Functioning Malignant Carcinoid: A Review of Nine Cases

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THE malignant appearance but benign course of tumors arising from the argentaffin cells of the small intestine was emphasized by Oberndorfer1 in 1907 when he named these tumors ‘carcinoids.’

In 1954, Thorsen and associates2 described a syndrome that consisted of malignant carcinoid of the small intestine with metastases to the liver, valvular disease of the right side of the heart (pulmonary stenosis and tricuspid regurgitation without septal defects), peripheral vasomotor symptoms, bronchoconstriction, and an unusual type of cyanosis. They reported 16 cases that partially or fully demonstrated these criteria and concluded that, “In view of the rarity of both pulmonary stenosis and malignant carcinoid of the small intestine . . . the fact that several cases have been reported to suffer from both conditions simultaneously makes this rather unsuspected combination a clinical and pathologic syndrome . . .”.

In the 10 years immediately following the published report of the carcinoid syndrome, nine patients with functioning malignant carcinoid have been treated and studied at the Cleveland Clinic. One patient with a malignant adenoma of the bronchus and functioning metastases to the liver was previously reported3 as having a variant of the typical carcinoid syndrome. A review of the case material illustrates the protean manifestations of the syndrome, and the validity of Sjoerdsma and Melmon’s4 recent suggestion that the term carcinoid spectrum be used to designate functioning and disseminated malignant carcinoids.

Clinical Material

The clinical data of the nine patients examined at the Cleveland Clinic in the years 1955 through 1964 are . . .



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