Sarcoidosis presenting with massive splenomegaly: 2 cases without pulmonary involvement

Area of Science:

• Internal Medicine
• Pathology
• Radiology

Background:

• Sarcoidosis is a chronic systemic granulomatous disease typically affecting lungs and mediastinal lymph nodes.
• Extrapulmonary sarcoidosis, particularly isolated splenic involvement without lung or mediastinal lymph node disease, is rare.
• Splenomegaly as an initial symptom poses diagnostic challenges, especially in regions with high prevalence of infectious or hematologic diseases.

Observation:

• Two young adults presented with massive splenomegaly and minimal systemic symptoms.
• No clinical or radiological evidence of thoracic sarcoidosis was found in either patient.
• Imaging revealed splenomegaly with multiple hypodense splenic lesions; liver and lymph node biopsies showed noncaseating granulomas.

Findings:

• Histological examination of liver and lymph node tissue revealed noncaseating granulomas, consistent with sarcoidosis.
• Infectious and autoimmune causes were ruled out through thorough investigation.
• Both patients responded well to oral corticosteroids, showing symptomatic relief and regression of splenic lesions.

Implications:

• Sarcoidosis should be considered in the differential diagnosis of splenomegaly of unknown etiology.
• Non-surgical management with corticosteroids can be effective in treating isolated splenic sarcoidosis.
• These cases highlight the importance of considering sarcoidosis to avoid unnecessary surgical interventions like splenectomy.