Malignant Solitary Fibrous Kidney Tumor with Peritoneal Disease: A Case Report

Cruz, Maria Isabel Rodríguez; Sánchez, José Emilio Hernández; Blázquez, Beatriz Segovia; Nogal, Sara Belén Prieto; Tejeda, Luis Miguel Gómez

doi:10.1159/000362539

Cited by 3 publications

(5 citation statements)

References 8 publications

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“…The cells were diffusely positive for CD34, CD99, Bcl2, vimentin, α-smooth muscle actin, NAB2, STAT6, and GRIA2 (Figs. 3c and 4 ) [ 3 , 4 , 26 , 29 ]. Ki-67 levels were very low in the tumor nuclei (0.6 to 0.7 %) (Fig.…”

Section: Case Presentationmentioning

confidence: 99%

“…It was first depicted in the pleura of the lung by Klemperer et al [ 2 ] in 1931. To date, SFTs have been reported in various locations other than the pleura, including the meninges, orbit, neck, nose, paranasal cavity, thyroid, mediastinum, adrenal gland, liver, pancreas, retroperitoneum, spermatic cord, skin, extremities, uterine cervix, prostate, urinary bladder and kidney [ 3 – 5 ]. Among these affected sites, renal SFT, first reported by Gelb et al [ 6 ] in 1996, is very rare.…”

Section: Introductionmentioning

confidence: 99%

“…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%

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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: Case Presentationmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2015

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“…The most essential prognostic factor is surgical resectability because complete resection of the tumor is curative in more than 90% of cases[ 1 , 42 ]. Poor prognosis is observed in patients with incomplete resection[ 43 ]. However, SFTs are hypervascular, complicating surgical resection[ 9 ] regardless of the tumor location.…”

Section: Discussionmentioning

confidence: 99%

“…Adjuvant chemoradiotherapy is not widely practiced or accepted as the standard of care[ 46 ]. Some experts have suggested radiotherapy or adjuvant chemotherapy, although poor outcomes have been reported for these treatments[ 43 ]. Some experts have used antiangiogenic agents as a therapeutic strategy in recent years.…”

Section: Discussionmentioning

confidence: 99%

Mesentery solitary fibrous tumor with postoperative recurrence and sarcomatosis: A case report and review of literature

Chiu¹,

Ishibashi²,

Wakama³

et al. 2022

WJCO

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BACKGROUND Solitary fibrous tumors are rare neoplasms of mesenchymal origin. They are often of low malignant potential and rarely metastasize. They frequently arise from the pleura and can occur at any soft tissue site in the body. However, these tumors rarely develop in the mesentery, peritoneal cavity or peritoneum. CASE SUMMARY We report on a scarce case of solitary fibrous tumor of the rectal mesentery showing sarcomatosis about 4 years after previous tumor resection. This 69-year-old male had no clinical symptoms but was transferred to our hospital because of a suspected tumor recurrence from follow-up abdominal computed tomography. Tumor markers (CEA, CA 19-9 and CA 125) were within the normal range. Open laparotomy showed sarcomatosis, and pathology confirmed its mesenchymal origin and diagnosis as the solitary fibrous tumor. Our case may be the second recurrent mesentery solitary fibrous tumor reported to date, and the only one with progression to sarcomatosis. There has been no evidence of recurrence in follow-up at the 28 th mo after extensive intra-operative peritoneal lavage and cytoreductive surgery. CONCLUSION Although there are few risk factors of cancer recurrence in this patient, careful long-term follow-up after cytoreductive surgery is necessary.

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Malignant solitary fibrous tumor of the kidney with IGF2 secretion and without hypoglycemia

Zhou,

Liu,

et al. 2024

World J Surg Onc

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Background Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal tumor that mostly involves the pleura and infrequently involves extra-pleural sites. De novo SFT of the kidney is uncommon, and malignant SFT is extremely rare. Case presentation We report a case of a 51-year-old man with a large malignant SFT in the left kidney. Pathological examination confirmed the diagnosis of SFT based on typical morphology, nuclear STAT6 expression, and NAB2-STAT6 gene fusion. The malignant subtype was determined by a large tumor size (≥ 15 cm) and high mitotic counts (8/10 high-power fields). KRAS mutation was identified by DNA sequencing. Insulin-like growth factor 2 (IGF2) was diffusely and strongly expressed in tumor cells, however, hypoglycemia was not observed. Hyperglycemia and high adrenocorticotropic hormone (ACTH) concentration were observed one month after surgery. Hormone measurements revealed normal blood cortisol and aldosterone levels, and increased urinary free cortisol level. A pituitary microadenoma was identified using brain magnetic resonance imaging, which may be responsible for the promotion of hyperglycemia. Conclusions We report a case of renal malignant SFT with a KRAS mutation, which was previously unreported in SFT and may be associated with its malignant behavior. Additionally, we emphasize that malignant SFT commonly causes severe hypoglycemia due to the production of IGF2. However, this effect may be masked by the presence of other lesions that promote hyperglycemia. Therefore, when encountering a malignant SFT with diffuse and strong IGF2 expression and without hypoglycemia, other lesions promoting hyperglycemia need to be ruled out.

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Malignant Solitary Fibrous Kidney Tumor with Peritoneal Disease: A Case Report

Cited by 3 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

Mesentery solitary fibrous tumor with postoperative recurrence and sarcomatosis: A case report and review of literature

Malignant solitary fibrous tumor of the kidney with IGF2 secretion and without hypoglycemia

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