Phenochromocytoma with large cystic calcification and associated sphenoid ridge malformation.

Abstract

Pheochromocytoma need not be considered a rare neoplasm since it is seen with relative frequency in nearly every large tumor clinic. Calcification within a pheochromocytoma, however, is quite unusual, and its occurrence in the region of the adrenal glands rarely leads to suspicion of such a tumor. We have been able to collect only 16 published cases in which calcium was radiographically demonstrable (2, 8, 15). The following case is the only one described to date with large cystic calcification. The concurrent presence of other radiographic stigmata of neuroectodermal dysplasia will be described and discussed. Case Report2 A 74-year-old white male was admitted to the private medical service of the Presbyterian Hospital of Pittsburgh Sept. 4, 1959, because of intermittent diffuse lower abdominal pain associated with palpitation and headaches, which had been present for approximately fifteen years. Two months prior to admission, the episodes of pain became increasingly frequent and severe and were at times associated with vomiting. They were not accompanied by chills, fever, or changes in color of skin, feces, or urine. Additional historical, familial, and social data were not relevant. Several previous studies elsewhere had failed to yield a satisfactory diagnosis. The patient appeared well developed, well nourished, and in no acute distress. The pulse rate was 100 and the blood pressure recorded on admission was 120/80. The remainder of the physical examination was not remarkable. During the initial radiologic examination, shortly following admission, the patient fainted and fell to the floor, striking his head. Upon questioning, he admitted to frequent similar episodes at home, characterized by total unawareness of the fall. Films of the skull were obtained and will be discussed below. Regular and frequent blood-pressure recordings subsequent to this episode revealed fluctuations of the arterial pressure from 60/0 to 230/120. An electrocardiogram following the syncope was interpreted as indicative of “possible ectopic atrial pacemaker” and “nonspecific RST segment changes possibly associated with old coronary disease.” The hematologic and serologic data on admission were within normal limits except for a mild polymorphonuclear leukocytosis and a slight increase of hemoglobin concentration to 17.6 mg. per 100 ml. Radiographic examination of the skull (Fig. 1) showed gross asymmetry of the calvarium with elevation of the left petrous pyramid and attenuation of the left sphenoid ridge markings. In addition, the orbits were quite asymmetrical. These findings were interpreted as indicative of neurofibromatosis with calvarial dysgenesis. Films of the chest were within normal limits for the patient's age.