**4.1 Elements of surgical technique and tactics**

The operating table must benefit from adequate mobility in order to allow an adequate angulation in order to optimally open the anatomical space from the costal rim to the iliac crest. The operating surgeon and the cameraman are positioned in front of the patient and the assistant on the opposite side. The patient can be placed on the operating table in a lateral decubitus with a 90-degree angle position to ensure the full retraction of the spleen and a partial retraction of the liver by the gravity force.

At the time of pneumoperitoneum installation, the patient is already lying on his side. The Veress needle will be inserted through the abdominal flank, being warned of the risk of visceral (liver or colon on the right side) or vascular (epigastric pedicle branches) injuries. Classic, there are three subcostal ports that can be positioned on the left and one epigastric port that is localized at the inferior margin of the liver. It is not unusual for the first trocar (corresponding to the left mid-clavicular line) to be inserted just a little bit slower than the other three subcostal trocars in order to offer a comprehensive view of the operative field and to identify the entire trajectory of the left colonic flexure (to facilitate the dissection of the colonic ligament for a lower positioning of the colon and to offer a better view over the left adrenal lodge).

For the left adrenalectomy, the spleen can be mobilized till the gastric fundus appears in the visual field, this can allow the spleen to retract more medially. This maneuvers can develop the plane between the spleen and the pancreas' tail, up to the left adrenal gland. The first maneuver is the incision made at the later splenic peritoneum. Further dissection is performed in a relatively avascular plane that is located close to the retropancreatic and anterior to the adrenal and renal capsule. Through this conjunctive structure's transparency, the adrenal tissue specific aspect is easily distinguished as having a specific yellowish color.

Sometimes, it is mandatory to cut the conjunctive tissue between the left colonic flexure (at its highest point) to the abdominal wall and to re-positioning the colon down below the level of the inferior pole of the spleen. After that, a broad view of this area is noticed. More than that, an easy discovery of the kidney superior left pole is possible, and this should be the start to identify (especially on obese patients) the groove between the left kidney and the pancreas – the normal localization of the left adrenal gland. For a pheochromocytoma dissection, the surgical gestures should be delicate, precise, and firm – a proper instrument of dissection should be used (sealing-cutting device, ultrasound device – electrocautery hook is to be avoided by the beginners and is a time-consuming device for an experienced surgeon).

The essential vascular anatomical elements that will be treated in left adrenalectomy are the central vascular pedicle and the upper vascular pedicle. The left central vein (LCV) goes into the left renal vein, most commonly into the common trunk with the lower-left diaphragmatic vein (LLDV), from which it should be disected before injuring or clipping. The sectioning of this common

trunk interpreted as LCV will result in the cranial interception of LLDV; a vascular element misinterpreted as an accessory left central vein. The LCV must be doubleclipped to the renal vein, for security reasons, and then sectioned with sharp surgical instrument – the latest surgical devices (like a sealing-cutting instrument with a computerized chip for measuring the impedance and secure the ligation) can be safely used with no clips (depending on surgeon's experience). The left middle adrenal artery comes from the aorta and will be highlighted later. It can be treated by titanium clip or bipolar electrosurgery. By following the upper margin of the adrenal gland, the upper adrenal pedicle branches will be highlighted and treated using an electrocoagulation procedure.

The complete disection of the entire adrenal gland is done by posterior dissection, in a very loose anatomical space, after witch is then separated from the upper renal pole and the muscular abdominal wall (up to the left quadratus lumborum muscle). The final steps of left laparoscopic adrenalectomy are made by the hemostasis, surgery gland extraction and local drainage.

Thoroughly attention should be given to the dissection of the superior part of the adrenal gland near the pancreas' inferior edge because important vessels are lying over there – including end-parts of the splenic vein and the veins' and arteries' network around the pancreatic tail. Finally, pancreatic parenchyma should be carefully avoided to be damaged during dissection to skip a postoperative pancreatic leakage.

For laparoscopic right adrenalectomy, four working trocars (3 of 10 mm and one of 5 mm) are required, arranged on a line parallel to the costal margin, developed between the sub-xiphoid region and the right anatomical flank (below the tip of the right tenth rib). Near the right kidney's upper pole covered by the right hepatic lobe can be found the the right adrenal gland, lying on the diaphragm, in close contact with the IVC. The right triangular ligament is disected, and the right liver lobe is then retracted with the instrument through the epigastric trocar. Displacemant of the right hepatic lobe depends a lot on obtaining a good and comfortable access in the adrenal gland's dissection space. The lifting of the hepatic love is performed by an atraumatic instument, of "snake" type or with atraumatic blades, inserted in the sub-xiphoid trocar. In the particular situation of a highly developed right hepatic lobe, additional maneuvers or additional tools are required to obtain a suitable elevation. A slightly reverse-Trendelenburg position also obtains a better position of the patient on the operating table. These exposure gestures are critical to prevent the application of excessive forces intended to widen the operating field. These traumatic maneuvers can lead to damage to the Glisson capsule and tears of the liver parenchyma. Consecutive bleeding significantly alters the dissection conditions, reducing the surgeon's ability to distinguish the specific appearance of the gland. The crimson-yellow specific gland color is easy to recognise under proper conditions of dissection. The anterior plane of the gland can be exposed by performing a blunt dissection or with the electrocautery. This can be performed in an extracapsular plane, anatomical space occupied by many loose and avascular lax fibrous tissue. The disection plane found between IVC and the right adrenal gland can be exposed. This will be the starting point of the surgical excision of a pheochromocytoma – the surgeon should prioritize the "attack" of the central vein expecially for the right adrenal gland. The arterial sources that approach the gland are anastomosing each other in the next presented way: first the central artery that arises from the aorta, second, the superior arterial pedicle that arises from the right inferior diaphragmatic artery and not the last, the inferior arterial pedicle from the right renal artery. Venous blood can be collected by the homonymous satellite veins, represented by the central vein that is collected directly into the IVC that should be well identified before any maneuver on the adrenal vessels. Any bloody source can

**53**

excellent hemostasis [4].

*Surgical Approach in Pheochromocytoma DOI: http://dx.doi.org/10.5772/intechopen.96066*

superior margin of the gland [4, 5].

hepatic lobe is placed for 24–48 hours [4].

emphasized.

be solved by IVC partial clamping only if it is exposed and have a good control. It is mandatory for a safe treatment of the vascular pedicles and for the achievement of complete glandular removal to have a complete exposure of the entire medial and

The central vein can be clipped twice at the inferior vena cava margin and with a single clip to the margin of the gland. In approximately 20% of patients, there can be found a central accessory vein, sometimes this is the cause of difficult to control hemorrhage. Due to the lateral decubitus position, after dissecting the central vein, the IVC is mobilized to the medial, and the retro-IVC extension of the gland can be much easily dissected. The central artery can be dissected and sectioned by the branches with electrocautery. Modern means of electrosurgery (sealingcutting devices or ultrasonic scalpel) offers additional operating comfort and efficiency, but the need to continuously follow the correct anatomical plans must be

A complete mobilization of the gland is achieved by posterior dissection, to or from the side, in a loose fibrous anatomical space which is interposed between the renal upper pole and diaphragm until the aspect of the right quadratus lumborum muscle appears in the operating field. It is mandatory not to touch the adrenal tissue, especially the pheochromocytoma tumor, to avoid spillage the tumor cells in the peritoneal cavity. The piece will be placed in a bag and is extracted by widening one of the access wall brackets (usually the one on the right anterior axillary line). After a thorough control of the hemostasis, a drainage tube beneath the right

For situations in which bilateral excision is required in the same operating session, the patient's position will be changed after removing one of the glands, the

Although this direction is time-consuming, involves the anesthetic-surgical team's synchronized effort, determines an increased consumption of materials by reorganizing the operating field, the benefit offered by the advantages of the transperitoneal lateral approach is entirely found in operating comfort [6, 7].

For cortical sparing/preservation technique, once the supraadrenal glands are exposed by the mobilization and dissection of the triangular hepatic ligament on the right side and the spleen on the left side, a mass can be seen in the retroperitoneum. Using the best imaging by ultrasound technique to identify the necessary anatomy, surgeons should try to preserve the adrenal veins to allow proper function of the adrenal remnants. It is recomanded to perform an ultrasound examination to assess relationship between the tumor and the adrenal veins and determin whether there are more nodules in patients with genetic predisposition. If there is only one nodule, a harmonic scalpel can be used to remove the nodule. Since the blood vessels of the adrenal gland are highly vigorous, harmonic activation is performed while the jaws are opened, and the adrenal tissue is slowly compressed to provide

Do not use any device to grab the nodule or adrenal gland in any position. Grasping the nodule or the adrenal glands with any device may cause a rupture that lead to pheochromocytomatosis. Grasping a portion of adipose tissue located near the adrenal glands or attached to it, or using gentle retraction will give enucleation appropriate exposure. After the nodule is removed, the remaining adrenal glands should be checked for hemostasis. For multiple nodules suitable for removal, the surgeon should consider proceed as before. However, if there are multiple nodules and the restant gland is less than 30% of the total gland, the surgeon should consider total adrenalectomy. The remaining glands may lose function, and the patient may benefit from total adrenalectomy to avoid the need for reoperation of relapse. After the tumor is removed, put the specimen in the bag and take it out [4–7].

entire device is reset to perform the contralateral adrenalectomy.

#### *Surgical Approach in Pheochromocytoma DOI: http://dx.doi.org/10.5772/intechopen.96066*

*Pheochromocytoma, Paraganglioma and Neuroblastoma*

treated using an electrocoagulation procedure.

stasis, surgery gland extraction and local drainage.

trunk interpreted as LCV will result in the cranial interception of LLDV; a vascular element misinterpreted as an accessory left central vein. The LCV must be doubleclipped to the renal vein, for security reasons, and then sectioned with sharp surgical instrument – the latest surgical devices (like a sealing-cutting instrument with a computerized chip for measuring the impedance and secure the ligation) can be safely used with no clips (depending on surgeon's experience). The left middle adrenal artery comes from the aorta and will be highlighted later. It can be treated by titanium clip or bipolar electrosurgery. By following the upper margin of the adrenal gland, the upper adrenal pedicle branches will be highlighted and

The complete disection of the entire adrenal gland is done by posterior dissection, in a very loose anatomical space, after witch is then separated from the upper renal pole and the muscular abdominal wall (up to the left quadratus lumborum muscle). The final steps of left laparoscopic adrenalectomy are made by the hemo-

Thoroughly attention should be given to the dissection of the superior part of the adrenal gland near the pancreas' inferior edge because important vessels are lying over there – including end-parts of the splenic vein and the veins' and arteries' network around the pancreatic tail. Finally, pancreatic parenchyma should be carefully avoided to be damaged during dissection to skip a postoperative pancreatic

For laparoscopic right adrenalectomy, four working trocars (3 of 10 mm and one of 5 mm) are required, arranged on a line parallel to the costal margin, developed between the sub-xiphoid region and the right anatomical flank (below the tip of the right tenth rib). Near the right kidney's upper pole covered by the right hepatic lobe can be found the the right adrenal gland, lying on the diaphragm, in close contact with the IVC. The right triangular ligament is disected, and the right liver lobe is then retracted with the instrument through the epigastric trocar. Displacemant of the right hepatic lobe depends a lot on obtaining a good and comfortable access in the adrenal gland's dissection space. The lifting of the hepatic love is performed by an atraumatic instument, of "snake" type or with atraumatic blades, inserted in the sub-xiphoid trocar. In the particular situation of a highly developed right hepatic lobe, additional maneuvers or additional tools are required to obtain a suitable elevation. A slightly reverse-Trendelenburg position also obtains a better position of the patient on the operating table. These exposure gestures are critical to prevent the application of excessive forces intended to widen the operating field. These traumatic maneuvers can lead to damage to the Glisson capsule and tears of the liver parenchyma. Consecutive bleeding significantly alters the dissection conditions, reducing the surgeon's ability to distinguish the specific appearance of the gland. The crimson-yellow specific gland color is easy to recognise under proper conditions of dissection. The anterior plane of the gland can be exposed by performing a blunt dissection or with the electrocautery. This can be performed in an extracapsular plane, anatomical space occupied by many loose and avascular lax fibrous tissue. The disection plane found between IVC and the right adrenal gland can be exposed. This will be the starting point of the surgical excision of a pheochromocytoma – the surgeon should prioritize the "attack" of the central vein expecially for the right adrenal gland. The arterial sources that approach the gland are anastomosing each other in the next presented way: first the central artery that arises from the aorta, second, the superior arterial pedicle that arises from the right inferior diaphragmatic artery and not the last, the inferior arterial pedicle from the right renal artery. Venous blood can be collected by the homonymous satellite veins, represented by the central vein that is collected directly into the IVC that should be well identified before any maneuver on the adrenal vessels. Any bloody source can

**52**

leakage.

be solved by IVC partial clamping only if it is exposed and have a good control. It is mandatory for a safe treatment of the vascular pedicles and for the achievement of complete glandular removal to have a complete exposure of the entire medial and superior margin of the gland [4, 5].

The central vein can be clipped twice at the inferior vena cava margin and with a single clip to the margin of the gland. In approximately 20% of patients, there can be found a central accessory vein, sometimes this is the cause of difficult to control hemorrhage. Due to the lateral decubitus position, after dissecting the central vein, the IVC is mobilized to the medial, and the retro-IVC extension of the gland can be much easily dissected. The central artery can be dissected and sectioned by the branches with electrocautery. Modern means of electrosurgery (sealingcutting devices or ultrasonic scalpel) offers additional operating comfort and efficiency, but the need to continuously follow the correct anatomical plans must be emphasized.

A complete mobilization of the gland is achieved by posterior dissection, to or from the side, in a loose fibrous anatomical space which is interposed between the renal upper pole and diaphragm until the aspect of the right quadratus lumborum muscle appears in the operating field. It is mandatory not to touch the adrenal tissue, especially the pheochromocytoma tumor, to avoid spillage the tumor cells in the peritoneal cavity. The piece will be placed in a bag and is extracted by widening one of the access wall brackets (usually the one on the right anterior axillary line). After a thorough control of the hemostasis, a drainage tube beneath the right hepatic lobe is placed for 24–48 hours [4].

For situations in which bilateral excision is required in the same operating session, the patient's position will be changed after removing one of the glands, the entire device is reset to perform the contralateral adrenalectomy.

Although this direction is time-consuming, involves the anesthetic-surgical team's synchronized effort, determines an increased consumption of materials by reorganizing the operating field, the benefit offered by the advantages of the transperitoneal lateral approach is entirely found in operating comfort [6, 7].

For cortical sparing/preservation technique, once the supraadrenal glands are exposed by the mobilization and dissection of the triangular hepatic ligament on the right side and the spleen on the left side, a mass can be seen in the retroperitoneum. Using the best imaging by ultrasound technique to identify the necessary anatomy, surgeons should try to preserve the adrenal veins to allow proper function of the adrenal remnants. It is recomanded to perform an ultrasound examination to assess relationship between the tumor and the adrenal veins and determin whether there are more nodules in patients with genetic predisposition. If there is only one nodule, a harmonic scalpel can be used to remove the nodule. Since the blood vessels of the adrenal gland are highly vigorous, harmonic activation is performed while the jaws are opened, and the adrenal tissue is slowly compressed to provide excellent hemostasis [4].

Do not use any device to grab the nodule or adrenal gland in any position. Grasping the nodule or the adrenal glands with any device may cause a rupture that lead to pheochromocytomatosis. Grasping a portion of adipose tissue located near the adrenal glands or attached to it, or using gentle retraction will give enucleation appropriate exposure. After the nodule is removed, the remaining adrenal glands should be checked for hemostasis. For multiple nodules suitable for removal, the surgeon should consider proceed as before. However, if there are multiple nodules and the restant gland is less than 30% of the total gland, the surgeon should consider total adrenalectomy. The remaining glands may lose function, and the patient may benefit from total adrenalectomy to avoid the need for reoperation of relapse. After the tumor is removed, put the specimen in the bag and take it out [4–7].

Laparoscopic adrenalectomy has been found to reduce the need for hospitalization, blood transfusion, postoperative analgesia and recovery. Laparoscopic adrenalectomy is more difficult to perform on the right side because of the exposure problems, the proximity of the gland to the IVC and the short right adrenal vein. Although there are more and more reports of laparoscopic resection of pheochromocytoma, but it should not be considered for malignant pheochromocytomas or tumors greater than 8 cm [2].

#### **4.2 Posterior retroperitoneal laparoscopic approach**

Place the patient in a jack-knife position and place a 1.5–2 cm transverse incision under the terlfth rib to access the retroperitoneum space. Digital palpation can be used to develop and disecting spaces. Guided by the surgeon's index finger, place another trocar along the lateral border of the paraspinous muscle. Similarly, place a side needle under the eleventh rib. Insert another 12 mm blunt balloon trocar into the first incision. When pneumoperitoneum is established, carbon dioxide needs to be injected and kept at 20–24 mmHg. The working space of superior border of the kidney is developed by dissecting the retroperitoneal areolar tissue and Gerota's fascia. After identifying the adrenal veins, dissect the upper adrenal glands laterally and inferiorly, and finally dissect the upper and mid-medial glands. Identify, dissect and remove the adrenal veins between the clamps. The adrenal gland is firstly dissected laterally and inferiorly and secondly the superior and medial adesions are dissected with the evidentiation of the adrenal vein. The adrenal vein is identified, dissected, and resected between clips. The next step is the dissection of the upper attachment, and then put the specimen into the Endo bag device and removed it from the abdomen [2].

#### **4.3 Open techniques**

Multiple incisions can be used to exposure the anatomy, including subcostal, midline, and the Makuuchi incision. The aim is to use subcostal incision two to three cm below the costal margin. This incision provides excellent liver and adrenal bed exposure. If a lymph node dissection is planned, the subcostal incision will provide the space betwen aorta and inferior vena cava and between the lymph nodes around the hilum. As with the laparoscopic technique, the triangular ligament on the right is also cut, and the spleen on the left is moved into the retroperitoneal cavity. Very important anatomical landmarks such as inferior vena cava, must be marked on the right, and the tail of the pancreas and the loose plane between the tail and the left adrenal gland on the left must be visualized. For boths sides, the adrenal veins must be identified and cut. Split all remaining attachments and remove the gland. The open transabdominal or thoracoabdominal approaches give the best exposure for resecting extensive tumors, for bilateral adrenalectomy or for metastatic disease [3].

#### **5. Perioperative/postoperative management**

Following surgical approach of pheochromocytoma, 80% of patients are suspected to be again normotensive. Persistent postoperative hypertension can be caused by the residual tumor, intraoperative injury to the kidney's renal artery or metastatic disease.

Intraoperative hypotension may be caused by (1) hemorrhage during surgery (2) insufficient hormone replacement after left and right adrenal excision (3) vascular

**55**

*Surgical Approach in Pheochromocytoma DOI: http://dx.doi.org/10.5772/intechopen.96066*

perfusions and vasopressors [3].

**5.1 Postoperative outcomes**

different procedures [18].

**6. Particular situations**

**6.1 Malignant pheochromocytoma**

fails to respond to sufficient volume replacement [16].

elective method for patients with VHL syndrome [19].

compliance changes and immediate reduction of catecholamine levels after tumor removal, (4) myocardial infarction (5) long-term residual effects of prolonged α-blockers before surgery. As shown, hypotension can be effectively controlled by replacing volume and blood transfusion. Use vasopressors only when hypotension

Postoperatively, the majority of patients that had an uneventful intraoperative course with or without hemodynamic instability do not necessitate intensive care supervision. In the first postoperative period, the patients that have pheochromocytoma have also a greater risk of hypoglycemia and hypotension [5]. After catecholamines are suddenly stopped, due to the relative increase in insulin sensitivity, hypoglecemia may occur. Therefore, blood glucose must be supervised every hour for the first three or four hours postoerative. When a total adrenalectomy is planned, glucocorticoid preparations can be taken before surgery, specifically for the patients that have bilateral tumors or a familial pheochromocytoma syndrome. After surgery,incidence of hypoglycemia droped from 15% to 4.2%. Independent predictors related to postoperative hypoglycemia include prolonged operation time and increased urine adrenaline in 24-hour period before surgery. Therefore, glicemia must be checked regularly in the first day after surgery. Isolated episodes of hypotension are very common and can be attribueted to the preoperative alpha blockade residual values, hypovolemia that can be due to intraoperative blood loss or/and preoperative volume contract,. Treatment must be aggressive in terms of IV

The rate of morbidity in untreated pheochromocytoma is very high and difficult to determin. 71% of patients can die from cardiovascular causes: myocardial infarction, hypertensive heart insuiciency, or hemodynamic instability occurring during

Postoperative good outcomes for cortical-sparing adrenalectomy is focused on preventing a steroid dependency. The benefit of not having a steroid necesity must be compared to the risk of recurrence in time. The necessity of extensive adrenalectomy depends on the genetic or familial predisposition. The patients that have familial syndromes such as MEN 2B, VHL or/and MEN 2A are the ideal candidates for cortical sparing adrenalectomies. Patients with MEN 2A or 2B syndroms who suffered cortical sparing adrenalectomy have a recurrence risk about 51.8% at ten years. The steroid dependency rate of patients who underwent unilateral or/and bilateral cortical sparing technique was 43%. Cortical sparing adrenalectomy is the

Patients with different type of pheochromocytomas should have a whole life follow-up program to avoid possible recurrence or the development of metastatic

As we all know, about 10% of adrenal pheochromocytomas tumors are malignant,

and about 30% of any extra-adrenal tumors are are more commonly malignant. Malignant pheochromocytoma is less frequent in children than in elderly and is mainly at extra-adrenal glands. Pheochromocytomas that are associated with some familial syndromes usually have an early diagnoses and are less malignant than

disease, which can occur up to 40 years after resectioning the tumor [17].

#### *Surgical Approach in Pheochromocytoma DOI: http://dx.doi.org/10.5772/intechopen.96066*

*Pheochromocytoma, Paraganglioma and Neuroblastoma*

**4.2 Posterior retroperitoneal laparoscopic approach**

tumors greater than 8 cm [2].

removed it from the abdomen [2].

**4.3 Open techniques**

metastatic disease [3].

metastatic disease.

**5. Perioperative/postoperative management**

Laparoscopic adrenalectomy has been found to reduce the need for hospitalization, blood transfusion, postoperative analgesia and recovery. Laparoscopic adrenalectomy is more difficult to perform on the right side because of the exposure problems, the proximity of the gland to the IVC and the short right adrenal vein. Although there are more and more reports of laparoscopic resection of pheochromocytoma, but it should not be considered for malignant pheochromocytomas or

Place the patient in a jack-knife position and place a 1.5–2 cm transverse incision under the terlfth rib to access the retroperitoneum space. Digital palpation can be used to develop and disecting spaces. Guided by the surgeon's index finger, place another trocar along the lateral border of the paraspinous muscle. Similarly, place a side needle under the eleventh rib. Insert another 12 mm blunt balloon trocar into the first incision. When pneumoperitoneum is established, carbon dioxide needs to be injected and kept at 20–24 mmHg. The working space of superior border of the kidney is developed by dissecting the retroperitoneal areolar tissue and Gerota's fascia. After identifying the adrenal veins, dissect the upper adrenal glands laterally and inferiorly, and finally dissect the upper and mid-medial glands. Identify, dissect and remove the adrenal veins between the clamps. The adrenal gland is firstly dissected laterally and inferiorly and secondly the superior and medial adesions are dissected with the evidentiation of the adrenal vein. The adrenal vein is identified, dissected, and resected between clips. The next step is the dissection of the upper attachment, and then put the specimen into the Endo bag device and

Multiple incisions can be used to exposure the anatomy, including subcostal, midline, and the Makuuchi incision. The aim is to use subcostal incision two to three cm below the costal margin. This incision provides excellent liver and adrenal bed exposure. If a lymph node dissection is planned, the subcostal incision will provide the space betwen aorta and inferior vena cava and between the lymph nodes around the hilum. As with the laparoscopic technique, the triangular ligament on the right is also cut, and the spleen on the left is moved into the retroperitoneal cavity. Very important anatomical landmarks such as inferior vena cava, must be marked on the right, and the tail of the pancreas and the loose plane between the tail and the left adrenal gland on the left must be visualized. For boths sides, the adrenal veins must be identified and cut. Split all remaining attachments and remove the gland. The open transabdominal or thoracoabdominal approaches give the best exposure for resecting extensive tumors, for bilateral adrenalectomy or for

Following surgical approach of pheochromocytoma, 80% of patients are suspected to be again normotensive. Persistent postoperative hypertension can be caused by the residual tumor, intraoperative injury to the kidney's renal artery or

Intraoperative hypotension may be caused by (1) hemorrhage during surgery (2) insufficient hormone replacement after left and right adrenal excision (3) vascular

**54**

compliance changes and immediate reduction of catecholamine levels after tumor removal, (4) myocardial infarction (5) long-term residual effects of prolonged α-blockers before surgery. As shown, hypotension can be effectively controlled by replacing volume and blood transfusion. Use vasopressors only when hypotension fails to respond to sufficient volume replacement [16].

Postoperatively, the majority of patients that had an uneventful intraoperative course with or without hemodynamic instability do not necessitate intensive care supervision. In the first postoperative period, the patients that have pheochromocytoma have also a greater risk of hypoglycemia and hypotension [5]. After catecholamines are suddenly stopped, due to the relative increase in insulin sensitivity, hypoglecemia may occur. Therefore, blood glucose must be supervised every hour for the first three or four hours postoerative. When a total adrenalectomy is planned, glucocorticoid preparations can be taken before surgery, specifically for the patients that have bilateral tumors or a familial pheochromocytoma syndrome. After surgery,incidence of hypoglycemia droped from 15% to 4.2%. Independent predictors related to postoperative hypoglycemia include prolonged operation time and increased urine adrenaline in 24-hour period before surgery. Therefore, glicemia must be checked regularly in the first day after surgery. Isolated episodes of hypotension are very common and can be attribueted to the preoperative alpha blockade residual values, hypovolemia that can be due to intraoperative blood loss or/and preoperative volume contract,. Treatment must be aggressive in terms of IV perfusions and vasopressors [3].

#### **5.1 Postoperative outcomes**

The rate of morbidity in untreated pheochromocytoma is very high and difficult to determin. 71% of patients can die from cardiovascular causes: myocardial infarction, hypertensive heart insuiciency, or hemodynamic instability occurring during different procedures [18].

Postoperative good outcomes for cortical-sparing adrenalectomy is focused on preventing a steroid dependency. The benefit of not having a steroid necesity must be compared to the risk of recurrence in time. The necessity of extensive adrenalectomy depends on the genetic or familial predisposition. The patients that have familial syndromes such as MEN 2B, VHL or/and MEN 2A are the ideal candidates for cortical sparing adrenalectomies. Patients with MEN 2A or 2B syndroms who suffered cortical sparing adrenalectomy have a recurrence risk about 51.8% at ten years. The steroid dependency rate of patients who underwent unilateral or/and bilateral cortical sparing technique was 43%. Cortical sparing adrenalectomy is the elective method for patients with VHL syndrome [19].

Patients with different type of pheochromocytomas should have a whole life follow-up program to avoid possible recurrence or the development of metastatic disease, which can occur up to 40 years after resectioning the tumor [17].

### **6. Particular situations**

#### **6.1 Malignant pheochromocytoma**

As we all know, about 10% of adrenal pheochromocytomas tumors are malignant, and about 30% of any extra-adrenal tumors are are more commonly malignant. Malignant pheochromocytoma is less frequent in children than in elderly and is mainly at extra-adrenal glands. Pheochromocytomas that are associated with some familial syndromes usually have an early diagnoses and are less malignant than

the sporadic forms. The histological criteria that are used to distinguish benign and malignant forms of the tumors are not very accurate, as it happens in others endocrine or glandular tumors. Malignant tumors depend on the clinical tumoral manifestations and are accurantly diagnosed when there is infiltration of adjacent organs, distant metastasis or recurrence. The most common metastasis occur in bones, local LN, peritoneum, lungs and liver. Malignant tumors are commonly much bigger, with a higher frequency of vascular and capsule infiltration. They are usually characterized by DNA tetraploidy or aneuploidy, increased mitosis, angiogenesis, higher serum levels of neuropeptide Y, tumor necrosis, c-myc expression and higher neuron-specific enolase levels. Most malignant pheochromocytomas show increased uptake of metaiodo-benzylguanidine (MIBG). The survival rate at 5 years, for malignant pheochromocytomas is about 44%. In cases of extra-adrenal localization of pheochromocytomas the prognosis is worse than in adrenal tumors. Patients that have associated pulmonary metastasis have a much worse prognosis [18].

#### **6.2 Pheochromocytoma in pregnancy**

There are very common undiagnosed pheochromocytomas during pregnancy, and their maternal and infant mortality rates are very high, up to 58% and 56%. If the diagnosis is madein time, during the pregnancy period, the mortality and morbidity rate can reach below 11%. When the diagnosis is made at the time of delivery, the maternal mortality rate is still high, about 40%. In case of pregnant women the diagnosis of pheochromocytoma can be suspected in case of severe forms of hypertension in the first two months of pregnancy. If hypertension is not controlled in the second trimester, or is related to orthostatic hypotension, or if unexplainable shock occurs suddenly befor delivery, the diagnosis should be focused. The diagnosis as been confirmed by biochemical testing. MRI is the preferred local imaging tehnique to avoid radiation risk. In these cases, the freguently used irritation test is contraindicated, but in some specific cases, the clonidine inhibition test can be used. If the diagnosis is made in first trimester of pregnancy, it is recommended to remove the tumor after proper control of hypertension. In the last trimester of pregnancy, it is recommended to combine pregnancy with selective cesarean C-section intervention and immediate tumor removal under the same anesthesia through medical management. Due to the increased risk of fetal complication and hypertension, spontaneous and vaginal natural delivery methods should be avoided [19].

#### **7. Conclusions/summary**

The surgical methods for pheochromocytomas approach must have in priority list the assesment of the best imaging, the identifying any germline genetic mutations, and of course the utilization of any minimally invasive techniques when feasible and indicated. Biochemical diagnosis and precise tumor localization are necessary.

The minimally invasive technique of the abdominal or retroperitoneal approach is the standard surgical method. For large tumors with risk of rupture and potential malignancy, open surgery is recomanded. The results after surgical resection have a real potential to reduce the incidence of cardiovascular disease. Complete surgical resection is the ultimate treatment for benign and malignant pheochromocytoma that have low morbidity and mortality rates.

Special cases of malignant pheochromocytoma or this pathology's occurrence in pregnancy must be suppervised and treated with the utmost care. There are not any very accurate of uniform histological criteria so far, to distinguish malignancy in these cases, which depends on the tumor's clinical behavior.

**57**

**Author details**

Radu Mihail Mirica1

'Carol Davila', Bucharest, Romania

'Carol Davila', Bucharest, Romania

provided the original work is properly cited.

\* and Sorin Paun<sup>2</sup>

\*Address all correspondence to: mirica\_rm@yahoo.com

1 Emergency Clinical Hospital 'Saint John', University of Medicine and Pharmacy

2 Emergency Clinical Hospital Bucharest, University of Medicine and Pharmacy

© 2021 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/ by/3.0), which permits unrestricted use, distribution, and reproduction in any medium,

*Surgical Approach in Pheochromocytoma DOI: http://dx.doi.org/10.5772/intechopen.96066*

The authors declare no conflict of interest.

**Conflict of interest**

*Surgical Approach in Pheochromocytoma DOI: http://dx.doi.org/10.5772/intechopen.96066*
