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SMARCA4-Deficient Carcinoma of Unknown Primary Presenting with Fatal Paraneoplastic Hypercalcemia in a Heart Transplant Recipient: First Report in a Male Patient
Joint Authors
Strobel, Deike
Agaimy, Abbas
Strecker, Thomas
Source
Issue
Vol. 2017, Issue 2017 (31 Dec. 2017), pp.1-5, 5 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2017-10-11
Country of Publication
Egypt
No. of Pages
5
Main Subjects
Abstract EN
Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT), is a rare SMARCA4-driven aggressive malignancy of young age characteristically associated with paraneoplastic hypercalcemia.
Comparable neoplasms/presentations have not been reported in males.
A 39-year-old male heart transplant recipient (HTX 40 months previously) presented with multiple liver nodules and hypercalcemic crisis.
The serum parathyroid hormone-related protein (PTHrp) was significantly elevated (241 pg/ml; reference value < 57).
Liver biopsy showed poorly differentiated partially rhabdoid neoplasm expressing pancytokeratin, CK20, and focally GATA3, SATB2, p63, and SALL4.
The tumor cells showed dual loss of SMARCA4 and SMARCA2.
He died of irreversible multiorgan failure one week after admission.
To our knowledge, this is the first report highlighting the rare occurrence of paraneoplastic hypercalcemia associated with SMARCA4-deficient malignancies in males.
Although the immunophenotype suggests urothelial or upper gastrointestinal tract origin, the exact primary tumor site could not be ascertained due to rapid death of the patient.
SMARCA4 immunohistochemistry should be included in the workup of neoplasms associated with hypercalcemia irrespective of gender and site.
American Psychological Association (APA)
Agaimy, Abbas& Strobel, Deike& Strecker, Thomas. 2017. SMARCA4-Deficient Carcinoma of Unknown Primary Presenting with Fatal Paraneoplastic Hypercalcemia in a Heart Transplant Recipient: First Report in a Male Patient. Case Reports in Pathology،Vol. 2017, no. 2017, pp.1-5.
https://search.emarefa.net/detail/BIM-1149113
Modern Language Association (MLA)
Agaimy, Abbas…[et al.]. SMARCA4-Deficient Carcinoma of Unknown Primary Presenting with Fatal Paraneoplastic Hypercalcemia in a Heart Transplant Recipient: First Report in a Male Patient. Case Reports in Pathology No. 2017 (2017), pp.1-5.
https://search.emarefa.net/detail/BIM-1149113
American Medical Association (AMA)
Agaimy, Abbas& Strobel, Deike& Strecker, Thomas. SMARCA4-Deficient Carcinoma of Unknown Primary Presenting with Fatal Paraneoplastic Hypercalcemia in a Heart Transplant Recipient: First Report in a Male Patient. Case Reports in Pathology. 2017. Vol. 2017, no. 2017, pp.1-5.
https://search.emarefa.net/detail/BIM-1149113
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1149113