A Forgotten cause of Hypertension: Pheochromocytoma

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NHL Journal of Medical Sciences/ July 2012/ Vol. 1/Issue 1

38

Case Report

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Rare Case Report

Dr. Tapan Shah** Dr. Dipen Patel* Dr. Yash Vaidhya* Dr. Jigar Jadeja* Dr. Sharad Patel***Dr. Asit Patel**** Assistant Professor** Resident*Asso.Professor ***Professor and HOD****

Department of General Surgery, Shardaben Hospital, Smt NHL Municipal Medical College, Ahmedabad

Introduction:

Pheochromocytoma is defined as chromaffin cells derived catecholamine-producing tumor of adrenal medulla. A rare tumor with incidence of only 0.1% even which is found incidently, occurs in 0.1-1% of hypertensive patients.

Majority of patients present with classic Triad: Pain (Headache), Perspiration, Palpitations

Pheochromocytoma is with five‟P‟s :

Pressure (Hypertension) Pain (Headache) Perspiration Palpitation Pallor

Abdominal Incidentaloma can be described as adrenal lesions found incidentaly on abdominal laparotomy or during screening without suspecting any adrenal lesion.

Why “10% Tumor”?

 10% extra-adrenal (closer to 15%)

 10% occur in children

 10% familial (closer to 20%)

 10% bilateral or multiple (more if familial)

 10% recur (more if extra-adrenal)

 10% malignant

 10% discovered incidentally

Mainly if familial Pheochromocytoma occurs with any of the following groups:

1. MEN 2 syndromes

2. VonHippel-Lindau‘s Diseases 3. Neurofibromatosis type 1

4. Familial Paraganglioma Syndrome

VMA in an acidic 24 hour Urine collection will confirm the diagnosis of Pheochromocytoma in 95% of cases. High Index of suspicion for diseases should also checked for fractionated Catecholamines and Metanephrines.

CT abdomen (Contrast) :

It is 95% accurate in localizing the tumor, but prior alpha blockers should be given to prevent sudden rise in the blood pressure.

In case of operable tumor (only 10% malignant) preoperative and post operative management is very much important, as Hypertension and Hypoglycemia can lead to lifethretning stage.

MIBG is also one of the useful diagnostic tool.

Case Report: 1

A 22 year old female experienced pain in the right side of the lower abdomen and fullness of the abdomen, with

recurrent first trimester abortions (due to PIH) and presented to the Gynecologist who advised USG and reffered with mass in the Right Lumber Region.

She did not have used any drugs nor there was any past medical history or family history.

On Examination:

Concious, oriented with Pulse: 98/min;

B.P. -170/98 mmof Hg.

General Examination : Pallor ++ S/E:

C.N.S.- concious, oriented. C.V.S. -heart sounds normal R.S. – clear AEEBS

Per Abdomen: Soft,

Tenderness in the Right Lumber Region ++ Mass Palpable in the Right Lumber Region Bowel Sounds ++

Blood Investigations:

Hb: 8.9 gm% Serum Creatinine: 1.9 ng/dl

24 hour Urinary Catecholamines level: VMA- 90 mg/day

USG Abdomen and Pelvis: 30 x 31 mm solid lesion in right perirenal space.

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The Patient was undertaken for surgery on emergency basis with good preoperative control of Blood Pressure and watch on the Blood Sugar level. Right Adrenalectomy done.

Fig.1 Intra-operative Tumor

Case Report :2

A 19 year old female experienced pain in the right side of the lower abdomen and fullness of the abdomen, with

recurrent spurts of rise in B.P. and Headaches and presented to the surgery department and advised routine investigations and CT Abdomen & MIBG.

She did not have used any drugs nor there was any past medical history or family history.

On Examination:

Concious, oriented with Pulse: 98/min;

B.P. -170/98 mmof Hg.

General Examination : Pallor ++ S/E:

C.N.S.- concious, oriented. C.V.S. -heart sounds normal R.S. – clear AEEBS

Per Abdomen: Soft,

Tenderness in the Right Lumber Region ++ Mass Palpable in the Right Lumber Region Bowel Sounds ++

Blood Investigations:

Hb: 9.6 gm% Serum Creatinine: 1.9 ng/dl

MIBG:

Right Adrenal Pheochromocytoma

CT Abdomen:

Heterogeneously enhancing lesion situated at right suprarenal region. Which is medial to IVC supromedial to

right kidney, free from aorta, causing mass effect on IVC. Size of lesion 4.6x 3.3x 5 cm., displacing right renal vein.

The Patient was undertaken for surgery with good preoperative control of Blood Pressure and watch on the Blood Sugar level. Intraoperatively there were many

fluctuations in blood pressure level, and postoperatively there was hypergycemia which was controlled by Insulin and other Oral Hypoglycemics. Right Adrenalectomy done.

Fig. 2 Excised Tumor

Potasium Dichromate test came positive for the chromafffin cells. Histopathological data correlated with Pheochromocytoma as well. Patient is still in follow up and has concieved and has 2 year old child, with controlled hypertension.

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Fig. 4 Pathologic View H&E stain

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Discussion:

The number of incidentally found adrenal lesions is growing. The most important problem in appraising adrenal incidental cases is diagnosing the lesion as benign or malignant. To distinguish malignant and benign lesions, screening methods must be used. CT is an ideal screening method for the adrenal gland and the most helpful technique for distinguishing cysts, bleedings, and myelolipomas. On the other hand, MRI is successful in distinguishing adrenal adenomas, carcinomas, and metastatic masses. But CT has gained widespread acceptance as the technique of choice for distinguishing between adrenal adenomas and metastasis1.

Pheochromocytoma is a rare catecholamine secreting tumor derived from chromaffin cells. It is thought that 5–7 % of functional adrenal incidental masses may be pheochromocytomas. Although pheochromocytoma is not one of the main causes of hypertension, it will be fatal if not controlled. About 10 % of pheochromocytoma cases are diagnosed incidentally during CT or MRI that are

screened for other reasons. Fifty percent of cases are diagnosed by autopsy, and this shows that the disease is seen frequently as well. Sometimes, prediagnosed pheochromocytoma is proposed, but rarely can the diagnosis really be pheochromocytoma. In one series of 300 cases, only one patient had pheochromocytoma. On the other hand, the proportion of malignant pheochromocytoma cases is 2.5–14 %, and these findings are diagnosed with metastases or local invasion.2,3

The literature states that incidental pheochromocytoma cases that are smaller than 1 cm have no clinic symptoms. Rarely, some large pheochromocytoma cases do not show any clinical symptoms. It is difficult to diagnose very small ones. Pheochromocytoma can be diagnosed during investigation of other diseases or adrenal incidentaloma 3,4 One of the important signs of silent pheochromocytomas is the occurrence of hypertension attacks during surgery or anesthesia. Our case diagnosed hypertensive on her visit to hospital5.Additionally, there were no abnormalities in echocardiography or the ECG. Consequently, Pheochromocytoma was not considered.

Pheochromocytoma can be diagnosed by establishing the

increase of catecholamines and metabolites in the plasma and urine. The level of catecholamines and metabolites in the plasma and urine provides 95 % of the evidence for the disease. Free catecholamines (epinephrine or norepinephrine) or metabolites (metanephrine, normetanephrine, or VMA) can be measured in the urine, and the levels of metanephrine or catecholamines can be measured from the plasma. Measurement of metanephrine in the urine is the most useful test. If the level of catecholamines in the urine 2 or 3 times as high as normal, a pheochromocytoma may be present6.

Since the operation material of the patient showed some findings related to pheochromocytoma, the patient was scanned for multiple endocronological neoplasia (MEN) in the postoperative period. Calcitonin and PTH levels detected for screening MEN. No clinical or diagnostic findings have been observed.

All incidental adrenal cases must be examined very carefully, because the chance of mortality is high when they are not diagnosed. Incidental pheochromocytoma cases have nonhomogenic, cystic, and hemoragic images in screening methods. Because our case was

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homogeneous, we did not consider pheochromocytoma as a diagnosis for our case. The image of a bilateral suprarenal incidental lesion is one of the most important symptoms of pheochromocytoma. The image of the left adrenal was normal in our case.

Conclusion :

Pheochromocytoma is a very rare neoplasm of adrenal gland, main stay of management is surgery , but Preoperative and Postoperative control and monitoring of Blood Pressure and Blood Sugar level is the utmost important. Changes unnoticed in these may be life-threatning.

References:

1. Pacak K, Linehan WM, Eisenhofer G, et al.: Recent advances in genetics, diagnosis, localization and treatment of pheochromocytoma. Ann Intern Med 2001, 134:315-20.

2. Goldfien Alan, Medulla Adrenal: Basic and Clinical Endocrinology. 6th edition. Edited by: Greenspan FS, Gardner DG. New York: Lange Medical Boks/Mc Graw-Hill; 2001:400-10.

3. Gross MD, Shapiro B: Clinically silent adrenal masses. J Clin Endocrinol Metab 1993, 77(4):885-8.

4. Kudva YC, Young WF, Thompson GB, et al.: Adrenal incidentaloma: an important component of the clinical presentation spectrum of benign sporadic adrenal pheochromocytoma. The Endocrinologist 1999, 9:77-81.

5. Shin S, Tsujihata N, Miyake O, et al.: Asymptomatic pheochromocytoma: a report of three cases. Hinyokika Kiyo 1994, 40(12):87-91.

6. Baily and Love‘s Short Practice of Surgery, 24th_Edition,