Transcript Case 1

LIRS Case Presentation
8 April 2014
44 y/o female with acute right flank pain; stone hunt
non-contrast CT ordered.
Submitted by Patrick H. Kobes, DO PGY-2
T1 C+: early arterial
T1 C+: 20 minute delay
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Common Benign Lesion of the Liver
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Demographic: Young woman taking OCPs
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Seen in males using anabolic steriods, Glycogen
storage disease (Von Gierke type 1a)
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Adenonomatosis (>10 Adenomas)
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Can be mistaken with HCC (lipid containing)
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Malignant transformation (HCC) in lesions > 10 cm
Imaging characteristics
CT: Heterogenous, Iso- to hypodense well defined lesion with early enhancement
Areas of High Attenuation: Hemorrhage
MR T1WI: Hyperintense signal (lipid/blood containing),
T2WI: Hypointense signal( recent hemorrhage),
Hyperintense signal (old hemorrhage, necrosis)
MR T1WI C+: Heterogenous enhancement
Delayed phase imaging: Hypointense to liver
Opposed phase GRE:
Inphase: Isointense
Out of phase: will show drop out (lipid containing)
Treatment:
Discontinue OCPs: resolution or cessation.
Avoid Pregnancy: Increased risk of rupture
Size:
Adenoma < 6 cm: observation
Adenoma > 6 cm, subcapsular, symptomatic:
*Surgical resection*
4 month old female presents with noisy
breathing.
Submitted by Erin McLaughlin, MD PGY-2
MIBG Scan and corresponding coronal CT
NEUROBLASTOMA
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Most common solid extra cranial tumor in
children and infants.
Third most common malignant neoplasm in
children.
The present anywhere sympathetic tissue is
located
14% present as a posterior mediastinal mass
(arising from the paravertebral sympathetic
chain)
Composed of neuroepithelial cell that show
glial or ganglionic differentiation.
More than half of all neuroblastomas
present before the age of 2
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CT is most commonly used for diagnosis
and staging.
 Characteristics: Necrosis, hemorrhage, coarse amorphous
calcifications, vascular involvement.
 In thoracic NBs look for neural foraminal widening.
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MRI is best used to evaluate for
involvement of the spinal cord.
Common metastatic patterns:
 Bone is most common. Liver, dura, lung, brain.
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MIBG may used to confirm diagnosis.
(But only about 70% will take up MIBG).
 Also used for metastatic surveillance
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David R, Lamki N, Fan S, Singleton EB, Eftekhari F, Shirkhonda A, Kumar R,
Madwell JE. The many faces of Neuroblastoma. Radiographics 9 (5): 85982.
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Lonergan GJ, Schwab CM, Suarez ES et-al. Neuroblastoma,
ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic
correlation. Radiographics. 22 (4): 911-34.
2 year-old male with left submandibular mass
and left posterior ear swelling x 1 week, not
improved on antibiotics. Also, with 1 day of
refusal to bear weight on left leg.
Submitted by Pnina Herskovits, MD PGY-2
Soft Tissue
Masses of
Head and Neck
with Cranial
Extension
Thymic Infiltration
Porta Hepatis Mass
Lytic Osseous
Lesions
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Annual Incidence: 2.6-5.4 / million children
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Presumed Pathophysiology: Uncontrolled immune
response wherein Langerhans cells (type of dendritic antigen
presenting cell) proliferate in response to an unknown
antigen. Cell proliferation is accompanied by inflammation
and formation of granulomas. Process can infiltrate any
tissue.
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Definitive Diagnosis: Biopsy
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Single System vs. Multisystem Disease
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Osseous involvement is present in the majority of patients
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Extraosseous Involvement:
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Skin 55%
CNS 35%
Hepatobiliary and Spleen 32%
Lungs 26%
Lymph Nodes 26%
Soft Tissues 26%
Bone Marrow 19%
Salivary Glands 6%
Digestive Tracts 6%
(% amongst 31 children with extraosseous LCH)
Shmidt, S et al. Extraosseous involvement of Langerhans’ cell histiocyotsis. Pediatr Radiol 2004.
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Treatment
 Multiagent chemotherapy and
immunosuppressants
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Prognosis
 High recurrence rate
 Low mortality rate
 “Risk organs” include lung, liver, spleen, and bone
marrow. Involvement of these organs is
associated with increased mortality rate
1. Jubran, R et al. Predictors of outcome in children with Langerhans cell
histiocytosis. Pediatr Blood Cancer 2005; 45: 37-42.
2. Schmidt S et al. Extraosseous involvement of Langerhans’ cell histiocyotsis.
Pediatr Radiol 2004; 34: 314- 32
3. Schmidt S et al. Extraosseous Langerhans cell histiocytosis in children.
Radiographics 2008; 28: 707-726.
4. Stalemark H, Laurencikas E, Karis J et al. Incidence of Langerhans cell
histiocytosis in children: A population- based study. Pediatr Blood Cancer. 2008;
51:76-81.
66M with history of petrous bone fracture (6
months ago), presents with fever, left ear
fullness and change in hearing.
Submitted by Elitsa Clark, MD PGY-2
CT Cisternography:
Coronal slice through the left mastoid
region
Axial image showing the left mastoid
defect.
Axial thin-section post-contrast
SPGR
Axial T2 weighted image.
Left decubitus scan (rotated into
standard orientation).
Positioning often done multiple
times to reposition subarachnoid
Axial C+ SPRG image
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An encephalocele is a protrusion of brain
tissue through a defect in the calvarium
Most encephaloceles are congenital.
 Failure of neural tube fusion.
 A smaller percentage are iatrogenic
▪ Postoperative or Post-traumatic
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Possible associated findings
 CSF leak +/- intracranial hypotension, meningitis,
epidural abscess, pneumocephalus
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Indium DTPA (diethylene triamine penta-acetic acid)
injected intrathecally
 Requires more time
 More sensitive in evaluating slow leaks
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CT or MR cisternogram
 Quicker, often challenging to redistribute contrast
throughout subarachnoid space
 Better spatial resolution- CT often needed for presurgical planning to repair the cranial defect.
50F with mutiple papular skin lesions on face
and upper torso.
Submitted by Peter Lee, MD PGY-2
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Langerhan Cell Histiocytosis
Lymphangioleiomyomatosis
Birt-Hogg-Dube Syndrome
Pneumatoceles
Pneumocystis pneumonia
Lymphocytic interstitial pneumonitis
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Rare, autosomal dominant, multisystem disorder.
Mutation in FLCN gene.
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Clinical findings:
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Pulmonary cysts, skin fibrofolliculomas, and renal tumors.
Pulmonary cysts (70-80%)
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Lower lung predominance.
Variable size and shape.
No additional parenchymal abnormalities.
Recurrent spontaneous pneumothoraces (25%).
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Skin fibrofolliculomas
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Hair follicle hamartomas on face and upper torso.
Arise during 3rd-4th decade of life.
Renal tumors (15-30%)
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Renal cell carcinoma (chromophobe or clear cell),
renal oncocytoma.
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Often multiple and bilateral.
Age of onset 25-75.
Angiomyolipoma- (as in this case)
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Agarwal, P. et al. Thoracic CT Findings in Birt-HoggDube Syndrome. AJR 2011; 196: 349-352.
Ayo, D. et al. Cystic Lung Disease in Birt-Hogg-Dube
Syndrome. Chest 2007; 132: 679-684.
Choyke, P. et al. Hereditary Renal Cancers.
Radiology 2003; 226: 33-46.
Menko F. et al. Birt-Hogg-Dube Syndrome:
Diagnosis and Management. Lancet Oncology 2009;
10: 1199-206.