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Flashcards in Mediastinal Diseases Deck (50):
1

diagnostic problems for mediastinal pathology

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

2

What is the most common type of mediastinal tumors?

neurogenic tumors

thymoma

cysts

lymphoma

germ cell tumors

3

structures found in the mediastinum

thumas

parathyroids

heart

lungs

trachea

esophagus

great vessels

lymph nodes

intercostal nerves and sympathetic ganglia

supporting mesenchymal and bony structures and mesothelium

4

Where are malignant mediastinal tumors mainly found?

anterior/superior compartment

5

Major tumors of the superior mediastinum

four T's:

thymoma

T-cell-rich lymphomas

teratomas (and other germ cell tumors)

thyroid (and parathyroid)

6

major pathological process of the middle mediastinum

bronchogenic/pericardial cyst

7

major pathological processes of the posterior mediastinum

neurilemmoma

neurofibroma

ganglioneuroma/neuroblastoma

malignant PNST

8

major pathological processes of the anterior mediastinum

thymoma

germ cell tumors

9

malignant vs. benign tumors of the mediastinum

80% of asymptomatic processes are benign

25% of 40% of mediastinal tumors are malignant

10

diagnostic techniques for mediastinal masses.

CR and MRI

ultrasound

nuclear scan

11

methods for mediastinum biopsy

CT-guided needle biopsy

cervical mediastinoscopy and parasternal mediastinotomy

VATS - video-assisted thoracoscopy

open thoracotomy

12

What are the types of thymus hyperplasia?

true hyperplasia

follicular thymic hyperplasia

13

true hyperplasia

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

compensatory hyperplasia observed after stressful events

14

follicular thymic hyperplasia

increase in B and T cells lymphodi follicles

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

15

thymoma

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

16

histological classification of thymomas

generally benign

classified as spindled or epithelioid

17

Type A thymoma

spindle cell, good prognosis

18

Type B thymoma

B1 - lymphocyte-rich, scattered epithelial cells

B2 - lymphocytes = epithelial cells

B3 - epithelial-rich, scattered lymphocytes

B3>B2>B1 in terms of prognosis

19

Type C thymoma

thymic carcinoma

poor prognosis

20

Masaoka stage 1

completely encapsulated thymoma macroscopically and microscopically

21

Masaoka stage 2

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

22

Masaoka stage 3

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

23

Masaoka stage 4

lymphatic or hematogenous metastasis, or disseminated pleural/ericardial spread

24

treatment for thymomas

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