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Pediatric vulvar malignancies: rare but important to know
Amanda L Strickland1, Oluwole Fadare2
1Department of Pathology, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA.
Seminars in Diagnostic Pathology
|September 18, 2020
Summary
Pediatric vulvar malignancies are rare, with rhabdomyosarcoma being the most common. Accurate classification is key for effective treatment of these rare childhood cancers.
Area of Science:
- Pediatric Oncology
- Gynecologic Pathology
- Rare Cancers
Background:
- Vulvar malignancies in children are exceptionally rare, limiting clinical insight.
- A comprehensive review of published pediatric vulvar cancer cases is needed.
Purpose of the Study:
- To summarize clinicopathologic profiles of pediatric vulvar malignancies.
- To analyze management and outcomes of these rare tumors.
Main Methods:
- Systematic review of English language literature from 1970-2020.
- Inclusion of all pediatric vulvar malignancy cases (≤21 years).
Main Results:
- Rhabdomyosarcoma comprises ~50% of cases; squamous cell carcinoma, yolk sac tumors, Ewing sarcoma/PNET, and melanoma each ~10%.
- Favorable outcomes for rhabdomyosarcoma; aggressive disease and poor outcomes noted for ES/PNET.
- Associations found between squamous cell carcinoma and HPV/immunosuppression, and melanoma and lichen sclerosus.
Conclusions:
- Pediatric vulvar malignancies are rare, primarily consisting of five entities.
- Accurate pathologic classification is essential for optimal patient management.
- Further research is needed to understand specific tumor behaviors and associations.
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