Primary Pulmonary Epithelioid Angiosarcoma: A Rare Case Presentation of Bilateral Pneumothoraces

Faiek, Saif; Tariq, Hafsa; Upparapalli, Deepti; Bansal, Aditya; Sompalli, Sreeja

doi:10.7759/cureus.6514

Cited by 4 publications

(4 citation statements)

References 7 publications

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“…MPPA is a high grade malignancy with only a few reported cases 1 . It represents a diagnostic challenge for pathologists and requires a meticulous cytological examination and a specific immunohistochemical panel to perform a correct diagnosis.…”

Section: Discussionmentioning

confidence: 99%

Proceedings of Annual Congress Siapec Iap 2021

Wang¹

2021

Pathologica

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Europa Donna Italia, tramite la sua rete territoriale, monitora costantemente la corretta applicazione delle linee di indirizzo nazionali per le Breast Unit 1 che prevedono la presenza di un anatomo-patologo specializzato all’interno dell’equipe multidisciplinare. Il problema del mancato accesso delle pazienti italiane ai test genomici nel percorso di cura del tumore al seno è stata l’occasione per una più intensa collaborazione tra Europa Donna Italia e SIAPEC, che insieme hanno promosso l’informazione al pubblico attraverso aggiornamenti scientifici sul sito e sui social del Movimento. Europa Donna Italia e SIAPEC hanno inoltre realizzato incontri di formazione 2 per le associazioni di pazienti sulla diagnosi personalizzata e sulla predittività e la prognostica dei test genomici nell’ambito del percorso formativo “Academy”. Sono state svolte anche campagne di informazione e sensibilizzazione sul diritto all’equità diagnostica e una raccolta di firme virtuali per sollecitare il Ministero della Salute ad emanare il decreto attuativo necessario a sbloccare il fondo nazionale destinato alle Regioni dalla legge di bilancio 2021. La campagna “Chemio: se posso la evito”, da gennaio a marzo 2021, ha raccolto 15.158 firme mentre da agosto a ottobre 2021 è stato svolto un monitoraggio nazionale per informare le donne sulla disponibilità dei test nelle varie Regioni a seguito dell’emanazione delle relative delibere. È nell’interesse delle pazienti che Europa Donna Italia intende proseguire l’attività di advocacy in collaborazione con SIAPEC affinché sia assicurata un’adeguata qualità di diagnosi dei tumori al seno e a ogni paziente sia garantito il diritto di consultare il patologo della propria Breast Unit.

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Section: Discussionmentioning

confidence: 99%

Proceedings of Annual Congress Siapec Iap 2021

Wang¹

2021

Pathologica

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“…Pulmonary metastasis from cutaneous and cardiac angiosarcomas occurs in 60%-80% of cases [ 8 ]. Primary pleural angiosarcomas have been shown to present in most segments of the pleura with invasion commonly to the lung parenchyma, blood vessels, pericardium, diaphragm, and bronchial wall [ 2 , 4 , 9 ]. Additionally, the neoplasm has shown the potential to significantly progress.…”

Section: Discussionmentioning

confidence: 99%

“…Angiosarcomas represent only 1% of all soft tissue malignancies [ 1 , 2 ]. Tumor arises from the endothelial cells of blood vessels.…”

Section: Introductionmentioning

confidence: 99%

Primary Pleural Epithelioid Angiosarcoma With Extensive and Rapid Metastasis to Brain and Bilateral Adrenal Glands

Sinkler

Ullah

Wehrle

et al. 2020

Cureus

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A 64-year-old male presents with shoulder pain, arm pain, and a chronic cough. CT imaging of the thorax shows a large 8.0 x 6.7 cm mass with central necrosis in the left upper lung lobe with invasion into the chest wall with partial destruction of the second and third ribs, and left axillary adenopathy. Bilateral adrenal nodules are identified via CT imaging and subsequently biopsied. Histologically, the mass reveals sheets of atypical epithelioid cells with round nuclei and abundant eosinophilic cytoplasm. Immunostaining is positive for CD31, CD34, FLI-1, AE1/AE3, and CK7, diagnostic of primary epithelioid angiosarcoma. The patient developed symptoms of confusion, dizziness, and ataxia. An MRI showed metastatic brain lesions. One month later, the patient had worsening symptoms. Repeat imaging demonstrates enlargement of the bilateral adrenal masses, a new lesion posterior to the left kidney, and doubling of the size of the brain lesions. This case illustrates the metastatic potential and pattern of the spread of an aggressive primary pleural angiosarcoma that is not described elsewhere in current literature. It also highlights the importance of timely intervention based on the rapid metastatic progression of this neoplasm.

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“…AS is a highly malignant soft tissue sarcoma originated from vascular endothelial cells, accounting for less than 1% of soft tissue sarcoma [1]. It often occurs in the skin, subcutaneous tissue of the head and face [9],breast, but also in the mouth [10], mediastinum [11], kidney [12], thyroid [13], lung and other anatomical sites [14]. AS in digestive system is rare, but in solid organs such as liver and spleen is relatively common [15].…”

Section: Discussionmentioning

confidence: 99%

Primary small intestinal epithelioid angiosarcoma: a potential diagnostic pitfall due to abnormal expression of cytokeratin

yuanyuan

Fang

Zhang

et al. 2020

Preprint

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Background: Primary small intestinal epithelioid angiosarcoma (EAS) is a very rare tumor, which originates from endothelial cell with high mortality. Only 66 cases of small intestinal angiosarcoma including primary or metastatic were reported in the current literature. Histologically, hemangiosarcoma with epithelioid cell morphology is more rare, so we report a case of EAS which is easily misdiagnosed as poorly differentiated carcinoma. We also reviewed the literature about small intestinal angiosarcoma.Case presentation: The patient was a 60-year-old man, who initially presented black stool with no obvious inducement for 7 days, gradually feeling dizzy and fatigue for 3 days. Abdominal CT showed that the intestinal wall of the upper jejunum was thickened near the duodenum. After enhancement, the intestinal wall could be enhanced evenly. With the time going on, the contrast media could be concentrated gradually, and the active hemorrhage was considered. Enteroscopy revealed a 30 mm × 20 mm red polypoid mass in the jejunum. A biopsy of polypoid mass exhibited malignant tumor, histopathology considered poorly differentiated cancer. The patient subsequently underwent resection of the jejunal neoplasm. Immunohistochemistry vascular markers and cytokeratin were postive，but C-MYC protein was negative, and showed no amplification of C-MYC gene by Fluorescence in situ hybridization （FISH）. The histopathology with immunohistochemical and FISH studies confirmed the diagnosis of primary small intestinal EAS. 24 months after surgery, the patient had no evidence of recurrence and metastasis.Conclusion: Primary small intestinal EAS is a rare malignancy of endothelial origin with uncertain etiology and often has a poor prognosis.The abnormal cytokeratin expression in epithelioid angiosarcomas represents a potential diagnostic pitfall for pathologists. FISH detection of C-MYC gene amplification may have some value in the diagnosis of primary or metastatic small intestinal AS.An accurate pathological diagnosis relies on histopathology with immunohistochemical stains of endothelial markers. Surgical resection remains the best treatment option for small intestinal angiosarcoma.

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Primary Pulmonary Epithelioid Angiosarcoma: A Rare Case Presentation of Bilateral Pneumothoraces

Cited by 4 publications

References 7 publications

Proceedings of Annual Congress Siapec Iap 2021

Proceedings of Annual Congress Siapec Iap 2021

Primary Pleural Epithelioid Angiosarcoma With Extensive and Rapid Metastasis to Brain and Bilateral Adrenal Glands

Primary small intestinal epithelioid angiosarcoma: a potential diagnostic pitfall due to abnormal expression of cytokeratin

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