We report two patients in whom pheochromocytoma was treated by subtotal adrenalectomy leaving a rim of vascularized cortical tissue in situ. Both patients are doing well without cortisol supplementation although they have subnormal cortisol responses to ACTH stimulation

Cedermark, Bjorn

Grondal, Staffan

Hamberger, Bertil

Hansson, Bengt-Goran

Telenius-Berg, Margareta

Werner, Sigbritt

English

Henry Ford Health System Scholarly Commons

Henry Ford Hospital Medical Journal Volume 35 Number 2 Second International Workshop on MEN-2 Article 14 6-1987 Subtotal Adrenalectomy in Multiple Endocrine Neoplasia Type 2 Bertil Hamberger Margareta Telenius-Berg Bjorn Cedermark Staffan Grondal Bengt-Goran Hansson See next page for additional authors Follow this and additional works at: https://scholarlycommons.henryford.com/hfhmedjournal  Part of the Life Sciences Commons, Medical Specialties Commons, and the Public Health Commons Recommended Citation Hamberger, Bertil; Telenius-Berg, Margareta; Cedermark, Bjorn; Grondal, Staffan; Hansson, Bengt-Goran; and Werner, Sigbritt (1987) "Subtotal Adrenalectomy in Multiple Endocrine Neoplasia Type 2," Henry Ford Hospital Medical Journal : Vol. 35 : No. 2 , 127-128. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol35/iss2/14 This Article is brought to you for free and open access by Henry Ford Health System Scholarly Commons. It has been accepted for inclusion in Henry Ford Hospital Medical Journal by an authorized editor of Henry Ford Health System Scholarly Commons. Subtotal Adrenalectomy in Multiple Endocrine Neoplasia Type 2 Authors Bertil Hamberger, Margareta Telenius-Berg, Bjorn Cedermark, Staffan Grondal, Bengt-Goran Hansson, and Sigbritt Werner This article is available in Henry Ford Hospital Medical Journal: https://scholarlycommons.henryford.com/hfhmedjournal/vol35/iss2/14 Subtotal Adrenalectomy in Multiple Endocrine Neoplasia lype 2 Bertil Hamberger,* Margareta Telenius-Berg, Bjorn Cedermark, Staffan Grondal, Bengt-Goran Hansson, and Sigbritt Werner We report two patients in whom pheochromocytoma was treated by subtotal adrenalectomy leaving a rim of vascularized cortical tissue in situ. Both patients are doing well without Cortisol sup-plementation although they have subnormal Cortisol responses to ACTH stimulation. (Henry Ford Hosp Med J 1987:35.727-8) The clinical management of adrenal disease in multiple endo-crine neoplasia type 2 (MEN-2) is controversial. Bilateral total adrenalectomy has been suggested to prevent risk of recurrence. However, patients then require lifelong steroid medication. Although only minimal morbidity from bilateral adrenalec-tomy has been reported (1), this has not been our experience. An altemative is to leave the apparently unaffected adrenal gland in place. In a series of patients undergoing only unilateral adrenalectomy, it has been demonstrated that contralateral adre-nalectomy could be postponed for several years (2). Total adre-nalectomy and transplantation of cortical tissue has so far been successful only in patients with Cushing's syndrome. We report here our experiences with two patients with the MEN-2 syn-drome on whom we performed subtotal adrenalectomy with the aim of removing all the adrenal medullary tissue. Technique The adrenal gland was widely exposed by a flank or dorsal approach. Part of the gland was mobilized and the adrenal vein ligated and divided. A rim of one adrenal gland, which by digi-tal palpation was felt to be thin, soft, and nontumorous and into which several small vessels can be seen entering, was identified. The rest of the gland was resected leaving only this rim of vascularized cortical tissue in situ (Figure). Case Reports Casel A 31-year-old man had undergone a left adrenalectomy for pheo-chromocytoma nine years previously. At a follow-up examination his remaining adrenal gland was enlarged and hypersecreting. This right adrenal gland was resected with about one third of cortical tissue being left in situ. The specimen contained two pheochromocytomas, 2.5 and 1 cm in diameter His urinary norepinephrine levels were within normal limits both preoperatively and postoperatively, but the epinephrine Figure—Schematic drawing of resection ofthe adrenal gland. A thin, well-vascularized rim ofthe adrenal gland is identified and left in situ and the rest ofthe gland is resected. Submitted for publication: March 2, 1987, Accepted for publication: April 13, 1987, 'Address correspondence lo Professor Hamberger, Department of Surgery, Karolinska Hospital. Stockholm, Sweden, Henry Ford Hosp Med J—Vol 35, Nos 2 & 3. 1987 Subtotal Adrenalectomy in MEN-2—Hamberger et al 127 Table Urinary Excretion of Norepinephrine, Epinephrine, and Cortisol after Subtotal Adrenalectomy Norepinephrine* Epinephrinet Cortisol^ Patient Preoperative Postoperative Preoperative Postoperative Postoperative 1 384 183 59 10 120 2 699 250 108 6 195 *NormaI level < 400 mmol/24 hours. tNormal level < 80 mmol/24 hours. tNormal level 80-300 mmol/24 hours. concentration was almost nondetectable postoperatively (Table). His postoperative Cortisol medication was withdrawn after one month. Since then, he has been without Cortisol supplementation except for a few periods with fever Two years postoperatively his urinary Cortisol level is normal, but the response to ACTH stimulation is subnormal. Case 2 A 31-year-old woman, who had been operated on for medullary thyroid carcinoma ten years earlier, was found to have a pheo-chromocytoma in the right adrenal gland. The tumor, which measured 3.5 cm in diameter, was removed along with the entire right adrenal gland. The left adrenal was resected leaving a rim of cortical tissue in situ. Urinary catecholamines were normal after operation (Table). Three months postoperatively Cortisol medication was withdrawn and after another six months basal plasma Cortisol and urinary Corti-sol levels were normal. However, her response to ACTH stimulation was subnormal. Conclusions In two patients with MEN-2 a rim of adrenal cortical tissue was left in situ when adrenalectomy was pierformed. Postoperatively, normal norepinephrine and very low epinephrine levels were de-tected in the urine. Both patients are doing well without Cortisol supplementation. Two more patients have recentiy been operated on with good immediate clinical results. However, follow-up eval-uations are not yet complete. In our opinion, subtotal adrenalec-tomy should be considered a valuable altemative in the treatment of patients with MEN-2. Acknowledgments This investigation was supported by the Swedish Medical Research Council (02330) and the Karolinska Institute. References 1. van Heerden JA, Sizemore GW, Carney JA, Grant CS, ReMine WH, Sheps SG. Surgical management of the adrenal glands in the multiple endocrine neoplasia type II syndrome. World J Surg 1984:8:612-21. 2. Tibblin S. Dymling JF, Ingemansson S, Telenius-Berg M. Unilateral ver-sus bilateral adrenalectomy in multiple endocrine neoplasia IIA. World J Surg 1983:7:201-8. "Man is a prisoner of his ideas . . . if he does not possess suitable and necessary tools." Claude Bernard—1865 128 Henry Ford Hosp Med J—Vol 35, Nos 2 & 3,1987 Subtotal Adrenalectomy in MEN-2—Hamberger et al 

Subtotal Adrenalectomy in Multiple Endocrine Neoplasia Type 2

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Subtotal Adrenalectomy in Multiple Endocrine Neoplasia Type 2

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