Mediastinal Diseases Flashcards

1
Q

diagnostic problems for mediastinal pathology

A

the mediastinum is a confined space that is difficult to clinically evaluate and adequately biopsy

there are many organs in this small compartment that can have many different pathologic processes

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2
Q

What is the most common type of mediastinal tumors?

A

neurogenic tumors

thymoma

cysts

lymphoma

germ cell tumors

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3
Q

structures found in the mediastinum

A

thumas

parathyroids

heart

lungs

trachea

esophagus

great vessels

lymph nodes

intercostal nerves and sympathetic ganglia

supporting mesenchymal and bony structures and mesothelium

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4
Q

Where are malignant mediastinal tumors mainly found?

A

anterior/superior compartment

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5
Q

Major tumors of the superior mediastinum

A

four T’s:

thymoma

T-cell-rich lymphomas

teratomas (and other germ cell tumors)

thyroid (and parathyroid)

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6
Q

major pathological process of the middle mediastinum

A

bronchogenic/pericardial cyst

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7
Q

major pathological processes of the posterior mediastinum

A

neurilemmoma

neurofibroma

ganglioneuroma/neuroblastoma

malignant PNST

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8
Q

major pathological processes of the anterior mediastinum

A

thymoma

germ cell tumors

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9
Q

malignant vs. benign tumors of the mediastinum

A

80% of asymptomatic processes are benign

25% of 40% of mediastinal tumors are malignant

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10
Q

diagnostic techniques for mediastinal masses.

A

CR and MRI

ultrasound

nuclear scan

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11
Q

methods for mediastinum biopsy

A

CT-guided needle biopsy

cervical mediastinoscopy and parasternal mediastinotomy

VATS - video-assisted thoracoscopy

open thoracotomy

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12
Q

What are the types of thymus hyperplasia?

A

true hyperplasia

follicular thymic hyperplasia

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13
Q

true hyperplasia

A

increase in all elements of th ethymus, so weight is greater than expected for age

compensatory hyperplasia observed after stressful events

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14
Q

follicular thymic hyperplasia

A

increase in B and T cells lymphodi follicles

observed in close to 75% o fpatients with myasthenia gravis and other autoimmune diseases

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15
Q

thymoma

A

most common tumor of the anterior and superior mediastinum

mostly asymptomatic, 1/3 have chest pain, cough, dyspnea, or superior vena cava syndrome

30-50% are associated with myasthenia gravis

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16
Q

histological classification of thymomas

A

generally benign

classified as spindled or epithelioid

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17
Q

Type A thymoma

A

spindle cell, good prognosis

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18
Q

Type B thymoma

A

B1 - lymphocyte-rich, scattered epithelial cells

B2 - lymphocytes = epithelial cells

B3 - epithelial-rich, scattered lymphocytes

B3>B2>B1 in terms of prognosis

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19
Q

Type C thymoma

A

thymic carcinoma

poor prognosis

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20
Q

Masaoka stage 1

A

completely encapsulated thymoma macroscopically and microscopically

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21
Q

Masaoka stage 2

A

thymoma macroscopically invades fatty tissue outside thymus, or microscopically invades through capsule

22
Q

Masaoka stage 3

A

tumor macroscopically invades into neighboring organs such as great vessels, lung, or pericardium

23
Q

Masaoka stage 4

A

lymphatic or hematogenous metastasis, or disseminated pleural/ericardial spread

24
Q

treatment for thymomas

A

thoracotomy and complete excision

complete excision and radiotherapy with/without chemotherapy

25
primary lymphoma of mediastinum and types of disease found here
commonly located anteriosuperior, and less often, middle mediastinum **Hodgkin's Lymphoma/Disease** - most common **acute lymphoblastic leukemia/lymphoma (NHL)** - most common in non-Hodgkin's lymphoma in children/adolescents **mediastinal large cell lymphoma (B-cell type)** - most common primary form of NHL in adults
26
Hodgkin's Lymphoma
most common, presents in young adult females as an anterosuperior mediastinal mass treated with chemotherapy and radiation Reed-Sternberg cells are diagnostic in the histology
27
acute lymphoblastic leukemia/lymphoma
T-cell type a rapidly progresssive lymphoma that quickly involves blood, and lymph nodes, and bone found in children/adolescent males SVC syndrome, respiratory distress treat with intense chemotherapy and bone marrow transplant
28
mediastinal large cell lymphoma
B-cell type found in young females usually tumor confined to chest majority go into complete remission with intensive chemotherapy wiht/without radiation
29
germ cell tumors
found in anterior mediastinum subtypes include: teratoma - most common germinoma (seminoma) other GCTs more commonly associatd with Klinefelter syndrome
30
teratoma
most common in adolescents neonates and young children have good prognosis in postpubertal patients prognosis is worse if associated with other malignant germ cells, immature elements, or somatic malignancy
31
histologic findings of teratoma
mucosa and skin adnexa with underlying mucinous glands
32
seminoma
most common malignant germ cell tumor almost exclusively in males most patients symptomatic, 10% have SVC good prognosis if treated with chemotherapy or radiation
33
histology of seminomas
epithelioid cells with cleared (glycogenated) cytoplasm and large nuclei with nucleoli arranged in nests surrounded by fibrovascular stroma with lymphocytes
34
beta-HCG
high levels strongly suggest the presence of highyl aggressive choriocarcinoma
35
What is the marker for yold sac tumors?
high levels of serum alpha-fetoprotein
36
neuroendocrine tumors o fthe mediastinum
**carcinoid tumors** located in the anteriosuperior mediastinum 35% functional, ACTH most comon hormone produced prognosis is poor, particularly in patients with tumors exhibiting metastatic spread
37
histology of carcinoid tumors
epithelial cells with rounded nuclei having finely granular (salt and pepper) chromatin rosette formation is common
38
neurogenic tumors
located in the posterior mediastinum scwannoma is most common neurofibroma is next - associated with neurofibromatosis type I
39
histology of as schwannoma
encapsulated, admixture of cellular Antoni A areas with Verocay bodies and hypocellular vascular antoni B areas dumbbell shaped tumor that can occasionally extend inside the spinal canal treated with complete resection
40
neurofibroma
condition associated with malignant peripheral nerve sheath tumors plexiform neurofibroma and malignant schwannoma
41
histology of plexiform neurofibromas
distended and enlarged nerve fascicles composed of Schwann cells, fibroblasts, and neurites in a myxoid stroma
42
histology of malignant schwannoma
cellular fascicles of highly atypical spindled cells with irregular nuclear contours stroma may be focally myxoid
43
ganglioneuroma
older children and adults usually asymptomatic, but may be associated with diarrhea diarrhea due to vasoactive intestinal polypeptide production by ganglion cells
44
histology of ganglioneuromas
fascicles of elongated Schwann cels and mature (but dysplastic) ganglion cells
45
cysts of the mediastinum
thymic cyst pericardial cyst bronchogenic cyst surgery is treatment of choice
46
thymic cyst
located in th eanterosuperior mediastinum generally asymptomatic in adults unilocular if congenital multilocular if acquired
47
histology of thymic cysts
inflammation, residual thymic tissue in 50% of cases, degenerative features influding presence of cholesterol clefts
48
lesions of the mediastinum
chronic fibrosing mediastinitis infectious granulomas sarcoidosis
49
chronic fibrosing mediastinitis
located in the middle/anterior mediastinum present with chest pain, fever, hemoptysis, SVC syndrome two important forms are infectious lymphadenitis (due to histoplasma) and IgG type 4 plasma-cell diseases (responds to steroids) can also be caused by other types of granulomatous disease, including TB
50
histology of fibrosing mediastinitis
lymphoplasmacytic inflammation and fibroblasts and dense fibrosis are histologic features associated with IgG type 4 lesions obliterative phlebitis is a common finding