1) This document contains information about 4 pediatric radiology cases presented by Dr. Kirsten Ecklund from Boston Children's Hospital. 2) The first case discusses a 7-year-old boy with fatigue and knee pain who is diagnosed with scurvy, a vitamin C deficiency. 3) The second case involves a 15-year-old boy with cough and weight loss found to have metastatic renal medullary carcinoma to the lungs. 4) The third case is about an 8-year-old boy with abdominal pain found to have an inflammatory myofibroblastic tumor causing small bowel obstruction. 5) The fourth case discusses an 11-year-old boy

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. Case 1: Diego Jaramillo

3. 7 y.o. boy with fatigue, knee pain and swelling

4. Additional images

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. 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. 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. 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. Case 2 : Cicero Silva

10. 15 y.o. African American male with cough, shortness of breath, 10 # weight loss

12. Additional images

13. Findings
• CXR – interstitial, septal
thickening
• Chest CT – septal
thickening, multiple small
nodules, ground glass opacities
• Abdominal CT –
infiltrative, indistinct left renal
mass

14. 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

15. 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

16. Case 3: Ilse Castro-Aragon

17. 8 y.o. boy with abdominal
pain, distention and vomiting

18. 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

19. 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

20. 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

21. Case 3: Michele Walters

22. 11 y.o. boy with 6 months of bilateral knee pain, right > left

23. Additional images

24. 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

25. 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

26. 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)