Malignant Glomus Tumor of the Kidney: A Case Report and Review of the Literature

Introduction/Objective Glomus tumors are rare neoplasms arising from the glomus body which is a specialized arteriovenous anastomosis that has a role in temperature regulation of the skin. They account for less than 2% of soft tissue tumors and typically manifest as painful nodules in the extremities, most commonly in the subungual region of the fingers and dermis of the wrist, forearm, and foot. They rarely arise in visceral organs as they lack glomus bodies. There are rare case reports of benign glomus tumors arising in the kidney, and malignant renal glomus tumors are exceedingly rare. Methods/Case Report We present a case of a 32-year-old man who was found to have a right renal mass incidentally after being involved in a motor vehicle accident. Computed tomography of the abdomen revealed a 5.3 cm enhancing mass in the posterior aspect of the inferior pole of the right kidney. Histological examination revealed a solid neoplasm composed of a pleomorphic population of cells with densely eosinophilic to pale cytoplasm, centrally located round to oval nuclei with fine nuclear chromatin and relatively inconspicuous nucleoli. Prominent lymphovascular invasion and 1 mitotic figure per 10 high power fields were also present. Areas composed of necrosis, sclerosis, hemosiderin deposition and calcification were identified. Immunohistochemical stains revealed the neoplastic cells to be positive for CD34, vimentin, smooth muscle actin, caldesmon, with focal reticulin staining around the tumor cells. Cells were negative for desmin, high and low molecular weight cytokeratins, synaptophysin, chromogranin, PAX8, and GATA3. The morphology and immunoprofile of the mass are consistent with the diagnosis of glomus tumor. Results (if a Case Study enter NA) NA Conclusion Although very rare, the presence of prominent vascular invasion, increased mitotic activity as well as necrosis are consistent with malignant behavior and support the classification as a malignant glomus tumor.

An eighth note

Venous stenosis is the most common cause of arteriovenous fistula (AVF) failure in hemodialysis patients. For patients with AVF stenosis, the pressure over the antecedent part of the AVF stenotic lesion will increase if arterial inflow is sufficient. We report a chronic hemodialysis patient who received an angiographic examination for the juxta-anastomosis stenosis of his AVF. A unique feature of a collateral venous branch antecedent to the stenotic lesion was noted, resembling a musical sign as the “eighth note.” After percutaneous transluminal angioplasty, the eighth note attenuated markedly at once. Of note, the eighth note sign is not seen frequently, and thus we postulate that the formation of an eighth note sign on the radiocephalic fistula should fulfill the following requirements, including a sufficient arterial inflow, an adjacent collateral branch close enough to the arteriovenous anastomosis, a severe juxta-anastomotic stenotic lesion, and an intact ulnar venous drainage system.

Patency and Efficacy of Anatomical Snuffbox Arteriovenous Fistula Compared with Wrist Fistula in Patients with End-Stage Renal Disease

Highlights Abstract Introduction: Snuffbox arteriovenous fistula (AVF) is one of the most common vascular accesses for hemodialysis. Despite all the advantages, it is not usually considered the first choice of treatment. This could be primarily due to difficulty in its technical procedure and a higher probability of failure than other sites such as cubital. Therefore, the present study aimed at comparing the efficacy and patency rate between the snuffbox and the wrist AVF. Methods: Seventy-eight patients with end-stage renal disease were enrolled in this study. They were randomly assigned into the snuffbox and the wrist groups. Each participant had an appropriate vein at the time of examination and was eligible for distal AVF creation. The procedure was conducted under local anesthesia, and arteriovenous anastomosis was done in an end-to-side configuration. AVF maturation was defined by initiation of successful dialysis through the AVF 8–10 weeks postoperatively. Results: The results showed that 61% of snuffbox AVFs and 65% of wrist AVFs became matured and were used for further dialysis. Diabetes and female sex had negative impacts on AVF patency. In addition, no significant relationship was found between the patency rate and demographic as well as clinical variables such as age, hypertension, and duration of the previous dialysis. Conclusions: No significant differences were found in the maturation and patency rate between the wrist and the snuffbox AVFs. Therefore, the snuffbox fistula is recommended in the first step, as there would be a possibility for re-creation on the ipsilateral wrist once the AVF fails.

Rapid growth of spinal epidural capillary hemangioma associated with isolated intraosseous lesion at the same level: A case report

A 47-year-old man presented with a spinal epidural capillary hemangioma manifesting as a 6-month history of worsening low back pain and lower limb pain. Computed tomography and magnetic resonance imaging (MRI) at a previous hospital showed intraosseous abnormal signals and an epidural lesion at the L3 level. One month later, MRI revealed rapid growth of the epidural lesion. Total resection of the epidural tumor and partial removal of the intraosseous tumor were performed. Macroscopically, the intraosseous lesion and epidural lesion seemed to be isolated from each other. Histopathological diagnosis was consistent with capillary hemangioma. Spinal epidural capillary hemangioma sometimes recurs via underlying arteriovenous anastomosis. Progressive clinical symptoms, as seen in our case, indicate the possibility of tumor growth even if the lesions are well demarcated, which suggests of benign tumor. Spinal epidural capillary hemangioma in addition to metastatic spinal tumors should be considered in the differential diagnosis of spinal epidural tumors associated with intraosseous lesions.

Ocular findings in two Chinese children with Loeys-Dietz syndrome

Background: Loeys-Dietz syndrome (LDS) is a type of connective tissue disease with systemic symptoms similar to Marfan syndrome. Ocular findings are rarely reported especially fundus and extraocular muscles. Case presentation: A 6-month old boy with systemic skeletal development delay was found peripheral non-perfusion and neovascularization in the both eyes, and gaven intravitreal injection of ranibizumab and laser. Fundus examination revealed a mild straightening of the temporal vessel in the both eyes. A 22-month old girl with confirmed connective tissue disorder presented to our hospital for strabismus and showed congenital hypoplasia of extraocular muscles. She also had arteriovenous anastomosis in the retinal. The diagnosis of LDS was supported by the genetic DNA examination. Conclusion: His is the first report of LDS with congenital hypoplasia of extraocular muscles, meanwhile, ocular examination especially fundus should be paid attention to.

Sono-angiography for dialysis vascular access based on the freehand 2D ultrasound scanning

Introduction: Dialysis vascular access, preferably an autogenous arteriovenous fistula, remains an end stage renal disease (ESRD) patient’s lifeline providing a means of connecting the patient to the dialysis machine. Once an access is created, the current gold standard of care for maintenance of vascular access is angiography and angioplasty to treat stenosis. While point of care 2D ultrasound has been used to detect access problems, we sought to reproduce angiographic results comparable to the gold standard angiogram (fistulogram) using ultrasound data acquired from a conventional 2D ultrasound scanner. Methods: A 2D ultrasound probe was used to acquire a series of cross sectional images of the vascular access including arteriovenous anastomosis of a subject with a radio-cephalic fistula. These 2D B-mode images were used for 3D vessel reconstruction by binary thresholding to categorize vascular versus non-vascular structures followed by standard image segmentation to select the structure representative of dialysis vascular access and morphologic filtering. Image processing was done using open source Python Software. Results: The open source software was able to: (1) view the gold standard fistulogram images, (2) reconstruct 2D planar images of the fistula from ultrasound data as viewed from the top, analogous to computerized tomography images, and (3) construct a 2D representation of vascular access similar to the angiogram. Conclusion: We present a simple approach to obtain an angiogram-like representation of the vascular access from readily available, non-proprietary 2D ultrasound data in the point of care setting. While the sono-angiogram is not intended to replace angiography, it may be useful in providing 3D imaging at the point of care in the dialysis unit, outpatient clinic, or for pre-operative planning for interventional procedures. Future work will focus on improving the robustness and quality of the imaging data while preserving the straightforward freehand approach used for ultrasound data acquisition.