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JAMA Surgery Clinical Challenge
October 05, 2016

A Massive Retroperitoneal Mass With Leukocytosis

Author Affiliations
  • 1Department of Surgery, Johns Hopkins University School of Medicine, Baltimore, Maryland
JAMA Surg. Published online October 5, 2016. doi:10.1001/jamasurg.2016.3180

A man in his 30s presented to his primary care clinician after 2 years of worsening abdominal discomfort and an increasingly large recurrent umbilical hernia. He described early satiety but had no nausea or vomiting. He reported night sweats and a 9.1-kg weight loss over the prior 6 months. His medical history was unremarkable. His social history was notable for a 15-pack-year smoking history and consumption of 6 to 8 beers per night. He had no family history of malignant disease. On examination he had no fever, a reducible umbilical hernia, a palpable left-sided abdominal mass, and significant bilateral lower extremity edema. Laboratory evaluation revealed anemia for which the patient received several blood transfusions, leukocytosis (white blood cell count, 57 000/μL), and hyponatremia (sodium level, 128 mEq/L). He was first referred to the hematology service, and a blood smear showed 74% neutrophils. A bone marrow biopsy specimen showed a hypercellular marrow with a myeloid left shift but no increase in blasts or dyspoiesis. Tests for BCR/ABL and peripheral blood JAK2 mutation were negative. Computed tomography revealed splenomegaly, hydronephrosis, and a 25-cm left-sided retroperitoneal mass (Figure). Surgical consultation was requested.

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