QUESTION-1:
Findings
CT scan shows large anterior (prevascular) mediastinal soft tissue mass with he:Correct Answer: Hodgkin lymphoma or Hodgkin disease (HD)
- Is a neoplasm of B lymphocytes characterized by the presence of Reed-Sternberg cells.
- Peak incidence occurs in two main age groups: young adults in the third decade and adults older than 50 years. It is slightly more common in men than in women.
- Intrathoracic involvement in HL is common and occurs most often in the form of lymph node enlargement.
- HL typically manifests as painless lymphadenopathy, involving the cervical and/or supraclavicular regions in 60% to 80% of patients, or as an incidental mediastinal mass on chest radiograph.
- HL involving the anterior mediastinum or paratracheal lymph nodes typically manifests radiographically as widening of the superior mediastinum by a lobulated mass
- Enlarged lymph nodes most commonly have homogeneous. In approximately 20% of cases, particularly in patients with large, coalescent masses, the involved lymph nodes or thymus may contain areas of low attenuation representing necrosis, hemorrhage, and cystic degenerations soft tissue attenuation.
- HL also frequently involves the thymus and may result in diffuse thymic enlargement. Thymic involvement may be difficult to distinguish from anterior mediastinal lymph node involvement.
Differential Diagnosis:
- Lymphoma hyperplasia
- Thymic neoplasms
- Substernal thyroid mass
- Germ cell tumors
- Mediastinal tuberculous lymphadenopathy
- Lymphangioma.
Pearls:
- Peak incidence occurs in young adults in the third decade and adults older than 50 years.
- Involving the cervical and/or supraclavicular regions in 60% to 80% of patients.
- HL involving the anterior mediastinum or paratracheal lymph nodes typically manifests radiographically as widening of the superior mediastinum by a lobulated mass.
References:
- https://radiopaedia.org/articles/hodgkin-lymphoma
- https://radiologykey.com/hodgkin-lymphoma-5/
- https://radiologyassistant.nl/pediatrics/pediatric-chest-ct/nonvascular-mediastinal-masses
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Correct Answer Explanation
QUESTION-2:
What’s the following MRI Image representative of:
Correct Answer: Mucoid impaction.
- Mucus plugs or mucoid impaction can mimic the appearance of lung nodules or a mass.
- Sometimes differentiating mucus impaction from a lung cancer can be difficult.
- Mucoid impaction is commonly seen in patients with bronchiectasis, as in cystic fibrosis (CF) and allergic bronchopulmonary aspergillosis (ABPA).
- It is also seen in bronchial obstruction caused by an obstructing tumor or bronchial atresia.
- The mucus in the dilated bronchi looks like the fingers in a glove (It is a common presentation).
Radiographic Features:
- Mucoid impaction may appear as a branching tubular opacity that is distinct from the normal vascular shadows. This classic feature is the finger-in-glove sign, and is also seen on CT. It can sometimes prove difficult to diagnose on plain radiography.
CT
- The appearance on CT can be variable, depending on where the mucous plugging occurs (central or peripheral airways).
- Mucous plugs appear as fluid density material often located dependently within the bronchus, sometimes with internal gas.
- The classic feature seen when plugged mucus extends along large branching airways is the finger-in-glove sign.
- Depending upon the orientation of the airways with respect to the image, airway impaction will appear as circular or tubular structures that connect to more central bronchi.
- Several key additional features can also occur:
- Distal lung collapse: from collateral air drift; this has not yet developed in infants and young children, which is why the distal lung is rather hyperlucent in this population (congenital bronchial atresia, foreign body aspiration).
- Distal airway dilatation
- Double artery sign
Plain radiograph
Differential Diagnosis:
- Endobronchial blood or blood clot appears similar to mucous plugging on CT.
- In some situations, there can be overlap between some endobronchial tumors per se (rather than with associated mucoid impaction).
Pearls:
- The appearance on CT can be variable, depending on where the mucous plugging occurs.
- The classic feature seen when plugged mucus extends along large branching airways is the finger-in-glove sign.
References:
- https://radiologyassistant.nl/chest/chest-x-ray/lung-disease#nodules-and-masses-mucoid-impaction
- https://radiologykey.com/airways-diseases/
- https://radiopaedia.org/articles/mucoid-impaction-lung-1#:~:text=Mucoid%20impaction%2C%20also%20referred%20to,pathological%20finding%20in%20chest%20imaging.
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Correct Answer Explanation
QUESTION-3:
4 month history of cough shows:
Correct Answer: Broncho-alveolar CA
- An uncommon type of bronchial carcinoma which occurs most frequently among non-smokers, women and Asians.
- There is no significant gender predilection, unlike other lung cancer types which are more prevalent in men.
- Risk factors: a pre-existing focus of pulmonary fibrosis, e.g. tuberculosis scar, infarct, scleroderma.
- Presentation is often insidious, and a large proportion (50%) of patients may be asymptomatic at the time of detection. Alternatively, as these tumors can produce large quantities of mucus, patients may present with bronchorrhea.
- Persistent consolidation for weeks despite appropriate antimicrobial therapy should raise the suspicion of a neoplastic process.
Radiographic Features:
- Solitary nodule or mass (40% of patients)
- Focal or diffuse consolidation (30%)
- Diffuse ill-defined centrilobular nodules (30%) due to endobronchial spread.
Plain radiograph:
May show segmental or lobar consolidation with chronic unilateral airspace opacification and air bronchograms. Can also present as a pulmonary nodule, mass or a cluster of diffuse nodules. The nodular form (commonest) can be indistinguishable from another subtype of adenocarcinoma or inflammatory granuloma on plain film.
The appearance on CT depends on its pattern of growth; hence, it may appear as:
A peripheral nodule:- Commonest appearance.
- Typically solitary and well-circumscribed.
- The nodule may be surrounded by a halo of ground-glass opacity, the so-called fried egg sign.
Cavitation:
- Pseudo cavitation (presence of bubble-like lucencies) is recognized.
- Overt cavitary changes rarely occur (~7%).
- Cavitating pulmonary metastases may occur.
- A focal area of ground glass (early sign): heterogeneous attenuation.
- A region of ground glass, with or without consolidation.
- Hilar and mediastinal adenopathy and pleural effusion are uncommon.
- Air bronchograms may be seen (also known as the open bronchus sign).
There are three recognized radiographic patterns:
Differential Diagnosis:
- Sarcoidosis
- TB
- Alveolar carcinoma
- Lipoid PN
- Lymphoma
- Alveolar proteinosis
- Goodpasture’s Dz
- BOOP
- Aspiration
- Silicosis
- Hemosiderosis
CHRONIC AIR SPACE DISEASE:
Pearls:
- An uncommon type of bronchial carcinoma which occurs most frequently among non-smokers, women and Asians.
- There is no significant gender predilection.
- Risk factors: a pre-existing focus of pulmonary fibrosis.
- Plain radiograph: may show segmental or lobar consolidation with chronic unilateral airspace opacification and air bronchograms. Can also present as a pulmonary nodule, mass or a cluster of diffuse nodules. The nodular form (commonest) can be indistinguishable from another subtype of adenocarcinoma or inflammatory granuloma on plain film.
- The appearance on CT depends on its pattern of growth; hence, it may appear as:
- A peripheral nodule.
- Cavitation.
- A focal area of ground glass.
- Hilar and mediastinal adenopathy and pleural effusion are uncommon.
- Air bronchograms may be seen.
References:
- https://radiopaedia.org/articles/adenocarcinoma-in-situ-minimally-invasive-adenocarcinoma-and-invasive-adenocarcinoma-of-lung-1?lang=us
- https://radiologyassistant.nl/chest/hrct/basic-interpretation
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Correct Answer Explanation
QUESTION-4:
Follow up to incidental finding:
Correct Answer: Broncholithiasis.
- The term broncholithiasis is used to denote the presence of calcified or ossified material within the lumen of the bronchus, usually due to erosion from adjacent lymph nodes.
Causes:
- Erosion by and Extrusion of Calcified Peribronchial Lymph Nodes.
- Aspiration of Radiopaque Fragments or in Situ Calcification of Foreign Material.
- Erosion by and Extrusion of Calcified Bronchial Cartilage Plates.
Imaging Findings:
- Endobronchial or peri bronchial calcified nodule at CT associated with signs of bronchial obstruction.
- Most common right middle lobe and anterior segmental bronchi upper lobes.
- Broncholith.
- Distorts adjacent airway, which is narrowed but remains patent (50%) or
- Calcified lymph nodes completely obstructs lumen (50%)
- Majority of lymph node calcified, soft tissue component < 10%
- Usually solitary, rarely multiple.
- Extraluminal air nearly diagnostic of erosion into bronchus.
Differential Diagnosis:
- The differential diagnosis of high-attenuation endobronchial lesions can be narrowed by carefully obtaining the patient history and evaluating CT findings.
- In cases of broncholithiasis due to erosion by calcified peribronchial lymph nodes, the most common cause, CT usually shows lymph nodes with or without calcification at other locations and parenchymal changes due to infection by causative organisms, in addition to the typical findings of broncholithiasis.
- Sometimes peribronchial lymph nodes, which do not erode into the bronchial lumen, may mimic broncholiths.
- In cases of an aspirated foreign body, the airways and pulmonary parenchyma are usually normal except for the broncholith.
- Calcification of other parts of the tracheobronchial wall is usually seen in cases of broncholithiasis due to erosion and extrusion of calcified bronchial cartilage plates and tracheobronchopathia osteochondroplastica. Noncalcified thickening of the airway wall helps in the diagnosis of tracheobronchial amyloidosis.
- The detection of fat content within an endobronchial lesion is important in diagnosing endobronchial hamartoma.
- Careful examination of contiguous images, or images obtained at non-contrast-enhanced CT, may be necessary to differentiate a hypertrophied bronchial artery protruding into the bronchial lumen from a broncholith.
Pearls:
- Even though the most common cause of broncholithiasis is erosion of the bronchial wall and intraluminal protrusion of a calcified peribronchial lymph node caused by granulomatous infection such as tuberculosis and histoplasmosis, there are several other pathogeneses.
- In addition, endobronchial tumors with calcification or tracheobronchial disease with mural calcification can mimic broncholithiasis.
- Awareness of the various causes of broncholithiasis and its mimics, as well as awareness of the typical radiologic features, is helpful in obtaining an accurate diagnosis and providing adequate patient management.
References:
- https://pubs.rsna.org/doi/10.1148/radiographics.22.suppl_1.g02oc07s199
- https://radiologykey.com/broncholithiasis/
- https://radiopaedia.org/articles/broncholithiasis
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Correct Answer Explanation
QUESTION-5:
60 yo smoker working at a mining company:
Correct Answer: Silicosis with Progressive massive fibrosis.
- Progressive massive fibrosis (PMF) refers to the formation of large mass-like conglomerates, predominantly in the upper pulmonary lobes, associated with radiating strands. These classically develop in the context of certain pneumoconiosis (especially coal worker's pneumoconiosis and silicosis) although similar mass-like densities have occasionally been described with talcosis.
- Usually bilateral in upper lobes and superior segments of lower lobes.
- Typically more common in dorsal aspect of lung.
- Right hemithorax earlier and PMF larger than left hemithorax
- PMF > 4 cm nearly all contain areas of low attenuation due to central necrosis.
- If necrosis or cavitation is seen, mycobacterial infection should be considered.
- As PMF progresses, micronodules decrease in profusion.
- Initially develop in lung periphery and with time migrate towards hilum.
Radiographic Features:
- May be seen as large symmetric bilateral opacities with irregular margins in the upper lobes.
CT:
- Mass-like areas of lung opacification associated with radiating strands are seen; the "sausage-shaped" mass is characteristic. These regions commonly contain air bronchograms and calcifications. These areas can shrink over time and migrate towards the hilar regions.
MRI:
- Magnetic resonance imaging can be helpful for distinguishing between progressive massive fibrosis and lung cancer. The latter typically appears as T2-bright, whereas progressive massive fibrosis appears as T2-dark (compared to skeletal muscle).
- The most frequent MRI appearance are regions which have following signal characteristics:
T1:
- Iso- to hyperintense
T2:
- Hypointense (compared with skeletal muscle).
- Areas of internal high T2 signal.
- There may be rim enhancement.
Plain radiograph:
Differential Diagnosis:
- UPPER LOBE INTERSTITIAL DZ (CASSETE PH) Cystic Fibrosis Ankylosing Spondylitis Silicosis
- Sarcoid Eosinophilic Granuloma TB
- Extrinsic allergic alveolitis
- Pneumoconioses, PCP (old days)
- Histo
Pearls:
- Usually bilateral in upper lobes and superior segments of lower lobes.
- PMF > 4 cm nearly all contain areas of low attenuation due to central necrosis.
- Mass-like areas of lung opacification associated with radiating strands are seen; the "sausage-shaped" mass is characteristic. These regions commonly contain air bronchograms and calcifications. These areas can shrink over time and migrate towards the hilar regions.
References:
- https://radiopaedia.org/articles/progressive-massive-fibrosis
- https://radiologykey.com/silicosis-complex/
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QUESTION-6:
32 yo woman with exertional dyspnea:
Correct Answer: Lymphangiomyomatosis.
- Proliferation of atypical smooth muscle cells in lungs & lymphatics associated with pulmonary cysts.
- Rare disease, It almost exclusively affects women of childbearing age.
- Identical pulmonary changes seen in 1% of patients with tuberous sclerosis.
- Clinical findings:
- Majority of patients present with dyspnea.
- Chylous pleural effusions (40%), Pneumothorax (40%), hemoptysis (40%).
- Patients die within 10 years of the onset of symptoms.
- Pregnancy may exacerbate disease.
- Lymphangioleiomyomatosis is a multi-system disorder and can affect many organs.
Radiographic Features:
- Multiple thin-walled cysts throughout the lungs, usually with a uniform distribution. These are present in nearly all cases. If the cysts are small, they may be seen as diffuse coarse interstitial markings on plain films.
Radiograph:
- Chylothorax: chylous pleural effusion.
- Evidence of hyperinflation.
- Diffuse bilateral reticulonodular densities.
- Recurrent pneumothoraces in complicated cases.
HRCT:
- Thin-walled cysts of variable sizes surrounded by normal lung parenchyma can be seen throughout the lung.
- Interlobular septal thickening.
- May show a dilated thoracic duct.
- Hemorrhages may be seen as areas of increased attenuation.
Chest
Differential Diagnosis:
- Langerhans cell histiocytosis: > 90% are smokers, cysts have irregular shapes and the basal costophrenic angles are spared.
- Centrilobular emphysema: characterized by airspaces that have no perceptible wall, centrilobular artery seen as dot in the center.
- Lymphoid interstitial pneumonitis: smooth cysts with ground-glass attenuation and nodules, often associated with autoimmune disease (seen in patients with HIV and Sjogren syndrome).
Pearls:
- LAM is very similar to EG
- Eosinophilic Granuloma. HRCT findings: thin-walled cysts <5mm in size equally distributed in central and peripheral lung zones, centrilobular peribronchiolar nodules. HRCT findings in LAM include numerous randomly scattered thin-walled cysts of various sizes surrounded by normal lung parenchyma, bronchovascular bundles at periphery of cyst walls.
- Classic signs include: coarse reticular interstitial pattern, recurrent large chylothorax (50% to 75%), recurrent pneumothorax (40%), and increasing lung volume (only interstitial disease to develop increasing lung volumes).
- Sarcoidosis may cause progressive retraction and upper lobe fibrosis with bullae, but not cysts.
- Centrilobular emphysema is the most common type of emphysema and presents with bullae predominantly in the upper lobes, whereas the panacinar type is predominantly in the bases and is from alpha 1 antitrypsin deficiency.
- Dähnert 5th p 498 and 501, (515 – lymphangiomatosis)
- Rare disease, It almost exclusively affects women of childbearing age.
- Lymphangioleiomyomatosis is a multi-system disorder and can affect many organs.
- Chest: multiple thin-walled cysts throughout the lungs, usually with a uniform distribution. These are present in nearly all cases. If the cysts are small, they may be seen as diffuse coarse interstitial markings on plain films.
- CT: thin-walled cysts of variable sizes surrounded by normal lung parenchyma can be seen throughout the lung.
- Interlobular septal thickening. May show a dilated thoracic duct. Hemorrhages may be seen as areas of increased attenuation.
References:
- https://radiologykey.com/lymphangioleiomyomatosis/
- https://radiologyassistant.nl/chest/hrct/common-diseases
- https://radiopaedia.org/articles/lymphangioleiomyomatosis-1
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Correct Answer Explanation
QUESTION-7:
42 yo with incidental finding on chest radiograph:
Correct Answer: Thymolipoma
- A large asymptomatic tumor (lack of mass effect)
- Soft encapsulated tumor
- MC anterior inferior mediastinal mass
- Conforms to shape of mediastinum
- Mimics cardiomegaly on CXR
Thymic masses:
- MC thymoma (encapsulated MC, invasive)
- Thymic hyperplasia
- Thymolipoma
- Thymic cyst
- Thymic carcinoma
- Thymic carcinoid
- Invasive thymoma: local extension
- The extent of the tumor within the pleura may be so great as to mimic a mesothelioma
- Thymic carcinoma: hematogenous metastasis
Radiographic Features:
- CXR:
- Large well-defined right anterior inferior mediastinal mass that conforms to the shape of the adjacent mediastinum
CT: - Mass containing fat and soft tissue.
- Little mass on mediastinum, despite large size.
Differential Diagnosis:
- Lipoma
- Liposarcoma
- Omental herniation
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Correct Answer Explanation
QUESTION-8:
Which is least likely to occur in the upper lobes?:
Correct Answer: Alpha-1-antitrypsin deficiency
- Affects the whole lung, but more severe at lung bases
- Dahnert 5th 484-486
- See Dahnert 5th 413-414 for mnemonics for upper and lower zonal predilictions
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Correct Answer Explanation
QUESTION-9:
59 yo male with chronic sinusitis:
Correct Answer: A. Kartagener’s Syndrome
- Case findings:
- CXR: situs inversus, diffuse overinflation and bilateral rounded opacities in lung bases
- HRCT: bilateral bronchiectasis, dilated bronchi in the lower lobes are filled with mucus
- Abnormal ciliary structure with impaired mucociliary clearance
- Triad: situs inversus, paranasal sinusitis bronchiectasis
(ciliary dyskinesia syndrome, immotile cilia syndrome)
Differential Diagnosis:
- BRONCHIECTASIS (saccular & cylindrical types) Infectious - aspergilla, TB, Swyer-James Congenital - cystic fibrosis, Kartagener syn
- Agammaglobulinemia, Wiliams-Campbell,
- Mournier-Kuhn Obstructive - neoplasm, nodes, Chemical - aspiration, inhalation Fibrotic - COPD
Pearls:
- Alpha-1-antitrypsin deficiency: b. Alpha-1-antitrypsin deficiency: affects the whole lung, but is more severe at lung bases Dänhert 5th p. 485
- Interstitial lung disease distribution: (Dänhert 5th p413) upper lobe predominance CASSETTE: cystic fibrosis, ankylosing spondylitis, silicosis, sarcoidosis, eosinophilic granuloma, TB (and centrilobular emphysema)
- Lower lobe predominance: BAD LASS RIF: bronchiectasis, aspiration, dermatomyositis, lymphangitic spread, asbestosis, sarcoid, scleroderma, rheumatoid arthritis, IPF, furadantin
References:
- https://bit.ly/3fmI1kH
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Correct Answer Explanation
QUESTION-10:
50 yo female with hoarseness, dyspnea, and stridor/wheeze.:
Correct Answer: Relapsing polychondritis
- Case findings:
- Tracheal walls are thickened and contain calcifications
- Lumen of the trachea is irregular, deformed and narrowed
- Recurrent inflammatory episodes that affect cartilages of the ears, nose, larynx, trachea and joints
- Result is fragmentation of the cartilages which are replaced by fibrosis
- Diffuse thickening of tracheobronchial wall with diffuse smooth narrowing of trachea and main bronchi
Differential Diagnosis:
- TRACHEAL NARROWING “SPRAT”
- Saber Sheath
- Postintubation Stenosis
- Relapsing Polychondritis
- Amyloidosis
- Tracheobronchopathia Osteochondroplastica - benign
- Calcified nodules
Pearls:
- TRACHEAL DILATION
- Mounier-Kuhn Syndrome (Cong. Tracheobronchomegaly)
- Ehler’s-Danlos Syndrome
- Ehlers-Danlos syndrome hereditary connective tissue disease.
- Clinically manifests with:
- skin hyperelasticity and fragility
- joint hypermobility
- blood vessel fragility with bleeding diathesis
- poor tissue healing with delayed healing with tissue paper-like scarring
References:
- https://bit.ly/3xlhvhY
- https://bit.ly/2VvRwXG
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Correct Answer Explanation
QUESTION-11:
What is the most likely manifestation of thymic carcinoid?:
Correct Answer: Cushing’s syndrome
- Adapted from:
Radiographics. 1999;19:707-736.
CONTINUING EDUCATION
From the Archives of the AFIP
Thoracic Carcinoids: Radiologic-Pathologic Correlation - Approximately one-half of thymic carcinoids are functionally active and manifest with clinical hormone syndromes.
- Cushing syndrome: 33%–40% of affected individuals.
- Type 1 MEN (Wermer syndrome):19%–25% of patients with thymic carcinoids. Type 1 MEN syndrome is characterized by:
- hyperparathyroidism (90% of cases), islet cell tumors of the pancreas (80%), and pituitary adenomas (65%). Carcinoids, adrenal adenomas or carcinomas, lipomas, or follicular thyroid adenomas are less frequently associated neoplasms .
- Additional associated conditions found in patients with thymic carcinoids include type 2 MEN syndrome, inappropriate secretion of antidiuretic hormone, polymyositis, clubbing, polyarthropathy, and myocarditis.
- There are no reports of the carcinoid syndrome in patients with thymic carcinoids.
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Correct Answer Explanation
QUESTION-12:
50 years old male complaining from hemoptysis:
Correct Answer: Squamous cell carcinoma
- Squamous cell carcinoma accounts for ~30-35% of all lung cancers and in most instances is due to heavy smoking.
- Central tumors with invasion and obstruction of bronchi typically result in the distal collapse which may have superimposed infection. Chronic cough and hemoptysis may be present.
- Metastatic disease may be the first sign of malignancy.
Four subtypes are recognized 4: - Papillary
- Clear cell
- Small cell (not to be confused with small cell lung cancer)
- Basaloid
Description :
Radiographic Features:
- Faint veiling opacity on the frontal
- Obliteration of the aortic arch by the collapsed apicoposterior segment
- Displacement of the major fissure and filling in of the anterior clear space on the lateral
Indirect Signs:
- Elevation of the diaphragm
- Juxtaphrenic peak
- Hilar displacement
- Mediastinal shift
- Narrowing of rib interspaces
- Compensatory Hyperaeration
- “Luftsichel” sign: crescentic lucency formed by the superior herniation of the hyperinflated superior segment left lower lobe.
LUL collapses anterior and superior:
Differential Diagnosis:
- Depends on the location of the lesion:
- Hilar masses eg lymph nodes if the lesion is central in position
- Solitary pulmonary nodule differential if it is peripheral location
Pearls:
- squamous carcinomas are encountered more frequently in male smokers.
- The appearance of CT can be variable, depending on whether it is central or peripheral in location.
References:
- https://radiopaedia.org/articles/squamous-cell-carcinoma-of-the-lung
- https://radiologykey.com/tumors-of-the-lung/
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Correct Answer Explanation
QUESTION-13:
50 years old male complaining from cough and fever:
Correct Answer: Bronchocele
- Mucoid impaction from accumulated inspissated secretions within the bronchial lumen. Usually associated with bronchial dilatation.
- Associated with bronchial obstruction – neoplasm, adenoma, and atresia.
- Associated without bronchial obstruction – asthma, cystic fibrosis, and infection
Radiographic Features:
- Accumulation of secretions distal to a long-standing segmental bronchial obstruction
- Causes:
- Congenital atresia
- Bronchial adenoma
- Bronchogenic carcinoma
- Asthma and ABPA
- Fusiform, round or oval densities which demonstrate branching
Differential Diagnosis:
- Endobronchial blood or blood clot appears similar to mucous plugging on CT.
- In some situations, there can be overlap between some endobronchial tumors per se (rather than with associated mucoid impaction).
Pearls:
- Mucoid impaction(bronchocele) may appear as a branching tubular opacity that is distinct from the normal vascular shadows. This classic feature is the finger-in-glove sign and is also seen on CT.
- CT can demonstrate the underlying cause of obstruction.
References:
- https://radiopaedia.org/articles/mucoid-impaction-lung-1
- https://www.cambridge.org/core/books/az-of-chest-radiology/bronchocele/2C14D36D3C44FFA1BA27501280D3CB0D
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Correct Answer Explanation
QUESTION-14:
45 year old female with progressive shortness of breath:
Correct Answer: Lymphangioleiomyomatosis
- Lymphangioleiomyomatosis (LAM) is a rare idiopathic disease that affects women and is characterized by nonneoplastic peribronchial, perivascular, and perilymphatic proliferation of atypical smooth muscle resulting in vascular and airway obstruction, cyst formation, and a progressive decline in lung function
- Commonly presents with spontaneous pneumothorax (up to 50%)
Associated findings:
- Renal angiomyolipoma (up to 57%)
- Mediastinal and retroperitoneal adenopathy
- Ascites
Radiographic Features:
- Round, thin-walled cysts (2-5 mm) surrounded by normal lung parenchyma which may increase in size as the disease progresses
- Diffuse distribution, including costophrenic angle involvement
- Nodules are rare
- Pleural effusions (chylous)
- Pneumothorax
Differential Diagnosis:
- For pulmonary manifestations, the primary differential to be considered is:
- Langerhans cell histiocytosis (LCH)
- tends to happen in children and young adults with a history of heavy cigarette smoking
- mid to upper lobe distribution with preservation of the costophrenic angles
- cysts in LCH tend to be more irregular in contour
- has a much more favorable prognosis compared with LAM
- Lymphocytic interstitial pneumonitis (LIP): smooth cysts with ground-glass attenuation and nodules, often associated with autoimmune disease
Pearls:
- More common in females
- Commonly presents with spontaneous pneumothorax (up to 50%)
References:
- https://pubs.rsna.org/doi/10.1148/rg.253055006
- https://radiopaedia.org/articles/lymphangioleiomyomatosis-1
- https://emedicine.medscape.com/article/358917-overview
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Correct Answer Explanation
QUESTION-15:
5 years old female complaining from seizures and skin pigments:
Correct Answer: Tuberous sclerosis
- TS has a wide variety of clinical and radiologic manifestations. It should be suspected when some of the common manifestations are found, including CNS involvement, renal AML, and cardiac rhabdomyoma, even if clinical signs are not obvious. Familiarity with the clinical course, sites of potential involvement, and frequency of involvement can allow correct treatment and improvement in quality of life. Recognition of radiologic features of various organ manifestations is essential for making the correct diagnosis and is helpful for detecting additional abnormalities.
- Definitive TS complex: either 2 major features or 1 major and 2 or more minor
- Possible TS complex: either 1 major or ≥2 minor
Major Features:
- angiofibromas (3 or more) or fibrous cephalic plaque
- non-traumatic ungual or periungual fibroma (2 or more)
- hypomelanotic macules (3 or more, at least 5 mm diameter)
- shagreen patch
- multiple retinal nodular hamartomas
- cortical dysplasias (include tubers and cerebral white matter migration lines)
- subependymal nodule
- subependymal giant cell astrocytoma
- cardiac rhabdomyoma
- lymphangioleiomyomatosis (LAM)
- angiomyolipomas (2 or more)
Minor Chnages:
- dental enamel pits: 3 or more for the entire dentition
- intraoral fibromas (2 or more)
- non-renal hamartomas
- retinal achromic patch
- 'confetti' skin lesions
- multiple renal cysts
Radiographic Features:
- Pulmonary findings are indistinguishable from LAM
- Findings:
- CNS: Subependymal nodules, giant cell astrocytoma and cortical tubers
- Skin: Adenoma sebaceum, Shagreen patch, ungual fibromas
- GU: Angiomyolipoma, cysts, renal cell carcinoma
- Cardiac: Rhabdomyoma
Differential Diagnosis:
- Depends on the location of the lesions.
Pearls:
- Tuberous sclerosis was classically described as presenting in childhood with a pathognomonic triad (Vogt triad) of:
- seizures: absent in one-quarter of individuals
- intellectual disability: up to half have normal intelligence
- adenoma sebaceous: only present in about three-quarters of patients
References:
- https://radiopaedia.org/articles/tuberous-sclerosis-diagnostic-criteria-1?lang=us
- https://pubs.rsna.org/doi/10.1148/rg.e32
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Correct Answer Explanation
QUESTION-16:
40 years old man complain from shortness of breath::
Correct Answer: Eosinophilic granuloma
- Eosinophilic granuloma, also known as pulmonary histiocytosis X (PHX) or pulmonary Langerhans cell histiocytosis X (PLCH), is an uncommon interstitial lung disease that is epidemiologically related to tobacco smoking. It chiefly affects young adults, primarily occurring in the third or fourth decades of life.
Radiographic Features:
- Nodular (1-15 mm) or reticulonodular pattern on chest radiograph with a middle and upper lung predominance
- Subsequent necrosis and cavitation of the nodules is best visualized with CT, and later becomes the dominant finding
- Irregular cystic airspaces (typically 1- 2 cm, up to 5 cm)
- The resultant increase in lung volumes with disease progression
- Adenopathy and pleural effusions are rare
- Spontaneous pneumothoraces (6-25%)
Differential Diagnosis:
- Differential depends on whether the nodular or cystic change is the dominant feature.
Early in the disease, when nodules are the dominant feature, consider:
- granulomatous disease
- granulomatosis with polyangiitis
- sarcoidosis
- metastases
- miliary tuberculosis
Later in the disease, when cysts are prominent, consider:
- lymphangiomyomatosis (LAM)
- cystic bronchiectasis from ABPA
- centrilobular emphysema
- Pneumocystis jiroveci pneumonia
- idiopathic pulmonary fibrosis
- lymphocytic interstitial pneumonitis (LIP)
References:
- https://emedicine.medscape.com/article/298601-overview
- https://radiopaedia.org/articles/pulmonary-langerhans-cell-histiocytosis
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Correct Answer Explanation
QUESTION-17:
40 years male worker complaining from cough::
Correct Answer: Mesothelioma
- Malignant pleural mesothelioma (MPM) is the most common primary tumor of pleura and its association with asbestos exposure has been well established.
- Imaging plays an important role in the diagnosis, treatment planning, and post-treatment assessment of MPM. Although CT is the first-line imaging modality for the evaluation of MPM, it has its limitations. A variety of benign and malignant pleural diseases may cause pleural abnormalities that resemble MPM, and thus, early diagnosis is often difficult.
Radiographic Features:
- Nodular pleural thickening, which may coalesce to form a diffuse circumferential rind of lobular soft tissue with associated pleural fluid
- Ipsilateral volume loss without mediastinal shift
- Invasion of chest wall, mediastinum, diaphragm and upper abdomen
Differential Diagnosis:
- pleural effusion (especially if loculated): on radiographs
- pleural metastases (especially with pleural carcinomatosis)
- peripheral bronchogenic carcinoma
- solitary fibrous tumor of pleura
- other pleural based tumors
- tumor like conditions of the pleura
- benign asbestos-related pleural disease
- pleural fibrosis from infective/inflammatory source (e.g. actinomycetes, tuberculosis)
Pearls:
- Mesothelioma has close relation to asbestous exposure
References:
- https://radiopaedia.org/articles/mesotheliomahttps://radiologykey.com/tumors-of-the-lung/
- https://www.kjronline.org/DOIx.php?id=10.3348/kjr.2016.17.4.5
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Correct Answer Explanation
QUESTION-18:
35years old male complaining from dyspnea:
Correct Answer: Sarcoidosis
- Sarcoidosis is a non-caseating granulomatous multisystem disease with a wide range of clinical and radiographic manifestations. ##3Pulmonary involvement is reported in up to 90% of patients with sarcoidosis and generally manifests as asymptomatic mediastinal adenopathy Hilar adenopathy is easily recognized on chest radiographs. However, CT is superior for demonstrating subtle mediastinal lymphadenopathy and associated parenchymal involvement. Because the prevalence of pulmonary involvement in patients with sarcoidosis is extremely high.
Radiographic Features:
- Eggshell Node Calcification: Annular peripheral nodal calcification found in:
- Silicosis
- Sarcoid
- Blastomycosis and Histoplasmosis (rare)
Differential Diagnosis:
- Lymphoma
- Silicosis
Pearls:
- Bilateral hila lymphadenopathy with fine calcifications is the most common chest manifestation of sarcoidosis, sometimes associated with pleural effusion or pulmonary infiltrates.
References:
- https://radiopaedia.org/articles/sarcoidosis-1
- https://pubs.rsna.org/doi/10.1148/rg.241035076
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Correct Answer Explanation
