KEYWORDS
Carney cpmlex (CNC), Cardiac myxoma,
Recurrence
A 72-year-old woman had endured more than 5 months with chest tightness
and difficulty breathing. Her previous medical history was that she had
interventional occlusion surgery for atrial septal defects 8 years ago.
On physical examination we found that she had a full moon face and
central obesity signs, spotty skin pigmentation lesions,while urinary
17-hydroxycorticosteroid measurements were significantly elevated. Based
on various clinical findings and data, we diagnosed this subject as
Cushing’s syndrome.
Emergency bedside ultrasound showed right atrial tumor, which was
confirmed by coronary computed tomography angiography (CCTA). CCTA
showed uneven low-density clumps attached to the edge of the blocker,
diastolic phase protruding into the right chamber, and systolic phase
completely retracting into the right atrium (Panels A, white arrow,
Supplementary material online, Videos S1 and S2). Pulmonary artery
multiple filling defect, was attached to the wall, free and completely
blocked the lumen, embolization index 70% (Panels C, white arrow).
The patient underwent surgical resection of the right atrial and
pulmonary artery tumors, partial removal of atrial septal occluder and
atrial septal repair. To ensure complete removal of the tumor, we
resected with a portion of tumor-free atrial septum, followed by primary
reconstruction of the septum. During the operation, we found that the
right chamber was gray, jelly sample, brittle tissue, attached to the
atrial septum and the edge of the blocked umbrella. Histopathological
examination showed stellate mesenchymal cells in a myxoid stroma (Panels
B, Stellate-shaped myxoma cells).
For CCTA review before discharge, the right atrial surface of the atrial
septum had less low-density shadow and 5mm thickness, (Panels E, white
arrow, Videos S3), No abnormal density was observed in each cardiac
cavity. However, the CCTA was reviewed in 6 months after surgery, the
low density shadow significantly increased, the thickness was 11mm
(Panels F, white arrow, Videos S4). Pulmonary embolism improved, with an
embolization index of 50% (Panels G, white arrow). New nodules were
found in both lungs, short diameter of 3mm-20mm(Panels H, white
arrow,Contrast the preoperative CCTA,Panels D), lung dissemination
should be suspected. The patient gave consent for her anonymous clinical
data to be published in this report.
Discussion:
Tumor recurrence is uncommon, although not unknown, after complete
excision. Recurrence following surgical excision is rare, with an
incidence of only 1%–3% of all myxomas, and the postoperative course
is generally good 1. However, some myxomas occur as
part of a syndrome known as Carney complex, which have some unusual
features. According to Wilbring, et al., elegantly report a 60-year-old
female patient with Carney complex and history of previous 4 cardiac
operations for recurrent myxoma 2.
Carney complex (CNC) is a rare autosomal dominant syndrome,
characterized by mucocutaneous pigmentation, cardiac, cutaneous myxomas
and endocrine overactivity. The criteria for the clinical diagnosis of
Carney complex are two or more major manifestations such as spotty skin
pigmentation lesions, myxomas, endocrine tumors or overactivity,
schwannoma, or breast ductal adenoma. In addition the diagnosis can be
made if there is one of these major manifestations in combination with
the supplementary criteria of either an affected first-degree relative
or inactivating mutation of the PRKAR1A gene 3. They
face a mottled skin pigmentation, and gorgeous features of Cushing’s
syndrome. Myxomas recurrence have been reported in numerous reports4,5,6. In contrast with other reported cases, a
specific characteristic of the present case was the short-term
recurrence of Cardiac myxoma and newly grown nodules in the lungs.
Patients received our recommendations for lifetime anticoagulation
therapy and long-term follow-up observations.
Figure 1. Computed tomography angiography(CTA) imaging and pathological
finding.
CTA revealed uneven low-density clumps attach to the edge of the
blocker(A). The pathological finding revealed stellate mesenchymal cells
in a myxoid stroma(B). Computed tomography pulmonary arteriography(CTPA)
demonstrated massive pulmonary embolism before surgery (C).Chest CT
showed no lung nodules before surgery(D). One month after surgery, the
CTA showed a low-density filling defect in the right atrial septum (E).
Six months after surgery, the CTA showed a large number of low-density
filling defect in the right atrial septum (F). Six months after surgery,
the CTPA demonstrated pulmonary embolism was improved (G). Six months
after surgery, Chest CT showed multiple new round lung nodules in the
lungs(F).
Supplementary material online, Videos S1
CTA revealed uneven low-density clumps attach to the edge of the
blocker,Cross the tricuspid valve into the right ventricle during the
diastolic phase and return to the right atrium during the systolic
phase.
Supplementary material online, Videos S2
CTPA confirmed massive pulmonary embolism in the pulmonary arteries.
Supplementary material online, Videos S3
One month after the operation, CT of the mediastinal window showed a new
growth low-density filling defect on the right side of the atrial
septum, and the CT of the lung window showed no pulmonary nodules in the
lungs.
Supplementary material online, Videos S4
Half a year after the operation, ct of the mediastinal window showed a
new growth low-density filling defect on the right side of the atrial
septum, which was significantly larger than before, and the lung window
CT showed multiple new round lung nodules in the lungs.
Reference:
1.McCarthy PM, Piehler JM, Schaff HV, et al. The significance of
multiple, recurrent, and “complex” cardiac myxomas. J Thorac
Cardiovasc Surg 1986; 91: 389-96.
2.Wilbring M,Wiedemann S,Kappert U,et al.A complicated case of Carney
complex: fifth reoperative cardiac surgery for resection of recurrent
cardiac myxoma.[J].J Thorac Cardiovasc Surg,2013,4:e22-4.
3. Stratakis CA, Kirschner LS, Carney JA. Clinical and molecular
features of the Carney complex: diagnostic criteria and recommendations
for patient evaluation. J Clin Endocrinol Metab 2001; 86: 4041-6.
4. Ando T,Goto H,Date K,et al.Recurrence of cardiac myxoma in the right
atrium with Carney complex following resection of myxomas in both
ventricles.[J].Gen Thorac Cardiovasc Surg,2019,10:891-893.
5. Wei K,Guo HW,Fan SY,et al.Clinical features and surgical results of
cardiac myxoma in Carney complex.[J].J Card Surg,2019,1:14-19.
6.Tamura Y,Seki T.Carney complex with right ventricular myxoma following
second excision of left atrial myxoma.[J].Ann Thorac Cardiovasc
Surg,2014,:882-4.