# Leaving the Excision Site Open After Complete Excision of a Large Paracervical Leiomyoma With Fumarate Hydratase (FH)-Deficient Morphology May Decrease the Risk of Developing Renal Cell Carcinoma

## TL;DR

Leaving the excision site open after removing a specific type of uterine tumor may reduce the risk of kidney cancer in patients with a rare genetic syndrome.

## Contribution

Suggests that leaving the excision site open after removing FH-deficient leiomyomas may lower renal cell carcinoma risk in HLRCC patients.

## Key findings

- A case of HLRCC was identified through a large FH-deficient leiomyoma in an unusual uterine location.
- Leaving the excision site open after removal may decrease the risk of developing renal cell carcinoma.
- The case highlights a potential pathophysiological link between FH-deficient leiomyomas and renal cell cancer.

## Abstract

Hereditary leiomyomatosis and renal cell cancer (HLRCC) syndrome is a rare autosomal dominant disorder characterized by cutaneous leiomyomas, uterine leiomyomas, and an increased risk of renal cell carcinoma. HLRCC may be preliminarily suspected through characteristic histopathologic features, but definitive diagnosis requires germline fumarate hydratase (FH) mutation testing. We report the case of a 41-year-old African American carrier of HLRCC phenotype who was diagnosed following hysterectomy and pathologic evaluation of a large leiomyoma in an unusually low-lying location of the uterus, demonstrating FH-deficient morphology. This case highlights potential links to the pathophysiology of how HLRCC-associated renal cell cancers may arise from FH-deficient leiomyomas around a decade later. Leaving the excision site open after complete excision of this leiomyoma with FH-deficient morphology may decrease the risk of developing renal cell carcinoma.