Ectopic Adrenocortical Tissue: An Incidental Finding

Fetal and Pediatric Pathology, Early Online:1–6, 2012 C Informa Healthcare USA, Inc. Copyright ISSN: 1551-3815 print / 1551-3823 online DOI: 10.3109/15513815.2012.659414

Ectopic Adrenocortical Tissue: An Incidental Finding During Inguinal Surgery in Children

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Dusanka S. Dobanovacki,1 Dusan M. Maric,1 Dusica L. Maric,2 Nada Vuckovic,3 Radoica R. Jokic,1 Andjelka R. Slavkovic,4 and Sanja V. Skeledzija Miskovic1 1

Institute for Children and Youth Health Care of Vojvodina, Pediatric Surgery Clinic, Novi Sad, Serbia; 2 Medical Faculty, Department of Anatomy, Novi Sad, Serbia; 3 Clinical Centre of Vojvodina, Centre for Pathology and Histology, Novi Sad, Serbia; 4 Clinic for Pediatric Surgery and Orthopedics, Nis, Serbia

Adrenal rests are usually unrecognized during operation, and the incidence of ectopic adrenal cortical tissue in pediatric patients during inguinal surgery procedures is unknown. We performed 3028 groin surgical explorations in 2680 patients aged 1 month to 17 years. Ectopic adrenal tissue was found in 69 inguinal operations (2.2%): 37 during 1.524 orchiopexy (2.4%), 23 during 1.115 herniectomy (2.0%), and 9 during 389 hydrocoela operation (2.3%). Statistically there were no signiﬁcant diﬀerences among those three groups. No adrenal rests were detected in females. Although a few reported cases with hormonal activity of ectopic adrenocortical tissue (EACT), the recommendation is to remove them if found. Keywords adrenal gland, ectopic tissue, inguinal surgery, adrenal rest tissue

INTRODUCTION The adrenal cortex is of mesodermal origin and develops in the sixth week of fetal life from the coelomic epithelium.At the very beginning, it exists as a chain-cord of cell buds, but shortly afterward most of them fuse into a large cellular mass lateral to the aorta becoming the adrenal cortex [1]. Later, medulla invades the cortical primordium and becomes encapsulated within it [2, 3]. Ectopic adrenocortical tissue (EACT) (synonyms: ectopic, heterotopic, accessory, or aberrant tissue or adrenal rests tissue) are right-yellow nodules a few millimeters in diameter. Microscopically, they are presented as lipid-rich cortical cells with no medullary component (4–6). They may be found in the region of adrenal, kidney, testes, and ovaries, along the retroperitoneal fascia, spermatic vessels, or broad ligament [1]. Ectopic adrenocortical tissue has been observed in a variety of places and reported in occurrence 1–9.3% in pediatric patients as an incidental finding during inguinal surgery [7–11]. Some unusual locations of the EACT were described in literature, such as in the thorax [12, 13], nervous system [14], placenta, liver [4], in the testis [15], the large intestine [16], etc. The explanations for their ectopic place is either in the fact that they originate

Address correspondence to Prof. Dusanka S. Dobanovacki, Institute for Children and Youth Health Care of Vojvodina, Pediatric Surgery Clinic, Hajduk Veljkova 10, Novi Sad, 21000 Serbia. E-mail: [email protected]

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D.S. Dobanovacki et al.

from penetration of medulla and defragmentation of cortical primordial, or in cortical cell chain-buds are not fused. The fate of yellow-bodies is to disappear or form rests that usually could be an incidental finding during surgical operations or autopsy. Autopsy reports differ substantially from the actual occurrence: more than 50% in neonates and children [5, 17–19] and lower in adults 1–32% [20, 21]. It supports the theory of gradual disappearance of rests during body development. In the majority of cases reported in the literature, EACT are represented by nonsecreting (hormonal inactive) remnant tissue found along the path of embryonic migration within urogenital tract. In rare cases, it is important to consider EACT as ectopic corticosteroid-secreting tumors in the corticotropin-independent Cushing’s syndrome [12, 22–24]. Ectopic tissue may also be encountered as a tumor mass, and some authors have reported neoplastic changes in this type of tissue [2, 11, 12]. A special category is testicular adrenal rest tumors (TART) detectable within the rete testis. They are benign tumors. Histologically, TART resembles adrenocortical tissue and sometimes is difficult to distinguish against malignant Leydig cell tumors [15, 25]. PATIENTS AND METHODS A retrospective (1999–2002) prospective (2003–2009) study dealt with detecting EACT during elective inguinal surgical procedures in children (male and female) aged 1 month to 17 years. The goal was to assess the frequency of EACT found during inguinal surgery, and to analyze the underlying diagnosis, age, and sex. Standard procedures of inguinal surgery were used: inguinal skin incision, opening the external oblique fascia, dissecting the spermatic cord elements cranially as it was necessary, and orchidopexy when indicated. Careful examinations of the spermatic cord during operation were provided in search for EACT. The fact is that more operations were done by general pediatric surgeons than by pediatric urologists.Inguinal explorations were classified according to the following operation diagnoses: inguinal herniectomy (H), ligation of the communicating processus vaginalis (hydrocoele) (PV), and undescended testis (UDT). In cases with undescended testes and hernial sac, they were included in a UDT group. Reoperation of the hernia or undescended testis, and also emergency cases (acute scrotal swelling or injury) were excluded. All detected ectopic adrenocortical bodies were easily and carefully removed, and later underwent histologic expertise using standard hematoxylin-eosin stain procedure. Patient’s database and the student t-statistical test were used for comparison of frequency of the three groups of patients analyzed. RESULTS The total number of 3028 inguinal elective operations was performed on 2.680 patients during the 1999–2009 time periods. At the time of operation, the patients were 1 month to 17 years of age, median age being 7.9. There were 2.253 boys and 427 girls. Median age of the patients for hernia and hydrocoele repair was 2.9, and of the cryptorchid patients 5.05. The types of surgical procedures were as follows: inguinal hernia 1.115, communicating hydrocoele 389, and undescended testis 1.524. The EACT were found in 69 cases located at the upper pole of the testis or along the spermatic cord: 37 diagnosed during orchiopexy (2.4%), 23 during herniectomy (2.0%), and 9 during hydrocoele operation (2.3%). Statistically, there were no significant differences among these groups: using the student t-statistical test for Fetal and Pediatric Pathology


Ectopic Adrenocortical Tissue 

FIGURE 1 Intraoperative finding during hydrocoele repair: arrow marks the clearly defined two yellow nodules located above the epididymis on the spermatic cord.

comparison of frequency of EACT in the three groups of our research, no statistically significant values were found for p < 0.001. Bilateral spermatic location of the bodies was found only in a 5-year-old boy who underwent bilateral orchiopexy. No adrenal rests were detected in the female population. Ultrasound examination and volumetry were applied preoperatively for patients with UDT but no case with TART was detected. Clinical data have shown that in the retrospective group, 1.092 inguinal explorations were performed both by general pediatric surgeons and pediatric urologists. In that group, 18 cases of EACT were detected (1.6%). In the prospective group, 1.936 groin operations were performed: 1.095 by general pediatric surgeons and 841 by pediatric urologists. In the whole group, adrenal rests were found in 51 explorations (2.6%). Detailed analyses revealed that in the group operated by general pediatric surgeons, only 7 rests were documented (0.6%) and in the one operated by pediatric urologist 44 (5.2%). Macroscopically, the tissues were 2–3 mm in diameter, yellowish, and spherical in shape (Figure 1). A histologic section of the excised nodules was made in all cases using hematoxylin-eosin staining, and examined by light microscopy. All the nodules had a typical appearance: a bright-yellowish nodule one to three millimeter in diameter. Histologic expertise showed the structure of a typical adrenal cortical tissue: zona glomerulosa, fasciculata, and reticularis were clearly demonstrated, surrounded by a thin, fibrous capsule (Figure 2). Microscopic examination revealed no adrenal medulla in either of the cases.

DISCUSSION Ectopic adrenal cortical tissue is usually a benign lesion and it is well known that it can be found in the upper part of the abdomen or anywhere along the path of the descent of the gonads, sometimes in some unusual places. Morgagni 1740 [18] (cited in Schechter) first described a yellow nodule at the spermatic cord, and Felix Marchand C Informa Healthcare USA, Inc. Copyright


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FIGURE 2 Histologic section (H & E × 40) of ectopic nodules; adrenal cortex tissue surrounded by capsule. No adrenal medullary tissue is observed. Inset picture: typical cortical cells (H& E × 200).

1883, found it at the border of the broad ligament and the kidney (Marchand’s body) [3]. During the course of development, cortical tissue may remain in various locations to form an accessory collection of cells. Adrenocortical rests are commonly registered in children and are most often found as bright yellow nodules along the route of descent of the gonads, in hernial sacs, or in gonads themselves. Although routine search for this lesion is not indicated, it should be resected when found during surgical procedure. The overall prevalence of EACT along the spermatic cord in published retrospective or prospective studies are very different, ranging 1–9.3% [7, 8], but the frequency in neonates is much higher, up to 15% [5]. The total prevalence in our study was 2.2% and adrenal rests were not registered among the neonates and children under 1 year of age. Also, the rests were not found in girls who underwent inguinal operations, as in Sullivan, Gohel, and Kinder [27] and Altin, Gundo, and Aksoy studies [8]. On the contrary, Oguzkurt, Oz, and Kayaselcuk [11] registered the frequency of 1.3% in boys and 3% in girls operated for inguinal hernia. Mares et al. [5] reported ectopic tissue in 3.8% of children operated upon for inguinal pathology and in the same study, it was found that in children operated upon for undescended testis, the frequency rose up to 9.3%. In our series, the frequency of overall number of ectopic adrenal tissue associated with inguinoscrotal region was 2.4%. This finding is less than publicated [5]. The higher number of found ectopic tissue in orchidopexy surgery may be due to a more thorough inspection of the spermatic cord. We only registered higher prevalence of EACT (5.6%) in the group of children operated by the pediatric urologists (orchidopexy is more frequent in inguinal pathology than hernia or hydrocoele) than in the other, and it could be that they pay more attention to funicular structures during operation. In published literature, there are different numbers of EACT with respect to the three diagnoses: inguinal hernia - communicating hydrocoele—undescended testis. In Sullivan’s study [27], EACT occurrence was 0.7% at inguinal herniotomy, 4.1% at ligation of the patent processus vaginalis for communicating hydrocele, and 3.3% at exploration for undescended testes. In Lochbuhler’s prospective study [28], aberrant cortical tissue was found in 5.2% of inguinal hernias and in 10.9% of undescended testes. Our analyses revealed no statistical significance in groups inguinal hernia (2.0%)—communicating hydrocoele (2.3%)—undescended testes (2.4%) with p < 0.001. Fetal and Pediatric Pathology

Ectopic Adrenocortical Tissue  We agree with the opinion that the difference in prevalence might arise through a more thorough dissection in one operation than in another [27]. Although the degree of dissection during all three types of operation is similar in practice, the vast majority of these operations were performed by different surgeons, which may be a reason for such different results. Clinical practice revealed that these aberrant implants of adrenal tissue may become neoplastic, benign, or malignant. We have no experience with malignant tumor or abnormal function due to EACT.


CONCLUSION We have concluded that the number of found EACT depends heavily on the surgeon’s knowledge and careful examination of the spermatic cord during inguinal operation. We also believe that EACT could be revealed as a concomitant occurrence in many more cases. Pediatric surgeons and urologists should bear in mind the fact that EACT can be seen as a small nodule during groin exploration, so excising it is recommended. A special search for it, however, has not been advised. Declaration of interest The authors have no conflict of interest. The authors alone are responsible for the content and writing of this article.

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