TY - JOUR
T1 - Superficial CD34-positive fibroblastic tumor
T2 - Report of 18 cases of a distinctive low-grade mesenchymal neoplasm of intermediate (borderline) malignancy
AU - Carter, Jodi M.
AU - Weiss, Sharon W.
AU - Linos, Konstantinos
AU - Dicaudo, David J.
AU - Folpe, Andrew L.
N1 - Copyright:
Copyright 2014 Elsevier B.V., All rights reserved.
PY - 2014/2
Y1 - 2014/2
N2 - 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=5), knee (N=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 follow-up 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.
AB - 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=5), knee (N=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 follow-up 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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U2 - 10.1038/modpathol.2013.139
DO - 10.1038/modpathol.2013.139
M3 - Article
C2 - 23887307
AN - SCOPUS:84893749156
SN - 0893-3952
VL - 27
SP - 294
EP - 302
JO - Modern Pathology
JF - Modern Pathology
IS - 2
ER -