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Pulmonary Artery Sarcoma: A Rare Entity.

Shomaf M, Obeidat N, Al-Fares F, Najjar S - J Investig Med High Impact Case Rep (2014)

Bottom Line: The patient was first diagnosed with pulmonary embolism but she did not improve on anticoagulant therapy.The tru-cut biopsy revealed an undifferentiated sarcoma.The patient died 10 months after her initial presentation.

View Article: PubMed Central - PubMed

Affiliation: The University of Jordan, Department of Pathology.

ABSTRACT
Pulmonary artery sarcomas (PAS) are extremely rare sarcomas of uncertain histogenesis that often mimic pulmonary thromboemboli. This is a report of a 60-year-old female patient who presented with recurrent chest pain and cough. The patient was first diagnosed with pulmonary embolism but she did not improve on anticoagulant therapy. Follow-up imaging studies revealed a mass in the left hilar region extending into the pulmonary trunk and branches of the left pulmonary artery. The tru-cut biopsy revealed an undifferentiated sarcoma. The patient died 10 months after her initial presentation.

No MeSH data available.


Related in: MedlinePlus

Hematoxylin–eosin stain showing a poorly differentiated sarcoma with marked cellularpleomorphism, 200× magnification.
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fig4-2324709614529416: Hematoxylin–eosin stain showing a poorly differentiated sarcoma with marked cellularpleomorphism, 200× magnification.

Mentions: A bilateral lower limb Doppler ultrasound proved to be negative for thrombosis.Thrombophilia workup revealed a homozygous mutation of A1298C MTHFR by polymerase chainreaction. The patient received anticoagulant therapy but showed no improvement. Four monthslater a chest (spiral) CT scan with contrast was done and showed a large lobulated hypodensemass in the left hilar and perihilar region extending into the pulmonary trunk, bothpulmonary arteries, and branches of the left pulmonary artery (Figure 2). On this admission the echocardiogramrevealed a diastolic dysfunction, pulmonary valve regurge, and pulmonary hypertension withpulmonary artery pressure of 42 mm Hg. A bronchoalveolar lavage was performed with anendobronchial biopsy, which was reported as superficial respiratory epithelium. On herfollow-up visit, a chest CT scan showed a large mass involving the left lung with extensionto the pulmonary trunk and mediastinal shift to the right, hilar lymph nodes enlargement,and moderate left pleural effusion were present (Figure 3). A CT-guided tru-cut biopsy was performed andthe histopathology revealed a poorly differentiated sarcoma with extensive necrosis (Figure 4). The tumor cells show briskmitotic activity including abnormal forms. Immunohistochemical stains were done and showedpositivity for vimentin (Figure 5),but CK, LCA, CD31, CD34, actin, caldesmon, desmin, and β-catenin stains were negative (Figure 6 and 7). The diagnosis of undifferentiated pulmonary arteryintimal sarcoma was made based on these findings.


Pulmonary Artery Sarcoma: A Rare Entity.

Shomaf M, Obeidat N, Al-Fares F, Najjar S - J Investig Med High Impact Case Rep (2014)

Hematoxylin–eosin stain showing a poorly differentiated sarcoma with marked cellularpleomorphism, 200× magnification.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License 1 - License 2 - License 3
Show All Figures
getmorefigures.php?uid=PMC4528857&req=5

fig4-2324709614529416: Hematoxylin–eosin stain showing a poorly differentiated sarcoma with marked cellularpleomorphism, 200× magnification.
Mentions: A bilateral lower limb Doppler ultrasound proved to be negative for thrombosis.Thrombophilia workup revealed a homozygous mutation of A1298C MTHFR by polymerase chainreaction. The patient received anticoagulant therapy but showed no improvement. Four monthslater a chest (spiral) CT scan with contrast was done and showed a large lobulated hypodensemass in the left hilar and perihilar region extending into the pulmonary trunk, bothpulmonary arteries, and branches of the left pulmonary artery (Figure 2). On this admission the echocardiogramrevealed a diastolic dysfunction, pulmonary valve regurge, and pulmonary hypertension withpulmonary artery pressure of 42 mm Hg. A bronchoalveolar lavage was performed with anendobronchial biopsy, which was reported as superficial respiratory epithelium. On herfollow-up visit, a chest CT scan showed a large mass involving the left lung with extensionto the pulmonary trunk and mediastinal shift to the right, hilar lymph nodes enlargement,and moderate left pleural effusion were present (Figure 3). A CT-guided tru-cut biopsy was performed andthe histopathology revealed a poorly differentiated sarcoma with extensive necrosis (Figure 4). The tumor cells show briskmitotic activity including abnormal forms. Immunohistochemical stains were done and showedpositivity for vimentin (Figure 5),but CK, LCA, CD31, CD34, actin, caldesmon, desmin, and β-catenin stains were negative (Figure 6 and 7). The diagnosis of undifferentiated pulmonary arteryintimal sarcoma was made based on these findings.

Bottom Line: The patient was first diagnosed with pulmonary embolism but she did not improve on anticoagulant therapy.The tru-cut biopsy revealed an undifferentiated sarcoma.The patient died 10 months after her initial presentation.

View Article: PubMed Central - PubMed

Affiliation: The University of Jordan, Department of Pathology.

ABSTRACT
Pulmonary artery sarcomas (PAS) are extremely rare sarcomas of uncertain histogenesis that often mimic pulmonary thromboemboli. This is a report of a 60-year-old female patient who presented with recurrent chest pain and cough. The patient was first diagnosed with pulmonary embolism but she did not improve on anticoagulant therapy. Follow-up imaging studies revealed a mass in the left hilar region extending into the pulmonary trunk and branches of the left pulmonary artery. The tru-cut biopsy revealed an undifferentiated sarcoma. The patient died 10 months after her initial presentation.

No MeSH data available.


Related in: MedlinePlus