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AP reviewer > Mediastinum > Flashcards

Mediastinum Flashcards

1
Q

Tumors of anterior mediastinum (8).

A

Thymoma, thymic cyst.
Lymphoma.
Thyroid lesions.
Parathyroid adenoma.

Germ-cell tumors.
Paraganglioma.
Hemangioma.
Lipoma.

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2
Q

Tumors of the posterior mediastinum (2).

A

Neurogenic tumors.

Gastroenteric cyst.

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3
Q

Tumors of the middle mediastinum (3).

A

Pericardial cyst.

Bronchial cyst.

Lymphoma.

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4
Q

Thymic cyst: Most common location.

A

Anterior mediastinum.

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5
Q

Acquired thymic cyst: Associations (5).

A

Lymphomas, esp. Hodgkin’s lymphoma.

Germ-cell tumors.

Other thymic tumors.

Congenital syphilis.

Previous thoracotomy.

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6
Q

Thymic cyst: Types based on gross pathology (2).

A

Unilocular: Thin wall; serous contents; congenital.

Multilocular: Thick wall; thick, turbid contents; acquired.

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7
Q

Thymic cyst: Lining (2).

A

Unilocular: Flat or cuboidal cells.

Multilocular: Variable but usually lined by flat cells.

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8
Q

Thymic cyst: Wall (3).

A

Unilocular: Usually no thymic remnants.

Multilocular: Thymic remnants.

Both: Inflammation, lymphoid follicles, cholesterol clefts; no differentiated soft tissue.

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9
Q

Cystic hygroma: Histology.

A

Cavernous spaces lined by flattened endothelium.

Collagenous stroma.

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10
Q

Thymic cyst vs. cystic thymoma.

A

Cystic thymoma: Thymomatous portion consists of disorganized thymic epithelium and T lymphocytes.

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11
Q

Bronchial cyst:

A. Age group.
B. Clinical sign.
C. Location.

A

A. Adults.

B. Movement with respiration.

C. Attached to trachea or main bronchus.

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12
Q

Bronchial cyst:

A. Lining.
B. Wall.
C. Contents.

A

A. Respiratory columnar epithelium, sometimes with squamous metaplasia.

B. Smooth muscle and cartilage.

C. Mucinous.

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13
Q

Esophageal cyst:

A. Synonym.
B. Age group.
C. Location.

A

A. Esophageal duplication.

B. Children and young adults.

C. At level of mid-esophagus.

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14
Q

Esophageal cyst:

A. Lining.
B. Wall.
C. Contents.

A

A. Squamous or columnar mucosa.

B. Double layer of smooth muscle is at least focally present.

C. Mucinous.

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15
Q

Enteric cyst:

A. Synonym.
B. Age group.

A

A. Foregut duplication cyst.

B. Infants and children.

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16
Q

Enteric cyst: Clinical associations (2).

A

Male sex.

Congenital anomalies of vertebrae, intestines, heart.

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17
Q

Enteric cyst: Location.

A

Posterior mediastinum, attached to vertebral column.

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18
Q

Enteric cyst:

A. Lining.
B. Wall.

A

A. Resembles lining of stomach, intestine, colon, or esophagus, complete with lamina propria.

B. Muscularis mucosae and muscularis propria; may contain ganglion cells.

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19
Q

Enteric cyst: Possible complications (3).

A

Respiratory symptoms.

Dysphagia due to compression.

Hemoptysis due to gastric acid.

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20
Q

Mesothelial cyst: Clinical presentation.

A

Usually asymptomatic.

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21
Q

Mesothelial cyst:

A. Lining.
B. Wall.
C. Contents.

A

A. Mesothelium.

B. Fibrous tissue; no smooth muscle.

C. Serous or (in some pericardial cysts) mucinous.

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22
Q

True thymic hyperplasia:

A. Age groups.
B. Associations (3).

A

A. Children; adults exposed to chemotherapy.

B. Myasthenia gravis, hyperthyroidism, other autoimmune diseases.

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23
Q

True thymic hyperplasia: Histology.

A

Same as that of normal thymus, but in an enlarged gland.

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24
Q

Thymic follicular hyperplasia: Associations.

A

Autoimmune diseases.

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25
Q

Thymic follicular hyperplasia:

A. Gross pathology.
B. Histology.

A

A. Usually normal size and weight.

B. Many follicles with germinal centers containing B lymphocytes.

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26
Q

Thymic follicular hyperplasia vs. normal thymic medulla.

A

Normal thymic medulla contains Hassall’s corpuscles.

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27
Q

Thymolipoma:

A. Clinical presentation.
B. Histology.

A

A. Dyspnea and cough due to compression.

B. Mature fat mixed with strands of normal thymic tissue.

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28
Q

Thymolipoma vs. thymic involution.

A

The involuted thymus is normal in size or small for the patient’s age.

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29
Q

Thymolipoma vs. lipoma.

A

Lipoma contains no thymic tissue.

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30
Q

Thymoma: Locations.

A

Typical: Anterior or superior mediastinum.

Others: Thymic rests elsewhere in the mediastinum or in the thyroid gland.

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31
Q

Thymoma: Association with myasthenia gravis (2).

A

About 10% of patients with MG have thymoma.

About 25-50% of patients with thymoma have MG.

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32
Q

Thymoma: Other associations (4).

A

Lambert-Eaton syndrome.

Pure red-cell aplasia.

Hypogammaglobulinemia.

Collagen-vascular diseases.

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33
Q

Thymoma: Most important predictor of clinical course.

A

Presence or absence of invasion of adjacent structures.

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34
Q

Thymoma: Staging system.

A

Stage I: Completely encapsulated.
Stage IIa: Microscopic breach of capsule.
Stage IIb: Macroscopic invasion into fat or pleura.
Stage III: Macroscopic invasion into pericardium, great vessels, or lung.
Stage IVa: Pericardial or pleural implants.
Stage IVb: Hematogenous or lymphatic spread.

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35
Q

Thymoma: Cellular components.

A

Neoplastic thymic epithelial cells:
− Oval or spindled, with bland nucleus.
− Epithelioid, with a vesicular nucleus and a large nucleolus.

Non-neoplastic T lymphocytes.

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36
Q

Thymoma, WHO type A: Histology.

A

Composed mainly of bland spindle cells.

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37
Q

Thymoma, WHO type AB: Histology.

A

Bland spindle cells mixed with many lymphocytes.

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38
Q

Thymoma, WHO type B: Definition.

A

A thymoma that consists mainly of epithelial cells, with variable numbers of mature and immature T lymphocytes.

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39
Q

Thymoma, WHO type B1: Histology.

A

Many lymphocytes and few epithelial cells.

The epithelial cells show minimal atypia.

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40
Q

Thymoma, WHO type B2: Histology.

A

Lymphocytes and epithelial cells are about equal in proportion.

The epithelial cells show mild to moderate atypia.

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41
Q

Thymoma, WHO type B3: Histology.

A

Few lymphocytes and many epithelial cells.

The epithelial cells show significant atypia, discrete cell borders, and rare mitotic figures.

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42
Q

WHO classification of thymomas: Correlation with histologic regions of the thymus.

A

Type A: Medullary.

Type AB: Mixed.

Type B: Cortical.

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43
Q

Thymoma: Histologic predictors of worse prognosis (4).

A

Predominance of epithelial cells.

Atypia of epithelial cells.

Loss of fibrous compartmentalization.

Loss of perivascular spaces.

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44
Q

Thymoma: Immunohistochemistry of the epithelial cells.

A

Positive: Pancytokeratin; p63, PAX8 (nuclear).

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45
Q

Thymoma: Flow cytometry.

A

In a lymphocyte-rich thymoma, flow cytometry can contribute to a misdiagnosis of lymphoblastic lymphoma.

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46
Q

Thymoma vs. lymphoblastic lymphoma.

A

Lymphoblastic lymphoma:

  • Younger patients.
  • Clonal T lymphocytes.
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47
Q

Spindle-cell thymoma vs. other spindle-cell tumors.

A

Spindle-cell sarcomas and solitary fibrous tumor do not express cytokeratin.

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48
Q

Thymic carcinoma: Associations (2).

A

May arise from thymoma.

Does not cause myasthenia gravis or pure red-cell aplasia.

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49
Q

Thymic carcinoma: Relative incidence.

A

Les common than secondary tumors of the thymus.

Primary thymic carcinoma is a diagnosis of exclusion.

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50
Q

Thymic carcinoma: Gross pathology (2).

A

Usually no capsule.

May be desmoplastic but has none of the fibrous septa of thymoma.

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51
Q

Thymic carcinoma vs. thymoma: Basic histologic differences (2).

A

Thymic carcinoma is frankly malignant.

Thymic carcinoma has lost all organotypic features.

52
Q

Thymic carcinoma: Grading.

A

Low-grade vs. high-grade.

53
Q

Thymic carcinoma: Frequent subtypes (6).

A

Keratinizing SCC.
Basaloid carcinoma.
Spindle-cell (sarcomatoid) carcinoma.
Clear-cell carcinoma.

Mucoepidermoid carcinoma.
Lymphoepithelioma-like carcinoma.

54
Q

Lymphoepithelioma-like thymic carcinoma: Viral association.

A

Rarely related to EBV.

55
Q

Spindle-cell thymic carcinoma: Association.

A

Often arises from spindle-cell thymoma.

56
Q

Thymic carcinoma: Subtypes that are typically low grade (2).

A

Basaloid carcinoma.

Papillary carcinoma.

57
Q

Thymic carcinoma: Subtypes that are typically high grade (5).

A

Clear-cell carcinoma.

Lymphoepithelioma-like carcinoma.

Anaplastic carcinoma.

Spindle-cell carcinoma.

Spindle-cell carcinosarcoma.

58
Q

Thymic carcinoma: Non-epithelial immunohistochemical markers (2).

A

CD5, CD117.

59
Q

Thymic carcinoma vs. epithelioid hemangioendothelioma.

A

Epithelioid hemangioendothelioma:

  • Cytoplasmic vacuoles.
  • Expression of vascular markers.
60
Q

Thymic carcinoma: Prognosis.

A

Low-grade thymic carcinomas are cured by excision.

All others have a poor prognosis.

61
Q

Neuroendocrine tumors of the thymus: Epidemiology.

A

Significantly more common in males.

62
Q

Thymic carcinoid tumor:

A. Clinical behavior.
B. Paraneoplastic syndromes.

A

A. More aggressive than bronchial carcinoid; can metastasize.

B. Cushing’s syndrome, SIADH, Lambert-Eaton syndrome.

63
Q

Well-differentiated neuroendocrine tumor of the thymus: Histology (4).

A

Fewer than 3 mitotic figures per 10 high-power fields.

Minimal atypia.

Classic organoid pattern.

No more than pinpoint foci of necrosis.

64
Q

Moderately differentiated neuroendocrine tumor of the thymus: Histology (3).

A

3-10 mitotic figures per 10 hpf.

Loss of organoid architecture in most cases.

Moderate cytologic atypia with large nucleoli.

65
Q

Poorly differentiated neuroendocrine tumor of the thymus: Histology (4).

A

More than 10 mitotic figures per 10 hpf.

Marked atypia or areas of small-cell carcinoma.

Total loss of organoid architecture.

Extensive necrosis.

66
Q

Neuroendocrine tumor of the thymus vs. metastatic medullary carcinoma of the thyroid.

A

Medullary carcinoma expresses CEA.

N.B.: Neuroendocrine tumors can have amyloid-like stroma and scattered expression of calcitonin.

67
Q

Neuroendocrine tumor of the thymus vs. paraganglioma.

A

Paraganglioma does not express cytokeratin.

68
Q

Neuroendocrine tumor of the thymus: Clinical predictors of poor prognosis (2).

A

Association with MEN syndrome.

Production of ACTH.

69
Q

Chronic mediastinitis:

A. Site.
B. Age group.

A

A. Anterior-superior mediastinum.

B. Most common in young adults.

70
Q

Chronic mediastinitis: Causes (5).

A

Fungi: Histoplasma, Aspergillus.

Nocardia.

Mycobacteria.

Methysergide.

Idiopathic.

71
Q

Chronic mediastinitis:

A. Radiography.
B. Possible complication (2).

A

A. Widened mediastinum.

B. Superior-vena-cava syndrome; compression and thrombosis of pulmonary vein.

72
Q

Chronic mediastinitis: Histology.

A

Dense, hyalinized fibrous tissue.

Granulomas.

73
Q

Hodgkin’s lymphoma of the thymus: Typical site.

A

Anterior mediastinum.

74
Q

Hodgkin’s lymphoma of the thymus: Primary vs. secondary disease.

A

Primary disease: Diagnosis requires presence of classic Reed-Sternberg cells in a background consistent with Hodgkin’s lymphoma.

Secondary disease: Presence of mononuclear Reed-Sternberg-type cells in a background consistent with Hodgkin’s lymphoma is sufficient.

75
Q

Hodgkin’s lymphoma of the thymus vs. sclerosing large-cell lymphoma (4).

A

Sclerosing large-cell lymphoma:

  • Sclerosis is diffuse rather than in broad bands.
  • Few or no small lymphocytes, plasma cells, or eosinophils.
  • Tumor cells express B-cell markers.
  • Negative for CD15 (but may express CD30).
76
Q

Lymphoblastic lymphoma: Age group.

A

Occurs mainly (but not exclusively) in older children and adolescents.

77
Q

Lymphoblastic lymphoma: Frequent clinical presentation.

A

Acute respiratory distress due to rapid growth of tumor.

78
Q

Lymphoblastic lymphoma: Cytology (3).

A

Absent or inconspicuous nucleoli.

Nucleus may be convoluted and resemble a transverse section of a brain.

Necrosis can show starry-sky pattern or be extensive.

79
Q

Lymphoblastic lymphoma: Immunophenotype (5,2,1).

A

Positive: CD1a, CD3, CD43, CD99, TdT.

Variable: CD4, CD8.

Negative: CD20.

80
Q

Lymphoblastic lymphoma: Limitations of flow cytometry.

A

May not distinguish lymphoblastic lymphoma from the benign immature lymphocytes of lymphocyte-rich thymomas.

81
Q

Castleman’s disease: Types (3).

A

Hyaline-vascular.

Plasma-cell (including multicentric variant).

Mixed.

82
Q

Castleman’s disease: Possible signs and symptoms (7).

A

Fever.

Hepatosplenomegaly.

Anemia, pancytopenia.

Hypergammaglobulinemia, increased ESR.

Proteinuria.

83
Q

Castleman’s disease: Malignant associations (3).

A

Non-Hodgkin’s lymphoma.

Carcinoma.

Kaposi’s sarcoma.

84
Q

Castleman’s disease: Viruses (2).

A

Multicentric variant: HIV, HHV-8.

85
Q

Castleman’s disease, hyaline-vascular type: Histology (4).

A

Hyalinization of blood vessels that supply germinal centers.

Small, hyalinized germinal centers, sometimes many within one follicle.

“Onion-skin” pattern of mantle zones.

Rich network of capillaries in the interfollicular zone.

86
Q

Castleman’s disease, plasma-cell type: Histology.

A

Sheets of plasma cells between the follicles.

Large, reactive germinal centers.

87
Q

Castleman’s disease, mixed type: Histology.

A

Combines features of hyaline-vascular and plasma-cell types.

88
Q

Castleman’s disease: Immunohistochemistry.

A

CD5-positive lymphocytes congregate at the peripheral of the abnormal follicles.

89
Q

Castleman’s disease: Clonality.

A

Typically polyclonal, being a reactive process; however, monoclonal populations of plasma cells have been described.

90
Q

Severe combined immunodeficiency: Thymic histology (3).

A

No corpuscles of Hassall.

Few or no lymphocytes.

Lobules of spindled or polygonal epithelial cells.

91
Q

AIDS: Thymic histology (2).

A

Severe depletion of lymphocytes.

Hassall’s corpuscles may be lost.

92
Q

Graft-versus-host disease: Thymic histology (3).

A

Severe depletion of lymphocytes.

Hassall’s corpuscles may be lost.

Thymic epithelial cells may undergo apoptosis.

93
Q

Most frequent mediastinal germ-cell tumor.

A

Teratoma.

94
Q

Teratoma with additional malignant components: Types (4).

A

Type I: Teratoma with carcinoma.

Type II: Teratoma with another germ-cell tumor.

Type III: Teratoma with sarcoma.

Type IV: Teratoma with any combination of the above.

95
Q

Mediastinal germinoma:

A. Typical patient.
B. Possible clinical presentations (2).

A

A. Male, second through fourth decade.

B. Cervical lymphadenopathy, superior-vena-cava syndrome.

96
Q

Mediastinal germinoma:

A. Nucleoli of tumor cells.
B. Location of lymphocytes.

A

A. Described as “spiked”.

B. In the fibrous septa.

97
Q

Mediastinal germinoma: Processes that can obscure the tumor cells (3).

A

Granulomatous reaction.

Follicular hyperplasia.

Stromal sclerosis and hyalinization.

98
Q

Mediastinal germinoma: Immunohistochemistry.

A

Positive: PLAP, CD117, OCT-4.

Paranuclear dotlike expression of cytokeratin in 80% of cases.

99
Q

Mediastinal germinoma: Special stain.

A

PAS.

100
Q

Nonseminomatous germ-cell tumors of the thymus: Preoperative indicators of a choriocarcinomatous component.

A

Gynecomastia.

hCG >500 IU.

101
Q

Nonseminomatous germ-cell tumors of the thymus: Associated syndrome.

A

Kleinfelter’s syndrome.

102
Q

Yolk-sac tumor: Reticular pattern.

A

Netlike arrangement of variably sized spaces; also known as the microcystic pattern.

103
Q

Yolk-sac tumor: Polyvesicular-vitelline pattern.

A

Saclike structures in a myxoid or fibrous stroma.

104
Q

Yolk-sac tumor: Feature common to all types.

A

Intra- or extracellular hyaline globules that contain α₁-antitrypsin.

105
Q

Choriocarcinoma: Cellular components.

A

Cytotrophoblasts.

Giant syncytiotrophoblasts.

106
Q

Nonseminomatous germ-cell tumors: Frequency of immunohistochemical reactivity for ___.

A. Cytokeratin.
B. PLAP.
C. AFP.

A

A. All tumors.

B. About half of tumors.

C. 30% of embryonal carcinomas, most yolk-sac tumors.

107
Q

Nonseminomatous germ-cell tumors: Mutation.

A

Isochromosome 12p.

108
Q

Nonseminomatous germ-cell tumors of the thymus: Histologic indicators of poor prognosis (2).

A

Extrathymic invasion or metastasis on presentation.

Sarcomatoid transformation.

109
Q

Nonseminomatous germ-cell tumors of the thymus: Association with hematological malignancy.

A

Yolk-sac tumor may give rise to AML (acute myelomonocytic or acute megakaryoblastic).

110
Q

Neurogenic tumors of thymus:

A. Special property.
B. Most common.

A

A. More likely to show differentiation than neurogenic tumors of other sites.

B. Ganglioneuroma.

111
Q

Neuroblastoma:

A. Age group.
B. Prognosis.

A

A. 90% of patients are <5 years old.

B. Depends on stage; average 3-year survival is 30%.

112
Q

Neuroblastoma: Histology.

A

Small, round, blue cells (neuroblasts) forming Homer Wright pseudorosettes.

113
Q

Ganglioneuroblastoma: Histology.

A

Neuroblastoma + ganglion cells, the latter making up more than half of the tumor.

114
Q

Ganglioneuroma: Histology.

A

Maturing: Differentiating neuroblasts, ganglion cells, neuropil.

Mature: Sheets of Schwann cells and clusters of mature ganglion cells.

115
Q

Immunohistochemical profile of ___.

A. Neuroblasts (3).
B. Schwann cells (3).

A

A. Neurofilament, synaptophysin, chromogranin.

B. S100, GFAP, myelin basic protein.

116
Q

Neuroblastoma: Genetic findings associated with better prognosis (4).

A

Near-triploidy or hyperdiploidy.

Presence of 1p36 tumor-suppressor gene.

No amplification of N-MYC.

No expression of TRKA factor receptor.

117
Q

Neuroblastoma: Genetic findings associated with worse prognosis (3).

A

Tetraploidy or near-diploidy.

Loss of heterozygosity for 1p36.

Amplification of N-MYC (>10 copies).

118
Q

Most common tumor of the posterior mediastinum.

A

Schwannoma.

119
Q

Schwannoma: Periphery.

A

Well-formed fibrous capsule.

120
Q

Ancient schwannoma: Histology (3).

A

Stromal sclerosis.

Atypical nuclei.

Little or no mitotic activity.

121
Q

Cellular schwannoma: Histology (3).

A

Highly cellular, consisting exclusively of Antoni A areas.

Variable nuclear atypia.

Low mitotic activity.

122
Q

Schwannoma: Immunohistochemistry (3).

A

Positive:

  • S100 (strong in cellular schwannoma).
  • CD57.
  • GFAP.
123
Q

Schwannoma: Classic finding on electron microscopy.

A

Luse bodies, which consist of collagen fibrils with a periodicity of 130 nm.

124
Q

Metastases to the thymus:

A. Most common primary sites (4).
B. Distinction from primary tumors of the thymus.

A

A. Trachea, bronchi, lung, esophagus.

B. Immunohistochemistry may not help; clinical history is always essential.

125
Q

Tumors of the superior mediastinum (4).

A

Thymoma, thymic cyst.

Lymphoma.

Thyroid lesions.

Parathyroid adenoma.

AP reviewer (17 decks)

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