Rare localization of low-grade fibromyxoid sarcoma to the nail region

Cabibi, Daniela; Mustacchio, Vincenzo; Rodolico, Vito; Barresi, E; Martorana, Annamaria; Amato, Steven

doi:10.1111/j.1365-2133.2005.06829.x

Cited by 11 publications

(17 citation statements)

References 11 publications

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“…1). We confirmed that our reported tumor exhibited an immunohistochemical profile positive for CD99 and negative for CD34 and EMA, which was compatible with those of LGFMS described by Dr Cabibi 2 …”

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confidence: 92%

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Kusumi¹,

Nishikawa

Tanaka³

et al. 2007

Pathology International

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supporting

confidence: 92%

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Kusumi¹,

Nishikawa

Tanaka³

et al. 2007

Pathology International

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“…Therefore we would like to add that, beyond the six cases in the hand and foot reported by Folpe et al 2 . and the two cases in the foot reported by Mertens et al ., 3 we recently described two cases localized in the nail region of the fingers 4 . We found that the immunohistochemical profile (positivity for vimentin, and negativity for neuron‐specific enolase, epithelial membrane antigen, cytokeratin, desmin, α‐smooth muscle actin, CD34, CD68, and S‐100 protein) is in keeping with the immunohistochemical findings reported by the authors.…”

supporting

confidence: 90%

“…Therefore we would like to add that, beyond the six cases in the hand and foot reported by Folpe et al 2 and the two cases in the foot reported by Mertens et al, 3 we recently described two cases localized in the nail region of the fingers. 4 We found that the immunohistochemical profile (positivity for vimentin, and negativity for neuron-specific enolase, epithelial membrane antigen, cytokeratin, desmin, a-smooth muscle actin, CD34, CD68, and S-100 protein) is in keeping with the immunohistochemical findings reported by the authors. We would underline that the more important differential diagnosis of acral low-grade fibromyxoid sarcoma (LGFMS) could be 'superficial acral fibromyxoma' described by Fetsch et al, 5 which produced positive immunostaining for CD99; Fetsch et al concluded that the findings were compatible with a specialized fibroblastic cell type due to the positivity of CD34 and CD99 in subpopulations of fibroblasts.Our patients also had CD99-positive immunostaining but they were negative for CD34.…”

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confidence: 89%

Low‐grade fibromyxoid sarcoma arising in the big toe

Cabibi¹

2007

Pathology International

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“…Low-grade fibromyxoid sarcoma is a tumor of deep soft tissue that rarely occurs superficially or on fingers and toes. 5,7,12,18 In contrast to digital fibromyxoma, low-grade fibromyxoid sarcoma is rarely and focally positive for CD34. 19 Both tumors can occasionally stain with SMA, and although EMA stains 43% to 91% of low-grade fibromyxoid sarcoma, such staining is often limited.…”

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confidence: 94%

Digital Fibromyxoma (Superficial Acral Fibromyxoma)

Hollmann

Bovée

Fletcher

2012

American Journal of Surgical Pathology

132

227

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Digital fibromyxoma (first described by Fetsch and colleagues as superficial acral fibromyxoma) is a distinctive soft tissue tumor with a predilection for the subungual or periungual region of the hands and feet. This report details the histologic, immunophenotypic, and clinical findings in 124 cases of digital fibromyxoma. The study group included 70 male and 54 female patients (1.3:1, M:F), ranging in age from 4 to 86 years (mean, 48 y; median, 49 y). Mean tumor size was 1.7 cm (range, 0.5 to 5 cm; median, 1.5 cm). Nearly half of the patients (41%) presented with a painful mass. Tumors arose on the hands (52%) or feet (45%), with rare tumors arising on the ankle or leg. Most tumors occurred on the digits (94% of hand tumors, 82% of foot tumors), with the majority growing in close proximity to the nail (97% on fingers, 96% on toes). Histologically, 80% of cases were poorly marginated; 70% infiltrated the dermal collagen, 27% infiltrated fat, and 3% invaded bone. In cases in which imaging studies were available, bone involvement by an erosive or lytic lesion was more frequent (9/25, 36%). All tumors were composed of spindle-shaped or stellate-shaped cells with palely eosinophilic cytoplasm and a random or loosely fascicular growth pattern. The tumor cells were separated by dense hyaline collagen alternating with myxoid stroma. Most (86%) of the tumors showed alternating areas of fibrous and myxoid stroma, 11% showed predominantly fibrous stroma, and 3% had predominantly myxoid stroma. Increased mast cells were noted in 88% of tumors. All tumors comprised cells with minimal atypia, occasionally showing scattered larger cells with so-called "degenerative change." Mitotic figures were infrequent, and all tumors lacked necrosis, pleomorphism, or neural/perineural infiltration. Multinucleate stromal cells were occasionally seen. Tumor cells were reactive for CD34 in 42/61 cases (69%), with rare tumors showing focal reactivity for EMA (3/40, 7.5%), smooth muscle actin (5/42, 12%), and desmin (1/18, 6%). All tumors were negative for S100 (0/66), MUC4 (0/11), GFAP (0/10), AE1/AE3 (0/4), Cam5.2 (0/2), PanK (0/2), Claudin (0/4), and NFP (0/3). Follow-up in 47 cases ranged from 1 to 252 months (mean, 35 mo). Ten tumors (24%) recurred locally (all near the nail unit of the fingers or toes) after a mean interval of 27 months. One tumor recurred twice. All recurrent tumors had positive margins on initial biopsy or subsequent excision and no other clinical or pathologic features correlated with recurrence/persistence. To date, no tumor has metastasized. Finally, sequencing of 8 digital fibromyxomas failed to reveal mutations in exon 8 or 9 of GNAS1, in contrast to intramuscular or cellular myxoma.

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Rare localization of low-grade fibromyxoid sarcoma to the nail region

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Cited by 11 publications

References 11 publications

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Low‐grade fibromyxoid sarcoma arising in the big toe

Digital Fibromyxoma (Superficial Acral Fibromyxoma)

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