Adrenogenic Testicular Tumor

Jou1-u,L\L 01, Unor,ony Vol, 79, No. 3, 'viarch )958

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fJrintcd

V.8.A.

ADRENOGENIC TESTICULAR TUMOR ROBERT D. CROUCH ihe Department of Urology, Duke

Hospital, Durham,

School ol 1lfed1:cine and fhtke

. C.

·

This is the case of a testicular tumor with unusual physiological and patho logical characteristics. The tumor was unquestionably adrenogenic, but the classificatiou pathologically could not be determined. The clinical course and gross surgical findings are as follmvs: A three-year-old ·white rn_ale child was first admitted to Duke Hospital 01, Augn::;t J95Z) with the chief complaint of swelling of ihe left side of the scrotum of three months' duration. At the onset of the swelling, he had been treated his Joeal physician with parenteral penicillin to no avail. Jle was ultimately referred to Dnke Hospital with a diagnosis of probable malignancy of the left testiele. The tumor had doubl0d in size from the onset of the lesion until the time of ad mission. Previous and developmental history were entirely negative, a;-, ,Yai3 the The p0rtinent findings on physical examination 1rnre as follows. Blood pressure ranged from 135/90 to 150/100; pulse 104 to 120 per minnte; respirations 20 per minute; temperaturs on admission 37A centigrade. The patient was ,,·ell developed, obese and in no distress. SeveraJ diffm-m, .small, n:rncular lesions, some of which were pustular, were noted over Lhe shins. There was no evidence of regional or peripheral lymphadenopathy. Tbe heart was normal in and there was no evidence of abnormality on auscultation. The liver was palpable to 2 cm. below the right costal margin. Examination of the external genitalia revealed a norm.al uncircumcised penis. The right testicle and scrotal c:cmtents were normal. There ,Ya:s a large mass in the left scrotum about, the size of a large orange (G to 8 cn1. in diameter) which was firm, non-tender, and did not transilluminato light. The clinical diagnosis on admission \\·as 1) testicular tumor, 2) acne, secondary to hormonal imbalance, 3) hypcrteusio11, and 4) exogenous obesity and nutritional auP1nia, Figure 1 .,;hmm the original appearance of the patient. Laboratory findings revealed a hemoglobin of 9.7 gm. vVhitc blood count Yrns 6,400 with a normal differential. Urine and stool exaniinatiom, were norrnaL te8t for syphilis and the 0. T test, 1: 1000, were negative. Serurn calcium ,n1,'-' U.8 aud the carbon dioxide combining power was 2G rneq/L. Serum chloride was 104.:3 ; sodium was 143.3 and potassium 4 rneq/L. blood sugar was 8!) mg, per cent and two hour p. c. gluco;:;e wa:c; ] 05 mg. per cenL Eosinophil count ,rns 277 per cubic millimeter. The chest x-ra.y and plain x-ra.y of the abdomen ,Yere negati-s:e. Skull fihm, and x-ray of the right hand and foot revealed normal bones for his age. PreoperatiYe urinary steroid studies showed 17-ketosteroids of 18 mg./24 hours and 17-hydroxycorticoids of lG.7 mg./24 hours. Intravenous urogrnrns revealed prompt and nonnal excretion hilatora.lly with a. hifid pelvis and upper ureter on the left. Accepted for publication October 8, 1957. Prcse11:. nddress: 101 Office Bldg., Frederick, Mel.

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ROBER'l' D. CROUCH

Frn. 1. Adrenogenic testicular tumor

After one week in the hospital to complete the studies, the ehild was taken to the operating room and the left testicular mass was removed without difficulty. There was no attachment to the ,mrrounding tissue and the entire spermatic cord wm; removed to the internal inguinal ring. He received 250 cc blood during and after surgery, but 24 hmm, pm,toperatively his blood pressure began to drop and tachycardia and signs of shock appeared. He was girnn 200 mg. intravenous hydrocortisone with good response. The following day he was started on ACTH, 20 units every 12 hours and cortisone, 50 mg. intramuscularly every six hours. For the next five days the cortisone was tapered gradually and ACTH stopped six days postoperatively. His blood pressure stabilized around 130/90. An elevation of temperature occurred two days after surgery for \Yhich achromycin and penicillin were prescribed throughout the remainder of his hospital stay. Hemoglobin was 13.5 gm. on discharge. His acneform dermatitis became much worse, but cleared 8omewhat on calamine lotion. At the time of discharge the pathological diagnosis was incomplete other than an undifferentiated carcinoma of the testicle which flecreted adrenal hormoues. Pathological report of the excised Rpecimen was as follows: "The gross specimen is identified as the left testicle. It meai':iure,; approximately 9 by 6 by 6 cm. and has a rubbery, soft consistency. The sectioned surface has a lobular, yellowish, granular appearance. Some areas are soft and appear necrotic whereas other areas are somewhat morn firm. Representative sections of the specimen are submitted as blocks I-V. It should be stated that the testicular architecture on cut surface has been completely replaced by the aforedescribed tissue." "Microscopic: The left testicle has been essentially completely replaced by a highly cellular tumor ·which has been sepa,rated into nodules by numerous

ADRE!NOGENIC TESTICULAR TUMOR

629

fibrour, trabeculae. LU the periphery of the testicle areas are normal testicular seminiferous tubules are identified. The tumor nr,c.c.,c,nt" no particular picture . There are areas in which the tumor is composed of sheets of relatively uniform round cells which have for the most part ill-defined cellular borders and scant .,~,nu,uu,. The nuclei of these cells contain loosely and ,veU-defined borden:L In areas of this mitotic chromatin but figures are numerous. Fibrous stroma i:;; flcant. Other areas of the tumor are characterized by marked plemnorphism of the cell:o. Some of the cells are and vary from a rounded to elongated and fusiform strap cells. Some of these large cells contain hyperehromatic nuclei and acidophilie granular Other cells contain dear \·acuolated cytoplasm. Intermingled with these cells are many pleomorphic cells of much :omaHer size. The nuclei of these cells are hyperchromatic and well-defined. 11ito:;;es am numerous. N (nvhere in the tumor is there any pattern to the cellular structure. There are areas of degeneration and necrosis. Scattered throughout the tumor are numerous vacuoleR which :otain for fat. There is no specific cell ·IVhich makes specific elastoification impo:osihle." Figure 2 is a photomicrograph of a section through the tumor. On September 18-19 the urine 17-kctosteroid determinatiou was 1.2 hours, and tho 17-hydroxycorticoid,; I .2 mg./24 hourn. This was th1, first steroid determiuation foHmYing surgery. The patient was discharged from the hospital on September 19.55. He was follcnrnd closely in the out-patient (:linic, and 011 October 25-26 the 17-ketosteroids were 1.2 mg./24 hours and 17 xycorticoid" were 1.0 mg./24 hours. These were repeated for the foH01\·ing 24 hours and were uncha11ged. On .January 17, 1%6 thP urinary 17-ketosteroids

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ROBERT D. CROUCH

were 7.9 mg./24 hours, and the 17-hydroxycorticoids were 4.6 mg./24 hours. The striking rise in the steroid excretion during the preceding two months suggested the probability of metastatic malignancy. This was confirmed in May 1956, when a palpable mass was found in the left side of the abdomen. The patient was again admitted to Duke Hospital on May 21, following x-ray therapy at another hospital closer to his home. The blood pressure on admission was 150/120. The mass in the left side of the abdomen was easily palpable. The urinary 17-ketosteroids were 15.8 mg. and the 17-hydroxycorticoids were 9.0 mg./24 hours. A second intravenous urogram was interpreted as normal. A glucose tolerance test was normal and the eosinophil count was 88 per cubic millimeter. Skull x-rays were negative. The patient showed evidence of a sexual and somatic precocity to a degree which had not been seen on the first admission. Endocrine studies indicated a functioning, adrenal-like tumor that had not responded to x-ray therapy. After these preliminary laboratory studies, he was placed on intramuscular injections of cortisone acetate to prevent the adrenal insufficiency that occurred following removal of the primary tumor. On May 28 a retroperitoneal exploration was done, and a large tumor mass adjacent to the aorta and immediately below the lower pole of the left kidney was easily removed. There were numerous enlarged nodes along the aorta and the iliac vessels, several of which were removed for biopsy. At the time of surgery it was believed that the nodes were metastatically involved; however, permanent pathological sections showed that they contained no evidence of metastatic tumor. The large tumor mass contained no demonstrable lymphoid tissue, and was histologically identical with the original tumor of the left scrotum. Postoperatively, the child was treated with cortisone acetate, which was gradually diminished in doses and replaced with ACTH gel. The postoperative course was not remarkable. The blood pressure stabilized at about 100/70. The child was discharged on June 4, 1956 with the final pathological diagnosis still indefinite. The urinary 17-ketosteroids and 17-hydroxycorticoids were 2.8 mg. and 6.7 mg. per 13 hours, respectively, the day prior to discharge, indicating that there were probably other metastatic lesions present. The steroid excretions continued to rise through the last visit to Duke Hospital on September 7, 1956. Although the exact location of the metastatic lesions could not be determined at this time, it was believed that generalized carcinomatosis was present. It was later learned that the patient expired at his home on September 22, 1956. An autopsy was not obtained. DISCUSSION

The foregoing case report is obviously that of an adrenogenic testicular tumor, but of an unusual type from a pathological viewpoint. Dr. Earl T. Engle of Columbia University College of Physicians and Surgeons reviewed the case history and the microscopic slides, and the concensus of opinion of Dr. Engle and his associates was that the tumor must be classified as a very embryonic and undifferentiated teratocarcinoma. In view of the pathological characteristics, it is impossible to explain the increased steroid excretion. However, the patient ex-

ADRENOGENIC 'l'ESTICTJLAR TUMOR

hibited adrenal insufficiency following removal of the original testicular tumor. He responded to treatment with cortisone acetate and the prophylactic adminis .. tration prior to removal of the large retroperitoneal mass apparently prevented recurrence of adrenal insufficiency. It is also of interest to uote the absence of metastatic lesion in the enlarged nodes that were removed adjacent to the retro .. n1ass. SUMMARY

This has been the presentation of a rare and extraordinary testicular turnor, which ('.Ould only be classified as an undifferentiated carcinoma pathologicaUy with adrenogenic function. The author would like to thank others for their suggestions, and efforts in this case· namely, Dr. E. P. Alyea, professor of urology, Duke UniSchool of Medicine, Dr. :Frank L Engle and his Department of Endoand many 1nembers of the staff of the Department of Pediatries of Duke HospitaL