CASE REPORT
Aberrant adrenocortical tissue in hernia sac occurring in an adult: case report and review of the literature
D. El Demellawy . A. Nasr . A. Samkari .
P. Pastolero · S. Alowami
Received: 21 December 2008 / Accepted: 20 March 2009 / Published online: 15 April 2009 @ Springer-Verlag 2009
Abstract Aberrant adrenal tissue is rare, particularly in adults. To the best of our knowledge, only eight cases have been reported in the English literature. Awareness of the possible occurrence of adrenal tissue in hernial sac is cru- cial, as these rests may have several clinical implications. These potential implications include hyperplasia, adrenal insufficiency, neoplastic transformation and misdiagnosis as metastatic clear cell carcinoma. We report a rare case of aberrant adrenocortical tissue in hernial sac. We review the literature and highlight the clinical implications of this inci- dental finding.
Keywords Hernia sac . Aberrant adrenal tissue · Accessory adrenal · Adrenal heterotopia
D. El Demellawy Department of Pathology and Laboratory Medicine, William Osler Health Center, Brampton Civic Hospital, Northern Ontario School of Medicine, 2100 Bovaird East Drive, Brampton, ON, Canada
e-mail: dina.demellawy@sympatico.ca
A. Nasr Department of Pediatric Surgery, Sick Kids Hospital, University of Toronto, Toronto, ON, Canada
A. Samkari · S. Alowami Department of Pathology and Molecular Medicine, Hamilton Health Sciences Center, McMaster University, Hamilton, ON, Canada
P. Pastolero Faculty of Science, University of Toronto, Toronto, ON, Canada
Introduction
The foci of aberrant adrenal tissue are reported uncom- monly. The most common location of these foci is the kid- neys, being in close approximation to the adrenal glands. Out of 5,000 autopsies, eight cases showed aberrant adrenal tissues in the kidneys [1].
The foci of aberrant adrenal tissue in the hernia sac are rare, with most of the reported cases occuring in children. Aberrant adrenal tissue is extremely rare and, to the best of our knowledge, only eight cases have been reported in the English literature (Table 1).
We report a case of aberrant adrenal tissue in hernial sac. We review the literature and highlight the clinical and path- ological implications of this incidental finding.
Case report
A 78-year-old man presented with lower quadrant abdomi- nal discomfort of chronic onset non-progressive course dat- ing since 1 year previously. He had been suffering from chronic constipation. His past medical and family histories are non-significant, except for coronary artery disease. Examination shows absence of distress, soft abdomen and non-complicated left direct inguinal hernia. There is no evi- dence of masses and his genitalia were normal with two descended testicles.
Ultrasound and abdominal computed tomography (CT) confirmed the presence of left inguinal hernia, containing a short segment of sigmoid colon. There was no evidence of associated bowel obstruction and the rest of his pelvi- abdominal organs were normal. The laboratory findings were within normal range, including blood picture and serum electrolytes.
| Case reports | Age (years) and gender | Laterality hernia | Focality | Size (mm) |
|---|---|---|---|---|
| Schechter (1967) | 19 M | Left indirect inguinal | 1 | 2 |
| Gutowski and Gray (1979) | 58 M | Right inguinal | 1 | 4 |
| Gutowski and Gray (1979) | 66 M | Right inguinal | 1 | 10 |
| Gutowski and Gray (1979) | 44 M | Right inguinal | 2 | 2,5 |
| Gutowski and Gray (1979) | 45 M | Left inguinal | 1 | 3 |
| Gutowski and Gray (1979) | 34 M | Right inguinal | 1 | 2 |
| Prasad (1984) | 31 M | Right indirect inguinal | 11 | 8 |
| Iyengar and Pittman (2007) | 54 M | Right direct inguinal | 1 | 4 |
| El Demellawy et al. (2009) | 78 M | Left direct inguinal | 1 | 7 |
The case’s working diagnosis was diverticular disease and incidental left direct inguinal hernia. Left inguinal her- nia repair was performed. Gross examination revealed a well-defined tan yellow nodule measuring 7 x 5 mm. His- tologically, there was adrenocortical tissue surrounded by thick fibrous capsule (Fig. 1) but there was no evidence of adrenal medullary tissue. The adrenocortical tissue shows tabecular arrangement with layers simulating zona granu- losa and zona fasciculata (Fig. 2). The tissue expressed antibodies against Melan A (Fig. 3), Cam5.2 and vimentin by immunohistochemistry, indicating a similar immunopro- file to native adrenal cortex. Significant pathological abnor- mality including hyperplasia and neoplasia was absent. The case was diagnosed as hernial sac with aberrant adrenocor- tical tissue. The patient received no further treatment and, during the follow-up period of 2 years, is doing well.
Discussion
Choriostoma and heterotopia are synonymous terms defi- ned as the presence of normal tissue within an abnormal
location. In most cases, it is an incidental finding; however, in some cases, it leads to serious clinical manifestations. In general, heterotopias are rare. In one study, among 11,265 surgical pathology cases, only 29 cases were diagnosed with heterotopia [2]. The majority were pancreatic heterotopy,
followed in decreasing order by gastric, adrenal and osse- ous heterotopy [2].
Ectopic adrenal tissue was first described within the vicinity of the adrenal gland by Morgagni as early as 1740 [3]. Ectopic adrenal cortex is most commonly located in the retroperitoneum and hilus of gonads [4]. Aberrant adrenal tissue involving the hernia sac is quite rare, with a reported incidence of 0.3-0.7% [5, 6]. The incidence of aberrant adrenal tissue in the inguinoscrotal region, in general, is still rare but higher. In such cases, which include those requiring inguinal exploration for inguinal hernia, hydro- cele and hydrocele of the spermatic cord, the incidence of aberrant adrenal tissue is 1.63% [7]. In cases with unde- scended testicle, the incidence of aberrant adrenal tissue is 5.1-9.3% [8, 9] and, in general, the incidence in groin exploration is 2.7% [6]. The nodules of the adrenal cortex are located along the spermatic cord, in the apex of the her- nial sac and between the testes and the epididymis [9].
The majority of the reported cases were in males, with a male to female ratio of 100:0-1:17 [6, 9].
Most of the cases reported within the literature are in paediatric age group, with mean age of diagnosis of 5.6 years [10]. In fact, in our search in the literature, we found only eight case reports of aberrant adrenocortical tis- sue in the adult population (Table 1) [3, 4, 11, 12].
The higher incidence of ectopic adrenal tissue in the pae- diatric male population and its relative association with tes- ticular pathology is not surprising. Adrenal cortical tissue develops from the mesoderm medial to the developing gonads. Hence, the foci of the developing cortex may be car- ried with the descending gonads and, hence, lie within its vicinity. The adrenal medulla develops from the neuroecto- derm, thus, it is less likely to be in proximity to the gonads.
Several pathologic entities have been described within the hernial sac, such as testes [13], ovaries [14, 15], fallo- pian tubes [14, 15], uterus [15], urinary bladder [14], ectopic appendix [14, 16], epiploic appendagitis [17, 18], glandular inclusions [19, 20], endometriosis [21], parasitic granuloma [22], metastatic carcinomas [23-25] and sar- coma [26].
The surgical implication in cases of ectopic adrenal tis- sue include neoplastic and hyperplastic potentials and the possibility of adrenal insufficiency after the removal of ectopic tissue in case of normal glands being absent. There is no evidence of evolution with age [6].
Recognition of aberrant adrenal tissue is mandatory to avoid their misdiagnosis as metastatic clear-cell carcinoma or malignant melanoma. Though their removal if encoun- tered incidentally is recommended as they may be associ- ated with neoplastic and hyperplastic changes, routine searches for them is not recommended [9].
Excising the adrenocortical ectopic tissue during the groin surgery can usually be easily achieved; however,
meticulous dissection of the spermatic cord should not be performed in order to avoid damage to the spermatic ves- sels and deferens [10]. The nodule is usually embedded in the cremasteric fibres of the spermatic cord, very close to the deferens and attached to the hernia sac, and it is very simple to have it dissected free without vascular injury [10].
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