Active clinical trials for "Paraganglioma"

The objectives of this study are: To assess the efficacy of lanreotide given every 4 weeks in participants with advanced or metastatic paraganglioma/ pheochromocytoma. To assess the toxicity and safety of lanreotide in participants with advanced or metastatic paraganglioma/ pheochromocytoma. To document the effects of lanreotide on markers of biochemical activity in participants with advanced or metastatic paraganglioma/ pheochromocytoma. Primary endpoints: • Assess efficacy by estimating the tumor growth rate while a patient is enrolled on study and comparing the growth rates on lanreotide to the pre-enrolment growth rate. Secondary endpoints include measurement of: Overall survival (OS) Progression-free survival (PFS) Overall response rate (ORR) according to RECIST defined as partial response (PR) + complete response (CR) Magnitude of reduction in levels of 24-hour urinary metanephrines, catecholamines and magnitude of reduction in serum chromogranin A, evaluated every two months while enrolled on study.

This is an open-label phase II study of an investigational drug, anlotinib in participants with advanced malignant paraganglioma or pheochromocytoma. Pheochromocytoma and paraganglioma (PPGL) are tumors originating from the adrenal medulla or adrenal diplomatic sensory chain, respectively, which can synthesize and secrete large amounts of catecholamines. In this study, participants whose disease has advanced or spread despite prior standard therapy, will receive anlotinib for 2-weeks followed by a 1-week rest period, until disease progression (PD) or drug toxicity intolerance. Anlotinib is an investigational drug, which has been shown to shrink tumours in several tumour models. The study will evaluate the efficacy as well as the toxicity profile of anlotinib when used as an alternative treatment for participants with PPGL tumours.

This phase II trial studies the effectiveness of anlotinib hydrochloride in the neoadjuvant therapy of locally advanced, or unresectable pheochromocytoma or paragangliom(PPGL). Anrotinib is used preoperatively in order to change unresectable tumors to resectable and reduce the high risk of surgery.

This phase II trial studies the effectiveness oftemozolomide in the neoadjuvant therapy oflocally advanced,or unresectable pheochromocytoma or paragangliom(PPGL). Temozolomide (TMZ) is a novel oral alkylation chemotherapeutic agent. Inthisstudy,temozolomidewill be used preoperatively in order to change unresectable tumors to resectable and reduce the high risk of surgery.

The aim of this study is to evaluate the diagnostic performance and tumor burden of 18F-metafluorobenzylguanidine (18F-MFBG) positron emission tomography (PET) in patients with neuroendocrine tumors mainly in pheochromocytoma and paraganglioma (PPGL) and neuroblastoma (NB).

Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors originating from catecholamine producing chromaffin cells in the adrenal medulla and extra-adrenal paraganglia. The overall age-standardized incidence rate is 0.18 per 100,000 person-years in Korea. The definitive treatment of PPGL is surgical excision of tumor. However, surgery is associated with a high risk of perioperative hemodynamic instability (HI). To avoid perioperative HI in patients diagnosed with PPGL, preoperative management including routine use of alpha blockade and volume expansion has been advocated by several guidelines. While unstable hypertension and tachycardia should be controlled in patients with PPGL, there is controversial that all patients diagnosed with PPGL should undergo preoperative pharmacological treatment, especially alpha blockade. The most important risk of preoperative alpha blockade use is perioperative hypotension. A recent study reported that patients diagnosed with PPGL postoperatively may have no further higher risk of intraoperative hypertension than those diagnosed preoperatively despite insufficient preoperatively management of PPGL. Therefore, it is a very important to study the relationship between HI and preoperative alpha blockade in normotensive patients diagnosed with PPGL. The aim this study is to analyze the effect and safety of omitting preoperative alpha-adrenergic blockade for normotensive pheochromocytoma through a prospective randomized controlled trial. The patients is divided into two groups. The patients in control group take a phenoxybenzamine at least 2 to 5 weeks before surgery. The patients in case group do not take a phenoxybenzamine. Primary outcome is to evaluate the percentage of time during surgery with systolic blood pressure more than 160mmHg or average blood pressure less than 60mmHg. And secondary outcomes are to evaluate hemodynamic instability in preoperative ward and postoperative ward.

Target population: Patients with (1) newly diagnosed or (2) past history of pheochromocytomas and paragangliomas (PPGL) or (3) carrier of genetic mutations in known PPGL susceptibility genes. International multicenter prospective cohort study with randomized intervention (special care follow-up vs. standard care follow-up). All patients will receive instructions about follow-up at the time point of study inclusion. Patients randomized to the standard care follow-up group will be advised to return annually for follow-up according to current routine practice (without active re-scheduling). In contrast, patients randomized to the special care follow-up group will also be advised to return annually for follow-up but these patients will be actively invited, re-scheduled and reminded by the centers to meet scheduled follow-up appointments.

This phase II trial studies nivolumab and ipilimumab in treating patients with rare tumors. Immunotherapy with monoclonal antibodies, such as nivolumab and ipilimumab, may help the body's immune system attack the cancer, and may interfere with the ability of tumor cells to grow and spread. This trial enrolls participants for the following cohorts based on condition: Epithelial tumors of nasal cavity, sinuses, nasopharynx: A) Squamous cell carcinoma with variants of nasal cavity, sinuses, and nasopharynx and trachea (excluding laryngeal, nasopharyngeal cancer [NPC], and squamous cell carcinoma of the head and neck [SCCHN]) B) Adenocarcinoma and variants of nasal cavity, sinuses, and nasopharynx (closed to accrual 07/27/2018) Epithelial tumors of major salivary glands (closed to accrual 03/20/2018) Salivary gland type tumors of head and neck, lip, esophagus, stomach, trachea and lung, breast and other location (closed to accrual) Undifferentiated carcinoma of gastrointestinal (GI) tract Adenocarcinoma with variants of small intestine (closed to accrual 05/10/2018) Squamous cell carcinoma with variants of GI tract (stomach small intestine, colon, rectum, pancreas) (closed to accrual 10/17/2018) Fibromixoma and low grade mucinous adenocarcinoma (pseudomixoma peritonei) of the appendix and ovary (closed to accrual 03/20/2018) Rare pancreatic tumors including acinar cell carcinoma, mucinous cystadenocarcinoma or serous cystadenocarcinoma. Pancreatic adenocarcinoma is not eligible (closed to accrual) Intrahepatic cholangiocarcinoma (closed to accrual 03/20/2018) Extrahepatic cholangiocarcinoma and bile duct tumors (closed to accrual 03/20/2018) Sarcomatoid carcinoma of lung Bronchoalveolar carcinoma lung. This condition is now also referred to as adenocarcinoma in situ, minimally invasive adenocarcinoma, lepidic predominant adenocarcinoma, or invasive mucinous adenocarcinoma Non-epithelial tumors of the ovary: A) Germ cell tumor of ovary B) Mullerian mixed tumor and adenosarcoma (closed to accrual 03/30/2018) Trophoblastic tumor: A) Choriocarcinoma (closed to accrual) Transitional cell carcinoma other than that of the renal, pelvis, ureter, or bladder (closed to accrual) Cell tumor of the testes and extragonadal germ tumors: A) Seminoma and testicular sex cord cancer B) Non seminomatous tumor C) Teratoma with malignant transformation (closed to accrual) Epithelial tumors of penis - squamous adenocarcinoma cell carcinoma with variants of penis (closed to accrual) Squamous cell carcinoma variants of the genitourinary (GU) system Spindle cell carcinoma of kidney, pelvis, ureter Adenocarcinoma with variants of GU system (excluding prostate cancer) (closed to accrual 07/27/2018) Odontogenic malignant tumors Pancreatic neuroendocrine tumor (PNET) (formerly named: Endocrine carcinoma of pancreas and digestive tract.) (closed to accrual) Neuroendocrine carcinoma including carcinoid of the lung (closed to accrual 12/19/2017) Pheochromocytoma, malignant (closed to accrual) Paraganglioma (closed to accrual 11/29/2018) Carcinomas of pituitary gland, thyroid gland parathyroid gland and adrenal cortex (closed to accrual) Desmoid tumors Peripheral nerve sheath tumors and NF1-related tumors (closed to accrual 09/19/2018) Malignant giant cell tumors Chordoma (closed to accrual 11/29/2018) Adrenal cortical tumors (closed to accrual 06/27/2018) Tumor of unknown primary (Cancer of Unknown Primary; CuP) (closed to accrual 12/22/2017) Not Otherwise Categorized (NOC) Rare Tumors [To obtain permission to enroll in the NOC cohort, contact: S1609SC@swog.org] (closed to accrual 03/15/2019) Adenoid cystic carcinoma (closed to accrual 02/06/2018) Vulvar cancer (closed to accrual) MetaPLASTIC carcinoma (of the breast) (closed to accrual) Gastrointestinal stromal tumor (GIST) (closed to accrual 09/26/2018) Perivascular epithelioid cell tumor (PEComa) Apocrine tumors/extramammary Paget's disease (closed to accrual) Peritoneal mesothelioma Basal cell carcinoma (temporarily closed to accrual 04/29/2020) Clear cell cervical cancer Esthenioneuroblastoma (closed to accrual) Endometrial carcinosarcoma (malignant mixed Mullerian tumors) (closed to accrual) Clear cell endometrial cancer Clear cell ovarian cancer (closed to accrual) Gestational trophoblastic disease (GTD) Gallbladder cancer Small cell carcinoma of the ovary, hypercalcemic type PD-L1 amplified tumors Angiosarcoma High-grade neuroendocrine carcinoma (pancreatic neuroendocrine tumor [PNET] should be enrolled in Cohort 22; prostatic neuroendocrine carcinomas should be enrolled into Cohort 53). Small cell lung cancer is not eligible (closed to accrual) Treatment-emergent small-cell neuroendocrine prostate cancer (t-SCNC)

CABATEN is a multicohort phase II study of cabozantinib plus atezolizumab in advanced and progressive tumors from endocrine system. The primary objective is to assess the efficacy of cabozantinib plus atezolizumab combination by means of radiological objective response rate (ORR) evaluated following RECIST v1.1 criteria in advanced endocrine tumors. Endocrine tumors from different origins (thyroid, lung, pancreas and digestive tract, adrenal gland and paraganglia) are characterized by being remarkably vascular and expressing several growth factors including vascular endothelial growth factor (VEGF), platelet-derived growth factor (PDGF), insulin-like growth factor 1 (IGF-1), basic fibroblast growth factor (BFGF), and transforming growth factor (TGF)-α and -β. The (over) expression of some of these factors has been linked to poor prognosis. Cabozaninib, a VEGF inhibitor, in combination with atezolizumab, an inhibitor of PD-L1, may be active in endocrine tumors by overcoming the resistance to prior antiangiogenic drugs. The trial will include patients with advanced and refractory tumors of endocrine system and patients would be allocated to six different cohorts according to the following tumor types.

This pilot phase II trial studies how well cabozantinib s-malate works in treating patients with pheochromocytomas or paragangliomas that have spread from the primary site to other places in the body and cannot be removed by surgery. Cabozantinib s-malate may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth and by blocking the growth of new blood vessels necessary for tumor growth.