Management of Papillary Endothelial Hyperplasia on the Tongue
Complete surgical excision is the recommended treatment for papillary endothelial hyperplasia (PEH) of the tongue to prevent recurrence and to definitively rule out angiosarcoma.
Understanding Papillary Endothelial Hyperplasia
Papillary endothelial hyperplasia (PEH), also known as Masson's tumor, is a benign vascular lesion characterized by papillary fronds lined by proliferating endothelium. It represents approximately 2% of vascular tumors of the skin and subcutaneous tissue 1. When occurring in the oral cavity, the lips and tongue are most frequently affected sites, with a female predilection 2.
Diagnostic Considerations
PEH has significant clinical importance due to its microscopic resemblance to angiosarcoma, which can lead to misdiagnosis and inappropriate treatment 3. Key distinguishing features of PEH include:
- Confinement entirely within vascular lumens
- Absence of mitosis and necrosis
- Lack of true solid cellular areas without vascular differentiation
- Immunohistochemical positivity for endothelial markers CD34 and CD31 1
Management Approach
1. Surgical Management
- Complete surgical excision is the treatment of choice for PEH 1
- The procedure should aim for complete removal with clear margins
- The size of the lesion and proximity to critical landmarks generally do not affect surgical outcomes in terms of functional damage 1
2. Histopathological Confirmation
- Thorough histopathological evaluation is essential to differentiate PEH from angiosarcoma 4
- Typical features include:
- Well-circumscribed lesion with numerous small papillary structures
- Papillae with hyalinized hypocellular cores covered by flattened endothelium
- Positive endothelial markers (CD34, CD31) on immunohistochemistry 1
3. Post-Excision Follow-up
- Regular follow-up is recommended to monitor for potential recurrence
- Most cases show no recurrence after complete surgical excision 1
- Follow-up period should be at least 12 months post-surgery
Special Considerations
Differential Diagnosis
The most critical differential diagnosis is angiosarcoma, which requires a completely different treatment approach. Careful histopathological examination is crucial to avoid misdiagnosis 3, 4.
Recurrence Risk
Complete surgical excision generally results in excellent prognosis with minimal risk of recurrence. In a study of extravascular PEH, no recurrence was observed during a mean follow-up period of 22 months 5.
Functional Outcomes
When planning surgical excision of tongue lesions, consideration should be given to preserving tongue function. Similar to approaches used for other tongue lesions, the goal should be complete removal while maintaining mobility and swallowing function 6.
Conclusion Points
- PEH is a benign vascular lesion that can mimic malignancy
- Surgical excision is both diagnostic and therapeutic
- Complete removal offers excellent prognosis with low recurrence risk
- Careful histopathological examination is essential to differentiate from angiosarcoma