Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1
Malignant triton tumors (MTTs) are rare and aggressive sarcomas categorized as a subgroup of malignant peripheral nerve sheath tumors (MPNSTs). MTTs arise from Schwann cells of peripheral nerves or existing neurofibromas and have elements of rhabdomyoblastic differentiation. We report the occurrence...
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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Oncological Medicine |
| Online Access: | http://dx.doi.org/10.1155/2015/405351 |
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| author | Basnet Alina Jofre A. Sebastian Capo Gerardo |
| author_facet | Basnet Alina Jofre A. Sebastian Capo Gerardo |
| author_sort | Basnet Alina |
| collection | DOAJ |
| description | Malignant triton tumors (MTTs) are rare and aggressive sarcomas categorized as a subgroup of malignant peripheral nerve sheath tumors (MPNSTs). MTTs arise from Schwann cells of peripheral nerves or existing neurofibromas and have elements of rhabdomyoblastic differentiation. We report the occurrence of MTTs in two sisters. The first patient is a 36-year-old female who presented with left sided chest wall swelling. She also had clinical features consistent with neurofibromatosis type 1 (NF-1). Debulking of the mass showed high-grade malignant peripheral nerve sheath tumor with skeletal muscle differentiation (MTT). The patient was treated with ifosfamide and adriamycin along with radiation. Four years after treatment, she still has no evidence of disease recurrence. Her sister subsequently presented to us at the age of 42 with left sided lateral chest wall pain. Imaging showed a multicompartmental retroperitoneal cystic mass with left psoas involvement. The tumor was resected and, similarly to her sister, it showed high-grade malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (MTT). The patient was started on chemotherapy and radiation as described above. |
| format | Article |
| id | doaj-art-7d7f7ed7f037428d847ffbf0eb3673e5 |
| institution | Kabale University |
| issn | 2090-6706 2090-6714 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Oncological Medicine |
| spelling | doaj-art-7d7f7ed7f037428d847ffbf0eb3673e52025-08-20T03:35:18ZengWileyCase Reports in Oncological Medicine2090-67062090-67142015-01-01201510.1155/2015/405351405351Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1Basnet Alina0Jofre A. Sebastian1Capo Gerardo2Trinitas Regional Medical Center, Seton Hall University of Health and Medical Sciences, Elizabeth, NJ 67202, USATrinitas Regional Medical Center, Seton Hall University of Health and Medical Sciences, Elizabeth, NJ 67202, USATrinitas Regional Medical Center, Seton Hall University of Health and Medical Sciences, Elizabeth, NJ 67202, USAMalignant triton tumors (MTTs) are rare and aggressive sarcomas categorized as a subgroup of malignant peripheral nerve sheath tumors (MPNSTs). MTTs arise from Schwann cells of peripheral nerves or existing neurofibromas and have elements of rhabdomyoblastic differentiation. We report the occurrence of MTTs in two sisters. The first patient is a 36-year-old female who presented with left sided chest wall swelling. She also had clinical features consistent with neurofibromatosis type 1 (NF-1). Debulking of the mass showed high-grade malignant peripheral nerve sheath tumor with skeletal muscle differentiation (MTT). The patient was treated with ifosfamide and adriamycin along with radiation. Four years after treatment, she still has no evidence of disease recurrence. Her sister subsequently presented to us at the age of 42 with left sided lateral chest wall pain. Imaging showed a multicompartmental retroperitoneal cystic mass with left psoas involvement. The tumor was resected and, similarly to her sister, it showed high-grade malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (MTT). The patient was started on chemotherapy and radiation as described above.http://dx.doi.org/10.1155/2015/405351 |
| spellingShingle | Basnet Alina Jofre A. Sebastian Capo Gerardo Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1 Case Reports in Oncological Medicine |
| title | Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1 |
| title_full | Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1 |
| title_fullStr | Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1 |
| title_full_unstemmed | Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1 |
| title_short | Malignant Triton Tumors in Sisters with Clinical Neurofibromatosis Type 1 |
| title_sort | malignant triton tumors in sisters with clinical neurofibromatosis type 1 |
| url | http://dx.doi.org/10.1155/2015/405351 |
| work_keys_str_mv | AT basnetalina malignanttritontumorsinsisterswithclinicalneurofibromatosistype1 AT jofreasebastian malignanttritontumorsinsisterswithclinicalneurofibromatosistype1 AT capogerardo malignanttritontumorsinsisterswithclinicalneurofibromatosistype1 |