Pathology of Central Nervous System Posttransplant Lymphoproliferative Disorders: Lessons from Pediatric Autopsies

Gheorghe, Gina; Radu, Oana M.; Milanovich, Samuel; Hamilton, Ronald L.; Jaffe, Ronald; Southern, James F.; Ozolek, John A.

doi:10.2350/12-01-1148-oa.1

Cited by 9 publications

(7 citation statements)

References 23 publications

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“…While EFS was only 50 ± 10%, OS was relatively good at 74 ± 9% for the whole cohort and even higher for the post‐renal transplant patients at 89 ± 10%. Due to the retrospective and heterogeneous nature of this dataset, the reasons for the favourable OS are difficult to discern, but are in contrast to the literature in which prognosis for primary paediatric CNS PTLD is generally reported as being poor 4‐7,9,10 . Our 25 patients were treated with a variety of modalities at the discretion of the local treating institutions, including RIS, systemic and intrathecal chemotherapy and rituximab, rarely radiotherapy and EBV‐specific CTLs in a few instances (three of four alive).…”

Section: Resultsmentioning

confidence: 96%

Primary post‐transplant lymphoproliferative disorder of the central nervous system: characteristics, management and outcome in 25 paediatric patients

et al. 2021

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Summary Primary central nervous system (CNS) post‐transplant lymphoproliferative disorder (PTLD) in childhood is rare. Twenty‐five patients were retrieved from nine European Intergroup for Childhood Non‐Hodgkin’s Lymphoma and/or international Berlin–Frankfurt–Münster Study Group members. Types of allografts included kidney (n = 11), liver (n = 4), heart (n = 5), bowel (n = 1) and haematopoietic stem cells (n = 4). Eighteen were male, 16 ≥ 10 years old, 21 had monomorphic disease and 24 solid intracranial tumour masses. Four‐year event‐free and overall survival rates were 50% ± 10% and 74% ± 9% respectively. This report represents the largest paediatric series of CNS PTLD reported to date, showing favourable survival odds following systemic and intrathecal chemotherapy and rituximab administration.

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Section: Resultsmentioning

confidence: 96%

Primary post‐transplant lymphoproliferative disorder of the central nervous system: characteristics, management and outcome in 25 paediatric patients

et al. 2021

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“…However, despite PTLD being the most common and best described malignant complication in children after ITx, with an incidence of 20%, its manifestation in unusual locations can remain undetected and therefore yield a poor prognosis.…”

Section: Discussionmentioning

confidence: 99%

Atypical malignancies after intestinal transplantation in children: A European single‐centre experience

Gerlach

Morland

Hobin

et al. 2020

Pediatric Transplantation

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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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“…Lung.-Sole involvement of the lungs is rare; however, lung involvement can be part of widely spread PTLD (46). In pediatric lung transplant recipients, a solitary pulmonary mass is seen in 50% of cases of lung allograft PTLD, nodules occur in 50% of patients (Fig 11), and airspace consolidation is seen in 42% of patients (47)(48)(49). Solitary or multiple lung nodules measuring 1-4 cm can be seen on chest radiographs (48).…”

Section: Chestmentioning

confidence: 99%

“…In children, PTLD of the central nervous system is rare and manifests with nonspecific symptoms that also suggest other conditions such as primary central nervous system tumors, infection, inflammation, and hemorrhage (49). Tissue biopsy is generally required for the diagnosis, because other investigations such as cerebrospinal fluid and neurologic and ophthalmologic studies are usually nondiagnostic (54).…”

Section: Central Nervous Systemmentioning

confidence: 99%

Posttransplant Lymphoproliferative Disorder in Children: A 360-degree Perspective

Marie¹,

Navallas²,

Navarro³

et al. 2020

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Posttransplant lymphoproliferative disorder (PTLD) is the most common malignancy that can occur after organ transplant in children. PTLD arises because the patient's immune system has been suppressed to protect the graft and may fail to provide an adequate immune check for transformed malignant or premalignant lymphocytes. PTLD risk factors and pathogenesis are not completely understood; however, Epstein-Barr virus is identified in many patients with PTLD and may contribute to its evolution. PTLD is a clinical challenge because the biologic behavior and clinical manifestations are diverse, and there is no standardized treatment. Treatment options include reduction of immunosuppression therapy, chemotherapy, radiation therapy, and surgical resection of localized lesions, depending on factors including the type and extent of disease, whether the patient has positive test results for the Epstein-Barr virus, and patient comorbidities. A multidisciplinary approach is essential for the appropriate management of PTLD. Diagnostic imaging modalities such as radiography, US, CT, MRI, and PET are essential in diagnosis, assessment of therapeutic response, and monitoring of this heterogeneous disease entity. When imaging findings are suggestive of PTLD, prompt tissue biopsy to identify the PTLD subtype is essential for appropriate treatment. It is important to know the proper indications and limitations of different diagnostic imaging techniques in the management of PTLD. In addition, familiarity with the imaging features of PTLD and its mimics narrows the differential diagnosis and may facilitate decision making about patient treatment. ©

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Pathology of Central Nervous System Posttransplant Lymphoproliferative Disorders: Lessons from Pediatric Autopsies

Cited by 9 publications

References 23 publications

Primary post‐transplant lymphoproliferative disorder of the central nervous system: characteristics, management and outcome in 25 paediatric patients

Primary post‐transplant lymphoproliferative disorder of the central nervous system: characteristics, management and outcome in 25 paediatric patients

Atypical malignancies after intestinal transplantation in children: A European single‐centre experience

Posttransplant Lymphoproliferative Disorder in Children: A 360-degree Perspective

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