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
Q

primary lymphoma of mediastinum and types of disease found here

A

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
Q

Hodgkin’s Lymphoma

A

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
Q

acute lymphoblastic leukemia/lymphoma

A

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
Q

mediastinal large cell lymphoma

A

B-cell type

found in young females

usually tumor confined to chest

majority go into complete remission with intensive chemotherapy wiht/without radiation

29
Q

germ cell tumors

A

found in anterior mediastinum

subtypes include:

teratoma - most common

germinoma (seminoma)

other GCTs more commonly associatd with Klinefelter syndrome

30
Q

teratoma

A

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
Q

histologic findings of teratoma

A

mucosa and skin adnexa with underlying mucinous glands

32
Q

seminoma

A

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
Q

histology of seminomas

A

epithelioid cells with cleared (glycogenated) cytoplasm and large nuclei with nucleoli arranged in nests surrounded by fibrovascular stroma with lymphocytes

34
Q

beta-HCG

A

high levels strongly suggest the presence of highyl aggressive choriocarcinoma

35
Q

What is the marker for yold sac tumors?

A

high levels of serum alpha-fetoprotein

36
Q

neuroendocrine tumors o fthe mediastinum

A

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
Q

histology of carcinoid tumors

A

epithelial cells with rounded nuclei having finely granular (salt and pepper) chromatin

rosette formation is common

38
Q

neurogenic tumors

A

located in the posterior mediastinum

scwannoma is most common

neurofibroma is next - associated with neurofibromatosis type I

39
Q

histology of as schwannoma

A

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
Q

neurofibroma

A

condition associated with malignant peripheral nerve sheath tumors

plexiform neurofibroma and malignant schwannoma

41
Q

histology of plexiform neurofibromas

A

distended and enlarged nerve fascicles composed of Schwann cells, fibroblasts, and neurites in a myxoid stroma

42
Q

histology of malignant schwannoma

A

cellular fascicles of highly atypical spindled cells with irregular nuclear contours

stroma may be focally myxoid

43
Q

ganglioneuroma

A

older children and adults

usually asymptomatic, but may be associated with diarrhea

diarrhea due to vasoactive intestinal polypeptide production by ganglion cells

44
Q

histology of ganglioneuromas

A

fascicles of elongated Schwann cels and mature (but dysplastic) ganglion cells

45
Q

cysts of the mediastinum

A

thymic cyst

pericardial cyst

bronchogenic cyst

surgery is treatment of choice

46
Q

thymic cyst

A

located in th eanterosuperior mediastinum

generally asymptomatic in adults

unilocular if congenital

multilocular if acquired

47
Q

histology of thymic cysts

A

inflammation, residual thymic tissue in 50% of cases, degenerative features influding presence of cholesterol clefts

48
Q

lesions of the mediastinum

A

chronic fibrosing mediastinitis

infectious granulomas

sarcoidosis

49
Q

chronic fibrosing mediastinitis

A

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
Q

histology of fibrosing mediastinitis

A

lymphoplasmacytic inflammation and fibroblasts and dense fibrosis are histologic features associated with IgG type 4 lesions

obliterative phlebitis is a common finding