Mediastinal Disorders



Fig. 5.1.
Medial view of the left hemithorax. Used with permission from the McGill University Health Centre Patient Education Office.



A325685_1_En_5_Fig2_HTML.gif


Fig. 5.2.
Medial view of the right hemithorax. Used with permission from the McGill University Health Centre Patient Education Office.



Table 5.1.
Differential diagnosis of a mediastinal mass based on compartment.





















































































































Anterosuperior compartment

Middle compartment

Thymus

Thymic hyperplasia

Lymph node

See anterosuperior compartment

Thymoma

Cyst

Bronchial cyst

Thymic carcinoma
 
Pericardial cyst

Carcinoid tumor
 
Enteric (duplication) cyst

Small-cell carcinoma

Esophagus

See posterior compartment

Thymolipoma

Posterior compartment

Thymic cyst

Neurogenic

Schwannoma

Germ cell

Teratoma
 
Neurofibroma

Dermoid cyst
 
Ganglioneuroma

Seminoma
 
Ganglioneuroblastoma

Choriocarcinoma
 
Neuroblastoma

Embryonal cell carcinoma

Esophagus

Duplication cyst

Yolk sac tumor
 
Diverticulum

Lymph node

Inflammatory
 
Benign tumor

Infectious
 
Malignant tumor

Malignant (metastasis)

Other

Meningocele

Lymphoproliferative disorder
 
Paraganglioma

(e.g., lymphoma)
   

Mesenchymal

Fibroma/fibrosarcoma
   

Lipoma/liposarcoma
   

Lymphangioma
   

Hemangioma
   

Endocrine

Thyroid
   

Parathyroid
   

Vascular

Aneurysm
   



  • Anterior Compartment: anterior to pericardium and reflection over the great vessels.



    • Includes: thymus gland, lymph nodes, fat


    • In adults, approximately 50 % of mediastinal masses are located in the anterosuperior compartment


    • >90 % consist of thymomas, ectopic thyroid tissue, germ cell tumors, or lymphomas


  • Middle Compartment: bound by anterior and posterior edges of the pericardium



    • Includes: heart, pericardium, ascending and transverse aorta, brachiocephalic vessels, vena cavae, pulmonary arteries and veins, phrenic and vagus nerves, trachea, bronchi, and lymph nodes


  • Posterior Compartment: posterior to pericardium, heart, and trachea and extends to the thoracic vertebral column and paravertebral gutters



    • Includes: esophagus, descending aorta, azygos and hemiazygos veins, thoracic duct, sympathetic chain, and lymph nodes




      Clinical Presentation (Table 5.2):


      Table 5.2.
      Clinical presentation of a patient with a mediastinal mass.












































































      Locoregional symptoms

      Systemic symptoms

      Somatic

      Chest pain

      Constitutional Symptoms

      Night sweats

      Pulmonary

      Cough
       
      Fatigue

      Wheezing
       
      Weight loss

      Stridor
       
      Pel–Ebstein fevers

      Dyspnea

      Systemic

      Thyrotoxicosis

      Hemoptysis

      Syndromes

      Hypercalcemia

      Post-obstructive pneumonitis
       
      Hypoglycemia

      Recurrent pneumonia
       
      Osteoarthropathy

      Cardiovascular

      Superior vena cava syndrome
       
      Autoimmune syndrome

      Pericardial tamponade
       
      Paraneoplastic syndrome

      Congestive heart failure
       
      Yolk sac (endodermal cell) tumor

      Neurogenic

      Hoarseness
         

      Horner’s syndrome
         

      Phrenic nerve paralysis
         

      Brachial plexopathy
         




      • Majority are asymptomatic and discovered incidentally (especially benign lesions)


      • Symptomatology related to local mass effect, invasion of surrounding structures, and immunologic and hormonal factors related to the lesion. Systemic symptoms (e.g., Type B symptoms) are also seen in lymphoma.


      • Physical examination: a full head-to-toe examination, including peripheral lymph nodes and testes in men.


      • Diagnosis made by considering the patient’s age, location of the mass, presence or absence of locoregional and distant clinical manifestations:


      Workup



      • Laboratory: full blood panel, including thyroid function tests, tumor markers (α-fetoprotein (AFP), β-human Chorionic Gonadotropin (βhCG)), cardiac enzymes for chest pain, and autoantibody assays for suspected autoimmune syndromes.


      • Imaging:



        • Contrast enhanced CT is the modality of choice for detailed characterization of the mass (Fig. 5.3).

          A325685_1_En_5_Fig3_HTML.jpg


          Fig. 5.3.
          Axial cuts from contrast enhanced CT scan of thymoma in the anterior compartment of the mediastinum.


        • MRI used as an adjunct to provide additional information about the tissue planes and margins, as well as to differentiate between tumor compression and invasion of surrounding structures.


        • FDG-PET:



          • High uptake more likely to correlate with invasiveness and is seen in thymic carcinoma and invasive thymoma [1, 2].


          • Comparable sensitivity and specificity to CT scan.


          • Significantly higher sensitivity compared with gallium-67 scintigraphy for non-Hodgkin’s lymphoma and Hodgkin’s lymphoma [3, 4].


      • Tissue diagnosis:



        • Routine needle biopsy (FNA and core-needle) is typically avoided and the choice of resection or biopsy is made according to the most likely diagnosis based on workup.



          • If lymphoma is suspected, biopsy is required.


          • Well-encapsulated lesions unlikely to be lymphoma can be directly resected.


          • Locally invasive and unresectable lesions (other than lymphoma) are typically biopsied and evaluated for possible neoadjuvant therapy (e.g., thymic carcinoma).


        • Complications of biopsy include: pneumothorax (20–25 %), hemoptysis (5–10 %), significant hemorrhage (rare), and tumor seeding along needle tract (extremely rare).


        • Incisional and excisional biopsies may also be performed under general anesthesia. This may not be possible for patients with high risk of cardiopulmonary compromise (e.g., posture-related dyspnea, SVC syndrome). Options include:



          • Mediastinoscopic biopsy


          • 2nd/3rd intercostal space parasternal mediastinotomy (Chamberlain procedure)


          • Transcervical approach


          • VATS



      Thymoma




      Overview



      • Most common neoplasm of the anterosuperior compartment in adults.



        • Incidence: 0.15 per 100,000 person-years in the USA; M = F [5]


        • Rare in the first 2 decades of life


        • Incidence peaks at ages 30–40 (with associated myasthenia gravis (MG)) and 60–70 (without MG) [6]


      • Slow-growing epithelial tumor that spreads by local invasion. Extra-thoracic metastases are uncommon [7]


      Pathology



      • Controversial distinction between thymomas and thymic carcinomas (Table 5.3).


        Table 5.3.
        Classification of mediastinal masses originating from the thymus.


























        Thymus hyperplasia
         

        Epithelial neoplasms

        Thymoma

        Thymic carcinoma

        Thymic neuroendocrine tumors

        –  Carcinoid tumor

        –  Small-cell carcinoma

        Thymolipoma
         

        Thymic cyst
         




        • WHO Classification (Table 5.4) stratifies them along a continuum [8].


          Table 5.4.
          WHO classification of thymomas and thymic carcinomas [8].






































          WHO classification

          Epithelial cell shape

          Epithelial cell atypia

          Lymphocyte

          Organotypic “(Thymus-like)”

          Incidence (%) [29]

          A

          Spindle

          Minimal

          Poor

          Yes

           9

          AB

          Spindle/polygonal

          Minimal

          Moderate

          Yes

          24

          B1

          Polygonal

          Minimal

          Abundant

          Yes

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          Sep 23, 2016 | Posted by in CARDIOLOGY | Comments Off on Mediastinal Disorders

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