Low-Grade Fibromyxoid Sarcoma: Clinical Study and Case Report

Tang, Zhen; Zhou, Zhen; Lv, Chun-tang; Qin, Li-Yin; Wang, Yue; Tian, Gang; Luo, Xiaolong; Zhu, Qiang; Xu, Xiaogang

doi:10.1016/j.joms.2009.04.136

Cited by 33 publications

(26 citation statements)

References 67 publications

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“…LGFMS occurs most commonly in the deep soft tissues of & Morgan L. Cowan mcowan6@jhmi.edu the proximal extremities and trunk, with only isolated head and neck cases reported [7,[9][10][11][12][13][14]. Most LGFMSs harbor a characteristic gene fusion of FUS/CREB3L2, and recently MUC4 immunostaining has been found to be highly sensitive and specific for the diagnosis [15,16].…”

Section: Introductionmentioning

confidence: 99%

Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature

Cowan

Thompson

Leon

et al. 2015

Head and Neck Pathol

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Low-grade fibromyxoid sarcoma (LGFMS) is a deceptively bland malignancy with potential for late recurrence and metastasis, which usually occurs in the deep soft tissues of the extremities and trunk. Most LGFMSs harbor a characteristic gene fusion of FUS-CREB3L2, and recently MUC4 immunostaining has been found to be highly sensitive and specific for the diagnosis. We present a dedicated series of head and neck LGFMS, including the first reported laryngeal case, as well as a review of reported head and neck cases. The surgical pathology archives of our three institutions were searched for cases of LGFMS arising within the head and neck, and four cases were identified. The H&E slides were reviewed, and immunohistochemistry were performed for pancytokeratin, p63, p40, EMA, S100 protein, β-catenin, actin, CD34, and MUC4. The patients were 6, 43, 45, and 73 years old (mean 41.8 years) and included three males and one female. The tumors were located in the posterior cervical spine, facial skin, mandible, and larynx. The tumors were treated with surgical excision, and all four had histologic features typical for LGFMS including alternating myxoid and fibrous areas with prominent curvilinear vasculature. All tumors were MUC4 positive (100%), 2/4 (50%) were p63 positive, 1/4 (25%) showed focal EMA positivity; all 4 were negative for pancytokeratin, p40, S100 protein, β-catenin, actin, and CD34. LGFMS is a low grade sarcoma that rarely develops in the head and neck. Due to its rarity, a pathologist may not consider LGFMS in the differential diagnosis of spindle cell neoplasms within the head and neck. Immunohistochemical staining is helpful, but stains should be selected carefully to avoid misdiagnosis.

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Section: Introductionmentioning

confidence: 99%

Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature

Cowan

Thompson

Leon

et al. 2015

Head and Neck Pathol

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“…The median age of onset for LGFMS is 34 years [3], although patients of any age can be affected, and 13-19% of cases occur in patients 18 years and younger [3,4]. However, LGFMS is rare in children during the first 5 years of life [2].…”

Section: Introductionmentioning

confidence: 99%

Deeply located low-grade fibromyxoid sarcoma with FUS-CREB3L2 gene fusion in a 5-year-old boy with review of literature

et al. 2014

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BackgroundLow-grade fibromyxoid sarcoma (LGFMS) is a rare soft tissue tumor typically affecting young to middle-aged adults. Despite its otherwise benign histologic appearance and indolent nature, it can have fully malignant behavior, and recurrence and metastasis may occur even decades later.Case historyWe report a case of LGFMS in the left lower leg of a 5-year-old Japanese boy. A magnetic resonance imaging (MRI) uncovered a well-demarcated intra-gastrocnemial tumor measuring 27 × 20 mm with a slightly high intensity on T1WI and heterogeneously high intensity on T2WI. Histologically, the tumor was composed of bland spindle-shaped cells with a whorled growth pattern. The tumor stroma was variably hyalinized and fibromyxoid with arcades of curvilinear capillaries and arterioles with associated perivascular fibrosis. Although LGFMS is known to affect children under 18 years of age, it is extremely rare in infants and children under 5 years of age. Despite the young age, this patient was accurately diagnosed by the typical histology and the detection of a FUS-CREB3L2 gene fusion.ConclusionAlthough LGFMS in children tends to be located superficially, this case presented with an intramuscular tumor in the region of the gastrocnemius. To the best of our knowledge, this is the first case of deep LGFMS arising in a child younger than 5 years of age. The patient is still alive with no evidence of the disease 4 months after diagnosis.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_163

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“…Surgical resection remains the standard treatment for low-grade fibromyxoid sarcoma, with wide surgical resection being the most effective strategy [10] [14].…”

Section: Discussionmentioning

confidence: 99%

“…Chemotherapy is reserved for patients whose tumors recur locally or spread to distant sites [14] [15]. No adjuvant treatments were administered to our patient as they would have been unconventional for low-grade fibromyxoid sarcoma.…”

Section: Discussionmentioning

confidence: 99%

Low-Grade Fibromyxoid Sarcoma in the Left Gluteal Region Presenting as Sciatic Nerve Pain

Hashimoto¹,

Toriumi²,

Hara³

et al. 2018

OJO

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Low-grade fibromyxoid sarcoma is a slowly growing soft tissue neoplasm, mostly affecting young individuals. It usually arises in a deep soft tissue of the lower limbs and trunk, but few cases of low-grade fibromyxoid sarcoma that presents sciatic nerve pain have been reported. We report a 34-year-old woman with a low-grade fibromyxoid sarcoma originating in the left gluteal region that initially presented as sciatic nerve pain; she had experienced this type of pain for 5 years before visiting our hospital. Magnetic resonance imaging revealed a tumor in the left gluteal region. After needle biopsy revealed it to be a low-grade fibromyxoid sarcoma, we performed the wide resection. Intra-operative findings revealed no tumor invasion into the sciatic nerve. No recurrence or metastasis has been detected 6 months post-surgery. Oncologists who encounter patients with sciatic nerve pain should consider the possibility of less common causes such as the low-grade fibromyxoid sarcoma found in our patient.

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Low-Grade Fibromyxoid Sarcoma: Clinical Study and Case Report

Cited by 33 publications

References 67 publications

Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature

Low-Grade Fibromyxoid Sarcoma of the Head and Neck: A Clinicopathologic Series and Review of the Literature

Deeply located low-grade fibromyxoid sarcoma with FUS-CREB3L2 gene fusion in a 5-year-old boy with review of literature

Low-Grade Fibromyxoid Sarcoma in the Left Gluteal Region Presenting as Sciatic Nerve Pain

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