Malignant paraganglioma in children treated with embolization prior to surgical excision

Abstract

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.

Pheochromocytomas and paragangliomas in children: Data from the Italian Cooperative Study (TREP)

“…The use of alternatives to surgical excision (angiographic embolization, radiofrequency ablation, or cryoablation) has been mostly described for metastatic PC/PGL 30 . A recent systematic review 31 underlined how ablation techniques have been used in adults with different aims (palliative care, symptom control, or treatment of unresectable lesions, with or without other associated systemic/local therapies), while, in the pediatric age group, their application is still anecdotal 32,33 …”

Retroperitoneal Paraganglioma-Induced Cardiogenic Shock Rescued by Preoperative Arterial Embolization

“…However, it can be performed under strict monitoring to reduce vascular supply in large, hypervascularized tumors, thus facilitating surgical excision [ 13 – 16 ]. However, there are no indications that this procedure significantly reduces perioperative blood loss, surgical time, or hospital stay [ 17 , 18 ].…”

Malignant extra-adrenal pelvic paraganglioma in a paediatric patient

“…It is considered that between 10% and 20% of cases are diagnosed in the paediatric age group, with an estimated incidence of 0.011 for each 100,000 children under 18 years of age. Of these, approximately 12% are considered malignant [ 1 – 3 ]. Accordingly, the only widely accepted criterion is the presence of metastases in organs where normally there are no chromaffin cells, as is the case of lymph nodes [ 3 , 4 ].…”

“…Of these, approximately 12% are considered malignant [ 1 – 3 ]. Accordingly, the only widely accepted criterion is the presence of metastases in organs where normally there are no chromaffin cells, as is the case of lymph nodes [ 3 , 4 ]. Extra-adrenal paragangliomas are usually diagnosed in the retroperitoneum, head, and neck.…”

“…Those located in the abdomen and pelvis are associated with the sympathetic nervous system, with catecholamine hypersecretions being the cause of the signs and symptoms during the clinical presentation, as well as the possible effects of mass on neighbouring organs [ 5 , 6 ]. The treatment of choice for this type of neoplasm is surgical resection and eventually, tumour embolisation prior to surgery or the administration of combined chemotherapy [ 3 , 4 ].…”