Preoperative transarterial embolization of a recurrent orbital solitary fibrous tumor with significant hypervascularity: a case report.

BACKGROUND: Orbital solitary fibrous tumors (SFTs) are rare neoplasms. Recurrent, hypervascular, malignant variations of orbital SFTs have recently been noted and can present a surgical challenge. CASE PRESENTATION: We describe a case of a 53-year-old Chinese woman with a history of a resected orbital SFT. She presented with proptosis, limited eyeball movement, and visual loss in the right eye, suggestive of a recurrent SFT. Ocular examination with multimodal imaging revealed a large, nonpulsatile, noncompressible, hypervascular mass behind the eyeball. The patient underwent preoperative transarterial embolization of the main blood supply to the tumor in order to control intraoperative blood loss, followed by ocular enucleation to optimize exposure and enable complete resection of the tumor. Embolization of the right ophthalmic artery and the distal branch of the right internal maxillary artery caused an immediate, substantial reduction of vascular flow, which allowed us to enucleate the eyeball and resect the tumor with minimal blood loss and no complications. CONCLUSIONS: Our case is so far the first Chinese case of successful preoperative embolization of the main blood supply to a large, recurrent, hypervascular orbital SFT. This case also described a different surgical approach to achieve total removal of an orbital SFT without osteotomy.

Avoidance of scapular winging while approaching tumors of the middle scalene region.

BACKGROUND: Large tumors arising from the middle scalene region can displace the middle scalene muscle and distort regional anatomy, placing nerves at risk. Understanding the surgical anatomy of these nerves is key to approaching pathology of the middle scalene muscle and avoiding damage to the dorsal scapular, long thoracic, and spinal accessory nerves, each of which can cause scapular winging and associated morbidity if injured. METHODS: IRB approval was obtained for this study, allowing cases with relevant pathology to be reviewed and presented to highlight the relevant surgical technique. Anatomical depictions were created to correlate intraoperative images with known anatomical relationships. RESULTS: Key to this approach is consideration of the regional anatomy in a standard supraclavicular approach, the superficial plane, containing the anterior scalene muscle and brachial plexus, and the oblique plane containing the middle scalene muscle, long thoracic, spinal accessory, and dorsal scapular nerves. Identification and mobilization of each of these structures prior to lesion removal can not only provide likely boundaries of the tumor, but also allow for protection of the nerves to avoid injury that may lead to scapular winging with associated morbidity and functional impairment of the upper extremity. CONCLUSIONS: Lesions of the middle scalene region often split two important anatomical planes, the superficial and deep, creating an advantageous surgical corridor through an anterolateral approach. Through early identification of known anatomy, these two planes can be developed, and a safe approach to the lesion of the middle scalene region can be exploited.

Pazopanib efficacy in recurrent central nervous system hemangiopericytomas.

INTRODUCTION: There is currently no treatment for solitary fibrous tumors/hemangiopericytomas (SFT/H) of the central nervous system recurring after multiple surgeries and radiotherapies. The NAB2-STAT6 gene fusion is the hallmark of these tumors, and upregulates Early Growth Factor, activating several growth pathways. METHODS: We treated two patients presenting pluri-recurrent meningeal SFT/H with Pazopanib, a broad-spectrum tyrosine kinase inhibitor. We analyzed the exome and RNA sequencing data of one of them and, in addition to another meningeal SFT/H, compared it to the transcriptomic profiling of 5 systemic SFT/H. RESULTS: A dramatic clinical and radiological response was observed in both cases, respectively 84 and 43% decrease after 3 months. As a comparison, Pazopanib has only a stabilizing effect in systemic SFT/H. Indeed, central nervous system SFT/H show overexpression of different tyrosine kinases targeted by Pazopanib. CONCLUSIONS: Two consecutive patients with untreatable central nervous system SFT/H showed a spectacular partial response to Pazopanib, an unprecedented result in SFT/H. This result could be explained by differences in expression profiles and calls for a confirmation in a larger cohort of patients.

[Establishment of Preoperative Diagnosis Helpful in Choosing Minimal Surgical Procedure for Resecting Intrapulmonary Solitary Fibrous Tumor;Report of a Case].

Intrapulmonary solitary fibrous tumor (SFT) arising from the parenchyma of the lung is very rare. Few limited surgery have been performed because preoperative and intraoperative diagnosises of SFT are so difficult. We here report a case of intrapulmonary SFT which was able to be resected by segmentectomy by preoperative diagnosis. A 77-year-old man, who was found to have an abnormal nodule in right lower lobe on computed tomography (CT), was admitted to our hospital. Fluorodeoxyglucose-positron emission tomography (PET) showed a slight uptake in the nodule. By CT guided needle biopsy, the nodule was diagnosed as intrapulmonary SFT pathologically. We could choose segmentectomy as a surgical procedure by preoperative diagnosis.

A huge solitary fibrous tumor localized in the pancreas: a young women.

CONTEXT: Solitary fibrous tumor is an uncommon spindle cell tumor which were first described in 1931 at pleura; it should be seen rarely in extra-pleural localization. CASE REPORT: We report the ninth case of pancreatic solitary fibrous tumor in a 24-year-old woman who presented with mild epigastric pain radiating to the back and chronic constipation. Imaging studies confirmed a solitary mass in the epigastric region that begins from posterior of stomach, fills little curvature and extends to pelvis, invades vascular structures by encircling them and extends to retroperitoneal regions that was considered as it may have mesenchymal origin. The patient underwent an enucleation of the mass which was diagnosed as solitary fibrous tumor, supported by immunohistochemical studies showing positivity for CD34, vimentin and SMA. CONCLUSION: There is limited data regarding biological behavior of solitary fibrous tumors with extra-pleural localization, because they are rare tumors. They are generally asymptomatic and slow growing tumors and it is difficult to distinguish them from other mesenchymal tumors. These issues as well as the prior nine cases are discussed.

Malignant solitary fibrous tumors of the left atrial endocardium.

Solitary fibrous tumors are typically benign and usually develop in the pleura. We herein report the first case of a solitary fibrous tumor that was pathologically malignant and developed in the left atrial endocardium. A 24-year-old woman underwent resection of a malignant solitary fibrous tumor in her right forearm at another hospital. Computed tomography demonstrated a mass in her right pleura 2 months after the surgery. She was referred to our hospital, and a tumor in her left atrium was subsequently found. She underwent resection of these tumors, and pathological examination showed that they were both malignant solitary fibrous tumors.

Solitary fibrous tumour of the sigmoid colon mesentery.

Solitary fibrous tumours (SFTs) are rare tumours arising from mesenchymal tissues. Despite of their more frequent occurrence in the pleura, SFT can present anywhere in the body. Only a few cases have been described arising from the mesentery. Most tumours have a benign nature; however, up to 20% of them can spread, most commonly to liver, lung and bone. Surgical excision including all surrounding tissues remains the treatment of choice; however, there is no consensus regarding the need for adjuvant therapies. We present a 79-year-old man with abdominal pain who was found to have a SFT in the mesentery of the sigmoid colon, treated with en bloc resection. A multidisciplinary team including surgeons, medical and radiation oncologists is recommended in the care of these patients.

A solitary fibrous tumour mimicking an aggressive angiomyxoma/liposarcoma.

We present a case of a solitary fibrous tumour (SFT) resembling an aggressive angiomyxoma/liposarcoma on radiological imaging, causing significant diagnostic challenges preoperatively. A 76-year-old male was incidentally found to have a large pelvic mass on a CT scan. Further evaluation with an MRI scan confirmed a presacral mass containing fat and soft tissue components. It was inseparable from the sacrococcygeal spine, rectal serosa and the posterior wall of the urinary bladder, but no evidence of invasion was seen. A prominent vascular pedicle arising from the epidural vasculature was also noted. Differentials discussed at the multidisciplinary tumour board were an aggressive angiomyxoma versus a liposarcoma. The patient underwent wide resection of the pelvic tumour, anterior resection and end colostomy. Intraoperatively, a large 20 cm pelvic mass involving the sigmoid mesocolon and presacral fascia was found. Final histology reported an SFT with extensive adipocytic metaplasia.

Solitary Fibrous Tumors: 2016 Imaging Update.

Optimal management of solitary fibrous tumor requires a multidisciplinary approach with proper histopathological mapping and use of various imaging modalities for exact delineation of primary tumor and metastatic disease if present. In this article, the authors present a comprehensive review of the spectrum of imaging findings of solitary fibrous tumors involving various organ systems and discuss the role of molecular targeted therapies in the management of metastatic disease.

Solitary fibrous tumor/hemangiopericytoma of the penis: Report of the first case.

Hemangiopericytoma (HPC) is an uncommon perivascular tumor, first described in 1942, occurring most frequently in the extremities (pelvis, meninges, head and neck), and rarely affecting the urogenital system. In 1870, Wagner published the first histological description of a Solitary Fibrous Tumor (SFT) of the pleura. It is now thought that the majority of lesions previously called hemangiopericytomas (HPCs) are essentially indistinguishable from solitary fibrous tumors (SFTs). Nowadays, the new WHO classification of soft tissue tumors categorizes most HPCs as SFTs. We report the first case of penile SFT-HPC in a 44-year-old man, presenting with a 3-year history of slow-growing penile mass. The patient underwent a tumor excision. Six months after surgery he is free of local recurrence and distant metastasis.

Solitary fibrous tumor of the lung.

Solitary fibrous tumors are mesenchymal entities integrated in a mixed group of hemangiopericytoma-like neoplasms. Although classically presented as a pleura-based mass, there are extrapleural sites including the lung. We present the clinical, imaging, and histological features of a solitary fibrous tumor of the lung.