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.
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Other rare paraneoplastic syndromes are hyperreninemic hyperaldosteronism, erythropoietin-associated polycythemia, and leukocytosis (caused by chemokine release from the tumor).
“Other rare paraneoplastic syndromes are hyperreninemic hyperaldosteronism, erythropoietin-associated polycythemia, and leukocytosis (caused by chemokine release from the tumor) (82–84).”
From Adrenocortical carcinoma (2014) · cites this paper as [83]
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Else, T., Kim, A. C., Sabolch, A., Raymond, V. M., Kandathil, A., Caoili, E. M., Jolly, S., Miller, B. S., Giordano, T. J., & Hammer, G. D. (2014) · Endocrine Reviews · doi:10.1210/er.2013-1029 · Cited by 892 · see citing works
Hypoxia signaling pathway: A central mediator in endocrine tumors
Watts, D., Jaykar, M. T., Bechmann, N., & Wielockx, B. (2023) · Frontiers in Endocrinology · doi:10.3389/fendo.2022.1103075 · Cited by 15 · see citing works
“Frontiers in Endocrinology 07 reported (125).”
Sobolewska, J., Respondek, W., & Witek, P. (2025) · Frontiers in Endocrinology · doi:10.3389/fendo.2025.1533711 · Cited by 5 · see citing works
“However, cases of cancer-related hypoglycemia in patients with ACC have been described (11) or hyperrenin hyperaldosteronism (12) and erythropoietin-related polycythemia (13).”
Erythropoietin‐secreting metanephric adenoma presenting as erythrocytosis
Liu, A., Kremyanskaya, M., Mehrazin, R., Si, Q., Robinson, B. D., Cushing, M. M., Hoffman, R., & Ginzburg, Y. Z. (2023) · American Journal of Hematology · doi:10.1002/ajh.27047 · Cited by 1 · see citing works
Clinical Presentation and Initial Diagnosis
Allolio, B., & Faßnacht, M. (2009) · Springer eBooks · doi:10.1007/978-0-387-77236-3_4 · Cited by 1 · see citing works
“High secretion of renin by an ACC may contribute to hypertension [27], whereas secretion of erythropoietin may induce erythrocytosis [ 28 ], which is reversed after tumor removal.”
BackgroundLin, Y., Yang, W. P., Qin, H., Cheng, H., Shen, Y., Ren, Q., Xiao, S., Yu, L., & Wang, H. (2023) · Pediatric Blood & Cancer · doi:10.1002/pbc.30588 · Cited by 1 · see citing works
Mohapatra, D., Tripathi, M., Ojha, S., et al. (2022) · doi:10.22541/au.167212462.24535484/v1
Urologic Paraneoplastic Syndromes
Chappidi, M. R., Bhadwaraj, M., & Psutka, S. P. (2026) · Springer eBooks · doi:10.1007/978-3-031-52619-0_17
- Authors
- Oka, T., Onoe, K., Nishimura, K., Tsujimura, A., Sugao, H., Takaha, M., & Kurata, A.
- Affiliations
- Osaka National Hospital (JP)
- Venue
- Urologia Internationalis
- Publisher
- S. Karger AG
- Published
- 2010-02-03
- Volume
- 56
- Issue
- 4
- Pages
- 246-249
- ISSN
- 0042-1138 (ISSN-L)
- 1423-0399
- OpenAlex
- W1981053452
- Cited by
- 8
- References
- 0
- Citations by year
- 2026: 1 · 2025: 1 · 2023: 3 · 2022: 1 · 2013: 1
- FWCI
- 0.34
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Urologia Internationalis (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
- Health Sciences › Medicine › Hematology › Erythropoietin and Anemia Treatment
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Renal cell carcinoma treatment
- Keywords
- adrenocortical carcinoma, erythrocytosis, erythropoietin, adrenal tumors, lung metastasis, liver metastasis, adjuvant chemotherapy
- MeSH terms
- Adrenal Cortex Neoplasms, Combined Modality Therapy, Erythropoietin, Humans, Male, Middle Aged, Radioimmunoassay, Tomography, X-Ray Computed, Adrenocortical Carcinoma
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