Stauffer syndrome: a comprehensive review of the disease and diagnostic plan proposal

Elseidy, Sheref A; Awad, Ahmed K.; Mandal, Debvarsha; Elbadawy, Merihan A.; Iqbal, Amir

doi:10.1186/s43162-022-00128-6

Cited by 4 publications

(6 citation statements)

References 28 publications

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“…30,31 The most frequent finding in liver histology in SS is the presence of cholestasis defined as canalicular or bland cholestasis. 20 This type of injury, also observed in other liver conditions, is characterized by the presence of hepatocyte cholestasis and dilatation of the bile canaliculi with the presence of bile thrombus, but without evidence of ductal injury and interface hepatitis or portal inflammation. 13,14 In this case report, the patient presented with a type 1 pRCC of the right kidney associated with symptomatic cholestasis due to the presence of jaundice and pruritus.…”

Section: Discussionmentioning

confidence: 90%

“…14,20 Increased levels of interleukin (IL)-6 and/or IL-18 have been associated with SS and could act synergistically. 14,20 The production of IL-6 by RCC in vivo has shown a high correlation with paraneoplastic manifestations, such as fever, weight loss, elevated C-reactive protein, and haptoglobin. [21][22][23] A complete resolution of the hepatic biochemical alterations in SS has been observed using anti-IL-6 monoclonal antibodies.…”

Section: Discussionmentioning

confidence: 99%

“…9,14,19 The pathophysiology of SS is not fully clarified and would result from the following proposed mechanisms: (i) release of humoral procholestatic substances by tumor cells, (ii) release of humoral factors by tumor microenvironment, and (iii) immune-mediated cholestatic injury. 14,20 Increased levels of interleukin (IL)-6 and/or IL-18 have been associated with SS and could act synergistically. 14,20 The production of IL-6 by RCC in vivo has shown a high correlation with paraneoplastic manifestations, such as fever, weight loss, elevated C-reactive protein, and haptoglobin.…”

Section: Discussionmentioning

confidence: 99%

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Icteric Variant of Stauffer Syndrome as a Paraneoplastic Manifestation of Type 1 Papillary Renal Cell Carcinoma

et al. 2023

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Intrahepatic cholestasis as a paraneoplastic manifestation was first described by Dr. Maurice H. Stauffer in 1961. This paraneoplastic manifestation was primarily associated with renal cell carcinoma characterized by abnormal liver enzymes without hepatic metastasis. Stauffer syndrome is classified into 2 types: classical and jaundice variants. Indeed, the jaundice variant is extremely rare and only described in 13 published cases. We report a case of intrahepatic cholestasis associated with a type 1 papillary renal cell carcinoma with complete resolution after surgical treatment.

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Section: Discussionmentioning

confidence: 90%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Icteric Variant of Stauffer Syndrome as a Paraneoplastic Manifestation of Type 1 Papillary Renal Cell Carcinoma

et al. 2023

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“…The diagnostic algorithms utilized were comparable to those recommended by Elseidy et al in 2022 [ 17 ]. The tests confirmed liver dysfunction with a cholestatic pattern linked to jaundice.…”

Section: Discussionmentioning

confidence: 94%

Stauffer Syndrome: Another Strange Case of the Internist's Tumor

Muñoz Rossi,

Gallo,

Guaiquil

et al. 2023

Cureus

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Stauffer syndrome is a paraneoplastic disorder associated with renal cell carcinoma (RCC), which often can be the first manifestation of the tumor. It is characterized by nephrogenic hepatic dysfunction that reverses after removing the primary tumor. Stauffer syndrome has been associated with a worse prognosis in patients with RCC. We are presenting a case of a patient with an uncommon cholestatic jaundice variant. The patient also had hepatic profile alterations, but no metastasis existed. These findings meet the diagnostic criteria for Stauffer syndrome in the context of RCC as the first clinical manifestation in the diagnostic approach to malignancy-associated disease. A 74-year-old patient, with a history of obesity, hypertension, and type 2 diabetes mellitus, went for advice due to right upper abdominal pain, icterus accompanied by emetic episodes, and a cholestatic pattern in the hepatic profile. Autoimmune hepatitis was ruled out based on immunological testing. Imaging revealed evidence of a mass in the lower pole of the right kidney, suspicious of malignant neoplasia, and a distant paraneoplastic syndrome consistent with the cholestatic variant of Stauffer syndrome. This is an exciting case of Stauffer syndrome as the initial presentation of RCC associated with a cholestatic variant, providing relevant information about this uncommon condition in our setting.

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“…According to a recent review from Elseidy et al [ 19 ] (from 2022), diagnostic criteria for Stauffer syndrome are currently lacking. A stepwise diagnostic plan was proposed that includes liver function tests, abdominal ultrasound, MRCP and contrast-enhanced CT of the abdomen and pelvis.…”

Section: Discussionmentioning

confidence: 99%

Renal cell carcinoma presented with a rare case of icteric Stauffer syndrome: A case report

Popov¹,

Antonov²,

Atanasova³

et al. 2022

WJCC

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BACKGROUND Paraneoplastic syndromes remain poorly understood and manifest as multifaceted clinical symptoms, making their diagnosis difficult. Cholestasis can be observed in various malignancies. In rare cases, it can be a paraneoplastic manifestation, most often associated with renal cell carcinoma and other urogenital tumors, as well as with bronchial carcinoma. The classical form of Stauffer syndrome presents with a reversible anicteric increase of cholestatic liver function tests, thrombocytosis, coagulation impairment, and hepatosplenomegaly, without any proven hepatobiliary obstruction or metastases. CASE SUMMARY We report a patient who presented with elevated liver enzymes, cholestatic jaundice, weight loss and pruritus, in whom renal cell carcinoma was incidentally found during hospitalization. Clinical, laboratory, and imaging tests excluded primary hepatic cause or metastatic disease. Jaundice and laboratory abnormalities reversed completely a few months after nephrectomy. This case is an example of the many sides of renal cell carcinoma, and it focuses the clinicians’ attention on the differential diagnosis of cholestasis, including Stauffer syndrome and its variant. Thus, the correct diagnosis can be straightforward and the associated malignancy can be treated promptly. All cases should be followed up with a multidisciplinary team. Interleukin (IL)-6 is proposed to contribute to the pathophysiology of the condition. The probable mechanism is proinflammatory activity by the IL-6 cytokine, causing elevation of C-reactive protein and haptoglobin and inhibition of hepatobiliary transporter gene expression, impairing biliary outflow. CONCLUSION Despite being rare, Stauffer syndrome is a potentially reversible paraneoplastic condition, when the primary cause is treatable. This syndrome should be considered by clinicians because of the remediable liver disturbance, after successful treatment of the underlying malignancy.

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Stauffer syndrome: a comprehensive review of the disease and diagnostic plan proposal

Cited by 4 publications

References 28 publications

Icteric Variant of Stauffer Syndrome as a Paraneoplastic Manifestation of Type 1 Papillary Renal Cell Carcinoma

Icteric Variant of Stauffer Syndrome as a Paraneoplastic Manifestation of Type 1 Papillary Renal Cell Carcinoma

Stauffer Syndrome: Another Strange Case of the Internist's Tumor

Renal cell carcinoma presented with a rare case of icteric Stauffer syndrome: A case report

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