Pulmonary Hyalinizing Granuloma'·· PHILIP ENGLEMAN, AVERILL A. LIEBOW, JOHN GMELICH, and PAUL J. FRIEDMAN

SUMMARY ___________________________________~-------------------Twenty patients with pulmonary nodules cons1stmg of concentric hyaline lamellae, usually accompanied by perivascular collections of plasma cells and lymphocytes, were studied. In most instances, the lesions were multiple, bilateral, and mildly symptomatic. Many of these nodules showed all of the staining characteristics of amyloid, but others had an atypical birefringence pattern. No infectious agents were identified, and no consistent pattern of dysproteinemia was observed. Two of the patients had prior histories of tuberculosis. In other cases, the nodules were of unknown origin and pathogenesis. Four cases were complicated by sclerosing mediastinitis, and one, by retroperitoneal fibrosis and amyloidosis. Our current working hypothesis is that these lesions represent an exaggerated and, possibly, continuing immune response, perhaps to one of a number of agents.

Introduction Pulmonary hyalinizing granuloma is a syndrome that belongs in the differential diagnosis of pulmonary nodules of unknown origin. It is a puzzling disease process that simulates metastatic cancer radiographically and is often misinterpreted as a scar or healed infarct pathologically. The purpose of this paper is to describe the distinctive histologic architecture of pulmonary hyalinizing granuloma; to report the presentation and course of 20 patients with these nodules; and to raise some possibilities regarding its cause and pathogenesis.

years were reviewed. Twenty cases, morphologically subcategorized as "hyaline nodules," presented a remarkably uniform microscopic architecture. Histologic studies. Multiple sections were stained with hematoxylin and eosin, Gomori methenamine silver, Brown-Brenn stain, Ziehl-Neelsen stain for acid-fast bacilli, and Congo red stain for amyloid. Clinical and roentgenographic studies. Clinical questionnaires were sent to each referring physician; complete information was received for 90 per cent of the patients in the series. In addition, serial chest films were requested in all cases, with a response rate of approximately 50 per cent.

Materials and Methods Granulomatous pulmonary nodules of unknown cause received in consultation during the past 12

Clinical features of the cases of pulmonary hyalinizing granuloma are summarized in table l. The average age of presentation was 45 years. The youngest patient was 27 years of age; the oldest, 66 years of age. There was no significant sex or race predilection in the series. The typical patient was only mildly symptomatic or asymptomatic. Presenting symptoms included cough, hemoptysis, fatigue, fever, and pleuritic chest pain. Past medical history of these patients included 2 documented previous infections with pulmonary tuberculosis and one case diagnosed elsewhere as "'Wegener's granulomatosis." There

(Received in original form December 10, 1976 and in revised form March 15, 1977) 1 From the Department of Pathology, University Hospital, University of California Medical Center, San Diego, Calif. 92103. 2 Supported in part by Pulmonary Specialized Center of Research Grant HLI4169 and U. S. Public Health Service Cardiovascular Pathology Training Grant no. 5 TO! HL05858.

Results

AMERICAN REVIEW OF RESPIRATORY DISEASE, VOLuME 115, 1977

997

Rectal amyloidosis, S/P Dupuytren's contracture S/P, "Wegener's granulomatosis"

Multiple Multiple Multiple Solitary Multiple Solitary Multiple Multiple Solitary Multiple Multiple Multiple

Bilateral LU L, Ling, L hilum RML R hilum RML, RLL, R hilum LLL RML, RLL Bilateral RUL RUL, RML Bilateral Bilateral

Nodules Infiltrates Nodules NA Nodules Solitary nodule Infiltrates Nodules Infiltrate Nodules Nodules Nodules

Fever

None

Dyspnea, cough

NA

Hemoptysis, pleurisy

Cough, hemoptysis, pleurisy

NA

Fever, malaise

Cough. fatigue

Cough, pleurisy

None

NA

None

M

F

F

F

M

F

M

F

M

M

F

M

M

55

33

42

28

30

27

45

66

65

52

54

32

60

9

10

11

12

13

14

15

16

17

18

19

20

Definition of abbreviations: LLL; left lower lobe; S/P; status post; RUL; right upper lobe; PPD; purified protein derivative; LUL; left upper lobe; Ling; lingula; L hilum ; left hilum; NA; not available; RML; right middle lobe; R hilum; right hilum; R LL; right lower lobe; RU L; right upper lobe.

Positive reaction to PPD

Sclerosing mediastinitis

Sclerosing mediastinitis; positive cutaneous reaction to histoplasmin

Sclerosing mediastinitis; positive reaction to PPD

Multiple

Bilateral

8

Positive reaction to PPD

Multiple

Bilateral

Nodules Nodules

Cough, dyspnea

M

64

7

t"'

t= 0

Cor pulmonale

Multiple

Bilateral

Infiltrates

Dyspnea

F

58

6

Nodules

Hemoptysis, cough, pleurisy

M

30

5

Skin nodules showing "vasculitis"

Multiple

Bilateral

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