Neoplasms

All Conditions

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Rare Diseases

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Pituitary Diseases

Thyroid Neoplasms

Thyroid Diseases

Thyroid Nodule

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Goiter

Goiter, Nodular

Wilms Tumor

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Sex Cord-Gonadal Stromal Tumors

Pituitary Neoplasms

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Neoplasms by Histologic Type

Neoplasms, Connective and Soft Tissue

Endocrine System Diseases

Neuroectodermal Tumors, Primitive, Peripheral

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Female Urogenital Diseases and Pregnancy Complications

Kidney Diseases

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Endocrine Gland Neoplasms

Head and Neck Neoplasms

Brain Neoplasms

Central Nervous System Neoplasms

Nervous System Neoplasms

Lung Neoplasms

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Genital Neoplasms, Male

Genital Diseases

Genital Diseases, Male

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Genital Diseases, Female

Genital Neoplasms, Female

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Pineoblastoma

Rhabdomyosarcoma Embryonal

Pituitary Cancer

Pleuropulmonary Blastoma

Sertoli-leydig Cell Tumors

Gynandroblastoma

DICER1-related Pleuropulmonary Blastoma Cancer Predisposition Syndrome

Pineocytoma

Neuroepithelioma

Ovarian Cancer

Antineoplastic Agents

Antirheumatic Agents

All Drugs and Chemicals

Anti-Infective Agents

Cyclophosphamide

Doxorubicin

Liposomal doxorubicin

Vincristine

Ifosfamide

Dactinomycin

Isophosphamide mustard

Type I PPB is an early manifestation of this malignant disease, cured in some cases by surgery. Surgical guidelines are presented. It is unknown whether adjuvant chemotherapy improves cure rates for individuals with Type I PPB. If the treating physicians select adjuvant chemotherapy treatment, chemotherapy options include a 22-week regimen: 4 courses of vincristine, actinomycin D and cyclophosphamide (VAC) followed by 3 courses of vincristine and actinomycin D (VA). Therapy decisions are the responsibility of the treating institution.

Types II and III PPB are aggressive sarcomas. Surgery and chemotherapy are necessary in all cases. Surgical guidelines are presented. Many children with Types II or III PPB receive a single-arm multi-agent chemotherapy neo-adjuvant/adjuvant regimen of IVADo (ifosfamide, vincristine, actinomycin, doxorubicin) for 36 weeks. Second and possible 3rd look surgery may be considered for local control. Radiation therapy may be considered. Specific therapy decisions are the responsibility of the treating institution.

Type Ir (regressed) PPB is a unique, purely cystic tumor which lacks a primitive cell component. The International PPB/DICER1 Registry will enroll and follow participants with Type Ir PPB, regardless of age.

PPB and the associated conditions found in PPB families suggest a familial tendency to formation of tumors. The International PPB/DICER1 Registry for PPB, DICER1 and Associated Conditions study will enroll and follow participants who have the DICER1 gene mutations or conditions associated with PPB or DICER1.

Kris Ann P Schultz, MD

Paige HR Mallinger, MS

Anne K Harris, MPH

Pleuropulmonary blastoma (PPB) is a rare malignant neoplasm of the lung presenting in early childhood. Type I PPB is a purely cystic lesion, Type II is a partially cystic, partially solid tumor, Type III is a completely solid tumor. Treatment of children with PPB is at the discretion of the treating institution. This study builds off of the 2009 study and will also seek to enroll individuals with DICER1-associated conditions, some of whom may present only with the DICER1 gene mutation, which will help the Registry understand how these tumors and conditions develop, their clinical course and the most effective treatments.

PPB is a rare cancer of the lung presenting in early childhood, mostly commonly from birth to age \~72 months. PPB occurs within the lung or between the lung and the chest wall. There are three primary forms of PPB called Types I, II, and III PPB. PPB is related to an underlying change/mutation in a gene called DICER1 which impacts gene expression and cell growth. DICER1 mutations may also lead to the development of other tumors in children and adults.

The International PPB/DICER1 Registry offers information based on previous data from Registry participants and the medical literature and collaborative efforts with international rare tumor groups.

Retrospective and real-time central pathology review is encouraged. Therapy decisions remain at the discretion of the treating institution.

Children with Type I PPB require surgery and sometimes chemotherapy. Therapy decisions are the responsibility of the treating institution. Surgical guidelines are presented. It is unknown whether adjuvant chemotherapy improves cure rates for Type I PPB patients. Chemotherapy options include a 22-week regimen: 4 courses of vincristine, actinomycin D and cyclophosphamide (VAC) followed by 3 courses of vincristine and actinomycin D (VA).

Children with Types II and III PPB, require surgery, chemotherapy and sometimes radiation therapy. Many children with Types II or III PPB receive a single-arm multi-agent chemotherapy neo-adjuvant/adjuvant regimen of IVADo (ifosfamide, vincristine, actinomycin, doxorubicin) for 36 weeks. Second and possible 3rd look surgery may be considered for local control. Radiation therapy may be considered.

Tumor tissue, blood, urine, and cerebral spinal fluid (the latter only if a lumbar puncture is performed for clinical purposes) collected from enrolled individuals.

International PPB/DICER1 Registry

International Pleuropulmonary Blastoma/DICER1 Registry (for PPB, DICER1 and Associated Conditions)

The primary endpoint for statistical analysis will be time from start treatment to an event, defined as the occurrence of progression or recurrence of PPB, occurrence of a second malignant neoplasm, or death from any cause that is at least possibly related to the original disease or treatment.

The investigators will assess overall response to chemotherapy among participants with radiographically measurable tumor following initial surgery or biopsy.

The investigators will assess overall survival and time to death from any cause among participants.

Chemotherapy and surgery may have adverse effects on the quality of life outcomes. Multiple factors may impact quality of life for participants.This study will allow the investigators to assess the quality of life outcomes in participants with DICER1-related tumors and will compare outcomes to those with more common childhood cancers.

Chemotherapy and surgery may have adverse effects on cardiac outcomes. This study will allow the investigators to assess the cardiac outcomes as measured by ejection fraction and shortening fraction via echocardiogram of participants with DICER1-related tumors, and compare outcomes to those with more common childhood cancers.

Chemotherapy and surgery may have adverse effects on pulmonary outcomes. This study will allow the investigators to assess the pulmonary outcome of participants as ascertained by pulmonary function testing (forced vital capacity (FVC), FEV1(forced expiratory volume in 1 second)/FVC) with DICER1-related tumors, and compare outcomes to those with more common childhood cancers.

This protocol will include individuals with germline DICER1 mutations and will calculate incidence rates of specific neoplasms in this population

Washington University School of Medicine

ResourcePath, LLC

Beijing Children's Hospital

University of Cambridge

Emory University

Dana-Farber Cancer Institute

Phoenix Children's Hospital

Allina Health System

University of California, San Francisco

M.D. Anderson Cancer Center

University of Texas

Kaiser Permanente

UC Davis Children's Hospital

KK Women's and Children's Hospital

Louisiana State University Health Sciences Center Shreveport

Children's Healthcare of Atlanta

Dayton Children's Hospital

Akron Children's Hospital

Starship Children's Hospital of New Zealand

Ann & Robert H Lurie Children's Hospital of Chicago

Children's Hospitals and Clinics of Minnesota

Musculoskeletal Diseases

Ear, Nose, and Throat Diseases

Nutritional and Metabolic Diseases

Muscle Weakness

Paralysis

Paresis

Hypoparathyroidism

Vocal Cord Paralysis

Thyroid Cancer, Papillary

Hypocalcemia

Neurologic Manifestations

Parathyroid Diseases

Metabolic Diseases

Calcium Metabolism Disorders

Water-Electrolyte Imbalance

Laryngeal Diseases

Respiratory Tract Diseases

Otorhinolaryngologic Diseases

Vagus Nerve Diseases

Cranial Nerve Diseases

Adenocarcinoma

Adenocarcinoma, Papillary

Control (status/post-s/p total thyroidectomy-TT, without complication- demographics and BMI matched)

Experimental (s/p TT, with only vocal cord paralysis-VCP, uni or bilateral)

Experimental (s/p TT, with only hypocalcemia-H, transient or permanent)

Experimental (s/p TT, with both vocal cord paralysis-VCP and hypocalcemia-H);

Subgroups:

4.1. VCP (Permanent) + H (Permanent) 4.2. VCP (Transient) + H (Transient) 4.3. VCP (Permanent) + H (Transient) 4.4. VCP (Transient) + H (Permanent)

Please answer:

* Improvement in hypocalcemia also make a positive effect on voice? (any objective sign? Ca? PTH?)
* Return of voice is parallel with the improvement in hypocalcemia? (any objective sign? Ca? PTH?

Patients with thyroid diseases either benign (e.g. multinodular goitre) or malign (e.g. thyroid carcinoma) will be prepared for total thyroidectomy procedure and will be enrolled.

Total thyroidectomy

Ethem UNAL, MD, PhD, USMLE & IFSO-Certified, Board CSS

Kadir YILDIRAK, MD

Ethem UNAL, MD, PhD, USMLE & IFSO- Certified Board CSS

Kadir M YILDIRAK, MD

Sema YUKSEKDAG, MD

In the present study, the severity of recurrent laryngeal nerve injury (RLNI) and hypocalcemia (H) will be followed-up and the probable interrelation between them will be proposed considering the clinical situation of patients, e.g. improvement in hypocalcemia also make a positive effect on voice? (any objective sign? Ca? PTH?), return of voice is parallel with the improvement in hypocalcemia? Postoperative calcium (Ca), parathyroid hormone (PTH), regular vocal cord evaluations by ear-nose-throat (ENT) exams, deterioration-stability-improvement of clinical symptoms regarding both Ca metabolism and vocal cord function will be noted at regular intervals (postoperative day 1-3-first, weekly control/first month, monthly/first 6-month, 3-monthly/6-12 months) at outpatient controls. Serum Ca, PTH, ENT evaluation of vocal cords-noted.

Total thyroidectomy is currently the preferred surgical treatment modality for both thyroid carcinomas and benign disorders such as multinodular goitre, since it minimizes the risk of recurrence and eliminates the complication risks of repeat or completion surgery. Vocal cord paralysis due to injury to recurrent laryngeal nerve (RLN) is the most dreaded complication of total thyroidectomy. The reported incidence of temporary RLN injury (RLNI) varies between 0 and 12 %, while the incidence of permanent RLNI has been reported to be much lower (0-3.5 %). In case of bilateral RLNI, respiratory distress and aspiration can develop rapidly and may result in mortality. Therefore, all precautions including close monitoring and tracheostomy should be undertaken without any delay. The best known technique to avoid injury to RLN is meticulous dissection of the nerve throughout its anatomic pathway. However, functional impairment of RLN is not visible macroscopically and intraoperative nerve monitoring (IONM) has been developed to monitor the nerve to avoid unnecessary dissection. Meticulous hemostasis can be achieved with harmonic sealing instrument, since improper hemostasis is known to increase the risk of RLNI. Despite the lack of evidence to support an advantage of IONM over the standard anatomic dissection of RLN, surgeons have adopted it in increasing ratios. The second most feared compliation of thyroidectomy is iatrogenic hypocalcemia. Transient symptomatic hypocalcemia after total thyroidectomy occurs in approximately 7% to 25% of cases, but permanent hypocalcemia is less common (0.4% to 13.8%). Size and invasion of tumor, operative trauma and vascular compromise determines the severity of symptoms.

Post-thyroidectomy Vocal Cord Paralysis Along With Hypocalcemia: STROBE - Guided Prospective Cohort

Post-thyroidectomy Vocal Cord Paralysis Along With Hypocalcemia: Prospective, Matched-randomized Observational Cohort Compatible With STROBE Guidelines

Study is open to high-volume INTERNATIONAL endocrine surgery centers, eager to join to present STROBE compatible observational study

Available upon email requests@drethemunal@gmail.com

Any improvement recorded? VCP? H? vice versa

Umraniye Education and Research Hospital

MD, PhD, USMLE & IFSO-Certified, BCSS, Associated Professor of General Surgery and Surgical Oncology

Eye Diseases

Immune System Diseases

Graves Disease

Hyperthyroidism

Exophthalmos

Orbital Diseases

Autoimmune Diseases

Patients will undergo thyroidectomy according to the guidelines American Thyroid Association (ATA), Randolph.

The following assessments will be performed within 10 days before the thyroidectomy procedure:

* informed consent;
* personal data and medical records;
* registration of medical therapy and post-procedural pharmacological protocol
* surgical check;
* supra-aortic trunks EchocolorDoppler;
* pre-procedural blood sampling.

The following assessments will be performed after 30 days, 6 months and 12 months from the thyroidectomy procedure:

* surgical checks;
* registration of drug therapy;
* recording of all adverse events that occurred during hospitalization
* supra-aortic trunks EchocolorDoppler and cIMT measurement;
* blood sampling.

Experimental

Renato Casana, MD

Daniele Bissacco, MD

Gianlorenzo Dionigi, MD, Prof

Antonella Pino, MD

Chiara Malloggi, PhD

The evaluation of carotid intima-media thickness (cIMT) has become a possible predictor of the future risk of cardiovascular diseases (CVD). Evidence to date shows that cIMT augmentation is correlated with the extent of atherosclerosis in the coronary arteries and with other risk factors for CVD. More recently, several studies have evaluated the association of cIMT with stroke, to determine whether this measure can also predict future cardiovascular events.

Although the published evidence is scarce and fragmentary, the cIMT retains an increasingly interesting role as a marker of atherosclerotic pathology.

The purpose of the study is to analyze cIMT in patients undergoing thyroidectomy to evaluate a possible correlation between the cIMT variation after the procedure and the cause of the thyroid disease, the levels of thyroid hormones, and the lipid or other markers of atherosclerosis levels.

The primary endpoint will be the variation of the measure of the cIMT before and after surgery.

Secondary endpoints will be:

* major cardiovascular adverse events (death, major stroke, minor stroke, transient ischemic attack) in the short-term period (≤30 days after the procedure);
* major cardiovascular adverse events (death, major stroke, minor stroke, transient ischemic attack) in the long-term period (\>30 days after the procedure);
* the technical success of the procedure;
* the rate of postoperative complications. To date, there are no data that differentiate patients based on the causes of thyroid disease. The results of the present study will allow for correlating the variation of the cIMT to the causes of thyroid disease, the levels of thyroid hormones, and the levels of lipid and other markers of atherosclerosis.

Results from the present study may provide insights into possible areas of quality improvement. It may also influence the economic impact associated with carotid revascularization techniques, in terms of hospital charges and discharges to skilled nursing and rehabilitation facilities.

Prospective, Observational Study on the Carotid Intima-media Thickness in Patients Undergoing Thyroid Surgery

Prospective, Observational Study on the Investigation of the Carotid Intima-media Thickness in Patients Undergoing Thyroid Surgery

cIMT is a measurement of the thickness of tunica intima and tunica media, the innermost two layers of the wall of an artery. The measurement is made by external ultrasound according to the recommendation of the Joint Carotid Intima-Media Thickness and Plaque Area Measurement in Ultrasound for Cardiovascular/Stroke Risk Monitoring.

Incidence of death, major stroke, minor stroke, transient ischemic attack

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
International PPB/DICER1 Registry	NCT03382158	Pleuropulmonary... Sertoli-Leydig ... DICER1 Syndrome Cystic Nephroma Wilms Tumor Pineoblastoma Renal Sarcoma Nodular Hyperpl... Nasal Chondrome... Ciliary Body Me... Neuroblastoma Pituitary Cance... Embryonal Rhabd... Ovarian Sarcoma Gynandroblastom... Thyroid Carcino... Embryonal Rhabd... Embryonal Rhabd... Embryonal Rhabd...		-	Children's Hospitals and Clinics of Minnesota
Post-thyroidectomy Vocal Cord Paralysis Along With Hypocalcemia: STROBE - Guided Prospective Cohort	NCT04396912	Iatrogenic Hypo... Iatrogenic Hypo... Vocal Cord Para... Vocal Cord Pare... Vocal Cord; Inj... Thyroid Cancer,... Thyroid Cancer Multinodular Go... Thyroid Neoplas... Thyroid Nodule Calcium Deficie... PTH	Total thyroidec...	17 Years -	Umraniye Education and Research Hospital
International PPB/DICER1 Registry	NCT03382158	Pleuropulmonary... Sertoli-Leydig ... DICER1 Syndrome Cystic Nephroma Wilms Tumor Pineoblastoma Renal Sarcoma Nodular Hyperpl... Nasal Chondrome... Ciliary Body Me... Neuroblastoma Pituitary Cance... Embryonal Rhabd... Ovarian Sarcoma Gynandroblastom... Thyroid Carcino... Embryonal Rhabd... Embryonal Rhabd... Embryonal Rhabd...		-	Children's Hospitals and Clinics of Minnesota
Prospective, Observational Study on the Carotid Intima-media Thickness in Patients Undergoing Thyroid Surgery	NCT05774535	Plummer Disease Multinodular Go... Graves Disease Hyperthyroidism Euthyroid Goite... Thyroid Cancer Basedow Disease	Experimental	18 Years - 100 Years	Istituto Auxologico Italiano
Parathyroid Reimplantation in Forearm Subcutaneous Tissue During Thyroidectomy: a Simple Way to Avoid Ipoparathyroidism and Evaluate Graft Function	NCT02194920	Thyroid Goiter Thyroid Carcino... Multinodular Go... Graves Diseases	Thyroidectomy a...	-	University of Roma La Sapienza
Prospective, Observational Study on the Carotid Intima-media Thickness in Patients Undergoing Thyroid Surgery	NCT05774535	Plummer Disease Multinodular Go... Graves Disease Hyperthyroidism Euthyroid Goite... Thyroid Cancer Basedow Disease	Experimental	18 Years - 100 Years	Istituto Auxologico Italiano
Prospective, Observational Study on the Carotid Intima-media Thickness in Patients Undergoing Thyroid Surgery	NCT05774535	Plummer Disease Multinodular Go... Graves Disease Hyperthyroidism Euthyroid Goite... Thyroid Cancer Basedow Disease	Experimental	18 Years - 100 Years	Istituto Auxologico Italiano

Spots Global Cancer Trial Database for multinodular goiter