Recurrent Disease in Patients With Sporadic Pheochromocytoma and Paraganglioma.

CONTEXT: Long-term follow-up has been recommended for patients with pheochromocytoma or paraganglioma (PPGL) due to potential for recurrent disease. However, the need to follow patients with sporadic PPGL has recently become controversial. OBJECTIVE: To investigate the prevalence of recurrence among patients with sporadic compared with hereditary PPGL and to identify predictors of recurrence for sporadic disease. METHODS: This multicenter study included retrospective data from 1127 patients with PPGL. In addition to sex and age at primary tumor diagnosis, clinical information included location, size, and catecholamine phenotype of primary tumors, genetic test results, and subsequent development of recurrent and/or metastatic disease. Patients with sporadic PPGL were defined as those with negative genetic test results. RESULTS: Prevalence of recurrence among patients with sporadic PPGL (14.7%) was lower (P < 0.001) than for patients with pathogenic variants that activate pseudohypoxia pathways (47.5%), but similar to those with variants that activate kinase pathways (14.9%). Among patients with sporadic recurrent PPGL, 29.1% and 17.7% were respectively diagnosed at least 10 and 15 years after first diagnosis. Multivariable regression analysis showed that a noradrenergic/dopaminergic phenotype (HR 2.73; 95% CI, 1.553-4.802; P < 0.001), larger size (HR 1.82; 95% CI, 1.113-2.962; P = 0.017) and extra-adrenal location (HR 1.79; 95% CI, 1.002-3.187; P = 0.049) of primary tumors were independent predictors of recurrence in sporadic PPGL. CONCLUSION: Patients with sporadic PPGL require long-term follow-up, as supported by the 14.7% prevalence of recurrent disease, including recurrences at more than 10 years after first diagnosis. The nature of follow-up could be individualized according to tumor size, location, and biochemical phenotype.

Complete resection of isolated hepatic metastatic uveal melanoma with a notably long disease-free period: A case report and review of the literature.

Although uveal melanoma frequently metastasizes to the liver, cases with a large solitary lesion with spontaneous intratumoral hemorrhage and necrosis are rarely encountered. Here, we report a case of metastatic hepatic melanoma that occurred in a 45-year-old Chinese male. The patient complained of the feeling of a full stomach for a month. Ten years earlier, the patient had undergone left ocular enucleation and artificial eye implantation at a different hospital. Postoperative pathology revealed choroidal melanoma without intrascleral or vascular involvement. Abdominal magnetic resonance imaging and computed tomography scan revealed a solitary lesion measuring 12 cm in diameter. A whole-body F-18-fluoro-2-deoxyglucose (FDG) positron emission tomography/computed tomography scan demonstrated a large solitary nodule with increased FDG uptake. Computed tomography angiography revealed that the huge mass had partially ruptured and was bleeding spontaneously. For diagnostic and therapeutic purposes, right hepatectomy was performed and histological examination revealed that the tumor was metastatic melanoma.