KCR 2014

October 9(Thu) - 11(Sat), 2014Hall D2, Convention Hall 3F, Coex

Case of the Day

Neuro by Hyung Suk Seo

40/F

Trismus & headache for 8 months

Question

What is your diagnosis?

1) Lymphoma

2) Meningioma

3) Pachymeningeal metastasis

4) Hypertrophic pachymeningitis

5) Venous infarction with venous sinus thrombosis

Answer

4) Hypertrophic pachymeningitis

─ 1 ─

Case 1.

Imaging Features

Peripheral lobulating lesion is noted along the right inferior frontal convexity. The enhancement is

predominantly at the meninges on contrast enhanced T1WI. T2WI shows hyperintense meningeal

thickening with segmental dark signal in the medial side, and diffuse T2 hyperintense brain parenchy-

ma at the right frontal lobe and corpus callosum genu. Decreased NAA peak and slightly increased

choline peak in the right frontal subcortical white matter are noted on MRS with 288 ms TE, and small

lactate peak was noted at 1.3 ppm.

Discussion

Hypertrophic pachymeningitis is a rare chronic inflammatory disorder which is characterized by

marked fibrous thickening of the cerebral or spinal dura matter. Chronic headache and multiple cra-

nial neropathies are the common clinical manifestation. Its etiology remains unclear but infection,

autoimmune disorder, sarcoidosis and neoplasms have been thought as possible causative factors.

This patient did not have any identifiable cause and the diagnosis can be called ‘idiopathic hyper-

trophic pachymeningitis’. The symptom of chronic headache and trismus due to motor disturbance of

the trigeminal nerve was typical.

Contrast-enhanced study is indispensable for the investigatin of hypertrophic pachymeningitis. MRI

is more sensitive than CT to show the linear or nodular thickened dura matter. The lesion appear

hypointense or isointense on T1WI and hypointense on T2WI or FLAIR image.

High dose of steroid therapy usually can induce dramatic improvement and change of symptom

and appearance on images. Cerebral edema and venous sinus thrombosis can complicate this dis-

ease process.

References

1. Shigeo Riku, et al. Idiopathic hypertrophic pachymeningitis. Neuropathology 2003;23:335-344.2. Imran A. Kazem, et al. Idiopathic Tumefactive Hypertrophic Pachymeningitis. RadioGraphics 2005;25:1075-

1080.3. Mark J. Kupersmith, et al. Idiopathic hypertrophic pachymeningitis. NEUROLOGY 2004;62:686-694.4. Mangsuo Zhao, et al. Idiopathic hypertrophic pachymeningitis: Clinical, laboratory and neuroradiologic fea-

tures in China. Journal of Clinical Neuroscience 2014;21:1127-1132.

─ 2 ─

Chest by Kiyeol Lee

F/ 34

What is your diagnosis?

1) Emphysema

2) Lymphangioleiomyomatosis (LAM)

3) Langerhans cell histiocytosis (LCH)

4) Lymphocytic interstitial pneumonia (LIP)

5) Birt-Hogg-Dube syndrome (BHD)

─ 3 ─

Case 2.

Answer

2) Lymphangioleiomyomatosis (LAM)

Image interpretation

Nnumerous discrete, round, thin-walled multiple air cysts are noted on CT images. There is no defi-

nite predominant distribution of the air cysts. Abdomen CT also demonstrates fatty masses in the left

kidney.

Discussion

Lymphangioleiomyomatosis (LAM) is a rare interstitial lung disease that affects women exclusively,

typically during their reproductive years. A small percentage of patients, also typically women, have

LAM in association with tuberous sclerosis complex (TSC). LAM is characterized by the abnormal

proliferation of smooth muscle cells (LAM cells) in the lungs and in the thoracic and retroperitoneal

lymphatics. Affected patients are at risk of developing renal hamartomas or angiomyolipomas.

Patients with LAM characteristically present with chronic dyspnea and cough and less commonly with

spontaneous pneumothorax. Tuberous Sclerosis Complex is an inheritable autosomal dominant dis-

order characterized by multifocal systemic hamartomas that may affect the central nervous system,

skin, heart, kidneys, and other organs. The Vogt triad of epileptic seizures, mental retardation, and

facial angiofibromas (formerly misnamed as adenoma sebaceum) is exhibited by only approximately

29% of affected patients. Relationship between TSC and LAM; Although histologic and clinical fea-

tures may be identical in women with LAM and patients with TSC-associated pulmonary involvement,

there are reported differences. Patients with TSC- associated LAM, compared with patients.

Relationship between TSC and LAM; Although histologic and clinical features may be identical in

women with LAM and patients with TSC-associated pulmonary involvement, there are reported differ-

ences. Patients with TSC- associated LAM, compared with patients with sporadic LAM, generally

experience a longer delay between onset of symptoms and diagnosis of pulmonary disease, exhibit

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chylothoraces less frequently, and are more likely to present with gradual onset of dyspnea.

Radiographic abnormalities in patients with LAM have been reported as a pattern of generalized,

symmetric, reticular, or reticulonodular opacities, seen in approximately 80%-90% of affected

patients. Lung volumes have been reported as normal at chest radiography in 55%-78% of affected

patients and increased in 22%-45%. Pneumothorax is a common clinical and radiographic present-

ing manifestation of LAM, reported in 39%-53% of patients. Pleural effusion (secondary to chylotho-

rax) is a common radiographic feature, occurring in 10%-20% of patients, and may be unilateral or

bilateral. The CT manifestations of LAM are distinctive, characterized by numerous thin-walled cysts

surrounded by normal lung parenchyma and distributed diffusely and bilaterally. Occasionally, a chy-

lous effusion is suggested at chest CT based on low-attenuation (- 17HU) fluid that may relate to the

presence of fat. Renal angiomyolipomas have been observed on abdominal CT scans of 20%-54%

of patients with LAM. These tumors are characterized by CT evidence of soft tissue, fat, and enhanc-

ing vessels in variable proportions within a renal mass. A variety of intracranial manifestations of TS

are known. Four common CNS abnormalities are cortical tubers, subependymal nodules,

subependymal giant cell astrocytomas (SGCAs), and white matter abnormalities. Except for SGCAs,

these abnormalities can be seen in almost all patients with TS.

References

1. Abbott GF, Rosado-de-Christenson ML, Frazier AA, Franks TJ, Pugatch RD, Galvin JR; From the archives ofthe AFIP: lymphangioleiomyomatosis: radiologic-pathologic correlation. Radiographics. 2005 May-Jun;25(3):803-28.

2. Johnson SR, Cordier JF, Lazor R, Cottin V, Costabel U, Harari S, Reynaud-Gaubert M, Boehler A, Brauner M,Popper H, Bonetti F, Kingswood C; Review Panel of the ERS LAM Task Force. European Respiratory Societyguidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010 Jan;35(1):14-26.

3. Pallisa E, Sanz P, Roman A, Maj J, Andreu J, C ceres J. Lymphangioleiomyomatosis: Pulmonary andAbdominal Findings with Pathologic Correlation. Radiographics. 2002 Oct;22 Spec No:S185-98. Review.

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Abdomen by Yang Shin Park

32/M

Right flank pain.

─ 6 ─

Case 3.

Fig. 3. Coronal HASTE Fig. 4. Coronal HASTE Fig. 5. Coronal HASTEFig. 3. Coronal HASTE

Fig. 6. Coronal 20-min hepatobiliaryphase

Fig. 4. Coronal HASTE Fig. 5. Coronal HASTE

Fig. 1. Axial CT Fig. 2. Coronal CT

Question

What is your diagnosis?

1) Choledocal cyst

2) Hydatid cyst

3) Intraductal papillary neoplasm with invasive adenocarcinoma

4) Biliary cystadenocarcinoma

5) Cystic metastasis

Answer

3) Intraductal papillary neoplasm with invasive adenocarcinoma

Imaging Features

Contrast enhanced CT scans (Figs. 1 and 2) show an approximately 17-cm lobulating multilocular

cystic mass at the right liver and left medial segment. On coronal HASTE MR images (Figs. 3--5),

some cysts show either tubular shaped or cystic shaped. The some portions appear to be connected

to other cystic portions. There are small nodular portions within the cystic mass. On coronal 20-min

hepatobiliary phase, the nodular portions show mild enhancement.

Brief Review

Intraductal papillary neoplasm of bile duct (IPNB) has been recently recognized as a new entity of

biliary neoplasm which includes previous categories of biliary papilloma and papillomatosis. IPNB is

considered as a premalignant lesion in the dysplasia-carcinoma sequence, and if there is an invasive

component, it would be referred to as IPN with an associated invasive carcinoma, and can be pro-

gressed to intraductal growing cholangiocarcinoma. Histologically, IPNB is characterized by the intra-

ductal papillary or villous growth of biliary epithelium covering fine fibrovascular stalks. IPNB is known

as a biliary counterpart of intraductal papillary mucinous neoplasm (IPMN) of the pancreas based on

their pathologic similarities, although macroscopic mucin production is less frequent in the IPNB than

in the IPMN of the pancreas. IPNB can appear as cyst-forming masses because cystic or aneurys-

mal dilatation of the bile duct occurs as a result of excessive mucin production by papillary tumor cells

lining the inner surface of the cyst. Because cystic IPNBs usually manifest as unilocular or multilocu-

lar cystic lesions in the liver, imaging differentiation from mucinous cystic neoplasms can be challeng-

ing. Prominent mural nodules in cystic tumors usually suggest the possibility of biliary cystadenocarci-

noma or cystic IPNB, whereas mural nodules are rare in cases of biliary cystadenoma. In addition,

the presence of dilated bile ducts distal to the cystic tumor, which indicates communication between

the bile ducts and cystic mass, favors a diagnosis of cystic IPNBs.

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References

1. Joo I, Lee JM. Imaging bile duct tumors: pathologic concepts, classification, and early tumor detection.Abdom Imaging 2013;38(6):1334-1350.

2. Lim JH, Zen Y, Jang KT, Kim YK, Nakanuma Y. Cyst-forming intraductal papillary neoplasm of the bileducts: description of imaging and pathologic aspects. AJR Am J Roentgenol 2011;197(5):1111-1120.

3. Kim SW, Kim HC, Yang DM, Won KY, Kim BS. Cystic intraductal papillary adenocarcinoma of the bile ductof the caudate lobe initially manifesting as a simple cyst on CT. Jpn J Radiol 2014;32(6):371-4.

─ 8 ─

Urology by Deuk Jae Sung

M/74

Gross hematuria and dysuria

─ 9 ─

Case 4.

Fig. 1. Initial contrast-enhanced CT.

Fig. 2. Three-month follow-up MRI.

Question

what is your diagnosis?

1) Renal cell carcinoma

2) Transitional cell carcinoma

3) Membranous glomerulonephritis

4) Xanthogranulomatous pyelonephritis

5) Renal tuberculosis

Answer

2) Transitional cell carcinoma

Imaging Features

Initial contrast-enhanced CT images show thrombus in right renal vein and poor excretion of con-

trast material in the collecting system (Fig. 1). Follow-up MR images demonstrate enhancing, ill-

defined mass and wall thickening along the collecting system of right kidney with caliectasis in lower

pole. In addition, MR images show an enlarged thrombus in the right renal vein with extension into

the IVC (Figs. 2 and 3).

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Fig. 3. Three-month follow-up MRI.

Brief Review

The clinical manifestations of renal vein thrombosis depend on the age of the patient, the specific

disease process, and the speed with which it occurs. The causes of renal vein thrombosis are either

neoplastic or nonneoplastic. Nonneoplastic renal vein thrombosis is usually caused by an underlying

abnormality of the clotting system or the kidney itself or, in infants, dehydration. In adults, the most

common underlying abnormality is membranous glomerulonephritis. Renal vein thrombosis is also a

complication of the hypercoagulable state in patients with SLE. But, renal vein thrombosis can be

associated with tumor and is frequently caused by direct tumor extension. Neoplastic renal vein

thrombosis occurs most commonly in cases of renal cell carcinoma and occasionally in cases of tran-

sitional cell carcinoma. In renal vein thrombosis associated tumors, CT or MRI can show an enhanc-

ing soft-tissue lesion in the renal parenchyma or renal pelvis, and inhomogeneous enhancement

within the thrombus. Tumor thrombus in the renal vein may also result from a left adrenal tumor.

References

1. CT evaluation of renovascular disease. Kawashima A, Sandler CM, Ernst RD, Tamm EP, Goldman SM,Fishman EK. Radiographics 2000;20:1321-1340.

2. Jung SI, Kim SH. Vascular diseases of the kidney. In: Kim SH, ed. Radiology illustrated uroradiology. 2nd ed.Berlin Heidelberg, Springer, 2012; 629-633.

─ 11 ─

Cardiac imaging by Sung Ho Hwang

74/M

Chief complaint: recently aggravated dyspnea

Question

What is your diagnosis?

1) Myocardial infarction

2) Viral myocarditis

3) Hypertrophy cardiomyopathy

4) Hypertensive cardiomyopathy

5) Cardiac amyloidosis

─ 12 ─

Case 5.

Fig. 1. T2 weighted MR image. Fig. 2. Cine MR image.

Fig. 3. Delayed enhancement MR image. Fig. 4. Delayed enhancement MR image.

Answer

5) Cardiac amyloidosis

Image Features

Cardiac MRI shows concentric left ventricular hypertrophy and minimal pericardial effusion. T2

weighted MRI shows no myocardial edema. But, Delayed enhancement MRI demonstrates global

subendocardial enhancements in the left ventricle.

Discussion

Cardiac amyloidosis is a type of secondary nonischemic cardiomyopathy that results from extracel-

lular accumulation of insoluble fibrillary amyloid proteins. Several different forms of cardiac amyloido-

sis are recognized, including light chain (amyloid Light-chain: AL), familial, and senile amyloidosis,

which can have widely varying clinical presentations. AL amyloidosis is the most common form, asso-

ciated with plasma cell dyscrasias and the production of light chain protein. Cardiac involvement is

present in half of plasma cell dyscrasias, and has a particularly poor prognosis with a median survival

of less than 6 months in untreated patients with symptoms of heart failure.

Cardiac magnetic resonance (MR) imaging in suspected amyloidosis should include delayed

enhancement MR sequence. Functional MR images show concentric ventricular hypertrophy,

enlarged atria, restricted diastolic filling, and normal to decreased ejection fraction. Expansion of the

extracellular space by the amyloid proteins results in gadolinium retention, which manifests as

delayed enhancement. In addition, the absence of contrast material within the blood pool on delayed

enhancement MR image results in the characteristic dark blood pool. As the distinct pattern of

delayed enhancement, the global transmural or global subendocardial enhancements correlate with

the greatest amount of amyloid deposition. In general, incomplete nulling of the myocardial signal on

the inversion recovery scout sequence can be seen. So, if difficulty with myocardial suppression on

the delayed contrast-enhanced image is noted, then, cardiac amyloidosis should strongly be consid-

ered as the cause.

References

1. Giesbrandt KJ, Bolan CW, Shapiro BP, et al. Diffuse diseases of the myocardium: MRI-pathologic review ofnondilated cardiomyopathies. AJR Am J Roentgenol. 2013;200(3):W266-73.

2. Penugonda N. Cardiac MRI in infiltrative disorders: a concise review. Curr Cardiol Rev. 2010;6(2):134-6.

─ 13 ─

Head & neck by Young Hen Lee

Question

An otherwise healthy 37-year-old woman admitted to ENT clinic due to her headache, facial pain,

and bloody-purulent rhinorrhea. Her symptoms got aggravated 2 weeks ago. Her physical examina-

tion revealed a large polypoid mass completely obstructing the left nasal airway, without evidence of

increased vascularity. What is the most likely diagnosis?

1) Mucocele

2) Inverted papilloma

3) Malignant lymphoma

4) Organized hematoma

5) Squamous cell carcinoma

─ 14 ─

Case 6.

Answer

4) Organized hematoma

Her non-contrast computed tomogram showed an expansile lesion centered in the left maxillary

sinus, and extending to the ipsilateral nasal cavity. On her T1-, T2-weighted axial images, it appears

as a round soft tissue lesion with mixed signal intensity, surrounded by very low signal rim, especially

on T2-weighted image. After Gd-administration, it shows markedly heterogeneous enhancement. A

biopsy specimen via endoscopic approach showed only chronic inflammation, hemorrhage and fibro-

sis, however, there was no evidence of malignancy. Because the clinical biopsy findings were unre-

vealing, sinonasal organized hematoma was considered as final diagnosis.

Organized hematoma of sinonasal cavity is a rare, non-neoplastic condition that can be locally

aggressive. Sometimes it is associated with history of facial trauma, surgery, or hemorrhagic

sinonasal lesion. About 70% of patients with sinonasal organized hematoma complain of his/her

recurrent epistaxis. The clinical presentation and radiographic appearance can mimic malignancy, but

definitive diagnosis is confirmed only after histopathologic evaluation.

On computed tomograms, the lesion typically appears as a primarily expansile soft tissue mass

smoothly eroding the bony wall. Magnetic resonance images usually demonstrate a inhomogeneous

mass on both T1- and T2-weighted images. Mostly, they show marked heterogenous enhancement

(ex: papillary or frond-like enhancement) representing sites of neovascularity that occur within an

organizing hematoma, Moreover, peripheral hypointense rim, as characteristic feature to suggest

fibrous pseudocapsule may be obvious on of T2-weighted image.

There are multiple hypotheses regarding the pathogenesis of an organized hematoma. One theory

suggests that intranasal bleeding causes blood accumulation in the maxillary sinus cavity. Another

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involves rupture of the vascular branch supplying the maxillary sinus or submucosal bleeding in the

maxillary sinus due to loss of mechanical integrity of an arterial branch. Regardless of the inciting

event, this condition evolves in several stages. Once blood accumulates in the maxillary sinus, a

hematoma develops there. Because of poor ventilation and drainage, the hematoma undergoes neo-

vascularization and fibrosis, thus leading to an organized hematoma. The mass tends to grow slowly

and eventually can even cause pain owing to compression of surrounding structures.

The differential diagnosis is extensive and includes infectious, inflammatory, and neoplastic lesions.

In cases in which there is no radiographic bone erosion, the differential diagnosis includes sinusitis,

extensive polyposis and cysts, inverting papilloma, hemangioma, and mucocele. In cases involving

radiographic bone expansion and destruction, the differential diagnosis includes both infectious and

neoplastic processes. Fungal sinusitis is an important infectious disease process that needs to be

taken into consideration. Malignant conditions include adenoid cystic carcinoma, squamous cell carci-

noma, embryonal rhabdomyosarcoma, lymphoma, and tumors of dental origin. Pathologic confirma-

tion of the diagnosis is essential. Histologic findings include hematoma, fibrosis, neovascularization,

and no evidence of malignancy.

References

1. Kim EY, Kim HJ, Chung SK, Dhong HJ, Kim HY, Yim YJ, Kim ST, Jeon P, Ko YH. Sinonasal organizedhematoma: CT and MR imaging findings. AJNR Am J Neuroradiol. 2008 Jun;29(6):1204-8

2. Lee HK, Smoker WR, Lee BJ, Kim SJ, Cho KJ. Organized hematoma of the maxillary sinus: CT findings. AJRAm J Roentgenol. 2007 Apr;188(4):W370-3.

3. Omura G, Watanabe K, Fujishiro Y, Ebihara Y, Nakao K, Asakage T. Organized hematoma in the paranasal si-nus and nasal cavity—imaging diagnosis and pathological findings. Auris Nasus Larynx. 2010 Apr;37(2):173-7.

─ 16 ─

Abdomen by Suk Keu Yeom

91y/M

Right inguinal pain

─ 17 ─

Case 7.

Fig. 1

Fig. 2 Fig. 3

Question

What is your diagnosis?

1) Dermoid cyst

2) Foreign body abscess

3) Amyand’s hernia

4) Obturator hernia

5) Deep vein thrombosis

Answer

3) Amyand’s hernia (Inguinal hernia with acute appendicitis)

Imaging features

A 91-year-old woman presented with a 3-day history of swelling and pain in the right inguinal

region. Computed tomographic imaging of the abdomen revealed a dilated appendix within a right-

sided indirect inguinal hernia. The edematous appendix measured 1.1 cm in diameter, narrowing at

orifice of inguinal canal, and was accompanied by a fluid collection.

Discussion

An inguinal hernia sac containing a vermiform appendix is known as Amyand’s hernia. Claudius

Amyand, sergeant-surgeon to King George II of England, is credited by some with performing the first

documented successful appendectomy, in which he removed a perforated appendix from a right

inguinal hernia sac in 1735 (1).

Amyand’s hernia is classically described to account for 1% of inguinal hernias and 0.1% of cases of

appendicitis. In the pediatric population, Amyand’s hernia is about 3 times more common, and its

prevalence can actually reach 1%, a difference probably based on certain anatomic characteristics.

Amyand’s hernia is, as expected, more common in male patients and presents a bimodal age distrib-

ution in neonates and in patients aged 70 years (2).

Preoperative diagnosis of Amyand’s hernia is feasible with US and CT. CT allows direct visualiza-

tion of the appendix inside the inguinal canal (3).

Classical treatment of Amyand’s hernia includes appendectomy, reduction of hernia, and hernio-

plasty through the same incision (2).

References

1. Malayeri AA, Siegelman SS. Images in clinical medicine. Amyand’s hernia. The New England journal of med-icine. 2011;364(22):2147. Epub 2011/06/03.

2. Michalinos A, Moris D, Vernadakis S. Amyand’s hernia: a review. American journal of surgery.2014;207(6):989-95. Epub 2013/11/28.

3. Luchs JS, Halpern D, Katz DS. Amyand’s hernia: prospective CT diagnosis. Journal of computer assisted to-mography. 2000;24(6):884-6. Epub 2000/12/06.

─ 18 ─

Musculoskeletal by Suk-Joo Hong

18/M

Chief complaint: Left posterior ankle pain

─ 19 ─

Case 8.

Fig. 2. Sagittal T1-weighted image

Fig. 3. Fat suppressed sagittal T2-weighted image

Fig. 1. Left foot lateral view

Question

What is the most like diagnosis?

1) Achilles tendon tear

2) Haglund syndrome

3) Os trigonum syndrome

4) Sever disease

5) Achilles insertional tendinopathy

Answer

2) Haglund syndrome

Imaging Feature

Foot lateral radiograph shows osseous prominence of posterosuperior margin of calcaneus, soft tis-

sue fullness in pre-Achilles bursa region. In sagittal T1-, T2-weighted MR images, fluid in pre-Achilles

bursa, inflammatory change in pre-Achilles and retro-Achilles fat, calcaneal bone marrow edema.

Insertional Achilles tendinopathy shows streaks of increased signal intensity not as bright as fluid.

Brief Reviews

Definition

* Hanglund deformity: Enlarged superior margin of calcaneal posterior process

* Haglund triad (Haglund syndrome): Haglund deformity + pre-Achilles (retrocalcaneal) bursitis +

Insertional Achilles tendinosis

Haglund symdrome is a common cause of posterior heel pain, characterized clinically by a painful

soft tissue swelling at the level of Achilles tendon insertion. Generally, this condition is secondary to

chronic bony deformity, along with the shoes in question, may lead to a mechanically induced inflam-

mation of the superficial bursa, Achilles tendinosis, and retrocalcaneal bursitis. These inflammatory

changes in the presence of the bony deformity are together known as Haglund syndrome. Hindfot

varus and pes cavus are predisposing factors. Most important is the presence of chronic stress. On

lateral radiograph, the Haglund deformity is manifested by a bony projection of posterosuperior

aspect of calcaneal tuberosity (the radiographic equivalent of the “pump bump”). Soft tissue fullness

in pre-Achilles bursa region, soft tissue infiltration in Kager’s fat fad, Achilles tendon swelling with ill-

defined margin, and retro-Achilles soft tissue swelling may be seen in lateral radiograph. In MRI, bone

marrow edema on posterior calcaneus including osseous bump area, pre-Achilles or retro-Achilles

bursitis, edema in Kager’s fat fad, Achilles peritendinitis, and Achilles insertional tendinopathy (streaky

areas of increased signal intensity within thickened tendon) may be seen.

─ 20 ─

References

1. Pavlov H, Heneghan MA, Hersh A, Goldman AB, Viqorita V. The Haglund syndrome: initial and differentialdiagnosis. Radiology 1982;144(1):83-88

2. Lawrence DA, Rolen MF, Morshed KA, Moukaddam H. MRI of Heel Pain. AJR 2013;200:845-8553. Sonin A, Manaster BJ, Andrews CL, Crim A, Tuite MJ, Zoga AC. DIAGNOSTIC IMAGING Musculoskeletal:

Trauma. Amirsys 2010; 7:171-173

─ 21 ─

Pediatric by Bo-Kyung Je

M/1 day

CC: abnormal finding on fetal US

Question

What is your diagnosis?

1) Bronchial atresia

2) Pulmonary Sequestration

3) Pleuropulmonary blastoma

4) Congenital Lobar Overinflation (CLO)

5) Congenital Pulmonary Airway Malformation (CPAM)

Answer

5) Congenital Pulmonary Airway Malformation (CPAM)

─ 22 ─

Case 9.

Fig. 1. Fetal US (4 chamber view) at 22+1 weeks of ges-tational age.

Fig. 3. Chest CT scans at 2 months of age.

Fig. 2. Chest AP view at first day.

Imaging Features

Fetal US (Fig. 1) shows multiple hypoechoic cysts and intervening areas of increased homoge-

neous echogenicity. Postnatal chest AP radiography (Fig. 2) shows heterogeneous radiolucency in

the right lung. Chest CT scans in a lung window setting (Fig. 3) demonstrates the multiple cystic

lesions in the right lower lobe. The larger cysts are measured more than 2 cm, surrounded by smaller

cysts, suggesting type I CPAM.

Brief Review

CPAM is characterized by a heterogeneous group of congenital cystic and noncystic lung masses

that communicate with an abnormal bronchial tree lacking supporting cartilage. Type I CPAM is most

common with one or more cysts larger than 2 cm in diameter, usually lined by ciliated columnar

epithelium. Type II CPAM consists of multiple thin-walled cysts, smaller than 2 cm and lined with ciliat-

ed cuboidal or columnar epithelium. Type III CPAM manifests as a bulky, firm mass of glandular-like

tissue. Type IV CPAM consists of thin walled large cyst with non-ciliated flattened, alveolar lining cells.

The generalized consensus is that symptomatic CPAMs should be removed by lobectomy, but in case

of prenatally diagnosed, asymptomatic, small CPAMs, there is a controversy about the timing of

resection. There is a potential risk of associated complications, such as infection, pneumothorax, and

the small risk of malignant transformation, particularly in the case of CPAM type 1.

References

1. Coley BD, Caffey’s Pediatric Diagnostic Imaging, 12th Ed. Mosby-Elsevier, 20132. Shimohira M, Hara M, Kitase M, et al. Congenital pulmonary airway malformation: CT-pathologic correla-

tion. J Thorac Imaging 2007;22:149-53.

─ 23 ─

Breast by Kyu Ran Cho

F/45

Chief Complaint: Palpable lump in the right breast

─ 24 ─

Case 10.

Fig. 1. Mammography (MG). Fig. 2. Ultrasonography (US).

Fig. 3. MRI (T2 Weight image). Fig. 4. MRI (Early subtraction MPR image).

Question

What is the most probable diagnosis?

1) Phyllodes tumor

2) Pseudoangiomatous stromal hyperplasia

3) Metaplastic carcinoma

4) Mucinous carcinoma

5) Tubular carcinoma

Answer

4) Mucinous carcinoma

Imaging features

Mammography (Fig. 1) shows heterogeneously dense parenchymal pattern. A 2 cm sized lobular

circumscribed and partially microlobulated isodense mass is seen at the inner portion of right breast,

which is area where the patient complained of palpable lump. No associated calcifications is noted

within the mass. Utrasonography (Fig. 2) demonstrates a relatively circumscribed and partially

microlobulated isoechoic mass with posterior acoustic enhancement. MRI of T2-WI (Fig. 3) depicts

well-defined smooth margined round mass with bright high signal intensity and this shows peripheral

rim enhancement on early subtraction MPR image (Fig. 4). On time-intensity curve (Fig. 5), this is a

gradually enhancing mass (type I curve). Ultrasound-guided biopsy and subsequent surgery revealed

mucinous carcinoma.

Brief review

1. Clinical characteristeics

1) About 2% of all invasive breast cancer

2) Slow growth rate, a favorable prognosis

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Fig. 5. Time- Intensity Curve.

3) Age-related : 7% (> 75 yrs) / 1% (< 35 years)

4) If palpable, tend to manifest as soft masses

2. Radiologic findings

1) Mammography

- Manifests as a low-density, relatively well-defined or microlobulated oval or lobular mass

- Ill-defined rather than sharply defined on spot compression views

2) Ultrasoography

- Circumscribed or microlobulated margins / Isoechoic or hypoechoic

- Presence of posterior enhancement on US (46%)

- Vascularity (33%)

3) MRI Morphology

- Lobular, oval or round shaped, well-defined and smooth marginated

- Very high SI on T2WI compared with other histologic types of invasive ductal carcinoma due

to the watery nature of mucin

3. Histology

1) Pure mucinous carcinoma (> 90% of mucin contents)

better prognosis, lower incidence of axillary LN metastasis

2) Mixed mucinous carcinoma (< 90% of mucin contents),

75% of mucinous carcinoma of the breast

References

1. Shuichi M, Masaki Y, Toshiko S, et al. Mucinous Carcinoma of the Breast: MRI Features of Pure and MixedForms with Histopathologic Correlation AJR 2009; 192: 125-131

2. Lam WW, Chu WC, Tse GM, Ma TK. Sonographic appearance of mucinous carcinoma of the breast. AJR2004;182:1069-1074

3. Kawashima M, Tamaki Y, Nonaka T,et al. MR imaging of mucinous carcinoma of the breast. AJR2002;179:179-183

4. Memis A, Ozdemir N, Parildar M, Ustunn EE, Erhan Y. Mucinous breast cancer: mammographic and US fea-tures with histologic correlation. Eur J Radiol 2000; 35:39-43

5. Matsuda M, Yoshimoto M, Iwase T, et al. Mammographic and clinicopathological features of mucinous carci-noma of the breast.Breast Cancer. 2000;7:65-70

6. Cardenosa G, Doudna C, Eklund GW. Mucinous (colloid) breast cancer: clinical and mammographic findingsin 10 patients. AJR 1994; 162:1077-1079

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