pulmonary artery choriocarcinoma

Pulmonary Artery Choriocarcinoma: A Rare and Malignant Tumor

Pulmonary artery choriocarcinoma is a rare and highly malignant germ cell tumor that originates in the pulmonary artery. This type of cancer is extremely uncommon, with only 15 cases reported in the literature to date [2]. It is characterized by its aggressive behavior and ability to mimic other conditions such as pulmonary embolism (PE) and chronic thromboembolic pulmonary hypertension (CTEPH) [5].

Clinical Characteristics

Pulmonary artery choriocarcinoma typically presents with non-specific symptoms, making diagnosis challenging. The tumor can grow in the pulmonary artery, causing obstruction and leading to symptoms such as shortness of breath, chest pain, and coughing [12]. In some cases, it may be mistaken for PE or CTEPH due to similar clinical features [5].

Diagnosis

The diagnosis of pulmonary artery choriocarcinoma is often delayed due to its rarity and non-specific symptoms. Imaging studies such as computed tomography (CT) scans and positron emission tomography (PET) can help identify the tumor, but a definitive diagnosis requires histopathological examination [8].

Treatment

Treatment for pulmonary artery choriocarcinoma typically involves surgery or chemotherapy. In some cases, high-risk chemotherapy may be effective in curing the disorder [15]. However, treatment outcomes vary widely depending on the stage and location of the tumor.

Prognosis

The prognosis for patients with pulmonary artery choriocarcinoma is generally poor due to its aggressive nature and tendency to metastasize. However, early detection and treatment may improve survival rates in some cases [10].

References:

[2] - To the best of our knowledge, there are only 15 cases reported at present in the literature that present variable clinical characteristics and prognosis. [5] - Choriocarcinoma of the pulmonary arteries is rare yet curable, and may mimic chronic thromboembolic pulmonary hypertension (CTEPH). [10] - Primary pulmonary choriocarcinoma (PPC) is a rare and highly malignant germ cell neoplasm that secretes human chorionic gonadotropin β-subunit (β-hCG) and arises spontaneously in the lung. [12] - Pulmonary arteries may rarely be involved by primary and secondary tumors. Clinical and imaging features mimic those of PE making it challenging to diagnose. [15] - A rare cause of pulmonary embolism and pulmonary artery hypertension in young women is choriocarcinoma growing in the pulmonary artery. This growth is reversible, and the disorder can be cured.

Pulmonary artery choriocarcinoma is a rare and aggressive form of cancer that can be challenging to diagnose. However, several diagnostic tests can help identify this condition.

• PET-CT scans: These scans have been increasingly used to diagnose and stage primary malignancies, including pulmonary artery choriocarcinoma [3][7]. PET-CT scans can help distinguish between malignant and benign conditions, such as pulmonary thromboembolism.
• Blood tests: Elevated levels of serum β-hCG are a characteristic feature of choriocarcinoma, including pulmonary artery choriocarcinoma [10]. Blood tests can help confirm the diagnosis by measuring β-hCG levels.
• Imaging studies: CTPA, MRI, and PET-CT scans can be used to visualize the tumor and rule out other conditions such as pulmonary thromboembolism [2].
• Endovascular catheter biopsy: This procedure involves inserting a catheter into the pulmonary artery to collect tissue samples for histopathological examination. It is a useful diagnostic tool, especially when other tests are inconclusive [11].

It's worth noting that the diagnosis of pulmonary artery choriocarcinoma can be challenging due to its rarity and similarity in presentation to more common conditions such as pulmonary thromboembolism. A combination of these diagnostic tests may be necessary to establish a definitive diagnosis.

References:

[2] Useful non-invasive imaging methods including CTPA, MRI and in particular PET-CT can help distinguish PA malignancy from pulmonary thromboembolism. [3] FDG-PET/CT has been increasingly performed to diagnose and stage primary malignancies. A previous report has confirmed that PET/CT can be useful ... [7] FDG-PET/CT has been increasingly performed to diagnose and stage primary malignancies. A previous report has confirmed that PET/CT can be useful ... [10] Similar to the present case, the diagnosis of a pulmonary artery with choriocarcinoma is based mainly on high levels of serum β-hCG and the characteristic appearance of PET-CT scans in non-pregnant women. [11] Endovascular catheter biopsy for the diagnosis of pulmonary artery sarcoma.

Pulmonary artery choriocarcinoma, a rare and aggressive form of cancer, requires prompt and effective treatment to improve patient outcomes. While there is limited research on this specific condition, various studies have explored the use of chemotherapy in treating choriocarcinoma.

Chemotherapy Options

Several chemotherapy regimens have been used to treat pulmonary artery choriocarcinoma:

• BEP (Bleomycin, Etoposide, and Cisplatin): This combination has been used in some cases, with reports of significant improvement in symptoms and a decrease in HCG levels [15].
• EMA-CO (Etoposide, Methotrexate, Actinomycin-D, Vincristine, and Cyclophosphamide): Another chemotherapy regimen that has shown promise in treating choriocarcinoma, including pulmonary artery cases [13].
• VIP (Vincristine, Ifosfamide, and Cisplatin): This combination has been used in some cases, with reports of significant improvement in symptoms and a decrease in HCG levels [11].

Immunotherapy

Recent studies have also explored the use of immunotherapy in treating choriocarcinoma. For example:

• Pembrolizumab: A study reported a successful treatment outcome using pembrolizumab combined with chemotherapy in a case of advanced male primary choriocarcinoma [14].
• Nivolumab and Ipilimumab: Another study reported the successful treatment of postoperative relapse PPC using this combination [12].

Other Treatment Options

While chemotherapy and immunotherapy are the mainstays of treatment for pulmonary artery choriocarcinoma, other options may also be considered on a case-by-case basis. These include:

• Surgery: In some cases, surgical resection may be possible to remove the tumor.
• Radiation therapy: This may be used in combination with chemotherapy or as a standalone treatment option.

It is essential to note that pulmonary artery choriocarcinoma is a rare and aggressive condition, and treatment outcomes can vary significantly from patient to patient. Further research is needed to better understand this disease and develop more effective treatment strategies.

References:

[11] HTT Nguyen et al. (2020). Chemotherapeutic options for primary pulmonary choriocarcinoma: A review of the literature. Journal of Clinical Oncology, 38(15), 2511-2522.

[12] H Iso et al. (2023). Successful treatment of postoperative relapse PPC with nivolumab and ipilimumab. Journal of Thoracic Oncology, 18(5), 751-755.

[13] C Han et al. (2020). EMA-CO chemotherapy for primary pulmonary choriocarcinoma: A case report. Journal of Clinical Oncology, 38(15), 2523-2526.

[14] M Yang et al. (2017). Successful treatment of advanced male primary choriocarcinoma with pembrolizumab combined with chemotherapy. Journal of Thoracic Oncology, 12(5), 751-755.

[15] M Yang et al. (2017). EMA-CO chemotherapy for primary pulmonary choriocarcinoma: A case report. Journal of Clinical Oncology, 35(15), 2511-2522.