Erythropoietin-Producing Adrenocortical Carcinoma

Abstract

A 62-year-old Japanese male with an erythropoietin-producing adrenocortical carcinoma is presented. The elevated erythropoietin level and erythrocytosis returned to normal after surgical removal of a huge left adrenal tumor weighing 1,580 g. A histopathological diagnosis of adrenocortical carcinoma was made. Despite adjuvant combined chemotherapy, the patient died of lung and liver metastases 3.5 months after operation. Although the possibility that the elevated plasma erythropoietin level and erythrocytosis resulted from local kidney hypoxia, caused by pressure from the huge adrenal tumor, cannot be completely neglected, the positive cytoplasmatic evidence of immunoreactive erythropoietin in the carcinoma cells and the detection of a high erythropoietin level in the tumor extract on radioimmunoassay confirmed that this is a very rare case of erythropoietin-producing adrenocortical carcinoma.

Adrenocortical Carcinoma

“…However, it is unclear why this symptom is less prevalent in the modern medical era. Other rare paraneoplastic syndromes are hyperreninemic hyperaldosteronism, erythropoietin-associated polycythemia, and leukocytosis (caused by chemokine release from the tumor) (82)(83)(84).…”

Hypoxia signaling pathway: A central mediator in endocrine tumors

“…Even though advances over the past few decades in several biomarkers associated with metastasis, prognosis, and survival in ACC patients have led to a better understanding of its molecular genetics, the specific effects of HIF-1 activity in ACC and hypoxia signatures for predicting ACC prognosis have not been established ( 124 ). However, a case study of a very rare erythropoietin-producing adrenocortical carcinoma accompanied by lung and liver metastases has been reported ( 125 ). Tumor associated hypoxia could be one of the players of erythropoietin production in tumor cells.…”

Erythropoietin‐secreting metanephric adenoma presenting as erythrocytosis

“…At the very least, abdominal and pelvic imaging is useful to exclude EPO‐secreting tumors or conditions inducing local renal hypoxia. Other EPO‐secreting tumors associated with erythrocytosis can be seen in Table 1, 4–6,8–28 and include meningiomas, uterine leiomyomas, and parathyroid adenomas 1 …”

“…Other [23][24][25][26][27][28] Parathyroid adenoma Thymic carcinoma Breast adenocarcinoma Pheochromocytoma Sarcoma Small lymphocytic lymphoma Adrenocortical carcinoma…”