Primary Pulmonary Paraganglioma

Huang, Xin; Liang, Qi‐Lian; Jiang, Liang; Liu, Qiu-Long; Ou, Wen‐Ting; Li, Da-Heng; Zhang, Huijie; Yuan, Gao‐Le

doi:10.1097/md.0000000000001271

Cited by 19 publications

(12 citation statements)

References 17 publications

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“…Malignancy is defined by the presence of distant metastases at sites where chromaffin cells are normally absent—bones and lymph nodes [19]. However, for lung and liver, it has to be considered that both organs normally contain ganglia, and therefore in these locations, it may be difficult to differentiate between metastases and primary PGLs, especially if past medical history is negative [20,21,22,23,24,25]. Validated prognostic pathological parameters for malignant PCCs/PGLs are lacking, although some risk stratification systems have been described, such as The Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) [26] and the more recently extended Grading of Adrenal Pheochromocytoma and Paraganglioma (GAPP) [27].…”

Section: Introductionmentioning

confidence: 99%

Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

Nölting

Ullrich

Pietzsch

et al. 2019

Cancers

136

201

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Pheochromocytomas and paragangliomas (PCC/PGLs) are rare, mostly catecholamine-producing neuroendocrine tumors of the adrenal gland (PCCs) or the extra-adrenal paraganglia (PGL). They can be separated into three different molecular clusters depending on their underlying gene mutations in any of the at least 20 known susceptibility genes: The pseudohypoxia-associated cluster 1, the kinase signaling-associated cluster 2, and the Wnt signaling-associated cluster 3. In addition to tumor size, location (adrenal vs. extra-adrenal), multiplicity, age of first diagnosis, and presence of metastatic disease (including tumor burden), other decisive factors for best clinical management of PCC/PGL include the underlying germline mutation. The above factors can impact the choice of different biomarkers and imaging modalities for PCC/PGL diagnosis, as well as screening for other neoplasms, staging, follow-up, and therapy options. This review provides a guide for practicing clinicians summarizing current management of PCC/PGL according to tumor size, location, age of first diagnosis, presence of metastases, and especially underlying mutations in the era of precision medicine.

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Section: Introductionmentioning

confidence: 99%

Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

Nölting

Ullrich

Pietzsch

et al. 2019

Cancers

136

201

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“…DW1 shows a high signal. The enhanced scan shows a significant enhancement of the tumor mass[12]. When physical examinations reveal lesions in the posterior mediastinum, in addition to considering common neurogenic tumors such as schwannomas, rare ectopic tumors should also be considered to avoid misdiagnosis.…”

Section: Discussionmentioning

confidence: 99%

Thoracotomy of an asymptomatic, functional, posterior mediastinal paraganglioma: A case report

Yin

Yang

Ahmed

et al. 2019

WJCC

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BACKGROUND Paragangliomas in the mediastinum are rare, accounting for only 1%-2% of all paragangliomas and < 0.3% of all mediastinal tumors. Most paragangliomas are nonfunctional, therefore, asymptomatic functional paragangliomas in the left posterior mediastinum are extremely rare. Perioperative management including preoperative preparation, careful intraoperative procedures, and strict postoperative care is important, and one-stage surgical resection should be performed only after appropriate perioperative measures are undertaken. Because those tumors are rare, it is necessary to report known cases to raise awareness regarding them. CASE SUMMARY We report the case of a 47-year-old male who was admitted to our hospital with the chief complaints of intermittent tearing pain on the left side of the chest and back for more than 10 mo. A chest contrast-enhanced computed tomography scan revealed a round, solid mass in the left posterior mediastinum, with low-density cystic lesions in the middle, and no enlarged lymph nodes in the hilum or mediastinum (Figure 1). After the diagnosis of paraganglioma, the patient was preoperatively given an oral adrenoceptor blocking drug (phenoxybenzamine), and intravenous fluid resuscitation for two weeks, subsequently the patient underwent a one-stage resection of lesions via left thoracotomy. The patient’s blood pressure increased to 220/120 mmHg when the tumor was touched, which could be relieved by symptomatic treatment such as accelerating liquid transfusion or other intervention to lower blood pressure. The patient recovered uneventfully after surgery, with no abnormal blood pressure or recurrence during one year of follow-up visits. CONCLUSION Surgical resection is the preferred treatment for asymptomatic functional paragangliomas.

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“…The lesions typically exhibit significant enhancement on enhanced MRI. [11] In addition to common neurogenic tumors, such as schwannomas, rare ectopic tumors should also be considered in the differential diagnosis of masses located in the posterior mediastinum, in order to avoid misdiagnosis.…”

Section: Discussionmentioning

confidence: 99%

Asymptomatic left posterior mediastinal functional paraganglioma

Yang

Hua

et al. 2019

Medicine

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Rationale: Paraganglioma refers to a set of neuroendocrine tumors derived from the chromaffin cells of the adrenal diplomatic ganglion. Paragangliomas can be classified as functional or nonfunctional based on the ability to synthesize and release catecholamines. Patient concerns: We report a 47-year-old man with a functional paraganglioma in the left posterior mediastinum and highlight the key elements of management of mediastinal paragangliomas. Diagnoses: A left posterior mediastinal mass was found by computed tomography (CT) scan and Chest-enhanced CT. Preoperative ultrasound-guided biopsy suggested the possibility of a paraganglioma. A diagnosis of paraganglioma was established by immunohistochemistry. Interventions: The patient underwent single-stage resection of the lesion via left thoracotomy after preoperative oral α-adrenoceptor (phenoxybenzamine) therapy and intravenous fluid resuscitation for two weeks. Outcomes: The postoperative period was uneventful. The patient exhibited no abnormal blood pressure or recurrence during the 12-month follow-up period. Lessons subsections as per style: Pathological examination alone cannot determine whether it was a benign or malignant paraganglioma, which can be determined by pathological examination combined with distant metastasis. Long-term follow-up is required to assess the treatment effect.

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Primary Pulmonary Paraganglioma

Cited by 19 publications

References 17 publications

Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

Current Management of Pheochromocytoma/Paraganglioma: A Guide for the Practicing Clinician in the Era of Precision Medicine

Thoracotomy of an asymptomatic, functional, posterior mediastinal paraganglioma: A case report

Asymptomatic left posterior mediastinal functional paraganglioma

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