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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

Immunohistochemical staining. a The tumor cells are markedly and diffusely positive for alpha-inhibin. Their cytoplasm shows positive staining for b Melan-A and c synaptophysin. d Diffuse fine granular reactivity with an anti-mitochondrial antibody is seen in eosinophilic cells
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Fig3: Immunohistochemical staining. a The tumor cells are markedly and diffusely positive for alpha-inhibin. Their cytoplasm shows positive staining for b Melan-A and c synaptophysin. d Diffuse fine granular reactivity with an anti-mitochondrial antibody is seen in eosinophilic cells

Mentions: The tumor cells were markedly and diffusely positive for vimentin, alpha-inhibin (Fig. 3a), Melan-A (Fig. 3b), synaptophysin (Fig. 3c), NSE and CD56, and focally positive for pan-cytokeratin (AE1/AE3), but negative for PAX8, S100 and chromogranin A. The sustentacular pattern of S100 staining, typical in paraganglioma, was not observed in this case. Rather, the immunohistochemical profile was consistent with an adrenocortical origin of this tumor. In addition, a fine granular AMA immunoreactivity was detected in diffusely arranged eosinophilic cells located in peripheral regions of the tumor (Fig. 3d). The Ki-67 labeling index was < 1 %.Fig. 3


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)

Immunohistochemical staining. a The tumor cells are markedly and diffusely positive for alpha-inhibin. Their cytoplasm shows positive staining for b Melan-A and c synaptophysin. d Diffuse fine granular reactivity with an anti-mitochondrial antibody is seen in eosinophilic cells
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4837621&req=5

Fig3: Immunohistochemical staining. a The tumor cells are markedly and diffusely positive for alpha-inhibin. Their cytoplasm shows positive staining for b Melan-A and c synaptophysin. d Diffuse fine granular reactivity with an anti-mitochondrial antibody is seen in eosinophilic cells
Mentions: The tumor cells were markedly and diffusely positive for vimentin, alpha-inhibin (Fig. 3a), Melan-A (Fig. 3b), synaptophysin (Fig. 3c), NSE and CD56, and focally positive for pan-cytokeratin (AE1/AE3), but negative for PAX8, S100 and chromogranin A. The sustentacular pattern of S100 staining, typical in paraganglioma, was not observed in this case. Rather, the immunohistochemical profile was consistent with an adrenocortical origin of this tumor. In addition, a fine granular AMA immunoreactivity was detected in diffusely arranged eosinophilic cells located in peripheral regions of the tumor (Fig. 3d). The Ki-67 labeling index was < 1 %.Fig. 3

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