Thorax (1971), 26, 296.

Unilateral hilar or paratracheal adenopathyin sarcoidosis: a study of 38 cases

RICHARD W. SPANN, EDWARD C. ROSENOW, III,RICHARD A. DEREMEE, and W. EUGENE MILLER

Mayo Clinic, Rochester, Minnesota

The diagnosis of pulmonary sarcoidosis should be considered when unilateral hilar enlargementor a paratracheal mass is present. With this diagnosis in mind, a scalene node biopsy ormediastinoscopy may prevent unnecessary thoracotomy. It is believed that the unilateral stageis only an evanescent stage before the development or regression of bilateral hilarlymphadenopathy.

Sarcoidosis is a disease of unknown cause that ischaracterized by the presence of non-caseousgranuloma. The diagnosis is made whenever thishistological finding occurs in the absence of otherknown causes. of non-caseating granulomas andwhenever compatible clinical and radiographicfindings are present. The usual chest radiographicfindings in sarcoidosis are (1) bilateral hilar andoccasionally right paratracheal adenopathy,(2) thoracic lymphadenopathy and pulmonarydensities, (3) pulmonary densities without lympha-denopathy, and (4) predominant pulmonaryfibrosis.

Occasionally, the clue as to the possible clinicaldiagnosis of pulmonary sarcoidosis is the presenceof bilateral hilar lymphadenopathy. Sarcoidosis isnot usually considered in the differential diagnosisof unilateral hilar enlargement or of right para-tracheal adenopathy alone, but it should be.

MATERIAL AND FINDINGS

A review of the chest radiographs in 800 histo-logically diagnosed cases of sarcoidosis at the MayoClinic from 1960 through 1969 disclosed 38 cases inwhich the initial unbiased radiographic interpretationwas unilateral right hilar enlargement (22 patients),left hilar enlargement (12 patients), or right para-tracheal mass (4 patients). A right paratracheal masswas noted in six of the patients with unilateral hilarlymphadenopathy; thus, a total of 10 of the 38patients had right paratracheal adenopathy. Of the800 patients whose chest radiographs were reviewed,472 had mediastinal adenopathy. Therefore, approxi-mately 8% of mediastinal lymphadenopathy presentedunilaterally.The differential diagnosis most commonly included

lymphoma or bronchogenic carcinoma and, less

frequently, sarcoidosis. The diagnosis was confirmedin all cases; cultures for acid-fast bacilli and fungiwere negative in all cases. In retrospect, in some ofthe radiographs, the opposite hilus appearedsuspiciously involved but was not considered so untilafter the diagnosis had been confirmed histologically.Patients considered initially to have bilateral hilarinvolvement were not included in this study.

Positive histological confirmation was obtained byscalene node biopsy in 26 cases, by thoracotomy in8, by mediastinoscopy in 2, and by bronchoscopyand muscle biopsy in 1 each. Follow-up radiographswere available from four months to six years in 26cases. The disease remained stationary in three, pro-gressed to bilateral involvement in three, andregressed to normal in 20. A purified protein deri-vative test was done in 24 patients, and four hadpositive reactions. Six patients presented witherythema nodosum.

THREE REPRESENTATIVE CASES

CASE 1 A 59-year-old man had evidence of a righthilar mass on a routine chest film (Fig. Ia) and wasreferred to the Mayo Clinic for further evaluation.He was asymptomatic, except for residuals of a recentchest cold, and there were no abnormal findings onphysical examination. Results of routine laboratorystudies were within normal limits. In retrospect, theleft hilus was suspiciously involved but was not con-sidered so until after the diagnosis had been estab-lished at thoracotomy. Thoracotomy was preceded bynegative bronchoscopy and negative scalene nodebiopsy. Cultures for acid-fast bacilli and fungi fromthe removed lymph nodes were also negative. Atomogram of the right hilus showed evidence of themass (Fig. lb).

CASE 2 A 56-year-old woman came to the MayoClinic for evaluation of an adenomatous goitre.

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ilateral hilar or paratracheal adenopathy in sarcoidosis: a study of 38 cases 297

(a)

(b)

FIG. 1. Case 1 (a) Chest radiograph showing evidence ofright hilar mass. Left hilus,in retrospect, is suspiciously involved. (b) Tomogram of right hilar mass.

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FIG. 2. Case 2. Chest radiograph showing evidence of left hilar mass.

FIG. 3. Case 3. Chest radiograph showing evidence of right paratracheal mass.Lateral chest radiograph disclosed that this mass was in the middle mediastinum.

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Unilateral hilar or paratracheal adenopathy in sarcoidosis: a study of 38 cases

Evidence of a left hilar mass was found on a routinechest film (Fig. 2). A colloid goitre was removed atthyroidectomy, at which time the surgeon alsoremoved lymph nodes from the upper mediastinum,which showed non-caseous granuloma. A chest radio-graph four months later revealed no change in theleft hilar mass.

CASE 3 A 29-year-old woman had evidence of aright paratracheal mass on a chest film taken forevaluation of a mild cough of six months' duration(Fig. 3). Results of physical examination were nega-tive, with the exception of mild hypertension. A lowhaemoglobin level of 11-5 g/100 ml was consideredto be due to an unrelated iron-deficiency anaemia.The serum calcium level was raised on threeoccasions, being 10-4, 10-1, and 10-5. mg/100 ml(normal 8-9 to 10-1 mg). One year later the serumcalcium level was 9-9 mg/100 ml. Tissue removed atmediastinoscopy showed non-caseous granuloma. Allcultures were negative. A purified protein derivativetest with 250 tuberculin units and histoplasmin skintests were negative. A chest radiograph one year laterrevealed that the mass had almost completely dis-appeared.

DISCUSSION

Recently, Kent (1965) stressed the importance ofconsidering sarcoidosis in the differential diag-nosis of unilateral hilar lymphadenopathy, alongwith tuberculosis, lymphoma, bronchogenic carci-noma, and metastatc carcinoma. His reportdescribed a patient in who in unilateral left hilarlymphadenopathy regressed and was followedthree years later by development of right hilarlymphadenopathy. Some authors have emphasizedthat hilar enlargement may be unilateral in sar-coidosis (Gendel, Young, and Greiner, 1952;Kerley, 1956; Longcope and Freiman, 1952;Turiaf, 1964). There are many other reports of oneto four patients with unilateral hilar involvement(Citron, 1958; Hedvall, 1960; James, 1961;Lofgren, 1953; Miech, Morand, Janser, Reys,and Witz, 1965; Moyer and Ackerman, 1950-;Nitter, 1953; Rudberg-Roos, 1962; Scadding,1950; Siltzbach, 1955; Susmano and Carleton,1970; Williams, 1961). Most of these reports areone part of a large series of patients with sar-coidosis. Ellis and Renthal (1962) noted 13 patientswith unilateral hilar lymphadenopathy out of 76patients with hilar lymphadenopathy. In many ofthe patients bilateral hilar lymphadenopathy sub-sequently developed.

It is believed that hilar lymphadenopathy is thefirst stage of development of pulmonary sar-coidosis; not unlikely, the lympadenopathy beginsas or regresses to a unilateral or an asymmetricstage.

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