dysembryoplasia

Smooth muscle hamartoma is an uncommon cutaneous dysembryoplasia usually diagnosed in infancy. Among the 61 cases published since 1923, 56 were congenital and 3 appeared in young adults. We report a case in which the lesions started at the age of 15 years as a papular plaque in the right mammary region of a young woman. A review of the literature showed that the usual clinical presentation is a frequently pigmented plaque made of often follicular papules and measuring 1 to 10 centimeters on average. Excessive hairiness is the most frequent sign, being observed in more than two-thirds of the cases, and Darier's pseudo-sign is present in about 53 p...

Cysts and canals found on the ventral surface of the penis have been ascribed to minor dysembryoplasia of the male genitalia. These lesions occur almost exclusively on the ventral surface of the penis along the median raphe and can extend from the urethral meatus to the anus. They are lined by stratified urothelium or squamous epithelium and they do not communicate with the urethra. Most cases are noted in early adulthood, they are usually asymptomatic but may become infected. Surgical removal is the adapted treatment...

Two cases of juvenile elastoma (nevus elasticus in disseminated tumors) were histologically and ultrastructurally investigated. The predominant components of this disseminated dysembryoplasia are abnormal elastic fibers. Both cases showed numerous large elastic fibers with an abundant background matrix. The elastic microfibrils were replaced by granular material. In one case, most of the abnormal elastic units remained isolated without forming elastic fibers.

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The authors report the case of a 40-year-old man who has presented for many years an intermittent progressive spastic paraplegia. Plain films of the spine show very important dysraphic abnormalities of the inferior dorsal column and the myelography shows a complete block suggesting an intramedullary space-occupying lesion at four levels above the vertebral abnormalities. The operation permits a total removal of an intramedullary "bronchogenic cyst". In the post-operative course, the neurologic deficit improves, six months later a spastic paraparesia remains...

Cystic hygroma is a benign dysembryoplasia of the lymphatic system of mainly cervical expression. Clinical findings of a stereotyped nature were found in 4 cases of this particular aspect of infantile surgical affections. On operation there was an obvious relation with the internal jugular vein, and an entire segment had to be removed in 2 cases. These data confirm conventional pathogenic theories.

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Occurrence of basal cell epithelioma and syringocystadenoma papilliferum on sebaceous nevi is well known. But many other adnexal tumors, such as pilar or sweat gland tumours may also be associated with this dysembryoplasia. Out of 99 cases of verruco-sebaceous nevi we find 12 associated basal cell epitheliomas, 7 syringocystadenomas papilliferum and 6 benign adnexal tumors. Our report is about these 7 cases. All of these tumors appeared in adult-hood and were clinically suggesting the diagnostic of basal cell epithelioma...

Anatomico-radiological study of 8 cases of intraosseous epidermoid cysts (1 in the skull, 6 in distal phalanges of the hand, 1 in distal phalanx of the foot with invasion of the adjacent joint). The epithelium was malpighian with hyperkeratosis (therefore the scales filled the cavity), sometimes with parakeratosis and even dyskeratosis but without signs of malignancy. The adjacent connective tissue and bone reactions were more active in the phalanx than in the skull and this could explain the frequent pain in the phalanges...

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Among the various congenital cervicofacial swellings observed in children, three types are rarely encountered in general practice: auriculobranchial cysts and fistulae, thymic cysts and cystic lymphangiomas. To resolve diagnostic and therapeutic problems related to these rare lesions it is essential that their etiopathogenic bases be understood. Auriculobranchial cysts and fistulae present clinical symptoms (cervical, parotid, auricular) that are only poorly evidenced and are related mainly to infection. Surgical excision requires a wide approach route of the parotidectomy type with preliminary isolation of the facial nerve...

A case of lipoma in the cerebellopontine angle is reported. Intracranial lipomas are very rare, especially in the cerebellopontine angle. To our knowledge, only four cases, including our own, have been operated upon. Until now, total extirpation has not been possible. The singular appearance on computed tomography scanning of this dysembryoplasia is presented together with a bibliography of this subject.

Of 41 children, 22 girls and 19 boys, with cervicofacial lymphangioma, 31 had congenital lymphangiomas, 30 lymphangiomas with cervical expression but only 9 were restricted to the neck region. Operation in 35 children showed that in 12 cases the lymphangioma was limited in extent and failed to extend to the neck or parotidojugular region, and excision was complete and there were no recurrences. Partial excision was performed in 23 children in whom the lymphangioma extended to various degrees into the tongue, pharynx or larynx, and tracheotomy was necessary in 10 of these cases...