Outcomes of nephron sparing surgery and cortical sparing adrenalectomy in the management of Von Hippel–Lindau syndrome

Abstract Background The management of Von Hippel–Lindau syndrome (VHLS) is multidisciplinary. The urologist is involved in treating the renal, epididymal tumors and often adrenal pheochromocytoma. Preservation of renal and adrenal function is a challenge. We present 17 cases of VHLS in a tertiary care center in South India. Methods A retrospective review of the patients who underwent surgical treatment under urology for VHLS from January 2009 to November 2018 was conducted. The demographic data, the spectrum of manifestation, treatment, change in glomerular filtration rate, adrenal insufficiency, and recurrence-free survival were analyzed. Results There were 17 patients diagnosed with VHLS. The median age of diagnosis was 39 years (range 23–41). The spectrum of clinical manifestation was: multifocal RCC (88%), pancreatic cysts/tumors (70%), cerebellar hemangioblastoma (59%), retinal angiomas (47%), epididymal cysts/tumors (47%), pheochromocytomas (41%), and spinal hemangiomas (30%). There were seven patients with ten pheochromocytoma lesions. Three underwent cortical sparing and seven total adrenalectomies; 13 patients underwent nephron sparing surgery (NSS), of which seven patients had bilateral tumors. The median duration of follow-up was 6.5 years (range 2–12 years). Following NSS, seven patients had a local recurrence, and one developed pancreatic metastasis. Two patients (11%) were lost to follow-up. Renal function was preserved in all patients at the last follow-up, and there was no postoperative adrenal crisis or mortality. Conclusion Nephron sparing surgery and cortical sparing adrenalectomy are the treatment of choice for multifocal RCC and pheochromocytomas in patients with VHLS providing good oncological outcomes and preservation of renal and adrenal function.

Download Full-text

Nephron sparing surgery in von Hippel-Lindau associated renal cell carcinoma; clinicopathological long-term follow-up

Familial Cancer ◽

10.1007/s10689-012-9525-7 ◽

2012 ◽

Vol 11 (3) ◽

pp. 387-394 ◽

Cited By ~ 9

Author(s):

C. A. Jilg ◽

Hartmut P. H. Neumann ◽

S. Gläsker ◽

O. Schäfer ◽

C. Leiber ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Renal Cell ◽

Nephron Sparing Surgery ◽

Von Hippel Lindau ◽

Nephron Sparing ◽

Long Term Follow Up

Download Full-text

Von Hippel-Lindau Syndrome Presenting as Pancreatic Cysts

Contributions to Nephrology - Rare Kidney Diseases ◽

10.1159/000060208 ◽

2001 ◽

pp. 325-330

Author(s):

F. Scolari ◽

B.F. Viola ◽

L. Grazioli ◽

R. Maiorca

Keyword(s):

Pancreatic Cysts ◽

Von Hippel Lindau ◽

Von Hippel Lindau Syndrome

Download Full-text

Clinical, surgical, pathological and follow-up features of kidney cancer in patients with Von Hippel-Lindau syndrome: Novel insights from a large European consortium

European Urology Open Science ◽

10.1016/s2666-1683(20)33904-5 ◽

2020 ◽

Vol 19 ◽

pp. e1937

Author(s):

U. Capitanio ◽

E. Selcuk ◽

I. Rowe ◽

K. Onder ◽

E. Roussel ◽

...

Keyword(s):

Kidney Cancer ◽

Von Hippel Lindau ◽

Von Hippel Lindau Syndrome

Download Full-text

Nephron Sparing Surgery for Renal Angiomyolipoma with Inferior Vena Cava Thrombus in Tuberous Sclerosis

Case Reports in Urology ◽

10.1155/2014/285613 ◽

2014 ◽

Vol 2014 ◽

pp. 1-8 ◽

Cited By ~ 5

Author(s):

Adrien Riviere ◽

Thomas Bessede ◽

Jean-Jacques Patard

Keyword(s):

Inferior Vena Cava ◽

Tuberous Sclerosis ◽

Inferior Vena ◽

Vena Cava ◽

Nephron Sparing Surgery ◽

Renal Angiomyolipoma ◽

Nephron Sparing ◽

Mean Size ◽

The Mean

Introduction. Angiomyolipoma is a common benign renal tumor. It is associated with Tuberous Sclerosis Complex (TSC) in 20% of patients. Angiomyolipomas are classically multiple, bilateral, and growing; they may lead to complications such as Wunderlich syndrome or, in rare cases, to venous extension.Observation. a 74-year-old woman with TSC presented with an angiomyolipoma of the right kidney with inferior vena cava (IVC) fatty thrombus. She underwent partial nephrectomy and thrombectomy. After a 7-year follow-up there was no evidence of recurrence or metastasis and her renal function was preserved.Review of Literature. It is the 44th reported angiomyolipoma associated with IVC thrombus. The mean size of angiomyolipomas was 86.1 mm and 67.4% of patients were symptomatic. Pulmonary embolism was found in 6 patients. There were 2 cases of recurrence/metastatic outcome after radical nephrectomy and thrombectomy. They were associated with epithelioid form. The mean size of epithelioid tumors was significantly bigger than in classical angiomyolipomas (127.1 mm versus 82.6 mm,P=0.037). With a median follow-up of 12 months, 91.3% of patients were recurrence and metastasis free, with 3 cases of nephron sparing surgery.Conclusion. Nephron sparing surgery for angiomyolipoma with IVC fatty thrombus can be safely performed in TSC, even in sporadic angiomyolipoma.

Download Full-text

MP50-02 IS FOLLOW UP BEYOND 2 YEARS NECESSARY FOR PT1A RENAL CELL CARCINOMA TREATED WITH NEPHRON SPARING SURGERY? AN ASSESSMENT OF LATE RECURRENCES AND SURVEILLANCE COSTS.

The Journal of Urology ◽

10.1016/j.juro.2015.02.1667 ◽

2015 ◽

Vol 193 (4S) ◽

Author(s):

Kamran Zargar-Shoshtari ◽

Tim Kim ◽

Ross Simon ◽

Pranav Sharma ◽

Binglin Yue ◽

...

Keyword(s):

Renal Cell Carcinoma ◽

Cell Carcinoma ◽

Renal Cell ◽

Nephron Sparing Surgery ◽

Nephron Sparing

Download Full-text

Hemangioblastomas of the central nervous system

Journal of Neurosurgery ◽

10.3171/jns.1989.70.1.0024 ◽

1989 ◽

Vol 70 (1) ◽

pp. 24-30 ◽

Cited By ~ 248

Author(s):

Hartmut P. H. Neumann ◽

Hans R. Eggert ◽

Klaus Weigel ◽

Hartmut Friedburg ◽

Otmar D. Wiestler ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Screening Program ◽

Family Background ◽

Renal Cysts ◽

Pancreatic Cysts ◽

Content Type ◽

Von Hippel Lindau ◽

The Central Nervous System ◽

Von Hippel Lindau Syndrome

✓ The findings of a 10-year study (1976 to 1986) conducted in southwest Germany on hemangioblastomas (HBL's) of the central nervous system (CNS) are presented. During that period, 47 HBL's were diagnosed and surgically removed in 44 patients, with a good postoperative survival rate and prognosis. The majority (83%) of these tumors were located in the cerebellum. By thorough clinical examination of the patients and careful evaluation of their family background, it was found that 23% of the HBL patients were afflicted with von Hippel-Lindau syndrome. In addition to the CNS tumors, 14 neoplastic or similar lesions were detected in other tissues. These included angiomatosis of the retinae, pheochromocytomas, pancreatic cysts, renal cysts, and renal carcinoma. The diagnosis of von Hippel-Lindau syndrome was thus established in seven families. The authors suggest the need for a screening program for patients with HBL of the CNS which is designed to confirm or exclude ocular or visceral lesions associated with von Hippel-Lindau syndrome.

Download Full-text

Elective open nephron-sparing surgery for renal masses: Single-center experience with 223 consecutive patients

Journal of Clinical Oncology ◽

10.1200/jco.2009.27.15_suppl.e16139 ◽

2009 ◽

Vol 27 (15_suppl) ◽

pp. e16139-e16139

Author(s):

F. Francesca ◽

G. Pomara ◽

G. Campo ◽

P. Casale

Keyword(s):

Postoperative Bleeding ◽

Oncologic Outcome ◽

Nephron Sparing Surgery ◽

Consecutive Series ◽

Renal Masses ◽

Single Center Experience ◽

Nephron Sparing ◽

Urinary Fistulas

e16139 Background: To present our experience with elective, open, nephron-sparing surgery for renal masses in a contemporary, consecutive series. Methods: In this retrospective study, records of all patients who underwent elective nephron-sparing surgery (E-NSS) between March 1997 and December 2007 at our institution were reviewed. The preoperative workup included laboratory analysis, renal ultrasonography and abdominal computed tomography. The histological findings, complications, and oncologic outcome were studied. Results: A total of 231 E-NSS were performed in 223 patients (82 females,141males; mean age 64 years). 62 “hot ischemia” procedures and 169 “cold ischemia”. The mean tumor size was 4.6 cm (1.1–12cm). 52 patients presented renal masses > 4cm. Renal cell carcinoma was present in 177 patients (76.6%), benign renal masses were diagnosed in in 54 pazienti (23.3%): angiomyolipoma (35%), oncocytoma (40%), complicated cyst (25%). Worthy of note among these 54 patients, pre-operative diagnosis was present in 12 patients. Moreover, 17 benign lesions (31%) were > 4 cm. Complication rate was 5.3% (12 pts): splenectomy (2.2%), nephrectomy because of postoperative bleeding (0.8%), urinary fistulas (0.8%). After a median follow-up of 84 months (range 5 to 120), no patient had developed local recurrence, 19 (8.9%) died for other causes, 2 (0.9%) died for other tumor. Conclusions: The results of this contemporary, monocenter experience underline the role of open, elective, nephron-sparing surgery for patients with renal masses, confirming good results even for renal masses > 4cm. These conclusions are particularly important considering that benign histologic findings were present in almost one forth of patients. No significant financial relationships to disclose.

Download Full-text