ABSTRACT
Resumen ANTECEDENTES: El cáncer de vulva es relativamente raro, representa menos del 1% de los tumores malignos de la mujer; su incidencia aumenta con la edad. La variedad más frecuente es el carcinoma escamoso (80 al 90%), seguido del melanoma. En este reporte se revisa un carcinoma de origen glandular, como el hidradenoma papilífero del tipo glándula mamaria (mammary-like) de la vulva. CASO CLÍNICO: Paciente de 50 años, con una lesión papular en la vulva de dos años de evolución, con crecimiento lento y progresivo, ocasional sensación de masa y dolor, con colposcopia negativa, sin antecedentes de patología mamaria y con una biopsia previa que reportó hidradenoma papilífero vulvar. Se trató con resección completa de la lesión, con anestesia regional, con bordes libres, no se identificó algún componente infiltrante. En la actualidad permanece sin evidencia de recaída ni requerimiento de tratamientos adicionales durante el seguimiento. CONCLUSIÓN: El hidradenoma papilífero es una lesión benigna, poco frecuente, relacionada con las glándulas anogenitales de tipo mammary-like, con buen pronóstico. El tratamiento recomendado es la escisión quirúrgica, que casi siempre es curativa.
Abstract BACKGROUND: Vulvar cancer is relatively rare, representing less than 1% of malignant tumors in women; its incidence increases with age. The most frequent variety is squamous cell carcinoma (80 to 90%), followed by melanoma. In this report we review a carcinoma of glandular origin, such as papilliferous hydradenoma of the vulva of the mammary gland (mammary-like) type. CLINICAL CASE: We present a 50-year-old patient with 2 years evolution of a papular lesion on the vulva with slow and progressive growth, intermitent sensation of mass and pain, with negative colposcopy, no history of breast pathology and with a previous biopsy that reported vulvar papilliferous hydradenoma. She was treated with complete resection of the lesion under regional anesthesia, with free margins, without identifying an infiltrating component and currently without evidence of relapse or requirement of additional treatments. CONCLUSION: Papilliferous hidradenoma is a rare benign lesion related to the mammary-like anogenital glands, with a good prognosis and its recommended treatment is surgical excision, which is generally curative.
ABSTRACT
El pilomatrixoma es un tumor benigno cutáneo, de frecuente aparición en los niños, que se origina en las células de la matriz del folículo piloso. Se presenta como un nódulo o tumor, aproximadamente, de 0,5 a 3 cm de tamaño, de consistencia cálcica, de bordes facetados, con piel suprayacente eritematoazulada. En ocasiones, la piel puede atrofiarse, dar la apariencia de una ampolla e, incluso, extruir material cálcico. El diagnóstico se sospecha por la presentación clínica característica y los estudios de imágenes complementarios. El diagnóstico definitivo es por los hallazgos histológicos. La extirpación quirúrgica es el tratamiento de elección. Se presentan los datos epidemiológicos, clínicos, estudios complementarios y técnicas quirúrgicas empleadas de 149 pilomatrixomas resecados en 137 pacientes tratados en los Servicios de Dermatología de los hospitales de pediatría Dr. Pedro de Elizalde y Prof. Dr. Juan P. Garrahan.
A pilomatrixoma is a benign skin tumor common in children, which develops from the matrix cells of hair follicles. It presents as a nodule or tumor of approximately 0.5-3 cm in size, with calcium-like consistency, faceted edges, and blue erythematous overlying skin. Sometimes, the skin may atrophy and look like a blister or even extrude calcium. Diagnosis is suspected based on the typical clinical presentation and supplementary imaging tests. The definite diagnosis is made according to histological findings. Surgical removal is the treatment of choice. Here we describe the epidemiological and clinical data, supplementary tests, and surgical techniques in relation to 149 pilomatrixomas resected in 137 patients treated at the Departments of Dermatology of two children's hospitals: Dr. Pedro de Elizalde and Prof. Dr. Juan P. Garrahan.
Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Surgical Procedures, Operative , Child , Pilomatrixoma , Neoplasms, Adnexal and Skin AppendageABSTRACT
Pilomatricomas can be confidently diagnosed cytologically due to their characteristic cytomorphological features. However, these lesions are rarely encountered by cytopathologists and thus pose a diagnostic dilemma to even experienced individuals, especially when the lesions are focally sampled. We describe two cases of histologically confirmed pilomatricoma. The first case is of a 13-year-old boy with posterior cervical ‘lymphadenopathy’, and the second one is of a 12-year-old girl with a lower cheek swelling. Both aspirates comprised predominantly atypical basal-like cells, with prominent nucleoli. ‘Ghost cells’ were readily identified by cell block in case two, but cell block in case one yielded no diagnostic material. In case two, pilomatricoma was accurately diagnosed pre-operatively. A cytological suspicion of a neoplastic process was raised in case one. Despite being diagnostically challenging, pilomatricoma can be diagnosed with careful observation of two unique cytological features of the lesions: (1) pathognomonic ‘ghost cells’ and (2) irregular, saw-toothed, loosely cohesive basaloid cells, with prominent nucleoli. The role of thorough sampling of the lesion, with multiple passes of various sites, cannot be overemphasized.