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)

A 7-month-old boy had a giant pigmented lesion involving the trunk and thighs that exhibited many hyperpigmented hairy and verrucous nevi. One of the nevi ulcerated and on histological examination consisted of pleomorphic rhabdomyosarcoma cells that stained for muscle-specific actin (HHF-35), desmin, and myoglobin. Around the tumor, in the dermis, benign pigmented nevus cells were observed. The occurrence of malignant tumors, other than malignant melanoma, in pigmented nevi is rarely described.
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PMID:Rhabdomyosarcoma in a congenital pigmented nevus. 137 40

A variety of malignancies have been reported to arise within congenital melanocytic nevi, most commonly malignant melanoma, but rarely rhabdomyosarcoma, liposarcoma, and malignant peripheral nerve sheath tumor as well. There have been only three documented cases of rhabdomyosarcoma arising within congenital melanocytic nevi: two embryonal rhabdomyosarcomas and one mixed liposarcoma and rhabdomyosarcoma. One of these cases was also associated with neurocutaneous melanosis. We report a fourth case of rhabdomyosarcoma originating from a congenital melanocytic nevus. A 4-year-old girl presented with a large ulcerated nodule that developed within a hairy congenital nevus on her left gluteal and sacral regions. Her parents refused postoperative adjuvant therapy, and she died 13 months after surgical excision. Histologic sections showed a lesion with two distinct components. There was an expansile proliferation of pleomorphic cells within a fibromyxoid stroma. The neoplastic cells were spindled, and some had abundant eosinophilic globular cytoplasm with occasional cross-striations characteristic of rhabdomyoblasts. They strongly expressed desmin and myoglobin and were negative for S-100 protein and HMB-45. The tumor merged with an adjacent congenital melanocytic nevus characterized by a proliferation of uniform nonatypical melanocytes. The finding of both rhabdomyoblastic and melanocytic differentiation within the same lesion lends support to the hypothesis of their derivation from common pluripotential stem cells or neural crest cells.
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PMID:Rhabdomyosarcoma arising in a congenital melanocytic nevus. 1180 77

HES6, a member of the hairy-enhancer-of-split family of transcription factors, plays multiple roles in myogenesis. It is a direct target of the myogenic transcription factor MyoD and has been shown to regulate the formation of the myotome in development, myoblast cell cycle exit and the organization of the actin cytoskeleton during terminal differentiation. Here we investigate the expression and function of HES6 in rhabdomyosarcoma, a soft tissue tumor which expresses myogenic genes but fails to differentiate into muscle. We show that HES6 is expressed at high levels in the subset of alveolar rhabdomyosarcomas expressing PAX/FOXO1 fusion genes (ARMSp). Knockdown of HES6 mRNA in the ARMSp cell line RH30 reduces proliferation and cell motility. This phenotype is rescued by expression of mouse Hes6 which is insensitive to HES6 siRNA. Furthermore, expression microarray analysis indicates that the HES6 knockdown is associated with a decrease in the levels of Transgelin, (TAGLN), a regulator of the actin cytoskeleton. Knockdown of TAGLN decreases cell motility, whilst TAGLN overexpression rescues the motility defect resulting from HES6 knockdown. These findings indicate HES6 contributes to the pathogenesis of ARMSp by enhancing both proliferation and cell motility.
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PMID:HES6 enhances the motility of alveolar rhabdomyosarcoma cells. 2298 28

There is a vast spectrum of pathology that afflicts the floor of mouth in children. These span inflammatory conditions, vascular malformations, developmental anomalies, benign tumors and malignancies. While this area is readily evaluated on clinical exam, imaging is often performed to better characterize the disorder prior to management. The imaging modalities most frequently utilized are US, CT and MR. The purpose of this article is to describe the primary conditions that occur in this location in children so that radiologists may provide an appropriate differential diagnosis. These include ranula, venolymphatic malformation, dermoid, teratoma, foregut duplication cyst, hairy polyp, thyroglossal duct cyst and rhabdomyosarcoma. For each pathological condition, there will be a focus on describing its imaging manifestation. Floor of mouth anatomy, imaging approach during both prenatal and postnatal life and etiologies will be discussed. Surgical considerations and operative photographs will also be presented.
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PMID:Imaging of pediatric floor of mouth lesions. 2342 4

Objective:To analysis the clinical features, diagnosis and treatment experience of nasopharyngeal neoplasm in infants.Method:Twenty infants cases of nasopharyngeal neoplasm, from June 2002 to March 2015, admitted in our department were retrospectively studied. Nasopharyngeal neoplasm in all cases were ablated with low temperature plasma radiofrequency ablation after anesthesia downlink preoperative 70 degrees endoscope, and new biological routine pathologic examination were done.Result:The low temperature plasma radiofrequency ablation has several advantages,such as simple and convenient operating, short operating time, intraoperative blood loss and clear vision.Five cases were diagnosed as postoperative pathological cyst,4 cases were teratoma, 4 cases were hairy polyps,3 cases were hamartoma, 2 cases were hyperplasia of lymphoid tissue, 1 case was glioma, and 1 case was rhabdomyosarcoma. Postoperative follow-up for 6 months to 12 years, recurrence were found in 2 cases, including 1 death case.Conclusion:Infants nasopharyngeal neoplasm are almost benign, clinical rare congenital nasopharyngeal tumor more than the early detection of an infant. CT and MRI examination has important significance to the diagnosis. Low temperature plasma radiofrequency ablation with Seventy degrees nasal endoscope is worth extending.
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PMID:[The clinical analysis and surgical management of infant nasopharyngeal neoplasms]. 2987 Nov 77