Pheochromocytoma Of Adrenal Gland Scaled Score Research Articles

Risk Factors for Perioperative Complications, Treatment Outcomes and Aggressive Behavior of the Tumor in Patients with Pheochromocytoma

Introduction. Pheochromocytomas are catecholamine-secreting tumors arising from chromaffin cells of the adrenal gland. Surgery is the only curative treatment with a high biochemical cure rate, low mortality and high risk of perioperative complications. Objectives. To study the demographic characteristics of patients with pheochromocytoma and to identify the risk factors for perioperative complications, treatment outcomes, and aggressive behavior of the tumor. Methodology. We retrospectively studied the data of pheochromocytoma patients registered from 2012 to 2022. Results. In our study, a total of 30 patients with pheochromocytoma were included. The mean age of presentation was 35 ± 12.8 years. Fifty-six percent were females, and the sex ratio was 1.3:1. Pheochromocytoma spells (60%) was the most common complaint, followed by abdominal pain (53%), orthostatic complaints (10%) and incidentalomas (6%). The baseline mean 24-hour urinary total metanephrines was 2963.7 ± 2658 mcg/24 hours, and the mean tumor size was 7.3±0.53 cm. Forty-three percent of patients underwent laparoscopic adrenalectomy, while the rest underwent open surgery. The mean Pheochromocytoma of Adrenal gland Scaled Score (PASS) was 3.41 ± 0.28, and 23% had a high risk for malignancy. Among perioperative complications, hypertension crisis (17%) was the most common, followed by posoperative hypotension (13%), hypoglycemia (3%) and right-sided pneumothorax (3%). These patients with complications had higher metanephrine levels (5490 vs. 1880mcg/24 hours, p=0.001). Blood pressure normalized in 50%, and this was associated with male sex, younger age (29.5 vs. 40 years, p=0.03), higher metanephrines (4619 vs. 1855mcg/24 hours, p=0.001) and smaller tumors (5.91 vs. 8.61cm, p=0.046). PASS score greater than or equal to 4 was associated with higher metanephrine levels (5104 vs. 2312mcg/24 hours, p=0.021) and larger tumors (9.28 vs. 6.68 cm, p=0.024). Biochemical cure rate was achieved in 76% of patients after surgery and was associated with older age (37.7 years vs. 27.7 years, p=0.047) and absence of pheochromocytoma spells (100% vs. 61%, p=0.014). Conclusion. Young age, smaller tumor size and higher metanephrine concentrations were associated with normalization of blood pressure post-surgery. On the other hand, older patients and those without pheochromocytoma spells had better biochemical cure rates. Patients with higher baseline metanephrine levels had increased perioperative complications. More aggressive tumor behavior was associated with higher metanephrine levels and larger tumors. Sex, baseline blood pressure and mode of surgery did not have any influence on treatment outcomes.

Disentangling of Malignancy from Benign Pheochromocytomas/Paragangliomas.

ObjectiveMany malignant tumors initially appear benign but subsequently exhibit extensive metastases. Early identification of malignant pheochromocytomas and paragangliomas (PPGLs) before metastasis is important for improved prognosis. However, there are no robust prognostic indices of recurrence and malignancy. The aim of this study was to identify the clinical and histopathological factors that predict malignant PPGLs.DesignRetrospective follow-up study.MethodsIn this study, we included 223 patients with pathologically confirmed PPGLs who were treated between 2000 and 2015 at the Seoul National University Hospital in South Korea.ResultsOf these patients, 29 were diagnosed with malignancy, 12 of whom presented with metastatic lesions at the initial diagnosis while 17 developed metastases during follow-up. Nineteen patients with recurrent PPGLs consisted of ones with malignant PPGLs (n = 17) and multifocal PPGLs (n = 2) who had VHL and RET mutations. The mean age at presentation for malignant PPGLs was significantly younger than that for benign PPGLs (43.0 vs. 49.0 years, respectively; p = 0.023). Tumor size was not a distinguishing factor between malignant and benign PPGLs (5.0 vs. 4.5 cm, respectively; p = 0.316) nor did it predict recurrence. Of 119 patients with available pheochromocytoma of adrenal gland scaled score (PASS) data, those with malignant PPGLs presented PASS values ≥4. Of 12 parameters of PASS, necrosis, capsular invasion, vascular invasion, cellular monotony, high mitosis, atypical mitotic figures, and nuclear hyperchromasia were significant predictors of malignancy.ConclusionsTumor size did not predict malignancy or recurrence of PPGLs. PPGL patients with characteristic pathologic findings and PASS ≥4 or germline mutations require close follow-up.

Malignant pheochromocytoma: new malignancy criteria

The pathological diagnosis of malignancy in pheochromocytomas remains a controversial issue. According to the WHO, malignancy is defined in the presence of metastasis. Multiparameter scoring systems such as PASS (Pheochromocytoma of Adrenal gland Scaled Score) have been used but remain controversial. The aim of this study was to search for new immunohistologic elements allowing determination of pheochromocytoma malignancy. Among 53 patients operated for pheochromocytoma between 1993 and 2009, we selected pheochromocytomas with proven metastasis, seven cases in group 1 (G1) and paired two others groups: group 2 (G2), patients who had "benign" pheochromocytoma with PASS ≥4 and group 3 (G3), patients who had "benign" pheochromocytoma with PASS <4. We retrospectively analysed PASS criteria, size, weight, tumour necrosis, Ki-67 and pS100 staining. The size and weight of the lesion were directly and significantly correlated to malignancy in all three groups: respectively 9.7cm and 292.0g (G1), 6.2cm and 83.8g (G2) and 3.8cm and 37.1g (G3) (p < 0.005 for both). Tumour necrosis (TN) was present in all G1 (p < 0.005) and respectively at 0% and 37.5% in G2 and G3. Ki-67 is directly correlated to presence of TN (p < 0.005) and malignancy (G1 14.1%, G2 1.8%, G3 2.6%; p < 0.001). All G1 had a Ki-67 index >4%, although one G3 presented an 11% Ki-67 index. There was an inverse statistically significant correlation between the three groups in staining using pS100 (p < 0.01). Size and weight of the pheochromocytoma are directly related to PASS and malignancy. The presence of tumour necrosis, Ki-67 index >4% and pS100 absence impose a close histopathological evaluation and follow-up with regard to cases presenting a high risk of malignancy/recurrence.