A 30-year-old man presented with a fairly large intramedullary mass lesion involving virtually the entire spinal cord. It was hyperintense on both T1W and T2W sequences, with signal suppression on fat-saturation images. Subsequent noncontrast CT scan of the spine confirmed the presence of fat and calcification within the lesion, thus leading to the diagnosis of an intramedullary dermoid.
A 36-year-old man presented with headache and right upper and lower limb weakness for 10 days. MRI revealed absence of the odontoid process of the C2 vertebral body, with resultant atlantoaxial dislocation along with myelomalacic changes involving the cervicomedullary junction.
Background
Diffuse intrinsic pontine glioma (DIPG) is a uniformly fatal brain tumor with no available cure. Indoximod blocks the IDO (indoleamine 2,3-dioxygenase) pathway, thereby reversing IDO-mediated immune suppression in the tumor microenvironment.
Methods
Patients aged 3 to 21 years with treatment-naive DIPG were eligible for this phase 1b dose-confirmation study of indoximod. The treatment regimen comprised continuous oral indoximod (38.4 mg/kg/day divided twice daily) with conformal photon radiation (54 Gy in 30 fractions), followed by cycles of indoximod with temozolomide (200 mg/m2/day, days 1–5 in 28-day cycles).
Results
Thirteen patients (median age 9 years, range 5 to 20 years) with DIPG were treated. Median OS was 14.5 months (follow-up ranged 4.8 to 29.3 months), 12-month OS was 61.5% (8/13), and 18-month OS was 30.8% (4/13), with 1 patient remaining in follow-up at the data cutoff. This compared favorably to expected median OS of approximately 10.8 months, 12-month OS of 45.3%, and 18-month OS of 16.2% taken from published historical data from the Pediatric Brain Tumor Consortium. Two patients showed near-complete responses lasting until relapsing after 7.6 months and 13.3 months of study therapy, respectively. Many patients had increased circulating non-classical monocytes (nc-Monos, CD16+, CD14neg, CD33+, HLA-DR+) within the first 3 treatment cycles, and elevation of this early pharmacodynamic marker was predictive of subsequent OS. Patients with nc-Monos >10% (n=7) had median OS of 19 months, whereas patients with nc-Monos below 10% (n=5) had median OS of 7 months (p=0.0047). No patients stopped therapy for toxicity. The most common indoximod-attributed adverse events were thrombocytopenia, neutropenia, nausea, vomiting, dizziness, and fatigue.
Conclusions
Adding indoximod immunotherapy to conventional radiation and chemotherapy for front-line treatment of pediatric patients with DIPG was well-tolerated. Improved outcomes were observed in patients having evidence of pharmacodynamic response. A follow-on phase 2 study is in progress (NCT04049669).
A 25-year old man presented with a mediastinal lesion which was initially diagnosed as a loculated pericardial collection on echocardiography. Subsequent imaging showed it to be a cystic mediastinal mass, and following surgery and histopathology, it turned out to be an epidermoid cyst.
Introduction: Sickle cell disease (SCD) is a genetic disorder that affects 1 in 600 black infants in the United States. The painful crisis is one of its most characteristic manifestations and consists of pain in the extremities, back, abdomen, or chest. The differential diagnosis for acute back pain in SCD patients includes occlusive crisis, osteomyelitis, fracture, vertebral collapse or bone marrow necrosis. The magnetic resonance imaging (MRI) is the investigation of choice in delineating the cause but very rarely it can be falsely negative. Here we present one such case where despite negative findings on the MRI, the patient was found to have osteomyelitis on biopsy. Case Description: Fourteen years old male with SCD presented with acute back pain radiating up from lumbar spine. He had point tenderness at the Thoracic region of T9-10 and was afebrile with elevated Erythrocyte Sedimentation Rate (ESR) and C-Reactive Protein (CRP). The MRI of the spine showed combination of old bone infarcts and bone marrow hematopoietic recruitment throughout thoracic segments. It was also noted to have chronic appearing compression deformities with typical fish mouth deformity of the vertebra, more prominent at T9 and T10 (figure 2). Prevertebral enhancing soft tissue at T10-T11 (figure 1) was believed to represent hematopoiesis without definite evidence of osteomyelitis. With high suspicion of osteomyelitis, the team obtained biopsy of the T10 vertebral body of the spine which showed the growth of methicillin sensitive staphylococcus aureus (MSSA). The patient underwent treatment with intravenous clindamycin for six weeks which significantly improved his back pain. Discussion: Osteomyelitis has similar presentation to pain crisis making it difficult to diagnose. Fever, chills and lower back pain are seen in both conditions. Laboratory tests routinely done are non-specific in diagnosing osteomyelitis. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) may be helpful in differentiating osteomyelitis from pain crisis. The MRI is the modality of choice with sensitivity of 82-100 percent in diagnosing the osteomyelitis. Although very rare, there have been cases of false negative findings on MRI. One such case led to below knee amputation of the limb with surgical pathology confirming the diagnosis. The present case prompts us to be aware of the rare false negative findings on MRI. Therefore, with lack of improvement of symptoms or high clinical suspicion, it is imperative to confirm the finding with a biopsy to effectively diagnose and treat the back pain in SCD patient.
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