Symptoms and General Pathology

All Conditions

Neoplasms

Rare Diseases

Recurrence

Sarcoma

Desmoplastic Small Round Cell Tumor

Neoplasms, Connective and Soft Tissue

Neoplasms by Histologic Type

Soft Tissue Sarcoma

DNA analysis

RNA analysis

gene expression analysis

mutation analysis

nucleic acid sequencing

polymerase chain reaction

laboratory biomarker analysis

pharmacogenomic studies

Pooja Hingorani, MD

RATIONALE: Studying samples of blood and tumor tissue from patients with cancer in the laboratory may help doctors learn more about changes that occur in DNA and identify biomarkers related to cancer.

PURPOSE: This research study is studying genes in samples from younger patients with desmoplastic small round cell tumor registered on COG-D9902 or COG-ABTR01B1.

OBJECTIVES:

* To perform whole-exome sequencing on desmoplastic small round cell tumor (DSRCT) samples and their available matched normal samples to identify novel mutations, single nucleotide polymorphisms, or copy number changes associated with these tumors.
* To identify regions of interest that may be involved in the pathogenesis (including the region of EWSR1-WT1 translocation) from the whole-exome sequencing and perform detailed resequencing and transcriptone sequencing to further define the molecular aberrations at the RNA level.

OUTLINE: This is a multicenter study.

Archived tumor tissue samples are analyzed for DNA sequencing, effects of RNA on gene expression levels, novel RNA isoforms, splice variants, and translocations.

Studying Genes in Samples From Younger Patients With Desmoplastic Small Round Cell Tumor Registered on COG-D9902 or COG-ABTR01B1

Comprehensive Genome Sequencing of Desmoplastic Small Round Cell Tumors

National Cancer Institute (NCI)

Children's Oncology Group

Skin and Connective Tissue Diseases

Urinary Tract, Sexual Organs, and Pregnancy Conditions

Gland and Hormone Related Diseases

Respiratory Tract (Lung and Bronchial) Diseases

Ear, Nose, and Throat Diseases

Nervous System Diseases

Breast Neoplasms

Carcinoma

Carcinoma, Squamous Cell

Ovarian Neoplasms

Carcinoma, Ovarian Epithelial

Liposarcoma

Melanoma

Head and Neck Neoplasms

Histiocytoma, Malignant Fibrous

Carcinosarcoma

Mixed Tumor, Mullerian

Sarcoma, Endometrial Stromal

Fibrosarcoma

Histiocytoma

Histiocytoma, Benign Fibrous

Hemangiosarcoma

Laryngeal Diseases

Leiomyosarcoma

Rhabdomyosarcoma

Sarcoma, Synovial

Neurofibrosarcoma

Sarcoma, Alveolar Soft Part

Mesenchymoma

Endometrial Neoplasms

Breast Diseases

Skin Diseases

Neuroendocrine Tumors

Neuroectodermal Tumors

Neuroectodermal Tumors, Primitive

Neoplasms, Germ Cell and Embryonal

Neoplasms, Nerve Tissue

Nevus, Pigmented

Nevus

Nevi and Melanomas

Skin Neoplasms

Genital Diseases, Female

Genital Diseases

Urogenital Diseases

Female Urogenital Diseases

Pregnancy Complications

Female Urogenital Diseases and Pregnancy Complications

Genital Neoplasms, Female

Urogenital Neoplasms

Uterine Neoplasms

Uterine Diseases

Ovarian Cancer

Ovarian Epithelial Cancer

Undifferentiated Pleomorphic Sarcoma

Malignant Mixed Mullerian Tumor

Ovarian Carcinosarcoma

Endometrial Stromal Sarcoma

Laryngeal Cancer

Hypopharyngeal Cancer

Synovial Sarcoma

Malignant Peripheral Nerve Sheath Tumor

Epithelioid Sarcoma

Uterine Sarcoma

Alveolar Soft Part Sarcoma

Malignant Mesenchymoma

Metastatic Squamous Neck Cancer With Occult Primary

Neuroendocrine Tumor

Neuroepithelioma

BI 2536

high performance liquid chromatography

mass spectrometry

pharmacological study

Patrick Schoffski, MD, MPH

RATIONALE: BI 2536 may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth.

PURPOSE: This phase II trial is studying the side effects and how well BI 2536 works in treating patients with recurrent or metastatic solid tumors.

OBJECTIVES:

* Investigate if BI 2536 demonstrates antitumor activity in the selected tumor types.
* Further document its safety profile in the treated patient population.
* Describe the plasma concentration time-course following administration of a single administration of BI 2536 in patients with different tumor types using an appropriate population pharmacokinetic model.

OUTLINE: This is a multicenter study.

Patients receive BI 2536 IV over 1 hour on day 1. Treatment repeats every 21 days in the absence of disease progression or unacceptable toxicity.

Blood is collected periodically during study. Plasma samples are analyzed for pharmacokinetic studies by HPLC and tandem mass spectrometry.

After completion of study treatment, patients are followed every 3 months.

BI 2536 in Treating Patients With Recurrent or Metastatic Solid Tumors

Multicenter Parallel Phase II Trial of BI 2536 Administered as One Hour IV Infusion Every 3 Weeks in Defined Cohorts of Patients With Various Solid Tumors. A New Drug Screening Program of the EORTC Network of Core Institutions (NOCI)

European Organisation for Research and Treatment of Cancer - EORTC

Osteosarcoma

Chondrosarcoma

Solitary Fibrous Tumors

Hemangiopericytoma

Antineoplastic Agents

All Drugs and Chemicals

Anti-Infective Agents

Doxorubicin

Liposomal doxorubicin

Trabectedin

Anti-Bacterial Agents

Antibiotics, Antitubercular

Enzyme Inhibitors

Antineoplastic Agents, Alkylating

Alkylating Agents

Doxorubicin administered on day 1 every 3 weeks for a maximum of 6 cycles

Trabectedin administered on day 1 every 3 weeks at the dose of 1.3 mg/m² until progression

Trabectedin administered on day 1 every 3 weeks at the dose of 1.5 mg/m² over 24 hours until progression

doxorubicin hydrochloride

trabectedin

quality-of-life assessment

Nguyen Binh Bui, MD

James E. Butrynski, MD

RATIONALE: Drugs used in chemotherapy, such as doxorubicin hydrochloride and trabectedin, work in different ways to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. It is not yet known whether trabectedin is more effective than doxorubicin hydrochloride in treating patients with advanced or metastatic soft tissue sarcoma.

PURPOSE: This randomized phase II/III trial is studying the safety of trabectedin compared with doxorubicin hydrochloride and to see how well they work in treating patients with advanced or metastatic soft tissue sarcoma.

OBJECTIVES:

* To evaluate whether trabectedin given as first-line chemotherapy for patients with previously untreated advanced or metastatic malignant soft tissue sarcoma prolongs progression-free survival as compared to doxorubicin hydrochloride.
* To identify and validate biomarkers (including, but not limited to, XPG, BRCA1, RAD51, BRCA2, ATM and CHK1) of sensitivity to trabectedin in order to allow the selection of patients that benefit most from trabectedin treatment. (Optional translational research)

OUTLINE: This is a multicenter, phase IIB study followed by a phase III study. Patients are stratified according to institution, age at registration (\< 60 years old vs ≥ 60 years old), and presence of liver metastases (yes vs no).

* Phase IIB (step 1): Patients are randomized to 1 of 3 treatment arms.

  * Arm I: Patients receive doxorubicin hydrochloride IV on day 1. Treatment repeats every 3 weeks for 6 courses in the absence of disease progression or unacceptable toxicity.
  * Arm II: Patients receive trabectedin IV over 3 hours on day 1. Courses repeat every 3 weeks in the absence of disease progression or unacceptable toxicity.
  * Arm III: Patients receive trabectedin IV continuously over 24 hours on day 1. Courses repeat every 3 weeks in the absence of disease progression or unacceptable toxicity.

At the end of step 1, the best regimen of trabectedin will be determined. Patients receiving the non-selected trabectedin regimen ("losing arm") are offered to cross over in order to receive the selected regimen of trabectedin.

* Phase III (step 2): Patients are randomized to 1 of 2 treatment arms.

  * Arm I: Patients receive trabectedin IV on day 1 using the preferred regimen determined in step 1. Courses repeat every 3 weeks in the absence of disease progression or unacceptable toxicity.
  * Arm II: Patients receive doxorubicin hydrochloride IV on day 1. Treatment repeats every 3 weeks for 6 courses in the absence of disease progression or unacceptable toxicity.

Patients complete quality of life questionnaire (EORTC QLQ-C30 version 3) at baseline, at 6, 12, 24, and 36 weeks during study, and at the end of study.

Tumor tissue block obtained at diagnosis may be analyzed to identify and validate biomarkers of sensitivity to trabectedin and for tissue microarrays.

After completion of study therapy, patients are followed up at 1 month, every 6 or 12 weeks until disease progression, and every 12 weeks thereafter.

Doxorubicin Hydrochloride or Trabectedin in Treating Patients With Previously Untreated Advanced or Metastatic Soft Tissue Sarcoma

TRUSTS: A Phase IIB/III Multicenter Study Comparing the Efficacy of TRabectedin Administered as a 3-Hour or 24-Hour Infusion to Doxorubicin in Patients With Advanced or Metastatic Untreated Soft Tissue Sarcoma

Sarcoma Alliance for Research through Collaboration

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Studying Genes in Samples From Younger Patients With Desmoplastic Small Round Cell Tumor Registered on COG-D9902 or COG-ABTR01B1	NCT01430637	Sarcoma	DNA analysis RNA analysis gene expression... mutation analys... nucleic acid se... polymerase chai... laboratory biom... pharmacogenomic...	- 50 Years	Children's Oncology Group
BI 2536 in Treating Patients With Recurrent or Metastatic Solid Tumors	NCT00526149	Breast Cancer Endometrial Can... Head and Neck C... Melanoma (Skin) Ovarian Cancer Sarcoma	BI 2536 high performanc... mass spectromet... pharmacological...	18 Years -	European Organisation for Research and Treatment of Cancer - EORTC
BI 2536 in Treating Patients With Recurrent or Metastatic Solid Tumors	NCT00526149	Breast Cancer Endometrial Can... Head and Neck C... Melanoma (Skin) Ovarian Cancer Sarcoma	BI 2536 high performanc... mass spectromet... pharmacological...	18 Years -	European Organisation for Research and Treatment of Cancer - EORTC
Doxorubicin Hydrochloride or Trabectedin in Treating Patients With Previously Untreated Advanced or Metastatic Soft Tissue Sarcoma	NCT01189253	Sarcoma	doxorubicin hyd... trabectedin laboratory biom... quality-of-life...	18 Years -	European Organisation for Research and Treatment of Cancer - EORTC

Spots Global Cancer Trial Database for adult desmoplastic small round cell tumor