Giant Low-Grade Fibromyxoid Sarcoma in the Neck

Park, Jong Min; Lim, Hye Rin; Kim, Jo Heon; Lee, Dong Hoon

doi:10.3342/kjorl-hns.2019.00857

Cited by 3 publications

(5 citation statements)

References 9 publications

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“…LGFMSs are aggressive tumors with recurrence, metastasis and death rates of 64%, 45% and 42%, respectively 6 . Nevertheless, treatment options for these tumors remain limited.…”

Section: Discussionmentioning

confidence: 99%

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Challenging Diagnosis of Low-grade Fibromyxoid Sarcoma of the Chest Wall: Case Report

Costa¹,

Mendes²,

Neto³

et al. 2023

Rev. Bras. Cancerol.

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Introduction: Low-grade fibromyxoid sarcoma (LGFMS) is a rare special subtype of fibrosarcoma, it is more common in the trunk and proximal limbs. First described by Evans in 1987, this tumor is also named Evans tumor. Diagnosis of LGFMS may be quite challenging, either due to the low suspicion rate of the tumor or low specificity of its morphological pattern and immunohistochemical profile, a phenomenon that is magnified when tumor location is less usual, such as in the chest wall. The present article presents a challenging case of LGFMS of the chest wall. Case report: Female, 58-year-old patient was referred to the oncology referral clinic with a breast tumor. Diagnostic investigation included imaging tests (breast US and chest CT scan) and core needle biopsy. CT scan revealed the epicenter of the tumor in the left anterior thoracic wall. Biopsy to evaluate the histologic type of tumor was carried out and the result was inconclusive. Therefore, surgical excision of the tumor was performed. Histopathology and immunohistochemistry studies of the surgical specimen confirmed the diagnosis of LGFMS of the chest wall. Currently, after 36 months of the surgical excision, the patient is still doing well and continues under clinical follow-up. Conclusion: Although the diagnosis of LGFMS of the chest wall is challenging, it should be done correctly, since these cases require a long and thorough clinical follow-up.

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“…LGFMSs are aggressive tumors with recurrence, metastasis and death rates of 64%, 45% and 42%, respectively 6 . Nevertheless, treatment options for these tumors remain limited.…”

Section: Discussionmentioning

confidence: 99%

“…Some individuals do not respond to the currently available chemotherapy and radiotherapy regimens. Surgical resection and long-term periodical clinical follow-up are still the only treatment modalities available 6,7 .…”

Section: Discussionmentioning

confidence: 99%

Challenging Diagnosis of Low-grade Fibromyxoid Sarcoma of the Chest Wall: Case Report

Costa¹,

Mendes²,

Neto³

et al. 2023

Rev. Bras. Cancerol.

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“…LGFMS is a benign tumor rarely seen in the head and neck region. Despite acting like a benign tumor with a slow course, it can exhibit malignant behaviors such as distant metastasis and local recurrence [8]. In the head and neck region, the maxillary sinus localization of LGFMS is even rarer, and our literature review revealed only five reported cases in total [1,[9][10][11].…”

Section: Discussionmentioning

confidence: 99%

“…On the other hand, recurrence after surgery is not the only problem caused by the genetic and histological features of LGFMS. Another factor that makes treatment difficult is the low mitosis rate, which is one of the most characteristic features of the tumor [8,18]. The low mitotic rate and fibrotic background also limit the effectiveness of radiotherapy and chemotherapy, which are highly effective in malignant tumors [19,20].…”

Section: Discussionmentioning

confidence: 99%

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Low-Grade Fibromyxoid Sarcomas with the Maxillary Sinus Localization: A Case Report and Review of the Literature

Doblan

2021

Indian J Otolaryngol Head Neck Surg

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A low-grade fibromyxoid sarcoma (LGFMS) is an extremely rare tumor first described by Evans in 1987.LGFMS is a type of soft tissue sarcoma characterized by a deceptively benign histological appearance but completely malignant behavior. LGFMS is usually seen in the deep soft tissues of the extremities and trunk. We have examined many reviews, case reports and case series previously published in PubMed and Google Scholar. To date, only five cases have been reported in the maxilla. LGFMS generally affects young adults, but it can also be seen in children and older adults. A radical surgical approach is the most recommended treatment option. LGFMS has a very low mitotic activity; therefore, it is considered that neither chemotherapy nor radiotherapy has a significant effect on long-term LGFMS prognosis. However, to date, there has been no study suggesting any protocol for the follow-up of patients with LGFMS. In this report, we present a case with LGFMS located in the maxillary sinus, in which despite radiotherapy following extensive surgical excision, the tumor recurred in a short period of three months and reached its former size.

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Head and Neck Low-Grade Fibromyxoid Sarcoma: A Clinicopathologic Study of 15 Cases

Gjeorgjievski

Fritchie

Thangaiah

et al. 2021

Head and Neck Pathol

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Giant Low-Grade Fibromyxoid Sarcoma in the Neck

Cited by 3 publications

References 9 publications

Challenging Diagnosis of Low-grade Fibromyxoid Sarcoma of the Chest Wall: Case Report

Challenging Diagnosis of Low-grade Fibromyxoid Sarcoma of the Chest Wall: Case Report

Low-Grade Fibromyxoid Sarcomas with the Maxillary Sinus Localization: A Case Report and Review of the Literature

Head and Neck Low-Grade Fibromyxoid Sarcoma: A Clinicopathologic Study of 15 Cases

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