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16523

Long-Term Outcomes After Partial Adrenalectomy In Pediatric Patients with Von Hippel-Lindau Syndrome

Saturday, October 20, 2012
Grand Ballroom A/B (Hilton Riverside)
Dawud O. Lankford, MD1, Nitin Yerram, BS2, Dmtry Volkin, BS2, Israel Franco, MD1 and Peter Pinto, MD2, (1)Urology, New York Medical College, Valhalla, NY, (2)Urologic Oncology, National Institutes of Health, Bethesda, MD

Purpose:  

Children with von Hippel-Lindau (VHL) syndrome are at an increased risk for developing bilateral pheochromocytomas. In an effort to illustrate our experience with partial adrenalectomy (PA) in this select population, we report the largest series on PA for pediatric VHL patients.

Methods:  

From 1994 to 2011, a database was reviewed to evaluate 10 pediatric patients with hereditary pheochromocytoma for PA.  Surgery was performed if there was clinical evidence of pheochromocytoma and normal adrenocortical tissue was evident on preoperative imaging.  Perioperative data were collected and patients were followed for postoperative steroid use and tumor recurrence.

Results:

Ten pediatric patients with a diagnosis of VHL underwent 18 successful PAs.  The median tumor size was 2.6 cm. Over a median follow up of 7.2 years additional tumors in the ipsilateral adrenal gland were found in two patients. One patient underwent completion adrenalectomy and the other received a salvage PA with ipsilateral resection. One patient required steroid replacement. At last follow up, 7 patients had no evidence of disease recurrence.

Conclusion:

At our institution, partial adrenalectomy is the preferred management for pheochromocytoma in the (VHL) pediatric population.  This surgical approach allows for removal of tumor while preserving adrenocortical function and minimizing the side effects of long term steroid replacement on quality of life and development.

Table 1. Clinical Characteristics and Outcomes

Case No

Age*

Gender

Largest lesion size (cm)

PA Operationsa

Recurrence site/time

Path

Follow up (yrs)

Steroids

 

1

17

M

3.0

lap-RPA

None

Pheo

15.63

No

 

2

13

M

2.7

lap-LPA, lap-BPA

None

Pheo

15.77

No†

 

3

13

M

2.5

open-LPA

None

Pheo

12.74

No

 

4

13

M

4.1

lap-LPA, lap-RPA

None

Composite

9.41

No

 

5

15

F

1.4

lap-LPA, lap-RPA

None

Pheo

2.56

No

 

6

6

M

3.5

lap-LPA, lap-RPA

Right/5mos

Composite

4.58

No

 

7

17

F

2.0

open-BPA

None

Pheo

**

**

 

8

13

M

1.5

lap-RPA

None

Pheo

5.00

No

 

9

16

M

6.5

open-RPA, lap-RPA

Right/1yr

Pheo

4.06

No

 

10

15

M

5.5

robo-LPA, robo-RPA

None

Pheo

4.42

Yes/2 yrs post-op

 

       * Age at first operation

   ** Post-operative death

        a   Operations are in chronological order.                                                       

     † Patient began steroids after removal of an ACTH secreting ectopic tumor.

lap - laparoscopic

rob - robotic

LPA – left partial adrenalectomy

RPA – right partial adrenalectomy

BPA – bilateral partial adrenalectomy

Table 2. Operative Characteristics

Total Number  (n)

Total adrenal units operated on

18

Open

4

Lap

12

Robotic

2

Median

Range

Gross tumor size (cm)

2.6

1.2 – 6.5

Operative time (min)

300

170 – 600

Open

353

255 – 450

Lap

300

170 – 600

Robotic

273

245 – 300

Median

Range

Estimated blood loss

150

50 – 750

Open

725

700 – 750

Lap

125

50 – 700

Robotic

100

50 - 150