Intrathoracic Sympathoblastoma: Producing the Symptomatology of a Superior Pulmonary Sulcus Tumor (Pancoast)

Abstract

In 1932 Pancoast (1) described a tumor occurring at the thoracic inlet and characterized clinically by ( a ) pain about the shoulder, high in the axilla, or down the inner side of the arm or the ulnar side of the forearm, ( b ) Horner9s syndrome, ( c ) loss of power and wasting of the muscles of the hand, ( d ) x-ray evidence of a small, homogeneous shadow at the apex of the lung with destruction of the posterior parts of one or more ribs and often of the adjacent vertebrae. To the growth presenting such clinical and roentgenographic features Pancoast applied the name superior pulmonary sulcus tumor since “this term implies its approximate location, and a lack of origin from the lungs, pleura, ribs, or mediastinum.” Pancoast further concluded that tumors presenting this characteristic picture formed a definite pathological entity, the tumor being epithelial in its histopathology, although of uncertain origin. An origin from an embryonal epithelial rest, such as a fifth pharyngeal pouch, was suggested. Of the seven cases reported, biopsies were obtained in two. Case No. 1 was first thought to be an endothelioma of the pleura, but upon later consideration the diagnosis was changed to carcinoma spino-cellulare “with groups of prickle cells.” Case No. 2 was diagnosed simply as carcinoma. Two other patients—cases 6 and 7—each had a previous history of carcinoma of the cervix, one four years and the other two years previously, treated by radiation, and showing local disappearance of the tumor without recurrence. Both were considered to have primary superior pulmonary sulcus tumors. No pathological studies were made, however, so that the possibility that these were secondary growths cannot be eliminated. No biopsies or autopsies were secured in the remaining three cases, so that the nature of the tumors is not known.