Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3902_Библиотеки_им_академика_М_И_Перельмана
.pdf
86
dysphagia requiring a gastric tube placement, she was eventu-
ally discharged home approximately 4weeks after her opera-
tion. Her final pathology noted high grade intimal sarcoma
with PD-L1 positive staining. She is now being followed by
radiation oncology and medical oncology and undergoing
treatment for residual/metastatic pulmonary artery sarcoma.
Radiographic Interpretation
From the outset, CT images in this case were very suggestive
of a vascular tumor. The selected images from CT pulmonary
angiography (Fig.9.1) demonstrated an expansile mass within
the main and left pulmonary artery (block arrow). The lesion
extends to the level of the pulmonary valve and is of uni-
F . Fusion PET CT images of the chest (see “Radiographic
Interpretation
” section for image explanation)
F . Surgical specimen removed at time of endarterectomy
D. G. Papamatheakis et al.
https://t.me/med1917
87
formly low attenuation with no calcification. Careful review
reveals breach of the pulmonary artery wall (thin arrow) with
extension of soft tissue in to the mediastinum. The loss of the
integrity of the vascular wall is highly suspicious for an
aggressive lesion such as a sarcoma. Chronic thromboembolic
disease can involve the main pulmonary arteries, but the CT
features were not typical of thrombus.
The chest radiograph (Fig.9.2) demonstrated enlargement
of the main pulmonary artery with reduced vascular mark-
ings in the left lung with few tiny foci of parenchymal
scarring.
On VQ scintigraphy (Fig.9.3) there was complete absence
of perfusion to the left lung. Surprisingly, there was also a
segmental wedge shaped mismatched perfusion defect in the
right lung; this could be due to tumor “thrombus” or vascular
metastases.
The positron emission tomography (PET) CT (Fig. 9.4)
confirmed high FDG uptake in the soft tissue within the pul-
monary arteries in keeping with a metabolically active intra-
vascular malignancy.
Clinical Comments
This case is a dramatic illustration that proximal pulmonary
intra-arterial filling defects are not universally thromboem-
bolic in nature. In fact, there are a number of historical and
radiographic hints, particularly on CT, that push the differen-
tial away from this being a large proximal vessel thromboem-
bolus. That this patient experienced a decline in exercise
capabilities over the months prior to presentation suggests a
more chronic and progressive process. An acute saddle
embolus of this size would not typically present in this man-
ner. Additionally, a thromboembolus would not be expected
to simply “halt” in the proximal main pulmonary artery, with
contrast seen to completely opacify a normal sized vessel
beyond the globular shaped lesion (Fig.9.1). Other features
that might suggest a pulmonary vascular tumor include
Chapter 9. Case 9: Pulmonary Artery Sarcoma …
https://t.me/med1917
88
“eclipsing” or “breach” of the pulmonary arterial wall from
the mass like lesion (see Gan etal. 2013), extension of the
mass involving the right ventricular outflow tract and pul-
monic valve, size progression of the mass despite anticoagula-
tion, and the presence of nodular lesions “within the
parenchyma” following the course of the pulmonary arteries.
Moreover, the presence of radiographic and/or echocardio-
graphic signs of pulmonary hypertension with such a large
burden of occlusive vascular disease would not be anticipated
with an acute massive pulmonary embolus, where cardiogenic
shock might be expected at the time of presentation.
An appreciation of some of the clinical and radiographic
aspects of pulmonary vascular tumors can reduce the time
interval to appropriate therapeutic intervention, including sur-
gical endarterectomy and adjuvant modalities appropriate to
the patient’s tumor type and clinical condition. A clinical sus-
picion of this unusual tumor might also prevent ineffectual and
potentially life threatening attempts at reperfusion therapy.
Points ofEmphasis
The differential for major pulmonary arterial mass lesions
includes vascular neoplasm. The clinical history and distinc-
tive radiographic findings, particularly with CT and metabolic
imaging, can be helpful in making this important distinction
from acute or chronic thromboembolic disease.
Diagnosis
1. Pulmonary artery sarcoma.
Further Reading
Gan H-L, Zhang J-Q, Huang X-Y, Yu W.The wall eclipsing sign on
pulmonary artery computed tomography is pathognomonic for
pulmonary artery sarcoma. PLoS One. 2013;8(12):e83200.
D. G. Papamatheakis et al.
https://t.me/med1917
89
Grazioli V, Vistarini N, Morsolini M, et al. Surgical treatment of
primary pulmonary artery sarcoma. J Thorac Cardiovasc Surg.
2014;148:113–8.
Mussot S, Ghigna MR, Mercier O, etal. Retrospective institutional
study of 31 patients, treated for pulmonary artery sarcoma. Eur J
Cardiothorac Surg. 2013;43:787–93.
Papamatheakis DG, Kerr KM. Pulmonary vascular tumors. In:
Peacock AJ, Naeije R, Rubin LJ, editors. Pulmonary circulation.
Diseases and their treatment. Boca Raton, FL: CRC Press (Taylor
& Francis group; 2016. p.736–42.
von Falck C, Meyer B, Fegbeutel C, etal. Imaging features of primary
sarcomas of the great vessels in CT, MRI and PET/CT: a single
center experience. BMC Med Imaging. 2013;13:25.
Chapter 9. Case 9: Pulmonary Artery Sarcoma …
https://t.me/med1917
91© Springer Nature Switzerland AG 2020
W. R. Auger, D. Gopalan (eds.), Clinical Cases in Chronic
Thromboembolic Pulmonary Hypertension, Clinical Cases in
Cardiology, https://doi.org/10.1007/978-3-030-17366-1_10
Case Presentation
A 65-year-old Caucasian female presented for evaluation of
one and a half years of progressive shortness of breath. One
year prior to presentation she was diagnosed with a right
sided pulmonary embolism (PE) in the setting of an acute
increase in symptoms and was started on warfarin for antico-
agulation. She experienced a mild, temporary improvement
but within 3 months again experienced dyspnea with ambula-
tion. The dyspnea was associated with leg swelling but no
chest pain, syncope or pre-syncope.
Chapter 10
Case 10: Chronic
Thrombus andMetastatic
Cancer—An Unexpected
CTEPH Mimic
AlisonS.Witkin andWilliamR.Auger
A. S. Witkin (*)
Division of Pulmonary and Critical Care Medicine, Massachusetts
General Hospital, Harvard Medical School, Boston, MA, USA
e-mail: aswitkin@mgh.harvard.edu
W. R. Auger
Lewis Katz School of Medicine, Temple University,
Philadelphia, PA, USA
e-mail: Bill.Auger@tuhs.temple.edu
https://t.me/med1917
92
In addition to the above, past medical history was notable
for a history of mild asthma and osteoporosis. She was up to
date on her age appropriate cancer screening. She was a for-
mer smoker, with a 55 pack-year history and quit 5 years
prior to presentation. There was no family history of venous
thromboembolism.
On exam, she was afebrile. Blood pressure was
137/75mmHg, pulse was 96 beats per minute, respiratory rate
was 18 and her oxygen saturation was 92% on room air. She
was well-appearing and able to speak in full sentences with-
out accessory muscle use at rest but became dyspneic with
one flight of stairs. Heart rate and rhythm were regular with
a 2/6 systolic murmur. Her lungs were clear and a pulmonary
artery bruit was audible over the left upper posterior lung
field. There was no palpable cervical, supraclavicular, or axil-
lary lymphadenopathy. She had no palpable hepatospleno-
megaly and her extremities were warm with trace edema.
A CT scan of the chest was available for review and
showed significant thickening of the blood vessel wall of the
left pulmonary artery, and occlusive thrombus in the right
interlobar pulmonary artery (Fig.10.1).
F . Computed tomography of the chest demonstrating
thickening of the wall of the left pulmonary artery (blue arrow),
occlusive thrombus in the proximal right PA (solid white arrow) and
vessel attenuation of the right lower lobe arteries (open white
arrow)
A. S. Witkin and W. R. Auger
https://t.me/med1917
93
Pulmonary function testing revealed an FEV1 of 81%
predicted, and FVC of 94% predicted with an FEV1/FVC of
62%. The DLCO was decreased at 45% predicted. A TTE
showed a left ventricular ejection fraction of 60–65%. The right
ventricle was dilated with right ventricular hypertrophy and a
moderate decrease in systolic function. The estimated pulmo-
nary artery systolic pressure was 75–80mmHg. A recent right
heart catheterization demonstrated pulmonary hypertension.
The right atrial pressure was 27mmHg, the pulmonary artery
pressure was 91/23(46) mmHg and the pulmonary capillary
wedge pressure was 10 mmHg. The cardiac output by
thermodilution was 4.2L/min and the pulmonary vascular resis-
tance was 8.5Woods units. A ventilation perfusion scan showed
multiple mismatched perfusion abnormalities (Fig.10.2).
The patient was diagnosed with chronic thromboembolic
pulmonary hypertension and admitted to the hospital for
further management.
The patient was admitted to the hospital and treated with
intravenous diuretics. Supplemental oxygen was used to main-
tain an oxygen saturation >95%. On day 4 of hospitalization
she underwent pulmonary thromboendarterectomy (Fig.10.3).
F . The ventilation-perfusion scan. The ventilation images
were normal. The perfusion scan demonstrated multiple defects
consistent with acute or chronic pulmonary embolism
Chapter 10. Case 10: Chronic Thrombus andMetastatic…
https://t.me/med1917
94
She had an excellent hemodynamic result with improve-
ment in her systolic pulmonary artery pressure from 85mmHg
at the start of the case to 45 mmHg at the completion. The
post-operative course was uneventful. She was extubated and
weaned of vasopressors on the first post-operative day (POD).
On POD 5, the surgical specimen pathology resulted with
islands of malignant cells within the endarterectomy speci-
men (Fig.10.4a). These cells stained positive for keratin and
p-40 and were negative for CD34, consistent with squamous
cell carcinoma (Fig.10.4b).
A PET-CT was done which showed intense uptake in the
region of the right pulmonary artery and paratracheal
lymph nodes as well as in the left eighth rib and right iliac
crest. No primary source was identified and the patient was
diagnosed with metastatic squamous cell carcinoma of
known primary.
The patient had an uneventful recovery from her surgery
with significant improvement in her functional capacity. After
3months of recovery, she was started on chemotherapy with
an excellent radiographic response to treatment.
F . The endarterectomy specimen from the patient
A. S. Witkin and W. R. Auger
https://t.me/med1917
95
a
b
F . (a) Islands of malignancy cells (arrow) within endarter-
ectomy specimen. (b) The malignant cells stain strongly positive for
keratin. A p-40 stain was positive as well (not shown)
Chapter 10. Case 10: Chronic Thrombus andMetastatic…
https://t.me/med1917
96
Clinical Comments
Pulmonary tumor emboli can present in a manner similar to
thromboembolic disease, with some of the more common pri-
mary tissue types include breast, stomach, lung, liver, prostate and
pancreas. Tumor cell emboli in the pulmonary vascular bed can
involve the proximal vessels and present acutely, or can primarily
involve the microvasculature with a more sub-acute, progressive
clinical course. This case is of interest in that the presentation was
entirely consistent with CTEPH, and the tumor cells were found
embedded in the organized thrombus removed at the time of
surgery—emphasizing the importance of a pathologic examina-
tion of endarterectomized material. Unclear is the mechanism by
which the tumor cells became incorporated within the clot—
whether there was an initial tumor embolus leading to an in-situ
thrombus, or the pulmonary thromboembolism was somehow
“seeded” by circulating tumor cells.
Points ofEmphasis
Metastatic neoplasm involving the pulmonary vessels can
present in various ways, this case illustrating an unusual
involvement with organized thrombus. It is essential that
material endarterectomized from the pulmonary vessels
undergo complete examination to ensure that coexisting
pathology is not present.
Diagnosis
1. Chronic thromboembolic pulmonary hypertension.
2. Metastatic squamous cell carcinoma, unknown origin.
A. S. Witkin and W. R. Auger
https://t.me/med1917
Соседние файлы в папке Библиотека им академика М.И. Перельмана