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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pilomatrix carcinoma is a rare, locally aggressive tumor with a tendency to recur. Distant metastases have been reported, with pulmonary lesions being the most frequent manifestation. Similar to pilomatrixoma, pilomatrix carcinoma typically presents as a nontender, firm dermal swelling and is found most commonly in the head and neck region. Although pilomatrixomas and pilomatrix carcinoma are well-recognized lesions, clinically they are frequently misdiagnosed as other skin conditions. By reviewing the literature over the past 10 years, the aims of this review are to analyze the cause, clinical presentation, histopathologic features, management and outcomes of pilomatrix carcinoma amongst children and adults.
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PMID:Pilomatrix carcinoma: 12-year experience and review of the literature. 2891 51

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.
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PMID:Not Your Average Skin Cancer: A Rare Case of Pilomatrix Carcinoma. 3288 18

Pilomatrix carcinoma (PC) is a rare malignant variant of pilomatrixoma, a skin adnexal tumor originating from hair matrix cells. It is most often located in the head, neck region, upper back and upper extremities. PC has a locally aggressive behavior but metastasis only occur in 10% of cases. Mutations in CTNNB1, the encoding gene of beta-catenin, have been found in both pilomatrixoma and PC, but other molecular alterations are unknown. The authors present a case of PC in the clitoris, the third known reported case located on the external genitalia. The tumor followed an unusual clinical course with the development of multiple metastases. Next-generation sequencing analysis of the tumor identified, in addition to a characteristic CTNNB1 mutation, pathogenic mutations in PTEN, PIK3CA, and ARID1A, which could explain the aggressive course of the disease. The diagnostic criteria of PC and the differential diagnoses of this unusual tumor in the genital area are discussed.
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PMID:Vulvar Pilomatrix Carcinoma: Morphologic and Molecular Features. 3325 3


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