Giant adrenal tumor presenting as Cushing’s syndrome and pheochromocytoma:A case report

Department of Urology,Gauhati Medical College Hospital,Guwahati,Assam,India

Giant adrenal tumor presenting as Cushing’s syndrome and pheochromocytoma:A case report

Puskal Kumar Bagchi*,Somor Jyoti Bora,Sasanka Kumar Barua, Rajeev Thekumpadam Puthenveetil

Department of Urology,Gauhati Medical College Hospital,Guwahati,Assam,India

We report a case of a 35-year-old lady who presented with Cushingoid features and associated raised urinary metanephrine.The patient underwent open adrenelectomy.Histopathological examination revealed adreno-cortical carcinoma with microscopic lymphovascular invasion.Postoperative period was uneventful and is on follow-up for the last one year and is doing well.

Giant adrenal tumor;

Cushing’s syndrome;

Pheochromocytoma

1.Introduction

Adrenal carcinoma is an aggressive malignant neoplasia arising from the adrenal cortex with poor prognosis.It represents 0.02%of all neoplasia.Global incidence is 0.5e2 per every 1,000,000 inhabitants[1].The age distribution is reported as bimodal with a f i rst peak in childhood and a second higher peak in the fourth and f i fth decade[2,3]. Women are more often affected than men in the ratio of 1.5[3e5].Tumors are classif i ed as functioning when they are associated with endocrine manifestations or elevated hormone levels.Cushing’s syndrome is due to hypercortisolism while pheochromocytoma is a catecholamine secreting tumor of the adrenal medulla or extra adrenal sites.Adrenal carcinoma accounts for approximately 33%e 53%of cases of Cushing’s syndrome[6,7].However,it is rare for adrenocortical carcinoma to present clinically as pheochromocytoma.We report a case of a 35-year-old lady who presented with Cushingoid features and associated raised urinary metanephrine.

2.Description of case

A 35 years old female presented with the chief complaint of altered menstrual symptoms for the last 10 months.Shealso complained of dull aching pain in the left f l ank without any radiation or shifting and frequent episodes of generalized headache,palpitation and anxiety for the last 5 months.The palpitation was abrupt in onset and lasts for about 30 min to 1 h,occurs 4e5 times per week,and was associated with day-to-day household activities.The patient gradually developed swelling of both her lower limbs and she had great diff i culty in getting up from the squatting position.On clinical examination,she was found to have chemosis with swelling of eye lids,f l ushing of face, increased facial hair distribution,hypertension,centripetal obesity,and bilateral pedal edema.On examination of her abdomen,a f i rm mass at left hypochondriac region approx 10 cm?7 cm in size was felt with smooth surface and well def i ned margins,and f i ngers could not be insinuated below the costal margin.Laboratory work-ups including full blood count,renal function tests,serum electrolytes,and liver function tests were within normal limit.Serum cortisol [morning e 31.79 mg/dL(normal:4.30e22.40),evening e 32.73 mg/dL(normal:3.09e16.66)]and 24 h-urine norepinephrine e 117.21 mg per 24 h(normal:12.10e85.50), dopamine e 592.82 mg per 24 h(normal:52.00e480.00) levels were raised.CT scan revealed a 18.3 cm?12 cm?16 cm left sided hypervascular retroperitoneal mass without any invasion of the adjacent organs and showing focus of calcif i cation and microscopic fat component (Fig.1).Preoperatively,the patient was placed on a blockers(Tab.Prazosin 5 mg at bedtime)for the effective management of blood pressure.After effective stabilization of blood pressure,she was taken up for exploration.On exploration of abdomen,a left adrenal mass of approx 21 cm?12 cm?8 cm in size,f i xed to left kidney with evidence of local invasion was found(Fig.2).The left adrenal vein was isolated and suture ligated before attempts were made to dissect out the adrenal mass.The mass weighed 380 g(Fig.3).The capsule of the mass was intact with hemorrhagic and necrotic areas seen at places on cross section.Intraoperatively there were f l uctuations of blood pressure,which was managed effectively with Nitroglycerine infusion.Histopathological examination revealed adreno-cortical carcinoma(Mitotic rate 60/50 high power fi eld)with microscopic lymphovascular invasion and invasion limited to the capsule and small vessel.There was extensive tumor necrosis with hemorrhage and calci fi cation (Fig.4).However,no microscopic features suggestive of phaeochromocytoma seen on histopathology of adrenal medulla.Postoperative period was uneventful and is on follow-up for the last one year and is doing well.

3.Discussion

Adrenocortical carcinoma is itself a rare disease,of which functional adrenocortical carcinoma accounts for 50%e79% of cases[8].Rapidly progressing Cushing’s syndrome with or without virilization is the most frequent presentation[9]. Adrenocortical carcinoma can present with dysfunctional uterine bleeding in women due to increased amounts of androstenedione and estrogens[10].However,adrenocortical carcinoma presenting with features of pheochromocytoma alone is a rare entity and it is rarest to have features of both Cushing’s syndrome as well as pheochromocytoma in the same patient with adrenal tumor.Despite extensive PubMed search,no reports of the existence of an adrenal tumor presenting with both features of Cushing’s syndrome as well as pheochromocytoma has been found till date.

4.Conclusion

A functional giant adrenocortical carcinoma with features of both Cushing’s syndrome and pheochromocytoma is a rare entity.Surgical extirpation is a good management option for giant,resectable adrenocortical carcinoma. Precise preoperative work-up and cautious pre-,peri-and postoperative management for the functional component is of utmost importance.A further long stringent follow-up will throw light into the behavior of this entity.

Conf l icts of interest

The authors declare no conf l ict of interest.

[1]National Institutes of Health.NIH state-of-the science statement on management of the clinically inapparent adrenal mass(“incidentaloma”).NIH Consens State Sci Statements 2002;19:1e25.

[2]Wajchenberg B,Albergaria PM,Medonca B,Latronico A, Campos CP,Ferreira AV,et al.Adrenocortical carcinoma: clinical and laboratory observations.Cancer 2000;88:711e36.

[3]Koschker AK,Fassnacht M,Hahner S,Weismann D,Allolio B. Adrenocortical carcinoma improving patient care by establishing new structures.Exp Clin Endocrinol Diabetes 2006;144: 45e51.

[4]Wooten MD,King DK.Adrenal cortical carcinoma.Epidemiology and treatment with mitotane and a review of the literature.Cancer 1993;72:3145e55.

[5]Icard P,Goudet P,Charpenay C,Andreassian B,Carnaille B, Chapuis Y,et al.Adrenocortical carcinomas:surgical trends and results of a 253-patient series from the French Association of Endocrine Surgeons Study Group.World J Surg 2001;25: 891e7.

[6]Ramzi C,Vinay K,Tucker C.Robbins and Cotran Pathologic Basis of Disease.London:WB Saunders;1999.

[7]Clark S,Orlo P,Komminoth P,Roth J,Schroder S.Endocrine tumours.NewYork:AmericanCancerSocietyAtlasof Oncology Series.Decker;2003.

[8]Wein AJ,Kavoussi LR,Novick AC,Partin AW,Peters CA. CampbelleWalsh urology.10th ed.Elsevier Medicine;2011. p.1715.table 57e15.

[9]Bertherat J,Bertagna X.Pathogenesis of adrenocortical cancer.Best Pract Res Clin Endocrinol Metab 2009;23:261e71.

[10]Fauci A,Braunwald E,Kasper D,Hauser S,editors.Harrison’s principles of internal medicine.Philadelphia:Mc Graw Hill; 2008.

Received 27 January 2015;received in revised form 5 May 2015;accepted 13 June 2015 Available online 6 July 2015

*Corresponding author.

E-mail address:puskalbagchi@yahoo.co.in(P.K.Bagchi).

Peer review under responsibility of Shanghai Medical Association and SMMU.

http://dx.doi.org/10.1016/j.ajur.2015.06.007

2214-3882/ª2015 Editorial Off i ce of Asian Journal of Urology.Production and hosting by Elsevier(Singapore)Pte Ltd.This is an open access article under the CC BY-NC-ND license(http://creativecommons.org/licenses/by-nc-nd/4.0/).