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Chondromesenchymal hamartoma
Joseph Junewick, MD FACR
over 11 years ago
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Case Detail

Anatomy: Gastrointestinal
Heather Borders, MD
Diagnostic Category: Neoplasia Malignant
Created: over 10 years ago
Updated: over 10 years ago
Tags: PEDS
Modality/Study Types: CT
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8 year old patient with a mitochondrial disorder and history of liver transplant two years ago. Presents with abdominal pain and an ultrasound was performed.

Case Images



Clinical Notes

Ultrasound revealed numerous liver nodules and two large abdominal masses.


Numerous low attenuation liver lesions and renal lesions, density compatible with soft tissue. Large retroperitoneal and mesenteric masses, homogenous in density. Encasing multiple vessels. Small epidural soft tissue mass (not shown). Para esophageal lymph node (not shown).


In this patient with a history of liver transplant the findings are most compatible with PTLD.

Most of the transplant patients who develop lymphoma are actively infected with Epstein-Barr virus. The virus directly infects B lymphocytes and induces a diffuse polyclonal B-lymphocyte proliferation. In immunocompetent patients this proliferation is ultimately reversed mainly by an intact cytotoxic T-cell function. In immunocompromised patients, weak or suppressed T-cell function leads to an excessive B-cell proliferation which results in a disease spectrum ranging from mild diffuse polyclonal adenopathy to malignant monoclonal lymphoma. This disease spectrum is referred to as posttransplant or posttransplantation lymphoproliferative disorder (PTLD).

The incidence of malignancies in organ transplant patients is approximately 6% and PTLD accounts for 20% of the tumors. The frequency of PTLD varies depending on the type of transplant: 2.2% of liver, 1% of kidney, 1.8% of heart, and 4.6% of heart-lung transplants.

PTLD occurs as early as 1 month after transplantation.

Reduction of immunosuppression is the major form of therapy for PTLD. This treatment may be combined with the administration of acyclovir, an antiviral agent that is given to combat the Epstein-Barr virus infection.

Extranodal disease (81%) is more common than lymphadenopathy (22%) in patients with PTLD. Single or multiple organ masses are the characteristic radiographic presentations of PTLD. Any of the solid organs, hollow viscera, abdominal, retroperitoneal and iliac lymph nodes, retroperitoneal musculature, or peritoneum of the abdomen can be involved in PTLD.

The liver was the most common site of PTLD involvement at CT in patients with abdominal disease. Three patterns of hepatic disease were seen. Discrete, low-attenuating nodular lesions, ranging from 1 to 4 cm in diameter and from one to more than 20 in number. An infiltrative pattern, consisting of a geographic or ill-defined region of low attenuation, with or without hepatomegaly and a third pattern, characterized by a porta hepatis mass with direct extension into the biliary tree.

Distinguishing between the polymorphic and monomorphic subtypes is important for treatment planning. This distinction is not reliably made on the basis of imaging features alone


AJR 2000; 175:183-188. CT Findings in Posttransplantation Lymphoproliferative Disorder of Renal Transplants.Thomas G. Vrachliotis

October 1999 Radiology, 213, 73-78. Posttransplantation Lymphoproliferative Disorder of the Abdomen: CT Evaluation in 51 Patients. Perry J. Pickhardt, MD and Marilyn J. Siegel, MD

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