Isolated Vulvar Langerhans Cell Histiocytosis

Clinical Case Study Published in Gynecologic Oncology Reports

Nathalie D. McKenzie, MD
Nnamdi Gwacham, MD
Gynecologic Oncology Program
AdventHealth Cancer Institute

Nnamdi I. Gwacham¹, David Ward2, Nathalie D. McKenzie¹

¹Gynecologic Oncology Program, AdventHealth Cancer Institute, ²Department of Pathology, AdventHealth Orlando, Orlando, FL 32804

Summary
Primary Langerhans cell histiocytosis (LCH) is very rare and is generally encountered more in the childhood and adolescent populations. Its etiology and pathophysiology as well as reliable modes of therapy remain elusive and variable amongst many institutions. It is characterized by proliferation of dendritic cells derived from myeloid precursors. These cells share immunophenotypic and morphologic characteristics with skin Langerhans cells. The disease carries variable presentation and is rarely encountered in the female reproductive tract. When encountered, the vulva is the most common site affected. We report a case of a 31-year-old woman who presented with isolated LCH of the vulva and clitoris that underwent wide local resection and metastatic workup, including robotic-assisted laparoscopic pelvic lymph node dissection and bone marrow biopsy. Metastatic workup was negative, and she remains without recurrence.

Highlights

• Primary vulvar LCH is rare with less than 40 reported cases.
• Diagnosis of vulvar LCH requires prompt metastatic workup to rule out multi-system involvement.
• Treatment protocols for isolated vulvar LCH vary widely, and there is not an established standard of care.
• Continue surveillance is warranted even after prolonged periods of remission.

For more information or to refer a patient, call GYN Oncology Nurse Navigator Althea Buckner, MSN, APRN-AOCNP, at Call407-303-5909.

Read the Case Study: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8134974/