Severe Cholestatic Jaundice (Stauffer Syndrome) as a Rare Paraneoplastic Manifestation in Adrenocortical Carcinoma.
IL-6
Stauffer syndrome
adrenocortical carcinoma
cholestasis
hyperbilirubinemia
jaundice
liver failure
paraneoplastic syndrome
Journal
Journal of the Endocrine Society
ISSN: 2472-1972
Titre abrégé: J Endocr Soc
Pays: United States
ID NLM: 101697997
Informations de publication
Date de publication:
01 Aug 2022
01 Aug 2022
Historique:
received:
15
04
2022
entrez:
11
7
2022
pubmed:
12
7
2022
medline:
12
7
2022
Statut:
epublish
Résumé
Adrenocortical carcinoma (ACC) is a rare malignancy arising from the adrenal cortex. While ACC can be associated with adrenal hormone excess syndromes, classic paraneoplastic syndromes are rarely seen. Stauffer syndrome, a paraneoplastic phenomenon characterized by reversible cholestasis in the absence of liver metastases, has been described with renal carcinoma and other malignancies but has not been previously reported in ACC. A 38-year-old man presented with emesis, painless jaundice, pruritus, and weight loss. Laboratory evaluation demonstrated elevated total bilirubin of 8.7 mg/dL (N < 1.3 mg/dL). Computed tomography revealed a 20.4-cm left adrenal mass without evidence of liver metastases. The patient's condition deteriorated rapidly with progressive renal failure and worsening hyperbilirubinemia. The patient underwent left adrenalectomy, nephrectomy, ureterolysis, and wedge liver biopsy. Histopathology showed necrotic ACC with tumor invasion into the adrenal capsule, no lymphovascular invasion, uninvolved margins, and Ki-67 of 40%. Kidney parenchyma exhibited diffuse pigment casts. The liver specimen contained diffuse bile deposits and minimal chronic inflammation in the portal tracts. He tested positive for the pathogenic variant of This is the first report of a unique presentation of paraneoplastic-related hyperbilirubinemia in the setting of ACC. While extremely rare, Stauffer syndrome should still be considered in differential diagnosis in patients with ACC with liver dysfunction and jaundice without evidence of liver metastases.
Sections du résumé
Background
UNASSIGNED
Adrenocortical carcinoma (ACC) is a rare malignancy arising from the adrenal cortex. While ACC can be associated with adrenal hormone excess syndromes, classic paraneoplastic syndromes are rarely seen. Stauffer syndrome, a paraneoplastic phenomenon characterized by reversible cholestasis in the absence of liver metastases, has been described with renal carcinoma and other malignancies but has not been previously reported in ACC.
Case Presentation
UNASSIGNED
A 38-year-old man presented with emesis, painless jaundice, pruritus, and weight loss. Laboratory evaluation demonstrated elevated total bilirubin of 8.7 mg/dL (N < 1.3 mg/dL). Computed tomography revealed a 20.4-cm left adrenal mass without evidence of liver metastases. The patient's condition deteriorated rapidly with progressive renal failure and worsening hyperbilirubinemia. The patient underwent left adrenalectomy, nephrectomy, ureterolysis, and wedge liver biopsy. Histopathology showed necrotic ACC with tumor invasion into the adrenal capsule, no lymphovascular invasion, uninvolved margins, and Ki-67 of 40%. Kidney parenchyma exhibited diffuse pigment casts. The liver specimen contained diffuse bile deposits and minimal chronic inflammation in the portal tracts. He tested positive for the pathogenic variant of
Conclusion
UNASSIGNED
This is the first report of a unique presentation of paraneoplastic-related hyperbilirubinemia in the setting of ACC. While extremely rare, Stauffer syndrome should still be considered in differential diagnosis in patients with ACC with liver dysfunction and jaundice without evidence of liver metastases.
Identifiants
pubmed: 35811575
doi: 10.1210/jendso/bvac101
pii: bvac101
pmc: PMC9261502
doi:
Types de publication
Case Reports
Langues
eng
Pagination
bvac101Informations de copyright
Published by Oxford University Press on behalf of the Endocrine Society 2022.
Références
Best Pract Res Clin Endocrinol Metab. 2009 Apr;23(2):273-89
pubmed: 19500769
J Clin Invest. 2019 Jul 15;129(8):3324-3338
pubmed: 31305264
J Urol. 2003 Jan;169(1):5-11
pubmed: 12478091
Eur Respir Rev. 2020 Sep 17;29(157):
pubmed: 32943413
Cureus. 2019 Oct 30;11(10):e6032
pubmed: 31824799
J Clin Endocrinol Metab. 2013 Dec;98(12):4551-64
pubmed: 24081734
Clin Endocrinol (Oxf). 2014 Jun;80(6):925-7
pubmed: 23848572
Mol Clin Oncol. 2018 Apr;8(4):609-612
pubmed: 29541472
Lancet Diabetes Endocrinol. 2020 Nov;8(11):894-902
pubmed: 33065059
Cureus. 2017 May 8;9(5):e1230
pubmed: 28611937
Semin Urol Oncol. 1996 Nov;14(4):216-22
pubmed: 8946620
N Engl J Med. 1990 Apr 26;322(17):1195-201
pubmed: 2325710
Nat Rev Urol. 2010 Dec;7(12):681-92
pubmed: 21139643
Turk J Urol. 2018 Sep 12;45(1):70-72
pubmed: 30668309
J Urol. 1998 Mar;159(3):718-22
pubmed: 9474133
Case Rep Endocrinol. 2016;2016:5731417
pubmed: 27957352
BMJ Case Rep. 2010;2010:
pubmed: 22461853
Endocr J. 2000 Apr;47(2):119-25
pubmed: 10943735
Eur Urol. 2014 Apr;65(4):832-8
pubmed: 24268504
Cancer. 2000 Feb 15;88(4):711-36
pubmed: 10679640
Case Rep Oncol. 2020 Jun 26;13(2):742-746
pubmed: 32774269
J Urol. 1978 Apr;119(4):468-71
pubmed: 650745
Gastroenterology. 1989 Nov;97(5):1319-22
pubmed: 2676684
J Clin Endocrinol Metab. 2001 Aug;86(8):3968-74
pubmed: 11502840
J Clin Endocrinol Metab. 2006 Jul;91(7):2650-5
pubmed: 16670169
BMJ Case Rep. 2018 Jun 21;2018:
pubmed: 29930184
R I Med J (2013). 2020 Dec 1;103(10):35-37
pubmed: 33261232
Urol Oncol. 2018 Jul;36(7):321-326
pubmed: 29657090
J Urol. 1992 Apr;147(4):1147-50
pubmed: 1552613
Case Rep Urol. 2019 Apr 22;2019:9745301
pubmed: 31179152