Dedifferentiation within solitary fibrous tumor is a rare and only recently characterized phenomenon. It differs from malignant solitary fibrous tumor in that there is abrupt transition between classical solitary fibrous tumor and the dedifferentiated component. The latter is a high-grade sarcoma, which can exhibit a number of morphologies, but heterologous differentiation is exceptionally rare. We report a case of dedifferentiated solitary fibrous tumor, with heterologous osteosarcomatous and rhabdomyosarcomatous elements, arising in the deep soft tissue of the thigh of a 59-year-old man. This comprised morphologically and immunohistochemically typical solitary fibrous tumor, juxtaposed to pleomorphic, high-grade malignant neoplasm of 2 distinct lineages. The sharp demarcation between well-differentiated and dedifferentiated components is typical of the dedifferentiation seen in other mesenchymal neoplasms. This expands the range of histopathology of this rare, newly characterized type of malignant progression in solitary fibrous tumor.

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.anndiagpath.2012.08.006DOI Listing

Publication Analysis

Top Keywords

solitary fibrous
32
fibrous tumor
32
heterologous osteosarcomatous
8
osteosarcomatous rhabdomyosarcomatous
8
rhabdomyosarcomatous elements
8
solitary
8
dedifferentiated solitary
8
fibrous
8
tumor
8
dedifferentiation solitary
8

Similar Publications

Solitary fibrous tumor (SFT) is an uncommon spindle cell neoplasm, which generally arises from the pleura. There have been documented a number of extrapleural origins including the head and neck in the literature. It is emphasized to make a diagnosis in a rare location such as the retropharyngeal space.

View Article and Find Full Text PDF

Background: Solitary fibrous tumors (SFTs) of the pleura are usually benign. We present a case of SFT of the pleura which grew rapidly after slow long-term progression.

Case Presentation: A 78-year-old man was referred to our hospital for left-sided back pain and shortness of breath.

View Article and Find Full Text PDF

Background: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-malignant disorder characterized by excessive proliferation of histiocytes, the cause of which remains unknown. Although the lymph nodes are the most commonly affected site, some patients may present with extranodal involvement, particularly in the skin, nasal cavity, eyes, and bones. In this report, we aim to present a unique case of RDD with pleural involvement in a 61-year-old patient.

View Article and Find Full Text PDF

Intracranial solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) are rare, aggressive tumors typically found along the dural sinuses. Despite their aggressive nature, complete surgical resection remains the most significant factor in reducing recurrence and improving survival. Here, we present the case of a 32-year-old male patient who presented with a new-onset headache and vertigo.

View Article and Find Full Text PDF

Purpose: The NAB2::STAT6 fusion is predominantly associated with solitary fibrous tumors (SFTs) and is utilized in diagnosing SFTs through nuclear STAT6 protein overexpression. Recent studies expanded the phenotypic spectrum of NAB2::STAT6 rearranged neoplasms, including adamantinoma-like and teratocarcinosarcoma-like phenotypes. We report a case of a NAB2::STAT6 rearranged epithelial tumor exhibiting sebaceous differentiation in the parotid gland.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!