International CardioOncology Society, North America

Types of Treatment

Cancer Types and Prescribed Treatment Paths

Cancer is scary. But we have found that one way to counter that fear is with knowledge — patients’ own knowledge of the disease, as well as the medical expertise of the care team. At ICOS International, we’re committed to giving you the knowledge, inspiration, and specialty care you need to boldly face your cancer. Because no diagnosis should stop you from doing the things you love, or from living each day to the fullest.

The information provided here comes from the National Comprehensive Cancer Network (NCCN) guidelines. click here .

Acute Lymphoblastic Leukemia

TKI
- Dasatinib, Imatinib, Ponatinib, Nilotinib, Bosutinib
Immunomodulatory
- Blinatumomab, Inotuzumab
Regimen
- MOpAD
  - MTX, Vincristine, Pegaspargase, dexamethasone, rituximab
- hyper-CVAD
  - Hyper fractionated cyclophosphamide, intensified vincristine, doxorubicin, intensified dexamethasone, and pegaspargase
- Cytarabine-containing
  - High-dose cytarabine, idarubicin, IT MTX
- Alkylator-containing
  - Etoposide, ifosfamide, mitoxantrone
- Clofarabine-containing
  - Clofarabine, cyclophosphamide, etoposide

Acute Myeloid Leukemia

- ATRA 45mg/m2 in divided doses daily + arsenic trioxide 0.15mg/kg IV daily (or other doses)
PLUS any of the following
- idarubicin 12mg/m2 on days 2, 4, 6, 8
  Daunorubicin 50mg/m2 x4 days (IV days 3-6) + cytarabine 200mg/m2 for 7 days (IV days 3-9)
  Arsenic trioxide + gemtuzumab 9mg/m2 day 1

AIDS-Related Kaposi Sarcoma

First-line
- Liposomal doxorubicin, preferred (20mg/m2 IV every 3 weeks)
Relapsed/Refractory
- Pomalidomide, preferred (5mg/d oral for 21 days of each 28-day cycle)
  - Bevacizumab (15mg/kg IV on days 1 and 8 and then every 3 weeks)
  - Etoposide (50mg/d oral for 7 days of each 2-day cycle)
  - Gemcitabine (1000mg IV every 2 weeks)
  - Imatinib (400mg/d oral)
  - Interferon (1 million International units SubQ daily)
  - Nab-paclitaxel (100mg IV days 1, 8, and 15 of each 28-day cycle)
  - Thalidomide (200mg/d oral as starting dose, titrate to effect)
  - Vinorelbine (30mg/m2 every 2 weeks)

Anal Carcinoma

Locoregional Disease
- Mitomycin + 5-FU + RT
  Mitomycin + Capecitabine + RT
  5-FU + Cisplatin + RT
Metastatic
- 5-FU +/- RT
  - Carboplatin/paclitaxel +/- RT
- mFOLFOX +/- RT
  - Oxaliplatin 85mg/m2 IV day 1
    Leucovorin 400mg/m2 IV day 1
    5-FU 400mg/m2 IV bolus on day 1, then 1200mg/m2/day x 2 days
- Nivolumab or Pembrolizumab -

Bladder Cancer

- Intravesicular Gemcitabine, preferred
  Mitomycin
  Cisplatin-based regimen for more advanced stages
DDMVAC
- Dose-dense MTX, vinblastine, doxorubicin, and cisplatin
CMV
- Cisplatin, MTX, vinblastine
Atezolizumab, Pembrolizumab, Nivolumab, Durvalumab, Avelumab
Ifosfamide

Bone Cancer

Chondrosarcoma
- Low/High grade
- Surgery and RT
Metastatic
- Either Ewing/Osteosarcoma treatments depending on the pathology
  Dasatinib
Chordoma
- Imatinib (w/ cisplatin or sirolimus), Erlotinib, Sunitinib, Lapatinib, Sorafenib,
  Dasatinib
Ewing sarcoma
- First-Line
- VDC/IE
  - Vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide +
    etoposide
- VAI
  - Vincristine, doxorubicin, and cyclophosphamide
- VIDE
  - Vincristine, ifosphamide, doxorubicin, and etoposide
Second-Line
- Cyclophosphamide + topotecan
  Irinotecan +/- temozolomide
  Ifosfamide (high dose) +/- etoposide
  fosfamide, carboplatin, and etoposide
  Docetaxel + gemcitabine
  Vincristine + irinotecan
- Giant Cell Tumor of the Bone
  - Denosumab
    Interferon alpha-2b
- Osteosarcoma
- First-line
  - Cisplatin + doxorubicin
- MAP
  - high-dose MTX, cisplatin, and doxorubicin
- Doxorubicin, cisplatin, ifosphamide, and high-dose MTX
- Ifosphamide, cisplatin, and epirubicin
Second-line
- Docetaxel + gemcitabine
  Cyclophosphamide + etoposide or topotecan
  Gemcitabine
  Ifosphamide, carboplatin, and etoposide
  High-dose MTX, etoposide, and ifosfamide
  Ra 223
  Sorafenib

Breast Cancer

Noninvasive
- Surgery +/- focal/whole breast RT
Invasive
- Receptor (+), HER2 (+)
  - Aromatase inhibitor
    Tamoxifen
    Trastuzumab
HER2 (-) and Postmenopausal
Taxanes
- Paclitaxel (175mg/m2 IV day 1, then q21 days, or 80mg/m2 IV day 1 weekly)
Anti-metabolites
- Capecitabine (1000-1250 mg/m2PO bid days 1-14, then q21 days)
  Gemcitabine (800-1200mg/m2 IV days 1, 8, and 15, then q28 days) '
Microtubule inhibitors
- Vinorelbine (25mg/m2 IV day 1 weekly)
  Eribulin (1.4mg/m2 IV days 1 and 8, then cycled q21 days)
PARP inhibitors (options for BRCA1/2 +) -
- Olaparib (300mg PO bid, then cycled q28 days)
  Talazoparib (1mg PO daily, then cycled q28 days)
Non-steroidal aromatase inhibitor (anastrozole, letrozole)
- Selective ER down-regulator (fulvestrant)
Tamoxifen or toremifene
Steroidal aromatase inactivator (exemestane)
CDK4/6 inhibitor (abemaciclib, palbociclib, ribociclib) + fulvestrant
Exemestane + Everolimus
Fulvestrant + Everolimus
Tamoxifen + Everolimus
HER2 (+) and Postmenopausal
- Preferred regimen
  - Pertuzumab (840mg IV day 1, followed by 420mg IV) + trastuzumab (8mg/kg
    IV day 1, followed by 6mg/kg IV) + paclitaxel (80mg/m2 IV day 1 weekly or
    175mg/m2 day 1, then cycled q21 days) or docetaxel (75-100mg/m2 IV day 1,then cycled q21 days)
Aromatase inhibitor, Fulvestrant, or Tamoxifen +/- trastuzumab and/or lapatinib
Phyllodes Tumor
- (treat like a soft tissue sarcoma)
Paget’s Disease
- Treat as invasive or noninvasive breast cancer
Breast Cancer During Pregnancy
- no chemo during 1st trimester. No radiation AT ALL. anti-HER2 therapies
  contraindicated during pregnancy.
  Combinations of doxorubicin, cyclophosphamide, and 5-FU
  Can given weekly paclitaxel after the 1st trimester
Inflammatory Breast Cancer
- Preoperative anthracycline + taxane

Central Nervous System Cancer

Supratentorial Astrocytoma/Oligodendroglioma
- Low Risk
  - Preferred clinical trial
    PCV (procarazine + lomustine + vincristine)
    TMZ (Temozolomide)
High Risk
- Preferred clinical trial
  RT + adjuvant PCV (procarazine + lomustine + vincristine)
  RT + adjuvant TMZ
  RT + concurrent and adjuvant TMZ
Adjuvant Treatment
- Anaplastic Oligodendroglioma
  - - RT w/ neoadjuvant PCV
    - RT w/ adjuvant PCV
    - RT w/ concurrent TMZ and adjuvant TMZ
Anaplastic Astrocytoma/oligoastrocytoma
- - RT, then adjuvant TMZ (12 cycles)
  - RT w/ concurrent TMZ and adjuvant TMZ
  - RT w/ neoadjuvant PCV
  - RT w/ adjuvant PCV
- Anaplastic Gliomas
  - TMZ
- Recurrent Therapy
  - • TMZ
    • Lomustine or carmustine
    • PCV
    • Bevacizumab
    • Bevacizumab + chemo (irinotecan, carmustine/lomustine, TMZ, carboplatin)
    • Irinotecan
    • Cyclophosphamide
    • Platinum-based regimens
    • Etoposide
Adult Intracranial Ependymoma
- Recurrence
  - • Platinum-based regimens (single agent or in combination)
    • Etoposide
    • Lomustine/carmustine
    • Bevacizumab
    • TMZ
Adult Medulloblastoma
- Weekly vincristine during craniospinal radiation therapy follow by:
  - - Cisplatin, cyclophosphamide, and vincristine
    - Cisplatin, lomustine, and vincristine
Recurrence Therapy
- No prior chemo
  - - High-dose cyclophosphamide +/- etoposide
    - Carboplatin, etoposide, and cyclophosphamide
    - Cisplatin, etoposide, and cyclophosphamide
    - consider high-dose chemo with autologous stem cell reinfusion
Prior chemo
- - High-dose cyclophosphamide +/- etoposide
  - Oral etoposide
  - TMZ
  - Consider high-dose chemo with autologous stem cell reinfusion
Primary CNS Lymphom
- Induction Therapy
- Systemic Therapy
  - - High-dose MTX (8g/m2) w/ Rituximab +/- TMZ
    - High-dose MTX (3.5g/m2) w/ TMZ, procarbazine, and rituximab or TMZ,
    rituximab, and post-RT TMZ
- Intra-CSF Therapy
  - MTX, Cystarabine, Rituxmab
Consolidation
- High-dose chemo w/ stem cell rescue
  - - Carmustine + thiopeta
    - Thiopeta, busulfan, and cyclophosphamide
High-dose cytarabine +/- etoposide
High-dose cytarabine
Relapsed/Refractory Disease
- • Retreat w/ high-dose MTX +/- rituximab
  • Ibrutinib
  • TMZ
  • Rituximab +/- TMZ
  • Lenalidomide +/- TMZ
  • Topotecan
  • Consider high-dose chemo w/ autologous stem cell reinfusion in eligible patient
  • High-dose cystarabine
  • Dexamethasone, high-dose cytarabine, cisplatin
  • Pemetrexed
Primary Spinal Cord Tumors
Meningiomas
- - Interferon alpha
  - Somatostatin analogue (if octreotide scan positive)
  - Sunitinib
  - Bevacizumab + everolimus
Brain Metastases
- Newly Diagnosed
- BRAF/MEK inhibitor combination (melanoma)
  - - Venmurafenib/cobimetinib
    - Dabrafenib/trametinib
Ipilimumab (melanoma or NSCLC)
- Pembrolizumab (8g/m2)
Recurrent Disease
- BRAF/MEK combined therapies
  - - Vemurafenib/cobimetnib
    - Dabrafenib/trametnib
Ceritinib, alectinib, brigatinib (ALK rearrangement-positive NSCLC)
Erlotinib, afatinib, gefitinib (EGFR-sensitizing mutation-positive NSCLC)
Osimertinib (EGFR T790M mutation-positive NSCLC)
Crizotinib (ALK rearrangement-positive or ROS1 rearrangement-positive NSCLC)
Leptomeningeal Metastases
- - Systemic focused on primary, emphasizing drugs with good CNS penetration
- Intra-thecal
  - • Cytarabine (lymphoma/leukemia)
    • MTX (lymphoma/leukemia, breast)
    • Thiotepa
    • Rituximab (lymphoma)
    • Topotecan
    • Etoposide
    • Interferon alpha
    • Trastuzumab (breast)
- High-dose MTX (lymphoma/breast)
- Weekly pulse erlotinib for EGFR exon 19 deletion or exon 21 L858R mutation (NSCLC)
- Metastatic Spine Tumor
  - Use regimen for disease-specific site

Cervical Cancer

Chemoradiation
- - Cisplatin, preferred (Carboplatin if intolerant)
  - Cisplatin/5-FU
Recurrent or Metastatic Disease
- First-line
  - • Cisplatin/paclitaxel/bevacizumab
    • Carboplatin/paclitaxel/bevacizumab
    • Topotecan/paclitaxel/bevacizumab
    • Cisplatin/paclitaxel
    • Carboplatin/paclitaxel
    • Topotecan/paclitaxel
Possible first-line single agent
- Cisplatin, preferred
  Carboplatin, paclitaxel
Second-line
- • Pembrolizumab for PD-L1-positive or MSI-H/dMMR tumors, preferred
  • Bevacizumab
  • Albumin-bound paclitaxel
  • Docetaxel
  • 5-FU
  • Gemcitabine
  • Ifosfamide
  • Irinotecan
  • Mitomycin
  • Pemetrexed
  • Topotecan
  • Vinorelbine

Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma

CLL/SLL without del(17p)/TP53 mutation
- First-line
- Frail patient with significant comorbidity
- Preferred
  - • Ibrutinib
    • Acalabrutinib +/- obinutuzumab
    • Venetoclax + obinutuzumab
- Others
  - • Bendamustine + anti-CD20 monoclonal antibody
    • High-dose methylprednisolone (HDMP) + rituximab
    • Obinutuzumab +/- chlorambucil
    • Chlorambucil
    • Rituximab
Age >65 years and younger patients with significant comorbidities
- Preferred
  - • Ibrutinib
    • Acalabrutinib +/- Obinutuzumab
    • Venetoclax + obinutuzumab
- Other
  - • Bendamustine + anti-CD20 monoclonal antibody
    • FCR (fludarabine, cyclophosphamide, rituximab)
    • Fludarabine + rituximab (FR)
    • HDMP + rituximab
    • Pentostatin, cyclophosphamide, rituximab (PCR)
Post First-Line Chemoimmunotherapy Maintenance Therapy
- Other
  - Consider lenalidomide for high-risk patients after first-line therapy
Age <65 years old without significant comorbidities
- Preferred
  - • Bendamustine + anti-CD20 monoclonal antibody
    • FCR (fludarabine, cyclophosphamide, rituximab)
    • Fludarabine + rituximab (FR)
    • HDMP + rituximab
    • Pentostatin, cyclophosphamide, rituximab (PCR)
- Other
  - • Bendamustine + anti-CD20 monoclonal antibody
    • FCR (fludarabine, cyclophosphamide, rituximab)
    • Fludarabine + rituximab (FR)
    • HDMP + rituximab
    12 of 43
    • Pentostatin, cyclophosphamide, rituximab (PCR)• Bendamustine + anti-CD20 monoclonal antibody
    • FCR (fludarabine, cyclophosphamide, rituximab)
    • Fludarabine + rituximab (FR)
    • HDMP + rituximab
    • Pentostatin, cyclophosphamide, rituximab (PCR)
Relapsed/Refractory
- Frail, significant comorbidities, or age >65
- Preferred
  - • Acalabrutinib
    • Ibrutinib
    • Venetoclax + rituximab
    • Duvelisib
    • Idelalisib + rituximab
Others
- • Acalabrutinib
  • Alemtuzumab +/- rituximab
  13 of 45
  • Chlorambucil + rituximab
  • Reduced-dose FCR/PCR
  • HDMP + rituximab
  • Idelalisib
  • Lenalidomide +/- rituximab
  • Obinutuzumab
  • Ofatumumab
  • Venetoclax
  • Dose-dense rituximab
  • Bendamustine, rituximab +/- ibrutinib or idelalisib
Age <65 years old without comorbidities
- Preferred
  - • Acalabrutinib
    • Ibrutinib
    • Venetoclax + rituximab
    • Duvelisib
    • Idelalisib + rituximab
Others
- • Acalabrutinib
  • Alemtuzumab +/- rituximab
  • Bendamustine + rituximab
  • FC + ofatumumab
  • FCR
  • HDMP + rituximab
  • Idelalisib
  • Lenalidomide +/- rituximab
  • Obinutuzumab
  • Ofatumumab
  • PCR
  • Venetoclax
  14 of 45
  • Bendamustine, rituximab + ibrutinib or idelalisib
- Post Second-Line Maintenance (for complete or partial response after relapse)
- Other
  - Consider lenalidomide for high-risk patients after first-line therapy
CLL/SLL with del(17p)/TP53 mutation
- First-line
- Preferred
  - - Acalabrutinib +/- obinutuzumab
    - Ibrutinib
    - Venetoclax + obinutuzumab
- Others
  - - Alemtuzumab +/- rituximab
    - HDMP + rituximab
    - Obinutuxumab
Post First-line Maintenance
- Others
  - Consider lenalidomide for high-risk patients after first-line therapy

Chronic Myeloid Leukemia

- Dasatinib 100mg daily
  Nilotinib 300mg bid
  Bosutinib 400mg daily
  Ponatinib, Omacetaxine, allogeneic HCT or clinical trial

Colorectal Cancer

- Continuum systemic therapy
Intensive therapy candidate
- - FOLFOX (Oxaliplatin 85mg/m2 IV day 1, Leucovorin 400mg/m2 day 1, 5-FU 400mg/m2 IV bolus on day 1 then 1200mg/m2/d x 2 days continuous infusion) +/- bevacizumab (5mg/kg IV days 1)
  - CAPEOX (Oxaliplatin 130mg/m2 IV day 1, Capecitabine 1000mg/m2 bid PO x14 days) +/- bevacizumab
  - FOLFOX + cetuximab (400mg/m2 IV over 2 hr first infusion then 250mg/m2 IV over 60 min weekly) or panitumumab (6mg/kg IV day 1)
  - FOLFOXIRI (FOLFOX + Irinotecan 180mg/m2 IV over 30-90 min day 1) +/- bevacizumab
  - 5-FU/leucovorin +/- bevacizumab
- Intensive therapy NON candidate
  - - 5-FU/leucovorin +/-bevacizumab
    - Capecitabine +/- bevacizumab
    - Cetuximab or panitumumab
    - Nivolumab (3mg/kg every 2 weeks) or pembrolizumab (2mg/kg every 3 weeks)
    - Nivolumab + ipilimumab (1mg/kg over 30 min q3 weeks for 4 doses)
Postoperative Adjuvant Therapy (Rectal CA)
- • mFOLFOX
  • 5-FU/leucovorin weekly
  • Capecitabine
  • CAPEOX
Concurrent Chemo/XRT (Rectal CA)
- • XRT + continuous infusion 5-FU
  • XRT + Capecitabine
  • XRT + 5/FU/leucovorin

Esophageal and Esophagogastric Junction Cancers

- • First-line (2 dress preferred, 3 drugs reserved for medically fit patients)
Preferred
- • 5-FU or Capecitabine + Cisplatin
  • 5-FU or Capecitabine + Oaxaliplatin
- Other
  - • Paclitaxel (50mg/m2 IV on day 1) + Cisplatin (75-100 mg/m2 IV on days 1 and 29) or Carboplatin (AUC 2 IV on day 1) weekly for 5 weeks
    • Docetaxel + Cisplatin
    • 5-FU (750-1000mg/m2 IV on days 1 and 29) or Capecitabine
    • Docetaxel (50 mg/m2 on day 1)
    • Paclitaxel
    • 5-FU and Irinotecan (65 mg/m2 IV)
    • DCF (Docetaxel, cisplatin, 5-FU)
    • ECF (Epirubin, cisplatin, 5-FU)
Second-line (Dependent on prior therapy and PS)
- Preferred
  - • Ramucirumab (8mg/kg Iv on days 1 and 15) and Paclitaxel for adenocarcinoma
    • Docetaxel (75-100 mg/m2 IV on day 1 cycled every 21 days)
    • Paclitaxel (various doses)
    • Irinotecan (various doses)
    • 5-FU and Irinotecan
    • Pembrolizumab (200mg IV on day 1 cycled every 21 days)
- Other
  - • Ramucirumab for adenocarcinoma
    • Irinotecan + Cisplatin
    • Pembrolizumab
    • Docetaxel + Irinotecan

Gastric Cancer

- • First-line (2 drugs preferred 2/2 lower toxicity, but 3 drugs for fit patients)
Preferred
- • 5-FU or Capecitabine + Cisplatin or Oxaliplatin
- Others
  - • Paclitaxel + Cisplatin
    • 5-FU or Capecitabine
    • Docetaxel or Paclitaxel
    • DCF modifications (Docetaxel, cisplatin, and 5-FU)
    • ECF modifications (Epirubicin, oxaliplatin, and 5-FU)
Second-line
- Preferred
  - • Ramucirumab and Paclitaxel
    • Docetaxel or Paclitaxel
    • Irinotecan
    • 5-FU and irinotecan
    • Pembrolizumab
- Others
  - • Ramucirumab
    • Irinotecan + cisplatin
    • Pembrolizumab
    • Docetaxel + irinotecan

Gestatonal Trophoblastic Neoplasia

Low-Risk GTN
- - MTX 0.4 mg/kg/day IV or IM (max 25mg/day) daily x5 days or 1 mg/kg qod x4 days
  - Dactinomycin 10-12 mcg/kg IV daily x5 days or 1.25 mg/m2
- High-Risk GTN
  - - EMA/CO (Etoposide 100 mg/m2/day IV on days 1 and 2, Dactinomycin 0.5 mg IV on days 1 and 2, MTX 300 mg/m2 on day 1, Leucovorin 15 mg PO or IM q12hr for 4 doses, Cyclophosphamide 600 mg/m2 on day 8, Vincristine 1mg/m2 on day 8)
- High-Risk GTN (MTX-resistant)
  - - EMA/EP (Etoposide, MTX, Dactinomycin/Etoposide, Cisplatin)
    - TP/TE (Paclitaxel, Cispltain, Etoposide)
    - BEP (Bleomycin, Etoposide, Cisplatin)
    - VIP (Etoposide, Ifosfamide, Cisplatin)
    - TIP (Paclitaxel, Ifosfamide, Cisplatin)
    - ICE (Ifosfamide, Carboplatin, Etoposide)

Hairy Cell Leukemia

- • Purine analog monotherapy (Cladribine or Pentostatin)
  • Purine analogs + Rituximab
  • Rituximab
  • Interferon-alpha
  • Vemurafenib +/- rituximab
  • Ibrutinib
  • Moxetumomab

Head and Neck Cancers

Primary Systemic therapy + concurrent XRT
- - High-dose Cisplatin
  - Cetuximab
  - Carboplatin/Paclitaxel
  - Cisplatin/5-FU
  - Carboplatin/Paclitaxel
Post-operative chemoradiation
- - Cisplatin
Sequential chemotherapy
- - Docetaxel/cisplatin/5-FU
  - Paclitaxel/Cisplatin/5-FU

Hepatobiliary Cancers

- First-line Therapy
Preferred
- • Sorafenib
  • Lenvatinib
Other
- Systemic Chemo
  FOLFOX (limited data), Oxaliplatin/5-FU/Leucovorin, Doxorubicin
Subsequent Therapy
- - Regorafenib, Cabozantinib, Ramucirumab, Nivolumab, Sorafenib, Pembrolizumab
Gallbladder Cancer
- - Gemcitabine/Cisplatin
  - Fluoropyrimidine-based (or other Gemcitabine-based regimens)
  - EBRT w/ concurrent fluoropyrimidine
  - XRT
  - Pembrolizumab
Same as GB cancer
- Intrahepatic Cholangiocarcinoma
  Extrahepatic Cholangiocarcinoma
Resected with negative margins
- Fluoropyrimidine (or gemcitabine-based) chemoradiation
Resected w/ positive margins or lymph nodes
- More prolonged and aggressive version of the above

Hodgkin Lymphoma

Classic
- - ABVD (Doxorubicin, Bleomycin, Vinblastine, and Dacarbazine) +/- ISRT
  - Stanford V (Doxorubicin, Vinblastine, Mechlorethamine, Etoposide, Vincristine, Bleomycin, and Prednisone)
  - Escalated BEACOPP (Bleomycin, Etoposide, Doxorubicin, Cyclophosphamide, Vincristine, Procarbazine, and Prednisone)
  - Escalated BEACOPP followed by ABVD w/ ISRT
Nodular Lymphocyte-Predominant
- - ABVD
  - CHOP (Cyclophosphamide, Doxorubicin, Vincristine, Prednisone) + Rituximab
  - CVP (Cyclophosphamide, Vinblastine, Prednisolone) + Rituximab
  - Rituximab
Refractory/Relapsed Disease
- Second-Line
- Classic
  - - Brentuximab
    - DHAP (Dexamethasone, Cisplatin, high-dose cytarabine)
    - ESHAP (Etoposide, methylprednisolone, high-dose cytarabine, cisplatin)
    - Gemcitabine, Bendamustine, Vinorelbine
    - GVD (Gemcitabine, Vinorelbine, Doxorubicin)
    - ICE (Ifosfamide, Carboplatin, Etoposide)
    - IGEV (Ifosfamide, Gemcitabine, Vinorelbine)
- NLPHL
  - - DHAP, ESHAP, ICE, IGEV
Subsequent (only for Classic)
- • Bendamustine
  • C-MOPP (Cyclophosphamide, Vincristine, Procarbazine, Prednisone)
  • Everolimus
  • GCD (Gemcitabine, Carboplatin, Dexamethasone)
  • Lenalidomide
  • MINE (Etoposide, Ifosfamide, MESNA, Mitoxantrone)
  • Mini-BEAM (Carmustine, Cystarabine, Etoposide, Mephalan)
  • Nivolumab
  • Pembrolizumab

Kidney Cancer

- Relapsed or Stage IV (Clear cell)
Initial
- • Favorable Risk
  - Preferred
  • Pazopanib or Sunitinib
Other
- • Ipilimumab + nivolumab, Cabozantinib
  • Axitinib
  • Bevacizumab + Interferon alpha
  • High-dose IL-2
• Poor/Intermediate Risk
- Preferred
  - • Ipilimumab + Nivolumab, Cabozantinib
- Other
  - • Pazopanib or Sunitinib
    • Axitinib
    • Bevacizumab + Interferon alpha
    • High-dose IL-2
    • Temsirolimus
Subsequent Therapy
- Preferred
  - - Cabozantinib, or Nivolumab, or Ipilimumab + Nivolumab
- Other
  - - Axitinib
    - Lenvatinib + Everolimus
    - Everolimus
    - Pazopanib
    - Sunitinib
    - Bevacizumab
    - Sorafenib
    - High-dose IL-2
    - Temsirolimus
Relapsed or Stage IV (Non-clear cell)
- Preferred
  - • Clinical Trial or Sunitinib
- Other
  - • Cabozantib or Everolimus
    • Axitinib, Bevacizumab, Erlotinib, Lenvatinib + Everlolimus, Nivolumab, Pazopanib, Bevacizumab + Erlotinib or Everolimus, Temsirolimus

Malignant Pleural Mesothelioma

First-Line
- - Pemetrexed (500 mg/m2 day 1) + Cisplatin (75 mg/m2 day 1) administered every 3 weeks
  - Pemetrexed (500 mg/m2 day 1) + Cisplatin (75 mg/m2 day 1) + Bevacizumab (15 mg/kg day 1) administered every 3 weeks x6 cycles followed by maintenance Bevacizumab every 3 weeks until disease progression
  - Pemetrexed (500 mg/m2 day 1) + Cisplatin (AUC 5 day 1) +/- Bevacizumab administered every 3 weeks x6 cycles +/- maintenance Bevacizumab
  - Gemcitabine (1000-1250 mg/m2 days 1, 8, 15) + Cisplatin (80-100 mg/m2 day 1) administered in 3-4 week cycles
  - Pemetrexed (500 mg/m2) every 3 weeks
  - Vinorelbine (25-30 mg/m2) weekly
Subsequent
- - Pemetrexed (if not given in first-line)
  - Vinorelbine
  - Gemcitabine
  - Nivolumab +/- ipilimuab
  - Pembrolizumab

Melanoma

- Cutaneous Melanoma
First-Line
- Immunotherapy
  - Anti PD-1 Monotherapy (Pembrolizumab, Nivolumab)
    Nivolumab/Ipilimumab
- Targeted therapy if BRAF V600 activating mutation
  - Combination Therapy
    Dabrafenib/Trametinib
    Vemurafenib/Cobimetnib
    Encorafenib/Binimetinib
Second-Line or Disease Progression
- Anti PD-1 Monotherapy
  - Pembrolizumab or Nivolumab
- Nivolumab/Ipilimumab
- Targeted therapy if BRAF V600 activating mutation
- Combination Therapy
  - Dabrafenib/Trametinib
    Vemurafenib/Cobimetnib
    Encorafenib/Binimetinib
    
    Ipilimumab
    High-dose IL-2
    Cytotoxic Regimens (Dacarbazine, Temozolomide, Paclitaxel, Carboplatin/Paclitaxel)
    Imatinib for tumors with activating mutations of KIT
Uveal Melanoma
- Metastatic Disease
- Anti PD-1 Monotherapy
  - - Pembrolizumab
    - Nivolumab
    - Nivolumab/Ipilimumab
    - Ipilimumab
- Cytotoxic Regimens
  - - Dacarbazine
    - Temozolomide
    - Paclitaxel
    - Carbo/Paclitaxel
- Target Therapy
  - Trametinib

Multiple Myeloma/Other Plasma Cell Neoplasms

Multiple Myeloma
- - Transplant Eligible
  • Preferred
  - Bortezomib/Lenalidomide/Dexamethasone
  - Bortezomib/Cyclophosphamide/Dexamethasone
  • Other
  - Bortezomib/Doxorubicin/Dexamethasone
  - Carfilzomib/Lenalidomide/Dexamethasone
  - Ixazomib/Lenalidomide/Dexamethasone
  • Circumstantial
  - Bortezomib/Dexamethasone
  - Bortezomib/Thalidomide/Dexamethasone
  - Cyclophosphamide/Lenalidomide/Dexamethasone
  - Lenalidomide/Dexamethasone
  - Dexamethasone/Thalidomide/Cisplatin/Doxorubicin/Cyclophosphamide/Etoposide (VTD-PACE)
  - Transplant Ineligible
  • Primary Therapy
  - Preferred
  • Bortezomib/Lenalidomide/Dexamethasone
  • Lenalidomide/low-dose Dexamethasone
  • Bortezomib/Cyclophosphamide/Dexamerthasone
  • Daratumumab/Bortezomib/Melphalan/Prednisone
  - Other
  • Carfilzomib/Lenalidomide/Dexamethasoned
  • Carfilzomib/Cyclophosphamide/Dexamethasone
  • Ixazomib/Lenalidomide/Dexamethasone
  - Circumstantial
  • Bortezomib/Dexamethasone
  • Cyclophosphamide/Lenalidomide/Dexamethasone
  • Maintenance Therapy
  - Preferred
  • Lenalidomide
  - Other
  • Bortezomib
Systemic Light Chain Amyloidosis
- - Transplant Eligible
  • Preferred
  - Bortezomib/Cyclophosphamide/Dexamethasone
  • Other
  - Bortezomib +/- Dexamethasone
  - Bortezomib/Melphalan/Dexamethasone
  - Lenalidomide/Cyclophosphamide/Dexamethasone
  - Lenalidomide/Dexamethasone
  - Oral Melphalan/Dexamethasone
  - Transplant Ineligible
  • Preferred
  - Bortezomib/Cyclophosphamide/Dexamethasone
  - Oral Melphalan/Dexamethasone
  • Other
  - Bortezomib +/- Dexamethasone
  - Bortezomib/Melphalan/Dexamethasone
  - Lenalidomide/Cyclophosphamide/Dexamethasone
  - Lenalidomide/Dexamethasone
  - Refractory/Relapsed Disease
  • High-dose Melphalan w/ stem cell transplant
  • Bortezomib +/- Dexamethasone
  • Bortezomib/Melphalan/Dexamethasone
  • Ixazomib +/- Dexamethasone
  • Lenalidomide/Cyclophosphamide/Dexamethasone
  • Lenalidomide/Dexamethasone
  • Oral Melphalan/Dexamethasone
  • Pomalidomide/Dexamethasone
Waldenstrom’s Macroglobulinemia/Lymphoplasmacytic Lymphoma
- - Preferred
  • Bendamustine/Rituximab
  • Bortezomib/Dexamethasone/Rituximab
  • Rituximab/Cyclophosphamide/Dexamethasone
  - Other
  • Bendamustine
  • Bortezomib/Dexamethasone
  • Bortezomib/Dexamethasone
  • Carfilzomib/Rituximab/Dexamethasone
  • Cladribine +/- Rituximab
  • Cyclophosphamide/Doxorubicin/Vincristine/Prednisone/Rituxmib
  • Fludarabine +/- Rituximab
  • Fludarabine/Cyclophosphamide/Rituximab
  • Ibrutinib +/- Rituximab
  • Rituximab
  • Rituximab/Cyclophosphamide/Prednisone
Myelodysplastic Syndromes
- Intermediate or high IPSS(-R)
  - - Transplant Candidacy
    • Yes
    - Allo-HCT
    - Azacitidine followed by Allo-HCT
    - Decitabine followed by Allo-HCT
    - High-intensity chemotherapy (Idarubicin, Cytarabine, Fludarabine, and Topotecan-based regimens) followed by Allo-HCT
    • No
    - Azacitidine (preferred)
    - Decitabine
    - Clinical Trial

Myeloproliferative Neoplasms

Myelofibrosis
- - Low-risk (if symptomatic)
  • Ruxolitinib
  • Interferon alpha-2b
  • Hydroxyurea
  • Clinical Trial
  - Intermediate-risk
  • Same as above + Allo-HCT
  - High-risk (Transplant Ineligible)
  • Ruxolitinib
  • Clinical Trial
Polycythemia Vera
- - High-risk
  • Ruxolitinib
  • Hydroxyurea
  • Interferons
  • Clinical Trial
Essential Thrombocytemia
- - High-risk
  • Hydroxyurea
  • Interferon
  • Anagrelide
  • Clinical trial

Neuroendocrine and Adrenal Tumors

- • Somatostatin Analogues (Octreotide or Lanreotide)
  • Everolimus 10mg PO daily
  • Sunitinib 37.5mg PO daily
  • PRRT w/ Lu-dotate
  • Cytotoxic chemotherapies
  - Temozolomide/Capecitabine
  - 5-FU/Doxorubicin/Streptozocin
  - Streptozocin/Doxorubicin
  - Streptozocin/5-FU

Non-Hodgkin’s Lymphomas

- Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma
B cell Lymphomas
- • Follicular Lymphoma
  - First-line
  • Bendamustine + Obinutuzumab or Rituximab
  • CHOP (Cyclophosphamide, Doxorubicin, Vincristine, Prednisone) + Obinutuzumab or Rituximab
  • CVP (Cyclophosphamide, Vincristine, Prednisone) + Obinutuzumab or Rituximab
  • Lenalidomide + Rituximab
  - First-line Elderly or Infirm
  • Rituximab
  • Chlorambucil or Cyclophosphamide +/- Rituximab
  • Ibritumomab
  - First-line Consolidation
  • Rituximab
  • Obinutuzumab
  - Second-line
  • Bendamustine + Obinutuzumab or Rituximab
  • CHOP (Cyclophosphamide, Doxorubicin, Vincristine, Prednisone) + Obinutuzumab or Rituximab
  • CVP (Cyclophosphamide, Vincristine, Prednisone) + Obinutuzumab or Rituximab
  • Lenalidomide + Rituximab
  • Ibritumomab
  • PI3K inhibitors (Idelalisib, Copanlisib, Duvelisib)
  - Second-line Subsequent Therapy
  • Rituximab
  • Chlorambucil or Cyclophosphamide + Rituximab
  • Chlorambucil or Cyclophosphamide
  • Ibritumomab
  - Secondary-line Consolidation
  • Rituximab
  • Obinutuzumab
  • Marginal Zone Lymphomas
  - First-line
  • Bendomustine + Rituximab
  • RCHOP (Rituximab + CHOP)
  • RCVP (Rituximab +CHOP)
  • Rituximab
  • Ibritumab
  • Lenalidomide + Rituximab
  - First-line for Elderly or Infirm
  • Rituximab
  • Chlorambucil or Cyclophosphamide +/- Rituximab
  - Second-line and Subsequent Therapy
  • Bendomustine + Rituximab
  • RCHOP (Rituximab + CHOP)
  • RCVP (Rituximab +CHOP)
  • Rituximab
  • Ibritumab
  • Lenalidomide + Rituximab
  • PI3K inhibitors (Idelalisib, Copanlisib, Duvelisib)
  • Ibritumomab
  - Second-line and Subsequent Therapy Elderly or Infirm
  • Chlorambucil or Cyclophosphamide +/- Rituximab
  • Rituximab
  - Second-line Consolidation
  • Obinutuzumab
  • Mantle Cell Lymphoma
  - Induction
  • Aggressive
  - First-line
  • Preferred
  - RDHA (Rituximab, Dexamethasone, Cytarabine) + Platinum (Carboplatin, Cisplatin, Oxaliplatin)
  - Alternating RCHOP/RDHAP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, Prednisone/Rituximab, Dexamethasone, Cytarabine, Cisplatin)
  - NORDIC (maxi-CHOP alternating w/ Rituximab + high-dose Cytarabine)
  - HyperVAD (Cyclophosphamide, Vincristine, Doxorubicin, and Dexamethasone alternating w/ high-dose MTX and Cytarabine) + Rituximab
  - Bendomustine
  • Maintenance (after Autologous Stem Cell Rescue)
  - Rituximab
  • Less Aggressive
  - Preferred
  • Bendamustine + Rituximab
  • VR-CAP (Bortezomib, Rituximab, Cyclophosphamide, Doxorubicin, Prednisone)
  • RCHOP
  • Lenalidomide + Rituximab
  • Modified Rituximab-HyperCVAD
  • RBAC (Rituximab, Bendamustine, Cytarabine)
  - Maintenance
  • Rituximab
  - Second-line
  • Short response prior to chemo
  - Preferred
  • Acalabrutinib
  • Ibrutinib or Lenalidomide +/- Rituximab
  • Venetoclax
  • Extended response prior to chemo
  - Preferred
  • Bendamustine +/- Rituximab
  • Bendamustine, Bortezomib, Rituximab
  • PEPC (Prednisone, Etoposide, Procarbazine, Cyclophosphamide)
  • RCHOP
  • VRCAP
  • Diffuse Large B-Cell Lymphoma
  - First-line
  • RCHOP
  • EPOCH (Etoposide, Prednisone, Vincristine, Cyclophosphamide, Doxorubicin) + Rituximab
  - First-line w/ poor LV function
  • RCEPP (Rituximab, Cyclophosphamide, Etoposide, Prednisone, Procarbazine)
  • RCDOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, Prednisone)
  • DA-EPOCH (Etoposide, Prednisone, Vincristine, Cyclophosphamide, Doxorubicin) + Rituximab
  • RCEOP (Rituximab, Cyclophosphamide, Etoposide, Vincristine, Prednisone)
  • RGCVP (Rituximab, Gemcitabine, Cyclophosphamide, Vincristine, PRednisone)
  - First-line and frail and/or >80 years old
  • RCEPP (Rituximab, Cyclophosphamide, Etoposide, Prednisone, Procarbazine)
  • RCDOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, PRednisone)
  • R-mini-CHOP
  • RGCVP (Rituximab, Gemcitabine, Cyclophosphamide, Vincristine, Prednisone)
  - First-line Consolidation
  • Lenalidomide
  - Concurrent CNS Disease
  • Parenchymal
  - Systemic MTX
  • Leptomeningeal
  - IT MTX + Cytarabine
  - Second-line and Subsequent Therapy (Transplant intended)
  • DHAP (Dexamtheasone, Cisplatin, Cytarabine) +/- Rituximab
  • DHAX (Dexamethasone, Cytarabine, Oxaliplatin) +/- Rituximab
  • ESHAP (Etoposide, methylprednisolone, Cytarabine, Cisplatin) +/- Rituximab
  • GDP (Gemcitabine, Dexamethasone, Cisplatin) +/- Rituximab or (Gemcitabine, Dexamethasone, carboplatin) +/- Rituximab
  • GemOx (Gemcitabine, Oxaliplatin) +/- Rituximab
  • ICE (Ifosfamide, Carboplatin, Etoposide) +/- Rituximab
  • MINE (MESNA, Ifosfamide, Mitoxantrone, Etoposide) +/- Rituximab
  - Second-line and Subsequent Therapy (Transplant not intended)
  • Bendamustine +/- Rituximab
  • Brentuximab
  • CEPP +/- Rituximab
  • CEOP +/- Rituximab
  • DA-EPOCH +/- Rituximab
  • GDP +/- Rituximab
  • GemOx +/- Rituximab
  • Gemcitabine, Vinorelbine +/- Rituximab
  • Ibrutinib
  • Lenalidomide +/- Rituximab
  • Rituximab
  • High-Grade B-Cell Lymphoma
  - Clinical Trial
  - RCHOP
  - DA-EPOCH-R
  - RHyper-CVAD
  - R-CDOX-M/R-IVAC (Rituximab, Cyclophosphamide, Vincristine, Doxorubicin w/ Ifosfamide/MTX, Etoposide, Cytarabine)
  • Burkitt Lymphoma
  - Induction
  • Low-risk
  - CODOX-M
  - EPOCH
  - HyperCVAD
  • High-risk
  - RICE (Rituximab, Ifosfamide, Carboplatin, Etoposide)
  - RIVAc (Rituximab, Ifosfamide, Cytarabine, Etoposide)
  - RGDP (Rituximab, Gemcitabine, Dexamethasone, Cisplatin)
  - High-dose Cytarabine + Rituximab
  • Hairy Cell Leukemia
  • Primary Cutaneous Lymphomas
  - Mycosis Fungoides/Sezary Syndrome
  • Brentuximab
  • Bexarotene
  • Interferons
  • MTX
  • Mogamulizumab
  • Romidepsin
  • Vorinostat
  • Gemcitabine
  • Doxorubicin
  • PRalatrexate
  - Primary Cutaneous T-Cell Lymphoproliferative DO
  • Clinical Trial
  • Brentuximab
  • T cell Lymphomas

Non-malignant Skin Cancers

Basal Cell Carcinoma
- - Surgical removal and local topical agents
Dermatofibrosarcoma Protuberans
- - If metastatic, then treat as soft tissue sarcoma
Merkel Cell Carcinoma
- - Local
  • No chemo
  - Regional
  • Clinical trial preferred
  • Cisplatin +/- Etoposide
  • Carboplatin +/- Etoposide
  - Disseminated
  • Clinical Trial preferred
  • Preferred chemo
  - Avelumab
  - Pembrolizumab
  - Nivolumab
  • Useful in certain circumstances
  - Cisplatin +/- Etoposide
  - Carboplatin +/- Etoposide
  - Topotecan
  - Cyclophosphamide, Doxorubicin (or epirubicin), and Vincristine (CAV)
  • Squamous Cell Carcinoma
  - Metastatic disease (not great data)
  • Anti-PD-1 (Nivolumab, Pembrolizumab)
  • Anti-CTLA-4 (Ipilimumab)
  • Cisplatin + 5-FU or Cetuximab or immune checkpoint inhibitors

Non-Small Cell Lung Cancer

Neoadjuvant/Adjuvant Chemotherapy
- - Healthy patients
  • Cisplatin + Vinorelbine or Etoposide or Gemcitabine or Docetaxel or Pemetrexed
  - At risk patients
  • Carboplatin + Paclitaxel or Gemcitabine or Pemetrexed
Chemotherapy with Radiation
- - Cisplatin + Etoposide or Vinblastine
  - Carboplatin + Pemetrexed
  - Paclitaxel + Carboplatin
Consolidation Therapy for nonresectable lesions
- - Durvalumab

ccult Primary

Adenocarcinoma
- - Paclitaxel + Carboplatin +/- Etoposide
  - Docetaxel + Carboplatin
  - Gemcitabine + Cisplatin or Docetaxel
  - CAPEOX
  - mFOLFOX6
  - Docetaxel + Cisplatin
  - Irinotecan + Carboplatin or Gemcitabine
  - FOLFIRI
Squamous Cell Carcinoma
- - Paclitaxel + Carboplatin
  - Cisplatin + Gemcitabine
  - mFOLFOX6
  - Docetaxel or Paclitaxel + Cisplatin or Carboplatin +/- 5-FU

Ovarian Cancer

Epithelial Ovarian/Fallopian tube/Primary Peritoneal
- - Stage I
  • Paclitaxel + Carboplatin
  • Carboplatin + Doxorubicin 30mg/m2
  • Docetaxel + Carboplatin
  - Stage II-IV
  • IP/IV Regimens
  - Paclitaxel + Carboplatin
  • IV
  - Paclitaxel or Docetaxel + Carboplatin
  - Carboplatin + Doxorubicin
  - Bevacizumab
Carcinosarcoma
- - Carboplatin or Cisplatin or Paclitaxel + Ifosfamide
Clear Cell Carcinoma
- - Paclitaxel + Carboplatin
Mucinous Tumors
- - Paclitaxel + Carboplatin
  - 5-FU + Leucovorin + Oxaloplatin
  - Capecitabine + Oxaliplatin
Endometrial
- - As above
  - Hormone Therapy (anastrozole, letrozole, exemestane), leuprolide, tamoxifen
Malignant Germ Cell Tumors
- - BEP (Bleomycin, Etoposide, Cisplatin)
  - Etoposide + Carboplatin
Malignant Sex Cord-Stromal Tumors
- - BEP
  - Paclitaxel + Carboplatin

Pancreatic Adenocarcinoma

Neoadjuvant (Resectable/Borderline Resectable)
- - Limited evidence for systemic chemo
  • Preferred
  - FOLFIRINOX
  - Gemcitabine + cisplatin
Adjuvant
- - Preferred
  • mFOLFIRINOX
  • Gemcitabine + Capecitabine
  - Other
  • Gemcitabine
  • 5-FU + Leucovorin
  • Capecitabine

Penile Cancer

Local
- - Neoadjuvant
  • Preferred
  - TIP (Paclitaxel, Ifosfamide, and Cisplatin)
  - Adjuvant
  • Preferred
  - TIP
  • Other
  - 5-FU + Cisplatin
Metastatic
- - First-Line
  • Preferred
  - TIP
  • Other
  - 5-FU + Cisplatin
  - Second-Line
  • Preferred
  - Clinical Trial
  - Pembrolizumab
  • Useful in certain circumstances
  - Paclitaxel
  - Cetuximab
  - Combination Therapy (with radiation)
  • Preferred
  - Cisplatin or Mitomycin +/- 5-FU
  • Other
  - Capecitabine

Prostate Cancer

Systemic Therapy
- - Chemotherapy
  • Docetaxel + Steroids
  • Cabazitaxel + Steroids
  - Immunotherapy
  • Sipuleucel-T
  • Pembrolizumab

Small Cell Lung Cancer

Primary or adjuvant therapy
- - Limited Stage
  • Cisplatin or Carboplatin + Etoposide + XRT
  - Extensive Stage
  • Carboplatin + Etoposide + Atezolizumab
  • Carboplatin or Cisplatin + Etoposide or Irinotecan
Subsequent Systemic therapy
- - Relapse < 6 months
  • Topotecan
  • Irinotecan
  • Paclitaxel
  • Docetaxel
  • Temozolomide
  • Pembrolizumab
  • Nivolumab +/- Ipilimumab
  • Venorelbine
  • Etoposide
  • Gemcitabine
  • Cyclophosphamide + Doxorubicin + Vincristine (CAV)
  • Bendamustine
  - Relapse > 6 months
  • Redo original regimen

Soft Tissue Sarcoma

Non-specific Histology
- - Combination Regimens
  • AD (Doxorubicin + Dacarbazine)
  • AIM (Doxorubicin + Ifosfamide + MESNA)
  • MAID (MESNA + Doxorubicin + Ifosfamide + Dacarbazine)
  • Ifosfamide + Epirubicin + MESNA
  • Gemcitabine + Docetaxel or Vinorelbine or Dacarbazine
  • Doxorubicin + Olaratumab
  - Single Agents
  • Doxorubicin, Ifosfamide, Epirubicin, Gemcitabine, Dacarbazine, Temozolomide, Vinorelbine, Eribulin, Trabectedin, Pazopanib, Regorafenib, Larotrectinib
  • GIST
  - Primary
  • Imatinib, Sunitinib, or Regorafenib
  - Progression
  • Sorafenib, Nilotinib, Dasatinib, Pazopanib, or Everolimus + TKI
  • Desmoid Tumors
  - Primary
  • Sunlinac or other NSAIDs
  • Tamoxifen +/- Sunlinac
  • Toremifene
  • MTX and Vinblastine or Vinorelbine
  • Low-dose Interferon
  • Doxorubicin
  • Imatinib
  • Sorafenib
Non-pleotropic Rhabdomyosarcoma
- - Combination Regimens
  • Vincristine + Dactinomycin or Doxorubicin + Cyclophosphamide or Ifosfamide
  • Cyclophosphamide + Topotecan
  • Ifofamide + Doxorubicin or Etoposide
  • Irinotecan + Vincristine
  • Carboplatin + Etoposide
  • Vinorelbine + low-dose Cyclophosphamide
  • Vincristine + Irinotecan + Temozolomide
Tenosynovial Giant Cell Tumor
- - Imatinib
Angiosarcoma
- - Paclitaxel/Docetaxel
  - Vinorelbine
  - Sorafenib/Sunitinib
  - Bevacizumab
Alveolar Soft Part Sarcoma
- - Sunitinib/Pazopanib
  - Pembrolizumab
Inflammatory Myofibroblastic Tumor
- - Crizotinib/Ceritinib
Well-Differentiated Liposarcoma or RP Sarcomas
- - Palbociclib
Solitary Fibrous Tumor/Hemangiopericytoma
- - Bevacizumab + Temozolomide
  - Sunitinib/Sorafenib/Pazopanib
PEComa, Recurrent Angiomyolipoma, Lymphangiomyomatosis
- - Sirolimus/Everolimus/Temsirolimus

Systemic Mastocytosis

- • Clinical Trial
  • Midostaurin
  • Claddribine
  • Interferons
  • Allo-HCT

Testicular Cancer

Germ Cell Tumors
- - Primary
  • Preferred
  - BEP (Bleomycin, Cisplatin, Etoposide)
  - EP (Etoposide, Cisplatin)
  - Other
  • VIP (Etoposide, MESNA, Ifosfamide, Cisplatin)
  - Secondary
  • Conventional-dose
  - TIP (Paclitaxel, Ifosfamide, MESNA, Cisplatin)
  - VeIP (Vinblastine, MESNA, Ifosfamide, Cisplatin)
  • High-dose
  - Carboplatin + Etoposide
  - Paclitaxel + Ifosfamide + Carboplatin + Etoposide

Thymomas and Thymic Carcinomas

First-line Therapy
- - CAP (Cisplatin + Doxorubicin + Cyclophosphamide)
  - CAP + prednisone
  - ADOC (Cisplatin + Doxorubicin + Vincristine + Cyclophosphamide)
  - PE (Cisplatin + Etoposide)
  - Etoposide + Ifosfamide + Cisplatin
  - Carboplatin + Paclitaxel
Second-line Therapy
- - Sunitinib
  - Pemetrexed
  - Everolimus
  - Paclitaxel
  - Octreotide +/- Prednisone
  - Gemcitabine
  - 5-FU + Leucovorin
  - Etoposide
  - Ifosfamide
Thyroid Carcinomas
Thyroid Carcinoma
- - Surgical excision, consider TKI
Papillary/Follicular/Hurthle Cell Carcinoma
- - Surgical excision, possible XRT and/or RAI ablation, no chemo
Medullary Thyroid Carcinoma
- - Metastatic
  • Vandatanib
  • Cabozantinib
  • Dacarbazine
  • TKI
  • Clinical Trial
Anaplastic Carcinoma
- - Dabrafenib/Trametinib
  - Larotrectinib
  - Paclitaxel + Carboplatin
  - Docetaxel + Doxorubicin
  - Paclitaxel or Doxorubicin monotherapy
Uterine Neoplasms
Endometrial Carcinoma
- - Preferred
  • Carboplatin + Paclitaxel +/- Trastuzumab (if HER2 positive)
  - Other
  • Cisplatin + Doxorubicin +/- Paclitaxel
  • Carboplatin + Paclitaxel + Bevacizumab
  • Ifosfamide or Cisplatin + Paclitaxel
  • Everolimus + Letrozole
  • Cisplatin
  • Carboplatin
  • Doxorubicin
  • Topotecan
  • Bevacizumab
  • Temsirolimus
  • Docetaxel
  - Hormone Therapy
  • Methylprogesterone/Tamoxifen (alternating)
  • Megestrol/Tamoxifen (alternating)
  • Aromatase inhibitors
  • Tamoxifen
  • Fulvestrant
Uterine Sarcoma
- - Preferred
  • Doxorubicin
  • Docetaxel + Gemcitabine
  • Doxorubicin + Olaratumab
  - Other
  • Doxorubicin + Ifosfamide or Dacarbazine
  • Gemcitabine + Dacarbazine or Vinorelbine
  • Dacarbazine or Gemcitabine or Epirubicin or Ifosfamide or Doxorubicine or Pazopanib or Temozolomide or Trabectedin or Eribulin
  - Hormone Therapy
  • Preferred
  - Aromatase inhibitors
  • Other
  - Fulvestrant
  - Megestrol
  - Medroxyprogesterone
  - GnRH analogs

Vulvar Cancer

Chemoradiation
- - Preferred
  • Cisplatin
  - Other
  • 5-FU + Mitomycin or Cisplatin
Recurrence
- - Preferred
  • Cisplatin or Carboplatin
  • Cisplatin or Carboplatin + Paclitaxel +/- Bevacizumab
  - Other
  • Paclitaxel
  • Cisplatin + Vinorelbine
  • Erlotinib
  • Cisplatin + Gemcitabine
  • Carboplatin + Paclitaxel + Bevacizumab
  - Certain useful circumstances
  • Pembrolizumab