Treatment of Malignant Cutaneous Adnexal Neoplasms. / Tratamiento de las neoplasias anexiales cutáneas malignas.

Malignant cutaneous adnexal neoplasms form a group of rare, typically low-grade-malignancy carcinomas with follicular, sebaceous, apocrine, or eccrine differentiation or a combination of the first 3 subtypes. Their clinical presentation is usually unremarkable, and biopsy is required to establish the differentiation subtype and the definitive diagnosis. Due to their rarity, no clear consensus has been reached on which treatment is most effective. Mohs micrographic surgery is considered to be the best option to prevent recurrence in the majority of patients. Radiotherapy and chemotherapy have been studied in very few cases and have rarely been shown to be effective.

Pilomatrixoma ampollar en caso pediátrico / Bullous pilomatrixoma in pediatric case

Se realiza la presentación de un caso clínico en edad pediátrica con diagnóstico de pilomatrixoma, variedad ampollar. Este tumor benigno originado en la vaina del folículo piloso muestra una incidencia elevada en las dos primeras décadas de vida. El objetivo de este artículo es presentar un caso con diagnóstico de un tumor, con características clínicas atípicas e inusuales. En ocasiones este tipo de tumor, conllevan al diagnóstico erróneo de la entidad, realizándose el diagnóstico de certeza mediante el estudio histopatológico una vez que se procede a la exéresis quirúrgica de la lesión (AU).

A clinical case with diagnosis of bullous variety pilomatrixoma in pediatric age is presented. This benign tumor originated in the sheath of the hair follicle shows a high incidence in the first 20 years of life. The aim of this work is presenting a case with tumor diagnosis, the clinical characteristics of which sometimes leads to an erroneous diagnosis of the entity in the atypical and unusual varieties. The accurate diagnosis was made through the pathologic study after the surgical excision of the lesion (AU).

Pilomatrixoma ampollar en caso pediátrico / Bullous pilomatrixoma in pediatric case

Se realiza la presentación de un caso clínico en edad pediátrica con diagnóstico de pilomatrixoma, variedad ampollar. Este tumor benigno originado en la vaina del folículo piloso muestra una incidencia elevada en las dos primeras décadas de vida. El objetivo de este artículo es presentar un caso con diagnóstico de un tumor, con características clínicas atípicas e inusuales. En ocasiones este tipo de tumor, conllevan al diagnóstico erróneo de la entidad, realizándose el diagnóstico de certeza mediante el estudio histopatológico una vez que se procede a la exéresis quirúrgica de la lesión (AU).

A clinical case with diagnosis of bullous variety pilomatrixoma in pediatric age is presented. This benign tumor originated in the sheath of the hair follicle shows a high incidence in the first 20 years of life. The aim of this work is presenting a case with tumor diagnosis, the clinical characteristics of which sometimes leads to an erroneous diagnosis of the entity in the atypical and unusual varieties. The accurate diagnosis was made through the pathologic study after the surgical excision of the lesion (AU).

Pilomatrix Carcinoma of the scalp. A case report and review of the literature.

INTRODUCTION: Pilomatrix Carcinoma (PC) is a rare and malignant dermo-hypodermic tumor. Only 11 cases were reported in patients younger than 18 years old and only 13 cases were reported on the scalp. CASE REPORT: We report the case of a 15-year-old woman who underwent cyst excision on the vertex. Anatomopathology shed light trichilemmal cyst. Five months later, she presented a first local recurrence. The tumor was removed with wide margin. Anatomopathology shed light PC. No adjuvant therapy was performed. The patient presented a second recurrence 3 months later with a parietal bone and superior sagittal sinus invasion and a lung metastasis. She underwent a craniotomy and radiochemotherapy. A third local recurrence was detected 4 months later. Three more lines of chemotherapy were performed without success. DISCUSSION: PC is a locally aggressive tumour, with a high rate of local recurrences and metastases. PC arises de novo or through malignant transformation of a pilomatrixoma. PC were observed frequently in the white male over 50 years old. The histological diagnosis is difficult to prove. Treatment consists of a wide surgical excision. Peritumoral margins are not codified. Because of most cases are on the face and neck, Mohs Micrographic Surgery seems to be a good modality to limit margins. Radiation therapy is an adjuvant treatment. Chemotherapy can be used in metastasis case. CONCLUSION: PC is a rare malignant tumor with high rate of disease relapse. Histological diagnosis is difficult and treatment is not standardized. Surgical procedure with wide margins is recommended to avoid the large recurrence when the staging shows no metastasis.

Pilomatrixoma anetodérmico / Anetodermic Pilomatricoma

El pilomatrixoma anetodérmico es una rara variante de pilomatrixoma, cuyo mecanismo etiopatogénico es desconocido. Se caracteriza por la presencia de una piel atrófica, plegada, con aspecto queloideo, que recubre un nódulo de consistencia dura. Su diagnóstico es clínico e histopatológico y el tratamiento es quirúrgico. Se reporta el caso de un paciente varón de 14 años con pilomatrixoma anetodérmico en omóplato derecho.

Anetodermic pilomatricoma is a rare variant of pilomatrixoma whose etiopathogenic mechanism is unknown. lts characterized by the presence of atrophic skin, fold, with keloidal appearance covering a hard nodule. The diagnosis is clinical and histopathological and the treatment is surgery. The case of a male patient of 14 years with anetodermic pilomatricoma in right shoulder blade is reported.

Pilomatrixoma / Pilomatrixoma

Niña de 6 años de edad que presenta una tumoración palpable en antebrazo derecho, de consistencia dura y coloración azulada, de aproximadamente diez meses de evolución, indolora. Se realiza una ecografía de partes blandas y se observa sobre formación palpable una imagen ovoidea, de 12 x 8 mm, de contornos regulares, con halo hipoecoico, de ecoestructura heterogénea con calcificaciones en su interior, con refuerzo de los ecos posteriores, ubicado en hipodermis. Diagnóstico presuntivo: pilomatrixoma. La paciente no presentaba enfermedades asociadas. Pasados seis meses no hubo cambios significativos y hasta el momento se mantiene conducta expectante.

Pilomatrixoma / Pilomatrixoma

Niña de 6 años de edad que presenta una tumoración palpable en antebrazo derecho, de consistencia dura y coloración azulada, de aproximadamente diez meses de evolución, indolora. Se realiza una ecografía de partes blandas y se observa sobre formación palpable una imagen ovoidea, de 12 x 8 mm, de contornos regulares, con halo hipoecoico, de ecoestructura heterogénea con calcificaciones en su interior, con refuerzo de los ecos posteriores, ubicado en hipodermis. Diagnóstico presuntivo: pilomatrixoma. La paciente no presentaba enfermedades asociadas. Pasados seis meses no hubo cambios significativos y hasta el momento se mantiene conducta expectante.(AU)

Pilomatricomas múltiples. Presentación de un caso / Multiple pilomatricomas. A case report

El pilomatricoma, o epitelioma calcificado de Malherbe, es un tumor cutáneo benigno de origen ectodérmico, relativamente raro, aunque es más común en la infancia, sobre todo antes de los 10 años; habitualmente es único y sólo el 3,5% de los casos son formas múltiples. Suelen ser pequeños (0,5-3 cm) y la localización más habitual es la cefálica. Se presentan como nódulos subcutáneos solitarios y asintomáticos, de consistencia pétrea. El diagnóstico de sospecha puede realizarse mediante una exploración clínica cuidadosa; sin embargo, con frecuencia el diagnóstico preoperatorio de pilomatricoma es incorrecto, fundamentalmente debido a la falta de familiaridad de los clínicos con este tumor. Este hecho y la poca frecuencia de los pilomatricomas múltiples nos motivaron a presentar este caso clínico (AU)

Pilomatricoma, or calcifying epithelioma of Malherbe, is a benign cutaneous tumour of ectodermal origin, relatively rare, although it is more common during childhood, especially in children under ten; it is normally unique and only 3.5% of the mare of multiple forms. They are usually small (0.5-3.0 cm) and the most usual location is the cephalic one. They are usually asymptomatic solitary subcutaneous nodules with a petreous consistency. The suspected diagnosis may be done with a very careful clinical examination; however preoperative diagnosis of pilomatricoma is often incorrect, mainly because the examining physician is not familiar with it. This event and the fact that multiple pilomatricomas are not very frequent are the reason for us to present this case report (AU)

Three cases of giant pilomatrixoma--considerations for diagnosis and treatment of giant skin tumours with abundant inner calcification present on the upper body.

BACKGROUND: Pilomatrixoma frequently occurs as a solitary, small tumour on the face or upper extremities of people younger than 20 years. METHODS: We report three cases of giant pilomatrixoma. In all these cases, outward appearances and imaging investigations suggested malignant tumours. Preoperative biopsies suggested that case 1 was a basal cell carcinoma, but cases 2 and 3 had no malignant features on biopsy. Two of the cases experienced rare complications - hypercalcaemia caused by parathyroid-related protein (PTHrP) production and multiple occurrences. RESULTS: All three tumours were removed with a 1-3-cm margin. The postoperative histopathologies showed pilomatrixoma in all three cases. CONCLUSIONS: Rarely, pilomatrixoma develops to a giant size with various atypical outward appearances consistent with a malignant tumour. Preoperative clinical appearances frequently lead to misdiagnosis, and preoperative examinations are unreliable. Therefore, when a giant tumour with abundant inner calcification is present in a young patient, the possibility of a pilomatrixoma should be considered.

Recurrent malignant pilomatrixoma invading the cranial cavity: Improved local control with adjuvant radiation.

We report the case of a 41-year-old mentally retarded male with recurrent pilomatrix carcinoma of the occipital region which invaded the occipital bone, left cerebellum and left temporal lobe. At his initial presentation the patient had a craniotomy and subtotal excision of the lesion with positive margins. He received no adjuvant therapy. After an early intracranial recurrence he had subtotal debulking and was referred for external beam radiotherapy. At 27 months follow-up after adjuvant external beam radiotherapy the intracranial component has not progressed and the patient remains clinically well.

Pilomatricoma: a review of six pediatric cases with nine lesions.

Pilomatricoma, although rare, is the second most frequent benign skin tumor in childhood. It is a hard, painless, slowly progressing subcutaneous tumor that is commonly located in the head-neck region, especially periorbital, and in the upper extremities. Histopathologic characteristics are distinctive. In this study, we performed a retrospective review of the records of our pediatric patients with pilomatricoma. We discuss herein the diagnosis and management of this uncommon skin tumor, and compare our experience with the previous literature. Pilomatricoma should be included in the differential diagnosis of superficial pediatric head and neck masses by any physician involved with pediatric tumors.

Pilomatricomas múltiples. Implicaciones diagnósticas y terapéuticas / Multiple pilomatricomas. Diagnostic and therapeutic implications

El pilomatricoma es el segundo tumor benigno superficial más frecuentemente extirpado en la infancia 1. Su aspecto clínico característico es casi diagnóstico. La presencia de múltiples pilomatricomas nos debe hacer descartar ciertas patologías asociadas a este tumor. Presentamos dos casos de pilomatricomatosis múltiple (AU)

Pilomatricoma is the second most commonly excised superficial mass in childhood 1. Its typical clinical appeareance is of diagnostic value. Multiple pilomatricomas prompt clinical evaluation for related syndromes. We present two cases of multiple pilomatricomas(AU)

Malignant transformation within benign adnexal skin tumours.

AIMS: To report five malignant trichogenic tumours arising in longstanding, previously benign adnexal neoplasms through malignant transformation. Malignant trichogenic adnexal tumours are extremely rare neoplasms. METHODS AND RESULTS: The patients were between 55 years and 79 years of age. Three of the tumours were located on the arms, two on the face. Three of our patients had a history of chronic lymphocytic leukaemia, one patient had a history of colonic adenocarcinoma. The duration of the tumour nodules was reported as between 20 and 40 years before sudden changes occurred. These changes included rapid growth, pain, itching, ulceration and bleeding. Histologically, all tumours were well circumscribed and encapsulated. There was a residual benign tumour component and morphological signs such as bone formation, dystrophic calcification and sclerosis suggesting long duration of the lesions. All patients except for one, who refused further clinical investigation due to her advanced age of 79 years, had an underlying systemic malignancy. CONCLUSIONS: The growth stimulus in these benign adnexal neoplasms resulting in malignant transformation may be attributed to the acquisition of additional genetic events or to immunosuppression due to an underlying neoplastic disease. Therefore, patients with systemic diseases or malignancy should be carefully examined and followed for sudden changes in pre-existing benign cutaneous tumours.

Malignant pilomatrixoma with multiple local recurrences and distant metastases: a case report and review of the literature.

Malignant pilomatrixoma is a rare malignant tumour of the hair matrix, which was first described in 1980. Only five patients with distant metastases have been reported. We report a sixth case with metastatic disease in a 52-year-old male, who also developed multiple local recurrences. We also review the previous five cases.

Pilomatrix carcinoma with multiple metastases: report of a case and review of the literature.

Pilomatrix carcinoma, the malignant counterpart of pilomatrixoma, is rare, with only 55 cases reported, and only four cases with visceral metastases described in the literature. Here we present a case report and a literature review on this rare tumour. A 74-year-old male with a pilomatrix carcinoma from the left temporal region presented in July 1996 and the tumour was excised. One month after diagnosis, metastases to both lungs and to a regional lymph node were found and histologically verified. The patient also developed metastases in the abdomen, back and thoracic spine. The latter resulted in spinal cord compression and paraplegia. Despite systemic chemotherapy with intravenous cisplatin and 5-fluorouracil and localised radiotherapy to the thoracic spine, progression and deterioration led to death within 3 months from time of diagnosis. Pilomatrix carcinomas are usually indolent. In our patient, however, the malignant disease progressed rapidly and it appeared to be resistant to both chemotherapy and irradiation.