An unusual presentation of giant pilomatrixoma in an adult patient

Abstract

Background: Pilomatrixoma (OMIM ID #132600) is a benign cutaneous tumor originating from the pilosebaceous follicle and characterized by the presence of subcutaneous nodules of up to 3.0 cm in diameter, usually on the head, neck and upper extremities. It is most common in the first two decades of life and after the age of 60.Main observations: An adult female patient was evaluated, presenting a solid tumoral lesion with erythematous surface and purplish tone, with approximately 7.5 x 5.0 cm in size, in the interscapulovertebral region. It was thought to be an epidermoid cyst, sarcoma, calcified hemangioma, giant dermatofibroma, or nodular basal cell carcinoma. The resection of the lesion was carried out and, at the histological examination, specific details were observed that led to the diagnosis of pilomatrixoma. Conclusion:Pilomatrixoma should be suspected in the differential diagnosis of giant adnexal tumors. (J Dermatol Case Rep. 2013; 7(2): 56-59) An unusual presentation of giant pilomatrixoma in an adult patient IntroductionPilomatrixoma (PM; OMIM ID #132600) was first described in 1880 by Malherbe and Chenatais as a "calcified epithelioma of Malherbe" originated from sebaceous glands. In 1961, Forbis and Helwig demonstrated that the tumor originated from the hair matrix; therefore, the name pilomatrixoma. 1-3 PM is the most common hair-follicle tumor and it accounts for one of every 500 specimens submitted by dermatologists. 4 Clinically, the lesion is a benign cutaneous tumor usually characterized by a solitary asymptomatic, firm, and skin-colored faint blue/red nodule in the deep dermis and subcutaneous tissue, with an average size of 0.5 to 3.0 cm. The lesions larger than 5.0 cm are defined as giant pilomatrixomas. Pain and tenderness may be associated symptoms. 3,5 The tumor is generally observed on the head, neck and upper extremities. 6 It is a common skin neoplasm in the pediatric population, but it may be found in all age groups with bimodal peaks under the age of 30 and in the sixth/seventh decades of life. 5,6 Uncommon clinical-pathological variants of PM have been reported, such as bullous like, anetodermic, exophytic, perforating/ulcerated and lymphagiectatic. Multiple and familial cases have been described in association with myotonic dystrophy, Turner's syndrome, Gardner's syndrome, xeroderma pigmentosum, basal cell nevus syndrome and Sotos syndrome. 6,7 The present paper reports an unusual and uncommon case of pilomatrixoma on the back of an adult female patient. Case ReportA 54-year-old female patient sought the medical specialties outpatient service at School of Medical Sciences at Universidade de Marília (UNIMAR) complaining about a back injury three years ago, with progressive growth and sporadic local pain. The patient had undergone a surgery for the removal of the lesion in another hospital two and a half years before, but there was local recurrence 3 months after surgery. Krausen et al. 9 described the first case of giant PM (GPM), few similar lesions were described 5 a...

Multiple and giant perforating pilomatrixoma: a case report

“…Multiple or recurring pilomatrixomas are rarely seen and can be associated with myotonic dystrophy, Gardner syndrome, Turner syndrome, trisomy 9, spina bifida or sarcoidosis [6]. The overlying skin may be normal or have a reddish or bluish hue in 24% of cases [1,3,8]. The diagnosis may be reached by physical examination, imaging and cytology; however, there is a large number of misdiagnosis [1,3,6].…”

“…It's a relatively rare condition [1] which is frequently misdiagnosed clinically and the correct diagnosis is only histological [1,2]. This tumor presents as a solitary asymptomatic, firm, and skin-colored faint blue/red nodule in the deep dermis and subcutaneous tissue, with an average size of 0.5 to 3.0 cm [3]. However, giant pilomatrixomas (more than 5 cm) have been reported in a few cases [4].…”

Successful Excision of a 15x20cm Giant Solitary Fibrous Tumor on Nape, Originating From Submesenchymal Cells

“…Solitary fibrous tumors are mostly benign vascular tumors that characterized with spontaneous regression in 80% of cases [1][2][3]. Solitary fibrous tumor is a commonly benign vascular proliferative lesion that can be present at any age [2][3][4].…”

“…Solitary fibrous tumor is a commonly benign vascular proliferative lesion that can be present at any age [2][3][4]. Lesions usually expand to the surrounding tissue and become ulcerative in several years.…”

The Diagnostic Value of Ultrasonography in a Case of Unusual Pilomatrixoma

“…1 However, in 1961, Forbis and Helwig renamed the lesion pilomatrixoma based on its proved origin in the hair follicle matrix. 2 Pilomatrixoma or pilomatricoma is a benign appendageal growth, originating from the cells of the hair cortex, representing 0.12% of cutaneous tumors. 3 Its highest incidence is in the first and second decades of life, although some patients have been reported in their sixties.…”