Esophageal leiomyoma, duplication cysts

1. Case capsule
Dr. Zeeshan

2. • 40 / M
• No comorbidities
• Epigastric pain associated with epigastric fullness
• Dysphagia to solids for 1 month
• Cough after food intake
• Dyspnoea on exertion
• ? Relevant H/O

3. NO SIGNIFICANT FINDINGS ON EXAMINATION

4. DIFFERENTIALS

5. • Esophageal pathology
- Intraluminal:
Benign : GERD, Esophageal webs, rings ,
Zencker’s diverticulum
Malignant : Ca esophagus
- Extrinsic compression

6. BLOOD INVESTIGATIONS WITHIN NORMAL
LIMITS

7. HOW WOULD YOU LIKE TO PROCEED?

9. WHAT NEXT?

10. • UGI scopy
• Barium swallow
• ? Importance of order

11. WHAT NEXT?

20. CT report
A well defined, thin walled, large, nonenhancing, fluid
density lesion, measuring 13 mm x 11 mm x 9.1 mm in
the middle and posterior mediastinum, extending from T4
to T9 vertebrae and predominantly on the right.
The lesion is displacing the trachea anteriorly, the lower
two third of the esophagus to the ipsilateral side, and
causing splaying of the carina, main bronchi and right
pulmonary artery
The lesion is seen compressing upon the esophageal
lumen; fat planes between the lesion and the
esophageal wall is indistinct.
The lesion causes compression of the left atrium and
the pulmonary veins are stretched over the anterior
surface of the lesion, however the fat plane is preserved

21. Approach to a mediastinal mass

22. Mediastinum

23. Compartments
• Anterior
• Middle
• Posterior

24. Anterior compartment
• Between sternum and anterior aspect of
pericardium
• Contents:
- Thymus
- Internal mammary A.
- Lymph node
- Connective tissue
- Fat

25. Anomalies of Ant. Compartment
• Terrible T’s
- Thymoma
- Teratoma
- (Terrible) lymphoma
- Thyroid tissue

26. Middle compartment
• Ant: Pericardium
• Posteriorly : Ventral surface of thoracic spine
Contents:
- Heart
- Airway
- Pericardium
- Ascending thoracic aorta
- SVC with azygos vein

27. Anomalies of middle compartment
• Lymphadenopathy : MC cause
- Sarcoidosis
- Lymphoma
- Metastatic lung malignancy

28. Posterior mediastinum
• Artery
– Thoracic part of descending Aorta
• Veins
– Azygos vein
– Hemiazygos, accessory hemiazygos veins
• Nerves
– Vagus nerve
– Splanchnic nerve
• Esophagus
• Thoracic duct

29. Anomalies of Post. Mediastinum
• Mass arising from Esophagus
• Aneurysms of descending aorta
• Neurogenic tumors

30. Importance of H/O
• Age
• Anatomic location
• Associated symptoms

31. Why age?
• Adults :
- Thymomas
- Lymphomas
- Germ cell tumors
- Only 1/3rd
lesion are symptomatic

32. • Children:
- Post. Neurogenic tumors
- Lymphomas
- Germ cell tumors
- 2/3rd
symptomatic

33. Anatomic location
• Chest X-ray
- AP
- Lateral
• CT thorax

34. WHICH PART OF MEDIASTINUM IS THE MASS
ARISING FROM??

43. Posterior mediastinum
• Differentials for a cystic mass in posterior
mediastinum:
- Bronchogenic cyst
- Esophageal duplication cyst

44. Bronchogenic cyst
• MC cystic lesion
• Abnormal budding of the lungs
• MC in men/ right paratracheal location
• Symptoms of cough, substernal pain,
recurrent infections, dyspnoea

45. Esophageal duplication cysts
• Third most common benign esophageal mass
• MC – Leiomyoma
• 2nd
MC – Polyp
• Three criteria to establish diagnosis:
1.Esophageal attachment
2.Presence of 2 layers of muscularis propria
3.Epithelium characteristic of GI tract

46. HOW WOULD YOU LIKE TO PROCEED

47. • EUS
• Preoperative biopsy : debatable
Not recommended

48. • Pt was posted for Thoracotomy proceed
• He underwent excision of posterior
mediastinal tumor with resection of thoracic
esophagus + stomach pullup.

49. Findings
• 15 x 25x12 cm sized mass arising from the posterior
mediastinum ? site of origin with the oesophagus stretched
over it laterally and the tumour was crossing the midline.
• The lower limit was about 4 cm from the oesophageal hiatus.
• The tumour contained jelly like material and was infiltrating
the posterior pericardium.
• There were few enlarged nodes in relation to the tumour.
• The tumour was very vascular.

53. Biopsy
• Smooth muscle tumor probably benign
• Leiomyoma
• Maximum tumor size – 11.2cm
• Tumor was adherent to wall of esophagus

54. Leiomyoma of esophagus

55. Incidence
• Most common benign tumors of the
esophagus
• 90% occur in the lower/middle third
• Arise from muscularis mucosa
• Male predominance

56. • Most lesion are intramural/submucosal
• Solitary
• Size : 2-8cm
• Slow growing tumors
• 50% patients asymptomatic

57. Symptoms
• Dysphagia – Most common symptom
• Retrosternal pain/discomfort
• Heart burn
• Weight loss

58. Diagnosis
• UGI scopy : Submucosal mass lesion
• Barium swallow : Smooth filling defect in the
esophageal lumen without a mucosal
abnormality
• CT Thorax: Well defined eccentric intramural
mass with homogenous enhancement after
administration of IV contrast

59. • EUS : Homogenous and hypoechoic lesion
with clear margins with surrounding
hyperechoic area

60. • Biopsy : To be avoided
- Cause scarring and hampers extramucosal
excision of tumor
- Usually non diagnostic

61. Management
• Asymptomatic/ small leiomyomas
• Monitor periodically using UGI scopy, EUS and
CT thorax
• Risk of malignant transformation < 1%

62. Indication for surgery
• Symptomatic leiomyomas
• Leiomyomas > 5cm

63. Modes of excision
• Thoracoscopic excision of lesion
• Open surgery

64. Open approach
• Middle third of esophagus – Right
thoracotomy
• Lower third of esophagus - Left thoracotomy/
Left thoraco-abdominal / Transhiatal
approach

65. Summary
• Leiomyomas are rare tumors of esophagus
• If <5cm patient usually kept on follow-up
• Large leiomyomas are excised with segmental
esophageal resections
• Malignant potential <1%