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Calcifying fibrous tumor of the clivus presenting in an adult.

ABSTRACT: Calcifying fibrous tumor is a benign fibrous tumor. It rarely occurs in the clivus. The present study describes a case of a 56-year-old female, who was admitted to Taihe Hospital with dizziness not accompanied with headache for 2 months. Brain computed tomography examination revealed a well-defined, partially calcified lytic-expansile lesion in the clivus, which corresponded to an enhancing mass on contrast-enhanced magnetic resonance imaging. The patient underwent endoscopic resection. Subsequent pathologic examination of the resected tissue confirmed that the tumor was calcifying fibrous tumor. The patient was followed up for 3 months after operation without recurrence or metastasis.

SUBMITTER: Zhang T

PROVIDER: S-EPMC6460248 | biostudies-literature | 2019 Jun

REPOSITORIES: biostudies-literature

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Calcifying fibrous tumor of the clivus presenting in an adult.

Zhang Tao T Xu Lin L Gu Liangrui L Chen Wen W Pandey Gaurab G Wang Jingjing J Wu Yijun Y

Radiology case reports 20190410 6

Calcifying fibrous tumor is a benign fibrous tumor. It rarely occurs in the clivus. The present study describes a case of a 56-year-old female, who was admitted to Taihe Hospital with dizziness not accompanied with headache for 2 months. Brain computed tomography examination revealed a well-defined, partially calcified lytic-expansile lesion in the clivus, which corresponded to an enhancing mass on contrast-enhanced magnetic resonance imaging. The patient underwent endoscopic resection. Subseque ...[more]

PMID: 31011376

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Project description:Solitary fibrous tumors (SFTs) are NAB2-STAT6 fusion-associated neoplasms. There are several subtypes of NAB2-STAT6 fusions, but their clinical significances are still unclear. Moreover, the mechanisms of malignant progression are also poorly understood. In this study, using 91 SFT cases, we examined whether fusion variants are associated with clinicopathological parameters and also investigated the molecular mechanism of malignant transformation using whole-exome sequencing. We detected variant 1b (NAB2ex4-STAT6ex2) in 51/91 (56%) cases and variants 2a/2b (NAB2ex6-STAT6ex16/17) in 17/91 (19%) cases. The NAB2-STAT6 fusion variant types were significantly associated with their primary site (P < 0.001). In addition, a TERT promoter mutation was detected in 7/73 (10%) cases, and it showed a significant association with malignant SFTs (P = 0.003). To identify molecular changes during malignant progression, we selected an index patient to obtain parallel tissue samples from the primary and metastatic tumors. In the metastatic tissue, 10 unique molecular alterations, including those in TP53 and APAF1, were detected. In vitro functional experiments showed that APAF1 depletion increased the tumor potency of cells expressing NAB2-STAT6 fusion protein under treatment with staurosporine. We found that TP53 immunopositivity (P = 0.006) and loss of APAF1 immunoreactivity (P < 0.001) were significantly associated with malignant SFTs. Our study suggests that dysfunction of TP53 and APAF1 leads to impaired apoptotic function, and eventually contributes toward malignant SFT transformation. KEY MESSAGES: We firstly found that the TERT promoter mutation was strongly associated with malignant SFTs (P = 0.003) and the representative 1b (NAB2ex4-STAT6ex2) or 2a (NAB2ex6-STAT6ex16) fusion variants similarly contribute to tumorigenicity. We also found that TP53 immunopositivity (P = 0.006) and loss of APAF1 immunoreactivity (P < 0.001) were significantly associated with malignant SFTs. Our study suggests that dysfunction of TP53 and APAF1 leads to impaired apoptotic function, and eventually contributes toward malignant SFT transformation.

Project description:Hyperplastic dental follicles (HDFs) represent odontogenic hamartomatous lesions originating from the pericoronal tissues and are often associated with impacted or embedded teeth. These lesions may occasionally feature unique calcifying bodies, known as calcifying whorled nodules (CWNs), characterized by stromal cells arranged in a whorled or spiral fashion. CWNs are typically observed in multiple calcifying hyperplastic dental follicles or regional odontodysplasia. In our study, we examined 40 cases of HDFs, including nine instances with characteristics of CWNs, referred to as calcifying hyperplastic dental follicles (CHDFs), which are infrequently accompanied by odontodysplasia. The median ages of the HDFs and CHDFs were 16 (ranging from 3 to 66) and 15 (ranging from 11 to 50) years, respectively. The lower third molars were the most frequently affected by HDSFs and CHDFs, followed by the upper canines. A histological examination was conducted on all 40 cases, with an immunohistochemical analysis performed on 21 of them. Among the cases with CWN, nine affected a single embedded tooth, with one exception. CWNs exhibited diverse calcifications featuring sparse or entirely deposited psammoma bodies, and some displayed dentinoid formation. Immunohistochemically, the stromal cells of HDFs were frequently positive for CD56 and nestin. By contrast, CWNs were negative for CD56 but positive for nestin as well as hairy and enhancer split 1 (HES1), with a few dentin sialoprotein (DSP)-positive calcified bodies. Our results revealed that hamartomatous CHDFs can impact multiple and single-embedded teeth. CWNs composed of nestin and HES1-positive ectomesenchymal cells demonstrated the potential to differentiate into odontoblasts and contribute to dentin matrix formation under the influence of HES1. This study is the first report documenting odontoblastic differentiation in HDFs. The rare occurrence of HDFs and CHDFs contributes to limited comprehension. To prevent misdiagnosis, a better understanding of these conditions is necessary.

Dataset Information

Calcifying fibrous tumor of the clivus presenting in an adult.

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Calcifying fibrous tumor of the clivus presenting in an adult.

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