Schwannoma (Mediastinal Mass)
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Schwannoma (Mediastinal Mass)

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Schwannoma (Mediastinal Mass) Schwannoma (Mediastinal Mass) Presentation Transcript

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  • Evaluation of Mediastinal Mass Leslie Proctor, M.D. November 21, 2008
  • Mediastinal Anatomy
    • Includes structures bound by:
      • the thoracic inlet
      • diaphragm
      • sternum
      • vertebral bodies
      • and pleura
    • Has 3 compartments
      • Anterior
      • Middle
      • Posterior
  • The differential diagnosis of a mediastinal mass depends upon the anatomic compartment in which it arises. Redrawn from Baue, AE, et al. Glenn's Thoracic and Cardiovascular Surgery. 5th ed. Appleton & Lange, Norwalk, CT, 1991.
  • Mediastinal Anatomy
    • Anterior Compartment includes:
    • Thymus
    • Extrapericardial aorta and its branches
    • The great veins
    • Lymphatic tissue.
    • Middle Compartment is bounded by:
    • The pericardium anteriorly
    • The posterior pericardial reflection
    • The diaphragm
    • The thoracic inlet.
    • This compartment includes the heart, intrapericardial great vessels, pericardium, and trachea.
      • Extends from the posterior pericardial reflection to the posterior border of the vertebral bodies and from the first rib to the diaphragm.
      • It includes the esophagus, vagus nerves, thoracic duct, sympathetic chain, and azygous venous system
    Posterior Compartment:
  • Anatomic Distribution of Masses
    • Anterior Mediastinum
    • Thymic tumors and cysts
    • Germ cell tumors
    • Lymphomas
    • Intrathoracic goiter and thyroid tumors
    • Parathyroid adenomas
    • Connective tissue tumors
      • lipomas and liposarcomas
      • lymphangiomas
      • hemangiomas
    • Thymoma
  • Anatomic Distribution of Masses
    • Middle Mediastinum
    • Thyroid tumor or goiter
    • Tracheal tumors
    • Aortopulmonary paraganglioma
      • paracardial cysts
      • bronchogenic cysts
      • lymphoma
    • Lymphadenopathy
    • Retrosternal Goiter
  • Anatomic Distribution of Masses
    • Posterior Mediastinum
    • Neurogenic tumors
      • including Schwannomas
    • Esophageal tumors
    • Hiatal Hernias
    • Neurenteric Cysts
    • And rarely
      • extramedullary hematopoiesis
      • pancreatic pseudocyst
      • achalasia
    • Paraspinal Ganglioneuroma
  • About Neurogenic tumors…
    • 9 to 39 percent of all mediastinal tumors
    • develop from mediastinal peripheral nerves, sympathetic and parasympathetic ganglia, and embryonic remnants of the neural tube.
    • most frequent in the posterior compartment of the mediastinum
    • Can cause neurologic symptoms by compression.
    • Benign Schwannoma is most common
      • often asymptomatic, but can be associated with Horner’s or Pancoast’s syndrome
      • Focal calcifications and cystic changes
      • can extend through an intervertebral foramen, resulting in dumbbell-shaped tumors, and neurologic symptoms of spinal cord compression
      • Gross Histology
        • encapsulated, solid, soft, yellow-pink nodule, with the capsule attached to the epineurium of the nerve that gives rise to the neoplasm
      • Microscopic histology
        • composed of spindle cells with elongated nuclei, forming interlacing bundles with focal nuclear palisading
        • nuclear atypia, and stromal sclerosis in older lesions
        • Mitotic figures are rare.
        • Immunohistochemical studies reveal a strongly positive reaction with S-100 protein.
  • Mediastinal Benign Schwannoma
  • Anatomic Distribution of Masses
    • A mass may extend beyond these boundaries as it grows in size
    • In adults, anterior compartment masses are more likely to be malignant
  • Age Distribution
    • Age can help predict etiology of the mass
      • infants and children, neurogenic tumors and enterogenous cysts are the most common mediastinal masses
      • In adults, neurogenic tumors, thymomas, and thymic cysts are most frequently encountered lesions
      • In 20-40 year olds, the likelihood of a mass being malignant is greater secondary to the increased incidence of lymphoma (Hodgkin’s and non-Hodgkin's) and germ cell tumors
  • Signs and Symptoms
    • Depend on location of mass
    • Asymptomatic
    • Vague symptoms
      • aching pain
      • cough
    • Children more likely to be symptomatic
      • respiratory difficulty
      • recurrent pulmonary infections
  • Signs and Symptoms
    • Airway compression
      • recurrent pulmonary infection
      • hemoptysis
    • Esophageal compression
      • dysphagia
    • Involvement of the spinal column
      • paralysis
    • Phrenic nerve damage
      • elevated hemidiaphragm
  • Signs and Symptoms
    • Recurrent laryngeal nerve involvement
      • Hoarseness
    • Sympathetic ganglion involvement
      • Horner’s Syndrome
        • Ptosis, miosis, anhidrosis
    • superior vena cava involvement
      • Superior vena cava syndrome
        • facial neck, and UE swelling, dyspnea, chest and UE pain, mental status changes
    Horner’s Syndrome
  • Signs and Symptoms
    • Can also be associated with systemic diseases
      • Thymoma: myasthenia gravis, immune deficiency, red cell aplastic anemia
      • Goiter: thyroxicosis
      • Thymic carcinoid: Cushing’s syndrome
      • Parathyroid: hyperparathyroidism
  • Evaluation: Imaging
    • 2 view PA/Lat Chest X-ray
      • comparisons with old x-rays important
    • Chest CT with contrast
      • most important method of evaluation
      • Can help determine location, morphology, size, and attenutation coefficient
      • Important for directing further therapy
    • MRI
      • when contrast allergy or renal failure present
      • when vascular or chest wall involvement is suspected
      • neurogenic tumors (especially helpful in detecting intraspinal component
    • Ultrasound
      • Differentiate cystic from solid masses and relate to surrounding structures
      • When mass is close to heart or pericardium
      • Transesophageal or transbronchial useful to evaluate lymph nodes, sometimes for biopsy
    • Radio nucleotide scanning
      • With radioactive iodine when thyroid tumor suspected
    • PET scanning
      • Can localize specific tumors (pheochromocytoma, paragangliomas, neuroblastomas, neurogangliomas by targeting their metabolic pathways
  • Evaluation: Laboratory
    • Depends on clinic setting, but may include:
      • Thyroid function tests
        • If goiter suspected
      • Chemistry panel including calcium and phosphate and PTH
        • If parathyroid adenoma suspected
      • Fractionated 24-hour urinary metanephrines and catecholamines
        • If paraganglionic tumor suspected
      • AFP/beta HCG
        • In all males with anterior mediastinal tumor because of concern for non-seminomatous germ cell tumor
  • Management
    • Tailored to specific or likely diagnosis
    • Must decide whether to excise, biopsy, or aspirate lesion
      • Excision should be done with teratomas, thymomas, and isolated masses likely to be benign (VATS, median sternotomy, thoracotomy)
      • Needle aspiration of cystic lesions
      • Diagnostic biopsy is procedure of choice when suspect lymphoma, germ cell tumor, or unresectable invasive malignancy
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  • References
    • Kallab, Andre MD. Superior Vena Cava Syndrome . Emedicine. August 10 2005. http://www.emedicine.com/MED/topic2208.htm
    • Gangadharan, Sidhu MD. Evaluation of Mediastinal Masses. UptoDate. October 7, 2008.
    • Parmar, Malvinder S, MB, MS. Horner’s Syndrome. Emedicine. June 5, 2008. http://www.emedicine.com/med/TOPIC1029.HTML
    • Strolls, DC, Rosado-de-Christenson, ML, Jett, JR. Primary mediastinal tumors. Part I: Tumors of the anterior mediastinum . Chest 1997; 112:511.
    • Strollo, DC, Rosado-de-Christenson, ML, Jett, JR. Primary mediastinal tumors: Part II. Tumors of the middle and posterior mediastinum . Chest 1997; 112:1344.
    • Medscape.com (multiple images)
    • Devouassoux-Shisheboran, Mojgan MD and Travis, William D MD. Pathology of Mediastnal Tumors. Uptodate. September 9 th , 2008.