Dedifferentiated Solitary Fibrous Tumor: A Concise Review

Olson, Nicholas; Linos, Konstantinos

doi:10.5858/arpa.2016-0570-rs

Cited by 75 publications

(59 citation statements)

References 31 publications

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“…In contrast, the 2nd most common variant, NAB2ex6-STAT6ex16/17, tends to be detected in deep-seated and extrathoracic sites, affecting younger patients [26]. No recurrent or specific fusion type associated with dedifferentiated SFT has been identified [27].…”

Section: Discussionmentioning

confidence: 99%

“…Some authors suggest that TERT promoter mutation status might not be a reliable predictor of clinical outcome by itself; however, by integrating it into existing multivariable risk stratification, it might help refine outcome predictions in intermediate-risk cases [29, 30]. As for dedifferentiated SFTs, there is limited available data regarding their clinical behavior and molecular findings, but it seems that these lesions are likely to behave more aggressively than conventional malignant SFTs [4, 27].…”

Section: Discussionmentioning

confidence: 99%

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A dedifferentiated solitary fibrous tumor of the parotid gland: a case report with Cytopathologic findings and review of the literature

2019

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Background Solitary fibrous tumor (SFT) is a ubiquitous mesenchymal neoplasm but it rarely occurs in the parotid gland. The histological features are variable, with the majority having spindle cell morphology and non-specific branching (staghorn) ecstatic vascular pattern. SFT ranges from benign to overtly malignant. Dedifferentiation within SFTs represents an abrupt transition from a well-differentiated component to a high-grade area, the latter most often including poorly differentiated epithelioid/round cell or high-grade spindle cell morphology. To the best of our knowledge, dedifferentiated SFT in the parotid gland has not been previously reported. Case presentation A 33-year-old woman presented with a soft tissue tumor in the right parotid gland that had been present for 6 months. Fine needle aspiration (FNA) cytology indicated epithelioid morphology in the dedifferentiated component of the tumor, along with metachromatic myxoid matrix. The tumor was initially interpreted as a salivary gland neoplasm of uncertain malignant potential (SUMP).Right partial parotidectomy was performed, and microscopic examination of the resected specimen revealed a malignant spindle cell tumor with a central epithelioid/anaplastic component. The tumor cells were diffusely positive for CD34, STAT-6 and FLI-1, and negative for pan-cytokeratin, CAM5.2, p63, S100 protein, CD31, SMA, and calponin.ERG and Ki67 immunostaining showed an accentuated nuclear staining pattern in the central dedifferentiated area. There was no overexpression of p53 or p16. The patient is currently undergoing regular follow-up and is 11 months postresection with no evidence of recurrence or distant metastasis. Conclusions Unlike the typical spindle cell morphology of conventional SFTs, malignant SFTs can show areas of dedifferentiation mimicking an epithelial neoplasm. FNA of dedifferentiated SFTs of the parotid gland may show, a combination of atypical epithelioid cells and metachromatic myxoid/collagenous matrix, which is a less emphasized cytological feature of SFT and may lead to misdiagnosis as a more common parotid gland epithelial neoplasm.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A dedifferentiated solitary fibrous tumor of the parotid gland: a case report with Cytopathologic findings and review of the literature

2019

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“…There is a wide histological spectrum. Histologic variants include myxoid SFT [ 86 ], lipomatous (fat-forming) SFT [ 87 ], giant cell-rich SFT [ 88 ], and dedifferentiated SFT ( Figure 5 ) [ 89 ]. Malignant SFTs show a high mitotic count (>4 mitoses per 10 HPFs), increased cellularity, cytological atypia, necrosis, and/or infiltrative growth.…”

Section: Rare Retroperitoneal Sarcomasmentioning

confidence: 99%

Retroperitoneal Sarcomas: An Update on the Diagnostic Pathology Approach

Choi

2020

Diagnostics

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Retroperitoneal sarcomas are a heterogenous group of rare tumors arising in the retroperitoneum. Retroperitoneal sarcomas comprise approximately 10% of all soft tissue sarcomas. Though any soft tissue sarcoma histologic types may arise in the retroperitoneal space, liposarcoma (especially well-differentiated and dedifferentiated types) and leiomyosarcoma do so most commonly. Retroperitoneal sarcomas are diagnostically challenging, owing to their diversity and morphological overlap with other tumors arising in the retroperitoneum. An accurate diagnosis is necessary for correct management and prognostication. Herein, we provide an update on the diagnostic approach to retroperitoneal sarcomas and review their key histologic findings and differential diagnoses.

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“…Extrapleural SFT is usually more aggressive than the pleural form, and might occur in the mediastinum, retroperitoneum, pelvis, meninges, and soft tissues [56]. Although SFT is usually considered as a clinically indolent neoplasm, the prognosis is substantially unpredictable and only partially related to morphological feature [56]. Primary SFT arising in bone are extremely rare and rarely metastasize.…”

Section: Spindle Cell Sarcomasmentioning

confidence: 99%

Rare Primary Malignant Bone Sarcomas

Palmerini

Righi

Staals

2020

Cancers

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Rare primary malignant bone sarcomas (RPMBS), other than osteosarcoma, chondrosarcoma, chordoma, and Ewing sarcoma, account for about 5–10% of primary bone tumors and represent a major diagnostic challenge. These tumors include spindle cell and round cell sarcoma entities, hemangiopericytoma-like and vascular tumors. Additionally, several histotypes, traditionally described in the soft tissues, such as myxofibrosarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor of bone, have been reported in patients with primary bone tumors. While wide surgical resection is the mainstay of local treatment, systemic therapy of these rare entities is controversial. Patients with undifferentiated spindle cell or pleomorphic high-grade tumors of bone, are usually treated with osteosarcoma-like chemotherapy, while patients with round cell and undifferentiated round cell tumors (URCTs), may respond to sarcoma treatment regimens for Ewing sarcoma patients. Studies on analogies and differences among these ultra-rare tumors have seldom been reported. This review describes relevance, clinical aspects, diagnostic procedures, staging, treatment recommendations, and current research in this composite tumor group.

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Dedifferentiated Solitary Fibrous Tumor: A Concise Review

Cited by 75 publications

References 31 publications

A dedifferentiated solitary fibrous tumor of the parotid gland: a case report with Cytopathologic findings and review of the literature

A dedifferentiated solitary fibrous tumor of the parotid gland: a case report with Cytopathologic findings and review of the literature

Retroperitoneal Sarcomas: An Update on the Diagnostic Pathology Approach

Rare Primary Malignant Bone Sarcomas

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