Thiago Souza1, Fabio Francesconi1, Daniel Auade1, Marcos Ricci1
Keywords: CYSTS, SURGICAL FLAPS, NEOPLASM RECURRENCE, LOCAL
A 54-year-old male patient sought care at the Oncology Center of the Amazonas Foundation in September 2010 with a 23 cm x 18 cm recurrent tumor – which had well-defined bor- ders, a lobulated surface and a hardened consistency – on his back. The tumor had been previously operated on in 2004 (Figure 1). In 2004, the histopathological analysis of the speci- men concluded it was a proliferating trichilemmal tumor (PTT). The preoperative CT scan revealed a heterogeneous, expansive oval lesion measuring 15.6 cm x 10.6 cm, with a lobulated shape, rough calcifications, and a blurring of the sub- jacent fat, adjacent to the paravertebral muscles. The tumor resection was performed with a 2 cm safety margin (Figures 2 and 3) and a bilateral myocutaneous rotation flap of the latissi- mus dorsi muscle (Figure 4) to close the extensive wound (Figure 5) . The anatomic pathologic examination of the speci- men described a tumor mass weighing 1,850g, with a diameter of 27 cm and a histology typical of TTP.
PTTs are an aggressive variant of trichilemmal cysts that often affect women over 40; they were first described as a "pro- liferating epidermoid cyst" by Wilson Jones in 1963.1-4 They have since been referred to in the literature by different names: invasive pilomatrixoma, trichoclamidocarcinoma, gigantic matrix capillar tumor and keratinizing trichilemmoma. 3,4 Originating in the hair isthmus, PTTs 4,5 are usually solitary and develop in areas with high concentrations of hair follicles, such as the scalp. 2-5 Other locations, such as the trunk, are occasional- ly described. 2-5 TPTs 2-5 present as well-circumscribed and lobu- lated masses, and the surrounding tissue may atrophy or ulcera- te.3-5 The lesions may be mistaken for squamous cell carcino- mas, both clinically and histologically. 3-5
Usually, PTTs raise controversy due to their clinical beha- vior – which is, in most cases, benign – despite their local infil- trative potential. 3 Tumors with marked cell atypia might behave as if they were benign. Likewise, in some cases there is an absen- ce of architectural atypia, however with aggressive clinical behavior, which may even infiltrate the surrounding tissue, with a possible local recurrence of the lesion. 3, 5 Lymph node metastases are described in rare cases. 3 Notwithstanding its local infiltrative potential, its clinical behavior is, in most cases, benign. 3 The treat- ment of choice is the complete resection of the tumor with sur- gical margins of at least 1 cm. 4 The patient described in this study stands out for being a man with a 27 cm lesion in an unu- sual location. 2-5 He underwent a complex surgical procedure that allowed the closure of the extensive surgical wound.
1 . Jones EW. Proliferating epidermoid cysts. Archivies of Dermatology. 1966; 94(1):11-19.
2 . Mathis ED, Honningford JB, Rodriguez HE, Wind KP, Connolly MM, Podbielski FJ. Malignant proliferating trichilemmal tumor. Am J Clin Oncol. 2001; 24(4):351-353.
3 . Folpe AL, Reisenauer TK, Mentzel T, Rutten A, Solomon AR. Proliferating trichilemmal tumors: clinicopathologic evaluation is a guide to biologic behavior. J Cutan Pathol. 2003; 30(8): 492-98.
4 . Karaca S, Kulac M, Dilek FH, Polat C, Yilmaz S. Giant proliferanting trichilemal tumor of the gluteal region. Dermatol Surg. 2005; 31(12):1734-6.
5 . Satyaprakash AK, Sheehan DJ, Sangueza OP. Proliferating Trichilemmal Tumors: A Review of the Literature. Dermatol Surg. 2007; 33(9):1102-8.