Case Report

Testicular Ultrasound in Carney Complex:Report of Three Cases

Ahalya Premkumar, MD,1 Constantine A. Stratakis, MD, DSc,2

Thomas H. Shawker, MD,1 Dimitris A. Papanicolaou, MD,2

George P. Chrousos, MD2

1 Diagnostic Radiology Department, Warren Grant Magnuson Clinical Center,National Institutes of Health, 10 Center Dr., Bethesda, Maryland 20892

2 Developmental Endocrinology Branch, National Institute of Child Health andHuman Development, National Institutes of Health, Bethesda, Maryland

Received 11 April 1996; accepted 4 November 1996

Carney complex is a familial multiple neoplasia tours in both testes. Several of these werechunky and large, measuring up to 1 cm in diam-syndrome first described in 1985.1 It describes

the association of pigmented lesions of the skin eter. Almost all of them were associated withacoustic shadowing. The patient did not have(lentigines and blue nevi) with a variety of myxo-

matous and endocrine tumors, including uterine any symptoms referable to the testes, nor wereany masses palpated on physical examination.myxoid tumors, and, in men, large-cell Sertoli

calcifying tumors of the testes.2 These testiculartumors are benign and, as they do not require Patient 2orchiectomy, must be differentiated from other

A 42-year-old man, maternal uncle of patient 1,neoplasms occurring in the gonads at a younghad a right atrial myxoma removed at the age ofage. In the present report, the sonographic find-16 years. He presented with dysphagia, causedings of three individuals with Carney complexby an intramural mass in the esophagus, whichare described. Multiple echogenic masses withwas found at surgery to be a psammomatouschunky calcifications are seen bilaterally in themelanotic schwannoma. A scrotal ultrasoundtestes. This appearance seems to be distinctiveshowed bilateral echogenic homogeneous massesfor this disorder. A review of findings in Carneyin both testes, ranging in size from a few milli-complex is also made.meters to 1 cm in diameter (Figure 2). All thelarger ones and most of the smaller ones showed

CASE REPORTS acoustic shadowing. These lesions were not de-tectable on physical examination.

Patient 1Patient 3A 20-year-old white man presented with a 2-year

history of symptoms compatible with acromeg- A 31-year-old man first presented at the age of 7aly. Physical examination revealed lentigines with precocious puberty. He developed normally,and nevi on the face, arms, and back. An echo- except for short stature, until the age of 19 whencardiogram showed dilatation of both ventricles he was found to have signs and symptoms ofbut no evidence of a myxoma. An MRI of the sella Cushing’s syndrome. A left adrenal mass wastunica showed a macroadenoma of the pituitary. seen on workup and was excised. Pathology re-Scrotal sonography (Figure 1) showed multiple vealed pigmented micronodular hyperplasia ofechogenic homogeneous masses with smooth con- the adrenal gland. At follow-up 12 years later,

the patient was noted to have a tender mass inthe right testis. A scrotal ultrasound revealed

Correspondence to: A. Premkumar multiple echogenic masses in both testes. TheseJ Clin Ultrasound 25:211–214, May 1997 measured up to 1.8 cm in diameter, with acoustic

shadowing seen from the larger masses (Figure 1997 John Wiley & Sons, Inc. CCC 0091-2751/97/040211-04

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A

B

FIGURE 1. Sonograms of the right (A) and left (B) testes show multiple echogenic masses with acousticshadowing from several of them (arrows).

3). The patient also had multiple pigmented len- 1 was 12 years old and had only the skin lesions,and patient 2 was not available for evaluation.tigines on the face and upper body.

FAMILY HISTORY DISCUSSION

Carney complex was first described as an inde-Two of these patients belong to a family of fourgenerations with Carney complex who were the pendent, autosomal dominant syndrome of mul-

tiple endocrine neoplasias, myxomas, and spottysubject of a report in 1987.3 At that time, patient

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CARNEY COMPLEX

A

B

FIGURE 2. (A,B) Longitudinal sonograms of the testes show bilateral echogenic masses (arrows) with acoustic shadowing.

skin pigmentation by Carney et al in 1985.1 Myx- The characteristic testicular lesion in the Car-ney complex is the large cell calcifying Sertoliomas may be intracardiac, cutaneous, or mam-

mary. The endocrine component of the complex cell tumor (LCCS), although Leydig cell tumorsmay also be seen. These may be asymptomatic orincludes primary pigmented adrenocortical dis-

ease (PPNAD) leading to Cushing’s syndrome, (rarely) cause sexual precocity.4 Testicular tu-mors are seen in about half of the male patientstesticular tumors, growth hormone-producing pi-

tuitary adenomas, thyroid nodules, and breast with the complex. They are often bilateral andmulticentric and associated with calcificationsneoplasms. Psammomatous melanotic schwanno-

mas are rare tumors which are also part of the and the absence of a significant soft tissue com-ponent.5–7 The mean age of occurrence is 14complex.

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FIGURE 3. Longitudinal sonogram of the testis shows large echogenic masses with acoustic shadowing.

years. The tumors are often felt as hard non- these masses are relatively benign. Routinephysical examination and ultrasonography aretender masses, but frequently they are asymp-

tomatic.2 They are of low malignant potential. recommended. Precocious puberty may occur asa result of HCG-producing testicular tumors inSonographically, bilateral testicular tumors

may be seen in lymphoma and leukemia and prepubertal children. In this case, surgery andmedical treatment of precocious puberty are rec-with adrenal rest tumors. These tumors, how-

ever, present as focal and predominantly hypo- ommended.echoic masses, and it is unusual to see calci-fications in them. Testicular tumors withcalcifications include teratomas, teratocarcino-

REFERENCESmas, and embryonal cell carcinomas. The calcifi-1. Carney JA, Gordon H, Carpenter PC, et al: Thecations are, however, spotty, and the tumors

complex of myxomas, spotty pigmentation and en-tend to be unilateral. Testicular microlithiasisdocrine overactivity. Medicine 64:270–283, 1985.can be seen to involve both testes, but again, the

2. Carney JA: The Carney complex (myxomas, spottycalcifications are small and no associated masspigmentation, endocrine overactivity and schwan-is seen. The sonographic appearance of multiplenomas). Dermatological Clinics 13:19–26. 1995.homogeneous echogenic masses with a smooth 3. Danoff A, Jormark S, Lorber D, Fleischer N: Adre-

contour and acoustic shadowing together with nocortical micronodular dysplasia, cardiac myxo-absence of an associated soft tissue component mas, lentigines, and spindle cell tumors. Arch In-seen bilaterally in the testes is unique for LCCS tern Med 147:443–448, 1987.tumors and should be recognized. 4. Stratakis CA, Carney A, Lin JP, et al: Carney com-

plex, a familial multiple neoplasia and lentiginosisIf LCCS tumors are seen on scrotal sonogra-syndrome: analysis of 11 kindreds and linkage tophy, the patient should also be evaluated forthe short arm of chromosome 2. J Clin Investother components of Carney complex, especially97:699–705, 1996.the cardiac myxomas which cause the most seri-

5. Gierke CL, King B, Bostwick DG, et al: Large-cellous consequences in this disease. Likewise, in acalcifying Sertoli cell tumor of the testis: appear-patient diagnosed with Carney complex, testicu-ance at sonography. AJR 163:373–375, 1994.lar ultrasound is routinely recommended because 6. Shawker TH, Doppman JL, Choyke PL, et al: In-

the masses are often not palpable. Following the tratesticular masses associated with abnormallyidentification of characteristic homogeneous functioning adrenal glands. J. Clin Ultrasoundround echogenic masses with acoustic shadowing 20:51–58, 1992.in the testes in a patient with Carney complex, a 7. Radin R, Kempf RA: Carney complex: report of

three cases. Radiology 196:383–386, 1995.conservative approach is recommended since

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