Adrenal Tumors in Young Adults: Case Reports and Literature Review

Abstract

The current high detection rate of adrenal tumors (4-10% of general population) is attributable to a widespread use of variety of imaging studies, especially a computed tomography. Most of them represent clinically silent and biologically indolent incidentalomas, but some adrenal tumors may pose a significant clinical challenge. Thus, in every patient with an adrenal tumor, a decision on further management is made after careful hormonal and radiological evaluation. All hormonally active tumors and those with radiological features suggesting malignancy are qualified for surgery. Approximately 80% of adrenal tumors are adrenocortical adenomas, hypertrophy, or nodular adrenocortical hyperplasia. Other histopathological diagnoses include pheochromocytoma, adrenocortical carcinoma, metastases, mesenchymal tumors, lymphomas, cysts, and ganglioneuromas. Adrenal tumors are more commonly diagnosed and better studied in elderly patients. In younger patients, under 40 years old, focal adrenal lesions are relatively rare, and histological distribution of diagnoses differs from that in elderly individuals. Younger patients are more likely to display endocrine symptoms, which raise the suspicion of an adrenal mass. In the current study, we present a case series of seven adrenal tumors occurring in young patients. The cases presented below, along with the literature review, demonstrate that the diagnosis and treatment of adrenal tumors are crucial due to endocrinopathy-derived complications and a potential risk of malignancy.

Keywords: adrenal tumor; endothelial cysts; lymphangioma; paraganglioma; pheochromocytoma.

Figure 1

Adrenal cortical adenoma. Abdomen CT, transverse cross-section.

Figure 2

An adrenal cortical adenoma is composed of cells with a foamy cytoplasm, which is rich in lipids, and resembles cells of the zona fasciculata of the adrenal cortex.

Figure 3

Pheochromocytoma. Abdomen CT, transverse cross-section.

Figure 4

Pheochromocytoma—cells with moderate nuclear pleomorphism, prominent nucleoli, and abundant granular cytoplasm that form irregular nests. Hematoxylin & eosin, 400× magnification (A). Pheochromocytoma cells show strong cytoplasmic immunoreactivity for synaptophysin. 200× magnification (B).

Figure 5

Endothelial cyst. Abdomen CT, transverse cross-section.

Figure 6

Multilocular endothelial cyst lined by endothelial cells with septal calcifications. In the lumen of the cyst, eosinophilic masses and cholesterol clefts are visible. The lesion probably represents dilated blood vessels with organized thrombi. Hematoxylin & eosin, 40× magnification.

Figure 7

Adrenal lymphangioma. Abdomen CT, transverse cross-section.

Figure 8

Adrenal lymphangioma. Abdomen CT, transverse cross-section, after eight years.

Figure 9

Adrenal lymphangioma—the tumor adjoins the adrenal cortex and consists of numerous cysts filled with proteinaceous content ((A), hematoxylin and eosin, 100× magnification), and lined with podoplanin-expressing lymphatic endothelium. Immunohistochemistry for podoplanin, 400× magnification (B).

Figure 10

Ganglioneuroma. Abdomen CT, transverse cross-section.

Figure 11

Ganglioneuroma—the tumor is mainly composed of Schwann cells forming fascicles in the myxoid stroma ((A), hematoxylin and eosin, 400× magnification). The mature ganglion cells are scattered throughout the tumor in small groups; they are characterized by abundant, eosinophilic cytoplasm and a round, eccentrically located nuclei ((B), hematoxylin and eosin, 400× magnification). Furthermore, focal lymphocytic infiltrates are visible (well visible in photo (A)).

Figure 12

Adrenal cortical carcinoma. Abdomen CT, longitudinal cross-section.

Figure 13

This adrenal tumor is composed of less than 25% of clear cells. Cells show moderate nuclear pleomorphism. A total of two blood vessels containing tumor emboli were found at the periphery of the tumor. According to the modified Weiss criteria, it suggests a malignant lesion. Hematoxylin and eosin, 40× magnification.