Giant pilomatrix carcinoma of the scalp with intracranial extension: A case report.

Key Clinical Message: A very rare skin cancer. Malignant variant of pilomatricoma. It is unique because of its highly aggressive behavior. Responds poorly to chemotherapy and radiotherapy. Treated with a wide surgical excision with safe margins. Abstract: Pilomatrix carcinoma is uncommon and locally aggressive tumor of the hair follicle matrix. It is a malignant variant of pilomatricoma. If left untreated for a long time may attain massive size and has the potential for distant spread. Only few cases with brain extension have been reported in the literature. Diagnosis of these tumors is established by histopathology. Although pilomatrixoma and pilomatrix carcinoma are well-recognized lesions, clinically they are frequently misdiagnosed as other skin conditions. The tumors respond poorly to chemotherapy and radiotherapy. Thus, recommended treatment is a wide surgical excision with safe margins. Herein, we report the case of a 39-year-old African woman with pilomatrix carcinoma of the scalp eroding the skull bone with intracranial extension.

Pilomatrix Carcinoma In A 4-Year-Old Child With An Unusual Presentation.

Pilomatrix carcinoma is a rare, locally aggressive variant of pilomatrixoma with a high rate of recurrence and risk of distant metastasis. We report an unusual presentation of a pilomatrix carcinoma in a 4-year-old male child who presented with recurrent lesions on his left cheek. At the age of 1 month of life, he presented with a soft tissue swelling on his left cheek. The lesion showed a circumscribed proliferation of basaloid cells with central areas of eosinophilic ghost shadow cells and intermediate cells. Basaloid nests showed round to oval, hyperchromatic nuclei with open nuclear chromatin, prominent nucleoli and frequent mitoses but no marked nuclear pleomorphism or infiltration was identified. The lesion recurred twice at the same site. Both recurrences showed similar morphology as the primary tumour however there were extensive areas of stromal necrosis, infiltrating edges, frequent mitoses with atypical forms, and lymphovascular invasion. There was no marked nuclear pleomorphism. Morphological features favoured a diagnosis of pilomatrix carcinoma. The child is still on follow-up and no recurrence has been identified to date. Pilomatric carcinoma is rarely reported in infants. Due to its rarity, aggressive histological features may be missed.

Not Your Average Skin Cancer: A Rare Case of Pilomatrix Carcinoma.

Pilomatrix carcinoma is a rare malignancy stemming from aberrant proliferation of matrical cells found in developing hair. This neoplasm demonstrates a bimodal age distribution and a proclivity for developing on the head or neck. Clinically, a firm, painless, violaceous nodule with overlying ulceration is commonly described. Pilomatrix carcinoma is considered a variable-grade malignancy that tends to be locally aggressive, though metastatic disease occurs in 10 to 16 percent of cases. Mortality rates range from 7 to 9 percent. Although there is no definitive treatment protocol, surgical intervention in the form of local excision or via Mohs micrographic surgery can be considered, with radiotherapy adopted as an effective alternative for nonsurgical, recurrent, or metastatic disease. Here, we describe the case of a 62-year-old man who presented for evaluation of a red, enlarging lesion on his forehead which became tender and started to bleed shortly before the patient presented to our clinic. The patient was ultimately referred to a tertiary care center for surgical excision and, at the time of this pubilcation, has been tumor-free for more than one year. This case of a rare and often unconsidered neoplasm underscores the importance of clinical suspicion and close patient follow up to prevent local recurrence, metastasis, and death.

Pilomatrix carcinoma of the right postauricular region: A case report and literature review.

INTRODUCTION: Pilomatrix carcinoma is a rare aggressive tumor with a high rate of local recurrence after surgical excision. Diagnosis is made by histopathology and when discovered, wide local excision has been shown to have the best results. PRESENTATION OF CASE: We report a case of a 74-year-old male incidentally found to have a large right postauricular mass and regional lymphadenopathy. The mass was biopsied and proven to be a malignant pilomatrixoma. Wide local excision and level II and III neck dissection with reconstruction using a right supraclavicular flap was performed. DISCUSSION: Pilomatrix carcinoma is a lesion first described in 1880 by Malherbe and Chenantais. It is unknown if these tumors arise de novo or arise through malignant transformation of a benign pilomatrixoma. There are similarities between the benign lesion and its malignant counterpart in terms of activating mutations in signaling pathways. A well-defined gold standard for surgical management has not been established, but currently wide local excision with safe margins is recommended along with regional lymph node dissection when metastasis is suspected. Currently, no chemotherapy regimen has been shown to be effective in local control or in preventing metastatic spread. CONCLUSION: Pilomatrix carcinoma, given its aggressive nature, has a high propensity for recurrence after excision. It is important to perform wide local excision to avoid an incomplete resection and higher recurrence rates. Further studies will be needed to create a more defined standard of treatment and to evaluate the role of adjuvant chemotherapy and radiation therapy.

Pilomatrix carcinoma of the vulva.

BACKGROUND: Pilomatrix carcinomas are rare, frequently occurring in older male patients. We report a case of vulvar pilomatrix carcinoma in a 30-year-old woman, the second known reported case occurring on the external genitalia. CASE: A 30-year-old female originally presented at an outside institution for the management of an asymptomatic vulvar mass that was biopsied and read as invasive squamous cell carcinoma. Pathology review at our institution reclassified the vulvar mass as a low-grade pilomatrix carcinoma. The patient underwent radical hemivulvectomy without an inguinal-femoral groin node dissection. She has remained without evidence of disease recurrence for more than 5 years since her diagnosis. CONCLUSION: Pilomatrix carcinoma can be confused for an invasive squamous cell carcinoma. Due to its low risk of metastases, a less radical surgical approach can be taken. Consideration of this unusual malignancy is important in the determination of appropriate management.