Bilateral adrenocortical carcinoma in a patient with multiple endocrine neoplasia type 1 (MEN1) and a novel mutation in the MEN1 gene

The incidence of adrenal involvement in MEN1 syndrome has been reported between 9 and 45%, while the incidence of adrenocortical carcinoma (ACC) in MEN1 patients has been reported between 2.6 and 6%. In the literature data only unilateral development of ACCs in MEN1 patients has been reported.

We report a 31 years-old female MEN1-patient, in whom hyperplasia of the parathyroid glands, prolactinoma, non functioning pancreatic endocrine carcinoma and functioning bilateral adrenal carcinomas were diagnosed. Interestingly, a not previously described in the literature data, novel germline mutation (p.E45V) in exon 2 of MEN1 gene, was detected.

The association of exon 2 mutation of the MEN1 gene with bilateral adrenal carcinomas in MEN1 syndrome, should be further investigated.

Author: John Griniatsos, Nikoletta Dimitriou, Athanassios Zilos, Stavroula Sakellariou, Konstantinos Evangelou, Smaragda Kamakari, Penelope Korkolopoulou, Gregory Kaltsas
Credits/Source: World Journal of Surgical Oncology 2011, 9:6

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Laparoscopic Resection is Inappropriate in Patients with Known or Suspected Adrenocortical Carcinoma

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Laparoscopic Resection is Inappropriate in Patients with Known or Suspected Adrenocortical Carcinoma

B. S. Miller¹ , J. B. Ammori¹, P. G. Gauger¹, J. T. Broome³, G. D. Hammer² and G. M. Doherty¹

(1)	Division of Endocrine Surgery, University of Michigan, 2920F Taubman Center, 1500 E. Medical Center Drive, Ann Arbor, MI 48109, USA

(2)	Division of Metabolism, Endocrinology, and Diabetes, University of Michigan, Ann Arbor, MI, USA

(3)	Division of Surgical Oncology and Endocrine Surgery, Vanderbilt University, Nashville, TN, USA

Published online: 7 April 2010

Abstract

Background

Complete surgical resection is the mainstay of treatment for patients with adrenocortical cancer (ACC). Use of laparoscopy has been questioned in patients with ACC. This study compares the outcomes of patients undergoing laparoscopic versus open resection (OR) for ACC.

 

Methods

A retrospective review (2003–2008) of patients with ACC was performed. Data were collected for demographics, operative and pathologic data, adjuvant therapy, and outcome. Chi-square analysis was performed.

 

Results

Eighty-eight patients (66% women; median age, 47 (range, 18–81) years) were identified. Seventeen patients underwent laparoscopic adrenalectomy (LA). Median tumor size of those who underwent LA was 7.0 (range, 4–14) cm versus 12.3 (range, 5–27) cm for OR. Recurrent disease in the laparoscopic group occurred in 63% versus 65% in the open group. Mean time to first recurrence for those who underwent LA was 9.6 months (±14) versus 19.2 months (±37.5) in the open group (p < 0.005). Fifty percent of patients who underwent LA had positive margins or notation of intraoperative tumor spill versus 18% of those who underwent OR (p = 0.01). Local recurrence occurred in 25% of the laparoscopic group versus 20% in the open group (p = 0.23). Mean follow-up was 36.5 months (±43.6).

 

Conclusions

ACC continues to be a deadly disease, and little to no progress has been made from a treatment standpoint in the past 20 years. Careful and complete surgical resection is of the utmost importance. Although feasible in many cases and tempting, laparoscopic resection should not be attempted in patients with tumors suspicious for or known to be adrenocortical carcinoma.

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B. S. Miller Email: barbram@umich.edu

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Laparoscopic management of adrenal lesions larger than 5 cm in diameter - Abstract

Monday, 11 January 2010

Muljibhai Patel Urological Hospital, Nadiad, Gujarat, India.

Laparoscopic adrenalectomy remains a controversial procedure for large tumors. The incidence of adrenocortical carcinoma increases and technical difficulty of adrenalectomy increases as the size increases. We examined the outcome and complications of laparoscopic adrenalectomy for such lesions.

 

Twenty-nine patients underwent laparoscopic adrenalectomy, of whom 19 had tumors larger than 5 cm in diameter, having a median tumor size of 7.0 cm. They were compared with patients whose adrenal tumors were smaller than 5 cm.

 

Patients with small tumors (< 5 cm) had a significantly shorter median operative time of 90 minutes as compared to 145 minutes in those with large tumors (> 5 cm). There was no significant difference in the median hemoglobin drop (1.05 g/dL versus 1.30 g/dL), time for starting oral intake (24 hours in both groups) or hospital stay (3.5 days versus 4.0 days) between patients with small and large tumors, respectively. There were no intra-operative complications except for 1 incidence of supraventricular tachycardia in a patient with a large pheochromocytoma. There were no major complications seen in any of the patients and no open conversions. Histopathology of large tumors revealed 16 benign tumors (8 pheochromocytomas, 4 adenomas, 2 ganglioneuromas, 1 pseudocyst, and 1 myelolipoma) and 3 malignancies, of which 1 was primary adrenocortical carcinoma and 2 were metastatic renal cell carcinoma.

 

In experienced hands, laparoscopic adrenalectomy is safe and feasible for large functioning adrenal tumors. Large adrenal tumors suspicious of harboring malignancy with no peri-adrenal involvement can be tackled laparoscopically.

 

Written by:
Sharma R, Ganpule A, Veeramani M, Sabnis RB, Desai M.   Are you the author?

 

Reference:
Urol J. 2009 Fall;6(4):254-9.

PubMed Abstract
PMID:20027553

UroToday.com Adrenal and Retroperitoneum Section

 

From http://www.urotoday.com/57/browse_categories/adrenal_and_retroperitoneum/laparoscopic_management_
of_adrenal_lesions_larger_than_5_cm_in_diameter__abstract01112010.html