Page 93 - Medicine and Surgery
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                                                                         Chapter 2: Cardiovascular oncology 89


                  Aetiology                                     Carotid body tumours
                  The aetiology of atrial myxoma is unknown.
                                                                Definition
                                                                Tumour arising from chemoreceptors at the bifurcation
                  Pathophysiology
                                                                of the carotid artery.
                  The tumour is usually located on a pedicle arising from
                  the atrial septum, and can grow up to about 8 cm
                                                                Incidence
                  across. The pedicle allows the tumour to move within
                                                                Rare
                  the atrium resulting in various symptom complexes. If
                  the tumour obstructs the mitral valve a picture similar to  Aetiology
                  mitral stenosis will occur. If the tumour passes through  More common in people living at high altitude; it is
                  the mitral valve, mitral regurgitation will occur. Com-  thought that long-term hypoxia is a predisposing factor.
                  plete mitral valve obstruction may cause syncope. The
                  tumour may also give rise to thrombosis due to altered  Pathophysiology
                  flow patterns and resultant systemic embolisation. Local  Carotid body tumours are hormonally inactive chemod-
                  invasion and distant metastasis do not occur.  ectomas arising from the chemoreceptor tissue of the
                                                                common carotid artery at the level of the bifurcation
                  Clinical features                             of the artery into internal and external carotid body
                  The clinical picture varies but the diagnosis is suggested  (anatomically at the level of the hyoid bone). The tu-
                  by features of mitral stenosis with variable cardiac mur-  mour tends to grow upwards towards the skull base.
                  murs and a loud first heart sound especially if coupled
                  toafever. Many patients have a ‘tumour plop’ in early  Clinical features
                  diastole. Thromboembolism may result from the abnor-  Patients present with a pulsatile swelling in the upper
                  mal flow pattern through the atrium. It occurs in 40%  neck at the medial border of the sternocleidomastoid
                  and is a common presenting feature.           muscle. Classically on palpation the lump is mobile from
                                                                side to side but not up and down, and there may be an
                                                                associated overlying carotid bruit. It is important to dis-
                  Investigations
                                                                tinguish the rare carotid body tumour from the more
                  ESR is usually raised. Echocardiography demonstrates
                                                                common metastatic lymph node from a head and neck
                  the mass lesion within the atrium.
                                                                tumour, which may have transmitted pulsation. Metas-
                                                                tasis of carotid body tumours is very rare.
                  Macroscopy
                  The tumour is usually a polypoid mass on a stalk, its sur-  Microscopy
                  face covered with thrombus. Histologically the tumour  The tumour has a rich blood supply. It is composed of
                  is made up of connective tissue, with a variety of cell  chief cells with clear cytoplasm and a round nucleus en-
                  typessurrounded by extracellular matrix.      closed in a fibrous tissue framework.

                                                                Investigations
                  Management                                    Angiography shows a splaying of the carotid bifurcation
                  The tumour is surgically removed under cardiopul-  (lyre sign). CT and MRI imaging demonstrate the tu-
                  monary bypass.                                mour.

                                                                Management
                  Prognosis
                                                                Surgical excision may be performed especially in young
                  Five per cent local recurrence within 5 years. Follow-
                                                                healthypatients.Inelderlypatientssurgicalremovalmay
                  up with regular echocardiography is therefore indicated
                                                                not be necessary.
                  even in asymptomatic patients.
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