Fifteen cases of primary pulmonary Hodgkin's disease are reported. This presentation of Hodgkin's disease occurred more frequently in women (2:l) and older individuals, and B symptoms were relatively common (7 of 13). The majority of patients had nodular lesions on chest radiographs. An unfavorable prognosis was linked to B symptoms, older age (>60 years) and multiplicity and bilaterality of lung lesions. The histologic differential diagnosis is discussed, particularly in regard to Wegener's granulomatosis and non-Hodgkin's lymphomas.Cancer 57:12 17-1 224, 1986.
HEREAS TRACHEOBRONCHIAL and mediastinalW lymph node in~olvementl-~ is the most common intrathoracic manifestation of Hodgkin's disease (HD), pulmonary involvement has been observed in over 50% of cases in necropsy ~e r i e s .~*~-~ When identified, pulmonary parenchymal HD has been associated almost uniformly with nodal involvement in both roentgenographic and autopsy studies."39 Nonetheless, sporadic case reports of primary pulmonary HD (defined below) have ap~eared."-'~ Since the study of Kern et al. in 196 1 in which a total of 18 cases were re~iewed,'~ no large series of primary pulmonary HD has been reported. We describe the clinicopathologic features of 15 cases of primary pulmonary HD diagnosed by open-lung biopsy or resection in which there was an absence of demonstrable extrapulmonary HD. In addition, the histopathologic features of Accepted for publication June 12, 1985. pulmonary HD disease are reviewed with an emphasis on differential diagnosis.
Materials and MethodsWe reviewed all cases of pulmonary HD in the Surgical Pathology files of Stanford University Medical Center and the consultation files of Charles B. Carrington, MD. The cases chosen for further study fulfilled the following criteria for primary pulmonary HDl5-l9: (1) histologic features of HD; (2) restriction of the disease to the lung without hilar lymph node involvement; and (3) adequate clinical and/or pathologic exclusion of disease at distant sites. Thus, all patients who presented with mediastinal masses or confluent intrathoracic lymphadenopathy were excluded, as were cases with demonstrable HD at extrathoracic sites. We staged these cases according to a recently proposed system for intrathoracic lymphoma, as Stage IE: disease localized to the pulmonary parenchyma.I6The following information was sought in hospital records or requested from the referring pathologists or clinicians: age, sex, presenting symptoms, roentgenographic findings (particularly with regard to hilar, paratracheal, or mediastinal lymphadenopathy), staging studies, therapy, length to and site of relapse, and current status. Follow-up information was available for 14 of the 15 patients reported. The following histologic variables were evaluated classification of HD, pattern of infiltration, location of Reed-Sternberg cells and infiltrate in relation to pleura, character of cellular infiltrate, presence of sclerosis, necrosis, granulomas, vessel wall infiltration (qngiitis), bronchial cartilage and muco...