Noonan syndrome (NS) is an autosomal dominant developmental disorder caused by mutations in the RAS-MAPK signaling pathway that is well known for its relationship with oncogenesis. An 8.1-fold increased risk of cancer in Noonan syndrome has been reported, including childhood leukemia and solid tumors. The same study found a patient with a dysembryoplastic neuroepithelial tumor (DNET) and suggested that DNET tumors are associated with NS. Herein we report an 8-year-old boy with genetically confirmed NS and a DNET. Literature review identified eight other reports, supporting the association between NS and DNETs. The review also ascertained 13 non-DNET brain tumors in individuals with NS, bringing to 22 the total number of NS patients with brain tumors. Tumor growth while receiving growth hormone (GH) occurred in our patient and one other patient. It is unknown whether the development or progression of tumors is augmented by GH therapy, however there is concern based on epidemiological, animal and in vitro studies. This issue was addressed in a 2015 Pediatric Endocrine Society report noting there is not enough data available to assess the safety of GH therapy in children with neoplasia-predisposition syndromes. The authors recommend that GH use in children with such disorders, including NS, be undertaken with appropriate surveillance for malignancies. Our case report and literature review underscore the association of NS with CNS tumors, particularly DNET, and call attention to the recommendation that clinicians treating NS patients with GH do so with awareness of the possibility of increased neoplasia risk.

Objective:To describe the imaging findings of patients treated with subchondroplasty (SCP) of the ankle and hindfoot. Materials and Methods: Eighteen patients (10 men, 8 women; age mean 43.1 years [range 20.1-67.7 years]) underwent ankle and hindfoot SCP at a single center over a 14-month period. Imaging data were reviewed retrospectively by 2 radiologists by consensus interpretation, including preoperative radiography (18), computed tomography (CT) (11), and magnetic resonance imaging (MRI) (13) and postoperative radiography (10), CT (4), and MRI (6). Follow-up imaging was acquired 1 month to 1.6 years following SCP. Results: Indications for SCP included symptomatic bone marrow lesions (BMLs) secondary to an osteochondral lesion (OCL) (16/18) or stress fracture (2/18). While focal radiodensity related to the SCP procedure was retrospectively identifiable on postoperative radiography in all except 1 case (10/11), postprocedural findings were not described by the interpreting radiologist in 6/11 cases. On CT, the average injected synthetic calcium phosphate (CaP) volume was 1.15 cm3 (SD = 0.33 cm3); mean CT attenuation of the injectate was 1220 HU (range 1058-1465 HU). In all patients who had pre- and postoperative MRI (5/18), BML size decreased on follow-up MRI. Extra-osseous extrusion of CaP was not seen on postoperative radiography, CT, or MRI. Conclusion: Physicians should be aware of the expanding preoperative indications and postoperative imaging findings of SCP, which is being performed with increasing frequency in the ankle and hindfoot. Levels of Evidence: Diagnostic, Level III: Retrospective cohort study

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