Alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation: report of 3 cases.
dc.contributor.author | Houreih, Mohammad Adib | |
dc.contributor.author | Lin, Amy Y | |
dc.contributor.author | Eyden, Brian P | |
dc.contributor.author | Menasce, Lia P | |
dc.contributor.author | Harrison, James | |
dc.contributor.author | Jones, David R | |
dc.contributor.author | Folberg, Robert | |
dc.contributor.author | Chejfec, Gregorio | |
dc.contributor.author | Banerjee, Saumitra S | |
dc.date.accessioned | 2009-06-05T10:15:34Z | |
dc.date.available | 2009-06-05T10:15:34Z | |
dc.date.issued | 2009-04 | |
dc.identifier.citation | Alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation: report of 3 cases. 2009, 17 (2):135-41 Int. J. Surg. Pathol. | en |
dc.identifier.issn | 1066-8969 | |
dc.identifier.pmid | 18611935 | |
dc.identifier.doi | 10.1177/1066896908319444 | |
dc.identifier.uri | http://hdl.handle.net/10541/69766 | |
dc.description.abstract | The aim of this study is to report the clinicopathologic characteristics of 3 cases of alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation. Specimens of 3 cases of alveolar rhabdomyosarcoma were studied using histologic, immunohistochemical, ultrastructural, and molecular genetic techniques. The patients were a 19-year-old man with metastatic alveolar rhabdomyosarcoma in a groin lymph node, a 16-year-old girl with alveolar rhabdomyosarcoma of the perineum, and a 20-year-old man with recurrent orbital alveolar rhabdomyosarcoma. Microscopically, case 1 was composed of compact sheets of medium to large tumor cells. Cases 2 and 3 were small blue round cell tumors. Cases 1 and 3 were solid throughout, whereas case 2 demonstrated alveolar and solid architecture. By immunohistochemistry, the following markers were positive: desmin (3/3), myogenin (3/3), synaptophysin (3/3), and chromogranin (2/3). Ultrastructurally, sarcomeric filaments were seen in all cases, while neuroendocrine granules were detected only in case 1. PAX:FKHR fusion transcript was identified in case 2, case 3 had a variant PAX3 transcript, and case 1 was negative. The data presented expands the known differentiation of alveolar rhabdomyosarcoma. | |
dc.language.iso | en | en |
dc.subject | Eye Cancer | en |
dc.subject | Orbital Cancer | en |
dc.subject | Genital Cancer | en |
dc.subject | Tumour Markers | en |
dc.subject.mesh | Adolescent | |
dc.subject.mesh | Cell Differentiation | |
dc.subject.mesh | Chromogranins | |
dc.subject.mesh | Desmin | |
dc.subject.mesh | Female | |
dc.subject.mesh | Genital Neoplasms, Female | |
dc.subject.mesh | Humans | |
dc.subject.mesh | Lymphoma | |
dc.subject.mesh | Male | |
dc.subject.mesh | Myogenin | |
dc.subject.mesh | Neuroendocrine Cells | |
dc.subject.mesh | Neurons | |
dc.subject.mesh | Orbital Neoplasms | |
dc.subject.mesh | Rhabdomyosarcoma, Alveolar | |
dc.subject.mesh | Synaptophysin | |
dc.subject.mesh | Tumor Markers, Biological | |
dc.subject.mesh | Young Adult | |
dc.title | Alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation: report of 3 cases. | en |
dc.type | Article | en |
dc.contributor.department | Department of Histopathology, Christie Hospital NHS Foundation Trust, Manchester, United Kingdom. | en |
dc.identifier.journal | International Journal of Surgical Pathology | en |
html.description.abstract | The aim of this study is to report the clinicopathologic characteristics of 3 cases of alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation. Specimens of 3 cases of alveolar rhabdomyosarcoma were studied using histologic, immunohistochemical, ultrastructural, and molecular genetic techniques. The patients were a 19-year-old man with metastatic alveolar rhabdomyosarcoma in a groin lymph node, a 16-year-old girl with alveolar rhabdomyosarcoma of the perineum, and a 20-year-old man with recurrent orbital alveolar rhabdomyosarcoma. Microscopically, case 1 was composed of compact sheets of medium to large tumor cells. Cases 2 and 3 were small blue round cell tumors. Cases 1 and 3 were solid throughout, whereas case 2 demonstrated alveolar and solid architecture. By immunohistochemistry, the following markers were positive: desmin (3/3), myogenin (3/3), synaptophysin (3/3), and chromogranin (2/3). Ultrastructurally, sarcomeric filaments were seen in all cases, while neuroendocrine granules were detected only in case 1. PAX:FKHR fusion transcript was identified in case 2, case 3 had a variant PAX3 transcript, and case 1 was negative. The data presented expands the known differentiation of alveolar rhabdomyosarcoma. |
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Pathology