nurofibromatosis as pancoast syndrome in ayoung boy

1. CENTRAL CASE
PRESENTATION

2. Dr. Tanveer Kamal Fahim
Resident , Phase B
Pulmonology
MU-VII, NIDCH
Evaluation of An
Apical Shadow of Lung in
a Boy

3. Particulars of the Patient
Master Jobayer Ahmed
16 years
Student of class IX
Kishoregonj
Admitted on 20/1/20 , MU-VII

4. Chief Complaints
Pain & weakness in the left upper limb & shoulder
for 6 months

5. History of Present Illness
Pain in the left upper limb & shoulder
Gradual onset , for 6 months
Started at left upper arm and shoulder & radiated
along inner aspect of fore arm and wrist
Recurrent , persists for days
Dull aching

6. Moderate to severe intensity
Not aggravated or relieved by movement , activity
or rest of the arm , shoulder , no diurnal variation
Not relieved fully by analgesic

7. WEAKNESS
 Weakness in the left upper limb for last 6 month
Initially progressively increasing for 2-3 months
Now , static
Not related with activity , exercise , posture
No diurnal variation , drooping of eyelid , muscle pain
No H/O of steroid intake

8. No History of
• Headache
• Trauma
• Visual impairment
• Shortness of breath
• Fever
• Involvement of lower limb
• Cough , Coughing out of blood
• Palpitation
• Voice change
• Impaired consciousness
• Heat or cold intolerance
• Passage of black tarry stool
• Abdominal pain
• Joint pain / swelling
• Rashes
• Abnormal muscle movement
• Bowel bladder abnormality
• Weight loss
• TB or contact with TB
patient

9. History of Past Illness :
Nothing significant
Family History :
Parents and one sibling ( brother )
All are in good health
No history of abnormal mode of death in family

10. Personal History :
Student of class IX
Socio-Economic History :
Middle class family
Pacca house , good sanitation , Arsenic free
water

11. Immunization History :
Well Immunized
Treatment History :
Analgesics , cant mention names

12. GENERAL EXAMINATION

13. Ill Looking
Lean thin
Co-Operative
Pulse : 96 beaths / min
Blood Pressure : 90/60 mm Hg
Temperature : 98.6 degree Fahrenheit
Respiratory rate : 18 / min

14. CAFÉ-AU-LAIT Spots
Multiple smooth , flat , non tender,
coffee like light brown colored
Hyperpigmented patches of various
size and shape present over lateral
surface of left shoulder , since birth

15. Ill-defined mass felt on palpation in the left
supraclavicular fossa
soft in consistency
smooth surface
non tender
margin not identified
3*3 cm diameter
overlying skin free , fixed with deeper
structures

16. RESPIRATORY SYSTEM EXAMINATION

17. Inspection :
Normal in shape and movement
Presence of Café-Au-Lait spots in lateral surface of
left shoulder
Palpation :
Trachea : Central
Apex beat : Left 5th ICS , medial to midclavicular line
Chest Movement : Symmetrical on both side
Vocal fremitus : Equal on both side

18. Percussion Note :
Resonant
Auscultation Note :
Breath sound : Vesicular
No Added sound
Vocal resonance : Equal on both side

19. NERVOUS SYSTEM EXAMINATION

20. Cranial Nerves :
2nd,3rd , 4th , 6th nerves : Partial ptosis &
miosis of left eye
Rest of the cranial nerves and fundoscopy :
Intact
 Higher Psychic Function : Normal

21. Motor System Examination of Upper Limb :
Generalized wasting of all the muscles of left arm,forearm &
small muscles of hand
Café-Au-Lait spots in lateral surface of left shoulder
Muscle tone : Reduced in LUL (both proximally & distally)
Normal in RUL(both proximally & distally)
Muscle Power : 5/5 in RUL (both proximally & distally)
4/5 in LUL (both proximally & distally)
Reflexes : Normal In RUL (both proximally & distally)
All jerks are reduced in LUL

23. •Sensory examination of Upper Limb : Intact
• Gait : Normal
• Cerebellar Function : Intact

24. Examination of other systems revealed no abnormality

25. PROVISIONAL DIAGNOSIS
Pancoast Syndrome ( Left )

26. INVESTIGATIONS

27. CBC 10/1/20
Hb 11.9 g/dl
ESR 25 mm in 1st
hr
TcWBC 8000/Cu.mm
Neutrophil 63%
Lymphocyte 26%
RBS 72 mg/dl
Creatinine 0.7 mg/dl
S. Bilirubin 0.8 mg/dl
USG W/A NAD
SGPT 51 mg/dl
MT Negative ( 6 mm )

30. CXR done
on 27/12/19

31. CXR Done on
5/1/20

32. USG OF CHEST

39. CT GUIDED FNAC

40. MRI Cervical Spine

44. MRI Brachial Plexus

49. C/F • Pain and weakness of left upper limb and shoulder
CXR • Left Apical shadow
USG Neck • Mass lesion at neck with intrathoracic extension
USG
FNAC
• Benign Mesenchymal Tumor
CT chest
• Left para-Tracheal Lynphadenopathy
CT FNAC • No Granuloma or Malignant cell
MRI Neck • Degenerative changes , disc herniation
MRI Brachial
plexus • Schwannoma ( suggestive of )

50. FINAL DIAGNOSIS
Pancoast Syndrome due to
Neurofibromatosis
(schwannomatosis )

51. DISCUSSION

52. PANCOAST TUMOR
Pancoast tumors are neoplasms of pulmonary origin located at
the apical pleuropulmonary groove (superior sulcus) . It may involve :

54. MESENCHYMAL TUMORS include entities originating from mesodermal-
derived precursor cells that develop into bone, cartilage, or other connective
tissues, such as blood vessels, adipose tissue, smooth muscle, or fibroblasts

57. Causes of Pancoast Syndrome :
Primary Lung Cancer
Usually non-small cell lung cancer
NSCLC; rarely small cell lung cancer
SCLC
Other Primary Thoracic
Neoplasms
• Sarcoma
• Mesothelioma
• Adenoid cystic carcinoma
• Hemangiopericytoma
Metastatic disease
• Laryngeal cancer
• Thyroid carcinoma
• Uterine cervical cancer
• Urinary bladder cancer
Hematologic Malignancies
• Lymphoma
• Plasmacytoma
• Lymphomatoid granulomatosis

58. Nerve Sheath Tumor
• Neurofibroma
• Schwannoma
Infection
Bacterial
• Staphylococcal sp.
• Pseudomonas sp.
• Actinomycosis
Fungal
• Aspergillosis sp
• Cryptococcus sp
Mycobacterium tuberculosis
Parasitic
Miscellaneous
• Asymmetric apical cap
• Asymmetric apical extrapleural
fat deposition
• Apical extension of or located
pleural effusion
• Cervical rib syndrome
Causes of Pancoast Syndrome:

63. CAFE AU LAIT spots

66. Take Home Message
•In case of Pancoast syndrome , Benign Lesion should also be
considered
•Typical radiological findings can be present in early age with a
rare diagnosis
•Circumstances while clinicians face diagnostic dilemma ,
Multidisciplinary Approach is a must