Authors' Affiliations

Hezborn Magacha, Department of Internal Medicine, James H. Quillen College of Medicine, East Tennessee State University, 1276 Gilbreath Dr., Box 70300, Johnson City, TN 37614-1700. Shahnawaz Notta, Department of Internal Medicine, James H. Quillen College of Medicine, East Tennessee State University, 1276 Gilbreath Dr., Box 70300, Johnson City, TN 37614-1700.

Location

D.P. Culp Center Ballroom

Start Date

4-5-2024 9:00 AM

End Date

4-5-2024 11:30 AM

Poster Number

51

Name of Project's Faculty Sponsor

Joseph David

Faculty Sponsor's Department

Nephrology Department

Classification of First Author

Medical Resident or Clinical Fellow

Competition Type

Competitive

Type

Poster Presentation

Presentation Category

Health

Abstract or Artist's Statement

We present a rare patient case of Stauffer syndrome (SS). SS manifests with many types of cancers including Renal Cell Carcinoma (RCC). Historically named nonmetastatic nephrogenic hepatic dysfunction syndrome, manifesting with reversible anicteric transaminitis, elevated alkaline phosphatase, thrombocytosis, elevated erythrocyte sedimentation rate, prolongation of prothrombin time, and in some cases hepatosplenomegaly in the absence of hepatobiliary obstruction; literature supports more than 100 cases of SS since its discovery in 1961. Most cases are associated with RCC with an incidence of 3% - 6% and soft tissue sarcomas or prostate cancer coming in second. SS was first described with renal cell carcinoma associated with hepatosplenomegaly and abnormality of liver function with characteristic features of cholestatic disease pattern with the key feature being the absence of metastasis to the liver. The mechanism of how SS occurs is poorly understood. However, the literature suggests that this may be due to increased IL-6 from renal cell carcinoma. The increased IL-6 seems to have an immuno-neuroendocrine effect on cells in the posterior pituitary causing the release of vasopressin by binding both soluble and insoluble IL-6R which activate complex cascades of transcription factors and affects the production of transport proteins made by biliary cells of the liver. One study that was treating wrongly suspected giant cell arteritis in a patient with SS used corticosteroids which regressed cholestasis and inflammatory markers. Now we would like to present a 76-year-old male with a history of prostate cancer, a left nephrectomy due to RCC, a left thigh mass, and other medical conditions who came to the hospital due to diminished oral intake and persistent diarrhea. On admission, he had hyponatremia with a sodium of 129 and elevated liver function tests with alkaline phosphatase of 240 and AST of 52. Previous workup of the hyponatremia revealed elevated urine osmolality that did not respond to IV fluid administration, which was consistent with SIADH (Syndrome of Inappropriate Anti Diuretic Hormone). A PET Scan was previously done and showed a mass on his left thigh and an MRI showed a 12.2 cm mass deep in the sartorius muscle. Labs prior to surgery were as follows: 10.8 WBC, 7.8 Hb, 344 PLT, 129 Na, 825 ALK, and 65 AST. The patient did not have jaundice during this time nor scleral icterus but had gradually worsening anemia, persistent hyponatremia due to SIADH, and persistently elevated alkaline phosphatase and AST. The patient's AST started to increase just before the renal cell carcinoma was discovered. After the patient's nephrectomy, the alkaline phosphatase started to increase, and the alkaline phosphatase and the AST remained elevated until a brief time of about two months after nephrectomy. The AST remained elevated and spiked right after the liposarcoma resection but both sodium and LFTs normalized 6 weeks later. The conclusion was that this patient had coinciding liposarcoma with clear cell RCC that led to hepatic syndromes twice without liver metastasis and SIADH of malignancy. The findings support this case as a variant of SS without jaundice or icterus.

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Apr 5th, 9:00 AM Apr 5th, 11:30 AM

SIADH and Stauffer Syndrome in a Patient with Renal cell carcinoma Coinciding with Liposarcoma

D.P. Culp Center Ballroom

We present a rare patient case of Stauffer syndrome (SS). SS manifests with many types of cancers including Renal Cell Carcinoma (RCC). Historically named nonmetastatic nephrogenic hepatic dysfunction syndrome, manifesting with reversible anicteric transaminitis, elevated alkaline phosphatase, thrombocytosis, elevated erythrocyte sedimentation rate, prolongation of prothrombin time, and in some cases hepatosplenomegaly in the absence of hepatobiliary obstruction; literature supports more than 100 cases of SS since its discovery in 1961. Most cases are associated with RCC with an incidence of 3% - 6% and soft tissue sarcomas or prostate cancer coming in second. SS was first described with renal cell carcinoma associated with hepatosplenomegaly and abnormality of liver function with characteristic features of cholestatic disease pattern with the key feature being the absence of metastasis to the liver. The mechanism of how SS occurs is poorly understood. However, the literature suggests that this may be due to increased IL-6 from renal cell carcinoma. The increased IL-6 seems to have an immuno-neuroendocrine effect on cells in the posterior pituitary causing the release of vasopressin by binding both soluble and insoluble IL-6R which activate complex cascades of transcription factors and affects the production of transport proteins made by biliary cells of the liver. One study that was treating wrongly suspected giant cell arteritis in a patient with SS used corticosteroids which regressed cholestasis and inflammatory markers. Now we would like to present a 76-year-old male with a history of prostate cancer, a left nephrectomy due to RCC, a left thigh mass, and other medical conditions who came to the hospital due to diminished oral intake and persistent diarrhea. On admission, he had hyponatremia with a sodium of 129 and elevated liver function tests with alkaline phosphatase of 240 and AST of 52. Previous workup of the hyponatremia revealed elevated urine osmolality that did not respond to IV fluid administration, which was consistent with SIADH (Syndrome of Inappropriate Anti Diuretic Hormone). A PET Scan was previously done and showed a mass on his left thigh and an MRI showed a 12.2 cm mass deep in the sartorius muscle. Labs prior to surgery were as follows: 10.8 WBC, 7.8 Hb, 344 PLT, 129 Na, 825 ALK, and 65 AST. The patient did not have jaundice during this time nor scleral icterus but had gradually worsening anemia, persistent hyponatremia due to SIADH, and persistently elevated alkaline phosphatase and AST. The patient's AST started to increase just before the renal cell carcinoma was discovered. After the patient's nephrectomy, the alkaline phosphatase started to increase, and the alkaline phosphatase and the AST remained elevated until a brief time of about two months after nephrectomy. The AST remained elevated and spiked right after the liposarcoma resection but both sodium and LFTs normalized 6 weeks later. The conclusion was that this patient had coinciding liposarcoma with clear cell RCC that led to hepatic syndromes twice without liver metastasis and SIADH of malignancy. The findings support this case as a variant of SS without jaundice or icterus.