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5. Stepwise pathologic progression of normal lung cellular
architecture to invasive adenocarcinoma follows which
of the following patterns?
A. Adenocarcinoma in-situ --> Minimally invasive ade-
nocarcinoma --> Lepidic predominate adenocarci-
B. Minimally invasive adenocarcinoma --> Adenocar-
cinoma in-situ --> Lepidic predominate adenocarci-
CHAPTER 19
C. Adenocarcinoma in-situ --> Lepidic predominate
adenocarcinoma --> Minimally invasive adenocarci-
D. Minimally invasive adenocarcinoma --> Lepidic pre-
dominate adenocarcinoma --> Adenocarcinoma insitu --> Invasive adenocarcinoma
Chest Wall, Lung, Mediastinum, and Pleura
6. The grade of neuroendocrine carcinoma (NEC) that is
associated with hemoptysis, pneumonia, and tumor cells
arranged in cords and clusters is:
A. Grade VI NEC.
B. Grade IV NEC.
C. Grade II NEC.
D. Grade I NEC.
Answer: A
The incidence of adenocarcinoma in the lung has increased
over the last several decades and is now the most common
type of lung cancer. It occurs more frequently in females
than in males and is the most frequent histologic subtype in
women, patients under the age of 45, and Asian populations.
Adenocarcinoma can be divided into mucinous and nonmucinous types. Based on consensus, the international working
group proposed a multidisciplinary approach, with standardized criteria and terminology for diagnosis in cytologic and
small biopsy specimens, and routine molecular testing for
known mutations, such as estimated glomerular filtration rate
(EGFR) and KRAS mutations. The new classification system
delineated a stepwise pathologic progression, from Atypical
adenomatous hyperplasia (AAH) to invasive adenocarcinoma based on the predominant histologic growth patterns;
the terms bronchioloalveolar carcinoma and mixed subtype
adenocarcinoma were eliminated in favor of more biologically
driven classification. (See Schwartz 11th ed., p. 669.)
Answer: D
Grade I NEC (classic or typical carcinoid) is a low-grade
NEC; 80% arise in the epithelium of the central airways. It
occurs primarily in younger patients. Because of the central
location, it classically presents with hemoptysis, with or without airway obstruction and pneumonia. Histologically, tumor
cells are arranged in cords and clusters with a rich vascular
stroma. This vascularity can lead to life-threatening hemorrhage with even simple bronchoscopic biopsy maneuvers.
Regional lymph node metastases are seen in 15% of patients,
but rarely spread systemically or cause death. (See Schwartz
11th ed., p. 672.)
7. CT imaging is used routinely in diagnosis of malignant
lung cancer. CT imaging findings associated with malignancy include all the following EXCEPT:
A. Size > 2 cm.
B. Irregular, lobulated, or spiculated edges.
C. Corona radiata sign.
D. Stippled or eccentric calcifications.
8. The most common pattern of benign calcification in
hamartomas is:
A. Solid.
B. Diffuse.
C. Central.
D. Popcorn.
Answer: A
Spiral (helical) CT imaging is the gold standard for definitive diagnosis of lung cancer. CT findings characteristic of
malignancy include growth over time. Increasing density
on CT scan (40% to 50% of partial solid lesions are malignant compared to only 15% of subcentimeter solid or nonsolid nodules). Size > 3 cm. Irregular, lobulated, or spiculated
edges. The finding of the corona radiata sign (consisting of
fine linear strands extending 4 to 5 mm outward and appearing spiculated on radiographs). Calcification that is stippled,
amorphous, or eccentric is usually associated with cancer.
(See Schwartz 11th ed., p. 678.)
Answer: D
CT findings characteristic of benign lesions include small
size, calcification within the nodule, and stability over time.
Four patterns of benign calcification are common: diffuse,
solid, central, and laminated or “popcorn.” Granulomatous
infections such as tuberculosis can demonstrate the first
three patterns, whereas the popcorn pattern is most common
in hamartomas. In areas of endemic granulomatous disease,
differentiating benign versus malignant can be challenging.
Infectious granulomas arising from a variety of organisms
account for 70% to 80% of this type of benign solitary nodules; hamartomas are the next most common single cause,
accounting for about 10%. (See Schwartz 11th ed., p. 685.)

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9. A patient diagnosed with lung cancer presents with
symptoms of dyspnea with exertion, hiccups, and right
shoulder pain. The most likely location of this tumor is:
A. Left lower lobe central squamous cell carcinoma.
B. Right upper lobe apical adenocarcinoma.
C. Right lower lobe peripheral large cell carcinoma.
D. Right middle lobe central squamous cell carcinoma.
10. Which of the following is NOT a known predictive or
prognostic tumor marker for adenocarcinoma?
A. Estimated glomerular filtration rate (EGFR)
B. KRAS mutation
C. AFP (Alpha feto protein)
D. EML4-ALK fusion gene
Answer: D
Nonpulmonary thoracic symptoms are often due to tumor
invasion into surrounding thoracic structures. This can lead
to symptoms of Horner syndrome (Pancoast tumors), pericardial tamponade, back pain, and recurrent laryngeal nerve palsy
leading to hoarseness and coughing. Phrenic nerve palsy is
due to invasion of a tumor into the phrenic nerve. The phrenic
nerve traverses the hemithorax along the mediastinum, parallel
and posterior to the superior vena cava and anterior to the pulmonary hilum. Tumors at the medial lung surface or anterior
hilum can directly invade the nerve; symptoms include referred
shoulder pain, hiccups, and dyspnea with exertion because of
diaphragm paralysis. Radiographically, unilateral diaphragm
elevation on chest radiograph is present. The diagnosis can be
confirmed by fluoroscopic examination of the diaphragm with
paradoxical motion with breathing and sniffing, also known as
the “Sniff Test.” (See Schwartz 11th ed., p. 680.)
Answer: C
Lung cancer management.
Role of histologic diagnosis and molecular testing. Establishing a clear histologic diagnosis early in the evaluation and
management of lung cancer is critical to effective treatment.
Molecular signatures are also key determinants of treatment algorithms for adenocarcinoma and will likely become
important for squamous cell carcinoma as well. Currently,
differentiation between adenocarcinoma and squamous cell
carcinoma in cytologic specimens or small biopsy specimens
is imperative in patients with advanced stage disease, as treatment with pemetrexed or bevacizumab-based chemotherapy
is associated with improved progression-free survival in
patients with adenocarcinoma but not squamous cell cancer. Furthermore, life-threatening hemorrhage has occurred
in patients with squamous cell carcinoma who were treated
with bevacizumab. Finally, EGFR mutation predicts response
to EGFR tumor kinase inhibitors and is now recommended
as first-line therapy in advanced adenocarcinoma. Because
adequate tissue is required for histologic assessment and
molecular testing, each institution should have a clear, multidisciplinary approach to patient evaluation, tissue acquisition,
tissue handling/processing, and tissue analysis (Fig. 19-4).
In many cases, tumor morphology differentiates adenocarcinoma from the other histologic subtypes. If no clear morphology can be identified, then additional testing for one
immunohistochemistry marker for adenocarcinoma and one
for squamous cell carcinoma will usually enable differentiation. Immunohistochemistry for neuroendocrine markers is
reserved for lesions exhibiting neuroendocrine morphology. Additional molecular testing should be performed on
all adenocarcinoma specimens for known predictive and
prognostic tumor markers (eg, EGFR, KRAS, and EML4ALK fusion gene). Ideally, use of tissue sections and cell
block material is limited to the minimum necessary at each
decision point. This emphasizes the importance of a multidisciplinary approach; surgeons and radiologists must work
in direct cooperation with the cytopathologist to ensure that
tissue samples are adequate for morphologic diagnosis as well
as providing sufficient cellular material to enable molecular
testing. (See Schwartz 11th ed., p. 683.)
CHAPTER 19
Chest Wall, Lung, Mediastinum, and Pleura

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CHAPTER 19
Chest Wall, Lung, Mediastinum, and Pleura
STEP 1
POSITIVE BIOPSY (FOB,
TBBx, Core, SLBx)
POSITIVE CYTOLOGY
(effusion, aspirate, washings,
brushings)
Histology: Lepidic, papillary, and/or
acinar architecture(s)
Cytology: 3-D arrangements, delicate
foamy/vacuolated (translucent)
cytoplasm,
Fine nuclear chromatin and often
prominent nucleoli
Nuclei are often eccentrically situated
Classic morphology:
ADC
ADC marker
and/or
Mucin +ve;
SQCC
marker –ve
(or weak in
same cells)
NE morphology, large cells,
NE IHC+
NE morphology, small cells, no
nucleoli, NE IHC+, TTF-1 +/–,
CK+
Keratinization, pearls
and/or intercellular bridges
No clear ADC or
SQCC morphology:
NSCLC-NOS
STEP 2
Apply ancillary panel of
One SQCC and one ADC marker
+/OR Mucin
NSCLC,
LCNEC
SCLC
Classic Morphology:
SQCC
NSCLC, favor SQCC
SQCC marker +ve
ADC marker –ve/or
Mucin –ve
IHC –ve and
Mucin –ve
ADC marker or Mucin +ve;
as well as SQCC marker +ve
in different cells
NSCLC, favor ADC
NSCLC NOS
Molecular analysis:
eg, EGFR mutation
FIG. 19-4. Algorithm for adenocarcinoma diagnosis in small biopsies and/or cytology. Step 1: When positive biopsies (fiberoptic
bronchoscopy [FOB], transbronchial [TBBx], core, or surgical lung biopsy [SLBx]) or cytology (effusion, aspirate, washings, and brushings)
show clear adenocarcinoma (ADC) or squamous cell carcinoma (SQCC) morphology, the diagnosis can be firmly established. If there is
neuroendocrine (NE) morphology, the tumor may be classified as small cell carcinoma (SCLC) or non-small-cell lung carcinoma (NSCLC),
probably large cell neuroendocrine carcinoma (LCNEC) according to standard criteria (+ = positive, − = negative, and ± = positive or
negative). If there is no clear ADC or SQCC morphology, the tumor is regarded as NSCLC -not otherwise specified (NOS). Step 2: NSCLC-NOS
can be further classified based on (a) immunohistochemical stains, (b) mucin (DPAS or mucicarmine) stains, or (c) molecular data. If the stains
all favor ADC-positive ADC marker(s) (ie, TTF-1 and/or mucin positive) with negative SQCC markers, then the tumor is classified as NSCLC,
favor ADC. If SQCC markers (ie, p63 and/or CK5/6) are positive with negative ADC markers, the tumor is classified as NSCLC, favor SQCC. If
the ADC and SQCC markers are both strongly positive in different populations of tumor cells, the tumor is classified as NSCLC-NOS, with a
comment it may represent adenosquamous carcinoma. If all markers are negative, the tumor is classified as NSCLC-NOS. †EGFR mutation
testing should be performed in (1) classic ADC, (2) NSCLC, favor ADC, (3) NSCLC-NOS, and (4) NSCLC-NOS, possible adenosquamous carcinoma.
In NSCLC-NOS, if EGFR mutation is positive, the tumor is more likely to be ADC than SQCC. Step 3: If clinical management requires a more
specific diagnosis than NSCLC-NOS, additional biopsies may be indicated. CD = cluster designation; CK = cytokeratin; DPAS = diastase-periodic
acid Schiff; DPAS +ve = periodic-acid Schiff with diastase; EGFR = epidermal growth factor receptor; IHC = immunohistochemistry; NB = of note;
TTF-1 = thyroid transcription factor-1; -ve = negative; +ve = positive. (Reproduced with permission from Travis WD, Brambilla E, Noguchi M, et al:
Diagnosis of lung cancer in small biopsies and cytology: implications of the 2011 International Association for the Study of Lung Cancer/American
Thoracic Society/European Respiratory Society classification, Arch Pathol Lab Med. 2013;137(5):668–684.)
†
STEP 3
If tumor tissue inadequate for molecular testing,
discuss need for further sampling — back to Step 1
NSCLC, NOS,
possible
adenosquamous ca

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11. Evaluation of mediastinal lymphadenopathy by noninvasive imaging is best performed by which of the following
modalities?
A. Positron emission tomography (PET) scan
B. CT scan
C. MRI(magnetic resonance imaging)
D. PET-CT
Answer: D
Mediastinal lymph node staging by PET scanning appears
to have greater accuracy than CT scanning. PET staging of
mediastinal lymph nodes has been evaluated in two metaanalyses. The overall sensitivity for mediastinal lymph node
metastasis was 79% (95% confidence interval [CI] 76%–82%),
with a specificity of 91% (95% CI 89%–93%) and an accuracy
of 92% (95% CI 90%–94%). In comparing PET with CT scans
in patients who also underwent lymph node biopsies, PET
had a sensitivity of 88% and a specificity of 91%, whereas CT
scanning had a sensitivity of 63% and a specificity of 76%.
Combining CT and PET scanning may lead to even greater
accuracy. In one study of CT, PET, and mediastinoscopy in
68 patients with potentially operable NSCLC, CT correctly
identified the nodal stage in 40 patients (59%). It understaged
the tumor in 12 patients and overstaged it in 16 patients. PET
correctly identified the nodal stage in 59 patients (87%). It
understaged the tumor in five patients and overstaged it in
four. For detecting N2 and N3 disease, the combination of
PET and CT scanning yielded a sensitivity, specificity, and
accuracy of 93%, 95%, and 94%, respectively. CT scan alone
yielded 75%, 63%, and 68%, respectively. Studies examining
combined PET-CT consistently show improved accuracy
compared to PET or CT alone; accuracy for PET-CT nodal
positivity confirmed by mediastinoscopy is approximately
75%, with a negative predictive value of approximately 90%.
(See Schwartz 11th ed., p. 687.)
CHAPTER 19
Chest Wall, Lung, Mediastinum, and Pleura
12. A 57-year-old non-small-cell lung cancer patient with a
potentially resectable tumor found on CT scan who can
walk on a flat surface indefinitely without oxygen or stopping to rest, secondary to dyspnea will most likely tolerate:
A. Lobectomy.
B. Pneumonectomy.
C. Single-lung ventilation.
D. Wedge resection.
Answer: A
Assessment of functional status. Patients with potentially
resectable tumors require careful assessment of their functional status and ability to tolerate either lobectomy or pneumonectomy. The surgeon should first estimate the likelihood
of pneumonectomy, lobectomy, or possibly sleeve resection,
based on the CT images. A sequential process of evaluation
then unfolds. A patient’s history is the most important tool
for gauging risk. Specific questions regarding performance
status should be routinely asked. If the patient can walk on a
flat surface indefinitely, without oxygen and without having
to stop and rest secondary to dyspnea, he will be very likely
to tolerate lobectomy. If the patient can walk up two flights
of stairs (up two standard levels), without having to stop and
rest secondary to dyspnea, she will likely tolerate pneumonectomy. Finally, nearly all patients, except those with carbon
dioxide (CO2) retention on arterial blood gas analysis, will be
able to tolerate periods of single-lung ventilation and wedge
resection. (See Schwartz 11th ed., p. 691.)

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13. A 45-year-old man with 20 pack-year history of smoking is diagnosed with squamous cell carcinoma of the
right middle lobe. Clinically the patient has symptoms of
hoarseness and coughing when drinking liquids. The T
staging for this tumor is:
A. T2a.
B. T2b.
C. T3.
CHAPTER 19
D. T4.
Chest Wall, Lung, Mediastinum, and Pleura
14. Patients with resectable tumors require assessment of
their pulmonary functional status to determine if they
are able to tolerate surgical resection. The two most reliable predictors of pulmonary functional capacity are:
A. FEV1 and DLCO.
B. RV and TLC.
C. RV and DLCO.
D. FEV1 a n d RV.
Answer: D
The staging of solid epithelial tumors is based on the TNM
staging system. The primary tumor “T” status provides
information about tumor size and relationship to surrounding structures; the “N” status provides information about
regional lymph nodes; and the “M” status provides information about the presence or absence of metastatic disease. The
designation of lymph nodes as N1, N2, or N3 requires familiarity with the lymph node mapping system. Based on clearly
delineated anatomic boundaries, accurate and reproducible
localization of thoracic lymph nodes is possible, facilitating
detailed nodal staging for individual patients and standardization of nodal assessment between surgeons. In the case
described, the patient’s symptoms of dysphagia and hoarseness
suggests involvement of the recurrent laryngeal nerve, which
confers him a T4 designation in the TNM classification system.
(See Schwartz 11th ed., p. 689.)
Answer: A
Pulmonary function studies are routinely performed when
any resection greater than a wedge resection will be performed. Of all the measurements available, the two most
valuable are forced expiratory volume in 1 second (FEV1) and
carbon monoxide diffusion capacity (DLCO). General guidelines for the use of FEV1 in assessing the patient’s ability to tolerate pulmonary resection are as follows: Greater than 2.0 L
can tolerate pneumonectomy, and >1.5 L can tolerate lobectomy. It must be emphasized that these are guidelines only. It
is also important to note that the raw value is often imprecise
because normal values are reported as “percent predicted”
based on corrections made for age, height, and gender. Notably it is not uncommon to encounter patients with significant
reductions in FEV1 and DLCO that are inconsistent with the
patient’s functional status. In these patients, exercise testing
that yields maximal oxygen consumption (V.O
) has emerged
2max
as a valuable decision-making technique to help patients
with abnormal FEV1 and DLCO. (See Schwartz 11th ed.,
p. 692.)
15. Which of the following is an indication for surgical
drainage of a lung abscess?
A. Abscess > 3 cm in diameter
B. Hemoptysis
C. Failure to decrease in size after 1 week of antibiotic
therapy
D. Persistent fever
Answer: B
Surgical drainage of lung abscesses is uncommon since drainage usually occurs spontaneously via the tracheobronchial
tree. Indications for intervention are listed in Table 19-1. (See
Schwartz 11th ed., p. 708.)
TABLE 19-1 Indications for surgical drainage procedures
for lung abscesses
1. Failure of medical therapy
2. Abscess under tension
3. Abscess increasing in size during appropriate treatment
4. Contralateral lung contamination
5. Abscess > 4–6 cm in diameter
6. Necrotizing infection with multiple abscesses, hemoptysis, abscess
rupture, or pyopneumothorax
7. Inability to exclude a cavitating carcinoma

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16. The most likely cause of aspiration pneumonia is:
A. A mixture of aerobes and anaerobes.
B. Aerobes only.
C. Anaerobes only.
D. Gram-negative bacteria.
17. The population most at risk for developing active tuberculosis is:
A. Elderly.
B. Minorities.
C. Urban residents.
D. HIV infection.
Answer: C
Microbiology. Normal oropharyngeal secretions contain many more Streptococcus species and more anaerobes (approximately 1 × 108 organisms/mL) than aerobes
(approximately 1 × 107organisms/mL). Pneumonia that follows from aspiration, with or without abscess development,
is typically polymicrobial. An average of two to four isolates present in large numbers have been cultured from lung
abscesses sampled percutaneously. Overall, at least 50% of
these infections are caused by purely anaerobic bacteria, 25%
are caused by mixed aerobes and anaerobes, and 25% or fewer
are caused by aerobes only. In nosocomial pneumonia, 60% to
70% of the organisms are gram-negative bacteria, including
Klebsiella pneumoniae, Haemophilus influenzae, Proteus spe-
cies, Pseudomonas aeruginosa, Escherichia coli, Enterobacter
cloacae, and Eikenella corrodens. Immunosuppressed patients
may develop abscesses because of the usual pathogens as well
as less virulent andopportunistic organisms such as Salmo-
nella species, Legionella species, Pneumocystis carinii, atypical
mycobacteria, and fungi. (See Schwartz 11th ed., p. 708.)
Answer: D
Epidemiology. Tuberculosis is a widespread problem that
affects nearly one-third of the world’s population. Between
8.3 and 9 million new cases of tuberculosis and 12 million
prevalent cases (range 10–13 million) were estimated worldwide in 2011 according to the World Health Organization.
Only 10,521 new cases were reported to the World Health
Organization in the United States in 2011. HIV infection is
the strongest risk factor for developing active tuberculosis.
The elderly, minorities, and recent immigrants are the most
common populations to have clinical manifestations of infection, yet no age group, sex, or race is exempt from infection.
In most large urban centers, reported cases of tuberculosis are
more numerous among the homeless, prisoners, and drugaddicted populations. Immunocompromised patients additionally contribute to an increased incidence of tuberculosis
infection, often developing unusual systemic as well as pulmonary manifestations. (See Schwartz 11th ed., p. 710.)
CHAPTER 19
Chest Wall, Lung, Mediastinum, and Pleura
18. The fungi associated with the highest mortality rate due
to invasive mycoses in the United States is:
A. Aspergillus.
B. Cryptococcus.
C. Candidia.
D. Mucor.
Answer: A
Aspergillosis. The genus Aspergillus comprises over 150 species
and is the most common cause of mortality due to invasive
mycoses in the United States. It is typically acute in onset
and life-threatening and occurs in the setting of neutropenia,
chronic steroid therapy, or cytotoxic chemotherapy. It can also
occur in the general intensive care unit population of critically ill
patients, including patients with underlying chronic obstructive
pulmonary disease (COPD), postoperative patients, patients
with cirrhosis or alcoholism, and postinfluenza patients, without any of these factors present. The species most commonly
responsible for clinical disease include A. fumigatus, A. flavus,
A. niger, and A. terreus. Aspergillus is a saprophytic, filamentous fungus with septate hyphae. Spores (2.5–3 μm in diameter)
are released and easily inhaled by susceptible patients; because
the spores are microns in size, they are able to reach the distal
bronchi and alveoli. (See Schwartz 11th ed., p. 711.)

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19. Previously, patients with severe primary pulmonary
hypertension were listed for lung transplantation due to
respiratory failure. The use of the following medication
has significantly altered the need for transplantation:
A. Epoprostenol.
B. Midodrine.
C. Dobutamine.
D. Nitrates.
CHAPTER 19
20. The most common benign chest wall tumor is:
A. Chondromas.
B. Osteochondromas.
C. Desmoid tumors.
D. Fibrous dysplasia.
Chest Wall, Lung, Mediastinum, and Pleura
21. An “onion-peel” appearance of a rib on computed
tomography is suggestive of:
A. Chondroma.
B. Ewing sarcoma.
C. Plasmacytoma.
D. Osteosarcoma.
Answer: A
Currently, virtually all patients with primary pulmonary hypertension are now treated with intravenous epoprostenol. Prostacyclin is a member of the endogenous prostanoid family and is
produced from arachidonic acid by the enzymes prostacyclin
synthase and cyclooxygenase (COX). It acts to increase pulmonary vasodilation within the lungs. Espoprostenol must be
given by continuous infusion. (See Schwartz 11th ed., p. 719.)
Answer: A
Chondroma. Chondromas, seen primarily in children and
young adults, are one of the more common benign tumors of
the chest wall. They usually occur at the costochondral junction anteriorly and may be confused with costochondritis,
except that a painless mass is present. Radiographically, lesion
is lobulated and radiodense; it may have diffuse or focal calcifications; and it may displace the bonycortex without penetration. Chondromas may grow to huge sizes if left untreated.
Treatment is surgical resection with a 2-cm margin. Large
chondromas may harbor well-differentiated chondrosarcoma
and should be managed with a 4-cm margin to prevent local
recurrence. (See Schwartz 11th ed., p. 722.)
Answer: B
Primitive neuroectodermal tumors (PNETs) and Ewing sarcoma. PNETs (neuroblastomas, ganglioneuroblastomas, and
ganglioneuromas) derive from primordial neural crest cells
that migrate from the mantle layer of the developing spinal
cord. Histologically, PNETs and Ewing sarcomas are small,
round cell tumors; both possess a translocation between the
long arms of chromosomes 11 and 22 within their genetic
makeup. They also share a consistent pattern of protooncogene expression and have been found to express the
product of the MIC2 gene. Ewing sarcoma occurs in adolescents and young adults who present with progressive chest
wall pain, but without the presence of a mass. Systemic symptoms of malaise and fever are often present. Laboratory studies reveal an elevated erythrocyte sedimentation rate and
mild white blood cell elevation.
Radiographically, the characteristic onion peel appearance
is produced by multiple layers of periosteum in the bone formation. Evidence of bony destruction is also common. The
diagnosis can be made by a percutaneous needle biopsy or an
incisional biopsy. (See Schwartz 11th ed., p. 725.)
22. Desmoid tumors are associated with which of the following gene?
A. KRAS
B. RET
C. P53
D. Adenomatous polyposis coli (APC)
Answer: D
Desmoid tumors. Soft tissue neoplasms arising from fascial
or musculoaponeurotic structures, desmoid tumors consist
of proliferations of benign appearing fibroblastic cells, abundant collagen, and few mitoses. Desmoid tumors possess
alterations in the APC/β-catenin pathway. Cyclin D1 dysregulation is thought to play a significant role in their pathogenesis. Associations with other diseases and conditions are
well documented, especially those with similar alterations in
the APC pathway, such as familial adenomatous polyposis
(Gardner syndrome). Other conditions with increased risk of
desmoid tumor formation include increased estrogen states
(pregnancy) and trauma. Surgical incisions (abdominal and
thorax) have been the site of desmoid development, either in
or near the scar. (See Schwartz 11th ed., p. 722.)

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23. Eosinophilic granulomas are associated with:
A. Langerhans cell histiocytosis.
B. Parasitic infections.
C. Crohn disease.
D. Gardner syndrome.
24. A patient with an anterior mediastinal mass and elevated
serum alpha fetoprotein most likely has:
A. A teratoma.
B. A nonseminomatous germ-cell tumor.
C. A seminomatous germ-cell tumor.
D. Metastatic hepatocellular carcinoma.
Answer: A
Eosinophilic granulomas are benign osteolytic lesions. Eosinophilic granulomas of the ribs can occur as solitary lesions or
as part of a more generalized disease process of the lymphoreticular system termed Langerhans cell histiocytosis (LCH).
In LCH, the involved tissue is infiltrated with large numbers
of histiocytes (similar to Langerhans cells seen in skin and
other epithelia), which are often organized as granulomas. The
cause is unknown. Of all LCH bone lesions, 79% are solitary
eosinophilic granulomas, 7% involve multiple eosinophilic
granulomas, and 14% belong to other forms of more systemic
LCH. Isolated single eosinophilic granulomas can occur in the
ribs or skull, pelvis, mandible, humerus, and other sites. They
are diagnosed primarily in children between the ages of 5 and
15 years. Because of the associated pain and tenderness, they
may be confused with Ewing sarcoma or with an inflammatory process such as osteomyelitis. Healing may occur spontaneously, but the typical treatment is limited surgical resection
with a 2-cm margin. (See Schwartz 11th ed., p. 722.)
Answer: B
The use of serum markers to evaluate a mediastinal mass can
be invaluable in some patients. For example, nonseminomatous
and seminomatous germ-cell tumors can frequently be diagnosed and often distinguished from one another by the levels of
α-fetoprotein (AFP) and human chorionic gonadotropin (hCG).
In >90% of nonseminomatous germ-cell tumors, either the
AFP or the hCG level will be elevated. Results are close to 100%
specific if the level of either AFP or hCG is >500 ng/mL. Some
centers institute chemotherapy based on this result alone, without biopsy confirmation of the diagnosis. In contrast, the AFP
level in patients with mediastinal seminoma is always normal;
only 10% will have elevated hCG, which is usually <100 ng/mL.
Other serum markers, such as intact parathyroid hormone level
for ectopic parathyroid adenomas, may be useful for diagnosing and also for intraoperatively confirming complete resection.
After successful resection of a parathyroid adenoma, this hormone level should rapidly normalize. (See Schwartz 11th ed.,
p. 728.)
CHAPTER 19
Chest Wall, Lung, Mediastinum, and Pleura
25. What is the most common anterior mediastinal tumor in
adults?
A. Lymphoma
B. Germ-cell tumor
C. Thymoma
D. Fibrosarcoma
Answer: C
The most common tumor of the anterior mediastinum is
thymoma. Thymomas are histologically characterized by a
mixture of epithelial cells and mature lymphocytes. Grossly
they are well encapsulated. Between 10% and 50% of patients
with thymoma will have symptoms suggestive of myasthenia
gravis or have circulating antibodies to acetylcholine receptors. Most patients with thymomas are asymptomatic. Thymectomy leads to improvement or resolution of symptoms
of myasthenia gravis in only about 25% of patients with thymomas. In contrast, in patients with myasthenia gravis and
no thymoma, thymectomy results are superior: up to 50% of
patients have a complete remission, and 90% improve. In 5%
of patients with thymomas, other paraneoplastic syndromes,
including red cell aplasia, hypogammaglobulinemia, systemic
lupus erythematosus, Cushing syndrome, or SIADH, may be
present. Large thymic tumors may present with symptoms
related to a mass effect, which may include cough, chest pain,
dyspnea, or SVC syndrome. (See Schwartz 11th ed., p. 730.)

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26. A chylothorax is likely to be present in a patient whose
pleural fluid analysis results show a triglyceride level of:
A. 80 mg/100 mL.
B. 100 mg/100 mL.
C. 45 mg/100 mL.
D. 130 mg/100 mL.
CHAPTER 19
27. All of the following are associated with increased risk of
mesothelioma EXCEPT:
A. Asbestos exposure.
B. Smoking.
C. Age > 40.
D. Male gender.
Chest Wall, Lung, Mediastinum, and Pleura
Answer: D
Laboratory analysis of the pleural fluid shows a high lymphocyte count and high triglyceride levels. If the triglyceride
level is >110 mg/100 mL, a chylothorax is almost certainly
present (a 99% accuracy rate). If the triglyceride level is
<50 mg/mL, there is only a 5% chance of chylothorax. (See
Schwartz 11th ed., p. 741.)
Answer: B
Malignant mesothelioma is the most common type of primary tumor of the pleura, with approximately 3000 cases per
year in the United States. Other, less common tumors include
benign and malignant fibrous tumors of the pleura, lipomas,
and cysts. The only known risk factor for mesothelioma is
exposure to asbestos, identified in >50% of cases. Exposure
is typically work-related in industries using asbestos in the
manufacturing process, such as shipbuilding and brake pad
linings. The risk extends to family members who are exposed
to the dust of the clothing or to the work environment. Asbestos exposure and smoking synergistically increase the risk for
lung cancer, but smoking does not increase risk for malignant
mesotheliomas. Male predominance is 2:1, and it occurs most
commonly after the age of 40. (See Schwartz 11th ed., p. 743.)

CHAPTER 20
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Congenital Heart Disease
1. The most common form of atrial septal defect (ASD) is:
A. Sinus venosus defect.
B. Ostium primum defect.
C. Ostium secundum defect.
D. Combined primum and secundum defect.
Answer: C
ASDs can be classified into three different types (Fig. 20-1):
(a) ostium secundum type defect (Fig. 20-1B,C) (deficiency
of septum primum), which are the most prevalent subtype,
comprising 80% of all ASDs; (b) ostium primum defects
(Fig. 20-1A), which may also be described as partial or transitional AV canal defect; and (c) sinus venosus type defects,
comprising approximately 5% to 10% of all ASDs. (See
Schwartz 11th ed., p. 752.)
A
FIG. 20-1. A. Echocardiogram of a patient with primum
type artial septal defect (`*’ points to the atrial septal
defect). B. Echocardiogram of a large secundum type
ASD (`*’ points to the defect). C. Intraoperative picture
during repair of atrial septal defect. A large fenestrated
atrial septum is seen. Bicaval venous cannulation has
been performed and a right atriotomy provides exposure
to the atrial septum.
B
C
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