AI Article Synopsis

  • Post-transplant lymphomas, particularly related to EBV infection, are a significant concern for intestinal transplant recipients, highlighting their increased risk for unusual malignancies compared to other transplant groups.
  • A study reviewed 98 intestinal transplants in children from 1989 to 2017, identifying four cases of unusual cancers at rare sites, including PTLD and angiosarcoma, which had poor outcomes except for one child.
  • The findings suggest that about 5% of children may develop these uncommon malignancies post-transplant, stressing the need for vigilance in diagnosis and treatment.

Article Abstract

Background: Post-transplant malignancies, that is, lymphomas, are a recognized complication in intestinal transplant recipients but are mostly secondary to EBV infection. There is an increased risk for malignancies in unusual sites in intestinal transplant recipients as compared to other solid organ transplants and the general population.

Objective: To evaluate the incidence, course, and outcome of unusual malignancies in children after ITx.

Methods: Retrospective analysis of children who underwent ITx for primary digestive disorders at Birmingham Children's Hospital between January 1989 and December 2017.

Results: Ninety-eight intestinal transplants were performed in 90 children (49 males and 41 females) with an underlying primary digestive disorder. Median age was 2.7 years (0.6-16.2), and median weight was 14.5 kg (5.7-53.2) at the time of transplant. Within this cohort, we identified four cases of unusual malignancies at rare sites of presentation. One patient developed cerebral PTLD, two patients were diagnosed with SMT, located at the stomal orifice and in cervicothoracic paravertebral area, respectively, and the last patient developed a retroperitoneal angiosarcoma. Unfortunately, the overall patient outcome was poor in all but one child with SMT, who currently survives with cytotoxic T-cell therapy.

Conclusion: Unusual malignancies can occur in approximately 5% of children following ITx. A high index of suspicion is required for a timely diagnosis and adequate treatment.

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Source
http://dx.doi.org/10.1111/petr.13697DOI Listing

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