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Posterior Mediastinal Lesions 
Alireza Kashani
Mediastinal Compartments 
 Posterior Mediastinum: 
 Also called paravertebral 
compartment 
 Defined by : 
 Posterior Pericardium 
 Anterior Spinal Ligament 
 Superiorly: T4 vertebral body 
 Inferiorly: Diaphragm 
 Contains: 
 Oesophagus, Descending Aorta, 
Sympathetic Chain, Vagus 
Nerves, Thoracic Duct, Azygos 
and Hemiazygos veins, and 
Lymph Nodes
Epidemiology 
 Adults: 
 65% anterosuperior 
 10% middle 
 25% posterior compartments 
 Children: 
 25% anterosuperior 
 10% middle 
 65% in posterior compartments 
 Generally the incidence of posterior mediastinal lesions is higher in children. 
 50% are asymptomatic and are diagnosed incidentally 
 The absence of symptoms is generally is a clue to a benign lesion 
 Symptomatic lesions: 
 Children: 60%-80% 
 Adults: 50%-60%
Diagnosis 
 CT Scan is the diagnostic modality of choice and is superior to even MRI in 
majority of the cases 
 MRI rarely indicated 
 Thoracic US is not helpful in posterior mediastinal lesions 
 Biopsy: is not necessary in majority of the cases and likelihood of a positive 
result parallels the presence of symptoms 
 If needed: 
 CT-guided 
 Thoracoscopic 
 Limited posterolateral thoracotomy
Neurogenic Tumours 
 Young adults and children 
 They are the most common malignant neoplasm in children but they are less 
common than anterior mediastinal malignancies in adults. 
 Lymphomas and Thymomas are more common in adults 
 Malignancy rate: 
 Children: 50% 
 Adults: 10% 
 They originate from embryonic neural crest cells around the spinal ganglia 
and from either sympathetic or parasympathetic components
Neurogenic Tumours 
 Source: 
 Intercostal nerve: 
 Neurofibroma : benign 
 Of patients with nerve sheath tumours 25%-40% have multiple 
neurofibromatosis (von Recklingausen’s disease) and the incidence of 
malignancy is more common in these patient 
 Neurilemoma (Schwannoma) : 
 Mostly benign but malignant schwannomas are rare 
 The most common neurogenic tumor 
 Neurogenic Sarcoma (rare) 
 Sympathetic Ganglia: can also have pheochromocytoma 
 Ganglioneuroma 
 Ganglioneuroblastoma 
 Neuroblastoma 
 Other: phrenic nerves, Vagus nerves
Neurogenic Tumours 
 Those with benign lesions are generally asymptomatic 
 Malignant tumors might present with symptoms such as: 
 Spinal cord compression 
 Cough 
 Dyspnea 
 Chest wall pain 
 Hoarseness 
 Rarely Horner’s syndrome 
 Rarely pheochromocytoma: 
 Neuroblastoma or ganglioneuroma 
 In all symptomatic patients, esp. those with significant hypertension it is 
necessary to check for 24 h urine HVA (homovanillic acid) and VMA 
(vanillylmandelic acid) 
 If elevated then prior to operation to avoid adrenergic crisis: 
 α-adrenergic blockers 
 beta-blockers
Neurogenic Tumours 
 Intercostal nerves: 
 Neurofibroma 
 Poorly encapsulated 
 Random arrangement of spindle-shaped cells 
 Neurilemomas 
 The most common neurogenic tumor 
 Well-encapsulated 
 Firm, gray-tan 
 Two morphologic types: 
 Antoni’s type A: 
 Organized architecture with a cellular palisading pattern of growth 
 Antoni’s type B: 
 Loose reticular pattern 
 Both of the above can happen as parts of neurofibromatosis type 1 (von 
Recklinghausen’s disease) and if left untreated can degenerate to neurosarcoma
Neurofibroma
Neurilemmoma – Mixed A and B
Neurilemmoma – Antoni A
Neurilemmoma – Antoni B
Sympathetic Source
Sympathetic Source 
 Neuroblastic Tumors: 
 Arising from primitive sympathetic 
ganglia 
 Comprise: 
 Neuroblastoma 
 Ganglioneuroblastoma 
 Ganglioneuroma 
 These differ in their degree of cellular 
and extracellular maturation 
 Immature tumors: 
 Tend to be aggressive 
 Occur in younger patients 
(median age < 2) 
 Mature tumors: 
 Older children (median age 7) 
 More benign 
 Anywhere sympathetic nervous 
tissue exists: 
 Neck 
 Posterior Mediastinum 
 Adrenal Gland 
 Retroperitoneum 
 Pelvis
Sympathetic Source 
 Ganglioneuroma: 
 The most common neurogenic tumor occurring in children 
 Benign 
 Composed of gangliocytes and mature stroma 
 Early age 
 Located in the paravertebral region 
 Ganglioneuroblastoma: 
 Composed of mature gangliocytes and immature neuroblasts 
 Intermediate malignant potential
Ganglioneuroma
Ganglioneuroma
Sympathetic Source 
 Neuroblastoma: 
 Children < 4 y 
 Small, round immature cells organized in rosette pattern 
 Highly invasive 
 Metastasized by the time the diagnosis is made: 
 Regional lymph nodes, bone, brain, liver, lung 
 Some may have benign course 
 Symptoms: 
 Cough, dysphagia, chest pain, and occasionally paraplegia 
 Paraneoplastic: 
 Profuse watery diarrhea due to VIP 
 Opsoclonus-Polymyoclonus syndrome 
 Pheochromocytoma-like syndrome 
 Prognosis: 
 Influenced by: DNA content, tumor proto-oncogenes, and catecholamine synthesis 
 The above aid in categorizing patients with neuroblastoma into low, intermediate, 
and high-risk 
 Rx: Resection +/- Chemotherapy
Neuroblastoma
Neuroblastoma
Investigations 
 CT: 
 Can help to identify the tumor and extent of involvement 
 Those from peripheral nerves: 
 Well-defined, round or oval masses 
 Noncalcified 
 Neurilemoma: 
 Variable enhancement: homogenous or heterogenous 
 On enhanced CT: variable attenuation 
 Neurofibroma: 
 Usually homogenous 
 Low-attenuation 
 On enhanced CT: homogenous enhancement or with early central blush 
 Malignant nerve sheath tumors show variable attenuation
Investigations 
 CT: 
 Sympathetic Chain Tumors: 
 Expand along the spinal axis 
 No calcification or bone changes 
 Ganglioneuroma: 
 Oblong homogenous low-attenuation lesions on both enhanced and 
unenhanced CT 
 Neuroblastoma: 
 Aggressive soft tissue lesions with calcification 
 Ganglioneuroblastoma: 
 Mixed features of above 
 Paragangliomas: 
 Appear in aortopulmonary window 
 High contrast enhancement
Investigations 
 MRI: for dumbbell tumors 
 FNA 
 Tumor Markers: S-100
Dumbbell Tumors 
 Tumors of the posterior mediastinum 
that extend into the spinal canal via 
the intervertebral foramen 
 It is necessary to identify these 
tumors to have a better planning, as 
inaccurate diagnosis would lead to 
intra-spinal hemorrhage and cord 
compression 
 MRI can identify these 
 Smoothly rounded, homogenous 
density abutting the vertebral column 
 Rarely present with cord 
compression symptoms 
 They comprise 10% of patients with 
neurogenic tumors 
 Only 1-2% are malignant
Management 
 Standard of Care: 
 Surgical resection via thoracotomy or thoracoscopy 
 Most of the neurogenic tumors can be resected via thoracoscopy; however, malignant 
transformation, local invasion, and tumors larger than 5 cm increase the likelihood of 
conversion to thoracotomy 
 In most cases the tumor can easily be mobilized via thoracoscopy but any difficulty 
necessitates the conversion to thoracotomy 
 Resection of the nerve root is necessary in most of the cases 
 A widened intervertebral foramen might be indicative of invasion of vertebral canal 
(dumbbell tumor) and in these cases it is necessary to involve a neurosurgeon to 
assist in full resection of the tumor via a laminectomy in one stage procedure 
 Neuroblastoma: 
 Spontaneous regression has been reported 
 Stage: 
 I (noninvasive): Surgical resection alone 
 II (locally invasive on same side of midline): Surgery + RTx 
 III (invasive across the midline): Debulking + RTx + ChemoTx + Second-look 
operation 
 Children < 1 : good prognosis, prognosis has inverse correlation with age
Oesophageal Masses 
 These include: 
 Neoplasm 
 Cysts 
 Diverticula 
 Hiatal Hernia 
 Mega-oesophagus 
 Oesophageal Varices
Cysts 
 Rare, benign, congenital 
 20% of all mediastinal lesions 
 Include: 
 Bronchogenic 
 Hydatid 
 Enteric 
 Intramural Oesophageal 
 Neuroenteric Cysts 
 CT is the most effective modality of diagnosis
Bronchogenic 
 60% of all mediastinal cysts 
 Location: 
 Lung parenchyma 
 Mediastinum 
 Histology: 
 Ciliated columnar epithelium 
 Cyst wall: cartilage, mucus glands, 
smooth muscle 
 Rarely communicate with the 
tracheobronchial tree 
 Symptoms: 
 Occasional compression on the 
adjacent structures 
 Recurrent infection 
 Pain, Cough, Hemoptysis 
 Rx: 
 If incidental finding in an otherwise 
asymptomatic patient: observation is 
accepted 
 If symptomatic esp. if pain, cough, or 
hemoptysis the resection is strongly 
advised.
Bronchogenic
Bronchogenic
Bronchogenic 
 Dysphagia: as a result of 
compression effect on the 
oesophagus 
 Presence of air-fluid level: suggests 
the possibility of communication with 
the tracheo-bronchial tree and 
increased chance of recurrent 
infections; hence, the possible need 
for resection 
 If it becomes infected, it is very likely 
that eradication is unsuccessful and 
it needs to be resected 
 Symptoms tend to develop with time 
and hence the resection is advised 
in a healthy state before becoming 
symptomatic. 
 They can be resected via 
thoracoscopy or if in superior 
mediastinum via mediastinoscope.
Bronchogenic
Bronchogenic
Gastroenteric Cysts 
 Gastroenteric or duplication cysts: 
peri-oesophageal lesions that arise 
from posterior division of the primitive 
foregut. 
 Features: 
 Posterior or middle mediastinal mass 
 Within or adjacent to the oesophageal 
wall 
 Communication with UGIT is 
uncommon 
 Histology: 
 Nonkeratinizing squamous, ciliated 
columnar, gastric, or small intestinal 
epithelium 
 Epithelium is not a good differentiating 
feature from bronchogenic cysts: 
 Presence of two muscular layers in 
oesophageal cysts 
 Cartilage and bronchial glands in 
bronchogenic cysts 
 Usually asymptomatic, but symptoms: 
 Resp: Cough, SOB, Recurrent 
Pulmonary Infections, Chest Pain 
 If gastric mucosa: perforation into the 
oesophagus can cause haematemesis, 
or erosion into the adjacent lung can 
cause an abscess 
 Dx: EUS, Chest CT, Technetium Tc 
99m (to identify gastric mucosa) 
 Rx: 
 Resection: symptomatic, unclear cysts 
– whether cystic or solid 
 Observation: clearly cystic lesions, 
otherwise asymptomatic
Gastroenteric Cysts
Gastroenteric Cysts
Neuroenteric Cysts 
 5-10% of foregut lesions 
 Infants younger than 1 
 Connection to the meninges usually through a stalk 
 Associated with the congenital defects of the spine 
 Endodermal and ectodermal or neurogenic elements 
 Failure of separation of the notochord from the primitive gut 
 CT evidence of mediastinal lesion with either of : Scoliosis, Hemivertebrae, 
Spina Bifida should prompt the possible Dx of neuroenteric cysts.
Other 
 Primary or metastatic lesions of 
thoracic spine 
 Lymphomas : esp. Hodgekins 
 Infections such as TB 
 Post-traumatic haematoma 
 Descending thoracic aneurysm 
 Extramedullary hematopoiesis 
 Castleman’s Disease 
 Angiomyolipoma 
 Extralobar Pulmonary Sequestration 
 Neuroendocrine Carcinoma 
 Mediastinal Ependymoma 
 Cellular Hemangiomas 
 Melanotic Paraganglioma 
 Pancreatic Pseudocyst Mediastinal 
Extension
Castleman’s Disease 
 Giant lymph node hyperplasia 
 Vascular tumors surrounded by 
large LAP 
 Types : 
 Localized: Hyaline Vascular, Plasma 
Cell 
 Generalized or Multicenteric 
 Hyaline Vascular: 90% of lesions 
 Incidental finding in otherwise 
asymptomatic 
 Spinal cord compression has also 
been reported 
 Rx: Surgical excision, RTx is 
ineffective 
 Plasma Cell: 
 More symptomatic: fever, fatigue, 
weight loss, hemolytic anemia, high 
ESR and hyper-γ- globulinemia as 
the result of the over production of 
IL-6 
 Resection is Rx of choice 
 Generalized: 
 Histologic features of both of the 
localized forms 
 Older patients 
 Symptoms: 
 Severe systemic symptoms 
 Generalized LAP 
 Hepatosplenomegaly 
 Mortality : 50% with median survival 
of 27 months and Progression to 
lymphoma
Castleman’s Disease - Hyaline
Castleman’s Disease - Plasma

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Posterior Mediastinal Lesions : A Short Review

  • 2. Mediastinal Compartments  Posterior Mediastinum:  Also called paravertebral compartment  Defined by :  Posterior Pericardium  Anterior Spinal Ligament  Superiorly: T4 vertebral body  Inferiorly: Diaphragm  Contains:  Oesophagus, Descending Aorta, Sympathetic Chain, Vagus Nerves, Thoracic Duct, Azygos and Hemiazygos veins, and Lymph Nodes
  • 3. Epidemiology  Adults:  65% anterosuperior  10% middle  25% posterior compartments  Children:  25% anterosuperior  10% middle  65% in posterior compartments  Generally the incidence of posterior mediastinal lesions is higher in children.  50% are asymptomatic and are diagnosed incidentally  The absence of symptoms is generally is a clue to a benign lesion  Symptomatic lesions:  Children: 60%-80%  Adults: 50%-60%
  • 4. Diagnosis  CT Scan is the diagnostic modality of choice and is superior to even MRI in majority of the cases  MRI rarely indicated  Thoracic US is not helpful in posterior mediastinal lesions  Biopsy: is not necessary in majority of the cases and likelihood of a positive result parallels the presence of symptoms  If needed:  CT-guided  Thoracoscopic  Limited posterolateral thoracotomy
  • 5. Neurogenic Tumours  Young adults and children  They are the most common malignant neoplasm in children but they are less common than anterior mediastinal malignancies in adults.  Lymphomas and Thymomas are more common in adults  Malignancy rate:  Children: 50%  Adults: 10%  They originate from embryonic neural crest cells around the spinal ganglia and from either sympathetic or parasympathetic components
  • 6. Neurogenic Tumours  Source:  Intercostal nerve:  Neurofibroma : benign  Of patients with nerve sheath tumours 25%-40% have multiple neurofibromatosis (von Recklingausen’s disease) and the incidence of malignancy is more common in these patient  Neurilemoma (Schwannoma) :  Mostly benign but malignant schwannomas are rare  The most common neurogenic tumor  Neurogenic Sarcoma (rare)  Sympathetic Ganglia: can also have pheochromocytoma  Ganglioneuroma  Ganglioneuroblastoma  Neuroblastoma  Other: phrenic nerves, Vagus nerves
  • 7. Neurogenic Tumours  Those with benign lesions are generally asymptomatic  Malignant tumors might present with symptoms such as:  Spinal cord compression  Cough  Dyspnea  Chest wall pain  Hoarseness  Rarely Horner’s syndrome  Rarely pheochromocytoma:  Neuroblastoma or ganglioneuroma  In all symptomatic patients, esp. those with significant hypertension it is necessary to check for 24 h urine HVA (homovanillic acid) and VMA (vanillylmandelic acid)  If elevated then prior to operation to avoid adrenergic crisis:  α-adrenergic blockers  beta-blockers
  • 8. Neurogenic Tumours  Intercostal nerves:  Neurofibroma  Poorly encapsulated  Random arrangement of spindle-shaped cells  Neurilemomas  The most common neurogenic tumor  Well-encapsulated  Firm, gray-tan  Two morphologic types:  Antoni’s type A:  Organized architecture with a cellular palisading pattern of growth  Antoni’s type B:  Loose reticular pattern  Both of the above can happen as parts of neurofibromatosis type 1 (von Recklinghausen’s disease) and if left untreated can degenerate to neurosarcoma
  • 14. Sympathetic Source  Neuroblastic Tumors:  Arising from primitive sympathetic ganglia  Comprise:  Neuroblastoma  Ganglioneuroblastoma  Ganglioneuroma  These differ in their degree of cellular and extracellular maturation  Immature tumors:  Tend to be aggressive  Occur in younger patients (median age < 2)  Mature tumors:  Older children (median age 7)  More benign  Anywhere sympathetic nervous tissue exists:  Neck  Posterior Mediastinum  Adrenal Gland  Retroperitoneum  Pelvis
  • 15. Sympathetic Source  Ganglioneuroma:  The most common neurogenic tumor occurring in children  Benign  Composed of gangliocytes and mature stroma  Early age  Located in the paravertebral region  Ganglioneuroblastoma:  Composed of mature gangliocytes and immature neuroblasts  Intermediate malignant potential
  • 18. Sympathetic Source  Neuroblastoma:  Children < 4 y  Small, round immature cells organized in rosette pattern  Highly invasive  Metastasized by the time the diagnosis is made:  Regional lymph nodes, bone, brain, liver, lung  Some may have benign course  Symptoms:  Cough, dysphagia, chest pain, and occasionally paraplegia  Paraneoplastic:  Profuse watery diarrhea due to VIP  Opsoclonus-Polymyoclonus syndrome  Pheochromocytoma-like syndrome  Prognosis:  Influenced by: DNA content, tumor proto-oncogenes, and catecholamine synthesis  The above aid in categorizing patients with neuroblastoma into low, intermediate, and high-risk  Rx: Resection +/- Chemotherapy
  • 21. Investigations  CT:  Can help to identify the tumor and extent of involvement  Those from peripheral nerves:  Well-defined, round or oval masses  Noncalcified  Neurilemoma:  Variable enhancement: homogenous or heterogenous  On enhanced CT: variable attenuation  Neurofibroma:  Usually homogenous  Low-attenuation  On enhanced CT: homogenous enhancement or with early central blush  Malignant nerve sheath tumors show variable attenuation
  • 22. Investigations  CT:  Sympathetic Chain Tumors:  Expand along the spinal axis  No calcification or bone changes  Ganglioneuroma:  Oblong homogenous low-attenuation lesions on both enhanced and unenhanced CT  Neuroblastoma:  Aggressive soft tissue lesions with calcification  Ganglioneuroblastoma:  Mixed features of above  Paragangliomas:  Appear in aortopulmonary window  High contrast enhancement
  • 23. Investigations  MRI: for dumbbell tumors  FNA  Tumor Markers: S-100
  • 24. Dumbbell Tumors  Tumors of the posterior mediastinum that extend into the spinal canal via the intervertebral foramen  It is necessary to identify these tumors to have a better planning, as inaccurate diagnosis would lead to intra-spinal hemorrhage and cord compression  MRI can identify these  Smoothly rounded, homogenous density abutting the vertebral column  Rarely present with cord compression symptoms  They comprise 10% of patients with neurogenic tumors  Only 1-2% are malignant
  • 25. Management  Standard of Care:  Surgical resection via thoracotomy or thoracoscopy  Most of the neurogenic tumors can be resected via thoracoscopy; however, malignant transformation, local invasion, and tumors larger than 5 cm increase the likelihood of conversion to thoracotomy  In most cases the tumor can easily be mobilized via thoracoscopy but any difficulty necessitates the conversion to thoracotomy  Resection of the nerve root is necessary in most of the cases  A widened intervertebral foramen might be indicative of invasion of vertebral canal (dumbbell tumor) and in these cases it is necessary to involve a neurosurgeon to assist in full resection of the tumor via a laminectomy in one stage procedure  Neuroblastoma:  Spontaneous regression has been reported  Stage:  I (noninvasive): Surgical resection alone  II (locally invasive on same side of midline): Surgery + RTx  III (invasive across the midline): Debulking + RTx + ChemoTx + Second-look operation  Children < 1 : good prognosis, prognosis has inverse correlation with age
  • 26. Oesophageal Masses  These include:  Neoplasm  Cysts  Diverticula  Hiatal Hernia  Mega-oesophagus  Oesophageal Varices
  • 27. Cysts  Rare, benign, congenital  20% of all mediastinal lesions  Include:  Bronchogenic  Hydatid  Enteric  Intramural Oesophageal  Neuroenteric Cysts  CT is the most effective modality of diagnosis
  • 28. Bronchogenic  60% of all mediastinal cysts  Location:  Lung parenchyma  Mediastinum  Histology:  Ciliated columnar epithelium  Cyst wall: cartilage, mucus glands, smooth muscle  Rarely communicate with the tracheobronchial tree  Symptoms:  Occasional compression on the adjacent structures  Recurrent infection  Pain, Cough, Hemoptysis  Rx:  If incidental finding in an otherwise asymptomatic patient: observation is accepted  If symptomatic esp. if pain, cough, or hemoptysis the resection is strongly advised.
  • 31. Bronchogenic  Dysphagia: as a result of compression effect on the oesophagus  Presence of air-fluid level: suggests the possibility of communication with the tracheo-bronchial tree and increased chance of recurrent infections; hence, the possible need for resection  If it becomes infected, it is very likely that eradication is unsuccessful and it needs to be resected  Symptoms tend to develop with time and hence the resection is advised in a healthy state before becoming symptomatic.  They can be resected via thoracoscopy or if in superior mediastinum via mediastinoscope.
  • 34. Gastroenteric Cysts  Gastroenteric or duplication cysts: peri-oesophageal lesions that arise from posterior division of the primitive foregut.  Features:  Posterior or middle mediastinal mass  Within or adjacent to the oesophageal wall  Communication with UGIT is uncommon  Histology:  Nonkeratinizing squamous, ciliated columnar, gastric, or small intestinal epithelium  Epithelium is not a good differentiating feature from bronchogenic cysts:  Presence of two muscular layers in oesophageal cysts  Cartilage and bronchial glands in bronchogenic cysts  Usually asymptomatic, but symptoms:  Resp: Cough, SOB, Recurrent Pulmonary Infections, Chest Pain  If gastric mucosa: perforation into the oesophagus can cause haematemesis, or erosion into the adjacent lung can cause an abscess  Dx: EUS, Chest CT, Technetium Tc 99m (to identify gastric mucosa)  Rx:  Resection: symptomatic, unclear cysts – whether cystic or solid  Observation: clearly cystic lesions, otherwise asymptomatic
  • 37. Neuroenteric Cysts  5-10% of foregut lesions  Infants younger than 1  Connection to the meninges usually through a stalk  Associated with the congenital defects of the spine  Endodermal and ectodermal or neurogenic elements  Failure of separation of the notochord from the primitive gut  CT evidence of mediastinal lesion with either of : Scoliosis, Hemivertebrae, Spina Bifida should prompt the possible Dx of neuroenteric cysts.
  • 38. Other  Primary or metastatic lesions of thoracic spine  Lymphomas : esp. Hodgekins  Infections such as TB  Post-traumatic haematoma  Descending thoracic aneurysm  Extramedullary hematopoiesis  Castleman’s Disease  Angiomyolipoma  Extralobar Pulmonary Sequestration  Neuroendocrine Carcinoma  Mediastinal Ependymoma  Cellular Hemangiomas  Melanotic Paraganglioma  Pancreatic Pseudocyst Mediastinal Extension
  • 39. Castleman’s Disease  Giant lymph node hyperplasia  Vascular tumors surrounded by large LAP  Types :  Localized: Hyaline Vascular, Plasma Cell  Generalized or Multicenteric  Hyaline Vascular: 90% of lesions  Incidental finding in otherwise asymptomatic  Spinal cord compression has also been reported  Rx: Surgical excision, RTx is ineffective  Plasma Cell:  More symptomatic: fever, fatigue, weight loss, hemolytic anemia, high ESR and hyper-γ- globulinemia as the result of the over production of IL-6  Resection is Rx of choice  Generalized:  Histologic features of both of the localized forms  Older patients  Symptoms:  Severe systemic symptoms  Generalized LAP  Hepatosplenomegaly  Mortality : 50% with median survival of 27 months and Progression to lymphoma