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THEODORE D. LIWONGAN
1. ANATOMY
The mediastinum comprises an anatomic space located between the thoracic inlet and
the diaphragm, and bordered on the left and right sides by the pleural cavities.
This central anatomic location houses or borders vital structures of almost every
major organ system including the heart and great vessels of the circulatory system,
the esophagus, and major airways of the aerodigestive tract, the thymus of the
immune system, and important nerves such as the phrenic and vagus nerves. Further,
various endocrine organs may project into it, distant malignancies may metastasize
to it, and infectious processes can manifest themselves within it.
3. DIAGNOSIS
Mediastinal masses rarely cause chest pain. Most attract attention by compression
of normal structures or are found incidentally on chest X-ray. Signs suggesting
mediastinal tumors are dyspnea from retrosternal goiter, hoarseness, and brassy
cough from compression of the recurrent laryngeal nerve, Horner syndrome (unilateral
ptosis, miosis, and anhidrosis) from involvement of the superior cervical ganglion,
edema of the arms and neck with cyanosis from obstruction of the SVC, and chylous
pleural effusion. Lymph nodes are enlarged in Hodgkin disease, non-Hodgkin lymphoma,
carcinoma, germ cell tumors, or tuberculosis. Other locations of neoplastic tissue
are retrosternal goiter, thymoma, and teratoma (dermoid cyst). When a dermoid forms
a tracheal fistula, it may produce trichoptysis, coughing up of hair.
THYMOMA
4.1.1. PATHOLOGY
Thymomas are neoplasms of thymic epithelial cells. They almost always
occur in adult life and most (80%) are benign.
Most thymomas are in the anterosuperior mediastinum, although a few
may occur elsewhere where thymic tissue is present, such as the neck, middle
and posterior mediastinum and pulmonary hilus. Benign
thymomas are irregularly shaped masses that vary from
a few centimeters to 15 cm or more. They are
encapsulated, firm and gray to yellow tumors that are
divided into lobules by fibrous septa. Large thymomas
may
have foci of hemorrhage, necrosis and cystic
degeneration. Sometimes, the entire thymoma is cystic and multiple sections
are required to identify the true nature of the lesion.
Thymomas contain a mixture of neoplastic epithelial cells and
nontumorous lymphocytes. The proportions of these elements vary from case to
case, and even among different lobules. The epithelial cells are plump or
spindle shaped, with vesicular nuclei. In cases in which epithelial cells
predominate, they may show organoid differentiation, including perivascular
spaces with lymphocytes and macrophages, tumor cell rosettes and whorls
suggesting abortive Hassall corpuscles.
4.1.4. STAGING
The staging system for thymoma was developed by Masaoka and colleagues.
It is an anatomic system in which the stage is increased on the basis
of the degree of invasiveness. The 5-year survival of patients in the
various stages is as follows: stage I, 96%; stage II, 86%; stage III,
69%; and stage IV, 50%. The French Study Group on Thymic Tumors (GETT)
has proposed modifications to the Masaoka scheme based on the degree of
surgical removal because the extent of surgery has been noted to be a
prognostic indicator. In their system, stage I tumors are divided into
A and B on the basis of whether the surgeon suspects adhesions to adjacent
structures; stage III tumors are divided into A and B based on whether
disease was subtotally resected or only biopsied. The concurrence between
the two systems is high.
Stage
I
Diagnostic Criteria
Macroscopically and microscopically completely encapsulated; no invasion through capsule
II
IIA Microscopic invasion outside the capsule
IIB Macroscopic invasion into surrounding fat or grossly adherent to pleura or pericardium
III
IIIA Macroscopic invasion into neighboring organs, pericardium, or pleura but not great vessels
IIIB Macroscopic invasion into neighboring organs that includes great vessels
IV
IVA Pleural or pericardial dissemination
IVB Lymphatic or hematogenous metastases
Stage Distribution, %
5-Year Survival, %
10-Year Survival, %
36
95100
86100
II
26
70100
50100
III
22
6889
4760
IV
10
4769
011
4.2.
5.1.
NEURILEMOMA (SCHWANNOMA)
5.2.
NEUROFIBROMA
5.3.
NEUROSARCOMA
6. THERAPY
6.1.
6.2.
6.3.
6.4.
SURGICAL
RADIOTHERAPY
CHEMOTHERAPY
RADIOCHEMOTHERAPY
Treatment for mediastinal tumors depends on the type of tumor and symptoms:
ATTACHMENT
1. Mediastinum
1.1.
Superior
1.2.1. Anterior
1.2.1.1. Loose areolar tissue
1.2.1.2. Lymphatic vessels which ascend from the convex surface of the
liver
1.2.1.3. Two or three anterior mediastinal lymph nodes
1.2.1.4. The small mediastinal branches of the internal thoracic artery
1.2.1.5. Thymus (involuted in adults)
1.2.2. Media
1.2.2.1. Pericardium
1.2.2.2. Heart
1.2.3. Posterior
1.2.3.1. Artery
1.2.3.1.1.
Thoracic part of the descending aorta
1.2.3.2. Veins
1.2.3.2.1.
Azygos vein
1.2.3.2.2.
The hemiazygos vein and the accessory hemiazygos vein
1.2.3.3. Nerves
1.2.3.3.1.
Vagus nerve
1.2.3.3.2.
Splanchnic nerves
1.2.3.3.3.
Sympathetic chain
1.2.3.4.
Esophagus
1.2.3.5.
Thoracic duct
1.2.3.6.
Some lymph glands
Structures
Common Lesions
Rare Lesions
Anterior compartment
Thymus
Thymomas
Lymphomas
Vascular lesions
Mesenchymal tumors
Endocrine tumors (e.g., ectopic
parathyroid, goiter)
Thyroid (occasional)
Castlemans disease
Middle compartment
Heart
Foregut cysts
Pericardium
Lymphoma
Castleman disease
Vascular tumors
Aorta
Superior and inferior
vena cava
Trachea and main bronchi
Lymph nodes
Posterior compartment
Descending aorta
Structures
Esophagus
Common Lesions
Neurogenic tumors
Rare Lesions
Mesenchymal tumors
Vagus nerves
Lymphatic lesions
Thoracic duct
Neurenteric cysts
Sympathetic chain
Lymph nodes
Resources:
LeBlond RF, Brown DD, Suneja M, Szot JF. The Chest: Chest Wall, Pulmonary, and
Cardiovascular Systems; The Breasts. In: LeBlond RF, Brown DD, Suneja M, Szot JF.
eds. DeGowins Diagnostic Examination, 10e. New York, NY: McGraw-Hill; 2015
Burt BM, Shrager JB. Benign and Malignant Neoplasms of the Mediastinum. In: Grippi
MA, Elias JA, Fishman JA, Kotloff RM, Pack AI, Senior RM, Siegel MD. eds.
Fishman's Pulmonary Diseases and Disorders, Fifth Edition. New York, NY: McGrawHill; 2015
Essential Clinical Anatomy 5
th
ed Moore