Primary pulmonary angiosarcoma presenting as Pancoast tumor

Sayan, Muhammet; Valiyev, Elgun; Akarsu, Irmak; Esendağlı, Güldal; Korpeoglu, Tugba; Çelik, Ali

doi:10.1177/0218492320988456

Cited by 2 publications

(5 citation statements)

References 8 publications

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“…Primary pulmonary angiosarcoma (PPA) is a rare tumor arising from arterial or venous pulmonary vessels of various sizes ( 9 ), and only ~30 cases of PPA have been reported to date ( 5 , 7 ). PPAs are more common in males and the majority of patients are aged 40 years or above ( 7 , 10 ).…”

Section: Discussionmentioning

confidence: 99%

“…Angiosarcoma originates from blood vessels or lymphatic vessels; thus, abnormal activation of vascular endothelial growth factor (VEGF) and its receptor VEGFR may result in angiosarcoma ( 6 ). According to previous reports, the risk factors for PPA are radiotherapy, thorium dioxide, poly-vinyl chloride, radon, thorotrast, copper mining dust, mastectomy, chronic empyema and tuberculosis ( 3 , 8 , 10-12 ). The patient of the present study received iodine 131 therapy.…”

Section: Discussionmentioning

confidence: 99%

“…PPA is associated with symptoms such as cough, hemoptysis, chest pain, shortness of breath, pneumothorax, spontaneous hemothorax, cyanosis, syncope or rarely massive pulmonary hemorrhage and weight loss ( 6-8 , 10 , 11 ). In addition, most patients present with cough and hemoptysis as the initial symptoms ( 6 ).…”

Section: Discussionmentioning

confidence: 99%

“…Angiosarcoma is a rare disease, and thus, there is currently no standard treatment plan, particularly not for PPA. Patients with pulmonary angiosarcoma have been treated with surgical resection ( 5 , 11 , 17 , 22 ), adjuvant radiotherapy ( 8 , 20 , 22 ), chemotherapy ( 20 , 26 ), immunotherapy ( 27 , 28 ) and combined therapy ( 10 , 16 , 20 ). It has been proposed that surgery was the most effective treatment, which should be considered as early as possible for resectable tumors ( 5 , 17 ).…”

Section: Discussionmentioning

confidence: 99%

“…Wilson et al ( 26 ) presented a case of PPA with complete radiographic response to gemcitabine and docetaxel, with a sustained complete response for over 12 months and markedly prolonged survival of 39 months. The other effective chemotherapy regimens include doxorubicin/ifosfamide/mesna ( 10 ), adriamycin/ifosfamide ( 29 ), cyclophosphamide/gemcitabine/docetaxel ( 30 ) and docetaxel/cisplatin ( 16 ). Previous studies reported that immunotherapy is effective against angiosarcoma ( 31 , 32 ).…”

Section: Discussionmentioning

confidence: 99%

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Primary pulmonary epithelioid angiosarcoma with thyroid tumor history: A case report and literature review

et al. 2022

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Primary pulmonary epithelioid angiosarcoma is a rare tumor type without any specific clinical and imaging features. Therefore, it is associated with high rates of misdiagnosis. The present study reports the case of a 54-year-old female patient who was admitted after complaining of cough, expectoration and bloody sputum for >5 months in May 2021. The patient reported a previous history of papillary thyroid carcinoma in 2003 and had undergone treatment through surgery, postoperative chemotherapy and iodine 131 therapy. Chest computed tomography (CT) was performed in May 2021, which indicated that the disease had progressed rapidly since February 2021. CT-guided lung biopsy and immunohistochemical staining of the tumor indicated positivity for CD31, CD34 and E26 transformation-specific-related gene markers. The tumor was negative for thyroid cancer-associated antibodies; thus, a diagnosis of primary pulmonary epithelioid angiosarcoma was made. The patient died 3 months after the diagnosis. Primary pulmonary epithelioid angiosarcoma is a rare tumor type with high recurrence and metastasis rates. This tumor has no specific clinical symptoms and signs and is thus easily misdiagnosed. Biopsy is essential for diagnosis of the disease, particularly if patients have a tumor history.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

Primary pulmonary epithelioid angiosarcoma with thyroid tumor history: A case report and literature review

et al. 2022

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Primary Pulmonary Angiosarcoma Found Incidentally in a Complicated Patient: A Rare Case Report

Basiri,

Ziaei Moghaddam,

Rikhtegar

et al. 2024

Clinical Respiratory J

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IntroductionPrimary pulmonary angiosarcoma (PPA) is a highly aggressive and rare malignancy originating from the endothelial cells of blood vessels in the lungs. PPA is an extremely rare subtype, with less than 30 cases reported to date. PPA is not only challenging to diagnose but also has a poor prognosis, often resulting in a high mortality rate within a year of diagnosis, regardless of the treatment approach.MethodWe present the case of a 33‐year‐old woman with no significant past medical history who presented with abdominal pain and was incidentally found to have a right hilar mass with pleural effusion and empyema. After undergoing surgery for a perforated gastric ulcer, her pulmonary lesions were further worked up. Despite an extensive diagnostic evaluation, including imaging, bronchoscopy, and thoracotomy, establishing a diagnosis was challenging. Ultimately, PPA was diagnosed on surgical lung biopsy, and the patient was started on pazopanib and paclitaxel chemotherapy but expired after 1 month due to multiple complications.ConclusionThis case highlights the difficulty in diagnosing this rare tumor and its poor prognosis regardless of therapy. Greater awareness of PPA and more research are needed to improve early detection and treatment options for this deadly disease.

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Primary pulmonary angiosarcoma presenting as Pancoast tumor

Cited by 2 publications

References 8 publications

Primary pulmonary epithelioid angiosarcoma with thyroid tumor history: A case report and literature review

Primary pulmonary epithelioid angiosarcoma with thyroid tumor history: A case report and literature review

Primary Pulmonary Angiosarcoma Found Incidentally in a Complicated Patient: A Rare Case Report

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