Dataset Information

From inoperable to back to life: a case report of successfully treated obstructive right ventricular primary cardiac lymphoma.

ABSTRACT:

Background

Cardiac masses include various tumourous and non-tumourous lesions. Primary cardiac tumours are very rare and most commonly benign. Primary cardiac lymphomas (PCL) account for 1-2% of malignant primary cardiac tumours. Only 197 cases of PCL have been reported between 1949 and 2009.

Case summary

We report a case of a 73-year-old patient who presented with atrial flutter. The diagnosis was a tumourous cardiac mass in the right atrium with signs of the infiltration of the tricuspid valve insertion and pericardium. There were no signs of extracardiac disease at the initial presentation. The patient was deemed to be inoperable by cardiac surgeons. Rapid tumour progression caused atrioventricular-block type Mobitz 2 with concomitant obstruction of the tricuspid valve and axillary lymph node metastasizing. Excision of the axillary lymph node revealed a diffuse large B-cell non-Hodgkin lymphoma. An epicardial right ventricle single lead pacemaker was sited, and chemotherapy was administered, resulting in complete remission.

Discussion

Cardiac masses are rare and challenging cases. Although current imaging procedures deliver extensive information, histological examination is still required in many cases. We encountered a tumourous mass with deep infiltration. After the patient was deemed inoperable, later lymph node invasion allowed histological examination, revealing PCL. Primary cardiac lymphomas are life-threatening tumours with rapid and aggressive growth. Treatment is based on chemotherapy consisting of anthracycline-containing regimens. This case report highlights the curative potential of chemotherapy, as we report a rapid regression of the tumour as well as the disappearance of arrhythmias and conduction disorders after treatment.

SUBMITTER: Al Mawed M

PROVIDER: S-EPMC8859631 | biostudies-literature | 2022 Feb

REPOSITORIES: biostudies-literature

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Publications

From inoperable to back to life: a case report of successfully treated obstructive right ventricular primary cardiac lymphoma.

Al Mawed Mohammad M Brockmeier Johannes J Haertel Dirk D Ellermeier Marc M Hartmann Frank F Gielen Stephan S

European heart journal. Case reports 20220203 2

<h4>Background</h4>Cardiac masses include various tumourous and non-tumourous lesions. Primary cardiac tumours are very rare and most commonly benign. Primary cardiac lymphomas (PCL) account for 1-2% of malignant primary cardiac tumours. Only 197 cases of PCL have been reported between 1949 and 2009.<h4>Case summary</h4>We report a case of a 73-year-old patient who presented with atrial flutter. The diagnosis was a tumourous cardiac mass in the right atrium with signs of the infiltration of the ...[more]

PMID: 35198851

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Project description:INTRODUCTION: Carney complex is a multiple neoplasia syndrome involving cardiac, endocrine, neural and cutaneous tumors with a variety of pigmented skin lesions. It has an autosomal dominant mode of inheritance. Approximately 7% of cardiac myxomas are related to the Carney complex. Myxomas that occur as part of the Carney complex affect both sexes with equal frequency. Cardiac myxomas with Carney complex are reported mostly in the left side of the heart and are less common on the right side. As per our review, this is the first reported case of Carney complex with right ventricle cardiac myxoma. CASE PRESENTATION: We present a rare case of recurrent cardiac myxoma in a patient later diagnosed to have Carney complex. A 46-year-old Caucasian man with a history of thyroid hyperplasia came to out-patient cardiology department with new onset atrial fibrillation. A transthoracic echocardiogram revealed a right ventricular mass attached to his interventricular septum, which was later seen on a transesophageal echocardiogram and cardiac magnetic resonance imaging. He underwent resection of the ventricular mass which on pathology revealed myxoma. He later developed skin lesions, pituitary adenoma and Sertoli cell tumor suggesting Carney complex. Two years later he developed a new mass within his right atrium which was later resected. CONCLUSIONS: Carney complex is a rare autosomal dominant disease with variable penetrance. Since it involves multiple organs, patients diagnosed with Carney complex should undergo serial endocrine workup, neural assessments, echocardiograms and testicular ultrasounds. Of the total number of cases of Carney complex, 65% are linked to PRKAR1A gene mutation. It is important for clinicians to be cognizant of a link between cardiac myxoma and Carney complex. The use of multi-imaging modalities allows better delineation of the mass before planned resection. Carney complex-related cardiac myxoma comprises 7% of all cardiac myxomas. Right ventricular cardiac myxomas are rare. This case report is the first to describe right ventricular myxoma with Carney complex.

Dataset Information

From inoperable to back to life: a case report of successfully treated obstructive right ventricular primary cardiac lymphoma.

Background

Case summary

Discussion

Publications

From inoperable to back to life: a case report of successfully treated obstructive right ventricular primary cardiac lymphoma.

Similar Datasets

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