The DOI matches its Crossref record; 17 of the 17 references it cites were found in a registry or an index.
An adrenal rest tumour in the perirenal region in a patient with congenital adrenal hyperplasia due to congenital 3β-hydroxysteroid dehydrogenase deficiency
Abstract
Background: In contrast to the high incidence of testicular adrenal rest tumours (TART) in adult male patients with congenital adrenal hyperplasia (CAH), ovarian adrenal rest tumours in female CAH patients are very rare and other locations of adrenal rest tumours have never been reported. Here, we report on an adult patient with CAH due to 3β-hydroxysteroid dehydrogenase (HSD) deficiency with bilateral TART and additionally a large perirenal adrenal rest tumour. Case report: The patient was known with CAH due to 3β-HSD deficiency and treated with hydrocortisone and fludrocortisone since the neonatal period. In puberty, there was lack of compliance with consequently high POMC concentrations. At the age of 16 years, bilateral TART were detected by scrotal ultrasound. Intensifying glucocorticoid medication did not result in decrease in POMC concentrations and shrinkage of size of the tumours. At the age of 23 years, abdominal ultrasound was performed because of abdominal complaints, showing a round inhomogeneous structure with a diameter of 4 cm near the left renal hilus. A computer tomography scan showed a multinodular lesion in the retroperitoneal region beside the left kidney. Histological investigation after removal of the tumour showed sheets of large polygonal cells with abundant eosinophilic cytoplasm, separated by dense fibrous tissue strands. The histological and immunohistochemical profile resulted in a diagnosis of an adrenal rest tumour. Conclusion: In adult CAH patients, ectopic adrenal rest tumours can be present outside the testicular region. Further investigations are necessary to determine whether regularly screening for these tumours is useful.
Overview
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
TART shrinkage and reversal of infertility are possible with glucocorticoid therapy.
“TART shrinkage and reversal of infertility are possible with glucocorticoid therapy; 120”
From Congenital adrenal hyperplasia (2017) · cites this paper as [120]
Citations
Cites
Stikkelbroeck, N. M. M. L., Otten, B. J., Pasic, A., Jager, G. J., Sweep, C. G. J. F., Noordam, K., & Hermus, A. R. M. M. (2001) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.86.12.8090 · Cited by 305
Self-citationLong Term Outcome in Adult Males with Classic Congenital Adrenal Hyperplasia
Cabrera, M. S., Vogiatzi, M. G., & New, M. I. (2001) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.86.7.7668 · Cited by 234
Stikkelbroeck, N. M. M. L., Suliman, H. M., Otten, B. J., Hermus, A. R., Blickman, J. G., & Jager, G. J. (2003) · European Radiology · doi:10.1007/s00330-002-1786-3 · Cited by 126
Self-citationder Grinten, H. L. C., Otten, B. J., Sweep, F. C. G. J., Span, P. N., Ross, H. A., MEULEMAN, E. J. H., & Hermus, A. R. (2007) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2007-0337 · Cited by 123
Self-citationIdentification of a novel population of adrenal-like cells in the mammalian testis
Val, P., Jeays-Ward, K., & Swain, A. (2006) · Developmental Biology · doi:10.1016/j.ydbio.2006.07.030 · Cited by 114
Activation of Testicular Adrenal Rest Tissue by Prolonged Excessive ACTH Production
Hamwi, G. J., Gwinup, G., Mostow, J. H., & Besch, P. K. (1963) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem-23-9-861 · Cited by 76
Ectopic adrenal tissue in the spermatic cord in pediatric patients: surgical implications
Méndez‐Gallart, R., Tellado, M., Argibay, I. S., Liras, J., Sánchez-Abuín, A., País, E., & Vela, D. (2006) · International braz j urol · doi:10.1590/s1677-55382006000200013 · Cited by 70
Massive Hyperplasia of Testicular Adrenal Rests in a Patient with Nelson’s Syndrome
Johnson, R. E., & Scheithauer, B. W. (1982) · American Journal of Clinical Pathology · doi:10.1093/ajcp/77.4.501 · Cited by 61
Ectopic localisation of adrenal cortex
Souverijns, G., Peene, P., Keuleers, H., & Vanbockrijck, M. (2000) · European Radiology · doi:10.1007/s003309900263 · Cited by 53
Adrenal Cortical Adenoma in the Spinal Canal of an 8-Year-Old Girl
Kepes, J. J., OʼBoynick, P. L., Jones, S., Baum, D., McMillan, J., & Adams, M. E. (1990) · The American Journal of Surgical Pathology · doi:10.1097/00000478-199005000-00008 · Cited by 52
Nonfunctioning Adrenal Rest Tumor of the Liver: Radiologic Appearance
Tajima, T., Funakoshi, A., Ikeda, Y., Hachitanda, Y., Yamaguchi, M., Yokota, M., Yabuuchi, H., Satoh, T., & Koga, M. (2001) · Journal of Computer Assisted Tomography · doi:10.1097/00004728-200101000-00018 · Cited by 51
Ovarian Adrenal Rest Tissue in Congenital Adrenal Hyperplasia - A Patient Report
der Grinten, H. L. C., de Kaa, C. A. H.-V., & Otten, B. J. (2006) · Journal of Pediatric Endocrinology and Metabolism · doi:10.1515/jpem.2006.19.2.177 · Cited by 35
Self-citationEctopic adrenocortical nodules along the spermatic cord of children
Savaş, Ç., Çandır, Ö., Bezir, M., & Çakmak, M. (2001) · International Urology and Nephrology · doi:10.1023/a:1014429119226 · Cited by 34
Ectopic adrenal tissue in the spermatic cord. Case report and review of the literature.
Russo, F., Pasquale, B. D., Romano, G., Vicentini, C., Manieri, C., Tubaro, A., & Miano, L. (1998) · PubMed · Cited by 29
Özel, Ş. K., Kazez, A., & Akpolat, N. (2006) · Pediatric Surgery International · doi:10.1007/s00383-006-1826-1 · Cited by 24
Ectopic Adrenal Cortex Tissue in the Spermatic Cord in Association with Testicular Cancer
Mari, M., Cagnazzi, E., Bollito, E., & Bellina, M. (2004) · Urologia Internationalis · doi:10.1159/000079705 · Cited by 14
Adrenal rests in the inguinal region in children
Altin, M. E. B., gdu, Z. H. G., & Aksoy, F. (1992) · Pediatric Surgery International · doi:10.1007/bf00178826 · Cited by 14
Flagged first, then by citations
Cited by
Sorted by citations
Congenital adrenal hyperplasia
El-Maouche, D., Arlt, W., & Merke, D. P. (2017) · The Lancet · doi:10.1016/s0140-6736(17)31431-9 · Cited by 669
Al Alawi, A. M., Nordenström, A., & Falhammar, H. (2019) · Endocrine · doi:10.1007/s12020-018-01835-3 · Cited by 77
Tiosano, D., Vlodavsky, E., Filmar, S., Weiner, Z., Goldsher, D., & Bar-Shalom, R. (2010) · Hormone Research in Paediatrics · doi:10.1159/000295722 · Cited by 65
Corcioni, B., Renzulli, M., Marasco, G., Baronio, F., Gambineri, A., Ricciardi, D., Ortolano, R., Farina, D., Gaudiano, C., Cassio, A., et al. (2021) · Translational Andrology and Urology · doi:10.21037/tau-20-998 · Cited by 39
Sonography and magnetic resonance imaging characteristics of testicular adrenal rest tumors
Yılmaz, R., Şahin, D., Aghayev, A., Erol, O. B., Poyrazoğlu, Ş., Saka, N., & Yekeler, E. (2018) · Polish Journal of Radiology · doi:10.12659/pjr.901986 · Cited by 35
Chen, H.-D., Huang, L.-E., Zhong, Z.-H., Su, Z., Jiang, H., Zeng, J., & Liu, J.-C. (2017) · Endocrine Pathology · doi:10.1007/s12022-016-9461-4 · Cited by 24
Bilateral Ovary Adrenal Rest Tumor in a Congenital Adrenal Hyperplasia Following Adrenalectomy
Zaarour, M. G., Atallah, D. M., Trak-Smayra, V. E., & Halaby, G. (2014) · Endocrine Practice · doi:10.4158/ep13092.cr · Cited by 23
Güven, A., & Polat, S. (2016) · Journal of Clinical Research in Pediatric Endocrinology · doi:10.4274/jcrpe.3306 · Cited by 18
The role of imaging in congenital adrenal hyperplasia
Teixeira, S. R., Elias, P. C. L., Andrade, M. T. S., Melo, A., & Elías, J. (2014) · Arquivos Brasileiros de Endocrinologia & Metabologia · doi:10.1590/0004-2730000003371 · Cited by 17
Fang, B., Cho, F. K. Y., & Lam, W. W. M. (2013) · Journal of Radiology Case Reports · doi:10.3941/jrcr.v7i12.1933 · Cited by 12
Kassaby, S., Velilla, R. E., & Shurbaji, M. S. (2016) · Human Pathology Case Reports · doi:10.1016/j.ehpc.2016.08.007 · Cited by 11
Testicular tumors of adrenogenital syndrome: From physiopathology to therapy
Naouar, S., Braiek, S., & Kamel, R. E. (2017) · La Presse Médicale · doi:10.1016/j.lpm.2017.05.006 · Cited by 10
Ectopic adrenal tissue in the kidney: A systematic review
Marchi, D. D., Tafuri, A., Mantica, G., Shakir, A., Scarfò, F., Passaretti, G., Smelzo, S., Proietti, S., Rigatti, L., Lucianò, R., et al. (2021) · Archivio Italiano di Urologia e Andrologia · doi:10.4081/aiua.2021.4.481 · Cited by 5
Woman with virilizing congenital adrenal hyperplasia and leydig cell tumor of the ovary
Salazar, R. F.-G., Muñoz-Darias, C., Haro‐Mora, J. J., Almaraz, M. C., Audı́, L., Martínez-Tudela, J., Yahyaoui, R., & Esteva, I. (2014) · Gynecological Endocrinology · doi:10.3109/09513590.2014.907260 · Cited by 5
Giant Macroorchidism in Congenital Adrenal Hyperplasia
Zadik, Z., Burundukov, E., & Zung, A. (2009) · Journal of Pediatric Endocrinology and Metabolism · doi:10.1515/jpem.2009.22.12.1095 · Cited by 3
Yıldız, M., Bayram, A., Baş, F., Karaman, V., Toksoy, G., Poyrazoğlu, Ş., Soysal, F. G., Önder, S. Y., Uyguner, Z. O., & Darendelıler, F. (2022) · European Journal of Endocrinology · doi:10.1530/eje-21-0913 · Cited by 3
Gawlik, Z., Preizner-Rzucidło, E., Kaleta, K., Szwarkowska, M., Wróblewska, M., Jurek, A., Książek, T., Jażdżewski, K., Siejka, A., Starzyk, J., et al. (2025) · Frontiers in Endocrinology · doi:10.3389/fendo.2025.1642051 · Cited by 1
Nationellt miljökvalitetsmål på lokal nivå
Engkvist, F. (2011)
Mazzeo, P., Tizianel, I., Galuppini, F., Sbaraglia, M., & Barbot, M. (2024) · BMC Endocrine Disorders · doi:10.1186/s12902-024-01635-z
Congenital Disorders of the Adrenal Gland
(2024) · American Registry of PathologyArlington, Virginia eBooks · doi:10.55418/9781933477473-02
Daga, A., Phadte, A., Karlekar, M. P., Sarathi, V., Lila, A. R., Hegishte, S. C., Memon, S. S., Barnabas, R., Shah, N., Mahadevan, S., et al. (2026) · Endocrine · doi:10.1007/s12020-026-04592-w
Metadata
- Authors
- Claahsen-van der Grinten, H. L., Duthoi, K., Otten, B. J., d'Ancona, F. C. H., Hulsbergen-vd Kaa, C. A., & Hermus, A. R. M. M.
- Venue
- European Journal of Endocrinology
- Publisher
- Oxford University Press (OUP)
- Published
- 2008-10
- Volume
- 159
- Issue
- 4
- Pages
- 489-491
- ISSN
- 0804-4643 (ISSN-L)
- 1479-683X
- OpenAlex
- W2118803760
- Cited by
- 21
- References
- 17
- Citations by year
- 2026: 1 · 2025: 1 · 2024: 2 · 2022: 1 · 2021: 2 · 2019: 1 · 2018: 1 · 2017: 2 · 2016: 3 · 2014: 3 · 2013: 1
- FWCI
- 0.41
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- European Journal of Endocrinology (publishedVersion)
- PubMed (publishedVersion)
- Radboud Repository (Radboud University) (submittedVersion)
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Sexual Differentiation and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
- Keywords
- congenital adrenal hyperplasia, 3β-hydroxysteroid dehydrogenase deficiency, proopiomelanocortin, glucocorticoid therapy, immunohistochemistry
- MeSH terms
- Adrenal Gland Neoplasms, Adrenal Hyperplasia, Congenital, Adrenal Rest Tumor, Adult, Humans, Kidney, Male, Retroperitoneal Neoplasms, Tomography, X-Ray Computed, 3-Hydroxysteroid Dehydrogenases
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
