Vulvar fetal rhabdomyoma mimicking 46XX sex differentiation disorder
| Autor: | Jesús Argente, Riñón C, de Prada I, Gabriel Á. Martos-Moreno |
|---|---|
| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
Pathology medicine.medical_specialty Endocrinology Diabetes and Metabolism Disorders of Sex Development Diagnosis Differential 03 medical and health sciences Tuberous sclerosis 0302 clinical medicine Endocrinology Fetal rhabdomyoma medicine Humans Sex organ Fetus Neurocutaneous Syndromes Sexual differentiation Vulvar Neoplasms business.industry Mesenchymal stem cell Rhabdomyoma medicine.disease Fetal Diseases 030104 developmental biology Subcutaneous nodule 030220 oncology & carcinogenesis Pediatrics Perinatology and Child Health Female business |
| Zdroj: | Journal of Pediatric Endocrinology and Metabolism. 29 |
| ISSN: | 2191-0251 0334-018X |
| DOI: | 10.1515/jpem-2015-0202 |
| Popis: | Rhabdomiomas are rare mesenchymal benign tumors of striated muscle origin. Setting aside the cardiac (most atrial) rhabdomiomas typically associated to neurocutaneous syndromes (tuberous sclerosis), extracardiac rhabdomyomas appear clinically as a subcutaneous nodule or as a submucosal polypoid lesion. Among them, three main histologic subtypes can be differentiated on the basis of the degree of tumor differentiation: 1) fetal rhabdomioma, usually diagnosed during childhood and almost exclusively located in the in the head and neck region with rare reports in other locations; 2) adult rhabdomioma; and 3) genital rhabdomioma, reported to occur in the lower genital tract of young and middle-aged women and, exceptionally, in children (5). |
| Databáze: | OpenAIRE |
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