What is a Solitary Fibrous Tumor?

Solitary Fibrous Tumor
Solitary Fibrous Tumor. Histologic and immunophenotypic characteristics of the tumor. (a) Sections of pleural-based solitary fibrous tumor showing a cellular spindle-cell neoplasm with a patternless architecture and "staghorn" vessels. (b and c) A nodular collection of epithelioid clear cells was incidentally found within the tumor. (d) These cells are negative for CD34 (in contrast to the solitary fibrous tumor on the left), but immunoreactive with (e) broad-spectrum cytokeratin, (f) vimentin, (g) CD10 and (h) paired box gene 2 (PAX2). Renal cell carcinoma metastasizing to solitary fibrous tumor of the pleura: a case report. Kragel C, Wei S - Journal of medical case reports (2011). Not Altered. CC.

The solitary fibrous tumor is a fibroblastic tumor.

What is the Pathology of Solitary Fibrous Tumor? 

The pathology of solitary fibrous tumor is: 

-Etiology: The cause of the solitary fibrous tumor is unknown. 

-Genes involved: NAB2-STAT6.

-Pathogenesis: The sequence of events that lead to the solitary fibrous tumor is the paracentric inversion involving chromosome 12q, resulting in NAB2-STAT6 gene fusion. 

-Histology: The histology associated with solitary fibrous tumors shows ovoid to fusiform spindle cells with indistinct cell borders arranged haphazardly or in short, ill-defined fascicles. 

How does Solitary Fibrous Tumor Present? 

Patients with solitary fibrous tumors typically affect both males and females at 40-70 years of age. The symptoms, features, and clinical findings associated with solitary fibrous tumors show painless lump with slow growth. 

How is Solitary Fibrous Tumor Diagnosed? 

A solitary fibrous tumor is diagnosed by histologic findings. Imaging features are non-specific.

How is Solitary Fibrous Tumor Treated? 

Solitary fibrous tumor is treated by surgical resection.

What is the Prognosis of Solitary Fibrous Tumor? 

The prognosis of solitary fibrous tumors is poor with a 10 – 30% (local or distant) recurrence rate.