Treatment options for a rare medical condition
A 41 year old female was diagnosed with a rare condition – a malignant tumor of the heart. Confused as to how to proceed with treatment of her condition, she received recommendations for medication and further tests from a Medical-Opinion online medical specialist.
The patient is a 41 year-old female diagnosed with cardiac angiosarcoma. She has no significant medical history, except for a slightly enlarged heart (cardiomegaly) since birth, periodically controlled and without pathological matching.
The patient had been experiencing pain in the pericardial region which was investigated but with no definite diagnosis. The patient scheduled an appointment at an outpatient hospital for further investigation and medical treatment.
In the meantime, the patient was hospitalized for suspected pericarditis, subsequent to the appearance of fever.
The performed CAT scan confirmed a neoformation affecting the right atrium. She then underwent a cardiac biopsy which diagnosed a cardiac angiosarcoma.
The patient was subsequently subject to an operation for the removal of the right endoatrial mass and for the reconstruction of the wall with autologous pericardium.
The histological slides were sent to a cancer institute, which confirmed the diagnosis of an Angiosarcoma of the epithelioid type.
The Pet, the bone scintigraphy and the MRI imaging performed afterwards have provided contrasting results with regard to eventual bone and hepatic metastasis.
Online Doctor Consultation – Medical Questions: The patient has asked for an online doctor consultation regarding an eventual treatment and/or further examinations to be performed.
Expert Report and Opinion
Summary: 41 year old woman in good health with pains in the left chest. Hospitalized with fever and the diagnosis of pericarditis. CT chest abdomen pelvis showed an abnormality in the right atrium. Transthorasic ECHO showed a right atrial mass measuring 4.4×3.4 cm and contiguous to the side wall. She had a biopsy of the atrial mass by catheterization, and the pathology revealed angiosarcoma. She then had resection of the right endoatrail mass with reconstruction with autologous pericardium, and she did well post op. The pathology was confirmed as Angiosarcoma, epithelial type at a cancer institute. PET CT showed increased uptake in the residual right side of the heart (which could reflect the recent surgery or be residual sarcoma), with increased uptake in D4, and in the pelvis near the right acetabulum thought to represent bone involvement, and a circumscribed area of increased activity in the rear margin of the second hepatic segment. The CT of the chest abdomen and pelvis had no abnormalities aside from the mass in the atium. I did not see a report of a bone scan or of an MRI of the spine in the records, or images on the accompanying CD. The margins were not noted on the path report.
Angiosarcoma arising in the right atrium, grossly resected, which by morphologic description appears to be high grade. Staging showed possible metastatic involvement by PET in D4 and the right acetabulum area, and possibly one small focus in the liver(with the CT c/a/p not revealing any obvious mets), with uptake in the right side of the heart (likely physiologic post-op).
Sarcoma of the heart including angiosarcoma of the heart is a very rare tumor. Gross resection may be possible but there is a high likelihood of distant dissemination hematogenously. There is no standard treatment approach because of the small number of cases. Our approach has been to treat adjuvantly with chemotherapy in patients with no obvious distant disease. We have used Adriamycin and Ifosphamide for 6 cycles if the ejection fraction is adequate. We have not used radiation to the heart. If the ejection fraction is poor, then the choice of drugs changes to Ifosphamide and VP-16. Angiosarcomas may have a good response to Taxol and treating with Taxol is reasonable. We have used Taxol given weekly to treat angiosarcomas which are metastatic with good success, but have not had a cardiac sarcoma that was of the angiosarcoma subtype.
If the patient has widespread disease then the prognosis is very grave, and the treatment would be palliative but would include the same drugs: Taxol; adriamycin and Ifosphamide; Ifosphamide and VP-16. Other possibilities which may have some activity are Gemzar with Taxotere; and ET-743 which is still experimental in the US, but is available on a compassionate basis. If not done, a bone scan and an MRI of the dorsal spine and the pelvis may help to determine if the patient has distant disease, but this could still remain equivocal. Treatment for 3 cycles followed by restaging may be helpful to determine the extent of disease.