ABSTRACT
Eccrine spiradenoma is one of the rare, benign dermal neoplasm showing eccrine sweat gland differentiation. The literature search accounts for less than 100 reported cases of eccrine spiradenoma. Clinically, the lesion presents as solitary, intradermal, circumscribed, round or oval, firm, painful or tender nodule measuring from 0.3 - 5 cm in diameter. Rarely if ES, presents as multiple lesions, in combination with other types of adnexal tumors such as cylindromas & trichoepitheliomas, it can be considered as a part of the Brooke-Spiegler syndrome. Majority of these tumours appear on the head/face & upper trunk region of the body. Spiradenomas arise in early adulthood in most instances with few reports also of congenital cases and no sex predilection seen. The definitive diagnosis is made by excisional biopsy of the tumour. Complete tumor excision with clear surgical margins is considered the primary treatment for these cases. Here, we present a rare case report of a 58 years old male presenting with a soft, cystic lesion in the left lumbar region of the abdomen for past 2 years duration. Surgical excision of the tumour was done with clear margins and the specimen was sent for histopathological examination. Due to the overlapping histomorphological features, this tumour is often confused with cylindromas and trichoepitheliomas along with other vascular tumours. Eccrine Spiradenoma, although a benign tumour, but malignant transformation has been described especially in long-standing cases or multiple lesions. So, early diagnosis is very important to prevent recurrence and to identify any onset of malignant transformation
ABSTRACT
@#<p style="text-align: justify;"><strong>INTRODUCTION:</strong> Trichoepithelioma is a benign hamartoma which may exhibit similar clinical and histopathological features with basal cell carcinoma. Since prognosis is dissimilar, differentiating between them is important. Literature reveals fifteen reports of malignant transformation of multiple trichoepitheliomas into basal cell carcinoma, with none in the local setting. In Brooke-Spiegler syndrome, the incidence rate of malignant transformation of benign neoplasms has been reported in 5-10 % of patients.</p><p style="text-align: justify;"><strong>CASE REPORT:</strong> A 53-year-old Filipino-American female presented with multiple discrete to coalesced, well-defined, skin-colored to hyperpigmented, smooth, dome-shaped, rubbery papules and nodules on the face since childhood. Throughout the years, lesions increased in number and size and spread to the scalp, chest, abdomen, back, upper and posterior right thigh. Some developed ulceration, telangiectasia and pigmentation.</p><p style="text-align: justify;">Chest radiography, radiographs of the jaw, whole abdominal ultrasound, cranial CT scan, ophthalmology and otorhinolaryngology assessments were normal. Based on clinical and histopathologic findings, the final diagnosis was Brooke-Spiegler syndrome with transformation into basal cell carcinoma. Carbon dioxide (CO2) laser was used to excise large and ulcerated lesions with good cosmetic results.</p><p style="text-align: justify;"><strong>CONCLUSION:</strong> A case of a Filipino-American adult female diagnosed clinically and histologically with Brooke-Spiegler syndrome with transformation into basal cell carcinoma was presented. Given the similarities in clinical and histopathologic features of trichoepithelioma and basal cell carcinoma, accurate diagnosis should be made because of their difference in prognosis. The use of an acceptable treatment modality such as carbon dioxide (CO2) laser in this case is an important emerging fi eld of study.</p><p style="text-align: justify;"><strong>KEYWORDS:</strong> trichoepithelioma, basal cell carcinoma, Brooke-Spiegler syndrome, malignant transformation, carbon dioxide laser</p>
Subject(s)
Carcinoma, Basal Cell , Lasers, GasABSTRACT
El síndrome de Brooke-Spiegler (SBS) es una entidad rara, autosómica dominante, que ocurre por mutaciones del gen CYLD, el cual funciona como supresor de tumores. Se presenta el caso de una mujer de 50 años de edad, con historia de aparición de lesiones características de tricoepiteliomas que predominaban en nariz, región interciliar y mentón, que iniciaron desde los 14 años de edad. Desde hace 5 años refiere aumento del tamaño de lesiones en alas nasales, y una lesión en punta nasal de 2 años de evolución. Al realizarse una correlación clínica e histológica, asociada a los antecedentes familiares de la madre y hermano de la paciente, se concluyó que el cuadro clínico era compatible con tricoepitelioma múltiple familiar, una variante especial del SBS, en este caso asociado a carcinoma basocelular, que aunque no es un hallazgo común, se ha visto que se puede presentar en esta enfermedad. El diagnóstico preciso de SBS requiere de una correlación clínico-histológica, y se debe hacer un seguimiento clínico cercano para detectar cambios en las lesiones en piel, que puedan indicar una transformación maligna
Brooke-Spiegler syndrome (BSS) is a rare autosomal dominant condition that occurs due to mutations in the CYLD gene, which functions as a tumor suppressor gene. The case of a 50-year-old woman with a history of characteristic trichoepitheliomas predominantly in the nose, glabella and chin that began at 14 years of age is presented. She reports an increase in the size of the nasal ala lesions for the past 5 years, and the appearance of a new lesion in the nasal tip 2 years ago. When performing a clinical and histological correlation, associated with family history in both the mother and brother, it was concluded that the diagnosis was compatible with multiple familial trichoepithelioma, a special variant of BSS, associated in this case with basal cell carcinoma, that although not a common finding, has been seen to coexist in this disease. The diagnosis of BSS requires a clinical and histological correlation, and a close clinical follow-up must be performed to detect changes in the skin lesions that may indicate malignant transformation