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Sarcomatous Change After Sellar Irradiation in a Growth Hormone-Secreting Pituitary Adenoma

  • Sujit S. Prabhu (a1) (a2), Kenneth D. Aldape (a3) (a2), Robert F. Gagel (a4), Robert S. Benjamin (a5), Jonathan C. Trent (a5) and Ian E. McCutcheon (a1) (a2)...

Abstract

Background:

Although the benefits of radiotherapy for pituitary adenomas are well-documented, post-irradiation sarcomas of the sella are rarely seen, with only 20 cases (mainly of fibrosarcoma) reported in the medical literature.

Method:

We describe a case of post-irradiation sarcoma five years after surgery followed by external-beam irradiation for an extensive and locally invasive growth hormone-secreting tumor. The patient was subsequently given pegvisomant, an antagonist of growth hormone receptor, to control symptoms of growth hormone excess.

Results:

The patient underwent transsphenoidal resection of the recurrent tumor, followed by adjuvant chemotherapy. This led to significant relief in the patient's symptoms including radiological evidence of tumor shrinkage, but the tumor regrew when, owing to dose-limiting toxicity, chemotherapy was stopped.

Conclusion:

Post-irradiation sarcomas of the pituitary are well-recognized but rare. They should be suspected in patients following sellar irradiation who show abrupt onset of new symptoms and appropriate radiological findings, and such tumors may respond to cytotoxic chemotherapy.

RÉSUMÉ: Introduction:

Bien que les bénéfices de la radiothérapie soient bien documentés dans le traitement des adénomes pituitaires, les sarcomes de la selle turcique postirradiation sont rares, seulement 20 cas ayant été rapportés dans la littérature médicale, surtout des fibrosarcomes.

Méthodes:

Nous décrivons un cas de sarcome post-irradiation cinq ans après une chirurgie suivie d’irradiation pour traiter une tumeur extensive avec envahissement local secrétant de l’hormone de croissance. Le patient a reçu par la suite du pegvisomant, un antagoniste des récepteurs de l’hormone de croissance, pour contrôler les symptômes causés par un excès d’hormone de croissance.

Résultats:

Le patient a subi une résection transsphénoïdale de la récidive tumorale suivie par une chimiothérapie adjuvante. Les symptômes du patient ont considérablement diminué et on a observé une régression de la tumeur. Cependant il y a eu récidive de la tumeur quand la chimiothérapie a dï être arrêtée à cause de sa toxicité.

Conclusions:

Les sarcomes de l’hypophyse après irradiation sont bien connus mais rares. On doit soupçonner leur présence chez les patients qui ont subi une irradiation de la selle turcique et qui présentent subitement de nouveaux symptômes et des signes radiologiques appropriés. Ces tumeurs peuvent répondre à la chimiothérapie cytotoxique.

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References

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Sarcomatous Change After Sellar Irradiation in a Growth Hormone-Secreting Pituitary Adenoma

  • Sujit S. Prabhu (a1) (a2), Kenneth D. Aldape (a3) (a2), Robert F. Gagel (a4), Robert S. Benjamin (a5), Jonathan C. Trent (a5) and Ian E. McCutcheon (a1) (a2)...

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