Metastatic Malignant Ectomesenchymoma Initially Presenting as a Pelvic Mass: Report of a Case and Review of Literature
Pediatric soft tissue sarcomas account for approximately 10% of all pediatric malignancies. Malignant ectomesenchymoma is rare biphasic sarcomas consisting of both mesenchymal and neuroectodermal elements. Approximately 64 cases have been reported in the literature and are believed to arise from plu...
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| Format: | Article |
| Language: | English |
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Wiley
2014-01-01
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| Series: | Case Reports in Pediatrics |
| Online Access: | http://dx.doi.org/10.1155/2014/792925 |
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| author | A. Nael P. Siaghani W. W. Wu K. Nael Lisa Shane S. G. Romansky |
| author_facet | A. Nael P. Siaghani W. W. Wu K. Nael Lisa Shane S. G. Romansky |
| author_sort | A. Nael |
| collection | DOAJ |
| description | Pediatric soft tissue sarcomas account for approximately 10% of all pediatric malignancies. Malignant ectomesenchymoma is rare biphasic sarcomas consisting of both mesenchymal and neuroectodermal elements. Approximately 64 cases have been reported in the literature and are believed to arise from pluripotent embryologic migratory neural crest cells. We report a 4-year-old boy who initially presented with a pelvic mass and inguinal lymphadenopathy at 6 months of age. Inguinal lymph node biopsy revealed a distinct biphasic tumor with microscopic and immunophenotypic characteristics diagnostic for both alveolar rhabdomyosarcoma and poorly differentiated neuroblastoma. The patient received national protocol chemotherapy against rhabdomyosarcoma with good response and presented with a cerebellar mass 21 months later. The metastatic tumor revealed sheets of primitive tumor cells and diagnostic areas of rhabdomyosarcoma and neuroblastoma were identified only by immunohistochemistry. Cytogenetic analysis of metastatic tumor demonstrated complex karyotype with multiple chromosomal deletions and duplications. The patient received national protocol chemotherapy against neuroblastoma and adjuvant radiotherapy after surgical resection of the cerebellar tumor with good response. He is currently off from any treatment for 18 months with no evidence of tumor recurrence or metastasis. |
| format | Article |
| id | doaj-art-ce54e42ae0014de78b0c098b2b05bae5 |
| institution | Kabale University |
| issn | 2090-6803 2090-6811 |
| language | English |
| publishDate | 2014-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pediatrics |
| spelling | doaj-art-ce54e42ae0014de78b0c098b2b05bae52025-02-03T01:11:47ZengWileyCase Reports in Pediatrics2090-68032090-68112014-01-01201410.1155/2014/792925792925Metastatic Malignant Ectomesenchymoma Initially Presenting as a Pelvic Mass: Report of a Case and Review of LiteratureA. Nael0P. Siaghani1W. W. Wu2K. Nael3Lisa Shane4S. G. Romansky5Department of Pathology and Laboratory Medicine, University of California Irvine Medical Center, 101 The City Drive, Orange, CA 92868, USADepartment of Pathology and Laboratory Medicine, University of California Irvine Medical Center, 101 The City Drive, Orange, CA 92868, USADepartment of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USADepartment of Medical Imaging, University of Arizona Medical Center, 1501 N. Campbell Avenue, P.O. Box 245067, Tucson, AZ 85724-5067, USAUniversity of California Irvine, Long Beach Memorial Care Health System, 2801 Atlantic Avenue, Long Beach, CA 908068, USAUniversity of California Irvine, Long Beach Memorial Care Health System, 2801 Atlantic Avenue, Long Beach, CA 908068, USAPediatric soft tissue sarcomas account for approximately 10% of all pediatric malignancies. Malignant ectomesenchymoma is rare biphasic sarcomas consisting of both mesenchymal and neuroectodermal elements. Approximately 64 cases have been reported in the literature and are believed to arise from pluripotent embryologic migratory neural crest cells. We report a 4-year-old boy who initially presented with a pelvic mass and inguinal lymphadenopathy at 6 months of age. Inguinal lymph node biopsy revealed a distinct biphasic tumor with microscopic and immunophenotypic characteristics diagnostic for both alveolar rhabdomyosarcoma and poorly differentiated neuroblastoma. The patient received national protocol chemotherapy against rhabdomyosarcoma with good response and presented with a cerebellar mass 21 months later. The metastatic tumor revealed sheets of primitive tumor cells and diagnostic areas of rhabdomyosarcoma and neuroblastoma were identified only by immunohistochemistry. Cytogenetic analysis of metastatic tumor demonstrated complex karyotype with multiple chromosomal deletions and duplications. The patient received national protocol chemotherapy against neuroblastoma and adjuvant radiotherapy after surgical resection of the cerebellar tumor with good response. He is currently off from any treatment for 18 months with no evidence of tumor recurrence or metastasis.http://dx.doi.org/10.1155/2014/792925 |
| spellingShingle | A. Nael P. Siaghani W. W. Wu K. Nael Lisa Shane S. G. Romansky Metastatic Malignant Ectomesenchymoma Initially Presenting as a Pelvic Mass: Report of a Case and Review of Literature Case Reports in Pediatrics |
| title | Metastatic Malignant Ectomesenchymoma Initially Presenting as a Pelvic Mass: Report of a Case and Review of Literature |
| title_full | Metastatic Malignant Ectomesenchymoma Initially Presenting as a Pelvic Mass: Report of a Case and Review of Literature |
| title_fullStr | Metastatic Malignant Ectomesenchymoma Initially Presenting as a Pelvic Mass: Report of a Case and Review of Literature |
| title_full_unstemmed | Metastatic Malignant Ectomesenchymoma Initially Presenting as a Pelvic Mass: Report of a Case and Review of Literature |
| title_short | Metastatic Malignant Ectomesenchymoma Initially Presenting as a Pelvic Mass: Report of a Case and Review of Literature |
| title_sort | metastatic malignant ectomesenchymoma initially presenting as a pelvic mass report of a case and review of literature |
| url | http://dx.doi.org/10.1155/2014/792925 |
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