Recurrent Sinonasal CD34-Negative Malignant Solitary Fibrous Tumor Diagnosed on STAT6 Immunohistochemistry and NAB2-STAT6 Fusion

Kakkar, Aanchal; Sakthivel, Pirabu; Rajeshwari, Madhu; Kairo, Arvind; Sharma, Mehar Chand

doi:10.1007/s12105-018-00999-8

Cited by 14 publications

(16 citation statements)

References 13 publications

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“…The positive STAT6 result highly indicated an SFT. Approximately 5-10% of SFTs are negative for CD34, 7 and most of these are MSFT, in other words, a negative CD34 result may be suggestive of a malignant SFT, as in the present case. FISH showed no gene translocation, either in EWSR1, SS18 or FUS, whose gene fusions are traditionally regarded as pathognomonic for diagnosis of Ewing sarcoma, synovial sarcoma and myxoid/round cell liposarcoma, respectively.…”

Section: Discussionsupporting

confidence: 61%

“…There is no difference in incidence rates between male and female patients, and SFT mostly occurs in those aged 50–70 years, with only a small number of cases in younger patients. 3 , 7 , 8 …”

Section: Introductionmentioning

confidence: 99%

“…There is no difference in incidence rates between male and female patients, and SFT mostly occurs in those aged 50-70 years, with only a small number of cases in younger patients. 3,7,8 The aetiology of SFT is still unknown, while some molecular changes are thought to be related to tumour progression, such as changes to NGFI-A binding protein 2 (NAB2)-signal transducer and activator of transcription 6 (STAT6) fusion and tumour protein 53 (TP53). 9,10 Preoperative diagnosis, such as imaging examination and even biopsy can be inaccurate.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Locally advanced malignant solitary fibrous tumour successfully treated with conversion chemotherapy, operation and postoperative radiotherapy: a case report

Liu

Chen

et al. 2021

J Int Med Res

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Preoperative diagnosis of solitary fibrous tumour (SFT) may not provide a complete tumour picture and may be inaccurate. There is no standard treatment for locally advanced or metastasised malignant SFT (MSFT). Here, the case of a 17-year-old male patient with final pathology diagnosis of MSFT is reported. Preoperative biopsy pathology results suggested an Ewing sarcoma that was positive for CD99 antigen, vimentin, friend leukaemia integration 1 transcription factor, apoptosis regulator Bcl-2, and synaptophysin; and negative for CD34 antigen, S-100 protein (S-100), smooth muscle antigen, cytokeratin, and Wilms tumour 1 associated protein. The Ki67 positive rate was 8%, so the patient initially received eight cycles of conversion chemotherapy (vincristine, etoposide, ifosfamide and pirarubicin for one cycle, and vincristine, doxorubicin, and cyclophosphamide/ifosfamide and etoposide for 7 cycles in total). The tumour shrunk significantly and was surgically removed. The final pathology diagnosis was MSFT that was positive for CD99 and signal transducer and activator of transcription 6, and negative for CD34, tumour protein 63, S-100, desmin, and epithelial membrane antigen. Fluorescence in situ hybridization showed no gene translocation in EWS RNA binding protein 1, SS18 subunit of BAF chromatin remodelling complex or FUS RNA binding protein. The patient finally accepted adjuvant radiotherapy of 5600 cGy. Disease-free survival has been > 1 year, with no recurrence or metastasis detected to date. MSFT is rare and treatment for locally advanced or metastatic MSFT remains controversial. The efficacy of the present therapeutic strategy requires further research.

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Section: Discussionsupporting

confidence: 61%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Locally advanced malignant solitary fibrous tumour successfully treated with conversion chemotherapy, operation and postoperative radiotherapy: a case report

Liu

Chen

et al. 2021

J Int Med Res

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“…Detectable via a variety of molecular diagnostics, STAT6 expression is a worthwhile adjunct for head and neck SFT [19]. Diffuse nuclear expression of STAT6 protein, likely representing the presence of the pathognomonic NAB2-STAT6 gene fusion, carries a diagnostic sensitivity estimated above 95% [13,[20][21][22][23]. While other mesenchymal neoplasms frequently exhibit both nuclear and cytoplasmic positivity, SFT demonstrate exclusive nuclear expression.…”

Section: Discussionmentioning

confidence: 99%

Solitary Fibrous Tumors in the Head and Neck: Comprehensive Review and Analysis

Stanisce

Ahmad

Levin

et al. 2019

Head and Neck Pathol

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Solitary fibrous tumors (SFT) arising in the head and neck region are uncommon yet well-recognized entities. Their biologic behavior and management still need to be elucidated. Systematically reviewing all published cases of SFT involving the head and neck region since 1991, a pooled meta-analysis was conducted to evaluate various demographic and tumor characteristics. 587 SFT in the head and neck have been reported; 343 met pooled analysis inclusion criteria. 61% of cases presented as a new mass; 89% were painless. Median onset of symptoms prior to evaluation was 8 months. Pre-operative local invasion and malignant histological features (hemorrhage, necrosis, mitoses > 4/10 hpf) were not statistically associated with decreased recurrence-free survival. Positive surgical margins was the only factor associated with shorter recurrence-free survival (p < 0.001). The evidence presented herein reveals novel associations between clinical presentation and tumor characteristics that provide otolaryngologists with new insight into SFT tumor behavior, thus prompting further investigations.

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“…Moreover, a NAB-STAT6 gene fusion has been found in SFTs. CD34 negative-MSFTs can be diagnosed clearly with the combined expression of STAT6 and the NAB2-STAT6 gene fusion [20]. However, some STAT6-positive cases lacked the NAB2-STAT6 gene fusion.…”

Section: Case Presentationmentioning

confidence: 93%

A pelvic cavity malignant solitary fibrous tumor with dedifferentiation and multifocal cytokeratin expression

Huang

Lin

et al. 2020

Preprint

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Background: Solitary fibrous tumor is an uncommon mesenchymal neoplasm that originates from fibroblasts and occurs predominantly in the visceral pleura. Pelvic cavity small sized malignant solitary fibrous tumor is very rare; it is easily misjudged by surgeons as a benign lesion and resected by laparoscopic surgery. When accompanied by dedifferentiation, malignant solitary fibrous tumor is difficult to diagnose by pathologists. Here, we describe a challenging case.Case presentation: A 47-year-old man was accidentally found to have a pelvic mass for three months, with pain and distension for seven days. The mass was adhered and compressed to the ureter and bladder. The right side of the pelvic cavity had a palpable and substantial mass, with no obvious associated pain. With an assumed diagnosis of a benign tumor, the patient underwent laparoscopic surgery to resect the tumor. Histologically, spindle cell areas and dedifferentiated areas were observed. Immunohistochemical analysis of dedifferentiated regions revealed cytokeratin showed multifocal expression. Vimentin and CD34 were abnormal negative. This case was diagnosed as a MSFT with dedifferentiation. The patient was well after the operation, but, unfortunately, he had a recurrence one year later.Conclusion: Pelvic cavity malignant solitary fibrous tumor is a rare tumor that is in frequently dedifferentiated and exhibiting cytokeratin expression. The diagnosis of this type of tumor can be confusing, and it should be distinguished from Synovial Sarcoma, Liposarcoma, and other malignant tumors. The expression of cytokeratin and the absence of vimentin and CD34 are pitfalls to diagnosis. In this case, there was still a high degree of malignancy despite the small size of the tumor. Clinical-image-pathological multidisciplinary analysis has a great effect on diagnosis and therapy of diseases. This case is a cautionary tale for surgeons and pathologists.

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Recurrent Sinonasal CD34-Negative Malignant Solitary Fibrous Tumor Diagnosed on STAT6 Immunohistochemistry and NAB2-STAT6 Fusion

Cited by 14 publications

References 13 publications

Locally advanced malignant solitary fibrous tumour successfully treated with conversion chemotherapy, operation and postoperative radiotherapy: a case report

Locally advanced malignant solitary fibrous tumour successfully treated with conversion chemotherapy, operation and postoperative radiotherapy: a case report

Solitary Fibrous Tumors in the Head and Neck: Comprehensive Review and Analysis

A pelvic cavity malignant solitary fibrous tumor with dedifferentiation and multifocal cytokeratin expression

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