Post-Transplant Lymphoproliferative Disorder

 Site of presentation may depend partially on transplanted organ


image Extranodal (80%) > nodal involvement (20%)


• Imaging findings of post-transplant lymphoproliferative disorder parallel those of non-Hodgkin lymphoma (NHL) in immunocompetent patients

• GI tract: Imaging findings are similar to NHL, including mass-like bowel wall thickening, aneurysmal dilatation, ulcerated polyploid mass, or submucosal nodules
image Increased prevalence of ulceration and bowel perforation

• Liver: Most frequently involved abdominal solid organ
image Single or multiple poorly enhancing masses, discrete mass in porta hepatis, or diffuse infiltration of liver

• Spleen: Splenomegaly ± discrete lesions (usually multiple, hypoattenuating, and variable in size)

• Kidney: Most common site in renal transplant recipients
image Heterogeneous mass surrounding hilar vessels, parenchymal masses, or diffuse infiltrative disease

• Nodal disease: Abdominal nodal involvement in only 15-20% of cases
image Nodal involvement much less common than in immunocompetent NHL




TOP DIFFERENTIAL DIAGNOSES




• Recurrent or new malignancy

• Opportunistic infections


PATHOLOGY




• Most cases are related to B-lymphocyte proliferation due to Epstein-Barr virus (EBV) infection


CLINICAL ISSUES




• High mortality, with survival rates of only 25-35%

• Treatment: Reduction or cessation of immunosuppression can be effective, although antiviral drugs, chemotherapy, or rituximab may be necessary

image
(Left) Axial CECT in a patient post liver transplant demonstrates a new hypodense mass image in the porta hepatis, as well as an enlarging portacaval lymph node image.


image
(Right) Axial CECT in the same patient demonstrates extensive retroperitoneal lymphadenopathy image. The findings of post-transplant lymphoproliferative disorder (PTLD) in this case are indistinguishable from traditional non-Hodgkin lymphoma (NHL) in an immunocompetent patient.

image
(Left) Axial NECT demonstrates mass-like wall thickening image of a segment of colon with aneurysmal dilatation.


image
(Right) Coronal NECT in the same patient again demonstrates the significant wall thickening image of the bowel segment with dilatation. This is a common appearance for both NHL in immunocompetent patients and PTLD.


TERMINOLOGY


Abbreviations




• Post-transplant lymphoproliferative disorder (PTLD)


Definitions




• Heterogeneous group of lymphoproliferative diseases that occur in post-transplant setting (either solid organ or stem cell transplants), ranging from abnormal lymphoid hyperplasias to frank malignancies


IMAGING


General Features




• Location
image Extranodal involvement (80%) is much more common than nodal involvement (20%)
– Unlike lymphoma in general population where nodal disease predominates

image Can occur nearly anywhere, with common locations including lungs, GI tract, and CNS
– Site of presentation may depend partially on type of transplanted organ

– Abdominal cavity is most frequently involved (up to 50% of all cases)

– May occur within renal and liver allografts
image Some studies have suggested that PTLD may preferentially affect allograft itself

• Size
image Masses and nodes range from < 1 cm to huge masses


Imaging Recommendations




• Best imaging tool
image CECT for initial diagnosis

image PET/CT for staging and follow-up


CT Findings




• Imaging findings of PTLD mostly parallel those of non-Hodgkin lymphoma (NHL) in immunocompetent patients

• GI tract
image Small bowel (distal > proximal) > colon > stomach > duodenum > esophagus

image Imaging findings are similar to NHL in immunocompetent patients
– Mass-like wall thickening (most common) with aneurysmal dilatation of lumen

– Dominant polyploid mass (often with ulceration) or multiple submucosal nodules

– May present with intussusception

image Unlike lymphoma in general population, there is a markedly increased prevalence of ulceration and perforation of bowel
– Spontaneous perforation may be 1st symptom of PTLD

• Liver
image Most frequently involved abdominal solid organ

image Several possible appearances
– Most often single or multiple low attenuation, poorly enhancing masses
image Lesions may vary in size (few mm to few cm)

– Diffuse or geographic infiltration of liver with no discrete lesions (liver appears steatotic)

– Discrete mass in porta hepatis (sometimes with extension into biliary tree or gallbladder)
image Unique manifestation of PTLD (not common with immunocompetent lymphoma)

• Spleen
image Spleen involved in 10-40% of cases (particularly common after liver transplant)

image Possible appearances
– Splenomegaly (most common) ± discrete parenchymal lesions

– Parenchymal lesions are typically multiple, low-attenuation, and variable in size

image Spontaneous rupture is possible complication

• Kidney
image Most commonly involved site in renal transplant recipients and may affect native kidneys or allograft

image Renal allograft involvement
– Heterogeneous mass surrounding hilar vessels

– Multifocal parenchymal masses

image Native kidney involvement
– Almost always unilateral

– Discrete round, hypoenhancing parenchymal lesions

– Diffuse infiltrative disease with nephromegaly

• Adrenal
image Adrenal involvement in 5%

image Diffuse infiltration with adrenal enlargement or discrete homogeneous hypoenhancing mass

• Pancreas
image Rare manifestation of PTLD only described in pancreatic allograft (not native pancreas)

image Diffuse enlargement of pancreas mimicking acute pancreatitis

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Nov 16, 2016 | Posted by in GASTROINTESTINAL IMAGING | Comments Off on Post-Transplant Lymphoproliferative Disorder

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