Primary Pericardial Sarcoma with Right Atrial Invasion and Multiple Bilateral Pulmonary Metastases in a Patient with Hereditary Nonpolyposis Colorectal Cancer

Our plain-English summary of Primary Pericardial Sarcoma with Right Atrial Invasion and Multiple Bilateral Pulmonary Metastases in a Patient with Hereditary Nonpolyposis Colorectal Cancer, by E. Wong, L.J. Oh, K. Nahar, et al (Case Reports in Oncological Medicine, 2016).

The study

• Authors: E. Wong, L.J. Oh, K. Nahar, A. Lee, S. Clarke
• Journal: Case Reports in Oncological Medicine
• Published: 2016;2016:6208029
• Cited by: 1 article, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Primary tumours originating from the pericardium are extremely rare. Previous studies have reported that these tumours account for only 6.7-12.8% of all mediastinal tumours with an overall prevalence of 0.001% to 0.007%. The majority of these tumours are benign lipomas or pericardial cysts. The most common pericardial malignancy is mesothelioma. Sarcomas are soft-tissue mesenchymal malignancies originating from various parts of the body but are extremely rare in this area. We report a case of a 52-year-old female who was diagnosed with a primary sarcoma with rhabdoid differentiation originating from the pericardium. The patient presented to her GP with a four-week history of progressive dyspnea and chest pain on exertion. Chest X-Ray demonstrated a prominent pericardial effusion and suspicious chest and pericardial lesions. Biopsies of the effusion and primary tumour identified on FDG/PET scans revealed the diagnosis of primary undifferentiated sarcoma. On thoracotomy, it was noted that the tumour had invaded the right atrium; therefore, pericardial window was aborted and a drain inserted instead. The patient was then started on chemotherapy; however, progression soon occurred and the patient died within 4 months, suggesting there is urgent need for efficacious treatments for sarcomatous lesions.

In plain English

This case report from Royal North Shore Hospital, Australia, described one patient. A 52-year-old woman had four weeks of worsening breathlessness on exertion and right-sided chest pain. She had been diagnosed with localised Hereditary Nonpolyposis Colorectal Cancer in 2005. The report stated she had no asbestos or smoking history. Scans showed a large mass in the front of the chest against the right side of the heart, fluid around the heart, and multiple spots in both lungs that were presumed to be metastases. A biopsy showed a malignant sarcomatoid tumour with some rhabdoid differentiation that could not be further classified. During surgery the tumour was found to have invaded the right atrium, so a drain was placed instead of the planned pericardial window. She received doxorubicin chemotherapy and reported improved breathing after the second cycle. Three months later the disease progressed, and she died two weeks after treatment stopped. The authors called this the first case they knew of linking such a sarcoma with this cancer history, though its relevance was uncertain. They concluded that new treatments are urgently needed.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: case report (single patient)
• Who was studied: One 52-year-old woman treated at Royal North Shore Hospital, St Leonards, NSW, Australia, with a past history of localised Hereditary Nonpolyposis Colorectal Cancer (2005)
• Exposure: No asbestos exposure: the report states the patient had no asbestos or smoking history. Her prior medical history was Hereditary Nonpolyposis Colorectal Cancer treated with colonic resection.
• Main results:
  • Biopsy showed a malignant sarcomatoid tumour expressing PAX8, myogenin, myoD1 and desmin, suggesting some skeletal or cardiac muscle differentiation
  • The tumour showed some rhabdoid differentiation but could not be further classified
  • FDG/PET scanning showed multiple FDG-avid pulmonary nodes in all lobes of both lungs, presumed to be metastases
  • At thoracotomy the tumour involved the pericardium and had invaded the right atrium, so a pericardial drain was inserted instead of a pericardial window
  • The patient received single-agent doxorubicin (75 mg/m2) with pegfilgrastim and reported improved breathing and quality of life after the second cycle
  • Three months later the disease progressed. Systemic therapy was stopped and the patient died two weeks later.
• The authors' conclusion: The authors concluded that, although initial chemotherapy gave some symptom benefit, the disease progressed quickly and the patient died within 4 months, suggesting an urgent need for new treatments for sarcomatous lesions.
• Limitations the authors state:
  • The authors stated that the relevance of the patient's history of Hereditary Nonpolyposis Colorectal Cancer to the sarcoma is uncertain

Topics

Medical terms in this study, explained in our Lexicon: Pericardial, Sarcoma, Bilateral, Colorectal Cancer, Pericardium, Mesothelioma, Rhabdoid, Dyspnea.

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