Endometrial Stromal Sarcoma With Hyalinizing Giant Rosettes, Mimicking Low-Grade Fibromyxoid Sarcoma

Kokohaare, Eva Kolson; Strauß, D.; Jones, Robin L.; Thway, Khin

doi:10.1177/1066896918767547

Cited by 2 publications

(1 citation statement)

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“…CD117, however, is negative in LGFMS 3,11. Rare cases of primary vulvovaginal low-grade endometrial stromal sarcoma (LGESS) have been reported,32,33 and LGESS with fibromyxoid features may resemble LGFMS,34 including rare cases of LGESS with collagen rosettes 35. High-grade endometrial stromal sarcoma with BCOR alterations may assume a multilobulated fibromyxoid appearance,36 but these are primary to the uterus and show brisk mitoses and necrosis; vulvovaginal forms of BCOR -altered high-grade endometrial stromal sarcoma have not been described.…”

Section: Discussionmentioning

confidence: 99%

Low-grade Fibromyxoid Sarcoma of the Vulva and Vagina

Costigan

Cin

Fletcher

et al. 2022

American Journal of Surgical Pathology

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Low-grade fibromyxoid sarcoma (LGFMS) is a malignancy with propensity for late relapse that principally affects deep soft tissues of the extremities and trunk. Its occurrence in the lower female genital tract is rare, and thus it may not be always considered in the differential diagnosis. We describe the salient features of 7 vulvovaginal LGFMS identified in the authors’ consultation files. Clinical information was obtained from referring pathologists. Archival slides were reviewed, and immunohistochemistry and fluorescence in situ hybridization were performed in cases with available material. Median age was 40 years (range, 34 to 58 y). Primary sites included vulva (n=6) and vagina (n=1). Tumors were 1.2 to 8.7 cm (median, 5.0 cm) in size and grossly circumscribed with firm to focally gelatinous cut surfaces. Microscopically, 5/7 had infiltrative edges. All tumors showed fibrous and myxoid areas, with lobulated myxoid foci in 5/7, comprising storiform, patternless, or (less often) fascicular arrangement of spindled to stellate cells with bland, slender to ovoid nuclei. In all cases, mitoses were <1/2.4 mm2, and necrosis was absent. Capillary “arcades” were seen in 3/7. Margins were positive in 3/6. Immunohistochemistry showed positive epithelial membrane antigen in 4/6 and MUC4 in 5/6. Fluorescence in situ hybridization detected FUS rearrangement in 5/7. Both tumors without FUS rearrangement were also negative for EWSR1 rearrangement. All 5 patients with available follow-up were alive and disease-free 10 to 150 months (median, 57 mo) after diagnosis. However, a review of vulvovaginal/pelvic LGFMS previously reported shows recurrences as late as 45 years after initial diagnosis. Pathologists need to be aware that LGFMS can arise in the vulvovaginal region. Tumor lobulation, capillary arcades, and positive MUC4 are helpful features distinguishing LGFMS from other bland myxoid spindle cell neoplasms in the lower female genital tract. Molecular testing can be useful in challenging cases. Complete excision is feasible for most vulvovaginal LGFMS. Long-term surveillance is required as local and/or distant spread can occur decades after diagnosis.

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