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NERRS Jan 2014 Pediatric Radiology Case Answers

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NERRS Jan 2014 Pediatric Radiology Case Answers

  1. 1. NERRS Jan 2014 Pediatric Radiology Unknown Cases Kirsten Ecklund, M.D. Clinical Vice Chair of Radiology Division Chief Musculoskeletal Imaging Boston Children’s Hospital Assistant Professor of Radiology Harvard Medical School, Boston, MA
  2. 2. Case 1: Diego Jaramillo
  3. 3. 7 y.o. boy with fatigue, knee pain and swelling
  4. 4. Additional images
  5. 5. DDX Multifocal Marrow Abnormalities • Neoplasm: Leukemia, metastatic dz (NB, rhabdomyosarcoma) • Infiltration: Gaucher’s, storage dz • Reactive: severe anemias (red marrow) • Infection/Inflammation: multifocal osteomyelitis, CRMO • Metabolic disease: Anorexia nervosa, nutritional deficiency
  6. 6. Scurvy • Vitamin C deficiency – Vit C facilitates hydroxylation of lysine, proline required for crosslinking collagen – Deficiency impairs enchondral ossification • Etiologies – Nutritional deficiency – Intestinal malabsorption – Over utilization (thyrotoxicosis) • Symptoms – Lethargy, fever, malaise – MSK sxs (80%) include bruising, joint pain
  7. 7. Scurvy • Xray findings (late) – Prominent ZPC (white lines of Frankel) – Subjacent lucent bands (scurvy lines) – Physeal widening – Epiphyseal (Pelkan) spurs – Metaphyseal fractures • MR findings are non-specific – Metaphyseal marrow changes – Periosseous soft tissue edema – Subperiosteal fluid/hematoma (late) • 3 very similar reported cases in last 3 years Skel Rad (2012) 41: 357-360
  8. 8. Scurvy • Intramedullary and subperiosteal hemorrhage • Gelatinous transformation of marrow • Our patient – Marrow bx – fibrosis, hemorrhage – Restricted diet: chicken nuggets, water, packaged cookies – Further eval: autism spectrum diagnosis • 2 new cases in last 3 months – Incidence of autism is increasing
  9. 9. Case 2 : Cicero Silva
  10. 10. 15 y.o. African American male with cough, shortness of breath, 10 # weight loss
  11. 11. Additional images
  12. 12. Findings • CXR – interstitial, septal thickening • Chest CT – septal thickening, multiple small nodules, ground glass opacities • Abdominal CT – infiltrative, indistinct left renal mass
  13. 13. Pulmonary lymphangitic metastases • Tumors: typically adenocarcinoma – Breast, lung, colon, thyroid, pancreas • CT Findings – Interlobular septal thickening – Nodules or reticulonodular changes – Ground glass opacities • DDX – Sarcoidosis – Idiopathic pulmonary fibrosis – Pulmonary edema
  14. 14. Metastatic renal medullary carcinoma • Demographics: – Seen almost exclusively in pts with sickle trait – Age range = 11 – 39, Male: female=3:1 • Symptoms: pain, hematuria, wt loss • Renal mass imaging features – Infiltrative, associated necrosis, caliectasis – More commonly right-sided • Often widely metastatic at dx – Pulm lymph spread is common
  15. 15. Case 3: Ilse Castro-Aragon
  16. 16. 8 y.o. boy with abdominal pain, distention and vomiting
  17. 17. Findings/ DDX • SBO due to distal ileal stricture with circumferential hyperemic soft tissue • DDX – Inflammation • IBD • Infection • Focal inflammatory/post inflammatory process – Neoplasm • Lymphoma • GIST • Sarcoma – Developmental • Omphalomesenteric duct remnant • volvulus
  18. 18. Inflammatory Myofibroblastic Tumor • Synonyms: inflammatory pseudotumor, plasma cell granuloma, pseudosarcoma • Sites: Lung, orbit most common, but anywhere • Etiology: ?, likely reactive • Path: No malignant features, spindle cells, mononuclear inflammatory cells • Symptoms: depend upon site – Abdomen: pain, fever most common • RX: surgical resection, chemo? • Prognosis – Good for local, resectable dz – Poor for infiltrative dz, esp abdominal, locally recurrent
  19. 19. IMT: imaging features • Dependent upon anatomic site • Typical: low density, +/- calc, focal mass vs infiltrative soft tissue • Bowel lesions: diffuse, infiltrative wall thickening • Overall, somewhat nonspecific
  20. 20. Case 3: Michele Walters
  21. 21. 11 y.o. boy with 6 months of bilateral knee pain, right > left
  22. 22. Additional images
  23. 23. Findings • Benign appearing tibial metaphyseal lesion – Vascular (enhancement) – No marrow edema or soft tissue mass – Cortically based, sclerotic margins • Systemic physeal widening – Faint ZPC – Metaphyseal fraying, osteopenia
  24. 24. Phosphaturic mesenchymal tumor (PMT) with oncogenic osteomalacia • Benign mesenchymal neoplasm • Secretes phosphatonin (FGF 23) – Inhibits renal phosphate reabsorption and conversion of 25-hydroxyvitamin D > 1,25 dihydroxyvitamin D – Result is osteomalacia (rickets)- paraneoplastic syndrome • Tumor locations: extremities, facial bones • Mesenchymal tumor types associated with OO: – PMT, mixed connective tissue type • Hemangiopericytoma – Osteoblastoma-like tumors – Ossifying fibroma-like tumors – Non-ossifying fibroma-like tumors
  25. 25. PMT with osteomalacia • Labs – low serum phosphate, normal calcium – high 25-hydroxyvitamin D, low 1,25 dihydroxyvitamin D – elevated FGF 23 • Imaging: Whole Body MR for occult lesions • RX: surgical resection (FGF levels normalize in 1wk)

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