Superficial CD34-positive fibroblastic tumor

Report of 18 cases of a distinctive low-grade mesenchymal neoplasm of intermediate (borderline) malignancy

Jodi Carter, Sharon W. Weiss, Konstantinos Linos, David J. Dicaudo, Andrew L. Folpe

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)294-302
Number of pages9
JournalModern Pathology
Volume27
Issue number2
DOIs
StatePublished - Feb 1 2014

Fingerprint

Neoplasms
Malignant Fibrous Histiocytoma
Desmin
S100 Proteins
Muscle Proteins
Keratins
Thigh
Fluorescence In Situ Hybridization
Sarcoma
Smooth Muscle
Actins
Knee
Necrosis
Referral and Consultation
Lymph Nodes
Neoplasm Metastasis
Recurrence
Growth

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Superficial CD34-positive fibroblastic tumor : Report of 18 cases of a distinctive low-grade mesenchymal neoplasm of intermediate (borderline) malignancy. / Carter, Jodi; Weiss, Sharon W.; Linos, Konstantinos; Dicaudo, David J.; Folpe, Andrew L.

In: Modern Pathology, Vol. 27, No. 2, 01.02.2014, p. 294-302.

Research output: Contribution to journalArticle

Carter, Jodi ; Weiss, Sharon W. ; Linos, Konstantinos ; Dicaudo, David J. ; Folpe, Andrew L. / Superficial CD34-positive fibroblastic tumor : Report of 18 cases of a distinctive low-grade mesenchymal neoplasm of intermediate (borderline) malignancy. In: Modern Pathology. 2014 ; Vol. 27, No. 2. pp. 294-302.
@article{c5a8d6d9733149fbb70d2ec565af9d93,
title = "Superficial CD34-positive fibroblastic tumor: Report of 18 cases of a distinctive low-grade mesenchymal neoplasm of intermediate (borderline) malignancy",
abstract = "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.",
author = "Jodi Carter and Weiss, {Sharon W.} and Konstantinos Linos and Dicaudo, {David J.} and Folpe, {Andrew L.}",
year = "2014",
month = "2",
day = "1",
doi = "10.1038/modpathol.2013.139",
language = "English (US)",
volume = "27",
pages = "294--302",
journal = "Modern Pathology",
issn = "0893-3952",
publisher = "Nature Publishing Group",
number = "2",

}

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

AU - Weiss, Sharon W.

AU - Linos, Konstantinos

AU - Dicaudo, David J.

AU - Folpe, Andrew L.

PY - 2014/2/1

Y1 - 2014/2/1

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.

UR - http://www.scopus.com/inward/record.url?scp=84893749156&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84893749156&partnerID=8YFLogxK

U2 - 10.1038/modpathol.2013.139

DO - 10.1038/modpathol.2013.139

M3 - Article

VL - 27

SP - 294

EP - 302

JO - Modern Pathology

JF - Modern Pathology

SN - 0893-3952

IS - 2

ER -