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Multiple endokrine Neoplasie Typ 1

Chirurgische Therapie des primären Hyperparathyreoidismus

Multiple endocrine neoplasia type 1

Surgical therapy of primary hyperparathyroidism

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Zusammenfassung

Der primäre Hyperparathyreoidismus (pHPT) tritt bei etwa 90% der Patienten mit multipler endokriner Neoplasie Typ 1 (MEN1) auf. Im Gegensatz zum sporadischen pHPT sind üblicherweise mehrerer Drüsen betroffen. Das geeignete Operationsverfahren ist Gegenstand kontroverser Diskussionen. Ziel der vorliegenden Studie war, die Ergebnisse der chirurgischen Therapie des pHPT bei Patienten mit genetisch gesicherter MEN1 zu analysieren. Hierzu wurden präoperative Befunde, operatives Vorgehen und Therapieergebnisse mit Langzeitverlauf des pHPT sowie mögliche Genotyp-Phänotyp-Korrelationen bei 34 Patienten mit genetisch gesicherter MEN1 im Rahmen einer Beobachtungsstudie analysiert. Nach diesen Ergebnisse scheint tPTX+T (total parathyroidectomy+thymectomy) mit Autotransplantation gegenüber der sDE (selektive Drüsenexstirpation) und der stPTX (subtotal parathyroidectromy) ohne zervikale Thymektomie bei Patienten mit MEN1-pHPT das überlegene Operationsverfahren zu sein, da hierbei Rezidive oder eine Persistenz der Erkrankung selten sind. Zur definitiven Beurteilung ist jedoch ein Vergleich der stPTX mit zervikaler Thymektomie und der tPTX mit zervikaler Thymektomie und Autotransplantation im Rahmen einer prospektiven randomisierten Multicenterstudie erforderlich. Eine Genotyp-Phänotyp-Korrelation wurde nicht gefunden.

Abstract

Primary hyperparathyroidism (pHPT) occurs in about 90% of patients with multiple endocrine neoplasia type 1 (MEN1). In contrast to sporadic pHPT, multiple gland disease is most common in MEN1. The appropriate surgical approach is still controversial. The aim of this study was to analyze the results of surgical therapy of pHPT in patients with genetically confirmed MEN1. In an observational study, preoperative data, operative procedures, long-term results, and a possible genotype-phenotype correlation were analyzed in patients with pHPT and genetically confirmed MEN1. According to our results, tPTX+T (total parathyroidectomy+thymectomy+autotransplantation) seems to be a more favorable surgical approach in patients with MEN1 pHPT than sDE (selective gland exstirpation) and stPTX (subtotal parathyroidectromy) without cervical thymectomy, because recurrences or persistence of the disease are rare. A prospective randomized trial is needed to compare stPTX including cervical thymectomy vs tPTX+T. A genotype-phenotype correlation could not be identified.

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Authors and Affiliations

  1. Klinik für Visceral-, Thorax- und Gefäßchirurgie, Philipps-Universität Marburg

    P. Langer, A. Wild, T. Schilling, C. Nies, M. Rothmund & D. K. Bartsch

  2. Klinik für Visceral-, Thorax- und Gefäßchirurgie, Philipps-Universität, Baldingerstraße, 35043 , Marburg

    P. Langer

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  1. P. Langer
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  2. A. Wild
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  3. T. Schilling
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  4. C. Nies
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  5. M. Rothmund
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  6. D. K. Bartsch
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Correspondence to P. Langer.

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Gefördert durch die Else-Kröner-Fresenius-Stiftung.

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Langer, P., Wild, A., Schilling, T. et al. Multiple endokrine Neoplasie Typ 1. Chirurg 75, 900–906 (2004). https://doi.org/10.1007/s00104-004-0838-4

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