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Ectopic adrenocortical adenoma in the renal hilum: a case report and literature review.

Liu Y, Jiang YF, Wang YL, Cao HY, Wang L, Xu HT, Li QC, Qiu XS, Wang EH - Diagn Pathol (2016)

Bottom Line: This case provides a reminder to pathologists to be aware of atypical cases of this benign tumor.A misdiagnosis of this benign condition as a malignant renal tumor may have severe consequences for the patient, including unnecessary radical nephrectomy.Preoperative biopsy and appropriate immunohistochemical staining will assist in determining the origin and nature of the tumor and in avoiding intraoperative uncertainty.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, The First Affiliated Hospital and College of Basic Medical Sciences, China Medical University, Shenyang, 110001, China. lyonliuyang@mail.cmu.edu.cn.

ABSTRACT

Background: Ectopic (accessory) adrenocortical tissue, also known as adrenal rests, is a developmental abnormality of the adrenal gland. The most common ectopic site is in close proximity to the adrenal glands and along the path of descent or migration of the gonads because of the close spatial relationship between the adrenocortical primordium and gonadal blastema during embryogenesis. Ectopic rests may undergo marked hyperplasia, and occasionally induce ectopic adrenocortical adenomas or carcinomas.

Case presentation: A 27-year-old Chinese female patient who presented with amenorrhea of 3 months duration underwent computed tomography urography after ultrasound revealed a solitary mass in the left renal hilum. Histologically, the prominent eosinophilic tumor cells formed an alveolar- or acinar-like configuration. The immunohistochemical profile (alpha-inhibin+, Melan-A+, synaptophysin+) indicated the adrenocortical origin of the tumor, diagnosed as ectopic adrenocortical adenoma. The patient was alive with no tumor recurrence or metastasis at the 3-month follow-up examination.

Conclusions: The unusual histological appearance of ectopic adrenocortical adenoma may result in its misdiagnosis as oncocytoma or clear cell renal cell carcinoma, especially if the specimen is limited. This case provides a reminder to pathologists to be aware of atypical cases of this benign tumor. Although uncommon, an ectopic adrenal lesion should be included in the differential diagnosis of tumors involving the renal hilum. A misdiagnosis of this benign condition as a malignant renal tumor may have severe consequences for the patient, including unnecessary radical nephrectomy. Preoperative biopsy and appropriate immunohistochemical staining will assist in determining the origin and nature of the tumor and in avoiding intraoperative uncertainty.

No MeSH data available.


Related in: MedlinePlus

Histological features. The tumor is circumscribed by a discontinuous capsule (a) and is clearly demarcated from the surrounding adipose tissue (b). c The tumor cells are mainly arranged in nests with an alveolar- or acinar-like configuration. These acini are surrounded by an open vascular network. d Foci of edematous stroma are seen within the tumor. e The focal infiltration of mature lymphocytes is especially prominent in the areas undergoing lipomatous or myelolipomatous metaplasia. f Bizarre nuclear forms are occasionally present, especially in the area of diffuse oncocytes. The cells contain eosinophilic nuclear pseudoinclusion bodies. g Lipofuscin pigmentation is detected in focal areas. h The peripheral adipose tissue contains foci of ectopic adrenal rests
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Fig2: Histological features. The tumor is circumscribed by a discontinuous capsule (a) and is clearly demarcated from the surrounding adipose tissue (b). c The tumor cells are mainly arranged in nests with an alveolar- or acinar-like configuration. These acini are surrounded by an open vascular network. d Foci of edematous stroma are seen within the tumor. e The focal infiltration of mature lymphocytes is especially prominent in the areas undergoing lipomatous or myelolipomatous metaplasia. f Bizarre nuclear forms are occasionally present, especially in the area of diffuse oncocytes. The cells contain eosinophilic nuclear pseudoinclusion bodies. g Lipofuscin pigmentation is detected in focal areas. h The peripheral adipose tissue contains foci of ectopic adrenal rests

Mentions: Histologically, the tumor was well circumscribed and composed of cells mainly arranged in nests forming an alveolar- or acinar-like configuration (Fig. 2a–c) and surrounded by an open vascular network. Foci of edematous stroma within the tumor (Fig. 2d) and oncocytes with a diffuse growth pattern along its periphery were observed. Focal infiltrations of mature lymphocytes were especially prominent in areas undergoing lipomatous or myelolipomatous metaplasia (Fig. 2e). The tumor was mainly composed of cells with an abundant eosinophilic cytoplasm. Focal areas of pale-staining clear cells with a lipid-filled cytoplasm were identified but were rare. Bizarre nuclear forms were occasionally seen in areas with diffuse tumor cells (Fig. 2f). Eosinophilic nuclear pseudoinclusion bodies and lipofuscin pigmentation were also detected (Fig. 2g). However, mitoses were exceptionally rare or absent. The peripheral adipose tissue contained foci of ectopic adrenal rests (Fig. 2h).Fig. 2


Ectopic adrenocortical adenoma in the renal hilum: a case report and literature review.

Liu Y, Jiang YF, Wang YL, Cao HY, Wang L, Xu HT, Li QC, Qiu XS, Wang EH - Diagn Pathol (2016)

Histological features. The tumor is circumscribed by a discontinuous capsule (a) and is clearly demarcated from the surrounding adipose tissue (b). c The tumor cells are mainly arranged in nests with an alveolar- or acinar-like configuration. These acini are surrounded by an open vascular network. d Foci of edematous stroma are seen within the tumor. e The focal infiltration of mature lymphocytes is especially prominent in the areas undergoing lipomatous or myelolipomatous metaplasia. f Bizarre nuclear forms are occasionally present, especially in the area of diffuse oncocytes. The cells contain eosinophilic nuclear pseudoinclusion bodies. g Lipofuscin pigmentation is detected in focal areas. h The peripheral adipose tissue contains foci of ectopic adrenal rests
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4837621&req=5

Fig2: Histological features. The tumor is circumscribed by a discontinuous capsule (a) and is clearly demarcated from the surrounding adipose tissue (b). c The tumor cells are mainly arranged in nests with an alveolar- or acinar-like configuration. These acini are surrounded by an open vascular network. d Foci of edematous stroma are seen within the tumor. e The focal infiltration of mature lymphocytes is especially prominent in the areas undergoing lipomatous or myelolipomatous metaplasia. f Bizarre nuclear forms are occasionally present, especially in the area of diffuse oncocytes. The cells contain eosinophilic nuclear pseudoinclusion bodies. g Lipofuscin pigmentation is detected in focal areas. h The peripheral adipose tissue contains foci of ectopic adrenal rests
Mentions: Histologically, the tumor was well circumscribed and composed of cells mainly arranged in nests forming an alveolar- or acinar-like configuration (Fig. 2a–c) and surrounded by an open vascular network. Foci of edematous stroma within the tumor (Fig. 2d) and oncocytes with a diffuse growth pattern along its periphery were observed. Focal infiltrations of mature lymphocytes were especially prominent in areas undergoing lipomatous or myelolipomatous metaplasia (Fig. 2e). The tumor was mainly composed of cells with an abundant eosinophilic cytoplasm. Focal areas of pale-staining clear cells with a lipid-filled cytoplasm were identified but were rare. Bizarre nuclear forms were occasionally seen in areas with diffuse tumor cells (Fig. 2f). Eosinophilic nuclear pseudoinclusion bodies and lipofuscin pigmentation were also detected (Fig. 2g). However, mitoses were exceptionally rare or absent. The peripheral adipose tissue contained foci of ectopic adrenal rests (Fig. 2h).Fig. 2

Bottom Line: This case provides a reminder to pathologists to be aware of atypical cases of this benign tumor.A misdiagnosis of this benign condition as a malignant renal tumor may have severe consequences for the patient, including unnecessary radical nephrectomy.Preoperative biopsy and appropriate immunohistochemical staining will assist in determining the origin and nature of the tumor and in avoiding intraoperative uncertainty.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, The First Affiliated Hospital and College of Basic Medical Sciences, China Medical University, Shenyang, 110001, China. lyonliuyang@mail.cmu.edu.cn.

ABSTRACT

Background: Ectopic (accessory) adrenocortical tissue, also known as adrenal rests, is a developmental abnormality of the adrenal gland. The most common ectopic site is in close proximity to the adrenal glands and along the path of descent or migration of the gonads because of the close spatial relationship between the adrenocortical primordium and gonadal blastema during embryogenesis. Ectopic rests may undergo marked hyperplasia, and occasionally induce ectopic adrenocortical adenomas or carcinomas.

Case presentation: A 27-year-old Chinese female patient who presented with amenorrhea of 3 months duration underwent computed tomography urography after ultrasound revealed a solitary mass in the left renal hilum. Histologically, the prominent eosinophilic tumor cells formed an alveolar- or acinar-like configuration. The immunohistochemical profile (alpha-inhibin+, Melan-A+, synaptophysin+) indicated the adrenocortical origin of the tumor, diagnosed as ectopic adrenocortical adenoma. The patient was alive with no tumor recurrence or metastasis at the 3-month follow-up examination.

Conclusions: The unusual histological appearance of ectopic adrenocortical adenoma may result in its misdiagnosis as oncocytoma or clear cell renal cell carcinoma, especially if the specimen is limited. This case provides a reminder to pathologists to be aware of atypical cases of this benign tumor. Although uncommon, an ectopic adrenal lesion should be included in the differential diagnosis of tumors involving the renal hilum. A misdiagnosis of this benign condition as a malignant renal tumor may have severe consequences for the patient, including unnecessary radical nephrectomy. Preoperative biopsy and appropriate immunohistochemical staining will assist in determining the origin and nature of the tumor and in avoiding intraoperative uncertainty.

No MeSH data available.


Related in: MedlinePlus