The treatment of pheochromocytoma associated with pseudo-obstruction and perforation of the colon, hepatic failure, and DIC.

Abstract

The case of a 59-year-old man with paralytic ileus (pseudo-obstruction) associated with pheochromocytoma is reviewed. Paralytic ileus is believed to have been the result of overstimulation of a and /3 receptors on the intestine by catecholamines. Phentolamine, bunazocin, propranolol, bethanechol and midaglizole in single administrations or in combination were administered. Phentolamine infusion clearly relieved the symptom, but ileus recurred, and the patient died of respiratory failure, liver dysfunction and disseminated intravascular coagulation syndrome. The significant role of catecholamines in causing these symptoms is discussed, and the management of this relatively rare complication is reviewed. Recently we treated a case of severe intestinal distension in a pheochromocytoma patient with multiple metasases. There is, as yet, no definite treatment regimen for paralytic ileus, thus the clinical course and autopsy findings of this case are presented. CASE REPORTA 59-year-old man, brought by ambulance, was admitted to our department because of severe constipation, abdominal distension and one week of jaundice. A diagnosis of malignant pheochromocytoma with multiple metastases had been made previously. The clinical history of the patient from the age of 38 is shown in Table 1. He was immediately transferred to the Intensive Care Unit of our hospital.The patient was drowsy, blood pressure was 130/90 mmHg, and pulse rate was 120/min. Skin and conjunctiva bulbi were icteric. No rale was audible and heart sounds were clear. The abdomen was quite distended, and tympanic. Livedo racemosa was present bilaterally in the upper and lower extremities.Chest X-rays revealed bilateral pleural effusion, enlargement of the heart shadow, and a mass in the right upper field which was thought to be a metastatic lesion of the third rib. Plain films of the abdomen (Fig. 1) demonstrated a distended intestine, but no mechanical obstruction of the colon was found in the endoscopic examination. Echo-

THERAPY OF ENDOCRINE DISEASE: Treatment of malignant pheochromocytoma and paraganglioma

“…Gastrointestinal bleeding and perforation have also been described in patients treated with tyrosine kinase inhibitors (27). Treatment for constipation may include oral fiber supplements, osmotic agents (e.g., lactulose and polyethylene glycol), lubricants and emollients (e.g., mineral oil and docusate), gastrointestinal stimulants (e.g., bisacodyl), a-blockers, a-methyl-para-tyrosine, and/or enemas (28). If constipation persists despite these measures, ileostomy is recommended (29).…”

Malignant pheochromocytoma associated with pseudo-obstruction of the colon

“…2,3 In patients with multiple metastases (as with our patient), the reduction of catecholamine levels is 4 Hashimoto et al, 5 Thurtle et al, 6 and Williams and Palmer 7 carried out treatments that primarily consisted of administering adrenaline receptor blocking agents, because they believed that intestinal obstruction would be relieved if the effects of catecholamines on the smooth muscle of the intestine were reversed.…”

“…This patient was switched to oral administration of an adrenergic blocking agent and had no recurrence during the following year. However, two other patients, reported by Hashimoto et al 5 and Thurtle et al, 6 died without having been switched from the phentolamine treatment.…”

“…2,3 In malignant cases, however, the treatment of intestinal symptoms is challenging because it is difficult to control the levels of catecholamines secreted from the metastatic foci of the tumor mass. [4][5][6][7][8][9][10] We report a case of malignant pheochromocytoma complicated by severe intestinal pseudo-obstruction in a patient in whom conservative treatment failed but surgical intervention was effective.…”

Constipation: an overlooked, unmanaged symptom of patients with pheochromocytoma and sympathetic paraganglioma

“…Among these, gastrointestinal manifestations may account for significant morbidity and mortality. Indeed, there are several case reports of PH/PG patients who have had severe constipation that resulted in paralytic ileus, bowel ischemia, gastrointestinal bleeding, and even colonic perforation (1,2,3,4,5).…”