 cm	CCPC	D3S1110, D3S1038 D3S1317, D3S1597	Absent	Term pregnancy	RSO, A&W 3 years
Present series Case 4	24	MS	Lt 5 cm	CCPC	D3S1038, D3S1597	NA	PCOD	BSO, LFU
Present series Case 5	36	MS	Rt 5.2, Lt 3 cm	CCPC	D3S1560	Familial VHL. Patient: ReA, RCC, Cy (R-L-P), He (C)	Grandmother, mother and sister died of VHLD-related complications	BSO, A&W 1 year

Site: MS, Mesosalpinx; BL, broad ligament, PU, Parauterine; OI, Omental implant. Side and Size: UL, unilateral, NA, not available. Histology: CCPC, clear cell papillary cystadenoma; ECAF, endometrioid cystadenofibroma of the broad ligament. Von Hippel Lindau disease: ReA, Retinal angioma; Cy, cysts; R, renal; L, liver; P, pancreas; RA, renal adenoma; RCC, renal cell carcinoma; He, hemangioblastoma; C, cerebellar and (Im) intramedullary; MET, middle ear tumour. Other lesions: PCOD, polycystic ovarian disease. Treatment and behaviour: TC, tumourectomy; USO, unspecified salpingoophorectomy; LSO/RSO, left or right salpingo-oophorectomy; TAH-BSO, total abdominal hysterectomy with bilateral salpingo-oophorectomy; A&W, alive and well; LFU, lost to follow-up.