Frantz’s tumor as incidental finding during Heller myotomy for achalasia: case report and review of literature

Abstract

Solid pseudopapillary tumor is a low-grade malignancy of the pancreas and predominantly affects young women. This neoplasm is a rare pancreatic entity with vague clinical presentation. Diagnosis is often incidental through imaging or even during surgical approach for another condition. A 22-year-old Brazilian female with gastrointestinal symptoms was diagnosed with achalasia and underwent Heller myotomy. Intra-operatory findings included an enlarging mass in distal pancreas. During follow-up for surgical approach of achalasia, a hypothesis of Frantz’s tumor was stated and spleen preserving distal pancreatectomy was performed. The pathological pathways of Frantz’s tumor is still unclear and its connection with chromosomal abnormalities is under investigation. Although the tumor has been reclassified over the years to solid pseudopapillary tumor, surgical resection remains as standard treatment. Despite a surgical challenge, surgery presents great prognosis in these patients and long-term survival. High suspicion and proper investigation are fundamental to diagnosis and early treatment.