open access
Epic battles in endocrinology — malignant pheochromocytoma: a case report
, 1, 2, 2, 2, 3, 4
Affiliations- Department of Endocrinology and Internal Medicine, Medical University of Gdańsk, Gdańsk, Poland
- Department of Endocrinology and Internal Medicine, Medical University of Gdansk, Poland
- Independent Clinical Practice, Sopot, Poland
- Department of Radiology, University of Warmia and Mazury, Olsztyn, Poland
open access
Abstract
The rarity of malignant pheochromocytoma coupled with the lack of definitive predictors of malignancy and the variability of clinical course, poses a significant diagnostic and therapeutic challenge. Since data on treatment is so scarce, case reports are a valuable source of knowledge for clinicians. This case report describes the medical history of a woman, aged 51 at the time of initial diagnosis and adrenalectomy.
Within over 5 years she presented with recurrent relapse of tumour in adrenal gland bed and multiple distant metastases to descending colon, abdominal wall, postoperative scars, and the peritoneum. Neither before diagnosis nor during the whole follow-up were symptoms associated with pheochromocytoma present.
The treatment administered to our patient consisted of numerous debulking surgeries along with administration of both hot and cold somatostatin analogues. We believe that debulking surgeries played a substantial role in enabling the patient to survive nearly 6 years despite aggressive clinical course of pheochromocytoma. She passed away in 2012 as a result of postlaparotomy complications.
We stress the role of debulking surgery in the treatment of malignant pheochromocytoma and summarise current
literature.
Abstract
The rarity of malignant pheochromocytoma coupled with the lack of definitive predictors of malignancy and the variability of clinical course, poses a significant diagnostic and therapeutic challenge. Since data on treatment is so scarce, case reports are a valuable source of knowledge for clinicians. This case report describes the medical history of a woman, aged 51 at the time of initial diagnosis and adrenalectomy.
Within over 5 years she presented with recurrent relapse of tumour in adrenal gland bed and multiple distant metastases to descending colon, abdominal wall, postoperative scars, and the peritoneum. Neither before diagnosis nor during the whole follow-up were symptoms associated with pheochromocytoma present.
The treatment administered to our patient consisted of numerous debulking surgeries along with administration of both hot and cold somatostatin analogues. We believe that debulking surgeries played a substantial role in enabling the patient to survive nearly 6 years despite aggressive clinical course of pheochromocytoma. She passed away in 2012 as a result of postlaparotomy complications.
We stress the role of debulking surgery in the treatment of malignant pheochromocytoma and summarise current
literature.
Keywords
malignant pheochromocytoma; debulking surgery; case report; lanreotide
About this articleTitle
Epic battles in endocrinology — malignant pheochromocytoma: a case report
Journal
Issue
Article type
Case report
Pages
38-44
Published online
2020-01-22
Page views
785
Article views/downloads
799
DOI
Bibliographic record
Arterial Hypertension 2020;24(1):38-44.
Keywords
malignant pheochromocytoma
debulking surgery
case report
lanreotide
Authors
Elżbieta Zych
Aleksandra Jaruszewska
Łukasz Obołończyk
Piotr Wiśniewski
Krzysztof Sworczak
Katarzyna Chełmińska
Jarosław Ćwikła
References (28)- Lenders JWM, Duh QY, Eisenhofer G, et al. Endocrine Society. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014; 99(6): 1915–1942.
- Wan WH, Tan KY, Ng C, et al. Metastatic malignant phaeochromocytoma: A rare entity that underlies a therapeutic quandary. Asian J Surg. 2006; 29(4): 294–302.
- Baudin E, Habra MA, Deschamps F, et al. Therapy of endocrine disease: treatment of malignant pheochromocytoma and paraganglioma. Eur J Endocrinol. 2014; 171(3): R111–R122.
- Giubellino A, Sourbier C, Lee MJ, et al. Targeting heat shock protein 90 for the treatment of malignant pheochromocytoma. PLoS One. 2013; 8(2): e56083.
- Sołtysiak M, Gołofit P, Łokaj M, et al. Biochemically silent pheochromocytoma — rare, but not uncommon. Case study. Art Hyperten. 2015; 19(4): 200–202.
- Januszewicz W, Jarząb B, Januszewicz A, et al. Malignant pheochromocytoma. Nadciśnienie Tętnicze. 2005; 9(2): 132–140.
- Yu J, Pacak K. Management of Malignant Pheochromocytoma. The Endocrinologist. 2002; 12(4): 291–299.
- Adjallé R, Plouin PF, Pacak K, et al. Treatment of malignant pheochromocytoma. Horm Metab Res. 2009; 41(9): 687–696.
- Zukauskaite R, Hjorthgau K, Poulsen PL, et al. Malignant pheochromocytoma and paraganglioma: three cases illustrating the use of molecular targeted diagnostics and therapy and possible role of new drugs. Acta Oncol. 2011; 50(8): 1255–1259.
- Tanabe A, Naruse M, Nomura K, et al. Combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine in patients with malignant pheochromocytoma and paraganglioma. Horm Cancer. 2013; 4(2): 103–110.
- Huang H, Abraham J, Hung E, et al. Treatment of malignant pheochromocytoma/paraganglioma with cyclophosphamide, vincristine, and dacarbazine: recommendation from a 22-year follow-up of 18 patients. Cancer. 2008; 113(8): 2020–2028.
- Watanabe D, Tanabe A, Naruse M, et al. Transcatheter arterial embolization for the treatment of liver metastases in a patient with malignant pheochromocytoma. Endocr J. 2006; 53(1): 59–66.
- Kim JK, Kim BH, Baek SM, et al. Incidentally detected inoperable malignant pheochromocytoma with hepatic metastasis treated by transcatheter arterial chemoembolization. Endocrinol Metab (Seoul). 2014; 29(4): 584–589.
- Edström Elder E, Hjelm Skog AL, Höög A, et al. The management of benign and malignant pheochromocytoma and abdominal paraganglioma. Eur J Surg Oncol. 2003; 29(3): 278–283.
- Jimenez C, Rohren E, Habra MA, et al. Current and future treatments for malignant pheochromocytoma and sympathetic paraganglioma. Curr Oncol Rep. 2013; 15(4): 356–371.
- Babinska A, Peksa R, Sworczak K. Primary malignant lymphoma combined with clinically "silent" pheochromocytoma in the same adrenal gland. World J Surg Oncol. 2015; 13: 289.
- Januszewicz W, Prejbisz A, Januszewicz A, et al. Pheochromocytoma — Disease that Can Stimulate Many Different Syndromes. Nadciśnienie Tętnicze. 2002; 6(3): 217 –228.
- López-Gómez J, Salazar-Álvarez MaA, Adame RY, et al. Metastatic pheochromocytoma to liver without elevation of metanephrines and catecholamines. Int J Surg Case Rep. 2016; 29: 71–75.
- Arnas-Leon C, Sánchez V, Santana Suárez AD, et al. Complete Remission in Metastatic Pheochromocytoma Treated with Extensive Surgery. Cureus. 2016; 8(1): e447.
- Favier J, Igaz P, Burnichon N, et al. Rationale for anti-angiogenic therapy in pheochromocytoma and paraganglioma. Endocr Pathol. 2012; 23(1): 34–42.
- Sun FK, He HC, Su TW, et al. Multi-targeted tyrosine kinase inhibitor sunitinib: a novel strategy for sporadic malignant pheochromocytoma. Chin Med J (Engl). 2012; 125(12 ): 2231–2234.
- Hata J, Haga N, Ishibashi K, et al. Sunitinib for refractory malignant pheochromocytoma: two case reports. Int Urol Nephrol. 2014; 46(7): 1309–1312.
- Rose B, Matthay KK, Price D, et al. High-dose 131I-metaiodobenzylguanidine therapy for 12 patients with malignant pheochromocytoma. Cancer. 2003; 98(2): 239–248.
- Rutherford MA, Rankin AJ, Yates TM, et al. Management of metastatic phaeochromocytoma and paraganglioma: use of iodine-131-meta-iodobenzylguanidine therapy in a tertiary referral centre. QJM. 2015; 108(5): 361–368.
- Lam MG, Lips CJM, Jager PL, et al. Repeated [131I]metaiodobenzylguanidine therapy in two patients with malignant pheochromocytoma. J Clin Endocrinol Metab. 2005; 90(10): 5888–5895.
- Gedik GK, Hoefnagel CA, Bais E, et al. 131I-MIBG therapy in metastatic phaeochromocytoma and paraganglioma. Eur J Nucl Med Mol Imaging. 2008; 35(4): 725–733.
- Chrisoulidou A, Kaltsas G, Ilias I, et al. The diagnosis and management of malignant phaeochromocytoma and paraganglioma. Endocr Relat Cancer. 2007; 14(3): 569–585.
- Lamarre-Cliche M, Gimenez-Roqueplo AP, Billaud E, et al. Effects of slow-release octreotide on urinary metanephrine excretion and plasma chromogranin A and catecholamine levels in patients with malignant or recurrent phaeochromocytoma. Clin Endocrinol (Oxf). 2002; 57(5): 629–634.