Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To the authors' knowledge, this is the first reported case of an odontogenic carcinoma with documented skeletal muscle differentiation (rhabdomyosarcoma). The histology and clinical features of this aggressive odontogenic neoplasm are described. Within the English-language literature, only 2 cases are reported of an odontogenic tumor with muscle differentiation: a benign odontogenic tumor (ameloblastoma) with differentiation into a rhabdomyosarcoma and an odontogenic sarcoma with smooth-muscle differentiation. The general practitioner should be aware that odontogenic lesions may be malignant, even though this is extremely rare, and all tissue removed from the oral cavity should be submitted for biopsy.
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PMID:Odontogenic carcinoma differentiation into rhabdomyosarcoma: report of a rare case. 1989 15

Melanotic neuroectodermal tumor of infancy is a rare and fast-growing neoplasm. In this study, we describe the case of a 6-month-old female patient, who presented swelling in the anterior maxilla. Tomographic reconstruction showed an unilocular hypodense and expansive area associated with the upper right central primary incisor. The presumptive diagnoses were dentigerous cyst, adenomatoid odontogenic tumor, melanotic neuroectodermal tumor of infancy and rhabdomyosarcoma, and an incisional biopsy was performed. Microscopically, the lesion revealed a biphasic cell population, consisting of small, ovoid, neuroblastic-like cells and epithelioid cells containing melanin. Immunohistochemically, the melanocyte-like component was strongly and diffusely positive for HMB-45 and Melan-A, but weakly positive for S100. Based on these findings, definitive diagnosis of melanotic neuroectodermal tumor of infancy was established. Then, enucleation of the lesion was performed by careful curettage. After 2 year follow-up, no clinical or radiographical evidence of recurrence was verified. The present case highlights the importance of early diagnosis and therapeutic intervention at the appropriate time to achieve a favorable outcome for the patient.
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PMID:Clinical and immunohistochemical study of melanotic neuroectodermal tumor of infancy in the maxilla. 2976 20