Malignant pilomatricoma

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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W8MD medical weight loss NYC and sleep center NYC

Malignant pilomatricoma
Malignant pilomatricoma
Synonyms	Pilomatrix carcinoma, Calcifying epitheliocarcinoma of Malherbe
Pronounce	N/A
Specialty	N/A
Symptoms	Skin nodule, ulceration, rapid growth
Complications	Metastasis, local recurrence
Onset	Typically in adults
Duration	Chronic
Types	N/A
Causes	Unknown, possibly related to genetic mutations
Risks	History of pilomatricoma, genetic predisposition
Diagnosis	Biopsy, histopathology
Differential diagnosis	Basal cell carcinoma, squamous cell carcinoma, dermatofibrosarcoma protuberans
Prevention	N/A
Treatment	Surgical excision, radiation therapy
Medication	N/A
Prognosis	Variable, depends on stage and treatment
Frequency	Rare
Deaths	N/A

Malignant pilomatricoma, also known as pilomatrix carcinoma, is a rare type of skin tumor that originates from the hair matrix cells. It is considered the malignant counterpart of the more common benign pilomatricoma.

Pathophysiology

Malignant pilomatricoma arises from the hair follicle matrix cells, which are responsible for the formation of hair shafts. These tumors are characterized by atypical cell proliferation and can invade surrounding tissues. Unlike benign pilomatricomas, malignant pilomatricomas have the potential to metastasize, although this is uncommon.

Clinical Presentation

Patients with malignant pilomatricoma typically present with a firm, nodular mass on the skin. These tumors are most commonly found on the head, neck, and upper extremities. The overlying skin may appear normal or may show signs of ulceration.

Diagnosis

The diagnosis of malignant pilomatricoma is primarily based on histological examination. A biopsy of the lesion reveals atypical basaloid cells, areas of necrosis, and infiltrative growth patterns. Immunohistochemical staining can aid in differentiating malignant pilomatricoma from other skin tumors.

Treatment

The primary treatment for malignant pilomatricoma is surgical excision with clear margins. Due to the potential for local recurrence and metastasis, wide local excision is often recommended. In some cases, adjuvant radiation therapy may be considered.

Prognosis

The prognosis for patients with malignant pilomatricoma depends on the extent of the disease at the time of diagnosis. Early detection and complete surgical excision are associated with a favorable outcome. However, the risk of recurrence and metastasis necessitates long-term follow-up.

See also

• Pilomatricoma
• Skin neoplasm
• Basal cell carcinoma
• Squamous cell carcinoma