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In Reply:

University of Michigan Medical School, Ann Arbor, MI

In the letter by Spunt and Pappo in this issue, exception is taken to my statement in the June 20, 2005, issue of the Journal of Clinical Oncology¹ that "medical oncologists giggle snidely at the phrase" nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). I assure them and every pediatric oncologist that I meant no offense. In that same editorial, I made another statement that "most medical oncologists are in awe of a cure rate of 25% in patients with distant metastatic sarcomas" reported by pediatricians. Spunt and Pappo reaffirm their preference for NRSTS.

Ferrari et al² coined the phrase "adult-type soft tissue sarcomas in pediatric age." I agreed that both terms are correct. Spunt argues, predominantly on epidemiologic grounds, that childhood NRSTS are not adult-type sarcomas. Ferrari offers that children with synovial sarcoma and malignant peripheral nerve sheath tumors (MPNSTs) are treated similarly as adult patients with the same tumors. Indeed, the medical treatment of teenagers and older children with synovial sarcoma or MPNSTs includes the same drugs, regimens, and doses as adults. Spunt argues that synovial sarcomas and MPNSTs are two of the most common sarcomas in children other than rhabdomyosarcoma, whereas the most common types of soft tissue sarcomas in adults are malignant fibrous histiocytoma, leiomyosarcoma, and liposarcoma. In fact, synovial sarcoma has a peak age of 30, and the ages of patients with MPNSTs are more similar to patients with liposarcoma than to those with synovial sarcoma. Both these tumors occur in older children and teenagers, but, obviously, there are many more adults than children.

The major thrust of my editorial, however, was not the naming of this group of tumors but rather a fervent plea for pediatric and medical oncologists, along with colleagues in orthopaedic oncology, surgical oncology, radiation oncology, sarcoma pathology, and imaging who are experts in the diagnosis and treatment of sarcomas, to work together and to jointly develop clinical trials. Sarcoma Alliance for Research through Collaboration is delighted with the recently approved Department of Defense project to treat patients with MPNSTs on a single protocol. As part of this study, we will collect the relevant biologic specimens and attempt to answer an important clinical question—whether chemosensitivity differs in patients with MPNSTs with or without neurofibromatosis. This protocol is histology-specific and addresses an important unresolved clinical question. We can compare children with adults who have MPNSTs, but I suspect differences in clinical course or outcome are unlikely to be significant.

Author's Disclosures of Potential Conflicts of Interest

The author or immediate family members indicated a financial interest. No conflict exists for drugs or devices used in a study if they are not being evaluated as part of the investigation. For a detailed description of the disclosure categories, or for more information about ASCO's conflict of interest policy, please refer to the Author Disclosure Declaration and the Disclosures of Potential Conflicts of Interest section in Information for Contributors.

Authors Employment Leadership Consultant Stock Honoraria Research Funds Testimony Other Laurence H. Baker Hope Foundation (A); Sarcoma Alliance for Research through Collaboration (A); Southwest Oncology Group Kanisa Pharmaceuticals Inc (A); Ascenta (A) Kanisa Pharmaceuticals Inc (A); Ascenta (A) Sanofi-Aventis (C); Eli Lilly (C); NIH (C); Robert Urich Foundation (B); Walther Foundation (C) Centacor (A); Bristol Myers Squibb (A); Ariad Pharmaceuticals (A)

Dollar Amount Codes (A) < $10,000 (B) $10,000-99,999 (C) $100,000 (N/R) Not Required

REFERENCES

1. Baker LH: Medical and pediatric oncology, not adult and pediatric oncology. J Clin Oncol 23:4003-4005, 2005[Free Full Text]

2. Ferrari A, Casanova M, Collini P, et al: Adult-type soft tissue sarcomas in pediatric-age patients: Experience at the Istituto Nazionale Tumori in Milan. J Clin Oncol 23:4021-4030, 2005[Abstract/Free Full Text]

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Related Correspondence

• Childhood Nonrhabdomyosarcoma Soft Tissue Sarcomas Are Not Adult-Type Tumors
  Sheri L. Spunt and Alberto S. Pappo
  JCO 2006 24: 1958-1959 [Full Text]

This Article Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Baker, L. H. Search for Related Content PubMed Articles by Baker, L. H. Related Articles Related Correspondence Social Bookmarking                            What's this?