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Desmoid Tumors
0022-534 7/87 /1381-0112$02.00/0 Vol. 138, July
THE JOURNAL OF UROLOGY
Copyright© 1987 by The Williams & Wilkins Co.
Printed in U.S.A.
Editorial DESMOID TUMORS The 3 articles in this issue of the Journal by Gianis and associates (page 152), Lattimer and associates (page 133), and Pasciak and Kozlowski (page 145), reporting on the postoperative development of large desmoid tumors, hold a message for the clinician. In this era of surgery often combined with chemotherapy for treatment of urological tumors evidence of subsequent abdominal tumor development is considered tantamount to relapse of the primary tumor. To assume reflexly primary tumor relapse and to invoke adjuvant treatment programs, such as radiotherapy or combination chemotherapy, in the absence of a tissue diagnosis would be major clinical mistakes. This is particularly true if there are no other stigmata of tumor progression, that is serum marker activity, negative chest findings, nutritional depletion, multiple organ involvement and so forth, and the recurrence is localized, albeit large in volume. There are valuable clues in these 3 reports to support the correct contention that surgical resection is the treatment of choice without other forms of pre-treatment. The relapse was long delayed and most malignant bulky tumor relapse is earlier. Malignant relapse often is directed at multiple organs and it was localized in the cases reported. There were no other stigmata of advanced disease and the patients had good nutritional status. Also, the tumors appeared to be based in the scar of prior incisions. As a general rule, the better differentiated a tumor, the less
112
sensitive it is to chemotherapy or radiotherapy (seminoma of course is a great exception). However, the mesenchymal group, particularly those of predominantly fibroblastic and desmoplastic composition, respond poorly if at all to chemotherapy or radiotherapy. These desmoid tumors predictably would be nonresponders. The mechanism of the development of desmoid tumors is intriguing. It seems to be an expression of abnormal regulation of healing connective tissue. The stimulus for this rare local response of confined deregulation appears to be surgical wound healing. It is not a hypertrophic response as in keloid formation but rather a continued proliferation of fibroblasts locally with no apparent down regulation with time or by local factors. Some of these tumors may progress to infiltrate or obstruct adjacent organs as noted in case 2 of the article by Lattimer and associates. Rare as these tumors are they illustrate a broader more common principle that a tissue diagnosis and total resection, if possible, are indicated as primary treatment of the atypical lesion developing late postoperatively.
John P. Donohue Department of Urology Indiana University Medical Center Indianapolis, Indiana
THE JOURNAL OF UROLOGY
Copyright© 1987 by The Williams & Wilkins Co.
Printed in U.S.A.
Editorial DESMOID TUMORS The 3 articles in this issue of the Journal by Gianis and associates (page 152), Lattimer and associates (page 133), and Pasciak and Kozlowski (page 145), reporting on the postoperative development of large desmoid tumors, hold a message for the clinician. In this era of surgery often combined with chemotherapy for treatment of urological tumors evidence of subsequent abdominal tumor development is considered tantamount to relapse of the primary tumor. To assume reflexly primary tumor relapse and to invoke adjuvant treatment programs, such as radiotherapy or combination chemotherapy, in the absence of a tissue diagnosis would be major clinical mistakes. This is particularly true if there are no other stigmata of tumor progression, that is serum marker activity, negative chest findings, nutritional depletion, multiple organ involvement and so forth, and the recurrence is localized, albeit large in volume. There are valuable clues in these 3 reports to support the correct contention that surgical resection is the treatment of choice without other forms of pre-treatment. The relapse was long delayed and most malignant bulky tumor relapse is earlier. Malignant relapse often is directed at multiple organs and it was localized in the cases reported. There were no other stigmata of advanced disease and the patients had good nutritional status. Also, the tumors appeared to be based in the scar of prior incisions. As a general rule, the better differentiated a tumor, the less
112
sensitive it is to chemotherapy or radiotherapy (seminoma of course is a great exception). However, the mesenchymal group, particularly those of predominantly fibroblastic and desmoplastic composition, respond poorly if at all to chemotherapy or radiotherapy. These desmoid tumors predictably would be nonresponders. The mechanism of the development of desmoid tumors is intriguing. It seems to be an expression of abnormal regulation of healing connective tissue. The stimulus for this rare local response of confined deregulation appears to be surgical wound healing. It is not a hypertrophic response as in keloid formation but rather a continued proliferation of fibroblasts locally with no apparent down regulation with time or by local factors. Some of these tumors may progress to infiltrate or obstruct adjacent organs as noted in case 2 of the article by Lattimer and associates. Rare as these tumors are they illustrate a broader more common principle that a tissue diagnosis and total resection, if possible, are indicated as primary treatment of the atypical lesion developing late postoperatively.
John P. Donohue Department of Urology Indiana University Medical Center Indianapolis, Indiana