KGMU

1. CASE PRESENTATION
• MODERATOR
• Dr. SUKRITI KUMAR
• Assist. Professor
• PRESENTER
• Dr. RAHUL KM. TIWARI
• JR III

2. CASE
• 22yr old male patient present with Scalp swelling
which is gradually increasing in size associated with
On and Off headache since 1 year.
• Few episodes of severe abdominal pain associated
with vomiting on exertional activities since 8
months.

3. USG

5. NCCT

6. CECT

8. MRI

9. Differentials
• Malignant Paraganglioma
• Ganglioneuroblastoma

10. Posterior Mediastinum
• Contains following structures: sympathetic ganglia,
nerve roots, lymph nodes, parasympathetic chain,
thoracic duct, descending thoracic aorta, small vessels
and the vertebrae.
• Most are NEUROGENIC in nature.
• Can arise from the sympathetic ganglia (eg neuroblastoma) or
from the nerve roots (eg schwannoma or neurofibroma).
• CYSTIC LESIONS will be either neuroenteric cysts,
schwannomas or meningoceles.
• FAT CONTAINING LESIONS will be extramedullary
hematopoiesis. When the anemia is resolved the
extramedullary marrow will stop producing blood and
become fatty.

11. Neurogenic Tumors
• MC cause of posterior mediastinal masses.
• 20% of all adult and 35% of all pediatric mediastinal neoplasms
are due to neurogenic tumors.
• Broadly, these lesions can be classified as:
1. TUMORS OF THE PERIPHERAL NERVES
(neurofibromas, schwannomas, malignant tumors of nerve sheath
origin),
2. TUMORS OF SYMPATHETIC GANGLIA
(ganglioneuromas, ganglioneuroblastomas,
neuroblastomas),
3. TUMORS OF PARASYMPATHETIC GANGLIA
(paraganglioma, pheochromocytoma).
• Peripheral nerve tumors are more common in adults,
• Sympathetic ganglia tumors are more common in children.

12. Sympathetic ganglia tumors
NEUROGENIC TUMORS
• They appear as well-circumscribed, smooth or lobulated
masses that may contain calcifications.
• Ganglioneuromas and ganglioneuroblastomas usually manifest
as well-marginated, elliptical, posterior mediastinal masses
that extend vertically over three to five vertebral bodies.
• Usually located lateral to the spine and may cause pressure
erosion on adjacent vertebral bodies

13. • CT has proven to be the superior imaging technique when
identifying tumor size, organ of origin, tissue invasion,
vascular encasement, adenopathy, and calcifications.
• MRI is the modality of choice for evaluating intraspinal
extension
• Measurement of catecholamine metabolites in the urine and
histological examination of biopsy specimens allow
definitive diagnosis.
• Distant metastases is assessed by using MIBG scintigraphy.

14. NEUROBLASTOMA
• They account for 8–10% of all tumors diagnosed in
pediatric patients and 80% of those found in children under 5
years of age .
• They rarely develop in children over 10.
• Males are affected more frequently than females .
• The tumors can arise wherever sympathetic nerve tissue is
present. The most common locations include the adrenal
glands (35%), paraspinal retroperitoneal ganglia (30–35%),
posterior mediastinum (20%), head and neck (1–5%), and
the pelvis (2–3%)

15. • Posterior mediastinum is also the most common extra-
abdominal location of neuroblastomas
• Neuroblastomas are highly malignant tumors that typically
occur in children younger than 5 years.
• A posterior mediastinal mass in this age group should be
considered a neuroblastoma until proved otherwise.
• On CT neuroblastomas manifest as paraspinal masses of
heterogeneous, predominantly soft tissue attenuation, contain
areas of hemorrhage, necrosis, cystic degeneration, and
calcification (30%).

16. • MR: heterogeneous signal
intensity on all pulse sequences
and show heterogeneous
enhancement following
gadolinium administration.
• Neuroblastomas also have a
tendency to cross the midline
• Around 1% of all neuroblastic
tumors metastasize, generally via
the vascular or lymphatic system.
• Common sites of metastatic
involvement are the liver, lung,
bone, and bone marrow. Patient
age and tumor stage at diagnosis
are major determinants of
outcome
T2-weighted MRI large mass in the posterior mediastinum

17. GANGLIONEUROBLASTOMA
• Ganglioneuroblastoma is a rare variety of peripheral neuroblastic
tumor that can arise anywhere along the sympathetic nervous
system.
• It occurs almost exclusively in the pediatric population usually 5-
10 years.
• It exhibit varying degrees of malignancy and is usually aggressive
with evidence of local and intraspinal invasion.
• Variable appearance on CT scans and can be cystic or solid.
• They may be small and homogenous or large and heterogenous .
• They appear heterogeneous on MRIs, with variable enhancement
and low signal-intensity on T1-weighted images and high signal-
intensity on T2-weighted images

18. • Ganglioneuromas can be
differentiated from more
aggressive neuroblastomas and
ganglioneuroblastomas by their
regular contours and lack of tissue
invasion and vessel encasing,
their occurrence in older patients,
and their discrete, punctate
calcifications on CT scans.
• Ganglioneuromas rarely
metastasize, whereas
neuroblastomas and
ganglioneuroblastomas can
metastasize to bone, skin, and
other organs.

19. PARAGANGLIOMAS
• Paragangliomas, sometimes called extraadrenal
pheochromocytomas, are rare neurogenic tumors
that arise from highly vascularized specialized
neural crest cells called paraganglia that are
symmetrically distributed along the aortic axis in
close association with the sympathetic chain in the
neck, chest, abdomen, and pelvis.
• The largest collection of paraganglia includes the
paired organs of Zuckerkandl that overlie the aorta
at the level of the inferior mesenteric artery
19

20. • Patients with paragangliomas present in the fourth
and fifth decades of life, although malignant
paragangliomas may sometimes arise in younger
patients.
• Men and women are affected equally.
• Up to 40% of paragangliomas are malignant, as
compared to 10% of adrenal pheochromocytomas.
• Paragangliomas may spread both via the lymphatics
and hematogenously, and the most common sites of
metastatic disease are lymph nodes, bone, lung, and
liver
20

21. LOCATION
Parasympathetic paragangliomas
• Parasympathetic paragangliomas
arise within paraganglia of the head
and neck in association with the
branches of the glossopharyngeal
and vagus nerve .
• Carotid body paraganglioma
• Juglotympanic paraganglioma
• Vagal paraganglioma
• Laryngeal paraganglioma
21

22. Sympathetic paragangliomas
• Arise in paraganglia below the level of the neck.
secrete catecholamines and can be intra- or extra-
adrenal.
• Extra-adrenal: arise outside the adrenal gland along
the length of the sympathetic chain
• abdomen
• organ of Zuckerkandl
• bladder base
• thorax (mediastinal paraganglioma)
• paravertebral (aortosympathetic paraganglia)
• great vessels of the chest (aortopulmonary
paraganglia)
• cardiac (extremely rare)
• intra-adrenal: arise within the adrenal medulla
• phaeochromocytoma
22

23. CLINICAL PRESENTATION
• Sympathetic paragangliomas present with features
of catecholamine-excess, such as headaches,
palpitations, diaphoresis and hypertension.
• Whereas, parasympathetic paragangliomas present
more commonly with mass-effect such as cranial
nerve palsies, a neck mass or tinnitus.
23

24. GENETICS
Paragangliomas are the most strongly hereditary group of tumors.
Most common genetic cause of hereditary paragangliomas are
mutations in the succinate dehydrogenase (SDH)
They are also associated with four clinical syndromes:
• Von Hippel-lindau Syndrome
• Multiple Endocrine NeoplasiaTypes 2AAnd 2B
• Neurofibromatosis Type 1
• Carney-stratakis Syndrome (AD Condition comprising of
familial paraganglioma and gastric stromal sarcoma)
24

25. Both anatomical and functional imaging of paragangliomas is
required for diagnosis and staging.
• Anatomical imagining includes CT and MRI.
• Functional imaging modalities includes: 123I-MIBG scintigraphy
, 18F-FDA PET, 18F-DOPA PET
• CT: typically heterogeneous and enhance
intensely after iv contrast administration.
• MR: Hypointense on T1WI
Hyperintense on T2WI
• Flow voids are noted sometimes s/o
high vascularity.

26. PARAGANGLIOMA
Points in Favour Points Against
• Hetrogenously
enhancing mass lesion
with central areas of
necrosis
• Bony,calvarial and lung
metastasis
• Clinical history- Severe
abdominal pain on
exertion
• Oblong hetrogenously
enhancing mass lesion
extending along 3 to 4
vertebral level.

27. GANGLIONEUROBLASTOMA
Points in favour Points against
• Heterogenously
enhancing oblong soft
tissue lesion
• Bony, calvarial and
lung metastasis
• Incidence –rare variety
• Age- 5-10 years
• Extension in spinal
canal causing widening
of NF
• Calcification

28. CASE SUMMARY
• 22 yr old male presented with slowly worsening
headache and scalp swelling along with severe
abdominal pain on exertional activities.
• On Imaging- het. enhancing mass lesion with
internal non enhancing necrotic area in posterior
mediastinum with calvarial, bony and lung
metastasis
• No e/o calcification or extension into neural
foramina is noted.

29. DIAGNOSIS
• NEUROGENIC TUMOR
(PARAGANGLIOMA)
HPE is awaited

30. QUESTIONS

31. Q1. which of the following is true about ganglioneuroma is ,
except:
A. Ganglioneuromas are the most common posterior mediastinal
mass in adolescents and young adults
B. They are malignant tumors originating from sympathetic
ganglia.
C. well-marginated posterior mediastinal masses that extend
vertically over three to five vertebral bodies.
D. usually located lateral to the spine and may cause pressure
erosion on adjacent vertebral bodies
Ref-John .R.Haaga’s CT and MRI of the whole body,6th edition, 2016,
1st volume pg 1058

32. • ANS 1-B
Q2. Which of the following signs indicate mass in posterior
mediastinum?
A. Widened paratrachel strip
B. Doughnut sign
C. Cervicothoracic sign
D. Obliteration of anterior junctional line
REF: Grainger and Allison’s diagnostic radiology, 6th edition,
2015, 1st vol ,pg233

33. • ANS 2-C
Q3. Which of the following is false about neuroblastoma?
A. Neuroblastomas are highly malignant tumors that typically
occur in children younger than 5 years
B. Neuroblastomas have a tendency to cross the midline
C. Common sites of metastatic involvement are the liver, lung,
bone and bone marrow
D. Calcification is not seen in neuroblastoma
Ref-John .R.Haaga’s CT and MRI of the whole body,6th edition, 2016,
1st volume pg 1058

34. • ANS 3-D
Q4.Which of the following sign is
shown in the given image ?
A. Hilum overlay sign
B. Anterior junctional line
C. Doughnut sign
D. Azygoesophageal recess
REF: Grainger and Allison’s diagnostic
radiology, 6th edition, 2015,
1st vol ,pg233

35. • ANS 4-C
Q 5. Which of the following sign is
shown in the given image ?
A. Hilum overlay sign
B. Cervicothoracic sign
C. Doughnut sign
D. Azygoesophageal recess
REF: Grainger and Allison’s diagnostic
radiology, 6th edition, 2015,
1st vol ,pg233

36. Ans5. B
Q6. A 52-year-old man with cough and
dysphagia On CECT images
demonstrate a low-density mass with a
cystic appearance in the subcarinal
region that has a mild mass eff ect on
the esophagus, which is seen between
the aorta and the cystic mass
A. Bronchogenic cyst (BC)
B. Esophageal (enteric) duplication
cyst
C. Pericardial cyst
D. Thymoma

37. Ans6. A
Q7. A 50-year-old man with back
pain.chest radiograph demonstrates a
large, well-circumscribed mass in the
left upper chest ,NCCT shows that the
mass has heterogeneous density. It
forms obtuse angles with the pleura,
suggesting an extrapulmonary
location.
A. Schwannoma
B. Meningocele
C. Lymphoma
D. Ganglioneuroma

38. Ans7. A
Q8.Most common site of paraganglioma in
retroperitoeum-
a). Anterior to aorta at the level of origin of superior
mesenteric artery
b). Anterior to aorta at the level of origin of inferior
mesenteric artery
c). Anterior to aorta at the level of origin of celiac axis
d).Posterior to aorta at the level of origin of inferior
mesenteric artery
Ref-radiographics, imaging of uncommon retroperitoneal
masses, July-August 2011

39. Ans8. B
Q9.Site of origin of ganglioneuroma-.
a). Parasympathetic ganglia
b).Sympathetic ganglia
c). Both A and B
d).Chemoreceptor
Ref-radiographics, imaging of uncommon retroperitoneal
masses, July-August 2011

40. Ans9. B
Q10. A 49-year-old woman
with muscle weakness.CT
chest demonstrates a well-
circumscribed, smooth,
hypodense mass in the anterior
mediastinum. There is no
evidence of vascular or pleural
involvement.
A. Thymoma
B. Seminomas
C. Thymic carcinoma
D. Teratoma

41. THANK YOU