Radiographic Exhibit
Ectopic ACTH Production and Mediastinal Lipomatosis 1 George F. Drasln, M.D., Theodore Lynch, M.D., and Gerald P. Ternes, M.D. The authors describe a case of mediastinal lipomatosis in a patient with ectopic ACTH production from carcinoma of the lung. Adrenals, hormones • Lipoma. Long neoplasms, 6 [0 ] .311 • Mediastinum. neoplasms (lipid deposition from steroids. 6[7].512)
INDEX TERMS :
Radiology
127:610, June 1978
HE postmortem findings in patients with Cush ing 's syndrome were described by Cushing in 1932 (4). Koerner and Sun (5) first described the roentgenologic findings in 3 patients with mediastinal fat deposition secondary to exogenous corticosteroids. Subsequent papers have dealt with mediastinal lipomatosis in patients with excess endogenous or exogenous corticosteroids (1,2,4,6-10). Mediastinal lipomatosis in a patient with ectopic ACTH production, though not reported previously to our knowledge, should not be unexpected. The following report describes such a case.
T
Fig. 1. Postero-anterior chest radiograph shows an uncalcified nodule in the right upper lobe. The upper mediastinumis widened, with iII-definedbut smooth borders. A small left apical fat pad is present.
CASE REPORT A 34-year-old white man first noticed leg weakness in January, 1976. This was followed by swelling of the face, neck, and stomach. The work-up was unrevealing except for erythrocytosis and ' mild hypertension. On retrospect, the chest radiograph showed minimal mediastinal widening and a small rightupper-lobe pulmonary nodule. Before his hospitalization in July, he noticed loss of scalp hair and libido, further facial swelling, and a tendency to bruise easily. On physical examination, his blood pressure was 170/110. His face was plethoric and moon-shaped, with swelling about the eyes . He had prominent adipose tissue on the trunk and neck and his extremities were wasted. Laboratory findings were: urinary 17-ketosteroids, 21 mg/24 hr. (normal, 9-22); urinary 17-ketogenic steroids, 35 mg/24 hr. (normal, 5-23); serum ACTH ; 188 pg/ml (normal, 0-100). Plasma cortisol was not suppressed by 48 hours of dexamethasone (2 mg every 6 hours). Fasting blood sugar was normal, but values of 268, 319 , and 144 mg/100 ml were recorded one, two, and three hours after the ingestion of glucose. A routine chest radiograph (Fig. 1) showed a nodule measuring 1.5 cm on the right upper lobe. The upper mediastinum was widened, with ill-defined borders. Tomography confirmed an uncalcified nodule and improved visualization of the mediastinal widening, more prominent on the left. A small fat pad was present at the left apex. Extensive exploration of the mediastinum through a right-sided med iastinotomy found only abundant fat tissue, with no normal nodes or neoplastic tissue. Five days later a right upper lobectomy was performed. Histological sections revealed a bronchial carcinoid with invasion of the surrounding alveoli. The peribronchial nodes contained small metastatic deposits. Three days after surgery his ACTH level fell to 23 pg/ml. Plasma cortisol, with the patient still on dexamethasone, was 5 mg/dl at 6:00 A.M. and 5.5 mg/dl at 11:00 P.M. Fourteen months after surgery he was tak ing no medications and was free of both cort isol excess and recurrent pulmonary tumor.
DISCUSSION Glucocorticoid excess, whatever its cause, can produce the centripetal obesity of Cushing's syndrome and mediastinal lipomatosis . Recognition of this as a cause of mediastinal widening is important in order to avoid diagnostic error and useless surgery . The radiographic features of such fat deposition (8, 10) include poor definition and greater lucency than other mediastinal masses with no tracheal distortion. Epipericardial fat pads may be the most reliable clue . Department of Radiology Jewish Hospital Louisville, Ky. 40202 REFERENCES 1. Bodman SF, Condemi JJ : Mediastinal widening in iatrogenic Cushing's syndrome. Ann Intern Med 67:399-403, Aug 1967 2. Cohen SL: The right pericardial fat pad. Radiology 60: 391-392, Mar 1953 3. Cushing H: The basophil adenomas of the pituitary bodyand their clinical manifestations (pituitarybasophilism). Bull JohnsHopkins Hosp 50:137-195, Mar 1932 4. FraserRG, PareJAP: Diagnosis of Diseasesof the Chest. An Integrated Study Basedon the AbnormalRoentgenogram. Philadelphia, Saunders, 1970. pp 558 and 1189 5. Koerner HJ, Sun DI-C: Mediastinal lipomatosis secondary to steroid therapy. Am J Roentgenol 98:461-464, Oct 1966 6. Price JE Jr, Rigler LG: Wideningof the mediastinum resulting from fat accumulation. Radiology 96:497-500, Sep 1970 7. Putte LBA van de. Wagenaar JP, San KH: Paracardiac lipomatosis in exogenous Cushing's syndrome. Thorax 28:653-656, Sep 1973 8. Santini LC, Williams JL: Mediastinal widening (presumably lipomatosis)in Cushing's syndrome. N EnglJ Med284:1357-1359, 17 Jun 1971 9. Strother CM: Cortisteroid-induced mediastinal widening in myasthenia gravis. Arch Neurol 32:702-703, Oct 1975 10. Teates CP: Steroid-induced mediastinal lipomatosis. Radiology 96:501-502 , Sep 1970
, From the Department of Radiology, Jewish Hospital (G.F.D.), and the Departments of Medicine (TL. Clinical Associate Professor, Section of Endocrinology) and Surgery (G.P.T., Clinical Associate Professor, Section of Thoracic Surgery), University of Louisville School of Medicine, Louisville. Ky. Accepted for publication in January 1978. sjh
610
Ectopic ACTH Production and Mediastinal Lipomatosis 1 George F. Drasln, M.D., Theodore Lynch, M.D., and Gerald P. Ternes, M.D. The authors describe a case of mediastinal lipomatosis in a patient with ectopic ACTH production from carcinoma of the lung. Adrenals, hormones • Lipoma. Long neoplasms, 6 [0 ] .311 • Mediastinum. neoplasms (lipid deposition from steroids. 6[7].512)
INDEX TERMS :
Radiology
127:610, June 1978
HE postmortem findings in patients with Cush ing 's syndrome were described by Cushing in 1932 (4). Koerner and Sun (5) first described the roentgenologic findings in 3 patients with mediastinal fat deposition secondary to exogenous corticosteroids. Subsequent papers have dealt with mediastinal lipomatosis in patients with excess endogenous or exogenous corticosteroids (1,2,4,6-10). Mediastinal lipomatosis in a patient with ectopic ACTH production, though not reported previously to our knowledge, should not be unexpected. The following report describes such a case.
T
Fig. 1. Postero-anterior chest radiograph shows an uncalcified nodule in the right upper lobe. The upper mediastinumis widened, with iII-definedbut smooth borders. A small left apical fat pad is present.
CASE REPORT A 34-year-old white man first noticed leg weakness in January, 1976. This was followed by swelling of the face, neck, and stomach. The work-up was unrevealing except for erythrocytosis and ' mild hypertension. On retrospect, the chest radiograph showed minimal mediastinal widening and a small rightupper-lobe pulmonary nodule. Before his hospitalization in July, he noticed loss of scalp hair and libido, further facial swelling, and a tendency to bruise easily. On physical examination, his blood pressure was 170/110. His face was plethoric and moon-shaped, with swelling about the eyes . He had prominent adipose tissue on the trunk and neck and his extremities were wasted. Laboratory findings were: urinary 17-ketosteroids, 21 mg/24 hr. (normal, 9-22); urinary 17-ketogenic steroids, 35 mg/24 hr. (normal, 5-23); serum ACTH ; 188 pg/ml (normal, 0-100). Plasma cortisol was not suppressed by 48 hours of dexamethasone (2 mg every 6 hours). Fasting blood sugar was normal, but values of 268, 319 , and 144 mg/100 ml were recorded one, two, and three hours after the ingestion of glucose. A routine chest radiograph (Fig. 1) showed a nodule measuring 1.5 cm on the right upper lobe. The upper mediastinum was widened, with ill-defined borders. Tomography confirmed an uncalcified nodule and improved visualization of the mediastinal widening, more prominent on the left. A small fat pad was present at the left apex. Extensive exploration of the mediastinum through a right-sided med iastinotomy found only abundant fat tissue, with no normal nodes or neoplastic tissue. Five days later a right upper lobectomy was performed. Histological sections revealed a bronchial carcinoid with invasion of the surrounding alveoli. The peribronchial nodes contained small metastatic deposits. Three days after surgery his ACTH level fell to 23 pg/ml. Plasma cortisol, with the patient still on dexamethasone, was 5 mg/dl at 6:00 A.M. and 5.5 mg/dl at 11:00 P.M. Fourteen months after surgery he was tak ing no medications and was free of both cort isol excess and recurrent pulmonary tumor.
DISCUSSION Glucocorticoid excess, whatever its cause, can produce the centripetal obesity of Cushing's syndrome and mediastinal lipomatosis . Recognition of this as a cause of mediastinal widening is important in order to avoid diagnostic error and useless surgery . The radiographic features of such fat deposition (8, 10) include poor definition and greater lucency than other mediastinal masses with no tracheal distortion. Epipericardial fat pads may be the most reliable clue . Department of Radiology Jewish Hospital Louisville, Ky. 40202 REFERENCES 1. Bodman SF, Condemi JJ : Mediastinal widening in iatrogenic Cushing's syndrome. Ann Intern Med 67:399-403, Aug 1967 2. Cohen SL: The right pericardial fat pad. Radiology 60: 391-392, Mar 1953 3. Cushing H: The basophil adenomas of the pituitary bodyand their clinical manifestations (pituitarybasophilism). Bull JohnsHopkins Hosp 50:137-195, Mar 1932 4. FraserRG, PareJAP: Diagnosis of Diseasesof the Chest. An Integrated Study Basedon the AbnormalRoentgenogram. Philadelphia, Saunders, 1970. pp 558 and 1189 5. Koerner HJ, Sun DI-C: Mediastinal lipomatosis secondary to steroid therapy. Am J Roentgenol 98:461-464, Oct 1966 6. Price JE Jr, Rigler LG: Wideningof the mediastinum resulting from fat accumulation. Radiology 96:497-500, Sep 1970 7. Putte LBA van de. Wagenaar JP, San KH: Paracardiac lipomatosis in exogenous Cushing's syndrome. Thorax 28:653-656, Sep 1973 8. Santini LC, Williams JL: Mediastinal widening (presumably lipomatosis)in Cushing's syndrome. N EnglJ Med284:1357-1359, 17 Jun 1971 9. Strother CM: Cortisteroid-induced mediastinal widening in myasthenia gravis. Arch Neurol 32:702-703, Oct 1975 10. Teates CP: Steroid-induced mediastinal lipomatosis. Radiology 96:501-502 , Sep 1970
, From the Department of Radiology, Jewish Hospital (G.F.D.), and the Departments of Medicine (TL. Clinical Associate Professor, Section of Endocrinology) and Surgery (G.P.T., Clinical Associate Professor, Section of Thoracic Surgery), University of Louisville School of Medicine, Louisville. Ky. Accepted for publication in January 1978. sjh
610
