Imaging Features of Primary Intracranial Sarcoma with DICER1 Mutation: A Multicenter Case Series.

Primary intracranial sarcoma, DICER1-mutant, is a rare, recently described entity in the fifth edition of the WHO Classification of CNS Tumors. Given the entity's rarity and recent description, imaging data on primary intracranial sarcoma, DICER1-mutant, remains scarce. In this multicenter case series, we present detailed multimodality imaging features of primary intracranial sarcoma, DICER1-mutant, with emphasis on the appearance of the entity on MR imaging. In total, 8 patients were included. In all 8 patients, the lesion demonstrated blood products on T1WI. In 7 patients, susceptibility-weighted imaging was obtained and demonstrated blood products. Primary intracranial sarcoma, DICER1-mutant, is a CNS neoplasm that primarily affects pediatric and young adult patients. In the present case series, we explore potential imaging findings that are helpful in suggesting this diagnosis. In younger patients, the presence of a cortical lesion with intralesional blood products on SWI and T1-weighted MR imaging, with or without extra-axial blood products, should prompt the inclusion of this entity in the differential diagnosis.

Parathyroid carcinoma: Imaging features of initial presentation and recurrence. A single center experience.

BACKGROUND AND PURPOSE: Parathyroid carcinoma is the rarest endocrine neoplasm with very few published data discussing its imaging appearance with primary focus on ultrasound imaging features. We present our 23 years institutional experience to highlight multimodality imaging evaluation at presentation and at recurrence. MATERIAL AND METHODS: Retrospective chart review of clinical and pathological diagnosis of parathyroid carcinoma patients presented for initial or recurrent disease management at M.D. Anderson Cancer Center between the period of January 2000 and February 2023 was performed. Imaging findings on US, CT, PET/CT, and technetium-99m sestamibi were analyzed for initial and local recurrent parathyroid carcinoma. We further assess patterns of distant recurrence and its location. RESULTS: Twenty three patients with pathological and clinical diagnosis of initial (14 patients) or recurrent parathyroid carcinoma (14 patients) were included in this study. US findings of parathyroid carcinoma were larger lesions, increased/irregular vascularity, and non-circumscribed margins. Multiphasic CT findings of parathyroid carcinoma included an arterially enhancing lesion that is hypoenhancing relative to the thyroid and demonstrates no washout on delayed imaging. Highly suggestive findings for recurrent disease included a hypoechoic solid nodule (91.67%) with increased vascularity on ultrasound (81.8%) with corresponding enhancement on CT. CONCLUSION: Parathyroid carcinoma is a rare malignancy often diagnosed after surgical resection. We provided CT and US imaging features that are helpful in suggesting the diagnosis of parathyroid carcinoma and detection of early local recurrence.

Good Gone Bad: Complications of Chemotherapy, Immunotherapy, and Radiotherapy on the CNS.

With recent advancements in cancer therapy, especially immunotherapy, overall survival of many cancers has increased and patient toxicity has been reduced. However, many complications of traditional cancer therapy are still prevalent and complications of novel therapies are just beginning to appear. The neuroradiologist may be the first to visualize signs of these complications on imaging. This article describes the notable imaging findings of several unique and characteristic complications of CNS cancer therapy, including toxicities of chemotherapies, immunotherapies, and radiotherapy. Complications of chemotherapeutic agents covered include methotrexate-induced and disseminated necrotizing leukoencephalopathy, and chemotherapy-induced myelopathy. Immunotherapy complications included are Tacrolimus-related Optic Neuropathy, Rituximab and Immune reconstitution inflammatory syndrome-associated Progressive Multifocal Leukoencephalopathy, Bevacizumab-associated late radiation-induced neurotoxicity, and Ipilimumab-induced hypophysitis. Lastly, radiation-induced neurotoxicities are covered, including myelopathy, radiation necrosis, cerebral atrophy, leukoencephalopathy, optic neuropathy, mineralizing microangiopathy, stroke-like migraine attacks, osteonecrosis, and vasculopathies. Neuroradiologists will increasingly encounter patients who have undergone treatment with more than 1 therapeutic modality, resulting in overlapping findings as well. Recognition of the common complications of these therapies on imaging is critical to minimizing the effects of these potential short- and long-term complications.

Head and Neck Sarcoma Tumor Board Survival Guide for Neuroradiologists: Imaging Findings, History, and Pathology.

Sarcomas of the head and neck carry a poor prognosis as diagnosis is often delayed until a late stage of the disease. Accordingly, it is essential to be familiar with the clinical and imaging features of sarcomas to suggest an appropriate differential diagnosis for collaborating surgeons and pathologists. However, as there are only 1000-1500 cases in the United States annually, many radiologists lack experience with pertinent imaging findings of sarcoma and lack knowledge of both treatment and necessary follow-up. In this review, a brief discussion of WHO definitions and histopathology is included to decode information provided by pathologists. Finally, staging and treatments are illuminated to aid the radiologist with initial imaging staging and follow-up care. This review aims to increase the comprehensive knowledge of a neuroradiologist and further their value to the multidisciplinary tumor board.

External Ear Diseases: A Comprehensive Review of the Pathologies With Neuroradiological Considerations.

The peripheral auditory system is subdivided into 3 compartments: the external, middle, and inner ear. Historically, the middle and inner ear have garnered more attention in the imaging literature, due to their intricate anatomy and complexity of pathologies. The external ear, however, has attained less recognition given its relatively straightforward anatomy and convenience of direct visual examination. The continued advancement in computed tomography and magnetic resonance imaging has expanded the role of radiology in the evaluation of the external ear lesions. The purpose of this article is to offer a comprehensive review of external ear pathologies, including congenital, inflammatory, infectious, traumatic, neoplastic, and rare disease entities and their imaging findings.

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.

Calvarial Langerhans cell histiocytosis in an Adult: Typical imaging findings in an atypical age group.

Langerhans Cell Histiocytosis (LCH) is a rare disorder characterized by neoplastic proliferation of Langerhans-type dendritic cells. LCH is most frequently encountered in the pediatric populations, and involvement of the skeletal system is a common manifestation. Herein, we report a case of LCH presented as an isolated skull lesion in a 66-year-old patient. This presentation has never been reported in the literature at this advanced age and suggests that, despite being exceptionally rare, clinicians should consider LCH in the differential diagnosis of skull lesions in the elderly with classical radiological appearance.