Nonfunctional ectopic adrenocortical carcinoma in the lung: A case report and literature review

Abstract

BackgroundEctopic adrenocortical tissues and neoplasms are rare and usually found in the genitourinary system and abdominal cavity. The thorax is an extremely rare ectopic site. Here, we report the first case of nonfunctional ectopic adrenocortical carcinoma (ACC) in the lung.Case presentationA 71-year-old Chinese man presented with vague left-sided chest pain and irritable cough for 1 month. Thoracic computed tomography revealed a heterogeneously enhancing 5.3 × 5.8 × 6.0-cm solitary mass in the left lung. Radiological findings suggested a benign tumor. The tumor was surgically excised upon detection. Histopathological examination using hematoxylin and eosin staining showed that the cytoplasm of the tumor cells was rich and eosinophilic. Immunohistochemical profiles (inhibin-a+, melan-A+, Syn+) indicated that the tumor had an adrenocortical origin. The patient showed no symptoms of hormonal hypersecretion. The final pathological diagnosis was non-functional ectopic ACC. The patient was disease-free for 22 months and is still under follow-up.ConclusionsNonfunctional ectopic ACC in the lung is an extremely rare neoplasm that can be easily misdiagnosed as primary lung cancer or lung metastasis, both preoperatively and on postoperative pathological examination. This report may provide clues to clinicians and pathologists regarding the diagnosis and treatment of nonfunctional ectopic ACC.

An Asymptomatic, Ectopic Mass as a Presentation of Adrenocortical Carcinoma Due to a Novel Germline TP53 p.Phe338Leu Tetramerisation Domain Variant

“…Usually, ACC leads to the excessive secretion of adrenocortical hormones resulting in Cushing's syndrome, virilisation, or feminisation, which are the triggers for investigations and eventually diagnosis. Non-secreting tumours may occur in older children, which account for less than 10% of cases and are difficult to determine [1, [16][17][18][19][20][21]. The data on ectopic ACC are very scarce and limited to case descriptions with the reported localisations including the bowel wall, ovaries, lungs, and spinal region [19,20,22,23].…”

“…Non-secreting tumours may occur in older children, which account for less than 10% of cases and are difficult to determine [1, [16][17][18][19][20][21]. The data on ectopic ACC are very scarce and limited to case descriptions with the reported localisations including the bowel wall, ovaries, lungs, and spinal region [19,20,22,23]. The potential occurrence of a non-functional ACC in atypical/ectopic localisation largely precludes any pre-operative ACC suspicion unless there is a cancer-predisposition trait in the individual or the family.…”