• Archives of Plastic Surgery
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• v.35 no.1
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• pp.107-109
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• 2008

Purpose: Desmoplastic trichilemmoma is a rare variant of trichilemmoma. Histologically, it has a typical characteristic of trichilemmoma with epithelium of central portion and dense fibrous interstitium. There are only 4 reported cases of desmoplastic trichilemmoma arised in a nevus sebaceus, so we present a case of desmoplastic trichilemmoma arised in a nevus sebaceus with literature review.Methods: A 46-year-old female was clinically diagnosed as nevus sebaceous on left side of the forehead. Considering the facial contour, the mass was totally excised elliptically and direct closure was feasible with undermining subcutaneous layer.Results: Pathology report confirmed desmoplastic trichilemmoma developed from a nevus sebaceus and all tumor margins were negative. There were no evidences of tumor recurrence after surgical treatment.Conclusion: Desmoplastic trichilemmoma developed from a nevus sebaceus is a very rare tumor. Variant tumors can be developed from nevus sebaceus, so surgeon should be cautious in excising the mass totally and confirm the final pathology report.

• Archives of Craniofacial Surgery
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• v.18 no.1
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• pp.46-49
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• 2017

Nevus sebaceus is a hamartoma of the sebaceous gland that occurs congenitally, from which various secondary tumors can arise with a prevalence of 5%-6%. Benign neoplasms commonly arise from nevus sebaceous, but they have a very low malignant potential. Two neoplasms may occasionally arise within the same lesion, but it is rare for three or more neoplasms to occur in a nevus sebaceus simultaneously. A 61-year-old male patient was admitted to our hospital for a $4cm\times2.5cm$ growing tumor in a verrucous form arising within a periauricular nevus sebaceus in the post auricle of the left ear that had developed 30 years earlier. The nodule was diagnosed as 3 different types of tumors: trichilemmoma, desmoplastic trichilemmoma, and basal cell carcinoma. To our knowledge, this is the first report of the coexistence of three different tumors arising from nevus sebaceous. It contain malignant neoplasm also. Surgeons should be aware of the need for close monitoring and early complete surgical excision of sebaceous nevus in order to improve patient outcomes.

• Journal of Breast Disease
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• v.6 no.2
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• pp.79-83
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• 2018

Cowden syndrome (CS), also known as multiple hamartomas syndrome, is a rare hereditary autosomal dominant disorder caused by a germline mutation in the phosphatase and tensin homolog (PTEN) gene mapped on chromosome 10. The clinical features of CS are variable, primarily presenting as mucocutaneous lesions (99%). A mucocutaneous lesion, such as trichilemmoma of the face or keratosis of the extremities, is an important diagnostic marker for CS. CS has been reported to increase the incidence of benign and malignant neoplasms in the breast, thyroid, and gastrointestinal tract. The risk of developing malignancy in individuals with CS is up to 10 times higher than general population throughout an entire life time.

• Archives of Craniofacial Surgery
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• v.23 no.2
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• pp.83-88
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• 2022

Nevus sebaceous of Jadassohn is a congenital cutaneous hamartoma with epidermal, sebaceous, follicular, and apocrine structures that usually appears at birth or in early childhood. It has the potential to generate a variety of secondary neoplasms of different lineages, and the risk increases with patient age. Although multiple neoplasms may occasionally arise within the same lesion, the coexistence of more than five secondary tumors is extremely rare. Here we report a case of seven secondary tumors including syringocystadenoma papilliferum, desmoplastic trichilemmoma, sebaceoma, trichoblastoma, pigmented trichoblastoma, sebaceous adenoma, and tumor of follicular infundibulum arising within a nevus sebaceous. The complete diagnosis relies on the histopathological analysis of multipoint biopsies and delicate pathological sections.