Other Benign Adrenal Gland Tumors

OTHER BENIGN ADRENAL GLAND TUMORS

Adrenocortical adenoma arising from aberrant adrenal gland is unusual (Figure 1) [1]. Figure 1 represent case that we have published [1] illustrating an adrenocortical adenoma initially presenting as a superiorly positioned adrenal tumor on the right side.  Intraoperative findings were consistent with  adrenocortical adenoma arising from aberrant adrenal gland and normally appearing adrenal gland on the right side (Figure 2). See my paper at the World Journal of Endocrine Surgery http://www.jaypeejournals.com/eJournals/ShowText.aspx?ID=1162&Type=FREE&...

Figure 1. MRI image of the right ectopic adrenal tumor, under the segment 5 of the liver

Figure 2. Operative image of the ectopic adrenal tumor on the right (A) in the process of dissection. Normal right adrenal gland (inferiorly) (B).  Tumor bed underneath the liver (C).

Aberrant or ectopic adrenocortical tissue is found along the path of embryonic migration of adrenal cortical rests. As adrenal migration occurs cortical tissue may separate leaving fragments of adrenal tissue in an ectopic location. Most, but not all, remain near the adrenal gland. Ectopic adrenal tissue has been reported in the testis, spermatic cord, broad ligament, kidney, retrocaval space, celiac region, lungs, central nervous system, colon, pancreas, and gallbladder. The most common location of heterotopic adrenal tissue is in the area of the celiac axis (32%) and broad ligament (23%). It was reported that ectopic adrenal tissue may undergo malignant transformation or become hormonally functional (2, 3). There are few reported cases of ectopic adrenal tumors. Leibowitz at al presented delayed recurrence, four years after initial surgery, of Cushing’s syndrome due to development of new cortisol secreting adrenal tumor from ectopic adrenal. This was resected 14 years after the initial procedure (3).

Accessory cortical masses are seen in approximately 50% of newborns.  Most do not persist.  Kirici at al reported a case of ectopically located adrenal gland in the right retrocrural space with compressive symptoms (4).

An ectopic aldosteron secreting tumor was described in association with adrenocortical adenoma, in the adrenal gland on the same side. The ectopic tumor was located in the retrocaval region, laterally to the body of the 12th thoracic vertebra (5).

Ayala at al reported case of a 63-yr-old woman who presented with new-onset corticotropin-independent Cushing's syndrome and imaging studies showing normal adrenal glands and enlargement of a left pararenal nodule incidentally observed 4 yr before the onset of symptoms. Surgical removal of the mass lead to resolution of the symptoms. Pathological exam confirmed adrenocortical adenoma in ectopic adrenal tissue (2).

Differential diagnosis between benign and malignant adrenal tumors by histology may be very difficult and surgical findings of the clearly defined margins without invasion is more important in predicting malignant potential of the tumor then just histological description.

Ganglioneuroma (9 cm) in the peri-adrenal retroperitoneal soft tissue tumor that can mimic adrenal tumor (Figure 3).

Figure 3. Peri-adrenal ganlioneuroma

References
  1. Shifrin AL, Zheng, M, Vernick, J “Ectopic Adrenocortical Adenoma.”  World Journal of Endocrine Surgery. January-April 2011;3(1) 45-6.
  2. Ayala AR, Basaria S, Udelsman R, Westra WH, Wand GS. Corticotropin-independent Cushing's syndrome caused by an ectopic adrenal adenoma.  J Clin Endocrinol Metab. 2000 Aug;85(8):2903-6
  3. Leibowitz J, Pertsemlidis D, Gabrilove JL. Recurrent Cushing's syndrome due to recurrent adrenocortical tumor fragmentation or tumor in ectopic adrenal tissue? J Clin Endocrinol Metab. 1998 Nov;83(11): 3786-9.
  4. Kirici Y, Mas MR, Saglamkaya U, Oztas E, Ozan H. Surg Radiol Anat. 2001 Jun;23(3): 201-3. Accessory suprarenal gland: report of a case.
  5. Mazza E, Papotti M, Durando R, Robecchi A, Camanni FJ. Ectopic aldosteronoma associated to another adrenocortical adenoma in the adrenal gland of the same side.  Endocrinol Invest. 1995 Nov;18(10): 809-12.