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1.
Rev. medica electron ; 39(3): 584-591, may.-jun. 2017.
Article in Spanish | CUMED | ID: cum-76927

ABSTRACT

El Síndrome de Muir-Torre (SMT) descrito desde 1967, es una genodermatosis con herencia autosómica dominante y penetrancia variable, caracterizada por la presencia de neoplasias cutáneas de origen sebáceo manifestado en áreas seborreicas, como la cara y cuero cabelludo. Los adenomas son los tumores sebáceos más frecuentes. Se han descrito también hiperplasias, neoformaciones sebáceas quísticas, epiteliomas o carcinoma. Los mismos pueden presentarse de manera aislada o múltiple; en conjunto con al menos una neoplasia visceral maligna. Entre las descritas con frecuencia están las gastrointestinales. Se pueden asociar a otras neoplasias, como las renales, las de endometrio, útero y de laringe. El diagnóstico clínico está dado por la presencia y asociación de un tumor visceral primario y otro de origen sebáceo. El tratamiento de esta entidad se basa en la exéresis de los tumores y el seguimiento especializado según el sistema afectado. Se presentó el caso de un hombre de 62 años de edad, cuya afección cutánea demostró múltiples lesiones de origen sebáceo de siete años de evolución por lo que se le realizaron complementarios para identificar entidades asociadas (AU).


The Muir-Torre syndrome, described since 1967, is a genodermatosis with dominant autosomal inheritance and variable penetrance, characterized by the presence of sebaceous skin neoplasias manifested in seborrheic areas as the face and scalp. Adenomas are the most frequent sebaceous tumors. Hyperplasia, cystic sebaceous neoformations, epitheliomas or carcinomas have been also described. They could appear in an isolated or multiple way, in all with at least one malignant visceral neoplasia. The gastrointestinal ones are among those frequently described. They might be associated to other neoplasias, as the renal ones, or the ones of the endometrium, the uterus and the larynx. The clinical diagnosis is given by the presence and association of one primary visceral tumor and another of sebaceous origin. The treatment of this entity is based on the tumors resection and the specialized follow-up according to the affected system. The case of a man, aged 62 years, is presented; his cutaneous condition showed multiple sebaceous lesions of seven years of evolution. Complementary exams were carried out for identifying associated lesions (AU).


Subject(s)
Humans , Male , Female , Middle Aged , Muir-Torre Syndrome/epidemiology , Skin Neoplasms/epidemiology , Sebaceous Gland Neoplasms/congenital , Sebaceous Gland Neoplasms/epidemiology , Skin Neoplasms/complications , Skin Neoplasms/diagnosis , Skin Neoplasms/pathology , Adenoma/epidemiology , Muir-Torre Syndrome/complications , Muir-Torre Syndrome/diagnosis , Muir-Torre Syndrome/genetics , Muir-Torre Syndrome/therapy
2.
Rev. medica electron ; 39(3): 584-591, may.-jun. 2017.
Article in Spanish | LILACS, CUMED | ID: biblio-902194

ABSTRACT

El Síndrome de Muir-Torre (SMT) descrito desde 1967, es una genodermatosis con herencia autosómica dominante y penetrancia variable, caracterizada por la presencia de neoplasias cutáneas de origen sebáceo manifestado en áreas seborreicas, como la cara y cuero cabelludo. Los adenomas son los tumores sebáceos más frecuentes. Se han descrito también hiperplasias, neoformaciones sebáceas quísticas, epiteliomas o carcinoma. Los mismos pueden presentarse de manera aislada o múltiple; en conjunto con al menos una neoplasia visceral maligna. Entre las descritas con frecuencia están las gastrointestinales. Se pueden asociar a otras neoplasias, como las renales, las de endometrio, útero y de laringe. El diagnóstico clínico está dado por la presencia y asociación de un tumor visceral primario y otro de origen sebáceo. El tratamiento de esta entidad se basa en la exéresis de los tumores y el seguimiento especializado según el sistema afectado. Se presentó el caso de un hombre de 62 años de edad, cuya afección cutánea demostró múltiples lesiones de origen sebáceo de siete años de evolución por lo que se le realizaron complementarios para identificar entidades asociadas (AU).


The Muir-Torre syndrome, described since 1967, is a genodermatosis with dominant autosomal inheritance and variable penetrance, characterized by the presence of sebaceous skin neoplasias manifested in seborrheic areas as the face and scalp. Adenomas are the most frequent sebaceous tumors. Hyperplasia, cystic sebaceous neoformations, epitheliomas or carcinomas have been also described. They could appear in an isolated or multiple way, in all with at least one malignant visceral neoplasia. The gastrointestinal ones are among those frequently described. They might be associated to other neoplasias, as the renal ones, or the ones of the endometrium, the uterus and the larynx. The clinical diagnosis is given by the presence and association of one primary visceral tumor and another of sebaceous origin. The treatment of this entity is based on the tumors resection and the specialized follow-up according to the affected system. The case of a man, aged 62 years, is presented; his cutaneous condition showed multiple sebaceous lesions of seven years of evolution. Complementary exams were carried out for identifying associated lesions (AU).


Subject(s)
Humans , Male , Female , Middle Aged , Skin Neoplasms/epidemiology , Muir-Torre Syndrome/epidemiology , Sebaceous Gland Neoplasms/congenital , Sebaceous Gland Neoplasms/epidemiology , Skin Neoplasms/complications , Skin Neoplasms/diagnosis , Skin Neoplasms/pathology , Adenoma/epidemiology , Muir-Torre Syndrome/complications , Muir-Torre Syndrome/diagnosis , Muir-Torre Syndrome/genetics , Muir-Torre Syndrome/therapy
3.
J R Coll Surg Edinb ; 46(5): 303-6, 2001 Oct.
Article in English | MEDLINE | ID: mdl-11697700

ABSTRACT

Sebaceous naevi are uncommon congenital skin lesions with a well-recognised potential for neoplastic change. They should be considered premalignant lesions as malignant degeneration, most commonly basal cell carcinoma and squamous cell carcinoma, occurs with a lifetime risk of between 5% and 22%. This incidence is equal to that of actinic keratosis and exceeds that of oral leukoplakia. Such change, however, is rare before puberty. Basal cell carcinoma may develop in children with naevoid basal cell carcinoma syndrome, xeroderma pigmentosum and rarely de novo but sebaceous naevus is the only solitary lesion in childhood associated with the development of basal cell carcinoma. We present two cases of malignant transformation in a congenital sebaceous naevus occurring in childhood and review the literature and discuss the evidence upon which to base management guidelines.


Subject(s)
Carcinoma, Basal Cell/pathology , Cell Transformation, Neoplastic/pathology , Facial Neoplasms/pathology , Nevus/pathology , Sebaceous Gland Neoplasms/pathology , Biopsy, Needle , Carcinoma, Basal Cell/surgery , Child , Facial Neoplasms/congenital , Facial Neoplasms/surgery , Female , Follow-Up Studies , Humans , Nevus/congenital , Nevus/surgery , Plastic Surgery Procedures , Sebaceous Gland Neoplasms/congenital , Sebaceous Gland Neoplasms/surgery , Skin Transplantation , Treatment Outcome
6.
G Ital Dermatol Venereol ; 124(1-2): 55-8, 1989.
Article in Italian | MEDLINE | ID: mdl-2767720

ABSTRACT

The case of a 7-year-old girl, born from a monochorionic biamniotic pregnancy (with healthy male twin) is presented. The patient showed a congenital brown-yellowish, raised, rough and oval-shaped nevic neoformation of the right temporo-zygomatic region. At 28 days of age she had a right hemiclonic status epilepticus, and from the 8th month of life she presented right sided partial motor seizures. Moreover from the beginning, her development milestones were delayed. When she was 5 years old an electroencephalogram displayed a sharp asymmetrical background activity, a continuous paroxysmal activity on the right hemisphere and independent focal irritative anomalies on the left posterior region. Her computed tomographic scan demonstrated megalencephaly on the right hemisphere. A biopsy specimen of her skin lesion showed the histological characteristics of a nevus sebaceous of Jadassohn. Taken together the clinical, neuroradiological and dermatological data led to the diagnosis of linear nevus sebaceous syndrome.


Subject(s)
Cerebral Cortex/abnormalities , Diseases in Twins , Facial Neoplasms/congenital , Nevus, Pigmented/congenital , Sebaceous Gland Neoplasms/congenital , Child , Female , Humans , Intellectual Disability/etiology , Male , Syndrome , Twins, Dizygotic
7.
Laryngoscope ; 97(8 Pt 1): 909-14, 1987 Aug.
Article in English | MEDLINE | ID: mdl-3613788

ABSTRACT

The nevus sebaceus of Jadassohn (SNJ) is a congenitally-occurring, hamartomatous disorder of the skin and its adnexa of infrequent occurrence. This presentation of five cases emphasizes the smooth, waxy, yellow-brown lesion's progression into a thickened sebaceous tumor of premalignant predilection. The incidence of neoplastic degeneration of these hamartomatous nevi may be as high as 30% with the capacity of metastasis occasionally reported. Because of malignancy risks as well as cosmetic considerations, early surgical removal is recommended. Previously unreported problems of dysphagia and malnutrition secondary to pulsion diverticulum at the esophageal inlet and cleft palate, obliterative aural stenosis with associated conductive hearing loss are documented. Regardless of SNJ's occurrence as either an isolated lesion or as the fully developed syndrome, including mental retardation and epilepsy, this congenital malformation of the skin, its hair, and sebaceous glands presents rare and histologically intriguing problems for the practitioner.


Subject(s)
Hamartoma/diagnosis , Head and Neck Neoplasms/diagnosis , Nevus/diagnosis , Scalp , Sebaceous Gland Neoplasms/diagnosis , Skin Neoplasms/diagnosis , Adolescent , Child , Female , Hamartoma/congenital , Hamartoma/pathology , Hamartoma/surgery , Head and Neck Neoplasms/congenital , Head and Neck Neoplasms/pathology , Head and Neck Neoplasms/surgery , Humans , Infant , Nevus/congenital , Nevus/pathology , Nevus/surgery , Sebaceous Gland Neoplasms/congenital , Sebaceous Gland Neoplasms/pathology , Sebaceous Gland Neoplasms/surgery , Sebaceous Glands/pathology , Skin Neoplasms/congenital , Skin Neoplasms/pathology , Skin Neoplasms/surgery , Syndrome
9.
J Am Acad Dermatol ; 6(6): 1078-80, 1982 Jun.
Article in English | MEDLINE | ID: mdl-7096670

ABSTRACT

A 7-week-old male infant with a seizure disorder and an extensive sebaceous nevus of the scalp was examined. Computerized axial tomography (CAT) scan identified an intracerebral mass which proved to be a hamartomatous malofrmation fo the brain. The association of intracranial masses with sebaceous nevi should be recognized. CAT scan is useful in the evaluation of infants with extensive lesions of the scalp or midline facial area, especially in the presence of neurologic abnormalities.


Subject(s)
Brain Neoplasms/congenital , Hamartoma/congenital , Neoplasms, Multiple Primary/congenital , Nevus/congenital , Sebaceous Gland Neoplasms/congenital , Humans , Infant , Male , Scalp , Seizures/congenital
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