Low-Grade Fibromyxoid Sarcoma: A Report of Two Metastasizing Neoplasms Having a Deceptively Benign Appearance

Evans, Harry L.

doi:10.1093/ajcp/88.5.615

Cited by 330 publications

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“…Most fibroblastic tumors of deep soft tissue fall into well-defined clinicopathological categories, such as desmoplastic fibroblastoma (collagenous fibroma), 1 adult fibrosarcoma, 2 low-grade fibromyxoid sarcoma, 3 and sclerosing epithelioid fibrosarcoma. 4 Although these tumors may also rarely occur in superficial (suprafascial) locations, superficially located fibroblastic tumors more often represent different entities, such as myxofibrosarcoma (myxoid malignant fibrous histiocytoma), 5,6 fibrosarcoma arising in dermatofibrosarcoma protuberans, 7 myxoinflammatory fibroblastic sarcoma (inflammatory myxohyaline tumor of distal extremities), 8,9 and pleomorphic hyalinizing angiectatic tumor.…”

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confidence: 99%

Superficial CD34-positive fibroblastic tumor: report of 18 cases of a distinctive low-grade mesenchymal neoplasm of intermediate (borderline) malignancy

et al. 2014

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Fibroblastic mesenchymal tumors show a spectrum of biological behavior, from benign to fully malignant. We report our experience of two decades with a distinctive, previously undescribed low-grade fibroblastic tumor of the superficial soft tissues. Eighteen cases were identified within our consultation files, previously coded as 'low-grade sarcoma, not further classified' and 'malignant fibrous histiocytoma, low grade'. The tumors occurred in adults (median age 38 years, range 20-76 years) of either sex (10 males and 8 females), ranged in size from 1.5 to 10 cm (mean 4.1 cm), and were confined to the superficial soft tissues of the thigh (N ¼ 5), knee (N ¼ 2), and other sites. Histological features included a fascicular growth pattern of the neoplastic spindled cells with striking, often bizarre cellular pleomorphism and variably prominent nucleoli. Necrosis was seen in one case. All cases showed strong, diffuse CD34 positivity and 68% of tested cases demonstrated focal cytokeratin expression. Desmin, ERG, FLI-1, smooth muscle actin, and S100 protein were negative. TP53 overexpression was absent. Fluorescence in-situ hybridization studies for TGFBR3 and/or MGEA5 rearrangements were negative in all tested cases. Clinical followup was available in 13 patients (median duration of 24 months; range 1-104 months). Twelve of 13 patients had no disease recurrence. One patient had regional lymph node metastases, 7 years after incomplete excision of the primary tumor. All patients are currently alive and disease free. The unique clinicopathological features of superficial CD34-positive fibroblastic tumor define them as a novel subset of low-grade fibroblastic neoplasms, best considered to be of borderline malignancy.

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Superficial CD34-positive fibroblastic tumor: report of 18 cases of a distinctive low-grade mesenchymal neoplasm of intermediate (borderline) malignancy

et al. 2014

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“…LGFMS is a hypocellular neoplasm composed of bland fibroblastic cells within a collagenized stroma alternating with myxoid foci, a whorled growth pattern, delicate capillary arcades, and few mitotic figures [15]. Occasional areas have increased cellularity, atypia or necrosis.…”

Section: Discussionmentioning

confidence: 99%

Sclerosing Epithelioid Fibrosarcoma of the Oral Cavity

Elkins

Wakely

2011

Head and Neck Pathol

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Sclerosing epithelioid fibrosarcoma (SEF) rarely occurs outside the somatic soft tissue. Until recently no consistently specific genetic alteration had been associated with SEF. Molecular testing of the FUS gene rearrangement involving chromosome 16 [at one time considered specific for low-grade fibromyxoid sarcoma (LGFMS) and its variant, LGFMS with giant collagen rosettes), may be a nonrandom abnormality in some cases of SEF.We present an example of a rare FUS-positive SEF that arose in the floor of mouth of a 56 year old male. Light microscopy, exhaustive immunohistology, and FISH examination showing chromosome rearrangement using the FUS break-apart probe led to an erroneous diagnosis of LGFMS with giant collagen rosettes. An outside expert agreed with that diagnosis citing the FISH results as confirmatory. Upon review almost 2 years later after local recurrence, the classic histopathologic features of SEF were noted instead. This example suggests that at least a subset if not most examples of SEF are part of the LGFMS ''family'' of neoplasms, and reiterates the value of careful histologic examination in an age of increasingly sophisticated and presumably specific molecular results.

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“…In Evan's LGFMS series, one case displayed anaplastic dedifferentiation at 30-years follow-up [5]. The presence of a rich and delicate vascularization of LGFMS suggests a potential target for additional postsurgical therapy [6].…”

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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Low-Grade Fibromyxoid Sarcoma: A Report of Two Metastasizing Neoplasms Having a Deceptively Benign Appearance

Cited by 330 publications

References 0 publications

Superficial CD34-positive fibroblastic tumor: report of 18 cases of a distinctive low-grade mesenchymal neoplasm of intermediate (borderline) malignancy

Superficial CD34-positive fibroblastic tumor: report of 18 cases of a distinctive low-grade mesenchymal neoplasm of intermediate (borderline) malignancy

Sclerosing Epithelioid Fibrosarcoma of the Oral Cavity

Low-grade fibromyxoid sarcoma: an unusual cardiac location

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