In fact, our study involves 40 patients, including 23 women (57,5%) and 17 men (42,5%). On the other hand, the average age at the time of diagnosis was 43.8 ± 16.8 years, with extremes that ranged from 10 to 74 years for the 40 patients.
Fourteen patients (35%) of our sample were found to be hypertensive for an average of 6 years before the diagnosis of PPGL (extremes: 6 months to 15 years). Moreover, different antihypertensive drugs were administered to patients, among whom six (42.8%) were on monotherapy, 3 on a combination therapy, 4 on a triple therapy and 1 on a quadritherapy.
On the other hand, the circumstances of discovery (COD) were mainly represented by adrenal incidentalomas (AI) in 23 cases (57,7%). Then, PHEO was discovered on the occasion of an investigation of hypertension (HT) among 12 patients (5 cases of HT among young patients, 5 cases of HT associated with paroxysmal PHEO signs, and 2 cases of drug-resistant HT). In addition, hypertension complications constituted a COD in 3 cases. Furthermore, an ectopic PPGL was discovered fortuitously during an anatomopathological examination of a biopsy of a right renal mass in a 63-year-old patient followed for a large cell B lymphoma. Finally, PPGL was diagnosed as a part of an extension review in a 46-year-old woman with syndromic features, wowing a multiple endocrine neoplasia type 2A (Medullary thyroid cancer, primary hyperparathyroidism, and cutaneous lichen amyloidosis).
In fact, different functional signs were reported by our patients. Palpitation was the most described sign (67.5%) followed by sweat (62.5%) then, headache (57.7%) while the Menard triad was present in 15 patients (37.5%). The UFM dosage was performed in 29 cases (72.5%). An elevation of this latter greater than 4 times the UCF was observed in 23 patients, while five patients showed an elevation between 1.4 and 3 times the UCF. Then, the amount of urine metanephrines was 18.2 times as high as that of the UCF (extreme: 1.4 − 80), while the average level of normetanephrine in urine was 23.2 times as high as that of the UCF (extremes: 1.5 − 165). On the other hand, PFM were dosed in 10 patients (25%). It was elevated in 90% of cases. Moreover, the levels of metanephrine (MNp) and normetanephrine (NMNp) in the blood were 5.7 times as high as those of the UCF for both parameters, with extremes ranging from 2 to 11 and 1.4 to 15 times, respectively. The elevation of PFM was 4 times as high as the UCF in 70% of cases and discrete between 1.4 and 3 times the UCF in 2 cases. On the other hand, the level of Chromogranine A (CGA) in the blood was dosed in seven cases, where it was normal in 2 patients and high in 5 patients with an average of 437.4 µg/l (extremes: 112–1650 µg/l). The Vanillylmandelic acid (VMA) was dosed in only one case in our series. This dosage allowed the diagnosis to be retained with an elevation of 3.8 times the upper reference limit. In general, the biological diagnosis in our series was based on the dosage of the UFM, PFM, and VMA, respectively, in 61.5%, 18% and 2.5% of cases. In fact, the associated dosages help retain the diagnosis at 18%.
The PPGL are a part of a genetic disease in 7,5% in our series (1 case of multiple endocrine neoplasia, type 2A, 1 case of multiple endocrine neoplasia, type 2B and 1 patient diagnosed with neurofibromatosis type 1).
An abdominal pan scan (CT) with contrast was performed in 39 patients. It was prescribed as a first-line imaging modality in 13 patients (32.5%) and preceded by an abdominal ultrasound in 26 patients. In fact, it helps locate the tumor in 100% of cases. The average tumor size was 55.2 mm (extreme: 16-157 mm). The mass was hypodense in 33.3% of cases and heterogeneous in 18% of cases. On the other hand, moderate to intense heterogeneous enhancement was objectified in 97.4% of cases. Abdominal magnetic resonance imaging (MRI) was performed on 15 patients (37.5%). MRI could help locate the tumor in 100% of cases. The tumor was found to have a low signal, T1 and a high signal T2 for all patients. Then, an enhancement was noted in 100% of cases. In total, CT and/or MRI were used to locate the tumor in all patients. The location was intra-adrenal in 92.5%: right adenoma in 23 patients, left adenoma in 11 patients and bilateral in 3 patients. Extra-adrenal location was objectified in 3 patients (pararenal, para-aortic and prostatic PGL). The MIBG scan was performed in 16 patients and increased radiotracer hyperfixation was noted in 93.7% of cases consistent with CT and/or MRI data.
Moreover, surgery was indicated in all patients: Thirty-two of our patients (80%) were operated, 6 patients refused the surgery, adrenalectomy was scheduled for 1 patient, and 1 patient died of a complicated pulmonary embolism with acute lung edema. All of our patients received a preoperative medical preparation for at least 7 days. Adrenergic blocking agents, also known as alpha-adrenergic antagonists, were prescribed as a first-line treatment in 8 cases then, associated with β-adrenergic receptor blockers or calcium channel blockers in 15 cases. The time between the biological diagnosis and the surgery was 6 months on average (extremes: 15 days–2 years). The gesture consisted of the following: a one-sided total adrenalectomy in 90% of cases (29 patients), a two-stage bilateral adrenalectomy was suggested for one patient and tumor resection was performed on 2 patients (2 PGL).
In fact, twenty-nine patients had at least one consultation for postoperative evaluation. The short-term evolution was marked by the disappearance of paroxysmal signs in almost all cases with the exception of one patient, who kept palpitations and for whom a β-blocker treatment was prescribed. An average weight gain of 4 kg (extremes: 1–10 kg) was objectified in 16 patients. Thirteen patients had normal blood pressure with the discontinuation of antihypertensive treatments after the surgery. Then, an improvement in blood pressure with therapeutic digression was observed in 6 patients, and persistent hypertension only in one patient (Fig. 1).
Moreover, metanephrines (urinary or plasma) were controlled in 24 patients during the first postoperative year. The results were as follows: Normalization in 21 patients (87.5%) and persistence of moderate elevation (< 4 times the upper limit) in three patients (12.5%). The CGA was dosed in 2 cases after the surgery but came back high in both cases. Therefore, an adrenal CT scan, performed on 5 patients (3 with high metanephrines and 2 with high levels of CGA), gave normal results in all cases. Moreover, fourteen patients were regularly monitored for long-term follow-up. The mean duration of postoperative follow-up was 3 years. Hence, a biological recurrence was observed in 3 cases (1 case with malignant PHEO, 1 patient with a normal radiological investigation and one patient whose investigation is ongoing).