North Shore – LIJ Health SystemHofstra University School of Medicine

LIRS Case Presentation8 April 2014

Case 1

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

Hepatic Adenoma - Ruptured

Common Benign Lesion of the Liver

Demographic: Young woman taking OCPs

Seen in males using anabolic steriods, Glycogen

storage disease (Von Gierke type 1a)

Adenonomatosis (>10 Adenomas)

Can be mistaken with HCC (lipid containing)

Malignant transformation (HCC) in lesions > 10

cm

Hepatic Adenoma

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)

Hepatic Adenoma ruptureTreatment:

Discontinue OCPs: resolution or cessation.

Avoid Pregnancy: Increased risk of rupture

Size:Adenoma < 6 cm: observationAdenoma > 6 cm, subcapsular,

symptomatic: *Surgical resection*

Case 2

4 month old female presents with noisy breathing.

Submitted by Erin McLaughlin, MD PGY-2

MIBG Scan and corresponding coronal CT

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

NEUROBLASTOMA

NEUROBLASTOMA IMAGING

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.

MRI is best used to evaluate for involvement of the spinal cord.

Common metastatic patterns: Bone is most common. Liver, dura, lung, brain.

MIBG may used to confirm diagnosis. (But only about 70% will take up MIBG). Also used for metastatic surveillance

REFERENCES

David R, Lamki N, Fan S, Singleton EB, Eftekhari F, Shirkhonda A, Kumar R, Madwell JE. The many faces of Neuroblastoma. Radiographics 9 (5): 859-82.

Lonergan GJ, Schwab CM, Suarez ES et-al. Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. Radiographics. 22 (4): 911-34.

Case 32 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

Langerhans Cell Histiocytosis

Annual Incidence: 2.6-5.4 / million children 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.

Definitive Diagnosis: Biopsy

Langerhans Cell Histiocytosis Single System vs. Multisystem Disease

Osseous involvement is present in the majority of patients

Extraosseous Involvement: 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.

Langerhans Cell Histiocytosis

Treatment Multiagent chemotherapy and

immunosuppressants 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

References

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.

Case 466M 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.

Clinical concern for CSF leak

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 contrast.

Axial C+ SPRG image

Encephalocele

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 Possible associated findings

CSF leak +/- intracranial hypotension, meningitis, epidural abscess, pneumocephalus

Imaging tests to evaluate for CSF leak

Indium DTPA (diethylene triamine penta-acetic

acid) injected intrathecally Requires more time More sensitive in evaluating slow leaks

CT or MR cisternogram Quicker, often challenging to redistribute

contrast throughout subarachnoid space Better spatial resolution- CT often needed for

pre-surgical planning to repair the cranial defect.

Case 5

50F with mutiple papular skin lesions on face and upper torso.

Submitted by Peter Lee, MD PGY-2

Differential Diagnosis

Langerhan Cell Histiocytosis Lymphangioleiomyomatosis Birt-Hogg-Dube Syndrome Pneumatoceles Pneumocystis pneumonia Lymphocytic interstitial pneumonitis

Birt-Hogg-Dube Syndrome

Rare, autosomal dominant, multisystem disorder.

Mutation in FLCN gene.

Clinical findings: Pulmonary cysts, skin fibrofolliculomas, and

renal tumors.

Pulmonary cysts (70-80%) Lower lung predominance. Variable size and shape. No additional parenchymal abnormalities. Recurrent spontaneous pneumothoraces

(25%).

Birt-Hogg-Dube Syndrome Skin fibrofolliculomas

Hair follicle hamartomas on face and upper torso.

Arise during 3rd-4th decade of life.

Renal tumors (15-30%) Renal cell carcinoma (chromophobe or

clear cell), renal oncocytoma. Often multiple and bilateral. Age of onset 25-75.

Angiomyolipoma- (as in this case)

References

Agarwal, P. et al. Thoracic CT Findings in Birt-Hogg-Dube 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.