TY - JOUR
T1 - Solitary fibrous tumor with neuroendocrine and squamous dedifferentiation
T2 - a potential diagnostic pitfall
AU - Lu, Chuanyong
AU - Alex, Deepu
AU - Benayed, Ryma
AU - Rosenblum, Marc
AU - Hameed, Meera
N1 - Publisher Copyright:
© 2018 Elsevier Inc.
PY - 2018/7
Y1 - 2018/7
N2 - Solitary fibrous tumor (SFT) is a ubiquitous mesenchymal neoplasm of deep soft tissue with variable and often unpredictable biological behavior. The lineage is presumed to be fibroblastic, and histological features range from benign to overtly malignant with rare tumors showing “dedifferentiation” or transformation to undifferentiated pleomorphic sarcoma. Dedifferentiation in mesenchymal neoplasms is a phenomenon of histologic progression of a well-differentiated neoplasm to a high-grade sarcoma, which can differentiate along divergent lines. It is extremely uncommon to encounter “transdifferentiation” to non-mesenchymal lineage and still maintaining the driver genetic event of the primary tumor. Herein, we report two diagnostically challenging SFTs with transformation to neuroendocrine and squamous phenotypes. The index case is a pelvic malignant SFT, which metastasized to the liver as a high-grade neuroendocrine carcinoma. The second case is a recurrent brain tumor initially presenting as a typical SFT and evolving into a dedifferentiated SFT with foci of squamous differentiation. Positive immunohistochemical stains for CD34 and STAT6 and the detection of NAB2-STAT6 fusion supported the diagnosis of dedifferentiated SFT in both cases. In the first case, molecular study also demonstrated that both the pelvic primary and liver metastasis harbored the same NAB2-STAT6 fusion. Dedifferentiation to a non-mesenchymal lineage/lineage infidelity can be a potential diagnostic pitfall in these tumors.
AB - Solitary fibrous tumor (SFT) is a ubiquitous mesenchymal neoplasm of deep soft tissue with variable and often unpredictable biological behavior. The lineage is presumed to be fibroblastic, and histological features range from benign to overtly malignant with rare tumors showing “dedifferentiation” or transformation to undifferentiated pleomorphic sarcoma. Dedifferentiation in mesenchymal neoplasms is a phenomenon of histologic progression of a well-differentiated neoplasm to a high-grade sarcoma, which can differentiate along divergent lines. It is extremely uncommon to encounter “transdifferentiation” to non-mesenchymal lineage and still maintaining the driver genetic event of the primary tumor. Herein, we report two diagnostically challenging SFTs with transformation to neuroendocrine and squamous phenotypes. The index case is a pelvic malignant SFT, which metastasized to the liver as a high-grade neuroendocrine carcinoma. The second case is a recurrent brain tumor initially presenting as a typical SFT and evolving into a dedifferentiated SFT with foci of squamous differentiation. Positive immunohistochemical stains for CD34 and STAT6 and the detection of NAB2-STAT6 fusion supported the diagnosis of dedifferentiated SFT in both cases. In the first case, molecular study also demonstrated that both the pelvic primary and liver metastasis harbored the same NAB2-STAT6 fusion. Dedifferentiation to a non-mesenchymal lineage/lineage infidelity can be a potential diagnostic pitfall in these tumors.
KW - Dedifferentiation
KW - Malignant transformation
KW - Neuroendocrine carcinoma
KW - Solitary fibrous tumor
KW - Squamous differentiation
KW - Transdifferentiation
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U2 - 10.1016/j.humpath.2017.12.024
DO - 10.1016/j.humpath.2017.12.024
M3 - Article
C2 - 29309807
AN - SCOPUS:85047825412
SN - 0046-8177
VL - 77
SP - 175
EP - 180
JO - Human Pathology
JF - Human Pathology
ER -