This document describes the case of a 23-year-old man who presented with a posterior mediastinal mass. He experienced coughing and difficulty breathing when climbing stairs but did not seek medical attention. Imaging revealed a large right para cardiac mass. He underwent surgery to remove the mass, and pathology found small round blue cells. Immunohistochemistry testing supported a diagnosis of ganglioneuroblastoma. He received chemotherapy but developed complications including pneumonia and hypomagnesaemia. Further treatment will include evaluation after completing four cycles of chemotherapy.

1. A 23 YEAR YOUNG MAN
PRESENTED WITH POSTERIOR
MEDIASTINAL MASS
Dr. S.M. Syfuzzaman
Phase B resident
Clinical Oncology
BSMMU

2. Particulars of the patient
 Name: Rezwan Islam
 Age: 23 years
 Sex: Male
 Religion: Islam
 Address: Jatrabari, Dhaka
 Marital status: Unmarried
 Occupation: Student

3. Background history
This patient was referred to us from cardiothoracic surgery with post-operative
malignant posterior mediastinal mass with history of incidental chest radiograph
findings of large right para cardiac mass.

4. On query patient informed us that he experienced dry cough, and difficulty in
respiration during climbing stairs. But he ignored the complain and did not
attend any physician. He had no H/O major physical or psychological illness.
All of his family members are in good health.
He is non-smoker.

5.  No history of -
 Fever
 Hemoptysis
 Yellow discoloration of sclera and urine
 Bony pain
 Trauma to the chest
 Abdominal pain
 Abdominal swelling
 Headache
 Seizure

6. General Examination
• Appearance: Normal
• Body built : Average
• Co-operation : Co-operative
• Decubitus : On choice
• Nutritional status : Average
• Height : 5 feet 5 inch

7. General Examination (contd.)
• Anaemia : (+)
• Jaundice : Absent
• Cyanosis : Absent
• Oedema : Absent
• Dehydration : Absent
• Koilonychia : Absent
• Leukonychia : Absent

8. General Examination (contd.)
• Clubbing : Absent
• Thyroid gland : Not enlarged
• JVP : Not raised
• Skin condition : Normal
• Hair distribution : Normal
• Bony tenderness : Absent

9. General Examination (contd.)
• Pulse : 80 beats/min
• BP : 110/70 mm Hg
• Temp. : 98.5°F
• Resp. rate : 16 breaths/min

10. Examination of Respiratory System
Inspection:
• Shape of the chest- Normal with there is a linear scar mark over
the posterior aspect of the right side of the chest
• Resp. rate- 16 breaths/min
• Chest movement- Restricted on right lower zone
• No visible pulsation
• No suprasternal, intercostal or subcostal indrawing

11. Examination of Respiratory System
Palpation:
• Trachea- Centrally placed
• Apex beat - Present in left 5th intercostal space just
medial to mid-clavicular line
• Chest expansion- Reduced on the right lower zone.
• Vocal fremitus- Deceased on the 8th ICS to
downwards along the dorsal scapular line.

12. Examination of Respiratory System
Percussion:
• Dull on the 8th ICS to downwards along the dorsal
scapular line.
Auscultation:
• Breath sound- Diminished on the 8th ICS to
downwards along the dorsal scapular line.
• Vocal resonance- Diminished on the 8th ICS to

13. Examination of the
other system reveals no
abnormality.

14. Pre-operative CT scan of chest with
contrast show
There is a fairly large wedge shaped pleural based soft
tissue density mass having internal calcification in medial
aspect of right hemithorax at paravertebral location
measuring about 15.2 * 9.2 cm.
The lesion having a sharp interface with surrounding lung
parenchyma. medially the lesion invades the mediastinum.
Superiorly the lesion extends into the right hilum. Vertically
the lesion extends from D6 to D11 level.
Post contrast scan revealed strong enhancement

16. At this stage patient underwent surgical excision of this mass
at cardiothoracic department, BSMMU. According to their OT
(19/3/23) note- About 80-90% of the tumour was removed.
And the mass attached to posterior chest wall which was
lobulated and firm in consistency.
.

17. And Post surgical histopathology report:
Section shows a malignant neoplasm composed of mostly
small round blue cells having scanty cytoplasm. Salt
and pepper chromatin and some having prominent
nucleoli

18. What is the Provisional
diagnosis.
?

19. Differential Diagnosis:
 NHL
 Neuroblastoma
 Rhabdomyosarcoma
 Small cell lung carcinoma
 Hepatoblastoma
 Ewing Sarcoma

22. Immunohistochemistry report
 Synaptophysin: (+ve)
 Chromogranin A: (+ve)
 CD 56 : (+ve)
 S100 Protein: : (+ve)
Comment: Ganglioneuroblastoma, Intermixed

24. Post-operative CT scan of chest with
contrast
Large hypodense solido-cystic mass lesion (15 * 7 cm) with
mild enhancement after contrast is seen in the posterior
mediastinum of right hemi-thorax with prevertebral and
right para spinal extension from D6 to D11 vertebrae.
Impression: Status post operative posterior mediastinal and
para spinal mass, present CT shows:
Suggestive of recurrence or residual tumour in the right
posterior mediastinum extending along the right pre
vertebral and para spinal region from D6 to D11 vertebrae.

26. Contrast enhanced CT scan of Abdomen:
Normal CT findings of upper abdomen.
MRI of Brain: Normal study
Routine biochemical and hematological investigation is within normal limit.

27. Confirm Diagnosis:
Neuroblastoma

29. Neuroblastoma
Neuroblastoma originates from primordial neural crest cells that normally give
rise to adrenal medulla and sympathetic ganglia.
Epidemiology:
 Most common extra cranial solid tumour in children.
 Accounting for 6% of all childhood malignancy.
 Can undergo spontaneous regression.
 Differentiate to benign neoplasm or exhibit extremely malignant behaviour.

30. Site
 Adrenal gland,
 Sympathetic chain
 Neck
 Thorax
 Retroperitoneum
 Pelvis

31. Pathology and biology:
 Diagnosis based on presence of characteristics, histopathologic features of
tumour tissue or presence of tumour cells in a bone marrow aspirate or
biopsy. Accompanied by elevated level urinary catecholamines.
 One of the small round blue cell tumour of childhood. Histologically favorable
or unfavorable.
Histologic spectrum:
- Neuroblastoma
- Ganglioneuroblastoma
- Ganglioneuroma

33. Genetics:
 Autosomal dominant pattern of inheritance.
 In familial cases median age decreases to 9 month.
 Amplification of NMYC oncogene seen in roughly 20%.
 Familial neuroblastoma have bilateral adrenal or multifocal primary tumour.

34. Clinical features
 Usually presents with an adrenal mass or a tumour arising in the sympathetic
neural chain.
 Asymptomatic abdominal mass with distant metastasis at the time of
diagnosis in 75% of cases.
 Clinical findings related to anatomic site of the tumour of origin.

38. Diagnosis:
 Routine hematological and biochemical test
 Urinary catecholamines
 Imaging: CT or MRI
 Tumour biopsy
 Metastatic workup:
 MRI of chest/Abdomen/Pelvis
 123I MIBG scintigraphy
 Bone marrow study
 PET scan

39. Staging:

40. Neuroblastoma in adolescent and
young adult
 Rarely Developed, <5%
 More indolent clinical course
 Shows De Novo chemoresistance.
 May also exhibit unusual clinic-pathological characteristics
 Large tumour
 Bilateral adrenal disease
 Pheochromocytoma like features
 Worse long time prognosis : 5 year overall survival rate is 38%

41. Contd:
 Aggressive chemotherapy and surgery have shown to achieve a minimal
disease state in 50% of patients
 Other modalities:
 Local radiation therapy, Autologous stem cell transplantation.
 Anti GD2 immunotherapy was good.
 Genetic abnormalities include:
 Somatic ATRX (58%) and ALK (44%) mutation but no MYCN amplification

42. Treatment options
 Low risks:
 Surgery followed by observation
 Observation +/- Biopsy
 CT +/- Surgery for Symptomatic disease or unresectable progressive disease after surgery.
 Intermediate risk:
 CT +/- Surgery (CT: Carboplatin, Doxorubicin, Etoposide – 2,4,8 cycles)
 Surgery and Observation
 RT
 High risk disease:
 CT followed by Surgery
 Myeloablative therapy and stem cell transplantation.
 RT
 Dinutuximab with GM-CSF and isotretinoin

43. Treatment of our patient
.

45. Systemic chemotherapy with OPEC/OJEC (4+3
cycles)→Evaluation for Surgery

47. PET Scan (26/4/23)
1. Non- avid soft tissue mass in postero- basal segment of right lower lung
extending down to retro-crural space at the level of D12 vertebra suggest post-
surgical post-chemotherapeutic residual fibrosis suggest good response to
therapy.
2. Low avid focal hypermetabolic uptake in posterior chest wall in between right
6th to 7th ribs suggest post surgical sequelae.
3. Hypermetabolic bilateral cervical level IB lymph nodes suggests reactive nodes
4. No other visceral, nodal, osseous hypermetabolic lesion is noted anywhere else
in the body.

48. Events of patient during chemotherapy:
 After 2nd cycle chemotherapy, patient developed severe respiratory distress
with fever and loose motion with hypertension. And diagnosed as right sided
pneumonia with hypomagnesaemia with thrombocytopenia and managed
accordingly at ICU outside BSMMU.
 During 3rd cycle CT, Patient developed hypertension and palpitation.
BP: 180/100mmHg
P: 150BPM

49. Investigation
 CK-MB: 19.2U/L
 S. creatinine: 0.95mg/dl
 Electrolytes:
 Sodium: 138mmol/L
 Potassium: 3.8mmol/L
 Chloride: 117mmol/L
 S. Troponin-I: <0.01ng/ml
 ECG: Sinus tachycardia
 And treated with Tab. Bisoprolol 5mg one daily.

50. What may be the
cause of this
scenario ?

51. Our further plan of treatment
Evaluation after 4th cycle of
systemic chemotherapy.

52. Thank you!