Med Bull Sisli Etfal Hosp. 2020; 54(4): 391-398 | DOI: 10.14744/SEMB.2020.58998

Pheochromocytoma and Paraganglioma: From Treatment to Follow-up

Nurcihan Aygun, Mehmet Uludag
Department of General Surgery, University of Health Sciences Turkey, Sisli Hamidiye Etfal Teaching and Research Hospital, Istanbul, Turkey

Pheochromocytomas (PCC) and paragangliomas (PGL) are rare neuroendocrine tumors arising from chromaffin cells in the adrenal medulla, sympathetic or parasympathetic ganglia. Currently, the only curative treatment option of pheochromocytomas/paraganglioma (PPGL) is surgical resection. Surgery aims to eliminate both risks of hypersecretion and tumor growth. The consequences of hypersecretion should be carefully controlled with medical therapy before and during the surgery. Postoperative major complications are hypotension and rebound hypoglycemia, and patients should be followed closely for 24-48 hours. The choice of surgical approach is determined based on multiple factors, including germline genetic test results, the size of the tumor, body mass index, surgeon's experience, and the likelihood of malignancy. Primary tumor resection does not completely eliminate the risk of tumor persistence and recurrence. Therefore, all patients with PPGL who are surgically treated should be followed for at least 10 years for recurrent disease and new tumor formation. Although surgical resection is the only curative treatment for PPGLs, surgical treatment is palliative except for resectable locoregional metastases in metastatic disease or for isolated distant metastases. The purpose of palliative treatment is to reduce hormone secretion and prevent metastasis-related complications in a critical anatomical location Combined and alfa;- and beta- adrenergic blockade is usually applied in patients with PPGL preoperatively. Some patients may present with pheochromocytoma multisystem crisis, which is a life-threatening condition that can involve cardiovascular, pulmonary, neurological, gastrointestinal, renal, hepatic and metabolic systems. Pheochromocytoma crisis may be spontaneous or may present with the tumor manipulation, trauma, corticosteroids, beta-blockers, anesthetic drugs, and the stimulation of non-adrenal surgical stress. These patients should be considered as medical emergencies rather than surgical emergencies. In this review, it was aimed to evaluate the pre-, per and post-operative management, curative and palliative surgical management, and postoperative outcomes and follow-up of the patients with PPGLs.

Keywords: Follow up, pheochromocytoma; paraganglioma; palliative; surgical; systemic treatment.

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Feokromasitoma ve paraganglioma: Tedaviden takibe

Nurcihan Aygun, Mehmet Uludag
Saðlýk Bilimleri Üniversitesi, Þiþli Hamidiye Etfal Eðitim ve Araþtýrma Hastanesi, Genel Cerrahi Kliniði, Ýstanbul

Feokromositoma (FK) ve paraganglioma (PG) adrenal medulla, sempatik veya parasempatik ganglionlardan kaynaklanan nadir nöroendokrin tümörlerdir. Günümüzde FK/PG (FPG) ’nin tek kuratif tedavi seçeneði cerrahi rezeksiyondur. Cerrahi hem hipersekresyon riskini hem de tümör büyümesini ortadan kaldýrmayý amaçlamaktadýr. Hipersekresyonun sonuçlarý cerrahiden önce ve cerrahi sýrasýnda medikal tedavi ile dikkatlice kotrol edilmelidir. Postopertaif major komplikasyonlar hipotansiyon, rebound hipoglisemi olup, hastalar bunun için 24-48 arasý yakýn takip edilmelidir.
Cerrahi yaklaþým tercihi germline genetik mutasyon sonuçlarý, tümör çapý, vücut kitle indeksi, cerrahýn deneyimi, malignite olasýlýðýný içeren multipl faktörler temelinde belirlenir. Primer tümör rezeksiyonu tümör persistan ve reküren riskini tam ortadan kaldýrmaz. Bundan dolayý, cerrahi tedavi edilen FPG’li bütün hastalar reküren hastalýk ve yeni tümör oluþumu için en az 10 yýl takip edilmelidirler. Cerrahi rezeksiyon PPGL'ler için tek küratif tedavi olmasýna raðmen, metastatik hastalýkta rezektabl lokorejyonel metastazlar veya izole uzak metastazlar dýþýnda cerrahi tedavi palyatiftir. Palyatif tedavide amaç hormon salgýlanmasýný azaltmak ve kritik bir anatomik lokalizasyondaki metastaz ile iliþkili komplikasyonlarý önlemektir.
PCC/PGL’li hastalarda genellikle kombine α- ve β- adrenerjik blokaj uygulanmaktadýr. Bazý hastalar, hayatý tehdit eden ve kadiyovasküler, pulmoner, nörolojik, gastrointestinal, renal, hepatik ve metabolik sistemleri tutabilen feokromositoma multisistem krizi ile baþvurabilir. Feokromositoma krizi spontan olabilir veya tümörün manipülasyonu, travma, kortikosteroidler, β blokerler, anestezik ilaçlar gibi deðiþik tedaviler, adrenal cerrahi dýþý cerrahi streslerin uyarýsý ile ortaya çýkabilir. Bu hastalar cerrahi acilden öte medical acil olarak düþünülmelidir.
Bu derlemede, FPG’li hastalarda preoperatif, peroperatif ve postoperatif tedavi, küratif ve palyatif cerrahi tedavi, postoperatif sonuçlar ve takibin deðerlendirilmesi amaçlandý. (SETB-2020-08-137)

Anahtar Kelimeler: pheochromocytoma, paraganglioma, surgical, palliative, systemic treatment, follow up

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