Extramedullary hematopoiesis (EH) is defined as hematopoiesis occurring in organs outside of the bone marrow. It occurs in diverse conditions, including fetal development, normal immune responses, and pathological circumstances. These sites of extramedullary hematopoiesis may present as masses mimicking malignancy or produce symptoms due to pressure effects. In the setting of an existing malignancy they may appear as metastatic deposits signifying progression of disease. It is essential to confirm this due to its prognostic and treatment implications. We report a 2-year-old little girl who presented initially with an acute history of ataxia, nystagmus, tremor, mydriasis and bruises on her left forehead. A solid left suprarenal mass was detected and a diagnosis of Stage 4 Neuroblastoma and Opsoclonus-Myoclonus syndrome was established. Subsequently she was on treatment which included chemotherapy, IVIG and stem cell transplant. On an MRI of the abdomen done a year later, a single lesion was detected in the right lobe of the liver. On subsequent short term follow up, innumerable scattered lesions were seen in the hepatic parenchyma and were thought to represent metastases. A liver biopsy showed that these hepatic lesions represented sites of extramedullary hematopoiesis. Extramedullary hematopoiesis has been uncommonly seen in the cranium and sacrum in the setting of Neuroblastoma. We believe this is a unique presentation with extramedullary hematopoiesis presenting as solid liver masses masquerading as metastases in a known case of Neuroblastoma.
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Meeting name:
SPR 2019 Annual Meeting & Postgraduate Course
, 2019
Authors:
Taori Abhijeet,
Shenouda Nazih
Keywords:
Neuroblastoma,
Extramedullary Hematopoiesis,
Metastases