What is the treatment for pilomatricoma?

Treatment of Pilomatricoma

Complete surgical excision is the standard treatment for pilomatricoma, which provides cure without recurrence in most cases. 1

Diagnosis and Clinical Features

• Pilomatricoma (formerly known as calcifying epithelioma of Malherbe) is a rare, benign skin tumor that develops from the cells of the pilar matrix 1
• It typically presents as a hard, slow-growing subcutaneous tumor, which may occasionally be associated with pain, inflammation (18%), or abscess/ulceration (11%) 2
• These tumors are most commonly found on the face (53%), neck (21%), parotid region (21%), and scalp (5%) 2
• Pilomatricomas are more common in children, with a mean age of presentation around 4.5 years 2

Treatment Approach

Surgical Management

• Complete surgical excision is the treatment of choice for pilomatricoma 1, 2
• Two main surgical approaches are used:
  • Direct approach with complete removal of the lesion, either with or without skin resection (most common) 2
  • Indirect approach via a parotidectomy-like incision (less common, used in specific anatomical locations) 2
• Overlying skin should be included in the excision when it is adherent to the tumor to ensure complete removal 2

Surgical Considerations

• Spontaneous regression is never observed, making surgical intervention necessary 2
• Preoperative imaging (such as CT scan) may be helpful for tumors located in complex anatomical regions like the parotid area 2
• For facial lesions, careful surgical planning is important to minimize cosmetic impact while ensuring complete excision 3

Prognosis and Follow-up

• The prognosis after complete surgical excision is excellent 1, 3
• Recurrence is rare when complete excision is performed 4
• Follow-up is recommended to monitor for potential recurrence, though this is uncommon 3

Special Considerations

• Multiple pilomatricomas can occur in some patients (approximately 12% of cases) 2
• Pilomatricomas may be associated with certain syndromes, such as constitutional mismatch repair deficiency syndrome (CMMRD), which requires annual skin examinations 5
• Differential diagnosis must include malignant pilomatricoma (trichomatrical carcinoma), which has significant aggressive potential 1

Common Pitfalls

• Pilomatricomas are frequently misdiagnosed preoperatively due to their clinical polymorphism 6, 4
• Incomplete excision may lead to recurrence, emphasizing the importance of complete removal 2
• The diagnosis should be confirmed histologically to rule out malignant variants 1

Surgical excision remains the definitive treatment for pilomatricoma, with excellent outcomes and minimal recurrence when performed properly.