Cystic Tumor of the Atrioventricular Node

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mandana Chitsazan, M.D. [2]

Synonyms and keywords: Mesothelioma of the atrioventricular node

Historical Perspective

Cystic tumor of the atrioventricular (AV) node was first reported by Armstrong and Mönckeberg in 1911.

Classification

There is no established system for the staging of the cystic tumor of the atrioventricular node.

Pathophysiology

Although the cystic tumor of the AV node is also called mesothelioma of the AV node, it appears to be a misnomer. The origin of the cystic tumor of the AV node is still controversial. It is believed that it has endodermal origin, derived from congenital rests during embryogenesis. (5-7). One study suggested that cystic tumor of the AV node and solid cell nests of the thyroid have similar structures and may share the same developmental process; therefore it is assumed that cystic tumor of the AV node is an ultimobrachial heterotopia.

On microscopic histopathological analysis, solid cell nests and cystic structures lined by flattened cuboidal or squamous epithelium mixed with rare neuroendocrine cells are characteristic findings of the cystic tumor of the AV node.

Causes

Disease name] may be caused by [cause1], [cause2], or [cause3]. OR Common causes of [disease] include [cause1], [cause2], and [cause3]. OR The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4]. OR The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click here.

Differentiating Cystic Tumor of the Atrioventricular Tumor from Other Diseases

Considering the location and histopathologic

Epidemiology and Demographics

CTANV is commonly diagnosed in the fourth decade of life. <ref name="pmid26725181">Burke A, Tavora F (2016). "The 2015 WHO Classification of Tumors of the Heart and Pericardium". J Thorac Oncol. 11 (4): 441–52. doi:10.1016/j.jtho.2015.11.009. PMID 26725181.</ref>

The female to male ratio is approximately 3 to 1 .

Risk Factors

There are no established risk factors for [disease name].

OR

The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].

OR

Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

OR

Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.

Screening

There is insufficient evidence to recommend routine screening for cystic tumor of the atrioventricular node.

Natural History, Complications, and Prognosis

If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

OR

Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].

OR

Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.

Diagnosis

Diagnostic Study of Choice

The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].

OR

The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].

OR

The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].

OR

There are no established criteria for the diagnosis of [disease name].

History and Symptoms

The majority of patients with [disease name] are asymptomatic.

OR

The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].

Physical Examination

Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].

OR

Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

The presence of [finding(s)] on physical examination is diagnostic of [disease name].

OR

The presence of [finding(s)] on physical examination is highly suggestive of [disease name].

Laboratory Findings

The cysts contain a clear to mucoid material and mucinous, ciliated, goblet and transitional type cells may also be present in the lining (Fig. 29). The cells express CK7, but not CK20, and also stain positive for EMA and CEA. Miller

Electrocardiogram

There are no ECG findings associated with [disease name].

OR

An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

X-ray

There are no x-ray findings associated with cystic tumor of the atrioventricular tumor.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

There are no CT scan findings associated with cystic tumor of the atrioventricular tumor.

MRI

There are no MRI findings associated with [disease name].

OR

[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with cystic tumor of the atrioventricular tumor.

Other Diagnostic Studies

There are no other diagnostic studies associated with cystic tumor of the atrioventricular tumor.

Treatment

Medical Therapy

There is no medical treatment for cystic tumor of the atrioventricular tumor.

Surgery

Because of the critical location of the tumor and its unfavorable prognosis, surgical excision is the mainstay of treatment for cystic tumor of the atrioventricular node. Anti-arrhythmic drugs and lifelong pacemaker placement may be necessary after excision to manage any post-surgical AV block.

Primary Prevention

There are no established measures for the primary prevention of cystic tumor of the atrioventricular tumor.

Secondary Prevention

There are no established measures for the secondary prevention of cystic tumor of the atrioventricular node.

References

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