Aortic Paraganglioma Masking as Intramural Hematoma

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Aortic paraganglioma initially misdiagnosed as an ascending aortic intramural hematoma (n=1). Intervention: Open-heart surgical excision of a para-aortic paraganglioma under cardiopulmonary bypass. Primary outcome: Complete surgical excision and postoperative clinical recovery without metastasis.

Q: What does this research mean for the field?

Aortic paraganglioma can mimic an intramural hematoma on computed tomography, and complete surgical excision can achieve full symptom resolution. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

Christopher Gaisendrees; Maximilian Luehr; Jana Siemanowski; Udo Siebolts; Ferdinand Kuhn‐Régnier; Thorsten Wahlers

doi:10.1016/j.jaccas.2023.101852

May 4, 2023JACC Case ReportsOpen Access

Aortic Paraganglioma Masking as Intramural Hematoma

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Key result

Surgical excision of an aortic wall paraganglioma mimicking intramural hematoma resolves symptoms promptly.

n=1

Figures

Graphical abstract published by JACC Case Reports — Graphical abstract · Published by JACC Case Reports · CC BY-NC-ND

Key Points

To report a case of an aortic paraganglioma misdiagnosed as an intramural hematoma on preoperative imaging and describe its surgical and diagnostic management.
Clinical evaluation of a 52-year-old woman presenting with dyspnea and angina using electrocardiography, transthoracic echocardiography, CT angiography, and coronary angiography.
Median sternotomy with cardiopulmonary bypass, cold blood cardioplegia, and aortic exploration to inspect the lumen and resect the outer aortic mass.
Postoperative histopathology, SDHB immunohistochemistry, next-generation DNA panel sequencing, and a 2-month surveillance positron emission tomography–CT scan.
Surgical inspection revealed no intimomedial intramural hematoma, but instead uncovered a 1.5 × 1.5 × 3.0-cm solid structure on the outer aortic wall that was completely excised.
Histopathology and sequencing confirmed an extra-adrenal paraganglioma with loss of nuclear SDHB expression, a pathogenic SDHB p.Arg90Ter mutation, and a tumor mutational burden of 0.8 mutations per megabase.
The patient was discharged without angina or dyspnea on postoperative day 7, and a follow-up PET-CT at 2 months showed no metastatic abnormalities.

Study Design

Type

Case Report (n=1)

Multicenter

No

Structured PICO

P

Population

1 female patient (age 52) presenting with NYHA class II dyspnea and angina, initially diagnosed with an ascending aortic intramural hematoma and found intraoperatively to have an extra-adrenal aortic paraganglioma, followed for 2 months.

I

Intervention

Open-heart surgery via sternotomy under general anesthesia, cardiopulmonary bypass, and cold blood cardioplegia (Buckberg), with exploratory aortotomy followed by extra-aortic dissection and complete surgical excision of a 1.5 x 1.5 x 3.0-cm solid para-aortic paraganglioma on the outer aortic wall.

O

Outcome

Histopathological and genetic diagnosis of the excised mass, resolution of angina and dyspnea at discharge on postoperative day 7, and absence of metastatic disease on positron emission tomography-CT at 2 months postoperatively.hard clinical

Rare para-aortic paragangliomas can mimic acute ascending aortic intramural hematomas on multimodality imaging, underscoring the need for high clinical suspicion, complete surgical resection, and postoperative genetic and PET-CT screening for metastasis.

Limitations

Preoperative multimodality imaging (echocardiography, CT angiography, and coronary angiography) failed to differentiate the aortic paraganglioma from an intramural hematoma, leading to an unnecessary aortotomy.
Even with multiple diagnostic imaging modalities (TTE, TEE, CT angiography, coronary angiography), a definitive preoperative diagnosis was not reached, resulting in an initial misdiagnosis of intramural hematoma.
Opening of the ascending aorta for inspection could have been avoided if the correct diagnosis of an extra-aortic mass had been established preoperatively.

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Implication

Consider paraganglioma in atypical aortic wall lesions; case extends differentials and leaves open standardized surveillance protocols.

Abstract

A 52-year-old woman presented dyspnea and angina. The computed tomography scan indicated an intramural hematoma, and the patient underwent surgery, during which a structure was excised that was identified as aortic paraganglioma. This case report underlines the importance of a multiprofessional interdisciplinary team to diagnose and treat cardiac masses. (Level of Difficulty: Advanced.)

Discussion

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Cite This Study

Gaisendrees et al. (2023) conducted a case report in Aortic paraganglioma initially misdiagnosed as an ascending aortic intramural hematoma (n=1). Open-heart surgical excision of a para-aortic paraganglioma under cardiopulmonary bypass was evaluated on Complete surgical excision and postoperative clinical recovery without metastasis. Surgical excision of a 1.5 x 1.5 x 3.0-cm outer aortic wall paraganglioma mimicking an intramural hematoma resulted in symptom resolution and discharge on postoperative day 7.

synapsesocial.com/papers/6a75f3198d9f8f61228b74ce https://doi.org/10.1016/j.jaccas.2023.101852

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Aortic Paraganglioma Masking as Intramural Hematoma

Figures

Key Points

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

Aortic Paraganglioma Masking as Intramural Hematoma

Figures

Key Points

Study Design

Structured PICO

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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