Spindle Cell Hemangioma and Atypically Localized Juxtaglomerular Cell Tumor in a Patient with Hereditary BRIP1 Mutation: A Case Report.

Spindle cell hemangioma is a benign vascular tumor typically occurring in the dermis or subcutis of distal extremities as red-brown lesions that can grow in both size and number over time. They can be very painful and potentially disabling. A family history of cancer or previous history may be relevant and must be taken into consideration. Juxtaglomerular cell tumor (reninoma) is an extremely rare cause of secondary hypertension diagnosed mostly among adolescents and young adults. Excessive renin secretion results in secondary hyperaldosteronism. Subsequent hypokalemia and metabolic alkalosis, together with high blood pressure, are clues for clinical diagnosis. Histological examination of the excised tumor leads to a definitive diagnosis. Reninoma is found in subcapsular localization, in most cases as a solitary mass, in imaging studies of kidneys. Exceptionally, it can be located in another part of a kidney. Both spindle cell hemangioma and reninoma are extremely rare tumors in children and adolescents. Herein, the authors present a case report of a patient with hereditary BRCA1 interacting protein C-terminal helicase 1 (BRIP1) mutation, spindle cell hemangioma, and secondary hypertension caused by atypically localized reninoma.

Experimental Electrophysiological and Pressure Responses of Urinary Bladder Detrusor to Lumbar to Sacral Nerve Rerouting - An Animal Study with Negative Results.

Background/Aims/Objectives: To verify the transfer of evoked potentials through anastomosis of an experimentally created micturition reflex arc and to detect said potentials directly on the detrusor and sphincter of rabbit urinary bladder. METHODS: During 2013-2015, 17 rabbits were operated upon and measurement followed during reoperation 3-16 months later. Suitable ventral spinal roots were electrophysiologically detected following laminectomy, and a somatic-central nervous system-autonomic micturition reflex arc was created. During reoperation, the ventral root was stimulated above and below the anastomosis, the evoked potentials on the bladder detrusor and sphincter were measured, and intravesical pressure was monitored. RESULTS: With stimulation above the anastomosis, 9 animals (53%) displayed a urinary bladder detrusor response and 7 (41%) a sphincter response. Four rabbits (24%) had elevated intravesical pressure. During the control stimulation below the anastomosis, we detected a detrusor response in 7 animals (41%), a sphincter response in 5 (29%), and elevated pressure in 4 (24%). Neither induction of micturition nor decrease in external sphincter activity occurred. CONCLUSIONS: Creation of a somatic-CNS-autonomic reflex arc is technically possible. However reflex activity transferring through the anastomosis is detectable on the detrusor only in some individuals, and is unable to induce a micturition reflex with or without accompanying detrusor-sphincter dyssynergia.