Pulmonary artery intimal sarcoma mimicking pulmonary thromboembolism

Abstract

Rationale: Pulmonary artery intimal sarcoma is a rare tumor with exceptionally high mortality and easily misdiagnosed as pulmonary thromboembolism pulmonary thromboembolism (PTE) due to the nonspecific clinical presentation and symptom. Misdiagnosis or untimely diagnosis makes the disease progress to an advanced stage and eventually leads to a poor prognosis. Patient concerns: A 37-year-old Chinese female presented with chest tightness and dyspnea for 3 months. Echocardiography and chest computed tomography revealed an intraluminal obstruction of the pulmonary arteries. Tests of serum tumor makers showed slight elevation for carbohydrate antigen-125, and α-fetoprotein. PTE was suspected according to the radiological and laboratory findings. Diagnosis: Microscopic findings of the presumed thrombus showed prominent myxoid and edematous background with atypical spindled cells and curvilinear vascularity. Immunohistochemical staining demonstrated that the atypical spindled cells were positive for vimentin but negative for CK, S100, SMA, desmin, CD68, STAT6, CD34, β-catenin, ALK-p80, p53, and MDM2. According to the radiological and pathological findings, the diagnosis of fibrosarcoma of pulmonary artery was made. Interventions: The patient underwent surgical resection and the mass was excised as completely as possible. Outcome: Follow-up information showed no evidence of recurrence or metastasis after 3 months postresection. Lessons: Because of the low incidence rate, nonspecific clinical symptoms, and radiological findings, primary fibrosarcoma of the pulmonary artery is commonly misdiagnosed as PTE. Pathological examination is necessary to confirm the diagnosis.

Not always embolism: A case of pulmonary artery intimal sarcoma – The role of the radiologist in early diagnosis

“…Clinical presentation depends on the disease stage and, therefore, on the degree of involvement of the pulmonary vessels and the possible distant progression of the disease: usually the symptoms and signs of right ventricular failure are the early features [ 4 ] that may lead to a difficult differential diagnosis with non-neoplastic conditions, such as chronic pulmonary embolism (CPE). PAIS and CPE demonstrate a strong overlap of the radiological findings; hence, diagnosis is complicated and often delayed [ [1] , [2] , [3] , [4] , [5] , [6] ]. The aim of this study is to illustrate contrast-enhanced computed tomography (CECT)-typical findings of PAIS, showing a case followed at our center, to help radiologists recognize the elements of differential diagnosis with CPE ( Table 1 ).…”

Pulmonary thromboembolic disease or pulmonary artery intimal sarcoma: Case report and literature review

“…It has been indicated that the manifestation of chronic right heart failure is frequently derived from PAIS-associated long-term occupation of the lumen in the pulmonary artery. The most common symptoms are dyspnea, cough, chest tightness and hemoptysis ( 10 ). The features associated with malignant tumors, such as fever, fatigue and weight loss, may also be present.…”

“…The potential of treatment improvement by adjuvant chemotherapy and radiotherapy remains controversial. Currently, no consensus exists on the effect of surgery combined with radiotherapy and chemotherapy on the overall survival rate of patients ( 10 ). Chemotherapy may be an option for patients with unresectable or recurring focal sarcoma ( 21 ).…”

“…transferring possibility Approximately 20% of cases demonstrate additional thoracic malignant metastases, involving lungs, kidneys, brain, lymph nodes and skin (9). symptoms and signs the classic symptoms include dyspnea, cough, chest tightness, hemoptysis, fever, fatigue and weight loss (10). Microscopy image the tumor cells mainly include spindle cells, whereas others are epithelial, and giant and multinucleated cells present in focal points (2).…”

“…treatment surgical resection is generally considered to be the best treatment option. Whether adjuvant chemotherapy and radiotherapy improve the treatment response remains controversial (10,20).…”