Hormonal and volumetric long term control of a growth hormone-releasing hormone-producing carcinoid tumor.

Abstract

A 25-yr-old female was referred for symptoms of acromegaly. Two months before admission she mentioned profuse sweating and complained of severe headache. Seven years earlier a large neuroendocrine tumor in the liver was detected. At that time she presented with secondary amenorrhea of 2-yr duration accompanied by fatigue, perspiration, diarrhea, weight loss, and disturbed liver tests (Table 1) and a large tumor in the right liver lobe palpable 10 cm below the right lower costal margin [23 3 18 3 25 cm by magnetic resonance imaging (MRI); Fig. 1A]. During laparatomy a highly vascular tumor was found invading the left liver lobe. Abdominal exploration did not reveal other tumor foci. The tumor biopsy showed characteristics of a neuroendocrine tumor (Fig. 2A) with positive chromogranin and neuronspecific enolase staining. Serum pancreatic polypeptide (PP), chromogranin, serotonin, and urinary 5-hydroxyindole acetic acid (5-HIAA) were markedly elevated. Urinary catecholamines (dopamine and norepinephrine) as well as its degradation products (homovanillic acid and vanillylmandelic acid) were elevated as well (Table 2). Somatostatin receptor scintigraphy with [In-DTPA]octreotide showed major, but heterogeneous, uptake by the hepatic tumor without evidence of other tumor sites (Fig. 3). The search for a primary tumor site, especially in the pancreas, was negative after investigation by computed tomography scan, MRI, endoultrasonography, and somatostatin receptor scintigraphy. The hepatic tumor was considered unresectable by its size and site; chemoembolization (with cisplatinum/lipiodol) was performed in 1992 and 1994 for relief of mass symptoms. Tumor volume was considered unchanged in July 1996. At the time of referral in 1996, soft tissue enlargement, an enlarged tongue, and prominence of the jaw pointed to the diagnosis of acromegaly, confirmed by the markedly elevated GH and insulin-like growth factor I (IGF-I) levels. Retrospective determination of the GH and IGF-I in samples collected at the time of the initial diagnosis showed comparable high values (Table 2). The patients’ visual fields were normal, but pituitary enlargement with chiasmatic compression was present on MRI of the sella (Fig. 4A).