Feocromocitoma y paraganglioma en la infancia: a propósito de 2 casos

Gorostidi, A. Mosquera; Ranera, A. Justo; Denis, Solène; Temprano, N. González; Uranga, M. Sagaseta de Ilúrdoz; Garicano, J. Molina

doi:10.1016/j.anpedi.2014.06.018

Cited by 4 publications

(6 citation statements)

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“…Nuclear medicine studies are reserved for patients with biochemical evidence of disease but with a negative CT or MRI and in suspected cases of metastatic disease. Even though there is no pathognomonic feature on CT scan to identify PGL, a heterogeneous, hypervascular mass with differential enhancement indicating areas of necrosis arising from the retroperitoneum is highly predictive [3]. …”

Section: Discussionmentioning

confidence: 99%

“…Preoperative biopsy should not be routinely performed due to the risk of an adrenergic crisis [5]. Alpha-adrenergic blockade for at least 4 weeks preoperatively may be administered in order to prevent and, if needed, to treat intra-operative catecholamine release [1, 3]. As neither signs nor symptoms of catecholamine release were observed in both cases prior to surgery and during biopsy, no alpha-adrenergic blockade was performed and no systemic repercussions were observed.…”

Section: Discussionmentioning

confidence: 99%

“…Laparoscopy can also be offered in cases of abdominal localization [3]. However, there are no data on the benefits of aggressive resection or debulking in the setting of locally invasive, metastatic, or recurrent disease [2].…”

Section: Discussionmentioning

confidence: 99%

“…The average age of diagnosis in the pediatric population is 11 years, but there are case reports as young as 5 years of age [3, 4]. There is a male predominance of 2:1 including adolescents.…”

Section: Introductionmentioning

confidence: 99%

“…The rate of metastatic disease is very variable in the literature, with reports ranging from 2.5 to 40 %. Recurrence, either regional or metastatic, usually occurs within 5 years of initial complete resection, but there are reports of recurrence up to 40 years postoperatively [3]. Although well described for the adult population, diagnosis and treatment of malignant pediatric paragangliomas are poorly described in the literature [4].…”

Section: Introductionmentioning

confidence: 99%

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Malignant paraganglioma in children treated with embolization prior to surgical excision

et al. 2015

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BackgroundParagangliomas (PGL) are rare tumors derived from neural crest cells, whose origins may vary along the chain of the sympathetic nervous system. Such tumors are often characterized by secretion of catecholamines, but sometimes they are biochemically inactive, which makes diagnosis often challenging. Malignant paraganglioma is defined by the presence of this tumor at sites where chromaffin cells are usually not found or by local invasion of the primary tumor. Recurrence, either regional or metastatic, usually occurs within 5 years of the initial complete resection but long-term recurrence is also described. Malignancy is often linked to a SDHB mutation. Preoperative embolization has been applied in the surgical management of PGLs with the objective to decrease intra-operative blood loss and surgery length without complications.Case PresentationWe report two cases of patients with abdominal or pelvic malignant PGLs who have been treated surgically at our center after preoperative embolization. Surgery was a very challenging procedure with multiple surgical teams involved and embolization did not prevent major blood loss and intraoperative complications. Patients required adjuvant treatment with either chemotherapy or radiotherapy.ConclusionsMany studies in the adult population have established recommendations for the diagnosis and therapeutic management of PGL, but few studies concern the pediatric population. Because malignant PGL is more important in the pediatric population, screening and early diagnosis of PGL is advisable in children with genetic predisposing. Surgical resection is the mainstay of treatment, but a multimodal approach is often required due to the complexity of cases. The role of preoperative embolization is not established and in our experience it has provided little benefit and major complications.

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Section: Discussionmentioning

confidence: 99%