Alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation: report of 3 cases.

2.50
Hdl Handle:
http://hdl.handle.net/10541/69766
Title:
Alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation: report of 3 cases.
Authors:
Houreih, Mohammad Adib; Lin, Amy Y; Eyden, Brian P; Menasce, Lia P; Harrison, James; Jones, David R; Folberg, Robert; Chejfec, Gregorio; Banerjee, Saumitra S
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.
Affiliation:
Department of Histopathology, Christie Hospital NHS Foundation Trust, Manchester, United Kingdom.
Citation:
Alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation: report of 3 cases. 2009, 17 (2):135-41 Int. J. Surg. Pathol.
Journal:
International Journal of Surgical Pathology
Issue Date:
Apr-2009
URI:
http://hdl.handle.net/10541/69766
DOI:
10.1177/1066896908319444
PubMed ID:
18611935
Type:
Article
Language:
en
ISSN:
1066-8969
Appears in Collections:
All Christie Publications ; Pathology

Full metadata record

DC FieldValue Language
dc.contributor.authorHoureih, Mohammad Adib-
dc.contributor.authorLin, Amy Y-
dc.contributor.authorEyden, Brian P-
dc.contributor.authorMenasce, Lia P-
dc.contributor.authorHarrison, James-
dc.contributor.authorJones, David R-
dc.contributor.authorFolberg, Robert-
dc.contributor.authorChejfec, Gregorio-
dc.contributor.authorBanerjee, Saumitra S-
dc.date.accessioned2009-06-05T10:15:34Z-
dc.date.available2009-06-05T10:15:34Z-
dc.date.issued2009-04-
dc.identifier.citationAlveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation: report of 3 cases. 2009, 17 (2):135-41 Int. J. Surg. Pathol.en
dc.identifier.issn1066-8969-
dc.identifier.pmid18611935-
dc.identifier.doi10.1177/1066896908319444-
dc.identifier.urihttp://hdl.handle.net/10541/69766-
dc.description.abstractThe 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.en
dc.language.isoenen
dc.subjectEye Canceren
dc.subjectOrbital Canceren
dc.subjectGenital Canceren
dc.subjectTumour Markersen
dc.subject.meshAdolescent-
dc.subject.meshCell Differentiation-
dc.subject.meshChromogranins-
dc.subject.meshDesmin-
dc.subject.meshFemale-
dc.subject.meshGenital Neoplasms, Female-
dc.subject.meshHumans-
dc.subject.meshLymphoma-
dc.subject.meshMale-
dc.subject.meshMyogenin-
dc.subject.meshNeuroendocrine Cells-
dc.subject.meshNeurons-
dc.subject.meshOrbital Neoplasms-
dc.subject.meshRhabdomyosarcoma, Alveolar-
dc.subject.meshSynaptophysin-
dc.subject.meshTumor Markers, Biological-
dc.subject.meshYoung Adult-
dc.titleAlveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation: report of 3 cases.en
dc.typeArticleen
dc.contributor.departmentDepartment of Histopathology, Christie Hospital NHS Foundation Trust, Manchester, United Kingdom.en
dc.identifier.journalInternational Journal of Surgical Pathologyen

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