Right ventricular calcified amorphous tumour

Abstract

Calcified amorphous tumour is a rare variety of benign cardiac intra cavitary tumour. Only a handful of cases have been reported till date. We report a case of a 42 years old male without any known comorbidities who presented with history of syncope and dyspnoea on exertion. Transthoracic echocardiography and cardiac magnetic resonance imaging revealed pedunculated mobile well-defined right ventricular mass attached to its free wall by a thin pedicle. He underwent surgical excision of mass under normothermic bypass. Microscopic examination revealed necrotic material with rim of fibrocollagenous tissue; focal dystrophic calcification was seen.

Introduction

The causative factors, pathogenesis and natural history of calcified amorphous tumour (CAT), which has been reported to occur rarely in heart, remain unclear [1]. It mostly presents with shortness of breath or is asymptomatic [2]. Definitive diagnosis requires histopathological evaluation of excised mass [3]. We report a case of right ventricular CAT in a 42 years old male who presented with history of syncope and dyspnoea on exertion.

Case report

A 42 years old male presented with history of loss of consciousness with spontaneous recovery and dyspnoea on exertion (New York Heart Association III) of 6 months duration. Clinical examination was unremarkable except for loud pulmonary component of second heart sound on auscultation. Transthoracic echocardiography showed mobile hyper echoic mass with echo shadows (suggestive of calcification). Cardiac magnetic resonance imaging (MRI) (Fig. 1) revealed a pedunculated mobile well-defined mass measuring 15 × 9 mm attached to the free wall of the right ventricle by a thin pedicle. He underwent surgical excision of mass (Fig. 2). After a median sternotomy, cardiopulmonary bypass via aorto-bicaval cannulation was established. The right ventricle was approached from right atrium and tricuspid valve. The CAT was visualised through tricuspid valve and was excised. He was then weaned from cardiopulmonary without difficulty. Post operative recovery was uneventful. Gross features (Fig. 3) showed single globular mass measuring 11 × 6 × 5 mm with calcified tissue which was hard to cut; cut surface showed calcified material. Microscopic examination revealed necrotic material with rim of fibrocollagenous tissue; focal dystrophic calcification was seen (Fig. 3).

Fig. 1.

Cardiac MRI revealed pedunculated mobile well-defined right ventricular mass attached to its free wall by a thin pedicle measuring 15 × 9 mm (yellow arrow) [RA- Right Atrium; RV- Right Venticle; LA- Left Atrium; LV-Left Ventricle]

Fig. 2.

Gross features showed single globular mass measuring 11 × 6 × 5 mm

Fig. 3.

Hematoxylin and eosin–stained section shows amorphous debris with admixed degenerated fibrin

Discussion

In 1997 Reynolds et al. [1] coined the term CAT in their report of a series of 11 patients with intracavitary cardiac masses, from 1965 to 1994. The histological examination consisted of calcified deposits with the background of amorphous degenerating fibrinous material [1]. Female preponderance has been seen in this lesion [4]. CAT affects all cardiac chambers, most commonly involved the left ventricle or mitral valve apparatus [4, 5]. Tumour size ranges from 1.7 mm to very large masses (20 × 90 mm) or even diffuse left ventricular (LV) infiltration [1, 4] However, our patient was 42 years which was younger than the mean age (54 years) [4]. The tumour was in the right ventricle which is one of the rare sites for CAT.

These patients most frequently present with dyspnoea and syncope. Pulmonary or systemic embolisation has been seen in about one-third of the cases. CAT is discovered incidentally in 17% of the patients. Our patient presented with syncope and dyspnoea on exertion with no systemic features of embolisation. Surgery is performed in most of the reported cases [2, 4]. Surgery had high rate of successful outcome [6–8]. Medical treatment is favoured in cases who have diffuse calcium infiltration of the ventricle [9, 10].

In summary, we report a case of CAT in the right ventricle in a 42-year-old male who was successfully treated by a complete resection of the mass. Cardiac CAT is a non-neoplastic cardiac tumour of unknown etiology. The literature review highlights that the tumour is commonly an incidental finding and the treatment of choice is complete surgical resection. Even though most cases are accompanied by other cardiac, renal and systemic comorbidities, it can also be seen in individuals without any previous comorbidities. Definitive diagnosis of CAT is difficult preoperatively. It requires histopathologic evaluation of excised mass.

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Declarations

Ethics approval

Institutional Ethical Committee, Army Hospital (R&R), Delhi Cantt IEC Regn no. 18/2021.

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Consent for publication

Written informed consent was obtained.

Conflict of interest

Nil.

Statement of human and animal rights

All human and animal studies have been approved by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.