Case Reports
HETEROTOPIC ADRENAL CORTICAL ADENOMA MASQUERADING AS METASTATIC RENAL CELL CARCINOMA
TONG WU, RICHARD BIHRLE AND MOO NAHM YUM
From the Departments of Pathology and Urology, Indiana University School of Medicine, Indianapolis, Indiana KEY WORDS: adenoma, adrenal cortical; carcinoma, renal cell; adrenal gland
It is known that a heterotopic adrenal gland may be located in the upper abdomen or anywhere along the path of descent of the gonads.1,2 Such adrenal glands may undergo hyper- plasia in response to increased levels of adrenocorticotropic hormone, and they may serve as a site of origin of a cortical neoplasm.3 Conceivably heterotopic adrenal cortical hyper- plasia and/or adenoma may be confused with certain primary or metastatic tumors. We report a case of adrenal cortical adenoma arising from the heterotopic adrenal cortex in the gastroesophageal junction that was misdiagnosed as meta- static renal cell carcinoma before and during radical nephrec- tomy for primary renal cell carcinoma. To our knowledge our case represents the first report of heterotopic adrenal cortical adenoma at the gastroesophageal junction mimicking meta- static renal cell carcinoma.
CASE REPORT
A 60-year-old black man presented with hematuria. Sub- sequently excretory urography and computerized tomogra- phy (CT) revealed a 12 × 12 cm. mass in the upper pole of the right kidney consistent with renal cell carcinoma (fig. 1). In addition, CT and esophagogastroduodenoscopic ultrasound showed a solid 2 x 3 cm. lesion at the gastroesophageal junction immediately outside the gastroesophageal wall. Transgastric fine needle aspiration of the mass demonstrated clusters of epithelial cells with clear cytoplasm consistent with metastatic renal cell carcinoma. Biopsy of the gastric mucosa revealed chronic active gastritis with no evidence of tumor in the gastric wall.
Therapeutic approaches were significantly complicated by these apparent gastroesophageal metastases. After consider- ing the risks, benefits and alternatives the patient elected right radical nephrectomy followed by exploration of the le- sion at the gastroesophageal junction. During surgery the 2.5 to 3.0 cm., soft, rubbery, bright yellow-orange mass was not
Accepted for publication September 18, 1998.
adherent to the gastroesophageal wall and it was easily enu- cleated from the surrounding soft tissue.
A small portion of the lesion was sent for frozen section evaluation. The diagnosis was metastatic carcinoma consis- tent with a renal or adrenal cortical primary tumor. Patho- logical examination of the resected right kidney confirmed the diagnosis of predominantly clear cell, grade 3 of 4 renal cell carcinoma with no evidence of capsular or vascular inva- sion (fig. 2, A).
Surprisingly, examination of tissue from the mass at the gastroesophageal junction contradicted the diagnosis of met- astatic renal cell carcinoma made on fine needle aspiration and frozen section. After sections were stained with hema- toxylin and eosin it was noted that the gastroesophageal junction lesion was comprised of cords and nests of benign polygonal cells with small ovoid nuclei and abundant foamy
A
B
to eosinophilic granular cytoplasm (fig. 2, B). The final patho- logical diagnosis of the mass at the gastroesophageal junction was adrenal cortical adenoma, likely derived from the hete- rotopic adrenal tissue. This diagnosis was supported by im- munohistochemical results. While cells of renal cell carci- noma in the right kidney stained positive for cytokeratin epithelial membrane antigen and vimentin, cells of the ad- renal cortical adenoma at the gastroesophageal junction stained negative for cytokeratin epithelial membrane anti- gen and positive for vimentin.
DISCUSSION
With the increasing use of body imaging for the manage- ment of renal cell carcinoma and other genitourinary malig- nancies it is likely that more cases of heterotopic adrenal
hyperplasia adenoma will be detected. The presence of a localized metastatic lesion in a patient with renal cell carci- noma should alert urologists and pathologists to the possi- bility of heterotopic adrenal hyperplasia and/or neoplasm. This distinction is important for the correct staging of renal cell carcinoma as well as therapeutic approaches, such as surgical resection and chemotherapy.
REFERENCES
1. Gutowski, T. and Gray, G. F .: Ectopic adrenal in inguinal hernia sacs. J. Urol., 121: 353, 1979.
2. Lack, E. E .: Atlas of tumor pathology: tumors of the adrenal gland and extra-adrenal paraganglia. AFIP, third series, fas- cicle 19, pp. 33-48, 1997.
3. Burke, E. F., Gilbert, E. and Uehling, D. T .: Adrenal rest tumors of the testes. J. Urol., 109: 649, 1973.