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Published on: June 15, 2020
Papillary endothelial hyperplasia (Masson's tumor) in children
Insights
Intravascular papillary endothelial hyperplasia (IPEH), or Masson's tumor, is a rare benign vascular tumor. This study highlights four pediatric cases, emphasizing diagnostic challenges and the need for histological confirmation in children.
Area of Science:
- Vascular Oncology
- Pediatric Pathology
- Dermatopathology
Background:
- Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's tumor, is a rare benign vascular proliferation.
- Typically found in adults, IPEH primarily affects the skin and subcutaneous vessels, often on the face and limbs.
Observation:
- This report details four pediatric cases of Masson's tumor with varied clinical presentations.
- Tumors presented as painful subcutaneous masses (1-5 cm) in unusual pediatric locations: abdominal muscle, foot, thigh, and axilla.
- Diagnostic imaging (MRI, Doppler ultrasound) was inconclusive, mimicking hematoma, lymphangioma, or hemangioma.
Findings:
- Histological examination was crucial for definitive diagnosis, distinguishing IPEH from other vascular lesions.
- Surgical excision was indicated due to pain or parental concern; incomplete resection led to a persistent residual lesion in one case.
- The study underscores the potential for IPEH in pediatric patients, including atypical presentations and locations.
Implications:
- Clinical suspicion for Masson's tumor should be raised in children presenting with sudden-onset, painful subcutaneous or intramuscular tumors with a vascular appearance.
- Accurate diagnosis relies on differentiating IPEH from malignant conditions like angiosarcoma through histological analysis.
- Early and complete surgical excision combined with histological confirmation is essential for managing pediatric Masson's tumors.
Abstract:
The intravascular papillary endothelial hyperplasia (IPEH/Masson's tumor) is a rare benign tumor of the skin and subcutaneous vessels. We report, in four pediatric cases, clinical presentation, care (diagnostic and surgical) of Masson's tumor in children. Two boys (two years) and two girls (four and six years) showed a pain subcutaneous tumor (one to five centimeters). They were in the transverse abdominal muscle, between two metatarsals, at the front of thigh and in the axilla. Imaging performed (MRI, Doppler ultrasound) evoked either a hematoma, a lymphangioma or hemangioma. The indication for removal was selected from pain and/or parental concern. The diagnosis was histologically. A lesion persisted in residual form (incomplete initial resection), and is currently not scalable for eleven years.
Discussion:
This tumor is characterized by excessive proliferation and papillary endothelial cells in the vessels, following a thrombotic event. It is found mainly in adults (no specific age), and preferentially localizes in the face and limbs. The clinical differential diagnosis of this tumor is angiosarcoma. The imagery has not allowed in our series to diagnose but still essential to eliminate differential diagnoses. Only surgical excision with histological examination can differentiate. Our study emphasizes the possibility of pediatric cases with two cases of unusual locations (abdominal and axilla). Clinical presentations we met, now lead us to direct our histologist looking for a Masson tumor in any child with a subcutaneous tumor and/or intramuscular pain, sudden onset, and vascular appearance (after excluding an arteriovenous malformation).
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