Case report: Undifferentiated carcinoma of unknown primary site after liver-small bowel transplant (2005)

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Record number: 
1888
Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
This case report describes carcinoma of donor origin after liver-intestine transplantation. In children, post transplant malignancy primarily associated with EBV PTLD, according the authors, at a frequency exceeding 25%. The tumor was an adenocarcinoma of small bowel - for which no data for further conclusions are available due to the low frequency in general population.
Time to detection: 
8 year post transplant. Carcinoma diagnosis followed numerous episodes of acute cellular rejection.
Alerting signals, symptoms, evidence of occurrence: 
EBV positive cells in surveillance endoscopy and biopsy. 9 months later, PTLD resolved and patient admitted with abdominal mass.
Demonstration of imputability or root cause: 
In situ hybridization for X and Y on tumor showed 202/203 cells containing two chromosome X centromeres (female donor genotype) in male patient.
Imputability grade: 
3 Definite/Certain/Proven
Groups audience: 
Suggest new keywords: 
malignancy
liver-intestine
Suggest references: 
Seda-Neto J, Macedo C, Jaffe R, Mazariegos GV, Sindhi R, Bond GJ, et al. Carcinoma of donor origin after liver-intestine transplantation in a child. Pediatric Transplantation. 2005;9(2):244-8.
Note: 
Please create a adverse occurence category of malignancy - other (please specifiy in text). Unfortunately we habe no category. ---- Add new MPHO type: combined liver small bowel; AO type: Carcinoma of unknown primary site (EP)
Expert comments for publication: 
Of note this exceptional case is difficult to classify: after pediatric liver intestine transplantation the recipient developed an adenocarcinoma with spread into the liver and intestine (multiple location) 8 years post transplant after exposure to intensified immunosuppression. In combination of debulking surgery and temporary cessation of immunosuppression the tumor "resolved", - unfortunately in this case causing further problems.