ABSTRACT
Introducción: El carcinoma adenoide quístico es una neoplasia maligna poco común y a menudo agresiva, que puede surgir en una variedad de órganos portadores de glándulas. Objetivo: Caracterizar la presentación de una neoplasia maligna agresiva infrecuente, el carcinoma adenoide quístico. Caso clínico: Paciente de 56 años que acudió a cuerpo de guardia con lesión en cuero cabelludo correspondiente con cilindroma maligno. Se estudia posteriormente y se constataron además lesiones a nivel de mamas, colon y recidiva en el cuero cabelludo, que dieron como resultado carcinoma adenoide quístico. Conclusiones: Es infrecuente; realizar su diagnóstico conlleva un alto índice de sospecha y su diagnóstico es histopatológico. Su pronóstico depende de la localización primaria y tiende a ser muy agresivo cuando se localiza fuera de la mama(AU)
Introduction: Adenoid cystic carcinoma is an uncommon and often aggressive malignant neoplasm that can arise in a variety of gland-bearing organs. Objective: To characterize the presentation of an uncommon aggressive malignancy, adenoid cystic carcinoma. Clinical case: 56-year-old patient who presented to the on-call department with a lesion on the scalp corresponding to malignant cylindroma. She was subsequently studied and lesions were also found in the breast, colon and recurrence in the scalp, which resulted in adenoid cystic carcinoma. Conclusions: It is infrequent; making its diagnosis involves a high index of suspicion and its diagnosis is histopathological. Its prognosis depends on the primary location and it tends to be very aggressive when located outside the breast(AU)
Subject(s)
Humans , Female , Middle Aged , Breast Neoplasms/diagnosis , Carcinoma, Adenoid Cystic/diagnosisABSTRACT
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
Subject(s)
Carcinoma, Basal Cell , Genes, Tumor Suppressor , Machado-Joseph Disease , Acrospiroma , Carcinoma, Adenoid Cystic , Deubiquitinating Enzyme CYLDABSTRACT
Brooke–Spiegler syndrome (BSS) is a rare autosomal dominant disease characterized by the development of multiple cutaneous adnexal neoplasms namely cylindroma, trichoepithelioma and spiradenoma. The neoplasms associated with this syndrome are generally benign, but rarely they may undergo malignant transformation. A 63-year-old male presented with an ulcerated nodular lesion over glabella and multiple asymptomatic nodular lesions over face, scalp, chest and limbs. His father, grandfather and paternal cousins had history of similar lesions. Histopathological examination revealed trichoblastic carcinoma arising from trichoepithelioma over glabella and cylindroma on the chest. With these findings we arrived at a diagnosis of BSS with malignant transformation of trichoepithelioma. Trichoblastic carcinoma arising in trichoepithelioma in a patient with BSS is extremely rare with only a single case reported in literature.
ABSTRACT
El cilindroma es un tumor anexial poco frecuente con diferenciación apocrina. Su localización es principalmente en frente y cuero cabelludo. Se los clasifica en dos grupos: los de carácter hereditario, que evolucionan con el tiempo a una gran masa tumoral llamada tumor en turbante, y los esporádicos solitarios. La histopatología es el estudio obligatorio a la hora de realizarel diagnóstico definitivo. Reportamos un caso de cilindroma solitario y planteamos sus diagnósticos diferenciales con otros tumores anexiales
Cylindroma is a rare adnexal tumor with apocrine differentiation usually located on forehead and scalp. They are classified into two groups: multiple hereditary tumors and solitary cylindromas which occur sporadically and typically are not inherited. Histopathology study is mandatory when making the final diagnosis. We report a case of solitary cylindroma and we propose their differential diagnosis with other adnexal tumors.
Subject(s)
Humans , Male , Adult , Carcinoma, Adenoid Cystic , Forehead , Neoplasms , Scalp , SkinABSTRACT
Adenoid cystic carcinoma (ACC) was first described by Billroth in 1856 and was called ‘cylindroma’ due to its characteristic histological appearance. ACC is the most common malignant neoplasm of the lacrimal gland, and the second most common type of carcinoma arising in the salivary glands, following mucoepidermoid carcinoma. Palate is the most commonly affected site followed by parotid gland, submandibular gland, antrum & tongue. Characteristic features include aggressive, slow growth, with insidious destruction of surrounding tissues, perineural invasion, prolonged clinical course and the tendency for delayed onset of the distant metastases which worsens the prognosis. Long term survival can be achieved particularly with combined surgery and radiotherapy. The most common pattern is the cribriform architecture. Histopathology is the gold standard for the diagnosis of ACC. CT & MRI are considered to be of almost similar significance in detection of perineural spread with preference to MRI because of its high soft tissue contrast. Here we have mentioned a case of Adenoid Cystic carcinoma arising from minor salivary glands of palate.
ABSTRACT
Cylindromas are rare benign adnexal tumors that arise primarily on the scalp. There are 2 distinct clinical forms: a solitary form, which is sporadic, and a multiple form, which is dominantly inherited. The lesions are pink-red, nodular, firm, and usually painless, and vary in size. On light microscopic examination, the tumors are nonencapsulated and are located in the upper dermis. They are composed characteristically of aggregations of islands of basaloid cells arranged in a jigsaw pattern and surrounded by hyaline sheaths. Malignant transformation occurs rarely, although some have argued that cylindromas are always benign. We report a man who developed a cylindroma on the upper left extremity, which is an unusual site.
Subject(s)
Carcinoma, Adenoid Cystic , Dermis , Extremities , Hyalin , Islands , ScalpABSTRACT
Cylindroma has been called dermal eccrine cylindroma and turban tumor since it was first described by Ancell in 1842. These tumor usually begin as small nodules and continue to grow slowly up to serveral centimeters. The tumor most commonly appears on scalp and face. They have been given the name turban tumor because they sometimes have covered the entire scalp like a turban. The tumor rarely undergone malignant degeneration. We have found a case of cylindroma that have malignant change with skull bone involvement, thus we present this case with review of the other reports.