mediastinal tumor

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Modern definition of mediastinal compartments
and the current approach to patients
with an anterior mediastinal mass
Pier Luigi FILOSSO, MD FECTS
Associate Professor
University of Torino Dept Thoracic Surgery

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Introduction
AIMS
 Mediastinum & Mediastinal Compartments: definition
 How to approach the mediastinal massess
 Diagnostic techniques of the mediastinum
 Evaluation and diagnostic approach according to the mediastinal
compartments
 Take home messages

THE MEDIASTINUM
The mediastinum extends from
the thoracic inlet (I°rib) to the
diaphragm, between the pleural
cavities

Mediastinal Compartments
Traditional
4-compartment model
Traditional
3-compartment model
Modified 3-
compartment model
(Shields)

Anterior
Between the back of the sternum and
the anterior surface of the pericardium
& the great vessels

Middle (visceral)
Includes the heart, great vessels, trachea,
esophagus, and it is bound posteriorly by
the anterior longitudinal ligament (the
anterior surface of the vertebral bodies)

Posterior (paravertebral)
Extends from the anterior longitudinal
ligament to the posterior ribcage

Mediastinal Masses
Mediastinal tumors are relatively
uncommon and there are many
different types

23-25%
20-25%
50-60%
IASLC, Thoracic Oncology, 2014
Mediastinal masses:
incidence by Compartments

 Incidence of Mediastinal Tumors is low
– Many years are needed to develop adequate clinical experience
 Includes a wide variety of lesions
 Clinical presumptive diagnosis is a matter of pattern recognition
– This is inherently difficult
– The approach used by authors is the opposite of what the clinician needs
Mediastinal Tumors:
Why is this difficult?

Approach to Mediastinal Tumors
 Known clinical data, always available:
• Age, gender
• Mediastinal compartment location
• Radiographic characteristics
 Often available:
• Duration of symptoms
 Sometimes available:
• Characteristic presentation: (Myasthenia Gravis, “B” symptoms, associated diseases)
• Laboratory identifiers

Symptoms and associated disorders
 Benign/slow growing neoplasms are often asymptomatic
 Malignant lesions are frequently symptomatic
Local signs/symptoms
Cough
Chest pain
SVC syndrome
Dyspnea, Dysphagia
Cardiac tamponade
Associated paraneoplastic diseases (MG, PRCA,
Hypogamma, SLE)
Systemic signs/symptoms
B symptoms
Fever
Night sweats
Weight loss

Symptoms and associated disorders
 Benign/slow growing neoplasms are often asymptomatic
 Malignant lesions are frequently symptomatic
Local signs/symptoms
Cough
Chest pain
SVC syndrome
Dyspnea, Dysphagia
Cardiac tamponade
Associated paraneoplastic diseases (MG, PRCA,
Hypogamma, SLE)
Systemic signs/symptoms
B symptoms
Fever
Night sweats
Weight loss
Duration of symptoms (rapid, intermediate or slow) is
of paramount importance to suggest a diagnosis

Mediastinum: diagnostic techniques
• CT
• CT/PET
• MRI
Non-invasive
(Imaging)
• Blind TBNA
• EBUS-TBNA
• EUS-TBNA
• TTNA
Minimally-invasive
pathologic staging
techniques
• Mediastinoscopy
• Mediastinotomy
• TEMLA, VAMLA
• VATS
Surgical
techniques

Approach to Mediastinal Tumors
Confirmation of diagnosis usually requires tissue, but approach may vary from:
 No biopsy (Cyst, NSGCT with + AFP, HCG)
 FNA
 Core Biopsy
 Surgical biopsy (Lymphoma)
 Resection (Stage I,II Thymoma, Teratoma, Schwannoma, Substernal Thyroid)

Mediastinum: surgical techniques
Mediastinoscopy Accessible regions

Anterior Mediastinotomy
(mammary vessels preservation)

Anterior Mediastinotomy
(with video-assistance)
Accessible regions

VATS (Video-assisted thoracic surgery)
 Access to all mediastinal regions
 Useful in case of associated pleural effusion
(lymphoma)
 (Theoretical) risk of seeding of the pleural
space/tract

Approach to Mediastinal Tumors:
the need for histological confirmation
1. In most cases CT findings (+ TM or clinical findings) are sufficient for the diagnosis
(teratoma, GCT, thymoma, neurogenic tumor, thyroid mass)
2. Cyto-histologic confirmation is needed in case of
 Equivocal CT findings
 Suspected lymphoma
 Unresectable or advanced thymic tumors (induction, exclusive CT/RT)
3. Non-surgical biopsy may be considered as first attempt (core > FNA)
4. Surgical biopsy is almost always diagnostic and the technique depends upon the
anatomical location

Anterior mediastinum
(epicenter anterior to
pericardium/great vessels)

Anterior Mediastinum Tumors All Patients
Courtesy of Prof. F. Detterbeck

Anterior Mediastinum Tumors Women > 40 years
ESTS Textbook of Thoracic Surgery
0%
20%
40%
60%
80%
100%
40-49 50-59 60-69 70-79 >80
Age (years)
Thymoma
Thyroid
Micellaneous
HD/MLC Lymph
LB NHL
Teratoma
NSGCT/TeratoCa
Seminoma
Thymoma and
substernal goitres are
the most frequent
tumor types

Anterior Mediastinum Tumors Men> 40 years
Thymoma and
substernal goitres are
the most frequent
tumor types
0%
20%
40%
60%
80%
100%
40-49 50-59 60-69 70-79 >80
Age (years)
Thymoma
Thyroid
Micellaneous
HD/MLC Lymph
LB NHL
NSGCT/TeratoCa
Teratoma
Seminoma

Anterior Mediastinum Tumors
Predominantly Thymoma and substernal Thyroid lesions
 Especially in women, esp. in older age groups
 A few are lymphoma (HD, MLC NHL > LB NHL)
 A few are teratoma in age 40-49
 ~15% comprised of miscellaneous rare tumors

THYMOMA
 Incidence 2.5-3.2/1.000.000 person
 Age onset: 30-40 yrs (with MG); 60-70 yrs (without MG)
 Histotypes: Thymoma (5 subtypes), Thymic carcinoma (TC)
and NETT
 Slow onset of symptoms (exception TC/NETT),
30% asymptomatic
 30-50% associated Myasthenia Gravis
 Paraneoplastic syndrome (PRCA, Hypogamma, SLE)
 CT signs suggestive for diagnosis

THYMOMA
Stage III Thymoma Stage IVa Thymoma

THYMOMA RECURRENCE
local: anterior mediastinum (25%)
regional: intrathoracic (60%)
distant: outside the chest or
intrapulmonary nodules (15%)
usually asymptomatic, incidentally discovered
during follow-up, several years after the resection
of the primary Thymoma

Anterior Mediastinum Tumors:
Thymic Cyst

Thymic Cyst
 Well-circumscribed
 Round/oval
 Homogeneous
 Water/fluid attenuation (HU 0-20)
the malignant potential of lesions appearing as thymic cyst is
CURRENTLY UNKNOWN

Cystic Thymoma
 Sometimes can manifest as
higher density and less regular lesions
 MRI should be therefore performed
(internal septations)
 Paraneoplastic syndrome(s) may also
be observed
 Age > 40 years

Substernal Goiter

Substernal Goiter
 Thyroid is easy to recognize, extending
down from neck on contrast-enhanced CT
(presence of calcifications, nodules and colloid)
 I131 scan not useful for 6 weeks after
contrast CT

Substernal Goiter
 Hyperdense (HU: 70-85 due to iodine)
 Enhances following IV contrast (prolonged
enhancement)
 Frequently demonstrates continuity with
cervical thyroid gland

 If it looks unusual or a lymphoma is
suspected:
biopsy (usually surgical)

LYMPHOMA
 2001 WHO classification
 Hodgkin disease (HD), non-Hodgkin lymphomas (NHL) and MALT lymphoma
 50% HD and 20% NHL involve ALSO the mediastinum
 3% HD and 6% NHL involve ONLY the mediastinum
 90% of mediastinal lymphomas are in the anterior (superior) region
 Hystotypes: Hodgkin disease, mediastinal B-large cell NHL and lymphoblastic
(T-cell) NHL, pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma

Clinical Scenario Level of
Confidence
% of All
1. Consider substernal goiter – Should be obvious on
CT Certain 10-30%
→ Resect if symptoms, airway narrowed;
rarely consider thyroid malignancy
2. If not substernal goiter → Thymoma most likely ~50%
a. If MG, other thymic paraneoplastic syndrome Certain
b. If lobulated, homogenous/slightly
heterogeneous mass High
c. If lobulated and with pleural nodules Very high
→ Resect if early stage, core biopsy first if higher stage
Male & women > 40 years

Male & women > 40 years
Confidence
% of
All
3. If it does not fit for goiter or thymoma
10-
40%
a. If well-circumscribed, round/oval/saccular,
homogeneous near thymic bed on CT → Consider thymic
cyst. get MRI
→ If purely cystic – simple thymic cyst, observe for ~2
years
→ If cystic but with soft tissue components on CT/MRI -
Multilocular cyst or cystic thymoma - Resection
Very high
Uncertain
<5%
<5%
b. If heterogeneous with fat (bone) → Benign teratoma High <5%
→ Resect if symptomatic or significant organ compression
c. If matted, multiple enlarged nodes → HD, MLC-NHL Moderate 2-10%
If “B” symptoms High
→ core (or surgical)l biopsy

Anterior Mediastinum Tumors Women age 10 – 39 years
0%
20%
40%
60%
80%
100%
10-19 20-29 30-39
Age (years)
HD/MLC Lymph
Thymoma
Teratoma
Miscellaneous
LB NHL
Thyroid
NSGCT/Teratoca
Seminoma
Lymphoma and
Teratoma are the most
frequent lesions

Hodgkin’s Lymphoma

Hodgkin’s disease
 Young adults , 16-30 years
 Female predominance (NS type)
 Intermediate onset of symptoms
 The most common type is nodular sclerosing (NS) type (60%)
 HIV-associated in mixed cellularity type (+ EBV)
 30% B symptoms (fever, night sweats, weight loss)
 Bulky involvement with symptoms in 30-35% of the patients
 Histology (surgical biopsy) is needed for subtype diagnosis

Mediastinal large cell non-Hodgkin lymphoma
 Broad age range, most young adults
 Female predominance
 Anterior region (>95%)
 Rapid onset of symptoms (pain, cough, SVC syndrome, pleural effusion)
 Type B symptoms in 30% of the pts
 Bulky involvement + pleural/pericardial effusion
 Extrathoracic involvement in 10-50% of the pts
 Surgical biopsy often needed (high sclerosis)

Germ cell tumors of the mediastinum
Primary (extragonadal) or secondary (from gonadal germ cell tumors)
3-5% of all germ cell tumors
Teratoma
 Mature teratoma,
 Teratomas with malignant elements
 Teratocarcinoma, with another germ cell tumor
Seminoma
Nonseminomatous
NSGCT
 Embryonal carcinoma
 Yolk sac tumor
 Choriocarcinoma
 Teratocarcinoma
 Mixed tumors

Teratoma
 Most common mediastinal germ cell tumor (60-70%)
 Equal M/F distribution, mean age 25-30 years
 More than one of three germ cell layers (ecto, meso, endoderm)
 Mostly asymptomatic
 Rarely malignant (“teratocarcinoma”)
 Surgery is the treatment of choice

Seminoma
 40% of malignant germ cell tumors of the mediastinum
 Men in third-fourth decades
 Only 10% have elevated β-HCG, not AFP
 Mostly symptomatic (compression symptoms, SVC syndrome)
 Highly sensitive to CT (cisplatin-based) and RT
 Nonsurgical therapy is curative in most patients with survival rates of 60-80%
(mostly CT, RT for small tumors)
 Surgery in selected cases (encapsulated forms, residual mass after CT or RT)

Nonseminomatous germ cell tumors of the
mediastinum (NSGCT)
 20-40 yrs of age, mostly symptomatic (compression, SVC, met)
 Elevated AFP and β-HCG levels
 Mets to regional LN, pleura, pericardium and distant sites (25%)
 Primary CT with bleomycin, etoposide and cisplatin (BEP, VIP)
 Surgery in case of resectable residual mass
 Salvage CT for disease progression or unresectable residual mass
 Challenging surgery and postop (bleomycin-damaged lung)
 2-year survival= 67%, 5-year= 50%

Teratoma

Mature Teratoma

Anterior Mediastinum Tumors: female 10-40 years
Most common is Lymphoma (HD, MLC ~ 40%)
Also thymoma in older pts, teratoma in younger pts
~ 10% have LB NHL, ~ 5% age 20-29 have NSGCT
Clinical Approach:
 Surgical Bx needed in most, esp if “B” symptoms
 Resection if confident of teratoma or stage I,II thymoma (? MG)
 FNA is useful if suspected LB NHL
 Rarely useful to get α-FP, β-HCG

Ant Mediast Lymphoma – Diagnosis
Nodular Sclerosing Hodgkins Disease (NSHD) or Mediastinal Large Cell (MLC)
NHL
 Both have prominent sclerosis
 Ability to establish diagnosis by FNA, especially the subtype of lymphoma, is
difficult
 Bone Marrow involvement rare (<2%)
 Surgical biopsy required in most centers
Lymphoblastic (LB) NHL
 Unique cytol & immunophenotypic charac on FNA
 + bone marrow in 50%, also + pl effus cytol

Ant Mediast Lymphoma – Diagnosis
Issues with Needle Bx of NSHD or MLC NHL
 prominent sclerosis  poor specimens
 Core Bx and ancillary studies may help, but:
 Ability to establish dx by FNAB varies from 13-90% (very center specific?)
 In most series used only as prelim test, to be confirmed w surg Bx to establish
subtype
 Most series incl FNAB done to Dx recurrence

women age 10-39 years
Confidence
% of All
1. If matted, multiple enlarged nodes, “B”
symptoms, ↑ LDH → HD, MLC-NHL
High 30-50%
→ core or surgical biopsy
2. If heterogeneous with fat (bone)
→ Benign teratoma
High 10-25%
→ Resect if symptomatic or significant organ compression
3. If MG and lobulated, homogenous mass
→ Thymoma
High 5-10%
→ Resect if early stage, core (or surgical) biopsy if higher stage

women age 10-39 years
Confidence % of All
4. If fulminant onset:
a. Large mass, pleural effusion, “B” symptoms,
↑ LDH → LB NHL
Moderate 5-10%
→ cytology of pleural effusion, bone marrow Certain
b. Heterogeneous mass, lung metastases
→ NSGCT
Moderate 2-5%
→ confirm by ↑↑ serum α-FP, β-HCG (± Bx) Certain
c. Everything else → various rare tumors Unclear 5-20%
→ core (or surgical)l biopsy

0%
20%
40%
60%
80%
100%
10-19 20-29 30-39
Age (years)
NSGCT/TeratoCa
HD/MLC Lymph
Miscellaneous
Thymoma
Teratoma
Seminoma
LB NHL
Thyroid
Anterior Mediastinum Tumors Men age 10 – 39 years

Men - Relatively even mix of:
 Malignant Germ cell (seminoma≈NSGCT)
 Lymphoma (HD>MLC)
 Thymoma (esp older groups)
 Teratoma (esp younger groups)
 Miscellaneous tumors
 Also LB NHL in age 10-19

Clinical Approach
• If onset of symptoms rapid 
suspect NSGCT (bulky inhomogeneous, pulm mets)
or LB NHL (bulky mass, freq extrathor invlmt, effus, “B” symptoms, 
LDH)
• Get α-FP, β-HCG / FNA of mass / bone marrow Bx, pleural fluid
cytology
• Occasionally MLC NHL (bulky, few other nodes, effus, “B” symp)

Mediastinal Germ Cell Tumor: Diagnosis
Nonseminomatous GCT
 ~90% have markedly abnormal serum markers α-FP, β-HCG
 Markers and typical presentation is sufficient for diagnosis without biopsy
Seminoma
 Normal markers (~10% low level  β-HCG)
 Get FNA for diagnosis

Ant Mediast Tumors – men Age 10-39
Clinical Approach
 If asymptomatic or indolent onset of sympt. 
suspect Thymoma (older age, ?MG)
or Teratoma (inhomogeneous, fat ± calcium density)
 Biopsy may not be needed

Ant Mediast Tumors – men Age 10-39
Clinical Approach
 If intermediate onset of symptoms  suspect Lymphoma
HD  cervical, hilar nodes, “B” symp; WBC,Alk 
MLC  bulky, inhomogeneous, “B” symp, pl/pericard effusion, few other nodes, ant
mediast only in 90%
or Seminoma (homogeneous, pulm mets)
 Surgical Biopsy will probably be needed

Ant Mediast Tumors – Children

Ant Mediast Tumors – Age < 10 years
 Vast majority of teratomas are benign
 Thymic lesions are generally cysts, hyperplasia
 LB NHL and HD/MLC NHL lymphomas occur in older children
 Teratomas, thymic lesions, other cysts, cystic hygromas usually in
younger children

Ant Mediast Tumors – Age < 10 years
 Issue is to differentiate lymphoma from other lesions
 If lymphoma, Bx only (may do FNA for LB NHL, or cytol of pleural fluid)
 Other lesions are generally resected due to compression of chest
structures
 This can usually be determined by age of onset, rapidity of growth, and
radiographic appearance

Posterior mediastinum
(Paravertebral Masses)

Vast Majority are benign:
 Schwannoma Neurofibroma Ganglioneuroma
 5-10% Neurofibrosarcoma
 Usually in setting of familial neurofibromatosis
 10-20% of all pts with neurogenic tumors have familial condition; of these 10-20%
will develop a malignant tumor
 Don’t forget about other systemic diseases
 TB, Hematopoetic disorders
Posterior Mediastinal Tumors: age 20 - 70

Posterior Mediastinal Tumors: Neurogenic Tumors
 Nerve sheath (adults, almost all benign)
 Benign, Neurilemmoma,Neurofibroma, Melanotic schwannoma, Granular cell t.
 Malignant, Neurofibrosarcoma
 Ganglion cell (children, young, 2/3 malignant)
 Benign, ganglioneuroma
 Malignant, Neuroblastoma, ganglioneuroblastoma (children)
 Paraganglionic tissue
 Benign, chemodectoma, pheochromocytoma
 Malignant, malignant pheochromocytoma

How to differentiate benign vs malignant:
 Not helpful:
 Symptoms (overall) ( 84% adults , 60% children are asympt.)
 10% dumbell (both adults/children, benign/malig)
 Bone changes (displacemen, deformationt seen in 33% of adults)
 VMA, HVMA (rarely if ever ↑ unless N/GN’blastoma)
 Signs of malignancy:
 Pain, Dyspnea,
 > 10 cm , major mediastinal shift, pleural effusion, bone destruction

Management - Potentially malignant tumors
 (Multiple neurofibromatosis, pain, radiographic signs)
 Chest CT; if close to spinal canal  MRI
 Other imaging (EUS, PET – not well defined)
 Resection, consider multimodality approach
 Long term survival is unusual
Management - Clinically benign, but NeuroFibromatosis:
 If solitary lesion, resect prophylactically
 If multiple, difficult to resect  Observation
 Poorly defined whether problem is rate of malignant degeneration or development of
new malignant tumor
 Rate of growth of benign lesions is hard to predict

Posterior Mediastinal Tumors: Neurinoma

Posterior Mediastinal Tumors: Neurinoma (MRI)

Posterior Mediastinal Tumors: Neuroblastoma
Courtesy of Prof. F. Detterbeck

Posterior Mediastinal Tumors: Age 0 – 19 years
Age 0-5:
 Neuroblastoma/Ganglioneuroblastoma in vast majority (esp age 0-1)
Age 5-19:
 Ganglioneuroma (Schwannoma, Neurofibroma)
 5-10% Neurofibrosarcoma or N/GN’blastoma

Middle mediastinum
(visceral compartment)

Majority are cysts (50%):
 Pericardial, bronchogenic, esophageal, undifferentiated cyst
 Communication with other structrues is rare (<10%)
Granulomatous/Inflammatory causes common (20%)
 TB, Sarcoidosis
 Other benign causes (silicosis, Castleman’s disease)
Malignant causes less common (20%)
 BALT,MALT Lymphoma in older pts
 SCLC/NSCLC in smokers
Other causes (10%)
 Aortic (pseudo)aneurysm, paraganglioma, thyroid, other lymphoma, fungal infection,
Middle Mediastinal Tumors

Conclusion 1/2
 Optimal evaluation and diagnosis of mediastinal tumors require an
integrated clinical, radiological and histological approach
 Clinical approach includes consideration of the following: age, sex,
presence/duration of symptoms and associated paraneoplastic conditions
 Radiological approach includes CT scan with intravenous contrast; CT scan
is essential to assign the tumor to one of the mediastinal anatomic
compartments. MRI and PET-CT are used on a selective basis.
 Histological confirmation is most often needed for suspected lymphomas
and in locally advanced/unresectable thymic tumors

Conclusion 2/2
 More than half of the tumors are in the anterior region, the remaining are
equally distributed in the visceral and paravertebral regions
 In the anterior region the most common tumors are thymomas in adults and
lymphomas in patients < 40 years. GCTs are mostly seen in young males
 In the visceral region more than half of the tumors are cysts. The remaining are
mediastinal involvement from lung cancer and granulomatous diseases.
 In the paravertebral region almost all tumors are from nervous structures.
Malignant forms are typical of infancy or in case of familial neurofibromatosis

Thank you very much
for your attention
Torino, Italy

mediastinal tumor

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