Myelolipoma associated with adrenal ganglioneuroma.
REPORT OF A CASE
A 20-year-old Hispanic woman presented with complaints of nausea and flank pain that had been present for 2 to 3 months. Computed tomographic scan of the abdomen revealed a left suprarenal solid mass. Her medical history included diabetes and mild hypertension, but no hematologic or endocrinologic disorders. Results of laboratory studies, including complete blood counts, erythrocyte sedimentation rates, and urine analysis were normal. Urinary vanillylmandelic acid and catecholamines were within normal limits. Since the patient did not have any features suggestive of endocrinologic dysfunction, laboratory evaluation of endocrine status was not investigated further A laparotomy was performed, revealing a large mass above the upper pole of the left kidney. There was no evidence of metastatic disease. Post-operative recovery was uneventful. The patient was well and free of disease 9 months after surgery.
Pathologic examination showed a 325-g, 11 x 9 x 6-cm, well-circumscribed solid tumor expanding the adrenal medulla. Residual adrenal gland was identified at one pole of the tumor and as a thin rim compressed at the periphery of the tumor. The sectioned surface of the tumor was gray-white and firm with a 1.5-cm, red-brown soft area at one edge of the tumor (Figure 1). On microscopic examination, the tumor was sharply circumscribed and consisted of interlacing bundles of neurofibromatous tissue with mature ganglion cells scattered throughout (Figure 2). Within the tumor, several foci (red-brown area on gross examination) of mature adipose tissue mixed with hematopoietic tissue were identified (Figure 3). These foci were mainly located in the peripheral portions of the tumor Additionally, strands of adrenal cortical cells, mainly of the reticularis type, were found mingled with the neurofibromatous tissue, some of which were seen in close proximity to the myelolipomatous foci (Figure 4).
[FIGURES 1-4 OMITTED]
The spectrum of myelolipomatous change in adrenal glands was reviewed by Plaut, (4) who noted that these lesions varied in size and "exhibited all gradations from almost entirely adipose to almost entirely myeloid tissue." Although the majority of myelolipomas present as isolated adrenal masses, (3) myelolipomatous foci have been reported in association with other adrenal pathologic conditions. (3,5) These associated adrenal pathologic conditions include adrenocortical hyperplasia, (3) adrenocortical adenomas, (3,5) adrenocortical carcinomas, (5) and endocrinologic dysfunctions, including Addison disease, (4) Conn syndrome, (5) 21-hydroxylase deficiency, (3) 17-hydroxylase deficiency, (6) and ectopic corticotropin production. (7) To our knowledge, this article represents the first report of myelolipoma occurring in association with an adrenal ganglioneuroma.
The histogenesis of myelolipoma remains obscure. Theories proposed include development from embryonic mesenchymal rests in the adrenal glands, development from hematogenously seeded bone marrow emboli, and metaplasia of reticuloendothelial cells. (4,8) The association of myelolipomas with hormonally active states and neoplastic conditions raises the possibility that the hormonal microenvironment may play a role in the development of myelolipomas. This view is supported by Selye and Stone's (8) experimental transformation of adrenal into myeloid tissue by using extracts of corticotropin and testosterone.
Ganglioneuroma is a benign tumor of the sympathetic nervous system. Although generally thought to be hormonally inactive, rare cases of ganglioneuromas associated with virilization and elevated testosterone levels have been reported in the literature. (9,10) Aguirre and Scully (10) identified "Leydig cells with crystalloids of Reinke," whereas Mack et al (9) hypothesized "the strands of hyperplastic adrenal-cortical cells, mainly of the reticularis type within the neurofibromatous stroma" as the possible source of testosterone secretion by the tumor. In both cases, the testosterone levels returned to normal postoperatively, confirming that the source of abnormal testosterone secretion had been removed. In the present case, the tumor showed numerous strands of adrenocortical cells within the neurofibromatous stroma, similar to the tumor described by Mack et al. (9) Although our patient did not have clinically evident endocrine dysfunction or virilization effects, the close proximity of myelolipomatous foci to strands of adrenal cortical cells within the stroma to ganglioneuroma suggests that the myelolipomatous foci may have been secondarily induced by the hormonal microenvironment within the ganglioneuroma.
In summary, myelolipomas probably represent hormonally induced choristomas derived from primitive mesenchymal elements. The presence of hormonally active cells in adrenal ganglioneuroma may explain this rare association of myelolipoma and ganglioneuroma.
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(2.) Oberling C. Les formations myelolipomatouses. Bull Cancer. 1929;18:234-236.
(3.) Kenny PJ, Wagner BJ, Rao P, Heffess CS. Myelolipoma: CT and pathologic features. Radiology. 1998;208:87-95.
(4.) Plaut A. Myelolipoma in the adrenal cortex (myelolipomatous structures). Am J Pathol. 1958;34:487-515.
(5.) Goetz SP, Niemann TH, Robinson RA, Cohen MB. Hematopoietic elements associated with adrenal glands: a study of the spectrum of change in nine cases. Arch Pathol lab Med. 1994;118:895-896.
(6.) Condom E, Villabona CM, Gomez JM, Carrera M. Adrenal myelolipoma in a woman with congenital 17-hydroxylase deficiency. Arch Pathol lab Med. 1985; 109:1116-1118.
(7.) Sieber SC, Gelfman NA, Dandurand R, Braza R Ectopic ACTH and adrenal myelolipoma. Conn Med. 1989;53:7-10.
(8.) Selye H, Stone H. Hormonally induced transformation of adrenal into myeloid tissue. Am J Pathol. 1950;26:211-233.
(9.) Mack E, Starto GE, Crummy AB, Carlson IH, Curet LB, Wu J. Virilizing adrenal ganglioneuroma. JAMA. 1978;239:2273-2275.
(10.) Aguirre P, Scully RE. Testosterone-secreting adrenal ganglioneuroma containing Leydig cells. Am J Surg Pathol. 1983;7:699-705.
Accepted for publication October 15, 2001.
From the Department of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston.
Reprints: Shakil H. Merchant, MD, 6001 Moon St, NE, #1624, Albuquerque, NM 87111 (e-mail: email@example.com).
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|Author:||Merchant, Shakil H.; Herman, Christopher M.; Amin, Mitual B.; Ro, Jae Y.; Troncoso, Patricia|
|Publication:||Archives of Pathology & Laboratory Medicine|
|Date:||Jun 1, 2002|
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