A cancer-prone kindred with four siblings afflicted by aggressive poorly differentiated bronchogenic carcinoma

The A. kindred enumerated ten siblings: 2 females and 8 males. During the years 1982-1988 four brothers, all smokers, at their fifth or early sixth decade, presented with carcinoma of the lung. All patients had their primary lesion located in the right hemithorax, with predominance of the upper lobe ( 3 4). The clinical course was relentless with survival ranging 3-14 and 2-11 months from diagnosis and from initiation of treatment, respectively. Symptomatic mediastinal, particularly pericardial disease was common: significant pericardial effusion occurred twice, accompanied by atrial fibrillation in one case. Epicardial deposits occurred in another case. Response to cisplatin-based chemotherapy was minimal. Poorly differentiated carcinoma was the common histologic denominator with apparent presentation of subtypes (squamous-, adeno- and large cell) by haematoxylin and eosin staining. One case had, questionably, small cell carcinoma. Immunoperoxidase staining, however, for neuron-specific enolase was uniformly (3 cases examined) negative. In contrast, tumor tissue ( 3 3) stained positively by cytokeratin and epithelial membrane antigens, supporting the diagnosis of non-small cell bronchogenic carcinoma. In addition to the aforementioned brothers afflicted with lung cancer, a female sibling succumbed to a primary brain tumor. Two probands had chronic obstructive pulmonary disease (COPD), which was the cause of death of another brother. Present knowledge lends support to the presumption that the A. kindred was genetically predisposed to bronchogenic cancer either by exogenous carcinogens or due to another hereditary trait. Definite studies are required in order to test this hypothesis.