Familial malignant retroperitoneal paraganglioma

Sebastian, J. P.; Williams, Steven E.; Wells, Michael; Peake, Mick

doi:10.1136/pgmj.65.768.781

Cited by 6 publications

(5 citation statements)

References 11 publications

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“…Each of these patients had a solitary extraadrenal paraganglioma, four of which occurred in association with adrenal pheochromocytoma. There have been isolated reports of familial paraganglioma in the literature [25][26][27][28][29][30]. Glowniak et al [26] in 1985 reported familial paraganglioma occurring at the same anatomic location in different family members and suggested that specific genetic abnormalities determine sites of tumor development.…”

Section: Discussionmentioning

confidence: 99%

Clinical Spectrum and Outcome of Functional Extraadrenal Paraganglioma

et al. 1996

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Functioning extraadrenal paragangliomas represent more than 10% of all pheochromocytomas, occur at diverse anatomic locations, and are said to have a higher malignancy rate than intraadrenal pheochromocytomas. Sixty-six patients had surgery for catecholamine-producing paragangliomas between 1952 and 1992. Median follow-up was 8.8 years. Median age was 40 years (11-67 years); the male/female ratio was 29:37. Familial disease occurred in 9 patients (13.6%), and 10 patients (15.2%) also developed adrenal pheochromocytoma. Solitary paragangliomas occurred in 52 patients: 46 abdominal, 4 thoracic, and 2 head and neck. Fifty-three tumors developed in 14 patients with multiple paragangliomas: 38 abdominal and 15 thoracic. Of 28 patients with solitary tumors undergoing localization studies over the past 10 years accurate localization was achieved in 27. There was one operative death; 15 patients had persistent disease; and 50 were cured postoperatively. Of those cured, nine developed recurrence, disease-free survival being 86%, 80%, and 80% at 5, 10, and 20 years. Metastatic disease was found in 14 patients (21%), 7 of whom have died. An additional 10 patients (15%) had locally invasive disease, of whom 4 have died. Cause-specific survivals at 5, 10, and 20 years were 90%, 83%, and 72%. Risk factors for death from pheochromocytoma were tumor size > 5 cm (p = 0.0002), metastatic disease (p = 0.001), and tumor invasion (p = 0.0023). Cause-specific survival for patients with tumors > 5 cm was 59% at 15 years compared to 100% among patients with tumors 5 cm and the occurrence of invasive or metastatic disease are strong predictors of outcome. Most tumors are abdominal, and imaging is highly successful for localization.

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Section: Discussionmentioning

confidence: 99%

Clinical Spectrum and Outcome of Functional Extraadrenal Paraganglioma

et al. 1996

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“…It is estimated that 10% of tumors occur outside the adrenal gland. Of extra‐adrenal tumors, chemodectomas and glomus jugulare tumors account for 90%, but they have been reported at many other sites within the head and neck, as well as the mediastinum, retroperitoneum, cauda equina, urinary and the gastro intestinal tract 3,32 …”

Section: Discussionmentioning

confidence: 99%

“…Of extra-adrenal tumors, chemodectomas and glomus jugulare tumors account for 90%, but they have been reported at many other sites within the head and neck, as well as the mediastinum, retroperitoneum, cauda equina, urinary and the gastro intestinal tract. 3,32 Histologically, paragangliomas are composed of spindle (sustentacular) cells and round or polygonal epithelioid cells arranged in a 'Zellballen' pattern. The spindle cells correspond to Schwann cells and are S-100 positive.…”

Section: Discussionmentioning

confidence: 99%

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First reported case of esophageal paraganglioma. a review of the literature of gastrointestinal tract paraganglioma including gangliocytic paraganglioma

Harries

Nunn

Shah

et al. 2004

Diseases of the Esophagus

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Paraganglioma and the variant gangliocytic paraganglioma are rare gastrointestinal tumors. We present the first reported case of an esophageal paraganglioma and a review of the literature. From this review it seems that these tumors can occur at any age and usually present with acute or chronic bleeding with or without abdominal pain. The majority of reported cases originated in the foregut, most commonly the second part of the duodenum. Macroscopically the tumor may be pedunculated, sessile or ulcerated and have been described up to 10 cm in size. There are no reported cases of gut paragangliomas shown to be producing clinically significant amounts of catecholamines. The majority of reported tumors have been benign, only 7% malignant at presentation and all with lymph node metastases. One case developed bone metastases 3 years after excision and another recurred locally. There has been no benefit seen from radiotherapy or chemotherapy to date and it is recommended that all of these tumors are widely excised together with a lymph node resection if possible.

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“…4 Intraoperatively the tumours appeared solid, homogeneous, and pale brown Paragangliomas are neuroendocrine tumors, and those occurring in the head and neck have well-recognized familial association. Retroperitoneal paragangliomas are uncommon, and their malignant potential has been very rarely reported in literature [4].…”

Section: Discussionmentioning

confidence: 99%

Multiple Retroperitoneal Paragangliomas

Rai¹,

Subas

Kini

et al. 2012

Indian J Surg

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Paragangliomas are rare with multicentricity being more common in patients with familial history. Early recognition of the characteristic symptoms of headache, palpitations, and diaphoresis in a patient with hypertension and prompt appropriate intervention can minimize the morbidity associated with such tumors and prevent a potentially fatal outcome. Paragangliomas represent neoplasms of neural crest origin that arise from paraganglia. Paragangliomas are tumours arising in extra-adrenal paraganglia either in the paravertebral space. We report a rare case of two discrete paragangliomas in the retroperitoneum of a young girl presenting with throbbing headache. Contrast Enhanced CT Abdomen showed the lesions as a large irregular heterogeneous and intensely enhancing mass lesions. Elevated levels of vanillylmandelic acid were found in the Urine. The lesions were removed by surgery and the histopathologic findings of both the lesions confirmed the diagnosis of multiple paragangliomas. Early identification and early surgery will minimize the potential risks and complications in this condition.

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Familial malignant retroperitoneal paraganglioma

Cited by 6 publications

References 11 publications

Clinical Spectrum and Outcome of Functional Extraadrenal Paraganglioma

Clinical Spectrum and Outcome of Functional Extraadrenal Paraganglioma

First reported case of esophageal paraganglioma. a review of the literature of gastrointestinal tract paraganglioma including gangliocytic paraganglioma

Multiple Retroperitoneal Paragangliomas

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