Challenges in the management of life-threatening complications caused by a rare case of sarcoid-lymphoma syndrome

Abstract

Purpose: The sarcoid-lymphoma syndrome is a rare condition where both entities co-exist in the same patient. Overlapping clinical manifestations, imaging findings, and pathological characteristics pose both a diagnostic as well as therapeutic challenge, especially in the setting of life-threatening complications. The objective of this case report is to highlight rare complications caused by abdominal sarcoidosis as well as a subsequently diagnosed non-Hodgkin lymphoma.

Methods: A 35-year-old man presented with massive upper gastrointestinal bleeding caused by a large retroperitoneal mass. Following several endoscopic and radiologic interventions, successful hemostasis could only be achieved by an emergency Whipple procedure. Biopsies of the mass showed an abdominal manifestation of sarcoidosis and corticosteroid therapy was started. The patient required several additional emergency surgeries due to a complicated bronchoperitoneal fistula, splenic abscess, perforation of the sigmoid colon, small bowel leakage, and repetitive intra-abdominal bleeding.

Results: Histopathological findings finally revealed a concurrent lymphoma, as clinically suspected earlier. Ultimately, polychemotherapy was administered.

Conclusion: A concurrent lymphoma can lead to emergency surgery and should be considered when sarcoidosis treatment does not improve symptoms, lymphadenopathy occurs, or hematologic changes persist under therapy.

Keywords: Bronchoperitoneal fistula; Gastrointestinal bleeding; Gastrointestinal sarcoidosis; Retroperitoneal mass; Sarcoid-lymphoma syndrome; Sarcoidosis malignancy.