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Table of Contents
CASE REPORT
Year : 2013  |  Volume : 17  |  Issue : 1  |  Page : 160-162

An unusual cause of toe necrosis


1 Department of General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Endocrinology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication27-Feb-2013

Correspondence Address:
Mayur S Bandawar
, Department of General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2230-8210.107872

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   Abstract 

Peripheral vascular disease is a rare feature of pheochromocytoma. This potentially catastrophic but curable tumor should be suspected in combination of distal necrosis with hypertension and palpable pulses. We report such an unusual case of pheochromocytoma presenting as toe necrosis.

Keywords: Catecholamine, peripheral vascular disease, pheochromocytoma, toe necrosis


How to cite this article:
Bandawar MS, Ansari MS, Behera A, Bhadada SK. An unusual cause of toe necrosis. Indian J Endocr Metab 2013;17:160-2

How to cite this URL:
Bandawar MS, Ansari MS, Behera A, Bhadada SK. An unusual cause of toe necrosis. Indian J Endocr Metab [serial online] 2013 [cited 2020 May 29];17:160-2. Available from: http://www.ijem.in/text.asp?2013/17/1/160/107872


   Introduction Top


Peripheral vascular disease (PVD) is a common vascular disease. PVD is commonly associated with absent and/or feeble peripheral pulses. The common causes of PVD are Buerger's disease, atherosclerosis, diabetes, and Raynaud's disease. Pheochromocytoma is associated with various cardiovascular problems like hypertension, cardiomyopathy, myocardial infarction, stroke, [1] and rarely associated with peripheral vascular disease, acute limb ischemia, [2],[3],[4] toe necrosis, [5] and intermittent claudication. [6],[7],[8] If no other signs and symptoms of pheochromocytoma are present, diagnosis is easily overlooked.

We report such a case of pheochromocytoma presenting as toe necrosis.


   Case Report Top


A 50-year-old male presented with history of pain in both lower limbs and ulcer in partially amputated left great toe since 8 months. He had history of pain in both lower limbs with blackish discoloration of left great toe for which he had undergone partial left great toe amputation by local practitioner before 8 months. He was a non-smoker. He had been treated for limb pain symptomatically before being referred to our institute. Before referral to the vascular surgery unit, CT angiography was done which incidentally detected left hypervascular suprarenal mass.

On examination, the patient was found to have tachycardia and hypertension. There were no peripheral signs of pheochromocytoma. Abdominal examination was unremarkable. Pulses were weak in both lower limbs as compared to hand, and great toe was partially amputated with ulcer [Figure 1].
Figure 1: Left great toe partially amputated with an ulcer

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Laboratory data showed hemoglobin (Hb) 11 gm/dl and random blood glucose 121 mg/dl. Coagulation profile and lipid profile were normal. Urine vanillylmandelic acid (VMA) was 10.3 (2-8 mg/24 h), urine epinephrine 16.8 (0-20 mg/24 h), urine norepinephrine 133.4 (15-80 mg/24 h), and serum cortisol after overnight dexamethasone suppression test was 46.72 (171-536 nmol/l).

CT angiography revealed relatively delayed runoff in lower limb arteries with marginally reduced caliber in right lower limb arteries and hypervascular left suprarenal mass suggestive of pheochromocytoma. Contrast-enhanced computed tomography (CECT) abdomen revealed a 5.2 × 3.5 × 5.8 cm hypervascular mass lesion replacing left adrenal, suggestive of pheochromocytoma [Figure 2].
Figure 2: Contrast-enhanced computed tomography abdomen revealed a 5.2 × 3.5 × 5.8 cm hypervascular mass lesion replacing left adrenal, suggestive of pheochromocytoma

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Patient was started on antihypertensive. After adequate alpha and beta blockade, he underwent left adrenalectomy [Figure 3]. Histological examination showed a well-circumscribed and encapsulated tumor with monomorphic cells arranged in alveolar and nesting pattern, separated by thin vascularized septae. Individual cells were monomorphic with round nuclei, granular chromatin, inconspicuous nucleoli, and moderate to abundant granular cytoplasm. The features were suggestive of a pheochromocytoma.
Figure 3: (a) Gross appearance of left suprarenal mass and (b) its cut section

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Postoperatively, his BP was normal. On follow-up, his toe ulcer healed completely.


   Discussion Top


Classical clinical triad of pheochromocytoma is formed by tachycardia, sweating, and headache. In addition to the classical triad, pheochromocytoma is commonly associated with hypertension, cardiomyopathy, myocardial infarction, and cerebrovascular accident. [1] Rarely, pheochromocytoma is also associated with peripheral vascular disease with intermittent claudication, non-healing ulcers, and necrosis of toes. Our patient presented with non-healing ulcer over partially amputated left great toe.

High levels of catecholamine production may be the pathologic mechanism causing extreme vasoconstriction or diffuse arterial vasospasm and critical peripheral ischemia. [1],[3],[5],[8],[9],[10] Significant negative correlation between plasma catecholamine concentration and skin blood flow has been demonstrated in pheochromocytoma. [10],[11] Resolution of ischemia and wound healing after removal of pheochromocytoma confirms the suggestion that excess catecholamine was responsible for the development of critical toe ischemia in our patient.

High index of suspicion and timely diagnosis is extremely important, as these tumors are curable by surgical removal, and any surgery in a patient with unsuspected pheochromocytomas carries high risk of morbidity and mortality.

In conclusion, we report an unusual case of pheochromocytoma presenting as toe ischemia and necrosis, and hypertension that was unsuspected because of rarity of the tumor presenting as toe necrosis. Diagnosis is mainly clinical. This potentially catastrophic but curable tumor should be suspected in combination of distal necrosis with hypertension and palpable pulses.

 
   References Top

1.Brouwers FM, Lenders JW, Eisenhofer G, Pacak K. Pheochromocytoma as an endocrine emergency. Rev Endocr Metab Disord 2003;4:121-8.  Back to cited text no. 1
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2.Engelman K, Horwitz D, Jaquier E, Sjoerdsma A. Biochemical and pharmacological effects of a-metyltyrosine in man. J Clin Invest 1968;47:577-94.  Back to cited text no. 2
    
3.Muehrcke DD, Bliss BP. Early presentation of pheochromocytoma as acute arterial disease. Br J Hosp Med 1988;39:534-5.  Back to cited text no. 3
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4.Borregana J, Lima RM, Oliveira E, Amador MG, Bicho M, Dias CA. Acute limb ischemia as manifestation of a pheochromocytoma. J Cardiovasc Surg (Torino) 1984;25:179-81.  Back to cited text no. 4
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5.Bessis D, Dereure O, Le Quellec A, Ciurana AJ, Guilhou JJ. Pheochromocytoma manifesting as toe necrosis. Ann Dermatol Venerol 1998;125:185-7.  Back to cited text no. 5
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6.Scharf Y, Nahir M, Plavnic Y, Ben-Arieh J, Lichting C, Gellei B. Intermittent claudication with pheochromocytoma. JAMA 1971;215:1323-4.  Back to cited text no. 6
    
7.Krane NK. Clinically unsuspected pheochromocytomas. Experience at Henry Ford Hospital and a review of the literature. Arch Intern Med 1986;146:54-7.  Back to cited text no. 7
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8.Tack CJ, Lenders JW. Pheochromocytoma as a cause of blue toes. Arch Intern Med 1993;153:2061.  Back to cited text no. 8
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9.Radtke WE, Kazmier FJ, Rutherford BD, Sheps SG. Cardiovascular complications of pheochromocytoma crisis. Am J Cardiol 1975;35:701-5.  Back to cited text no. 9
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10.Balbir-Gurman A, Nahir AM, Rozin A, Markovitz D, Braun- Moscovici Y. Toe necrosis and acute myocardial infarction precipitated by a pheochromocytoma in a patient with systemic sclerosis. J Clin Rheumatol 2007;13:331-3.  Back to cited text no. 10
    
11.Mikasa H, Sakuragi T, Higa T, Yasumoto M. Skin blood flow and plasma catecholamine concentrations during removal of a pheochromocytoma in a child. Br J Anaesth 2004;92:757-60.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


This article has been cited by
1 Cardiovascular Manifestations of Pheochromocytoma
Yu Wei Gu,Jennifer Poste,Mehta Kunal,Monica Schwarcz,Irene Weiss
Cardiology in Review. 2017; 25(5): 215
[Pubmed] | [DOI]



 

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