Superficial low-grade fibromyxoid sarcoma (Evans tumor): A clinicopathologic analysis of 19 cases with a unique observation in the pediatric population

Steven D. Billings, Georgeta Giblen, Julie C. Fanburg-Smith

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122 Citations (Scopus)

Abstract

Low-grade fibromyxoid sarcoma (LGFMS), usually a deeply situated mass in adults, is uncommon in superficial soft tissue and in children. Nineteen superficial LGFMS from our files were studied for clinicopathologic features, the latter including tumor size, growth pattern, cellularity, collagen rosettes, vascularity, nuclear atypia, mitotic rate, necrosis, and immunophenotype. The patients included 12 males and 7 females who ranged in age from 2 to 70 years (mean, 29 years). There were 7 children. Tumor locations included the lower extremity (8), buttock (3), trunk (3), vulva/inguinal region (2), upper extremity (2), and unspecified subcutis (1). Clinical and histologic submitting diagnoses were mainly benign except for 3 cases, submitted as low-grade sarcoma, with only one as superficial LGFMS. The mean tumor size was 4.2 cm (range, 1.6-18 cm). Of 15 with evaluable resections, 5 had focal ink on tumor and 2 of these had known negative wider reexcisions. The tumors were relatively well circumscribed with low to moderate cellularity. The tumors alternated from myxoid zones with prominent curvilinear vasculature to collagenous fascicular zones. Collagen rosettes with peripheral round epithelioid cells and focal ischemic necrosis were present in 6 cases each. Mitotic rate was low (mean 1.6/50 HPF). Tumor cells were positive for vimentin and some were focally positive for actins, CD68, and EMA. CD34, keratins, and S-100 protein were negative. Follow-up (mean, 44 months; range, 10-84 months) on 16 patients demonstrated 14 with no evidence for disease, 2 with local recurrences at 5 and 16 months, but no metastases. Superficial LGFMS is more common than previously recognized and may affect children at a higher rate (7 of 19,37%) than that for deep LGFMS. The prognosis is good and appears to be better than that for deep LGFMS.

Original languageEnglish (US)
Pages (from-to)204-210
Number of pages7
JournalAmerican Journal of Surgical Pathology
Volume29
Issue number2
DOIs
StatePublished - Feb 1 2005

Fingerprint

Sarcoma
Observation
Pediatrics
Population
Neoplasms
Necrosis
Collagen
Epithelioid Cells
Ink
Buttocks
Vulva
S100 Proteins
Groin
Vimentin
Keratins
Upper Extremity
Actins
Lower Extremity
Neoplasm Metastasis
Recurrence

All Science Journal Classification (ASJC) codes

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

@article{e207377b5503452783672965f7baca7d,
title = "Superficial low-grade fibromyxoid sarcoma (Evans tumor): A clinicopathologic analysis of 19 cases with a unique observation in the pediatric population",
abstract = "Low-grade fibromyxoid sarcoma (LGFMS), usually a deeply situated mass in adults, is uncommon in superficial soft tissue and in children. Nineteen superficial LGFMS from our files were studied for clinicopathologic features, the latter including tumor size, growth pattern, cellularity, collagen rosettes, vascularity, nuclear atypia, mitotic rate, necrosis, and immunophenotype. The patients included 12 males and 7 females who ranged in age from 2 to 70 years (mean, 29 years). There were 7 children. Tumor locations included the lower extremity (8), buttock (3), trunk (3), vulva/inguinal region (2), upper extremity (2), and unspecified subcutis (1). Clinical and histologic submitting diagnoses were mainly benign except for 3 cases, submitted as low-grade sarcoma, with only one as superficial LGFMS. The mean tumor size was 4.2 cm (range, 1.6-18 cm). Of 15 with evaluable resections, 5 had focal ink on tumor and 2 of these had known negative wider reexcisions. The tumors were relatively well circumscribed with low to moderate cellularity. The tumors alternated from myxoid zones with prominent curvilinear vasculature to collagenous fascicular zones. Collagen rosettes with peripheral round epithelioid cells and focal ischemic necrosis were present in 6 cases each. Mitotic rate was low (mean 1.6/50 HPF). Tumor cells were positive for vimentin and some were focally positive for actins, CD68, and EMA. CD34, keratins, and S-100 protein were negative. Follow-up (mean, 44 months; range, 10-84 months) on 16 patients demonstrated 14 with no evidence for disease, 2 with local recurrences at 5 and 16 months, but no metastases. Superficial LGFMS is more common than previously recognized and may affect children at a higher rate (7 of 19,37{\%}) than that for deep LGFMS. The prognosis is good and appears to be better than that for deep LGFMS.",
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