In cases with concurrent medullary thyroid carcinoma (MTC) and pheochromocytoma, discussion regarding a one-stage versus two-stage treatment strategy approach remains open. From 1975 to 1990, 11 of 25 multiple endocrine neoplasia type 2 (MEN 2) patients presented with biendocrinopathies or triendocrinopathies synchronously. All patients were treated surgically and followed subsequently in our hospital. Of the group of nine patients with concurrent MTC and pheochromocytoma, five were treated in one-stage and four in two-stage procedures. No patient had major complications intraoperatively. For the two-stage group, the total hospital stay (preoperatively and postoperatively) averaged 35 days. For the one-stage group, the total hospital stay averaged 25 days. In patients with increased operative risks (patients with higher age and impaired physical condition or if neck surgery includes transstemal cervicomediastinal lymphadenectomy), two-stage procedures should be selected. However, in young patients with the MEN 2 syndrome or syndromes with small tumors detected by family screening, thyroidectomy, cervical lymphadenectomy, and adrenalectomy may be performed in a one-stage procedure without increasing surgically related morbidity

Dralle, Henning

Scheumann, Georg F. W.

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Henry Ford Hospital Medical Journal Volume 40 Number 3 Article 32 9-1992 Surgical Approach of Synchronous Medullary Thyroid Carcinoma and Pheochromocytoma in MEN 2 Syndrome Georg F. W. Scheumann Henning Dralle 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 Scheumann, Georg F. W. and Dralle, Henning (1992) "Surgical Approach of Synchronous Medullary Thyroid Carcinoma and Pheochromocytoma in MEN 2 Syndrome," Henry Ford Hospital Medical Journal : Vol. 40 : No. 3 , 278-280. Available at: https://scholarlycommons.henryford.com/hfhmedjournal/vol40/iss3/32 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. Surgical Approach of Synchronous Medullary Thyroid Carcinoma and Pheochromocytoma in MEN 2 Syndrome Georg F. W. Scheumann,* and Henning Dralle' In cases with concurrent medullary thyroid carcinoma (MTC) and pheochromocytoma, discussion regarding a one-stage versus two-stage treatment strategy approach remains open. From 1975 to 1990, 11 of 25 multiple endocrine neoplasia type 2 (MEN 2) patients presented with biendo-crinopathies or triendocrinopathies synchronously. All patients were treated surgically and followed subsequently in our hospital. Of the group of nine patients with concurrent MTC and pheochromo-cytoma, five were treated in one-stage and four in two-stage procedures. No patient had major complications intraoperatively. For the two-stage group, the total hospital stay (preoperatively and postoperatively) averaged35 days. For the one-stage group, the total hospital .stay averaged 25 days. In patients with increased operative risks (patients with higher age and impaired physical condition or if neck surgery includes transstemal cervicomediastinal lymphadenectomy), two-stage procedures should be selected. However, in young patients with the MEN 2 syndrome or syndromes with .small tumors detected by family screening, thyroidectomy, cervical lymphadenectomy, and adrenalectomy may be performed in a one-stage procedure without increasing surgically related morbidity. (Henry Ford Hosp Med J 1992;40:278-80) The multiple endocrine neoplasia type 2 (MEN 2) syndrome appears in two variants. MEN 2A is defined as the MEN 2 syndrome without the mucosal neuroma phenotype, and MEN 2B as the MEN 2 syndrome with the mucosal neuroma pheno-type (1). The majority of hereditary cases belong to the MEN 2A (70%) or famdial non-MEN 2 A variant (17% to 24%); only 6% to 13% are of the mucosal neuroma phenotype (MEN 2B) (2-4). The syndrome is a genetically transmitted disease which pre-dominantly affects the thyroid, parathyroid, and adrenal glands. Although surgery is generally regarded as the treatment of choice for each stage ofthe disease, the extent of surgical resec-tion ofthe involved endocrine organs and the surgical time strat-egy in cases of synchronously involved glands is still debatable. In cases with concurrent medullary thyroid carcinoma (MTC) and hyperparathyroidism (HPT), the procedure with total thy-roidectomy and parathyroidectomy performed in a one-stage operation is generally accepted because the extent of resection and operative risk for the patient does not differ from that for one-gland disease (2,5). However, in cases with concurrent MTC and pheochromocy-toma, discussion regarding treatment strategy (one-stage versus two-stage approach) remains open. We present our surgical ap-proach in MEN 2 patients with regard to the synchronous ap-pearance of MTC and pheocfiromocytoma. patients were treated surgically and were followed subsequently at our hospital. The patients" data were stored, maintained, and evaluated by the Clinical Cancer Registry of the Medical School of Hannover. The information system used was developed and established under the DSM-11 (Digital Standard MUMPS) data base management and operating system. ("MUMPS" is the ac-ronym for the Massachusetts General Hospital Utility Multi-programming System which was originally developed at the Laboratory of Computer Science at Massachusetts General Hospital.) Results In the 11 patients (44% of all MEN 2 patients) with synchro-nous biendocrinopathies or triendocrinopathies, two patients suffered from synchronous MTC and HPT (patients 202 and 304). Another two patients showed MTC, HPT. and pheochro-mocytoma (patients 101 and 503) at the time of clinical presen-tation. In seven patients, MTC and pheochromocytoma were demonstrated concurrendy (Table). Four had bilateral pheo-chromocytoma. Bilateral pheochromocytoma occurred more frequentiy in patients with synchronous pheochromocytoma Patients and Methods From 1975 to 1990, 11 of 25 MEN 2 patients presented with biendocrinopathies or triendocrinopathies synchronously. Al l Submitted for publication; Oclober 14. 1991. Accepted for publication: January 27, 1992. *Klinik fur Abdominal und Transplantalionschirurgie der Medizinischen Hochschule Himnover, Hannover, Germany. Address correspondence to Dr. Scheumann, Klinik fur Abdominal und Transplaniation-schirurgie der Medizinischen Hochschule Hannover, Konstanty-Gutschow-Str. 8, 3000 Hannover 61, Germany. 278 Henry Ford Hosp Med J—Vol 40, Nos 3 & 4, 1992 Synchronous Surgery in MEN 2 Syndrome—Scheumann & Dralle Table Surgical Procedure in Nine Patients With Synchronous MEN 2/MTC and Pheochromocytoma (1975-1990) Number One-Stage Day of Discharge Complications Number Two-Stage Day of Di.scharge Complications 102 TT, parathyroidectomy, transperitoneal bilateral total adrenalectomy 302 TT, parathyroidectomy, transperitoneal left total and right sub-total adrenalectomy 305 TT, parathyroidectomy, transperitoneal left and right subtotal adrenalectomy 601 TT, transperitoneal left total adrenalectomy 1101 TT, transperitoneal left total adrenalectomy 17 1^ ) 24 13 101 502 503 504 December 1990, right translumbar total adrenalectomy March 1991, TT December 1986, TT July 1987, trans-lumbar right total adrenalectomy March 1987, TT May 1988, trans-peritoneal bilateral total adrenalectomy with adrenocortical autotransplantation left forearm December 1987, transperitoneal right total adrenalectomy April 1988, TT 4 2 7 8 15 22 Intermittent bradyarrhythmia Pneumonia TT = total thyroidectomy. and MTC, as opposed to those with metachronal pheochro-mocytoma and MTC (6). Ofthe group of nine patients with concurrent MTC and pheo-chromocytoma, five were treated in a one-stage and four in a two-stage procedure. No patient had major complications in-traoperatively. Preoperatively, all patients with synchronous MTC and pheochromocytoma were treated with a high dosage of a-receptor blockage (mean 270 mg phenoxybenzamine) for 13 to 25 days. Two-stage group The three females and two males had a mean age of 67 years (range 48 to 69 years). Two patients underwent thyroidectomy first because of symptomatic MTC (patients 502 and 503). They had been treated preoperatively with high dosage of a-receptor blockage without any intraoperative or postoperative complica-tions. The two other patients were treated first by adrenalectomy and then by thyroidectomy. One patient had pneumonia after the primary transperitoneal unilateral adrenalectomy (patient 504) and was discharged from hospital on the 22nd postoperative day. Another patient experienced a short period of intermittent bradyarrhythmia immediately after unilateral translumbar adre-nalectomy but without any delay in his hospital stay. The dura-tion of postoperative care averaged 12 days forthe single opera-tive procedure and a total of 17.5 days for the two operative pro-cedures for a total hospital stay (pre- and postoperative) averag-ing 35 days (Figure). Two stage OP I I One stage OP Figure—Comparison of patients' ages and length of hospital stays for one- and two-stage procedures. One-stage group The tfiree females and one male had a mean age of 29.4 years (range 15 to 54 years). The surgical procedure in one-stage oper-ations was cervical surgery followed by abdominal or lateral ap-proach to the adrenal gland. There were no postoperative com-plications. The mean postoperative stay was 16.4 days with a to-tal hospital stay (preoperatively and postoperatively) averaging 25 days (Figure). Henry Ford Hosp Med J—Vol 40. Nos 3 & 4. 1992 Synchronous Surgery in MEN 2 Syndrome—Scheumann & Dralle 279 Discussion Surgical management of MEN 2 patients includes preopera-tive diagnostic procedures identifying the organs involved (7,8), preoperative medical treatment of patients with pheochromocy-toma before adrenalectomy (9), and perioperative a-receptor blockade at the time of cervical intervention in patients with a pheochromocytoma diathesis not yet considered for adrenalec-tomy (6). Surgical strategy includes the management of patients with synchronous or metachronous multiorgan manifestations and the surgical technique of thyroid, parathyroid, and adrenal gland resection. Of our MEN 2 patients, 44% presented with synchronous bi-endocrinopathies or triendocrinopathies. This group is hetero-geneous, with an age range from 15 to 69 years, an MTC tumor stage variation from T l to T4, and pheochromocytoma with or without clinical symptoms and unilateral or bilateral disease. Therefore, a general strategy for surgical procedure could not be given. The decision between one-stage or two-stage procedure with adrenalectomy first as routine procedure, or thyroidectomy first in the case of dominating malignant cervical disease, must be made individually based on the patient's physical and MEN 2-related condition if appropriate a-receptor blocking pretreat-ment is followed (9-11). In patients with increased operative risks (i.e., higher age, impaired physical condition, or if neck surgery includes transstemal cervicomediastinal lymphadenec-tomy), two-stage procedures should be selected. However, in young patients with the MEN 2 syndrome (12) and syndromes such as pheochromocytoma and small MTC detected by family screening, thyroidectomy, cervical lymphadenectomy, and adre-nalectomy may be pertbrmed in a one-stage procedure with di-minished individual discomfort attended with a second surgi-cal intervention and without increased surgically related mor-bidity. References 1. Gagel RF, Jackson CE, Ponder BAJ, Raue F, Simpson NE, Ziegler R. Mul-tiple endocrine neoplasia type 2 syndromes: Nomenclature recommendation from the Workshop Organizing Committee. Henry Ford Ho.sp Med J 1989; 37:99. 2. Brunt LM, Wells SA Jr. Advances in the diagnosis and treatment of medul-lary thyroid carcinoma. Surg Clin North Am 1987;67:263-79. 3. Raue F. Spath-Roger M. Winter J. et al. A registry of medullary thyroid can-cer in West Germany. Med Klin 1990;3:113-6. 4. Saad MF, Ordonez NG, Rashid RK, et al. Medullary carcinoma of the thy-roid: A study ofthe clinical features and prognostic factors in 161 patients. Med-icine (Baltimore) 1984;63:319-42. 5. Block MA. Surgical treatment of medullary carcinoma of the thyroid. Oto-laryngol Clin North Am 1990;23:453-73, 6. Dralle H, Scheumann GFW, Kotzerke J, Brabant EG, Surgical management of MEN 2: Recent results in cancer research. Beriin: Springer Veriag (in press). 7. Wells SA Jr, Dilley WG, Famdon JA, Leight GS, Baylin SB. Early diagno-sis and treatment of medullary thyroid carcinoma. Arch Intem Med 1985; 145:1248-52. 8. Duh Q-Y, Sancho JJ, Greenspan FS, et al. Medullary thyroid carcinoma: The need for early diagnosis and total thyroidectomy. Arch Surg 1989; 124:1206-10. 9. Grosse H. Schroder D. Schober O. Hausen B, Dralle H. The importance of high-dose alpha-receptor blockade for blood volui-ne and hemodynamics in pheochrt-)mcx;ytoma. Anaesthesist 1990;39:313-8. 10. Tibblin ST, Dymling J-F, Ingemansson S, Telenius-Berg M. Unilateral versus bilateral adrenalectomy in multiple endocrine neoplasia IIA, World J Surg 1983;7:201-8, 11. Dralle H, Schurmeyer T, Kotzerke T, Kemnitz J, Gros.se H, von zur Muhlen A. Surgical aspects of familial pheochromocytoma. Horm Metab Res [Suppl] 1989;21:34-8. 12. Gagel RF, Tashjian AH Jr, Cummings T, et al. The clinical outcome of prospective screening for multiple endocrine neoplasia type 2A: An 18-year ex-perience. N Engl J Med 318:478-84. 280 Henry Ford Hosp Med J—Vol 40. Nos 3 & 4. 1992 Synchronous Surgery in MEN 2 Syndrome—Scheumann & Dralle 

Surgical Approach of Synchronous Medullary Thyroid Carcinoma and Pheochromocytoma in MEN 2 Syndrome

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Surgical Approach of Synchronous Medullary Thyroid Carcinoma and Pheochromocytoma in MEN 2 Syndrome

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