- Email: [email protected]
Parosteal osteosarcoma
ABSTRACTS
Clinical Aspects of the Rhabdoid Tumor of the Kidney: A Report of the National WUms" Tumor Study Group. N.F.
Palmer and W. Sutow. Med Pcdiatr Oncol 11:242 245, (July-August), 1983. Rhabdoid tumor of the kidney (RTK), originally described as a monoplastic sarcomatous variant of Wilms' tumor, is now recognized as a highly malignant, non-Wilms' tumor, possibly of neuroectodermal origin. Twenty-one National Wilms' Tumor Study patients with this tumor were treated in the years I969 through 1978. One patient only is continuously disease free, and another is surviving disease free following excision of bilateral pulmonary metastases. Eighteen of the 19 patients who relapsed died, 15 within one year of diagnosis, all with progressive tumor growth. The rapid appearance of metastases (mean 4 months), often to multiple sites, and short subsequent survival signal a very malignant tumor resistam to current treatment strategems. The authors clarify the establishment of the rhabdoid tumor of the kidney as a distinct entity and specifically not a Wilms' tumor. The young age of the involved children (mean age of 18 months) and the aggressive nature of the malignancy are emphasized. In addition, the small number of patients involved makes national or regional reporting of these cases essential to improvement in their care and outcome.--Elaine Wilt
201
Parosteal Osteosarcoma. M. CampanaccL P. Picci, F. Gher-
linzoni, et al. J Bone Joint Surg 66B:313-321, (May), 1984. From a study of 41 patients with parosteal osteosarcoma, the natural history of the condition, and particularly the prognosis as related to histological grading and surgical treatment, is described. Surgical excision was considered adequate when the tumor, together with a cuff of normal tissue completely encasing it, was removed. There were no local recurrences in the 12 cases in which this had been done. Thirteen tumors were classified as histological Grade I (the least malignant). Nine developed local recurrences but none pulmonary metastasis. Of the 14 classified as Grade II, 10 recurred locally, 5 of these developing pulmonary metastasis and subsequent death. Four of these tumors had intramedullary spread at the time of surgery. Eight patients had Grade III tumors. Seven had intramedullary spread at the time of their initial surgery. Four of these developed metastatic disease. There were no Grade IV tumors. On the basis of these resutts, the authors feel that treatment should consist primarily of local excision of the tumor together with a wide margin of normal tissue. In Grade III and IV tumors with intramedullary spread, chemotherapy should be considered.--G. C. Bennet
Yolk Sac Tumor of the Testicle: Is Retroperitoneal Lymph Node Dissection Necessary? Y. Homsy, F. Arrojo-Vila, N. IV-S Neuroblastoma: A Cooperative Study of 30 Children.
A.R. Mancini, P. Rosito, A. Vitelli, et al. Med Pediatr Oncol 12:218-220, 1984. The clinical features of 30 children with IV-S neuroblastoma have been reviewed. They were treated at 11 Italian pediatric institutions in the period 1970 to 1981. Patients with IV-S neuroblastoma comprised 6.1% of all the neuroblastoma cases observed during that period. The mean age at diagnosis was 3 months (range, birth to I I months). The adrenal gland was affected most often (57% of patients), but tumors of the chest and neck were also encountered as "primary" lesions. No specific "primary" lesion could be identified in 10 children. The most frequent site of widespread disease was the liver (87%). Neither the size of the "primary" tumor nor the number or size of subcutaneous nodules was of prognostic significance. Unfavorable features were age less than 2 months and clinical signs of pulmonary, renal, or hepatic enbarrassment. These data confirm the need for prompt intervention in the infant who is suffering from compression of these vital structures by the enlarged liver. Radiation therapy is preferred for this purpose because of the often severe toxicity that is associated with chemotherapy in infants. One, and possibly 2 babies in this series died of chemotherapy-related complications. This large series adds to the similar series of Evans et al to provide a large number of infants with this unique form of neuroblastoma. Both studies present complementary findings, and both encourage avoidance of chemotherapy. This study reports 2 cases of late recurrence in the "primary" tumor and underscores the recommendation for resection of the "primary" tumor when safely possible.--Elaine Wilt
Khoriaty, et al. J Urol 132:532-536, (September), 1984. Experience with 11 cases of yolk sac tumors that were treated between 1960 and 1982 is reported. The role of lymphadenectomy in yolk sac tumors ia analyzed critically and put into perspective with the use of serum alphafetoprotein. A flow sheet is presented for the management of yolk sac tumors. It seems that the indication for lymphadenectomy in yolk sac tumor should be restricted to patients with persistently elevated serum alpha-fetoprotein after inguinal orchiectomy and chemotherapy.--George Holcomb. Jr.
Infantile Hemangioendothelioma of the Liver: A RadiologicPathologic Clinical Correlation. A.H. Dachman, J.E. Lich-
tenstein, A.C. Friedman, et al. Am J Roentgenol 140:10911096, (June), 1983. The authors reviewed 25 cases of infantile hemangioendothelioma of the liver from the A F I P files. The patients included 18 girls and 9 boys; 13 presenting within the first month of life and 25 before 6 months of age. The most common presenting features were a palpable mass, hepatomegaly, or diffuse abdominal enlargement (26 patients) with only one presenting with concurrent congestive heart failure. Five of the 27 also had cutaneous hemangiomas. Plain radiographs, sonography, and nuclear scans often localized disease to the liver whereas contrast-enhanced C T scans and arteriography better defined the extent of the l e s i o n . Randall W. Powell
Clinical Aspects of the Rhabdoid Tumor of the Kidney: A Report of the National WUms" Tumor Study Group. N.F.
Palmer and W. Sutow. Med Pcdiatr Oncol 11:242 245, (July-August), 1983. Rhabdoid tumor of the kidney (RTK), originally described as a monoplastic sarcomatous variant of Wilms' tumor, is now recognized as a highly malignant, non-Wilms' tumor, possibly of neuroectodermal origin. Twenty-one National Wilms' Tumor Study patients with this tumor were treated in the years I969 through 1978. One patient only is continuously disease free, and another is surviving disease free following excision of bilateral pulmonary metastases. Eighteen of the 19 patients who relapsed died, 15 within one year of diagnosis, all with progressive tumor growth. The rapid appearance of metastases (mean 4 months), often to multiple sites, and short subsequent survival signal a very malignant tumor resistam to current treatment strategems. The authors clarify the establishment of the rhabdoid tumor of the kidney as a distinct entity and specifically not a Wilms' tumor. The young age of the involved children (mean age of 18 months) and the aggressive nature of the malignancy are emphasized. In addition, the small number of patients involved makes national or regional reporting of these cases essential to improvement in their care and outcome.--Elaine Wilt
201
Parosteal Osteosarcoma. M. CampanaccL P. Picci, F. Gher-
linzoni, et al. J Bone Joint Surg 66B:313-321, (May), 1984. From a study of 41 patients with parosteal osteosarcoma, the natural history of the condition, and particularly the prognosis as related to histological grading and surgical treatment, is described. Surgical excision was considered adequate when the tumor, together with a cuff of normal tissue completely encasing it, was removed. There were no local recurrences in the 12 cases in which this had been done. Thirteen tumors were classified as histological Grade I (the least malignant). Nine developed local recurrences but none pulmonary metastasis. Of the 14 classified as Grade II, 10 recurred locally, 5 of these developing pulmonary metastasis and subsequent death. Four of these tumors had intramedullary spread at the time of surgery. Eight patients had Grade III tumors. Seven had intramedullary spread at the time of their initial surgery. Four of these developed metastatic disease. There were no Grade IV tumors. On the basis of these resutts, the authors feel that treatment should consist primarily of local excision of the tumor together with a wide margin of normal tissue. In Grade III and IV tumors with intramedullary spread, chemotherapy should be considered.--G. C. Bennet
Yolk Sac Tumor of the Testicle: Is Retroperitoneal Lymph Node Dissection Necessary? Y. Homsy, F. Arrojo-Vila, N. IV-S Neuroblastoma: A Cooperative Study of 30 Children.
A.R. Mancini, P. Rosito, A. Vitelli, et al. Med Pediatr Oncol 12:218-220, 1984. The clinical features of 30 children with IV-S neuroblastoma have been reviewed. They were treated at 11 Italian pediatric institutions in the period 1970 to 1981. Patients with IV-S neuroblastoma comprised 6.1% of all the neuroblastoma cases observed during that period. The mean age at diagnosis was 3 months (range, birth to I I months). The adrenal gland was affected most often (57% of patients), but tumors of the chest and neck were also encountered as "primary" lesions. No specific "primary" lesion could be identified in 10 children. The most frequent site of widespread disease was the liver (87%). Neither the size of the "primary" tumor nor the number or size of subcutaneous nodules was of prognostic significance. Unfavorable features were age less than 2 months and clinical signs of pulmonary, renal, or hepatic enbarrassment. These data confirm the need for prompt intervention in the infant who is suffering from compression of these vital structures by the enlarged liver. Radiation therapy is preferred for this purpose because of the often severe toxicity that is associated with chemotherapy in infants. One, and possibly 2 babies in this series died of chemotherapy-related complications. This large series adds to the similar series of Evans et al to provide a large number of infants with this unique form of neuroblastoma. Both studies present complementary findings, and both encourage avoidance of chemotherapy. This study reports 2 cases of late recurrence in the "primary" tumor and underscores the recommendation for resection of the "primary" tumor when safely possible.--Elaine Wilt
Khoriaty, et al. J Urol 132:532-536, (September), 1984. Experience with 11 cases of yolk sac tumors that were treated between 1960 and 1982 is reported. The role of lymphadenectomy in yolk sac tumors ia analyzed critically and put into perspective with the use of serum alphafetoprotein. A flow sheet is presented for the management of yolk sac tumors. It seems that the indication for lymphadenectomy in yolk sac tumor should be restricted to patients with persistently elevated serum alpha-fetoprotein after inguinal orchiectomy and chemotherapy.--George Holcomb. Jr.
Infantile Hemangioendothelioma of the Liver: A RadiologicPathologic Clinical Correlation. A.H. Dachman, J.E. Lich-
tenstein, A.C. Friedman, et al. Am J Roentgenol 140:10911096, (June), 1983. The authors reviewed 25 cases of infantile hemangioendothelioma of the liver from the A F I P files. The patients included 18 girls and 9 boys; 13 presenting within the first month of life and 25 before 6 months of age. The most common presenting features were a palpable mass, hepatomegaly, or diffuse abdominal enlargement (26 patients) with only one presenting with concurrent congestive heart failure. Five of the 27 also had cutaneous hemangiomas. Plain radiographs, sonography, and nuclear scans often localized disease to the liver whereas contrast-enhanced C T scans and arteriography better defined the extent of the l e s i o n . Randall W. Powell