Der maligne solitäre fibröse Tumor der Niere: Fallbericht und kumulative Literaturanalyse

Martino, Michela de; Böhm, Malte; Klatte, Tobias

doi:10.1055/s-0030-1283853

Cited by 10 publications

(3 citation statements)

References 8 publications

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“…SFT in the kidney is mostly benign but has malignant potential. Recently, there has been an increase in the number of reports of malignant SFT, including metastasis to lymph nodes [ 12 ], lung [ 10 , 13 , 14 ], liver [ 13 , 14 ], pancreas [ 11 ] and bone [ 15 , 16 ], local recurrence [ 17 , 3 ], peritoneal implantation [ 3 ], and invasion to renal vein [ 18 ]. Microscopically, the diagnostic criteria for malignant SFT is the presence of increased cellularity with crowded and overlapping nuclei, pleomorphism, necrosis and hemorrhage, increased mitotic activity more than four mitoses per ten high power fields (HPFs) [ 19 , 8 , 7 ].…”

Section: Introductionmentioning

confidence: 99%

A GRIA2 and PAX8-positive renal solitary fibrous tumor with NAB2-STAT6 gene fusion

et al. 2015

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Solitary fibrous tumor (SFT) is a rare neoplasm composed of mesenchymal-derived spindle cells. Although SFT occurs anywhere in the body, they most frequently affects the thoracic region. Here, we reported an extremely rare case of an extrathoracic SFT occurring primarily in the kidney. To our knowledge, little information has been described on the immunohistochemistry (IHC) and genetics of renal SFT.A 41-year old Japanese female came to our hospital for further examination of a left kidney mass detected incidentally with ultrasound. Extensive investigation of the tumor, including physical, laboratory, and image examinations led to a clinical diagnosis of renal cancer (cT1aN0M0), which were in most parts imbedded in the lower polar parenchyma. The patient underwent laparoscopic radical nephrectomy. The mass was diagnosed pathologically as SFT originating from the kidney, but not as renal carcinoma. Microscopically, the tumor was composed of spindle-shape cells distributed variably in dense collagenous stroma and had a focal hemangiopericytomatous staghorn-like vascular pattern. Mitotic figures, atypical structures, necrosis and hemorrhage were not identified. No adjuvant therapies were given postoperatively. The patient has been free of tumor recurrence for 25 months since the surgery. IHC revealed that the tumor diffusely expressed CD34, CD99, Bcl2, PAX8, NAB2, STAT6, and GRIA2. The tumor stained negatively for desmin, S-100, c-Kit, CK-AE1/AE3, CDK4 and MDM2. A NAB2-SATA6 gene fusion was detected in tumor cells by reverse transcription-polymerase chain reaction, direct sequencing, and an in situ proximity ligation brightfield assay. The gene fusion occurred as an 831 bp truncation of exon 2 in NAB2 connected to the beginning of exon 3 in STAT6. We have reported a case of GRIA2 and PAX8-positive SFT occurring primarily in the kidney with such NAB2-STAT6 gene fusion for the first time. Diffuse expression of PAX8 in the tumor might present with a renal origin. Reportedly, benign histology of SFT cannot necessarily predict favorable clinical prognosis. Genetic alterations recently identified in SFT could possibly refer to risk stratification for tumor recurrence. However, malignant preponderance of extrathoracic SFT over thoracic SFT remains unexplained so far. Long-term follow-up after surgery should be performed in the present case.Electronic supplementary materialThe online version of this article (doi:10.1186/s13000-015-0386-x) contains supplementary material, which is available to authorized users.

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Section: Introductionmentioning

confidence: 99%

A GRIA2 and PAX8-positive renal solitary fibrous tumor with NAB2-STAT6 gene fusion

et al. 2015

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“…The histogenesis of this entity is still unknown, but recent studies suggest a primitive mesenchymal cells or level perivascular [10, 11]. …”

Section: Discussionmentioning

confidence: 99%

Malignant Solitary Fibrous Tumor of the Kidney: Report of the First Case Managed with Interferon

Cuello

Brugés

2013

Case Reports in Oncological Medicine

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Solitary fibrous tumors of the kidney are extremely rare tumors with unpredictable behavior. We describe a case of a patient with a solitary fibrous tumor of kidney with malignant findings with distant metastasis and nephrectomy managed with subcutaneous interferon achieving 23 months of progression-free survival. To date there is no prospective evaluation of any specific modality of treatment, but the surgical management and long-term followup are the only ones so far recommended strategies in the management of these patients. Studies are awaited with more patients to evaluate the different strategies of systemic therapy reported so far to allow adding survival benefit.

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“…Nearly 50 cases of SFT of the kidney have been reported so far in the literature. Most of these types of tumors have a benign character [3]. It is difficult to differentiate it from renal cell carcinoma with imaging techniques.…”

Section: Introductionmentioning

confidence: 99%

Solitary Fibrous Tumor of the Kidney: A Case Report

Demirtaş

Sabur

Akgün

et al. 2013

Case Reports in Urology

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Solitary fibrous tumor is a spindle cell neoplasm mostly originating from pleura; however, it has also recently been reported to be extrapleural. A 57-year-old man presented with left lumbal pain. Ultrasonography and computed tomography showed a cystic lesion of 14 × 11 cm with solid areas and septations in middle and lower poles of the left kidney. Radical nephrectomy was performed. Immunohistochemical studies showed strong reactions with CD34 and CD99. A nuclear positivity with Ki-67 was observed in less than 1% of cells. Despite repeated stainings with vimentin, no clear tumor evaluation could be made due to artifacts. The tumor was negative with Bcl-2, desmin, HMB-45, S100, FVIII, and CD31. Histopathological and molecular studies made the diagnosis of a solitary fibrous tumor. The patient is now currently free of disease at the 26th month of followup.

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Der maligne solitäre fibröse Tumor der Niere: Fallbericht und kumulative Literaturanalyse

Cited by 10 publications

References 8 publications

A GRIA2 and PAX8-positive renal solitary fibrous tumor with NAB2-STAT6 gene fusion

A GRIA2 and PAX8-positive renal solitary fibrous tumor with NAB2-STAT6 gene fusion

Malignant Solitary Fibrous Tumor of the Kidney: Report of the First Case Managed with Interferon

Solitary Fibrous Tumor of the Kidney: A Case Report

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