Patients must have histologically confirmed bone or soft tissue sarcoma by central pathology review\r\n* Patients must have histologically confirmed liposarcoma (LPS) (only dedifferentiated and pleomorphic; well differentiated not eligible), undifferentiated pleomorphic sarcoma (UPS)/malignant fibrous histiocytoma (MFH), or gastrointestinal stromal tumor (GIST)
Histologic documentation: Eligible patients must have histopathologically confirmed sarcoma of one of the subtypes listed, by central review
One of the following chemosensitive histologies as defined in the World Health Organization (WHO) classification of soft tissue tumors (with some evidence of good response to chemoradiation and of sufficient high risk of metastases, or clear evidence of metastases):\r\n* Unclassified soft tissue sarcomas that are too undifferentiated to be placed in a specific pathologic category in the WHO classification (often called “undifferentiated soft tissue sarcoma” or “soft tissue sarcoma not otherwise specified [NOS]”)\r\n* Synovial sarcoma\r\n* Angiosarcoma of soft tissue\r\n* Adult fibrosarcoma\r\n* Mesenchymal (extraskeletal) chondrosarcoma\r\n* Leiomyosarcoma\r\n* Liposarcoma (excluding myxoid liposarcoma)\r\n* Undifferentiated pleomorphic sarcoma\r\n* Embryonal sarcoma of the liver
Patients with the following histologies are only eligible for the chemotherapy cohort and cannot enroll on the non-chemotherapy cohort:\r\n* Unclassified soft tissue sarcomas that are too undifferentiated to be placed in a specific pathologic category in the WHO classification (often called “undifferentiated soft tissue sarcoma” or “soft tissue sarcoma NOS”) in patients < 30 years of age\r\n* Synovial sarcoma\r\n* Embryonal sarcoma of the liver
Patients with any size of grade 2 or 3 of the following “intermediate (rarely metastasizing)” or “malignant” tumors, as defined in the WHO classification of soft tissue tumors for which we have consensus data of chemotherapy-resistance are eligible only for the non-chemotherapy cohort:\r\n* So-called fibrohistiocytic tumors - plexiform fibrohistiocytic tumor, giant cell tumor of soft tissues\r\n* Fibroblastic/myofibroblastic tumors - solitary fibrous tumor, malignant solitary fibrous tumor, inflammatory myofibroblastic tumor, low grade myofibroblastic sarcoma, myxoinflammatory fibroblastic sarcoma, atypical myxoinflammatory fibroblastic tumor, myxofibrosarcoma, low grade fibromyxoid sarcoma, sclerosing epithelioid fibrosarcoma\r\n* Tumors of uncertain differentiation - epithelioid sarcoma, alveolar soft part sarcoma, clear cell sarcoma of soft tissue, angiomatoid fibrous histiocytoma, ossifying fibromyxoid tumor, myoepithelioma, myoepithelial carcinoma, extraskeletal myxoid chondrosarcoma, neoplasms with perivascular epithelioid cell differentiation (PEComa), intimal sarcoma, atypical fibroxanthoma, mixed tumor NOS, phosphaturic mesenchymal tumor, malignant ossifying fibromyxoid tumor, malignant mixed tumor, malignant phosphaturic mesenchymal tumor\r\n* Chondro-osseous tumors - extraskeletal osteosarcoma\r\n* Pericytic (perivascular) tumors - malignant glomus tumor\r\n* Nerve sheath tumors - malignant peripheral nerve sheath tumor, malignant granular cell tumor, epithelioid malignant peripheral nerve sheath tumor, malignant Triton tumor\r\n* Undifferentiated sarcomas (with a specific pathologic category in the WHO classification) - undifferentiated round cell sarcoma, undifferentiated epithelioid sarcoma, undifferentiated spindle cell sarcoma
Patients must have recurrent or refractory disease, or newly diagnosed disease with no known curative therapy or therapy proven to prolong survival with an acceptable quality of life; patients must have had histologic verification of one of the malignancies listed below at original diagnosis or at relapse:\r\n* Ewing sarcoma \r\n* Rhabdomyosarcoma (RMS)\r\n* Non-rhabdomyosarcoma soft tissue sarcomas (STS) including microphthalmia transcription factor associated STS (alveolar soft part sarcoma [ASPS] and clear cell sarcoma [CCS])\r\n* Osteosarcoma\r\n* Wilms tumor \r\n* Rare tumors \r\n** Medullary thyroid carcinoma (MTC)\r\n** Renal cell carcinoma (RCC)\r\n** Hepatocellular carcinoma (HCC)\r\n** Hepatoblastoma \r\n** Adrenocortical carcinoma\r\n** Pediatric solid tumors (including central nervous system [CNS] tumors) with known molecular alterations in the targets of XL184 (i.e., MET amplification, overexpression, activating mutation, MET translocation, MET exon skipping mutations, activating RET mutations, RET rearrangement, overexpression or activation of AXL); documentation of the alteration from a Clinical Laboratory Improvement Act (CLIA) certified laboratory will be required
Subjects with soft tissue sarcoma must have radiographic evidence of progression within the previous 3-4 months and must have received, or been intolerant to, prior treatment with an anthracycline +/- olaratumab or ifosfamide;
Patients must have had histologic verification of one of the malignancies listed below at original diagnosis or at time of relapse\r\n* Ewing sarcoma/peripheral primitive neuroectodermal tumor (PNET)\r\n* Osteosarcoma\r\n* Rhabdomyosarcoma\r\n* Non-rhabdomyosarcoma soft tissue sarcoma \r\n* Desmoplastic small round cell tumor\r\nNote: Patients with known involvement of the central nervous system (CNS) by malignancy will be included if there is no evidence of active bleeding or intratumoral hemorrhage on radiographic imaging
Have histologically or cytologically confirmed Kaposi's sarcoma or gastrointestinal stromal tumor (GIST).
Soft tissue sarcoma subtypes except GIST, desmoid tumors and pleomorphic rhabdomyosarcoma
Sarcoma
Diagnosis of Ewing sarcoma, rhabdomyosarcoma, osteosarcoma, non-rhabdomyosarcoma soft tissue sarcoma, Wilms tumor or other primary renal tumor (including clear cell and rhabdoid)
Presence of measurable disease as defined by the RECIST version 1.1 Cohort 2: Patient Population: Relapsed/Refractory Soft Tissue Sarcoma or Non-Kit GIST
Histologically confirmed diagnosis of advanced, unresectable, or metastatic soft tissue sarcoma not amenable to curative treatment with surgery or radiotherapy excluding: chondrosarcoma, neuroblastoma, osteosarcoma, embryonal rhabdomyosarcoma, or Kaposi sarcoma.
For patients with histiocytic sarcoma, interdigitating dendritic cell sarcoma, or follicular dendritic cell sarcoma only: disease that is not amenable to surgical resection and/or radiation therapy with curative intent
For histological specific cohorts, patients must have confirmed metastatic and/or locally advanced osteosarcoma, chondrosarcoma, undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma/high grade myxofibrosarcoma, vascular sarcoma, alveolar soft part sarcoma (ASPS), small blue round cell, or leiomyosarcoma (LMS) by the enrolling institution.
Patients must have had histologic verification of one of the malignancies listed below at original diagnosis or at relapse - a) Rhabdomyosarcoma, b) Non-rhabdomyosarcomatous Soft Tissue Sarcoma (including desmoplastic small round cell tumor), c) Ewing Sarcoma / Peripheral Primitive Neuroectodermal Tumor (PNET), d) Osteosarcoma, e) Neuroblastoma (Measurable), f) Neuroblastoma (Evaluable), g) Hepatoblastoma.
Stage 1: Histologically confirmed well-differentiated or de-differentiated liposarcoma; if stage 1 of the Simon II stage design fails to meet its endpoint for liposarcoma patients, an additional 16 patients will be enrolled, composed of 4 each of malignant peripheral nerve sheath tumor (MPNST), synovial sarcoma, alveolar rhabdomyosarcoma and alveolar soft part sarcoma (otherwise, an additional 16 patients with well-differentiated or de-differentiated liposarcoma would be enrolled); pathology review occurs at the center enrolling the patient on this trial
Soft-tissue sarcoma [excluding gastrointestinal stromal tumors (GIST)].
Sarcoma
INCLUSION - PROCUREMENT: Diagnosis of refractory or metastatic GD2-positive sarcoma not responsive to standard treatment; (patients with osteosarcoma do not require GD2 testing of their tumor)
INCLUSION - TREATMENT: Diagnosis of refractory or metastatic GD2-positive sarcoma not responsive to standard treatment; (patients with osteosarcoma do not require GD2 testing of their tumors)
Non-epithelial breast malignancies such as sarcoma or lymphoma.
Patients with gastrointestinal stromal tumor (GIST) tumors and Kaposi’s sarcoma are excluded
The participant is diagnosed with gastrointestinal stromal tumor (GIST) or Kaposi sarcoma.
Have an intermediate- or high-grade soft tissue sarcoma according to French Federation of Cancer Centers (FNCLCC) criteria
Has one of the following sarcoma subtypes where neoadjuvant chemotherapy is established as practice at our institution: extra-skeletal Ewing’s sarcoma, embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma (pleomorphic rhabdomyosarcoma is allowed, bone sarcomas including osteosarcoma, Ewings sarcoma and chondrosarcoma are not allowed)
Adult subjects with treatment naie primary or locally recurrent dedifferentiated liposarcoma (DDLPS) of the retroperitoneum or undifferentiated pleomorphic sarcoma (UPS) of the trunk or extremity will be eligible for inclusion in this study only if all of the following criteria apply.
Other terms for undifferentiated pleomorphic sarcoma (UPS) may include, but are not limited to: pleomorphic undifferentiated sarcoma, unclassified spindle cell sarcoma, spindle cell sarcoma not otherwise specified, pleomorphic spindle cell sarcoma, pleomorphic fibroblastic sarcoma, undifferentiated high-grade pleomorphic sarcoma, pleomorphic sarcoma with prominent inflammation, pleomorphic sarcoma with giant cells, malignant fibrous histiocytoma (including storiform-pleomorphic and inflammatory subtypes), fibrosarcoma, and myxofibrosarcoma (at least intermediate grade; located deep to the fascia in muscle).
Prior radiation therapy for sarcoma in the same area.
Are diagnosed with gastrointestinal stromal tumor or Kaposi sarcoma.
Phase 2: Participants must be diagnosed with histologically confirmed rhabdomyosarcoma (RMS), non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) or ewing sarcoma (EWS) which is relapsed or refractory having received at least 1 prior therapy, including primary treatment.
To limit the heterogeneity of the tumor population and reduce the risk of masking any clinical effect of the treatment regimen, multiple categories of STS subtypes will be excluded:\r\n* Patients with primarily bone-based sarcomas that can occur in the soft tissues, such as: \r\n** Extraskeletal Ewing sarcoma\r\n** Extraskeletal osteosarcoma\r\n** Peripheral chordoma\r\n** Extraskeletal myxoid chondrosarcoma\r\n** Mesenchymal chondrosarcoma\r\n* Patients with predominantly low-grade STS, such as:\r\n** Solitary fibrous tumor / hemangiopericytoma\r\n** Well-differentiated liposarcoma\r\n** Dermatofibrosarcoma protuberans\r\n** Kaposi’s sarcoma\r\n* Pediatric-type STS such as rhabdomyosarcoma\r\n* Gastrointestinal stromal tumors (GIST)
Have had a histologic diagnosis of osteosarcoma, Ewing sarcoma, or rhabdomyosarcoma or non-rhabdomyosarcoma soft tissue sarcoma either at diagnosis or relapse\r\n* Patients must have experienced relapse after front-line therapy, or have had documented disease progression during front-line therapy
Pathologically confirmed diagnosis of intermediate or high-grade soft tissue sarcoma for which single-agent doxorubicin is appropriate therapy, including but not limited to:\r\n* Synovial sarcoma\r\n* Fibrosarcoma\r\n* Undifferentiated sarcoma\r\n* Liposarcoma\r\n* Leiomyosarcoma\r\n* Angiosarcoma\r\n* Malignant peripheral nerve sheath tumor\r\n* Pleomorphic rhabdomyosarcoma\r\n* Myxofibrosarcoma\r\n* Epithelioid sarcoma\r\n* Undifferentiated pleomorphic sarcoma
Histologic diagnosis for which single-agent doxorubicin is NOT appropriate therapy, including but not limited to:\r\n* Alveolar or embryonal rhabdomyosarcoma\r\n* Ewings sarcoma or primitive neuroectodermal tumor (PNET)\r\n* Osteosarcoma\r\n* Gastrointestinal stromal tumor (GIST)
Patients must have a diagnosis of a locally advanced or metastatic sarcoma that is progressing; the following subtypes (considered genomically complex) will be eligible: leiomyosarcoma (well differentiated or poorly differentiated), undifferentiated pleomorphic sarcoma, myxofibrosarcoma, pleomorphic rhabdomyosarcoma, pleomorphic liposarcoma, malignant peripheral nerve sheath tumor, angiosarcoma or extraskeletal osteosarcoma; other potentially genomically complex soft tissue sarcoma (STS) subtypes may be included on a case-by-case basis after discussion with the principal investigator
Non-epithelial breast malignancies such as sarcoma/lymphoma
Has one of the following sarcoma subtypes where combining anthracyclines with other chemotherapies is established as the standard of care: osteosarcoma, Ewings sarcoma, embryonal rhabdomyosarcoma, alveolar rhabdomyosarcoma
Non-epithelial breast malignancies such as sarcoma or lymphoma
Histologically or cytologically confirmed sarcoma that fall into one of the following categories;  patients with low-grade tumors are eligible if there is definite evidence of metastasis or progression (defined as at least a 10% increase in the cumulative sum of the longest diameters within a 3 month period)\r\n* Adipocytic tumors (well-differentiated/dedifferentiated liposarcoma, myxoid liposarcoma, pleomorphic liposarcoma)\r\n* Vascular tumors (leiomyosarcoma, angiosarcoma)\r\n* Undifferentiated pleomorphic sarcoma\r\n* Synovial sarcoma\r\n* Osteosarcoma\r\n* Other sarcoma histologies
Must have received and have progressed, are refractory or intolerant to standard therapy appropriate for the specific sarcoma subtype, if there is a standard therapy for the subtype (i.e. progressing well-differentiated liposarcoma, clear cell sarcoma etc do not require prior therapy)
Histologically confirmed soft tissue sarcoma of the extremity/trunk
Non epithelial breast malignancies such as sarcoma or lymphoma
Histologically confirmed grade 2 or 3 soft tissue sarcoma that is unresectable or metastatic; surgery for primary or metastatic disease after chemotherapy following a response is allowed; patients with the following tumor types are eligible:\r\n* Undifferentiated pleomorphic sarcoma\r\n* Leiomyosarcoma\r\n* Malignant fibrous histiocytoma\r\n* Liposarcoma (myxoid/round cell, pleomorphic or dedifferentiated)\r\n* Synovial sarcoma\r\n* Myxofibrosarcoma\r\n* Angiosarcoma\r\n* Fibrosarcoma\r\n* Malignant peripheral nerve sheath tumor\r\n* Epithelioid sarcoma\r\n* Unclassified high-grade sarcoma (not otherwise specified)\r\n* Soft tissue sarcoma for which treatment with an anthracycline is appropriate at the approval of the principal investigator (PI)
Patients must have histologically or cytologically confirmed advanced/metastatic liposarcoma, osteogenic sarcoma, Ewing/Ewing-like sarcoma of soft tissue or bone, fusion-positive alveolar rhabdomyosarcoma or embryonal rhabdomyosarcoma/fusion-negative alveolar rhabdomyosarcoma
For Cohort 6 (subjects with epithelioid sarcoma undergoing mandatory tumor biopsy):
Morphology and immunophenotypic panel consistent with epithelioid sarcoma (e.g., CD34, EMA, Keratin, and INI1)
Histologically confirmed diagnosis of locally advanced STS that is unresectable with clear wide margins, for which preoperative radiotherapy is considered appropriate\r\n* Examples:\r\n** Resectable stage IIB, III, and IV disease that are not suitable for surgically resection alone due to inability to achieve clear margins\r\n** Including metastatic (stage IV) disease for which radiotherapy and surgical resection are indicated\r\n** Except certain histologic subtypes: gastrointestinal stromal tumor (GIST), Desmoid, Ewing sarcoma, Kaposi sarcoma, and bone sarcomas
Certain histologic subtypes: GIST, Desmoid, Ewing sarcoma, Kaposi sarcoma, and bone sarcomas
Documentation of Kirsten rat sarcoma viral oncogene homolog (K-Ras) mutational status
Non-epithelial breast malignancies such as sarcoma or lymphoma
Histologically or cytologically-confirmed Ewing sarcoma, rhabdomyosarcoma, neuroblastoma, or osteosarcoma
Patients must have histologically confirmed soft tissue sarcoma with evidence of metastatic or unresectable disease
The following specific histologic subtypes of soft tissue sarcomas will be excluded: gastrointestinal stromal tumor (GIST), Kaposi’s sarcoma, mesothelioma, dermatofibrosarcoma, chordoma, alveolar soft-part sarcoma; also, all bone sarcomas are excluded including Ewing’s sarcoma, osteosarcoma, GIST, low grade chondrosarcoma, and chordoma
Non-epithelial malignancies such as sarcoma or lymphoma.
Histologically confirmed diagnosis of metastatic or unresectable soft tissue sarcoma, excluding gastrointestinal stromal tumors, Kaposi‘s sarcoma, Ewing‘s family of tumors, and embryonal or alveolar rhabdomyosarcoma
Patients with concurrent Kaposi sarcoma requiring immediate cytotoxic chemotherapy
No prior radiation therapy for retroperitoneal sarcoma is allowed
Participants with sarcoma of head and neck, lung, heart or extremity origin; or histopathology demonstrating rhabdomyosarcoma, extraosseous primitive neuroectodermal tumor (PNET) soft tissue Ewing's sarcoma, osteosarcoma, Kaposi's sarcoma, angiosarcoma, aggressive fibromatosis (desmoid tumor), or dermatofibrosarcoma protuberans or chondrosarcoma other than extraskeletal chondrosarcoma; or well differentiated liposarcoma where the target volume cannot be adequately distinguished from the normal retroperitoneal fat are excluded
Non-epithelial breast malignancies such as sarcoma or lymphoma
Diagnosis of refractory HER2-positive sarcoma or metastatic HER2-positive sarcoma, not treatable by surgical resection
Diagnosis of refractory HER2-positive sarcoma or metastatic HER2-positive sarcoma, not treatable by surgical resection and with disease progression after receiving at least one prior systemic therapy
Phase II: patients must have histologically verified tumor at initial diagnosis and radiologically or histologically confirmed status at inclusion as indicated in the following: neuroblastoma, osteosarcoma, rhabdomyosarcoma or Ewing sarcoma.
Diagnosis: Histologically confirmed rhabdomyosarcoma (RMS), non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) (Grade 2 or 3), or Ewing sarcoma (EWS) which is relapsed or refractory (failed front line therapy)
Histopathology other than adenocarcinoma, e.g., sarcoma, lymphoma, carcinoid.
Histological diagnosis of synovial sarcoma
Histologically confirmed Ewing's Sarcoma Family of Tumors (ESFT).
Histopathologic documentation of synovial sarcoma or myxoid liposarcoma with the diagnosis of advanced or recurrent disease.
All sites of disease must be resectable or borderline resectable as assessed by a surgical oncologist with experience in retroperitoneal sarcoma resection after discussion in our institutional multidisciplinary sarcoma tumor board conference
Isolated myeloid sarcoma (meaning, patients must have blood or marrow involvement with AML to enter the study)
Excluded tumor types\r\n* Melanoma\r\n* Bone sarcomas\r\n* Soft tissue sarcomas, including angiosarcoma, primary cutaneous leiomyosarcoma, dermatofibrosarcoma protuberans\r\n* Leukemias\r\n* Myeloid sarcoma, leukemia cutis, and chloroma\r\n* Hodgkin’s lymphoma\r\n* B cell lymphoma
Patients must have histologically confirmed grade 2 or 3 tumors by the French Federation of Cancer Centers Sarcoma Group (FNCLCC) sarcoma grading system
Patients must have a sarcoma in the extremity or trunk in location, which is accessible to direct or ultrasound guided injections
Patient eligibility will be based on a diagnosis of Ewing sarcoma or PNET by institutional pathologist
Patients with pathologic diagnoses other than Ewing sarcoma will be excluded
Patients diagnosed with Ewing Sarcoma as a second malignant neoplasm are not eligible if they have received chemotherapy or radiation for the treatment of their primary malignancy
Patients with histology consisting of endometrial stromal sarcoma, leiomyosarcoma or malignant mixed Mullerian tumor (MMMT or carcinosarcoma)
Epithelioid sarcoma
Synovial sarcoma with SS18-SSX rearrangement
For subjects with synovial sarcoma only, the following test results must be available: Morphology consistent with synovial sarcoma, and cytogenetics or FISH and/or molecular confirmation (e.g., DNA sequencing) of SS18 rearrangement t(X;18)(p11;q11) For Dose Expansion Only:
Epithelioid sarcoma
Cohort 4 - Tumor types eligible for Cohorts 1 through 3 or synovial sarcoma with SS18-SSX rearrangement
For subjects with synovial sarcoma with SS18-SSX rearrangement (in Cohort 4 only) - have the following test results available:
Morphology consistent with synovial sarcoma, and
Locally advanced, relapsed, or metastatic sarcoma with measurable tumor burden following therapy, as defined by RECIST v1.1; the total of all lesions must be ?12 cm (for synovial sarcoma) or ?15 cm (for myxoid/round cell liposarcoma [MRCL]).
Tumor histology consistent with synovial sarcoma or MRCL.
Tumor histology consistent with one of the following: In Part 1, Dose Escalation - melanoma, NSCLC, ovarian cancer (including fallopian tube carcinoma), or sarcoma (any subtype). In Part 2, Patient Expansion - NSCLC, ovarian cancer (including fallopian tube carcinoma), or the sarcoma subtypes, synovial sarcoma or myxoid/round cell liposarcoma
Histologically confirmed diagnosis of nonresectable or metastatic soft tissue sarcoma; the following histologies are excluded: embryonal rhabdomyosarcoma, chondrosarcoma, osteosarcoma, Ewing tumors, primitive neuroectodermal tumors, gastrointestinal stromal tumors, dermatofibrosarcoma protuberans, inflammatory myofibroblastic sarcoma, and mixed mesodermal tumors of the uterus
Histologically confirmed Ewing sarcoma
Evidence of Ewing sarcoma translocation by FISH or RT-PCR.
Histologically confirmed unresectable soft tissue sarcoma (i.e., non-GIST, non-adipocytic) that has progressed following treatment with chemotherapy. Prior pazopanib is allowed if the drug was not discontinued for toxicity ( Phase 1 only)
For Part C: All participants must have histological evidence of advanced or metastatic specific subtypes of soft tissue sarcoma.
Colon cancer other than adenocarcinoma (e.g., sarcoma, lymphoma, carcinoid).
Non-epithelial breast malignancies such as sarcoma or lymphoma
Histologically confirmed diagnosis of advanced unresectable or metastatic soft tissue sarcoma not amenable to curative treatment with surgery or radiotherapy. Participants with Kaposi's sarcoma and gastrointestinal stromal tumors (GIST) will be excluded. Note: Evidence of disease progression is required for participants that are not newly diagnosed.
Diagnosis of GIST or Kaposi sarcoma.
Previously untreated Ewing sarcoma and rhabdomyosarcoma
Current evidence of GIST, alveolar soft part sarcoma, or dermatofibrosarcoma
Histologically confirmed, soft-tissue sarcoma: excluding rhabdomyosarcoma (pleomorphic rhabdomyosarcoma patients are eligible), Ewing’s, primitive neuroectodermal tumor (PNET), osteosarcoma, or gastrointestinal stromal tumor
Patients with any of the following sarcoma histologic subtypes will not be eligible for participation:\r\n* Alveolar soft-part sarcoma\r\n* Chondrosarcoma\r\n* Dermatofibrosarcoma\r\n* Ewing sarcoma\r\n* Gastrointestinal stromal tumor (GIST)\r\n* Kaposi sarcoma (non-human immunodeficiency virus [HIV] and HIV related disease)\r\n* Mixed mesodermal tumor/carcinosarcoma\r\n* Low grade (grade 1) sarcomas\r\n* Rhabdomyosarcoma (embryonal, alveolar)\r\n* Interdigitating dendritic sarcoma\r\n* Giant cell tumor of the bone
Histologically confirmed diagnosis of unresectable, recurrent, and/or metastatic high grade soft-tissue or bone sarcoma of one of the following subtypes: soft tissue sarcomas (leiomyosarcoma, poorly differentiated/de-differentiated liposarcoma, high grade pleomorphic undifferentiated sarcoma/MFH and synovial sarcoma), and bone sarcomas (Ewing sarcoma, osteosarcoma, and chondrosarcoma [de-differentiated or mesenchymal]).
Histologically confirmed diagnosis of metastatic or recurrent uterine cancer (endometrial carcinoma, carcinosarcoma, clear cell carcinoma, leiomyosarcoma, undifferentiated sarcoma, high grade endometrial stromal sarcoma) by Memorial Sloan Kettering Cancer Center; carcinosarcomas, endometrioid and clear cell carcinomas that appears to have arisen in the ovary/fallopian tube are also eligible; recurrence should not be amenable to curative approaches such as surgical resection or chemoradiotherapy
Group A: newly diagnosed patient less than 14 years of age at the time of diagnosis with histologically proven non-pelvic localized Ewing sarcoma family of tumor involving the bone or soft tissue
Participants must have a primary soft tissue sarcoma or isolated local recurrent sarcoma without prior radiation; open incisional biopsy or core biopsy should be performed to establish the diagnosis of soft tissue sarcoma; slides must be reviewed and assigned a histologic diagnosis and grade by an expert sarcoma pathologist
Participants must have histologically intermediate- or high-grade soft tissue sarcoma
Histologically confirmed solid tumor or lymphoma at original diagnosis:\r\n* Ewing sarcoma family of tumors (ESFT)\r\n* Gastrointestinal tumors\r\n* Germ cell tumors\r\n* Hepatic tumors (including hepatocellular carcinoma and hepatoblastoma)\r\n* Lymphoma (including Hodgkin and non-Hodgkin lymphoma)\r\n* Kidney tumors (including Wilms tumor, rhabdoid tumors, clear cell carcinoma, and renal cell carcinoma)\r\n* Melanoma\r\n* Neuroblastoma\r\n* Soft tissue sarcoma (including rhabdomyosarcoma and non-rhabdomyosarcoma soft tissue sarcoma)
Histologically or cytologically confirmed diagnosis of sarcoma of soft tissue; (patients with liposarcoma, bone sarcoma or gastrointestinal stromal tumor [GIST] will be excluded)
Patients with sarcoma, renal cell carcinoma, or melanoma or with known disease outside the lungs and/or thorax
Patients must have histologically confirmed relapsed/refractory Ewing's sarcoma or neuroblastoma
Well-differentiated liposarcoma or other low grade STS; Kaposi sarcoma, bone sarcomas, cartilage sarcomas and gastrointestinal stromal tumor (GIST)
Planned to receive neoadjuvant or adjuvant chemotherapy for current diagnosis of localized soft tissue sarcoma
For the purpose of the Pediatric study patients with histologic diagnosis of ESFT including: Ewing's sarcoma or primitive neuroectodermal tumor (malignant neuroepithelioma) of the bone or soft tissues, Askin's tumor of the chest and with central nervous system tumors are eligible.
Kaposi's Sarcoma.
Patients must have metastatic alveolar soft part sarcoma that is not curable by surgery; patients who have surgically resectable tumors with metastasis will be considered on a case-by-case basis
Group B: Primary sarcoma of bone or soft tissue of the lower extremity.
Histopathological documentation of sarcoma
Histological documentation of soft tissue sarcoma (biopsy may be historical and may have been obtained from primary tumor or a metastatic site).
Advanced and/or metastatic malignant soft tissue sarcoma of intermediate or high histologic grade. Excluded are the following sarcoma subtypes:
Ewing sarcoma of soft tissue or bone
Alveolar soft part sarcoma
Clear cell sarcoma
Kaposi sarcoma
No prior chemotherapy for current sarcoma, or
Current evidence/diagnosis of alveolar soft part sarcoma, extraskeletal myxoid chondrosarcoma, rhabdomyosarcoma, osteosarcoma, gastrointestinal stromal tumor (GIST), dermatofibrosarcoma (unless transformed to fibrosarcoma), Ewing's sarcoma, Kaposi's sarcoma, mixed mesodermal tumor, clear cell sarcomas.
Patients must have histologically confirmed soft tissue or bone/cartilage sarcoma. Patients with sarcoma of small round blue cell tumor types are allowed. Gastrointestinal stromal tumors (GIST) are excluded.
Histologically confirmed diagnosis of soft tissue sarcoma of high or intermediate grade with one of the following histological subtypes:
Adipocytic sarcoma, including:
Subjects should have received at least two standard systematic regimens for advanced soft tissue sarcoma one of which must have included an anthracycline (unless contraindicated).
Due to availability of effective first- and second-line therapies (as well as disease-specific clinical trials), patients with diagnosis of active Kaposi sarcoma will be excluded from study participation; however, persons with other active malignancy with prior history of Kaposi sarcoma can be considered for participation at the discretion of the Study Chair
Diagnosis:\r\n* Rhabdomyosarcoma: embryonal or alveolar\r\n* Ewing’s sarcoma family of tumors (ESFT), which include: classical, atypical, and extraosseous ESFT, peripheral primitive neuroectodermal tumors, peripheral neuroepithelioma, primitive sarcoma of bone, and ectomesenchymoma\r\n* Neuroblastoma: may be diagnosed via histology or the standard clinical evidence for increased catecholamines in the urine plus tumor cells in the bone marrow\r\n* Undifferentiated or embryonal sarcoma\r\n* Desmoplastic small round cell tumor\r\n* Synovial cell sarcoma
Diagnosis of Ewing sarcoma (ES), which includes: classical, atypical, and extraosseous ES, peripheral primitive neuroectodermal tumors, peripheral neuroepithelioma
Known or suspected current diagnosis of underlying malignancy including high grade sarcoma, osteosarcoma, fibrosarcoma, malignant giant cell sarcoma
Ewing’s sarcoma\r\n * Ewing's sarcoma, or other primitive neuroectodermal tumor (PNET) patients will be eligible if relapse occurs following an autologous transplant or if the patient relapses following initial treatment, but is not eligible for an autologous transplant; patients who do not achieve a complete response (CR) with initial therapy will also be eligible, provided they can be rendered free of bulky disease as defined above\r\n * Patients with Ewing’s sarcoma who present with bone or bone marrow metastasis will be eligible in first CR or partial response (PR)
Osteogenic sarcoma\r\n * Patients with osteogenic sarcoma will be eligible if they do not achieve a CR following initial therapy \r\n * Patients who relapse with pulmonary or bone metastases and do not achieve a CR with surgery and/or chemotherapy will also be eligible
Medical history including histopathological documentation of sarcoma
A diagnosis of synovial sarcoma or myxoid/round cell liposarcoma
If there is a patient with an NY-ESO-1 expressing soft tissue sarcoma who would be otherwise eligible for the trial, which is either not synovial sarcoma (SS) or myxoid/round cell liposarcoma (MRCL) or there is controversy about the diagnosis, eligibility will be decided by the PI
Lower extremity or pelvic soft tissue sarcoma necessitating radiation prior to surgical resection
Recurrent soft tissue sarcoma
(Part 2 only) Current evidence/diagnosis of alveolar soft part sarcoma, extraskeletal myxoid chondrosarcoma, rhabdomyosarcoma, osteosarcoma, gastrointestinal stromal tumor, dermatofibrosarcoma (unless transformed to fibrosarcoma), Ewing's sarcoma, Kaposi's sarcoma, mixed mesodermal tumor, or clear cell sarcomas.
Sarcoma clinic patient
Diagnosis of soft tissue sarcoma that has been histologically confirmed by an approved reference pathologist
Sarcoma of lower extremity location
Sarcoma location other than lower extremity
Patients must have a new diagnosis of osteosarcoma or Ewing’s sarcoma of bone
Patient to be treated with radiation therapy for a primary extremity soft tissue sarcoma or recurrent tumor after surgery, followed by surgical resection
Parent- or self-reported (for participants 18+ years old) physician diagnosis of sarcoma
PROVIDERS: At least 5 years' experience treating adolescents with sarcoma
Diagnosed with invasive malignancy including: breast, gastrointestinal track, female or male genitourinary system, sarcoma of bone or soft-tissue, leukemia and lymphoma
Patient is newly diagnosed with LE malignancy as shown by biopsy.; diagnoses include: osteosarcoma, Ewing's sarcoma, rhabdomyosarcoma, synovial sarcoma, malignant peripheral nerve sheath sarcoma, malignant fibrous histiocytoma of the bone and chondrosarcoma of the bone, or any other LE malignancy that requires surgical intervention
Participant must have histologically confirmed Ewing’s sarcoma
The Ewing’s sarcoma must have progressed following at least one standard prior chemotherapy regimen
Suspected or confirmed diagnosis of a bone sarcoma or osteomyelitis
This study will include only patients with sarcoma, prostate, breast, brain or metastatic cancer; in the future other patient groups may be included through amendment of this protocol
All St Jude patients with a known or suspected, newly diagnosed bone or soft-tissue sarcoma who will be treated on or as per disease specific protocols
Patients with tumors other than neuroblastoma must meet both the following criteria:\r\n* Have one of the following diagnoses (these tumors are known to express GD2 on cell surface):\r\n** Melanoma\r\n** Osteogenic sarcoma\r\n** Leiomyosarcoma\r\n** Ewing sarcoma\r\n** Liposarcoma\r\n** Fibrosarcoma\r\n** Malignant fibrous histiocytoma\r\n** Spindle cell sarcoma\r\n** Small cell lung cancer\r\n** Medulloblastoma metastatic to extracranial sites\r\n** Paraganglioma\r\n* Have refractory or relapsed or metastatic disease
Must have a biopsy-proven high-grade retroperitoneal or soft tissue extremity sarcoma confirmed by independent evaluation of a University of North Carolina (UNC) sarcoma specialized pathologist
Patients with biopsy and/or conventional imaging (CT/MRI) proven STS or bone sarcoma with a measurable soft tissue component; these include patients with extremity, retroperitoneal, chest wall, or head and neck primary sarcomas
Low-grade sarcomas as well as alveolar soft parts sarcoma, clear cell sarcoma, extraskeletal myxoid chondrosarcoma, well-differentiated liposarcoma, gastrointestinal stromal tumor, chordoma
-  15 years of age or older\n\n          -  Patients with metastatic sarcoma who have had no more than one prior systemic\n             treatment for metastatic disease\n\n          -  Patients with recurrent sarcoma at relapse\n\n          -  Patients with confirmed histological diagnosis of sarcoma or suspected diagnosis of\n             sarcoma. Some specific subtypes of sarcoma are NOT eligible (Gastrointestinal Stromal\n             Tumor (GIST), carcinosarcoma, sarcomatoid mesothelioma, and metastatic phyllodes\n             tumor)\n\n          -  If initial suspected diagnosis of sarcoma, must have sarcoma diagnosis confirmed prior\n             to proceeding with PDX drug sensitivity testing\n\n          -  Must have measureable disease for computed tomography (CT) scan or magnetic resonance\n             imaging (MRI) evaluation following biopsy or surgery to obtain tissue for PDX\n             development.\n\n          -  No plan for concurrent chemoradiation, for target lesions that will be used for drug\n             treatment correlation with PDX\n\n          -  Fresh tumor tissue available for PDX development\n\n          -  Eastern Cooperative Oncology Group performance status of 0-1\n\n          -  Life expectancy exceeds 6 months\n\n          -  Plan to receive systemic therapy\n\n          -  Informed consent
Subjects must have histologically proven osteogenic sarcoma, malignant fibrous histiocytoma (MFH), or Ewing sarcoma
A diagnosis of synovial sarcoma and myxoid/round cell liposarcoma