The High Frequency of Adenomas in Normal Adrenal Glands Can Make CAT Scans Difficult to Interpret

Pheochromocytoma is a delicate reality to diagnose, indeed when it occurs within the adrenal glands; still, when extra-adrenal pheochromocytomas do arise, it's frequently hard to label them as similar. Pheochromocytomas are most frequently honored due to elevated situations of catecholamines in blood/ urine samples and motorized tomography (CAT) reviews revealing that a mass exists. Pheochromocytomas are generally associated with headaches, sweating, pulsations and most frequently hypertension.

Pheochromocytoma is a delicate reality to diagnose, indeed when it occurs within the adrenal glands; still, when extra-adrenal pheochromocytomas do arise, it's frequently hard to label them as similar. Pheochromocytomas are most frequently honored due to elevated situations of catecholamines in blood/ urine samples and motorized tomography (CAT) reviews revealing that a mass exists. Pheochromocytomas are generally associated with headaches, sweating, pulsations and most frequently hypertension (1- 3).

 Low grade carcinoid excrescences aren't frequently associated with nasty hypertension. They can be set up anywhere in the body, but utmost generally in the small bowel, lung, or pancreas. Some arenon-secretory, but numerous cache active hormones. utmost famously, those tormented with the carcinoid pattern may have flash hypertension, not the type generally associated with pheochromocytomas.

The case, at the age of 53, was admitted to the sanitarium after complaining of briefness of breath and was in respiratory arrest when she arrived. She suffered from Type II grown-up onset diabetes mellitus, habitual obstructive pulmonary complaint( COPD), and a 40- pack- time history of smoking. At the time, she was set up to have pulmonary edema and cardiomyopathy. She was intubated, pulseless, and had a hypertensive extremity with a minimal blood pressure of240/120 mm Hg as she was being revived. She had a 24- hour urine test with metanephrine attention of 316 mcg, normetanephrines of 471 mcg, and total metanephrines of 831 mcg. She also had a 5- HIAA position of12.5 over the same 24- hour period.

To make a opinion of a pheochromocytoma, one or further of the following must be true on 24- hour urine collections

 Norepinephrine> 170 mcg/ 24 hours

 Epinephrine> 35 mcg/ 24 hours

 Dopamine> 700 mcg/ 24 hours

Norepinephrine> 900 mcg/ 24 hours or metanephrine> 400 mcg/ 24 hrs.

Dimension of tube catecholamines isn't accurate, and thus not employed).

She had a workup for a pheochromocytoma due to the high urinary vanillylmandelic acid ( VMA) situations observed, but the tests were negative. A CAT checkup revealed a4.4 x2.5 cm miscellaneous mass in the pulmonary hilum, bordering the descending thoracic aorta sitting roughly 3 cm from the carina. The case passed a vivisection and the mass had an absence of necrosis and presence of lower than 2 mitoses per high power field (HPF); therefore it was felt she had a low grade carcinoid excrescence. Neither low power nor high power views were harmonious with pheochromocytoma. Intraoperatively, the mass was actually intraparenchymal and it involved both lobes. The mass couldn't be removed entirely without a pneumonectomy; as a result, her entire left lung was removed. Pathology was harmonious with a low grade neuroendocrine excrescence.

Figure 1: Hematoxylin and eosin stains showing typical appearance of lowgrade carcinoid excrescence, with trabelulated groups of polygonal cells, high nuclear cytoplasm rate.

Figure 2: Advanced power view (300X).

Figure: Negative staining is also noted for vimentin( 100X).

Both pheochromocytomas and paragangliomas cache catecholamines and metanephrines. Extra-adrenal pheochromocytomas are delicate to diagnose. The high frequence of adenomas in normal adrenal glands can make CAT reviews delicate to interpret. Extra-adrenal pheochromocytomas do and can elevate systemic metanephrine situations, can beget hypertensive heads, tachycardia, or organ damage as a result of hypertensive heads. Due to the oddity of these excrescences, still, they frequently go undiagnosed and/ or misdiagnosed.

The excrescences are frequently only diagnosed rightly after junking. The question becomes, also, can other cases, those with carcinoid excrescences, present in a analogous fashion to those with pheochromocytomas? That is, can they present with the same constellation of symptoms and the same biochemical profile?

Pheochromocytomas are most frequently originally seen on CAT reviews. Previous to surgery, scrupulous control of hypertension both nascence and beta leaguer should be introduced. Long- term control of hypertension, indeed after the excrescence is removed, is occasionally necessary. With antihypertensive treatment the case can continue a normal life free of the numerous anguishing symptoms of the excrescence.

Our case had a pneumonectomy in 2002. She has been under constant surveillance and has had multiple CAT reviews since the operation. She's still entering multiple antihypertensives, but with no substantiation of rush. She's doing relatively well. With no recreating hypertensive heads or elevated metanephrine situations the case is likely cured. The case's excrescence was histologically a low grade carcinoid, though it conducted as if it were a pheochromocytoma or paraganglioma with endocrine and cardiovascular near catastrophes.

A excrescence's presenting position can be deceptive. Our case's excrescence appeared to be a low grade carcinoid forming in the lung, both by position and histologic appearance. still, with respect to clinical geste (nasty hypertension, respiratory failure and cardiac arrest at donation) and biochemical secretory autographs, we feel that this case most likely suffered from an extramedullary pheochromocytoma. Identification, excrescences can be removed with open or laparoscopic ways.   

1. Edstrom Elder E, Hjelm Skog AL, Hoog A and Hamberger B. The management of benign and malignant pheochromocytoma and abdominal paraganglioma. Eur J Surg Oncol. 2003; 29:278-83.  
2. Schlumberger M, Gicquel C, Lumbroso J, Tenenbaum F, Comoy E, Bosq J, Fonseca E, Ghillani PP, Aubert B, and Travagli JP et al. Malignant pheochromocytoma: clinical, biological, histologic and therapeutic data in a series of 20 patients with distant metastases. J Endocrinol Invest. 1992; 15:631-42.
3. Lowery AJ, Walsh S, McDermott EW and Prichard RS. Molecular and therapeutic advances in the diagnosis and management of malignant pheochromocytomas and paragangliomas. Oncologist. 2013; 18:391-407.