Intramuscular Low-grade Fibromyxoid Sarcoma: A Case Report
Low-grade fibromyxoid sarcoma (LGFMS) is a rare neoplasm that commonly arises in the deep soft tissues of the lower extremities, particularly in the thigh. LGFMS occurs preferentially in young male adults. The microscopic appearance of LGFMS exhibits bland fibroblastic spindle cells with a whorled o...
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Published in | The Kaohsiung journal of medical sciences Vol. 25; no. 8; pp. 448 - 454 |
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Main Authors | , , , , , , |
Format | Journal Article |
Language | English |
Published |
China (Republic : 1949- )
Elsevier B.V
01.08.2009
Wiley |
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Abstract | Low-grade fibromyxoid sarcoma (LGFMS) is a rare neoplasm that commonly arises in the deep soft tissues of the lower extremities, particularly in the thigh. LGFMS occurs preferentially in young male adults. The microscopic appearance of LGFMS exhibits bland fibroblastic spindle cells with a whorled or linear arrangement in fibrous and myxoid areas. Although LGFMS has a deceptively benign histologic appearance, local recurrence and late metastases have frequently been reported. Diagnosis of LGFMS is still difficult because of its characteristic bland-looking histologic features that can be confused with other benign or low-grade fibromyxoid lesions. Although immunohistochemical staining can offer an overview of the differential diagnosis of myxoid tumors of soft tissue, it is sometimes limited for diagnosis of LGFMS. However, recent cytogenetic and molecular analyses have provided significant improvements in the diagnosis of LGFMS. Such analyses have demonstrated that most cases of LGFMS have a characteristic t (7,16) (q33;p11) translocation, resulting in the FUS-CREB3L2 fusion gene. We report a 29-year-old female who presented with a LGFMS located in the soleus muscle of her left lower leg. Preoperative imaging suggested the possibility of an intramuscular histiocytoma of the left soleus muscle. In conclusion, diagnosis of LGFMS can be challenging in routine practice in surgical pathology because of its bland-looking features. The immunohistochemical and ultrastructural findings were consistent with the fibroblastic properties of LGFMS. Cytogenetic and/or molecular genetic analyses can be used as ancillary diagnostic tools for LGFMS. |
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AbstractList | Low‐grade fibromyxoid sarcoma (LGFMS) is a rare neoplasm that commonly arises in the deep soft tissues of the lower extremities, particularly in the thigh. LGFMS occurs preferentially in young male adults. The microscopic appearance of LGFMS exhibits bland fibroblastic spindle cells with a whorled or linear arrangement in fibrous and myxoid areas. Although LGFMS has a deceptively benign histologic appearance, local recurrence and late metastases have frequently been reported. Diagnosis of LGFMS is still difficult because of its characteristic bland‐looking histologic features that can be confused with other benign or low‐grade fibromyxoid lesions. Although immunohistochemical staining can offer an overview of the differential diagnosis of myxoid tumors of soft tissue, it is sometimes limited for diagnosis of LGFMS. However, recent cytogenetic and molecular analyses have provided significant improvements in the diagnosis of LGFMS. Such analyses have demonstrated that most cases of LGFMS have a characteristic t (7,16) (q33;p11) translocation, resulting in the
FUS‐CREB3L2
fusion gene. We report a 29‐year‐old female who presented with a LGFMS located in the soleus muscle of her left lower leg. Preoperative imaging suggested the possibility of an intramuscular histiocytoma of the left soleus muscle. In conclusion, diagnosis of LGFMS can be challenging in routine practice in surgical pathology because of its bland‐looking features. The immunohistochemical and ultrastructural findings were consistent with the fibroblastic properties of LGFMS. Cytogenetic and/or molecular genetic analyses can be used as ancillary diagnostic tools for LGFMS. Low-grade fibromyxoid sarcoma (LGFMS) is a rare neoplasm that commonly arises in the deep soft tissues of the lower extremities, particularly in the thigh. LGFMS occurs preferentially in young male adults. The microscopic appearance of LGFMS exhibits bland fibroblastic spindle cells with a whorled or linear arrangement in fibrous and myxoid areas. Although LGFMS has a deceptively benign histologic appearance, local recurrence and late metastases have frequently been reported. Diagnosis of LGFMS is still difficult because of its characteristic bland-looking histologic features that can be confused with other benign or low-grade fibromyxoid lesions. Although immunohistochemical staining can offer an overview of the differential diagnosis of myxoid tumors of soft tissue, it is sometimes limited for diagnosis of LGFMS. However, recent cytogenetic and molecular analyses have provided significant improvements in the diagnosis of LGFMS. Such analyses have demonstrated that most cases of LGFMS have a characteristic t (7,16) (q33;p11) translocation, resulting in the FUS-CREB3L2 fusion gene. We report a 29-year-old female who presented with a LGFMS located in the soleus muscle of her left lower leg. Preoperative imaging suggested the possibility of an intramuscular histiocytoma of the left soleus muscle. In conclusion, diagnosis of LGFMS can be challenging in routine practice in surgical pathology because of its bland-looking features. The immunohistochemical and ultrastructural findings were consistent with the fibroblastic properties of LGFMS. Cytogenetic and/or molecular genetic analyses can be used as ancillary diagnostic tools for LGFMS. Low-grade fibromyxoid sarcoma (LGFMS) is a rare neoplasm that commonly arises in the deep soft tissues of the lower extremities, particularly in the thigh. LGFMS occurs preferentially in young male adults. The microscopic appearance of LGFMS exhibits bland fibroblastic spindle cells with a whorled or linear arrangement in fibrous and myxoid areas. Although LGFMS has a deceptively benign histologic appearance, local recurrence and late metastases have frequently been reported. Diagnosis of LGFMS is still difficult because of its characteristic bland-looking histologic features that can be confused with other benign or low-grade fibromyxoid lesions. Although immunohistochemical staining can offer an overview of the differential diagnosis of myxoid tumors of soft tissue, it is sometimes limited for diagnosis of LGFMS. However, recent cytogenetic and molecular analyses have provided significant improvements in the diagnosis of LGFMS. Such analyses have demonstrated that most cases of LGFMS have a characteristic t (7,16) (q33;p11) translocation, resulting in the FUS-CREB3L2 fusion gene. We report a 29-year-old female who presented with a LGFMS located in the soleus muscle of her left lower leg. Preoperative imaging suggested the possibility of an intramuscular histiocytoma of the left soleus muscle. In conclusion, diagnosis of LGFMS can be challenging in routine practice in surgical pathology because of its bland-looking features. The immunohistochemical and ultrastructural findings were consistent with the fibroblastic properties of LGFMS. Cytogenetic and/or molecular genetic analyses can be used as ancillary diagnostic tools for LGFMS. |
Author | Huang, Wan-Ting Chai, Chee-Yin Chien, Song-Hsiung Wu, Chun-Chieh Liao, Kuo-Sheng Hsieh, Tsyh-Jyi Yang, Sheau-Fang |
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Cites_doi | 10.5858/2000-124-1179-HSCTWG 10.1097/00000478-199306000-00007 10.1097/00000478-200309000-00006 10.1038/modpathol.3800272 10.1097/00000478-200010000-00004 10.1097/PAS.0b013e3180321959 10.1097/00000478-199712000-00011 10.1111/j.1365-2559.1995.tb01436.x 10.1097/01.pas.0000157938.95785.da 10.1093/ajcp/88.5.615 10.1007/s00256-004-0876-5 10.1080/019131290882259 10.1002/gcc.20037 10.1002/(SICI)1097-0142(19990425)87:2<75::AID-CNCR6>3.0.CO;2-W |
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An immunohistochemical, ultrastructural, and cytogenetic analysis publication-title: Arch Pathol Lab Med doi: 10.5858/2000-124-1179-HSCTWG contributor: fullname: Bejarano PA – ident: e_1_2_1_4_2 doi: 10.1097/00000478-199306000-00007 – ident: e_1_2_1_13_2 doi: 10.1097/00000478-200309000-00006 – ident: e_1_2_1_15_2 doi: 10.1038/modpathol.3800272 – ident: e_1_2_1_6_2 doi: 10.1097/00000478-200010000-00004 – ident: e_1_2_1_9_2 doi: 10.1097/PAS.0b013e3180321959 – ident: e_1_2_1_3_2 doi: 10.1097/00000478-199712000-00011 – ident: e_1_2_1_7_2 doi: 10.1111/j.1365-2559.1995.tb01436.x – ident: e_1_2_1_14_2 doi: 10.1097/01.pas.0000157938.95785.da – ident: e_1_2_1_2_2 doi: 10.1093/ajcp/88.5.615 – ident: e_1_2_1_5_2 doi: 10.1007/s00256-004-0876-5 – ident: e_1_2_1_10_2 doi: 10.1080/019131290882259 – ident: e_1_2_1_12_2 doi: 10.1002/gcc.20037 – ident: e_1_2_1_8_2 doi: 10.1002/(SICI)1097-0142(19990425)87:2<75::AID-CNCR6>3.0.CO;2-W |
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Snippet | Low-grade fibromyxoid sarcoma (LGFMS) is a rare neoplasm that commonly arises in the deep soft tissues of the lower extremities, particularly in the thigh.... Low‐grade fibromyxoid sarcoma (LGFMS) is a rare neoplasm that commonly arises in the deep soft tissues of the lower extremities, particularly in the thigh.... |
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StartPage | 448 |
SubjectTerms | Adult Female fibromyxoid Fibrosarcoma - diagnosis Fibrosarcoma - pathology FUS-CREB3L2 Humans Internal Medicine low-grade fibromyxoid sarcoma Soft Tissue Neoplasms - diagnosis Soft Tissue Neoplasms - pathology |
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Title | Intramuscular Low-grade Fibromyxoid Sarcoma: A Case Report |
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