Low-grade Fibromyxoid Sarcoma: Treatment Outcomes and Efficacy of Chemotherapy

Chamberlain, Florence; Engelmann, Bodil Elisabeth; Al-Muderis, Omar; Messiou, Christina; Thway, Khin; Miah, Aisha; Zaidi, Shane; Constantinidou, Anastasia; Benson, Charlotte; Gennatas, Spyridon; Jones, Robin L.

doi:10.21873/invivo.11766

Cited by 30 publications

(39 citation statements)

References 23 publications

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“…The genes CREB3L2 and CREB3L1 play a critical role in codifying transcription factors and contributing to the pathogenesis of the disease. In a study of 14 patients diagnosed with LGFMS, Cesne et al found no association between Complex 1 and the metastatic or recurrent potential of the tumor [ 11 – 13 ].…”

Section: Discussionmentioning

confidence: 99%

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Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literature

et al. 2021

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Background Low-grade fibromyxoid sarcoma (LGFMS) is a rare tumor characterized by bland histological features and aggressive clinical course. The most common anatomic locations of occurrence are the lower extremities, thorax, inguinal area, and upper limbs. Primary mediastinal sarcomas are even rarer. To the best of our knowledge, only seven cases of primary mediastinal LGFMS have been reported in the literature. Here, we report a case of primary mediastinal LGFMS. Case presentation A 26-year-old Pakistani man presented with fever and vomiting for the past 2 months. On a routine chest x-ray, a mediastinal mass was incidentally found. Computed tomography (CT) scan showed a large circumscribed lobulated soft tissue density mass lesion in an anterior mediastinum. Grossly, the resected mass measured 17.0 × 12.0 × 11.0 cm. The cut surface was gray white with a whorled-like appearance and foci of calcification and cystic changes. Histologically, a spindle cell lesion was seen with alternating myxoid and hyalinized areas. The shaped cells were arranged in bundles. Immunohistochemical staining showed positive reactivity patterns with MUC4 and focally for epithelial membrane antigen (EMA). The diagnosis was confirmed as LGFMS. The patient is free of symptoms and recurrence 22 months after the surgery. Conclusion In conclusion, we report a rare case of primary mediastinal LGFMS in a young male patient that was discovered incidentally. Our patient is on regular follow-up to look for evidence of recurrence as these tumors are prone to recurrences.

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Section: Discussionmentioning

confidence: 99%

“…Approximately 20-25% of all sarcomas show some forms of chromosomal translocations. The majority of LGFMSs have a recurrent balanced translocation between chromosomes 7 and 16, involving the long arm (q) of chromosomes 32-34 and the short arms (p) 11, [11][12][13].…”

Section: Discussionmentioning

confidence: 99%

Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literature

et al. 2021

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“…Chamberlain et al recently described their experience with non-surgical therapies to treat LGFMS. According to the authors, systemic therapy has limited efficacy in advanced LGFMS 7 . Unlu et al , reported two cases of paratesticular LGFMS treated with simple orchiectomy.…”

Section: Discussionmentioning

confidence: 99%

“…Despite its deceptively indolent clinical behavior and benign histological appearance, LGFMS has a high tendency for local recurrence and late distant metastasis 6 . The current treatment includes surgical excision with clear margins for localized disease with or without radiotherapy, while conventional systemic therapy has limited efficacy in advanced LGFMS 7 .…”

Section: Introductionmentioning

confidence: 99%

Case Report: An extremely rare occurrence of recurrent inguinal low-grade fibromyxoid sarcoma involving the scrotum

et al. 2020

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Low-grade fibromyxoid sarcoma (LGFMS) is a rare sarcoma subtype. The most common tumor locations are the deep soft tissue of extremities or trunks. We report a rare case of recurrent LGFMS in the inguinal region involving the scrotum and both testicles. A 38-year-old male patient reported a history of multiple nodular lesions in the left inguinal region accompanied by local inflammation. The patient was submitted for local resection of the lesion at our institution, with histopathological diagnosis of LGFMS. He missed his follow-up, returning with a large bulge in the left inguinal region involving the scrotum with signs of tissue necrosis and local purulent discharge. Surgical exploration was performed and the patient underwent tumor resection in the left inguinal region and the entire scrotum, with bilateral orchiectomy, with the margins enlarged to the right inguinal region and proximal surface of the penis. Local reconstruction was performed with a left fascia lata tensor muscle flap and ipsilateral thigh coverage using partial skin graft. On microscopic examination, the tumor showed spindle cells arranged in bundles, with abundant collagen and myxoid stroma with interspersed prominent vessels. The immunohistochemical study carried out showed immunoreactivity with Ki67 (<5%), immunonegativity with desmin and S100, confirming the diagnosis of LGFMS. Postoperative recovery was good and no recurrence was seen after two years. The patient is in good health, realizing multidisciplinary outpatient follow-up and performing continuous testosterone replacement. Surgical resection with negative margins for localized disease remains the standard treatment for LGFMS.

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“…However, local recurrence can occur once or more after surgery, and the tumor can be even transferred to the lung for some patients. For patients with metastasis, surgical resection of the tumor is still the most effective method [4] , while radiotherapy and chemotherapy have no obvious bene ts in reducing the recurrence, metastasis and prolonging the survival time of LGFMS patients [7] . In a case series of 36 patients with LGFMS treated with surgical resection with curative intent, 5-and 15-year local control rates were 83% and 79%, respectively [5] .…”

Section: Discussionmentioning

confidence: 99%

Low-grade fibromyxoid sarcoma in inguinal region:a case report and literature review

Wang¹,

Yuan²,

Zhang³

et al. 2020

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Background: Low-grade fibromyxoid sarcoma (LGFMS) is a rare fibrous tumor in soft tissues. Case presentation: A 25-year-old patient was admitted to our hospital for "more than two years with a mass in the left inguinal region". During the operation, rapid pathological examination and frozen section microscopy supported that the mass may be a low-grade mesenchymal tumor. Therefore, the surrounding tissues were resected correspondingly, Pathological diagnosis: low-grade fibromyxoid sarcoma in the inguinal region.Conclusions: Combined with literature analysis, the diagnosis of LGFMS mainly depends on pathological diagnosis, and surgical resection is the most effective treatment.

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Low-grade Fibromyxoid Sarcoma: Treatment Outcomes and Efficacy of Chemotherapy

Cited by 30 publications

References 23 publications

Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literature

Low-grade fibromyxoid sarcoma incidentally discovered as an asymptomatic mediastinal mass: a case report and review of the literature

Case Report: An extremely rare occurrence of recurrent inguinal low-grade fibromyxoid sarcoma involving the scrotum

Low-grade fibromyxoid sarcoma in inguinal region:a case report and literature review

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