Primary intrathoracic low-grade fibromyxoid sarcoma

Jakowski, Joseph D.; Wakely, Paul E.

doi:10.1016/j.humpath.2007.08.017

Cited by 35 publications

(27 citation statements)

References 29 publications

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“…This includes the tumour originally designated as hyalinizing spindle cell tumour with giant rosettes [135]. Low-grade fibromyxoid sarcoma presents as a well-defined mass in deeper soft tissues of extremities (especially the thigh), trunk and rarely other locations, including viscera, in young adults of either sex [136][137][138][139][140][141][142]. Examples located superficially have a higher incidence in childhood [143].…”

Section: Low-grade Fibromyxoid Sarcomamentioning

confidence: 99%

Soft tissue sarcomas with non-EWS translocations: molecular genetic features and pathologic and clinical correlations

Fisher

2009

Virchows Arch

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Many soft tissue sarcoma subtypes have consistent chromosomal translocations with novel fusion genes, which result in disordered cellular function. The microscopic appearances, immunophenotype and behaviour of such tumours relate to the genetic events to a variable extent. This paper reviews the molecular pathology and related morphological and clinical features of sarcomas with non-EWS translocations. These include synovial sarcoma, alveolar rhabdomyosarcoma, alveolar soft part sarcoma, dermatofibrosarcoma protuberans, low-grade fibromyxoid sarcoma, infantile fibrosarcoma and inflammatory myofibroblastic tumour.

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Section: Low-grade Fibromyxoid Sarcomamentioning

confidence: 99%

Soft tissue sarcomas with non-EWS translocations: molecular genetic features and pathologic and clinical correlations

Fisher

2009

Virchows Arch

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“…The treatment involves the excision of the primary lesion, recurrent lesions, or metastases. [3,4,6] Close, long-term follow-up is essential. [3] …”

Section: Discussionmentioning

confidence: 99%

“…[2] However, only two cases with mediastinal location have been reported to date. [3,4] This report presents a third case of LGFMS in the mediastinum.…”

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confidence: 91%

Low-grade fibromyxoid sarcoma in the mediastinum: a case report

Gülhan¹

2012

Turk Gogus Kalp Dama

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Düşük dereceli fibromiksoid sarkom, nadir görülen bir yumuşak doku tümörüdür. Yirmi beş yaşında kadın hasta sırt ağrısı ve nefes darlığı yakınmaları ile kliniğimize başvurdu. Toraks bilgisayarlı tomografide posterior mediastinumda, en geniş boyutları yaklaşık 17x13x11 cm çaplı kitle lezyonu saptandı. Sağ torakotomi ile total kitle eksizyonu yapıldı. Histopatolojik incelemede düşük dereceli fibromiksoid sarkom saptandı. Mediastinum, her ne kadar düşük dereceli fibromiksoid sarkomun nadir yerleşim yeri olsa da bu gibi kitle varlığında akla getirilmelidir.Anah tar söz cük ler: Mediastinal tümör; mediastinum; sarkom.Low-grade fibromyxoid sarcoma is a rare type of soft tissue tumor. A 25-year-old female patient was admitted to our clinic with the complaints of back pain and dyspnea. Thorax computed tomography revealed a mass lesion of 17x13x11 cm in its largest diameter in the posterior mediastinum. Total mass excision was performed through right thoracotomy. Histopathological examination revealed low-grade fibromyxoid sarcoma. Although low-grade fibromyxoid sarcoma rarely involves mediastinum, it should be considered in the presence of such mass.

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“…According to our knowledge, this is the first described case of primary LGFMS of the heart. In 2008, Jakowski and Wakely [3] described a case of intrathoracic LGFMS as a mass attached to the external epicardium, with no myocardial involvement. LGFMS should be considered in the differential diagnosis with other primary myxoid cardiac tumors, including myxoma, low-grade myxoid fibrosarcoma or myxofibrosarcoma, and myxoid liposarcoma [1].…”

Section: Commentsmentioning

confidence: 99%

Low-grade fibromyxoid sarcoma: an unusual cardiac location

Ferlosio

Doldo

Polisca

et al. 2013

Cardiovascular Pathology

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We report the unusual cardiac localization of a primary low-grade fibromyxoid sarcoma of the right ventricle in a 57-year-old woman. Histological examination revealed a prevalent myxoid appearance with whorling growth pattern of small or spindle cells with bland features alternating with rare more collagenous hypocellular areas with rare atypical cells. Genomic polymerase chain reaction of genomic DNA revealed the typical FUS/Creb3L2 fusion gene products typical of low-grade fibromyxoid sarcoma. The tumor was surgically removed and recurred after 7 years as high-grade pleomorphic sarcoma. The patient died 6 months after the clinical manifestation of recurrence. Low-grade fibromyxoid sarcoma of soft tissues is a rare, distinctive variant of fibrosarcoma-typically arising in deep soft tissue of lower extremities and trunk-that rarely metastasizes. Clinically, low-grade fibromyxoid sarcoma is characterized by a longer survival rate compared to other sarcomas, suggesting its consideration in the differential diagnosis of cardiac tumors with a myxoid appearance.

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Primary intrathoracic low-grade fibromyxoid sarcoma

Cited by 35 publications

References 29 publications

Soft tissue sarcomas with non-EWS translocations: molecular genetic features and pathologic and clinical correlations

Soft tissue sarcomas with non-EWS translocations: molecular genetic features and pathologic and clinical correlations

Low-grade fibromyxoid sarcoma in the mediastinum: a case report

Low-grade fibromyxoid sarcoma: an unusual cardiac location

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