UNASSIGNED: Neurofibromatosis type 1 is a hereditary condition often associated with pheochromocytomas but rarely with malignant pheochromocytomas. Neurofibromatosis type 1 is often associated with bone lesions, which complicates the distinction between malignant and benign tumors.
UNASSIGNED: A 46-year-old man with a medical history of neurofibromatosis type 1 presented with right abdominal pain. Computed tomography revealed a right adrenal tumor, and metaiodobenzylguanidine scintigraphy showed accumulation in the right adrenal gland and thoracic vertebrae. He was diagnosed with pheochromocytoma, and a right adrenalectomy was performed. After surgery, a bone biopsy was conducted on the spinal lesion, confirming metastasis of pheochromocytoma, prompting irradiation. After that, lung and liver metastases emerged, and chemotherapy with cyclophosphamide, vincristine, and dacarbazine was initiated; however, the disease progressed, and he died 11 months after surgery.
UNASSIGNED: We report a case of malignant pheochromocytoma associated with neurofibromatosis type 1 in which bone metastasis was difficult to diagnose.

BACKGROUND: This case report documents a case of malignant pheochromocytoma manifested as vision changes with lung metastasis and recurrence.
METHODS: A 10-year-old Han Chinese girl presented with vision changes and was eventually diagnosed with pheochromocytoma by contrast-enhanced computed tomography, urine vanillylmandelic acid. After medication for hypertension and surgery, clinical symptoms disappeared. Malignant pheochromocytoma with lung metastasis was confirmed histologically using the Pheochromocytoma of the Adrenal Gland Scaled Score scoring system and genetically with succinate dehydrogenase complex iron sulfur subunit B mutation, and 3 months later, unplanned surgery was performed because of the high risks and signs of recurrence. She is asymptomatic as of the writing of this case report. Our patient\'s case highlights the importance of considering a diagnosis of malignant pheochromocytoma, and long-term follow-up for possible recurrence.
CONCLUSIONS: Although there are well-recognized classic clinical manifestations associated with pheochromocytoma, atypical presentation, such as vision changes in children, should be considered. In addition, malignant pheochromocytoma children with a high Pheochromocytoma of the Adrenal Gland Scaled Score and succinate dehydrogenase complex iron sulfur subunit B mutation require a long-term follow-up or even unplanned surgery because of the higher risk of recurrence.

Introduction: Bone metastasis of malignant pheochromocytoma is a rare disease. We report a patient with a 10-year history who underwent 18F-FDG PET/CT to detect bone metastasis and receive radiotherapy and chemotherapy with complete response for bilateral iliac pain. Case presentation: A 48-year-old male patient complained of dizziness, hypertension, and bilateral iliac pain for 2 months. The patient had a history of resection of bilateral malignant adrenal pheochromocytoma 10 years earlier, and all complaints were relieved immediately after operation. 18F-FDGPET/CT showed abdominal lymph node uptake and multiple bone uptake, as well as multiple brown fat uptake. A biopsy of the left ilium confirms the metastasis of malignant pheochromocytoma. Discussion: In our literature review, we discuss the metastasis of pheochromocytoma reported by some scholars, and the role of radionuclides such as 18F-FDG PET/CT, 18F-DOPA PET/CT, I-123MIBG, and 68Ga-DOTATATE PET, in the diagnosis of malignant pheochromocytoma. The patient above is a good case for clinicians in the diagnosis and treatment of metastatic pheochromocytoma, especially in some hospitals with only 18F-FDG imaging agents. Conclusion: A review of this case and similar rare cases in the literature illustrates the importance of 18F-FDG PET/CT in the diagnosis of malignant pheochromocytoma.

Pheochromocytoma is a catecholamine-producing tumor in the adrenal medulla and is often accompanied by hypertension, hyperglycemia, hypermetabolism, headache, and hyperhidrosis, and it is classified as benign and malignant pheochromocytoma. In addition, persistent hypertension is often observed in subjects with malignant pheochromocytoma.
A 52-year-old Japanese male was referred and hospitalized in our institution. He had a health check every year and no abnormalities had been pointed out. In addition, he had no past history of hypertension. In endocrinology markers, noradrenaline level was as high as 7,693 pg/ml, whereas adrenaline level was within normal range. Abdominal contrast-enhanced computed tomography revealed a 50-mm hyper-vascularized tumor with calcification in the right adrenal gland and multiple hyper-vascularized tumors in the liver. In 131I MIBG scintigraphy, there was high accumulation in the right adrenal gland and multiple accumulation in the liver and bone. In echocardiography, left ventricular ejection fraction was as low as 14.3%. In coronary angiography, however, there was no significant stenosis in the coronary arteries. Based on these findings, we finally diagnosed him as malignant pheochromocytoma accompanied by multiple liver and bone metastases and catecholamine cardiomyopathy. However, blood pressure was continuously within normal range without any anti-hypertensive drugs. Right adrenal tumor resection was performed together with left hepatic lobectomy and cholecystectomy. Furthermore, serum levels of vascular endothelial growth factor (VEGF) and parathyroid (PTH)-related protein were very high before the operation but they were markedly reduced after the operation.
This is the first report showing the time course of serum VEGF level in a subject with malignant pheochromocytoma, clearly showing that malignant pheochromocytoma actually secreted VEGF. In addition, this case report clearly shows that we should bear in mind once again that malignant pheochromocytoma is not necessarily accompanied by hypertension.

BACKGROUND: Malignant pheochromocytomas are rare endocrine tumors that develop within chromaffin tissue. The diagnosis of malignancy is based on neoplastic recurrence or the presence of metastasis in organs that lack chromaffin tissue. We report a series of four cases because of their diagnostic and therapeutic particularities.
METHODS: we describe four clinical cases of patients with malignant pheochromocytoma whose Menard triad \"headache-palpitations-sweating\" was present in three out of four patients, the methoxylated derivatives were measured in 4 patients, 3 of which had high values, all of our patients carried out a CT scan which objectified signs of malignancy, MRI was performed on a single patient; presenting with a neoplastic recurrence; looking for a locoregional invasion.
CONCLUSIONS: Pheochromocytoma (PC) is a rare neuroendocrine tumor derived from the chromaffin cells of the adrenal medulla. Its annual incidence is 2 to 8 per million adults. A peak frequency is observed between 30 and 40 years of age. Approximately 10% of pheochromocytomas are malignant and in 10% of cases, bilateral localization is observed. Criteria for malignancy include the invasion of neighboring organs, a large tumor, the presence of lymphadenopathy on imaging, or fixation on scintigraphy. Surgery for MAP is not always curative. In the case of multiple liver metastases, treatment is based on adrenalectomy, which can be effectively combined with chemoembolization, cryoablation, or radiofrequency techniques.
CONCLUSIONS: The main prognostic factors of the malignant pheochromocytomas are a large tumor volume, the existence or number of visceral metastases, and the presence of a mutation in the SDHB (Succinate dehydrogenase B) gene.

BACKGROUND: Malignant pheochromocytoma with cerebral and skull metastasis is a very rare disease. Combining our case with 16 previously reported cases identified from a PubMed search, an analysis of 17 cases of malignant cerebral pheochromocytoma was conducted. This literature review aimed to provide information on clinical manifestations, radiographic and histopathological features, and treatment strategies of this condition.
METHODS: A 60-year-old man was admitted with a progressive headache and enlarging scalp mass lasting for 3 mo. Radiographic images revealed a left temporal biconvex-shaped epidural mass and multiple lytic lesions. The patient underwent a left temporal craniotomy for resection of the temporal tumor. Histopathological analysis led to identification of the mass as malignant pheochromocytoma. The patient\'s symptoms were alleviated at the postoperative 3-mo clinical follow-up. However, metastatic pheochromocytoma lesions were found on the right 6th rib and the 6th to 9th thoracic vertebrae on a 1-year clinical follow-up computed tomography scan.
CONCLUSIONS: Magnetic resonance spectroscopy and histopathological examination are necessary to make an accurate differential diagnosis between malignant cerebral pheochromocytoma and meningioma. Surgery is regarded as the first choice of treatment.

BACKGROUND: Pheochromocytoma surgery is generally challenging for surgeons and anesthesiologists for cardiovascular complications.
METHODS: A 54-year-old Japanese man was found to have a large right pheochromocytoma infiltrating the posterior part of his liver and vena cava and multiple lung metastases. After retroperitoneal laparoscopic dissection of the dorsal side of the tumor and ligation of the feeding vessels, total resection of the primary tumor, extended posterior sectional hepatectomy, and partial vena cava resection were performed by open surgery via a thoracoabdominal approach. Abundant congestive bleeding with instability of vital signs occurred during transection. It could be finally controlled by dissect the remnant feeding artery in the inmost space. Prior control of arterial in-flow enabled successful completion of the planned surgical procedure. The patient has now survived for 27 months since resection of the primary lesion.
CONCLUSIONS: Ligation of the feeding arteries to this hypervascular catecholamine-releasing tumor via a retroperitoneal laparoscopic approach prior to performing combined organ resection facilitated successful excision of this large malignant pheochromocytoma.

Pleochromocytoma is a benign neuroectodermal tumor of the adrenal medulla. Ten to thirty percent of these tumors become metastatic. The bone is the most frequent site for metastasis but jaw involvement is reported rarely. A case of mandibular involvement is reported and a literature review in this topic is presented. Preoperative management of these patients undergoing general anesthesia is not a routine practice in maxillofacial surgery, so this topic is also is explained.

UNASSIGNED: Metastatic spinal pheochromocytoma (MSP) is very rare in clinical practice, with only a few case reports in the literature. Its low incidence makes it profoundly difficult for clinicians to determine appropriate treatment strategies and predict the prognosis. In this study, we analyzed the clinical characteristics, surgical procedure and prognosis of patients with MSP in one of the largest clinical investigations of this entity to date.
UNASSIGNED: In this study, we conducted a retrospective analysis of the clinical data of 10 patients with MSP who were treated in our department from 2012 to 2020. We performed a total of 14 operations using two types of surgery: open surgery and percutaneous vertebroplasty.
UNASSIGNED: Among them, nine patients underwent 14 spinal operations with satisfactory effect and without any perioperative complications. The mean time from the initial operation to detection of spinal metastasis was 85.3 (12-132) months. The average follow-up time was 27.3 months. Disease progression was detected in nine patients, and eight patients (80%) died during the follow-up period. Univariate analysis showed that extraosseous visceral metastasis (P = 0.022), Tomita score (P = 0.027), and number of spinal metastases (P = 0.024) were associated with overall survival (OS). In addition, extraosseous visceral metastasis (P = 0.030), Tomita score (P = 0.013), and number of spinal metastases (P = 0.026) were associated with progression-free survival (PFS).
UNASSIGNED: Surgical treatment is an effective option in treating MSP and plays an important role in improving patients\' quality of life, due to its efficacy in relieving pain, reconstruction of stability, and restoration of function. Extraosseous metastasis, Tomita score, and number of spinal metastases are all potential prognostic factors for OS and PFS.

Pheochromocytomas and paragangliomas (PPGLs) are rare tumors that cause refractory hypertension and hypertensive crisis. Although metastatic disease accounts for 30% of PPGLs, the diagnosis of malignancy is difficult without the presence of metastatic lesions. Here, we review several advancements in the diagnosis and treatment of PPGL. A nationwide epidemiological survey in Japan revealed that the annual number of patients with PPGL was 3000, which was higher than that reported previously. While plasma and urine fractionated metanephrines are recommended for use in specific biochemical testing for diagnosis, creatinine-corrected fractionated metanephrines in spot urine samples that had been widely used in Japan as a convenient screening test were shown to be as useful as 24-h urine fractionated metanephrines. Regarding imaging studies, a more specific functional imaging for PPGLs, 68Ga DOTATATE, was newly developed. 68Ga DOTATATE provides a clear image with high sensitivity and specificity. Currently, PASS or GAPP histological scores and SDHB immunostaining are clinically used to attempt to discriminate benign from malignant tumors. However, since this distinguishing process remains difficult, all cases were classified as malignant with the possibility of metastasis in the WHO classification of endocrine tumors updated in 2017. Approximately 60% of PPGLs have germline mutations in PPGL-related genes. Currently, the genes are classified into two clusters based on their mechanism for the etiology of tumorigenesis. Based on the possible mechanisms of tumor development associated with gene mutations, several molecular target drugs are under evaluation to explore more promising treatments for malignant PPGL.