Vol. 113, May Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright © 1975 by The Williams & Wilkins Co.
Single Case Reports ABERRANT ADRENAL TISSUE: AN INCIDENTAL FINDING DURING ORCHIOPEXY ARTHUR E. FELDMAN, RONALD S. ROSENTHAL
AND
JOHN L. SHAW
From the Department of Surgery, Division of Urology, Abington Memorial Hospital, Abington, Pennsylvania
ABSTRACT
Aberrant adrenal tissue is a not uncommon clinical observation. The embryology of the adrenal gland is briefly reviewed and the common origin of adrenal and gonadal tissue is emphasized to account for the sporadic appearance of adrenal rests associated with gonadal structures. A listing of the diverse sites of ectopic adrenal tissue is offered with mention of the significant clinical implications. Another case report of an adrenal rest in the spermatic cord is presented. In 17 40 Morgagni was the first to recognize the characteristic yellowish nodules of the adrenal in the near vicinity of the gland. 1 Several others later confirmed Morgagni's initial observations and, subsequently, there have been numerous reports of aberrant adrenal tissue in diverse locations. Recently, in the course of a routine orchiopexy for a congenital undescended testicle, we curiously encountered an adrenal rest on the spermatic cord. CASE REPORT
J. M., an 8-year-old white boy, was admitted to the urology service with a history of an undescended left testis since birth. The patient was the product of a normal vaginal delivery without complication and free of any significant medical or surgical history. Physical examination revealed a well developed, well nourished white boy with normal temperature, pulse and blood pressure. The patient was circumcised and examination of the genitalia demonstrated absence of the left testis in the scrotum and underdevelopment of the left hemi-scrotum. There was a barely palpable mass in the left inguinal region, possibly compatible with a testicle. The hemoglobin was 13.5 mg. per cent and the hematocrit was 36 per cent. The white blood count and differential were normal. Urinalysis was negative. The day following admission to the hospital the patient underwent exploration of the left inguinal region. A small indirect hernial sac was located and high ligation was accomplished in the usual fashion. A testis, somewhat smaller than its normal-sized counterpart, was identified in the mid inguinal canal and an orchiopexy, using a Dartos pouch, was performed. Of interest, a small golden yellow nodule was noted within a fragment of fibrofatty Accepted for publication August 16, 1974. 706
tissue adjacent to the spermatic cord. This nodule was excised and was thought to be a lipoma of the cord. Pathologic diagnosis of this specimen was ectopic adrenal cortical tissue. Microscopically, the nodule was composed of cords of uniform round epithelial cells with finely vacuolated and eosinophilic cytoplasm and small round nuclei (see figure). The nodule was golden yellow and 0.3 cm. in its greatest dimension. An excretory urogram 2 days postoperatively was normal. Convalescence was uneventful and the patient was discharged from the hospital 3 days postoperatively. DISCUSSION
The embryogenesis of the adrenal gland is well known and is important to the understanding of aberrant adrenal tissue. The adrenal gland has a double origin, arising from ectodermal elements (adrenal medulla) and mesodermal elements (adrenal cortex). The adrenal cortical substance, first noticeable at the 6 mm. size, or the beginning of the fourth week, ". . . arises from dorsal cells of the blastema cords at the medial borders of the mesonephric bodies, while ventral cells of the same region give rise to testicular interstitial cells or ovarian theca cells". 2 Aberrant adrenal glands or accessory adrenals are formed then when, at about the 7 to 8-week stage of development, the medullary components migrate into the cortical elements and "multiple primordia or secondarily separate fragments of the parent gland may split off'. 3 Generally these accessory glands are composed of cortical tissue only when formed at a distance removed from the parent gland, while those closer to the adrenal gland may contain medullary substance as well. Usually the accessory glands remain in geographic juxtaposition to their
ABERRANT ADRENAL TISSUE
707
Photomicrograph of adrenal cortical tissue. A, reduced from x 100. B, reduced from x 430
original site but it is logical that they may migrate down to the pelvis or scrotum in concert with the genital gland structures because of their common embryologic origin. According to Schechter accessory adrenals theoretically may exist with no parent adrenals in their usual anatomic location but this rarely occurs. Normally, " ... the accessories either involute entirely in early life or persist through adulthood". Adrenal cortical rests are said to be present in approximately 50 per cent of newborns but usually atrophy and disappear within a few years. 3 Because the myriad of foci of aberrant adrenal tissue are too numerous to elaborate in this text, they will simply be mentioned. O'Crowley and Martland found 8 cases of aberrant adrenal gland within the substance of the renal parenchyma in 5,000 consecutive autopsies.• Culp reported on 21 cases of true adrenal-renal heterotopia and presented a postmortem case of his own. 5 Also Mitchell and Angrist reported additional examples of adrenal rests in the kidney, further substantiating the predilection of the aberrant adrenal tissue for the kidney. 6 Schechter cited references to the many different foci of ectopic adrenal tissue: the broad ligament,
spermatic and ovarian vessels, spermatic cord, canal of Nuck and uterus. Abdominal structures containing aberrant adrenal substance have included pancreas, transverse colon, celiac plexus and capsule of the liver. He also noted several references to accessory tissue encountered in structures around the testis, that is epididymis and tunica albuginea. 3 Fifty-five years ago, MacLennan reported on finding nodules of adrenal tissue within 6 hernia sacs removed from 700 children, 600 of whom were boys. 7 Recently, Mininberg and Dattwyler presented a case of ectopic adrenal tumor presenting as torsion of the spermatic cord in a newborn infant. 8 For completeness the clinical implications of aberrant adrenal tissue must be cited. It has been adequately documented that adrenal accessories undergo compensatory hypertrophy and function after adrenalectomy for Cushing's syndrome, and advanced breast and prostatic carcinoma. Also, adrenal rests may be removed inadvertently during any one of a number of surgical procedures, resulting in adrenal insufficiency. For this reason, the clinician must be cognizant of the varied locations of aberrant adrenal tissue. Because of the acces-
708
FELDMAN, ROSENTHAL AND SHAW
sories' predilection for the kidney, " ... the accidental removal of heterotopic adrenal tissue is most likely to occur during nephrectomy". • And lastly, these aberrant implants of adrenal tissue may themselves become neoplastic, whether benign or malignant, and may be responsible for definite endocrinopathies observed clinically. REFERENCES
1. Morgagni, G. B.: Cited by Schechter.• 2. Glenn, J. F. and Boyce, W. H.: Urologic Surgery. New York: Harper & Row, Publishers, Inc., p. 9,
1969.
3. Schechter, D. C.: Aberrant adrenal tissue. Ann. Surg.,
167: 421, 1968.
4. O'Crowley, C.R. and Martland, H. S.: Adrenal heterotopia, rests, and the so-called Grawitz tumor. J. Urol., 50: 756, 1943. 5. Culp, 0. S.: Adrenal heterotopia. A survey of the literature and report of a case. J. Urol., 41: 303, 1939.
6. Mitchell, N. and Angrist, A.: Adrenal rests in the kidney. Arch. Path., 35: 46, 1943. 7. MacLennan, A.: On the presence of adrenal rests in the walls of hernial sacs. Surg., Gynec. & Obst., 29: 387, 1919.
8. Mininberg, D. T. and Dattwyler, B.: Ectopic adrenal tumor presenting as torsion of the spermatic cord in a newborn infant. J. Urol., 109: 1037, 1973.
THE JOURNAL OF UROLOGY
Copyright © 1975 by The Williams & Wilkins Co.
Single Case Reports ABERRANT ADRENAL TISSUE: AN INCIDENTAL FINDING DURING ORCHIOPEXY ARTHUR E. FELDMAN, RONALD S. ROSENTHAL
AND
JOHN L. SHAW
From the Department of Surgery, Division of Urology, Abington Memorial Hospital, Abington, Pennsylvania
ABSTRACT
Aberrant adrenal tissue is a not uncommon clinical observation. The embryology of the adrenal gland is briefly reviewed and the common origin of adrenal and gonadal tissue is emphasized to account for the sporadic appearance of adrenal rests associated with gonadal structures. A listing of the diverse sites of ectopic adrenal tissue is offered with mention of the significant clinical implications. Another case report of an adrenal rest in the spermatic cord is presented. In 17 40 Morgagni was the first to recognize the characteristic yellowish nodules of the adrenal in the near vicinity of the gland. 1 Several others later confirmed Morgagni's initial observations and, subsequently, there have been numerous reports of aberrant adrenal tissue in diverse locations. Recently, in the course of a routine orchiopexy for a congenital undescended testicle, we curiously encountered an adrenal rest on the spermatic cord. CASE REPORT
J. M., an 8-year-old white boy, was admitted to the urology service with a history of an undescended left testis since birth. The patient was the product of a normal vaginal delivery without complication and free of any significant medical or surgical history. Physical examination revealed a well developed, well nourished white boy with normal temperature, pulse and blood pressure. The patient was circumcised and examination of the genitalia demonstrated absence of the left testis in the scrotum and underdevelopment of the left hemi-scrotum. There was a barely palpable mass in the left inguinal region, possibly compatible with a testicle. The hemoglobin was 13.5 mg. per cent and the hematocrit was 36 per cent. The white blood count and differential were normal. Urinalysis was negative. The day following admission to the hospital the patient underwent exploration of the left inguinal region. A small indirect hernial sac was located and high ligation was accomplished in the usual fashion. A testis, somewhat smaller than its normal-sized counterpart, was identified in the mid inguinal canal and an orchiopexy, using a Dartos pouch, was performed. Of interest, a small golden yellow nodule was noted within a fragment of fibrofatty Accepted for publication August 16, 1974. 706
tissue adjacent to the spermatic cord. This nodule was excised and was thought to be a lipoma of the cord. Pathologic diagnosis of this specimen was ectopic adrenal cortical tissue. Microscopically, the nodule was composed of cords of uniform round epithelial cells with finely vacuolated and eosinophilic cytoplasm and small round nuclei (see figure). The nodule was golden yellow and 0.3 cm. in its greatest dimension. An excretory urogram 2 days postoperatively was normal. Convalescence was uneventful and the patient was discharged from the hospital 3 days postoperatively. DISCUSSION
The embryogenesis of the adrenal gland is well known and is important to the understanding of aberrant adrenal tissue. The adrenal gland has a double origin, arising from ectodermal elements (adrenal medulla) and mesodermal elements (adrenal cortex). The adrenal cortical substance, first noticeable at the 6 mm. size, or the beginning of the fourth week, ". . . arises from dorsal cells of the blastema cords at the medial borders of the mesonephric bodies, while ventral cells of the same region give rise to testicular interstitial cells or ovarian theca cells". 2 Aberrant adrenal glands or accessory adrenals are formed then when, at about the 7 to 8-week stage of development, the medullary components migrate into the cortical elements and "multiple primordia or secondarily separate fragments of the parent gland may split off'. 3 Generally these accessory glands are composed of cortical tissue only when formed at a distance removed from the parent gland, while those closer to the adrenal gland may contain medullary substance as well. Usually the accessory glands remain in geographic juxtaposition to their
ABERRANT ADRENAL TISSUE
707
Photomicrograph of adrenal cortical tissue. A, reduced from x 100. B, reduced from x 430
original site but it is logical that they may migrate down to the pelvis or scrotum in concert with the genital gland structures because of their common embryologic origin. According to Schechter accessory adrenals theoretically may exist with no parent adrenals in their usual anatomic location but this rarely occurs. Normally, " ... the accessories either involute entirely in early life or persist through adulthood". Adrenal cortical rests are said to be present in approximately 50 per cent of newborns but usually atrophy and disappear within a few years. 3 Because the myriad of foci of aberrant adrenal tissue are too numerous to elaborate in this text, they will simply be mentioned. O'Crowley and Martland found 8 cases of aberrant adrenal gland within the substance of the renal parenchyma in 5,000 consecutive autopsies.• Culp reported on 21 cases of true adrenal-renal heterotopia and presented a postmortem case of his own. 5 Also Mitchell and Angrist reported additional examples of adrenal rests in the kidney, further substantiating the predilection of the aberrant adrenal tissue for the kidney. 6 Schechter cited references to the many different foci of ectopic adrenal tissue: the broad ligament,
spermatic and ovarian vessels, spermatic cord, canal of Nuck and uterus. Abdominal structures containing aberrant adrenal substance have included pancreas, transverse colon, celiac plexus and capsule of the liver. He also noted several references to accessory tissue encountered in structures around the testis, that is epididymis and tunica albuginea. 3 Fifty-five years ago, MacLennan reported on finding nodules of adrenal tissue within 6 hernia sacs removed from 700 children, 600 of whom were boys. 7 Recently, Mininberg and Dattwyler presented a case of ectopic adrenal tumor presenting as torsion of the spermatic cord in a newborn infant. 8 For completeness the clinical implications of aberrant adrenal tissue must be cited. It has been adequately documented that adrenal accessories undergo compensatory hypertrophy and function after adrenalectomy for Cushing's syndrome, and advanced breast and prostatic carcinoma. Also, adrenal rests may be removed inadvertently during any one of a number of surgical procedures, resulting in adrenal insufficiency. For this reason, the clinician must be cognizant of the varied locations of aberrant adrenal tissue. Because of the acces-
708
FELDMAN, ROSENTHAL AND SHAW
sories' predilection for the kidney, " ... the accidental removal of heterotopic adrenal tissue is most likely to occur during nephrectomy". • And lastly, these aberrant implants of adrenal tissue may themselves become neoplastic, whether benign or malignant, and may be responsible for definite endocrinopathies observed clinically. REFERENCES
1. Morgagni, G. B.: Cited by Schechter.• 2. Glenn, J. F. and Boyce, W. H.: Urologic Surgery. New York: Harper & Row, Publishers, Inc., p. 9,
1969.
3. Schechter, D. C.: Aberrant adrenal tissue. Ann. Surg.,
167: 421, 1968.
4. O'Crowley, C.R. and Martland, H. S.: Adrenal heterotopia, rests, and the so-called Grawitz tumor. J. Urol., 50: 756, 1943. 5. Culp, 0. S.: Adrenal heterotopia. A survey of the literature and report of a case. J. Urol., 41: 303, 1939.
6. Mitchell, N. and Angrist, A.: Adrenal rests in the kidney. Arch. Path., 35: 46, 1943. 7. MacLennan, A.: On the presence of adrenal rests in the walls of hernial sacs. Surg., Gynec. & Obst., 29: 387, 1919.
8. Mininberg, D. T. and Dattwyler, B.: Ectopic adrenal tumor presenting as torsion of the spermatic cord in a newborn infant. J. Urol., 109: 1037, 1973.
