National Cancer Data Base report on malignant paragangliomas of the head and neck

Abstract

BACKGROUNDMalignant paragangliomas of the head and neck are rare, with previous reports limited to nine or fewer patients. The current review included 59 cases extracted from the National Cancer Data Base that were diagnosed between 1985–1996.METHODS The primary criterion for inclusion in the current study was verified metastatic spread from a paraganglioma of the head and neck. Patterns of presentation and treatment as well as clinically relevant associations were demonstrated in contingency tables. Relative survival was used for analysis of outcome.RESULTSThe average patient age at presentation was 44 years, and gender distribution was equivalent. Metastases were confined to regional lymph nodes in the majority of cases (68.6%), with carotid body tumors found to have an even higher rate of regional confinement (93.8%). Surgery was the most common treatment (76.3%). The use of adjuvant irradiation for regionally confined disease increased across time, from 27% (1985–1990) to 46% (1991–1996). The 5‐year relative survival rate was 59.5% (76.8% for regionally confined carcinoma and 11.8% for distant metastasis). Among patients who were followed until death, those treated with adjuvant irradiation had a longer median survival (45 months) compared with those patients who were treated with surgery alone (12 months).CONCLUSIONS Malignant paraganglioma represents metastatic spread of a tumor type that, when restricted to the site of origin, is considered benign. Metastases from malignant paragangliomas of the head and neck usually are regionally confined. The primary management of a recognized malignancy should be directed toward complete surgical removal of the primary tumor and regional lymph nodes. Postoperative irradiation may be beneficial in slowing the progression of residual disease. Cancer 2002;94:730–7. © 2002 American Cancer Society.DOI 10.1002/cncr.10252

Genetics and clinical characteristics of hereditary pheochromocytomas and paragangliomas

“…Currently, malignancy cannot be predicted with certainty, although some histological or gene expression features might be suggestive of malignancy (Strong et al 2008). The prognosis of malignant PCC and PGL is poor, with a 5-year mortality rate O50% (Lee et al 2002, Chrisoulidou et al 2007. There is currently no effective or curative treatment, but surgery, chemotherapy, and radiotherapy are beneficial in some patients.…”

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“…There is also a well-established familial association that has been observed in upward of 10% of PGLs, owing predominantly to mutations in three of the four succinate dehydrogenase subunit genes (SDHB, SDHC, and SDHD). 1,5 This genetic predisposition carries an increased risk for multifocal tumor development, with an 80% chance of multicentricity in familial cases as opposed to only a 10 to 20% chance in sporadic cases. 1,5 The genetic characteristics of familial PGLs have been well described in various publications, such as Martin et al 2007 and Papaspyrou et al 2011, although SDHD is the most commonly implicated gene in the development of benign familial PGLs of the head and neck, and SDHB mutations have the highest rate of developing malignant PGLs.…”

“…1,5 This genetic predisposition carries an increased risk for multifocal tumor development, with an 80% chance of multicentricity in familial cases as opposed to only a 10 to 20% chance in sporadic cases. 1,5 The genetic characteristics of familial PGLs have been well described in various publications, such as Martin et al 2007 and Papaspyrou et al 2011, although SDHD is the most commonly implicated gene in the development of benign familial PGLs of the head and neck, and SDHB mutations have the highest rate of developing malignant PGLs. 5, 12 Papaspyrou et al did report one case of their 175 patient series in which the patient was found to be positive for a mutation in the SDHC gene after presenting with multiple malignant PGLs of the ITF, carotid body, and jugulotympanic region.…”

“…1 Formerly known as glomus tumors, or chemodectomas, PGLs develop from the extraadrenal chromaffin and chief cells of autonomic paraganglia in various locations throughout the body, usually in association with the walls of blood vessels or specific nerves. 2,3 Although these generally benign lesions most often present by exerting local effects on surrounding structures, a smaller subset of tumors can retain their neuroendocrine functionality and present with signs and symptoms of excess circulating catecholamines.…”

“…1 The infratemporal fossa (ITF) represents an especially rare location for a PGL to emerge, with only a handful of cases in the literature describing such a presentation. 4,5 Given the complex anatomy of the anterior skull base and the critical neurovascular structures within the ITF, successful resection of such a highly vascular tumor within this space presents numerous perioperative and intraoperative challenges.…”