Radiological Category: Chest Radiology Principal Modality (1): Radiograph

Radiological Category: Chest Radiology Principal Modality (1): Radiograph

Radiological Category: Chest Radiology Principal Modality (1): Radiograph Principal Modality (2): MRI, CT, MIBG Case Report # 0887 Submitted by: Cantrell, Sarah M.D. Faculty reviewer: Ferguson, Emma M.D. Date accepted: 15 March 2012

Acknowledgements: Thank you to Dr. Michael Redwine, Dr. Susan Greenfield and Dr Caleb Richards for providing images Thank you to Dr. Brian Stewart, UT Department of Pathology, for providing histologic evaluation and images Case History 1 month-old with tachypnea and wheezing Radiological Presentations A (A) PA and (B) lateral chest radiographs B Radiological Presentations

A B C Axial CT of thorax in (A) lung, (B) mediastinal and (C) bone windows Radiological Presentations A B C Sagittal CT of thorax in (A) lung, (B) mediastinal and (C) bone windows

Radiological Presentations A B C (A)T1, (B)T1 post contrast and (C) T2 axial MRI of thorax Test Your Diagnosis Which one of the following is your choice for the appropriate diagnosis? After your selection, go to next page. Round pneumonia Bronchogenic cyst Extralobar sequestration CPAM

Neuroblastoma Findings PA and lateral radiographs demonstrate a well defined right sided extraparenchymal posterior chest mass without air bronchograms. Findings The lateral radiograph shows a well defined extraparenchymal mass in the posterior chest without air bronchograms. The mass demonstrates a cat under the rug appearance. The mass forms obtuse angles with the chest wall, suggesting an extraparenchymal location.

Findings A B C Contrast enhanced axial CT images demonstrate a well-defined, extraparenchymal mass in the posterior right hemithorax of low attenuation measuring 50 Hounsfield units without definite internal enhancement or extrathoracic extension. Findings A

B C Sagittal contrast enhanced CT images confirm the extraparenchymal location of the mass in the posterior chest, which demonstrates low attenuation measuring 50 Hounsfield units without definite internal enhancement or extrathoracic extension. Findings A B C (A) Axial T1 MR image shows a well circumscribed, extraparenchymal mass

with heterogenously low T1 signal in the right posteroinferior hemithorax (B) Axial T1 MR postcontrast image reveals heterogenous enhancement (C) Axial T2 MR image shows scattered foci of T2 hyperintensity Differential Diagnosis Differential diagnosis for posterior chest/mediastinal mass in a child <3 years old includes: Round pneumonia Bronchogenic cyst Extralobar sequestration CPAM Neuroblastoma Discussion Neuroblastoma Definition: Malignant tumor of neural crest cells

Demographics: Children <2 years Location Adrenal neuroblastoma is most common, though neuroblastoma may occur anywhere along the sympathetic chain Thoracic neuroblastoma involves the sympathetic chain in the posterior mediastinum Invasive: Neuroblastoma characteristically invades neural foramina to spread to the spinal canal where it encases vessels and nerves Discussion Neuroblastoma Radiograph: Solid well circumscribed posterior mediastinal mass without air bronchograms CT:

Calcification common Heterogenous appearance with hemorrhage, necrosis MRI: Useful for detecting invasion of neural foramina and spinal canal Heterogenous enhancement TWI1: low signal intensity T2WI: high signal intensity Nuclear MIBG: 70% of neuroblastomas produce catecholamines and are MIBG avid Useful for surveillance and evaluation of progression Discussion Neuroblastoma Clinical course Patient went to surgery at age 4 weeks and underwent resection of the right chest mass Postsurgical pathology showed Poorly differentiated neuroblastoma

Positive margins Vascular invasion Intermediate mitosis karyorrhexis index No n-myc amplification Discussion: poorly differentiated neuroblastoma Homer Wright Rosette Characteristic of sympathetic nervous system tumors including Central neuropil neuroblastomas and medulloblastomas Composed of halo-like clusters of cells in each rosette surrounding a central area of fiber-rich neuropil Halo-like cluster of cells Surgical pathology status post excision of right

chest mass demonstrates a neuroblastoma with favorable histology indicated by age <1.5 years, low to intermediate MKI (<196/ 5000 cells) and poorly differentiated to dedifferentiated histology. Thank you to Dr Brian Stewart, pathology, for providing slides and images Discussion Neuroblastoma Prior to surgery, urine HVA (homovanillic acid, a catecholamine produced by neuroblastoma) was elevated at 99.0 mg/g Cr (normal <35 mg/g Cr), indicating that urine HVA and MIBG would provide an effective method for surveillance Coronal whole-body images from MIBG tumor localization performed 2 weeks following surgery demonstrate:

No focal increased activity in the adrenal bed or midline Physiologic activity in the myocardium Discussion Neuroblastoma Prior to surgery, urine HVA (homovanillic acid, a catecholamine produced by neuroblastoma) was elevated at 99.0 mg/g Cr (normal <35 mg/g Cr), indicating that urine HVA and MIBG would provide an effective method for surveillance Sagittal whole-body images from MIBG tumor localization performed 2 weeks following surgery demonstrate: No focal increased activity in the adrenal bed or midline Physiologic activity myocardium Saggital T2

Discussion Neuroblastoma T2WI demonstrates increased signal within right 6-8th intercostal spaces Saggital T1 post Postcontrast T1WI demonstrates increased enhancement within right 6-8th intercostal spaces However, 8 weeks following

surgery HVA began to slowly rise HVA increased from 28 mg/g Cr postop to 52 mg/g at 16 weeks following resection MRI was performed and demonstrated small foci of residual tumor along the posterior 6th through 8th intercostal spaces Differential Diagnosis Differential diagnosis for posterior chest/mediastinal mass in a child <3 years old includes: Round pneumonia Bronchogenic cyst Extralobar sequestration CPAM

Neuroblastoma Discussion Round pneumonia Children <8 years Signs/symptoms of pneumonia (fever, cough, tachypnea) Location: most common, superior segment of lower lobes Radiography: Air bronchograms on radiograph Conform to lobar anatomy Acute angles with chest wall Bilateral round pneumonias: Opacities are intraparenchymal as evidenced by:

Acute angles with chest wall Completely surrounded by aerated lung parenchyma CT: CT usually not performed Enhancing vessels may course through parenchymal opacity, however, fluid filled alveoli will not demonstrate enhancement Discussion Round Pneumonia v. Neuroblastoma Round pneumonia Radiograph: Intraparenchymal opacity with air bronchograms

CT: Intraparenchymal opacity which may contain enhancing vessels coursing through it Neuroblastoma Radiograph: extraparenchymal opacity without air bronchograms CT Extraparenchymal opacity that displaces vessels and parenchyma Heterogenous content, may contain calcium and necrosis May demonstrate internal enhancement May demonstrate bony destruction

Discussion Bronchogenic Cyst Foregut duplication cysts Other foregut duplication cysts include neuroenteric and enteric cysts Result from abnormal ventral budding of the tracheobronchial tree between 26th-40th weeks of gestation

Location: middle mediastinum, subcarinal most common Mediastinal (majority)-middle mediastinal (hilar, subcarinal) Intraparenchymal Usually do not communicate with airway. Presence of air suggests infection. Thank you to Dr Susan Greenfield for providing Discussion Bronchogenic cyst v. Neuroblastoma Bronchogenic cyst

Neuroblastoma Middle mediastinum Posterior mediastinum CT CT Heterogenous content, may contain calcium and necrosis May demonstrate internal enhancement

May demonstrate bony destruction MRI Heterogenous content and enhancement T1WI: low signal intensity T2WI: high signal intensity Homogenous internal content May contain proteinaceous fluid but 50% have uniform fluid density No internal enhancement MRI TWI1: uniform low T1 signal T2WI: uniform high T2 signal No internal enhancement

Discussion Pulmonary Sequestration Abnormal lung parenchyma which does not communicate with the tracheobronchial tree Arterial supply from systemic arteries (descending thoracic aorta, abdominal aorta) Most commonly lower lobe Extralobar (10%) Intralobar (90%) Early childhood presentation Late childhood presentation

Systemic venous drainage (azygous, IVC) Drains to inferior pulmonary vein Separate pleural covering Other associated congenital lesions (CPAM) Visceral pleural covering No other associated congenital lesions

Thank you to Dr Susan Greenfield for providing Discussion: Pulmonary Sequestration v. Neuroblastoma Pulmonary sequestrum Neuroblastoma CT Systemic arterial supply, from thoracic aorta or abdominal aorta Calcium uncommon May contain heterogenous cystic/

air filled spaces if extralobar sequestration and CPAM are concurrent No bony destruction CT Heterogenous content, may contain hemorrhage and necrosis Calcium common May demonstrate internal enhancement May demonstrate bony destruction Discussion Congenital Pulmonary Airway Malformation

Multiple air filled cysts within the left hemithorax which shift the mediastinum rightward and exert mass effect on the left upper lobe. Dysplastic lung tissue with various degrees of cystic change Radiograph: Type I, most common Unilateral lucency with thin-walled cysts Expansile, may compress/shift adjacent lobes/mediastinum

CT Multiple cysts which may be fluid or air filled with thin or thick septa May displace adjacent structures Arterial/venous supply from pulmonary artery and vein Thank you to Dr. Caleb Richards for providing images Discussion Congenital Pulmonary Airway Malformation Axial T1 Coronal T1

Coronal single shot T2 Coronal single shot T2 Axial and coronal T1WI, axial and coronal single shot T2WI demonstrate multiple air filled cysts with minimal fluid within the left lower lobe which displace the mediastinum rightward and exert mass effect on the left upper lobe. Thank you to Dr Caleb Richards for providing Discussion Congenital Pulmonary Airway Malformation Discussion

CPAM v. Neuroblastoma CPAM Neuroblastoma Radiograph: intraparenchymal opacity with multiple air filled cysts Radiograph: solid extraparenchymal lesion CT CT

Solid lesion Multiple thin walled cysts with air or fluid -Calcium uncommon No bony destruction Heterogenous content, may contain hemorrhage and necrosis Calcium common May demonstrate bony destruction References Biyyam DR, Chapman T, Ferguson MR, Deutsch G, Dighe MK. Congenital lung abnormalities: embryologic features, prenatal diagnosis and postnatal radiologic pathologic correlation. Radiographics. 2010 Oct;30(6):1721-38. Berrocal T, Madrid C, Novo S, Gutirrez J, Arjonilla A, Gmez-Len N. Congenital

anomalies of the tracheobronchial tree, lung, aand mediastinum: embryology, radiology and pathology. Radiographics. 2004 Jan-Feb;24(1):e17. Epub 2003 Nov 10. David R, Lamki N, Fan S, Singleton EB, Eftekhari F, Shirkhoda A, Kumar R, Madewell JE. The many faces of neuroblastoma. Radiographics. 1989 Sep;9(5):859-82. Donnelly LF: Fundamentals of Pediatric Radiology. Phildelphia W.B. Saunders, 2001. Fitch SJ, Tonkin IL, Tonkin AK. Imaging of foregut duplication cysts. Radiographics. 1986 Mar;6(2):189-201. Jeung MY, Gasser B, Gangi A, Bogorin A, Charneau D, Wihlm JM, Dietemann JL, Roy C. Imaging of cystic masses in the mediastinum. Radiographics. 1990 Nov;10(6):105579. References Kao SW, Zuppan CW, Young LW. AIRP best cases in radiologic-pathologic correlation: type 2 congenital cystic adenomatoid malformation (type 2 congentital pulmonary airway malformation. Radiographics. 2011 MayJun;31(3):743-8. Kawashima A, Fishman EK, Kuhlman JE, Nixon MS. CT of posterior mediastinal masses. Radiographics. 1991 Nov;11(6):1045-67. Kawashima A, Fishman EK, Kuhlman JE, Nixon MS. CT of posterior mediastinal mass. Radiographics. 1993 Mar;13(2):425-41.

Lee EY, Boiselle PM, Cleveland RH. Multidetector evaluation of congenital lung anomalies. Radiology. 2008 Jun;247(3):632-48. Mata JM, Cceres J, Lucaya J, Garca-Conesa JA. CT of congenital malformations of the lung. Radiographics. 1990 Jul;10(4):651-74. References Panicek DM, Heitzman ER, Randall PA, Groskin SA, Chew FS, Lane EJ Jr, Markarian B. The continuum of pulmonary developmental anomalies. Radiographics. 1987 Jul;7(4):747-72. Raider L, Landry BA, Brogdon BG. The retrotracheal triangle. Radiographics. 2002 Oct;22 Spec No:S79-93. Rosado-de-Christenson ML, Stocker JT. Congenital cystic adenomatoid malformation. Radiographics. 1991 Sep;11(5):865-86. Rosado-de-Christenson ML, Frazier AA, Stocker JT, Templeton PA. Radiographics. 1991 Nov;11(6):1045-67. From the archives of AFIP: Extralobar sequestration: radiologic pathologic correlation. Rozovsky K, Koplewitz BZ, Krausz Y, Revel-Vilk S, Weintraub M, Chisin R, Klein M. Added value of SPECT/CT for correlation of MIBG scintigraphy and

diagnostic CT in neuroblastoma and pheochromocytoma. AJR Am J Roentgenol. 2008 Apr;190(4):1085-90. References Shady K, Siegel MJ, Glazer HS. CT of focal pulmonary masses in childhood. Radiographics. 1992 May;12(3):505-14. Tateishi U, Gladish GW, Kusumoto M, Hasegawa T, Yokoyama R, Tsuchiya R, Moriyama N. Chest wall tumors: radiologic findings and pathologic correlation part 2: malignant tumors. Radiographics. 2003 Nov-Dec;23(6):1491-508. Tsuchiya T et al: Bronchopulmonary foregut malformation diagnosed by threedimensional CT. Pediatr Radiol. 33(12):887-9, 2003 Winer-Muram HT, Kauffman WM, Gronemeyer SA, Jennings SG. Primitive neuroectodermal tumors of the chest wall (Askin tumors): CT and MR findings. AJR Am J Roentgenol. 1993 Aug;161(2):265-8. .

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