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Clear Cell Sarcoma of the Kidney Medication

  • Author: Nita Seibel, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
Updated: Oct 25, 2015

Medication Summary

Patients with clear cell sarcoma of the kidney (CCSK) are treated with combination chemotherapy. The addition of doxorubicin to chemotherapeutic regimens has been shown to improve disease-free survival rates. Physicians caring for a patient with clear cell sarcoma of the kidney should consult a pediatric oncologist affiliated with a cancer center that participates in national or international trials to determine the current standard treatment protocol and to determine whether the patient is eligible for an investigational protocol.


Antineoplastic agents

Class Summary

Cancer chemotherapy is based on an understanding of tumor cell growth and how drugs affect this growth. After cells divide, they enter a period of growth (ie, gap 1 [G1]), followed by DNA synthesis (ie, S phase), a premitotic phase (ie, gap 2 [G2]), and, finally, mitotic cell division (ie, M phase).

The rate of cell division varies among tumors. Most lesions of common cancers increase very slowly in size compared to normal tissues, and the rate of growth may even be slower in large tumors. This difference allows normal cells to recover more quickly from chemotherapy than malignant cells, and provides the rationale behind current cyclic dosage schedules.

Antineoplastic agents interfere with cell reproduction. Some agents are cell cycle specific, while others (eg, alkylating agents, anthracyclines, cisplatin) are not phase specific. Cellular apoptosis (ie, programmed cell death) is also a potential mechanism in many antineoplastic agents.

Refer to the specific protocols for duration of therapy with each drug and timing of administration within each treatment cycle.

Vincristine (Oncovin)


A vinca alkaloid that inhibits cellular mitosis by inhibiting intracellular tubulin function, binding to microtubules, and inhibiting the synthesis of spindle proteins.

Doxorubicin (Adriamycin, Rubex)


Inhibits topoisomerase II and produces free radicals, which may cause the destruction of DNA. The combination of these 2 events can, in turn, inhibit the growth of neoplastic cells.

Cyclophosphamide (Cytoxan, Neosar)


Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

Etoposide (Toposar, VePesid, VP-16)


Inhibits topoisomerase II and causes DNA strand breakage causing cell proliferation to arrest in late S or early G2 portion of the cell cycle.


Uroprotective antidote

Class Summary

Mesna is a prophylactic detoxifying agent used to inhibit hemorrhagic cystitis caused by ifosfamide and cyclophosphamide.

In the kidney, mesna disulfide is reduced to free mesna. Free mesna has thiol groups that react with acrolein, the ifosfamide or cyclophosphamide metabolite considered responsible for urotoxicity.

Mesna (Mesnex)


Inactivates acrolein and prevents urothelial toxicity without affecting cytostatic activity. Dose is dependent on dose of ifosfamide or cyclophosphamide, typically 60-100% of the antineoplastic agent used. May be administered as an initial bolus followed by IV continuous infusion or as intermittent IV infusions before and following chemotherapy regimen.


Colony-stimulating growth factors

Class Summary

These act as a hematopoietic growth factor that stimulates the development of granulocytes. They are used to treat or prevent neutropenia in patients receiving myelosuppressive cancer chemotherapy and to reduce the period of neutropenia associated with bone marrow transplantation. They are also used to mobilize autologous peripheral blood progenitor cells for bone marrow transplantation and to manage chronic neutropenia.

Filgrastim (Neupogen, G-CSF)


Granulocyte colony-stimulating factor that activates and stimulates production, maturation, migration, and cytotoxicity of neutrophils.

Contributor Information and Disclosures

Nita Seibel, MD Senior Investigator, Pediatric Section, Clinical Investigations Branch, Cancer Therapy Evaluation Program, Division of Cancer Treatment and Diagnosis, National Cancer Institute; Adjunct Professor of Pediatrics, George Washington University School of Medicine and Public Health; Attending Physician, Center for Cancer and Blood Disorders, Children's National Medical Center

Nita Seibel, MD is a member of the following medical societies: Alpha Omega Alpha, American Society of Clinical Oncology, American Academy of Pediatrics, American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD, FAAP Chief, Division of Hematology/Oncology/BMT, Gordon Teter Endowed Chair in Pediatric Cancer, Nationwide Children's Hospital; Professor of Pediatrics, Ohio State University College of Medicine

Timothy P Cripe, MD, PhD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Association for Cancer Research, American Pediatric Society, American Society of Gene and Cell Therapy, American Society of Pediatric Hematology/Oncology, Connective Tissue Oncology Society, Society for Pediatric Research, Children's Oncology Group

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American College of Healthcare Executives, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Kathleen M Sakamoto, MD, PhD Shelagh Galligan Professor, Division of Hematology/Oncology, Department of Pediatrics, Stanford University School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

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Large right-sided heterogeneous renal mass in a 9-month-old infant. Biopsy findings were consistent with clear cell sarcoma of the kidney.
Recurrent clear cell sarcoma of the kidney occurring in a lymph node 18 months after therapy.
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