The Forgotten Phacomatoses: A Neuroimaging Review of Rare Neurocutaneous Disorders.

Phakomatoses, or neurocutaneous syndromes, are a heterogeneous group of rare genetic disorders that predominantly affect structures arising from the embryonic ectoderm, namely the skin, eye globe, retina, tooth enamel, and central nervous system. Other organs are also involved in some syndromes, mainly cardiovascular, pulmonary, renal, and musculoskeletal systems. Currently, more than sixty distinct entities belonging to this category have been described in the literature. Common phakomatoses include conditions like Neurofibromatosis and Tuberous sclerosis. Several review papers have focused on various aspects of these common conditions, including clinical presentation, genetic and molecular basis, and neuroimaging features. In this review, we focus on rare neurocutaneous syndromes: Melanophakomatoses (Ie, Neurocutaneous Melanosis, and Incontinentia Pigmenti), Vascular Phakomatoses (Ie, Ataxia Telangiectasia and PHACE Syndrome), and other conditions such as Cowden Syndrome, Basal Nevus Syndrome, Schwannomatosis, Progressive Facial Hemiatrophy, Gomez-Lopez-Hernandez Syndrome, Wyburn-Mason Syndrome, CHILD Syndrome, and Proteus Syndrome. We also review the neuroradiologic manifestations of these conditions as a guide for neurologists and neuroradiologists in their daily practice.

It's About Quality, Not Quantity: Qualitative FDG PET/CT Criteria for Therapy Response Assessment in Clinical Practice.

OBJECTIVE. FDG PET/CT has emerged as an effective tool for the timely accurate assessment of how tumors respond to therapy. To standardize interpretation and reporting, numerous response criteria have been developed. This article will review the evolution of these criteria along with their strengths and weaknesses. CONCLUSION. Several qualitative assessments applicable to common malignancies have been developed in recent years that solve many of the challenges faced by their quantitative predecessors. These are reviewed, and information is provided regarding individual treatment efficacy and prognosis.

Review of 18F-Fluciclovine PET for Detection of Recurrent Prostate Cancer.

Fluorine 18 (18F) fluciclovine (anti-1-amino-3-18F-fluorocyclobutane-1-carboxylic acid [FACBC]) is a radiolabeled amino acid analog that takes advantage of the amino acid transport upregulation in several types of cancer cells. FACBC is taken up to a greater extent in prostate cancer cells than in surrounding normal tissue, providing an opportunity for its use in cases of this common cancer. In 2016, the U.S. Food and Drug Administration found the accuracy of FACBC PET to be superior to that of other molecular imaging techniques and subsequently granted approval for its use in PET of recurrent prostate cancer. As FACBC is an 18F radiotracer, an on-site cyclotron is not required for its production. This feature enables the widespread clinical availability of this agent and, in turn, an opportunity for improved patient care. The clinical pharmacology and imaging features of FACBC are reviewed, and the role of this agent in the imaging of recurrent prostate cancer, within the context of research that supports its effectiveness, is discussed. The administration of and image acquisition facilitated by using FACBC, as compared with 18F fluorodeoxyglucose, which is more widely used, are described. In addition, the criteria for interpreting FACBC imaging findings are outlined, with emphasis on common causes of false-positive and false-negative findings. ©RSNA, 2019.

Split-Dose 18F-FDG PET/CT-Guided Microwave Ablation for Liver Metastasis Recurrence with Immediate Treatment Assessment.

A 48-y-old man with a history of colon cancer presented with recurrent hepatic metastasis along a prior microwave ablation bed. Split-bolus, intraprocedural 18F-FDG PET was performed to guide repeat microwave ablation and immediately confirm complete treatment. PET-guided ablation is highly accurate for targeting and treating malignant hepatic lesions and feasible for nonspecialized tertiary care hospitals without an onsite cyclotron.

Multimodal imaging in the prenatal diagnosis of tuberous sclerosis complex.

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder in which benign hamartomas develop in multiple organ systems. Increasingly, stigmata of the disease, such as cardiac rhabdomyomas, are detected on routine prenatal ultrasound. Such a finding should prompt additional imaging studies in order to confirm diagnosis and to identify potential complications, which vary greatly from patient to patient. Early diagnosis allows for accurate parental counseling, coordination of high-level perinatal care, and subspecialty followup. We present a case of TSC in utero wherein access to and use of multiple imaging modalities confirmed diagnosis and allowed the patient to receive optimal care prior to birth.