**Conflict of interest**

*Pheochromocytoma, Paraganglioma and Neuroblastoma*

**6.2 Pheochromocytoma in pregnancy**

**7. Conclusions/summary**

that have low morbidity and mortality rates.

these cases, which depends on the tumor's clinical behavior.

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].

ous and vaginal natural delivery methods should be avoided [19].

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, spontane-

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

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

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The authors declare no conflict of interest.
