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Published by irianewsletter, 2021-10-10 04:44:22

11th Oct 2021

Case of the Week

Keywords: Case,week,IRIA,ICRI

ICRI CASE OF THE WEEK

Contributed By : Dr N. Eshwar Chandra

MD DNB FRCR

Virinchi Hospitals, Hyderabad

Copyright of the Case belongs to : Dr N. Eshwar Chandra

History

• 11 year old male child with shortness of breath and
right side chest pain

Frontal Chest Radiograph & CT Topogram

Axial CT Chest – Non-contrast

Axial CT Chest – Contrast

Axial CT Chest – Lung Window

What is your Diagnosis ?

1) Germ cell tumour
2) Pleuro-pulmonary blastoma
3) Rhabdomyosarcoma
4) Non Hodgkin's lymphoma

Answer

• Pleuro-Pulmonary Blastoma

Frontal Chest Radiograph & CT Topogram

Large homogeneous opacity filling almost the entire right hemithorax, causing
mediastinal shift to the left, and compressing the right lower lobe parenchyma

Axial CT Chest – Non-contrast

Large heterogeneous soft tissue mass in the right hemithorax, with foci of amorphous
calcifications involving the right upper lobe, and causing mediastinal shift to the left and
compressing the right lower lobe parenchyma

Axial CT Chest – Contrast

Heterogeneous enhancement of the mass is seen associated with moderate right
basal pleural effusion. Right lower lobe shows passive collapse

Axial CT Chest – Lung Window

Discussion

• Large thoracic masses in children can be classified based on their origin
in one of the three broad compartments:
1. Chest wall and pleura 2. Lungs and airways 3. Mediastinum

• Most common paediatric thoracic masses include lymphoma,
germ cell tumours, neurogenic tumours, congenital lesions
(such as foregut cysts, congenital pulmonary airway malformations),
vascular malformations, haemangiomas, chest wall sarcomas,
pleuropulmonary blastomas, inflammatory myofibroblastic tumour etc

Discussion: Differential Diagnosis

• Lymphoma is the most common anterior mediastinal mass in children.

• Hodgkin’s lymphoma typically occurs before the age of 10 years, 85% cases show
intrathoracic involvement and most commonly involves the antero-superior mediastinal
lymph nodes.

• Non-Hodgkin’s lymphoma is common in both first and second decades of life, 50% show
intrathoracic involvement and equally involve the anterior and posterior mediastinal
lymph node chains.

• Calcification is extremely rare in untreated tumours.

• Germ cell tumor is the 2nd most common anterior mediastinal tumour after
lymphoma, & teratomas account for 60% of tumours. Well defined with
presence of fat, fluid and calcification. Calcium is seen in 25% of the lesions.

• Rhabdomyosarcomas are the most common soft tissue tumours in children.
However thoracic involvement is uncommon (approx. 7%) and usually involve
the chest wall.

Discussion: Pleuro-Pulmonary Blastoma

• Primary lung neoplasms are rare in children. Lung masses in children
are approximately 10 times more likely to represent a benign
developmental or reactive lesion than a neoplasm, with a ratio of
primary tumours to metastatic tumours to non-neoplastic lesions of
1:5:60.

• The most common primary lung malignancies in children are
Pleuropulmonary blastoma and Carcinoid tumour

Discussion: Pleuro-Pulmonary Blastoma

• Pleuro-pulmonary blastoma (PPB) is the most common primary lung malignancy in
children. Pleuro-pulmonary blastomas are rare paediatric thoracic tumours most
often seen in the first two years of life and rarely after the age of 12 years

• Three types are described based on the morphology: type I (cystic), type II (mixed)
and type III (solid), with the solid type representing the most malignant form

• Pleuropulmonary blastomas are usually right-sided, pleural-based, without chest
wall invasion or calcification

• Radiographs reveal a opaque hemithorax with mediastinal shift to the opposite side
• CT reveals the lesion of mixed attenuation associated with pleural effusion and no

signs of chest wall infiltration

Discussion: Differential Diagnosis

• Type I tumours which are predominantly cystic: Other cystic
lesions such as Congenital pulmonary airway malformation (CPAM)

Type I CPAM & type I PPB are indistinguishable at imaging.
However, congenital lung malformations are seen most often in
foetuses during the second trimester, whereas PPB is more often
discovered postnatally

• Type III lesions: Neuroblastoma
Chest wall invasion favours neuroblastoma over PPB. Other rarer
differentials include intrathoracic soft tissue sarcoma, primitive
neuroectodermal tumour (PNET) of the thorax etc

References

1. Orman G, Masan P, Hicks J, Huisman TAGM & Guillerman RP
Pediatric thoracic masses: Beyond the common
Eur J Radiol Open 7 100240

2. Lichtenberger JP III, Biko DM, Carter BW, Pavio MA, Huppmann AR & Chung EM
Primary Lung Tumors in Children: Radiologic-Pathologic Correlation From the Radiologic
Pathology Archives
Radiographics 2018;38:2151-2172

3. Rangnath SH, Lee EY, Restrepo R & Eisenber RL.
Mediastinal masses in children
AJR 2012; 198: W917 – W216

4. Dishop M. K., Kuruvilla S.
Primary and Metastatic Lung Tumors in the Pediatric Population: A Review and 25-Year
Experience at a Large Children's Hospital.
Arch Pathol Lab Med 2008; 132 (7): 1079–1103


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