- Email: [email protected]
Congenital mesoblastic nephroma: Relationship to other renal tumors of infancy
218
ABSTRACTS
sis. The authors developed a technique of measuring epiphyseal extrusion beyond the confines of the acetabulum. The addition of an index of abnormal extrusion was found to be statistically very useful when added to the Catterall Classification in prognosticating the course of the involved femoral head. Anthony H. Alter Lengthening of the Lower Extremity by the Wagner Method: a Review of the Boston Childrens" Hospital Experiencce.
R. W. Ferd, D. J. Riseborough. J Bone Joint Surg 63:11221131, (September), 1981. Thirty eight patients who underwent forty leg lengthenings were studied. This included twenty four femoral and sixteen tibial lengthenings. The surgical technique was described using the Wagner instrumentation. Thirty five complications occurred in thirty eight patients, all of which were regarded as minor. The most common were loosening of fixation, minor pin tract infection, and various fractures through the segment a n d / o r fixation devices. The authors conclude that, although the complications are numerous and of low morbidity, the Wagner technique remains the procedure of choice for limb lengthening in cooperative patients for whom the severity of limb length inequality merits major surgical i n t e r v e n t i o n . Anthony H. Alter Congenital Pseudarthrosis of the Tibia. R. T. Morrissy, E. J.
Riseborough, J. E. Hall. J Bone Joint Surg 63B:367-375, (August), 198 I. In a review of 40 cases of congenital pseudarthrosis of the tibia, the methods of treatment and its results were assessed in an effort to find the most effective treatment and indications of when further treatment would be best avoided in favour of amputation. The surgical procedure of composite skin and bone pedicle grafting from the other leg was the superior technique. Poor results in terms of union or shortening were likely if grafting was still needed in older childreen beyond 6 yr or in those with rapid absorption of grafts. In these amputation yielded a more acceptable result, The presence of neurofibrornatosis in half of the patients did not affect the result but of the 20 so affected, gliomata of the central nervous system developed in five.--M. G. H. Smith. A Histochemical Study of Muscle in Club Foot. D. H. Gray, J. M. Katz. J Bone Joint Surg 63B:417-423, (August), 1981.
In a study of muscle biopsy material from 62 patients with club foot, evidence is produced that in soleus (but not other muscles) changes in the composition of the muscle and reduction in the number of fibers may have been caused by defective innervation in the developing leg.--M. G. H. Smith.
NERVOUS SYSTEM Spontaneous Intracranial Hemorrhage. P. Woo, C. B. Sed-
zimir, R. V. Jeffreys, and M. D. M. Shaw. J Neurol Neurosurg Psychiart 44:373, (April), 1981 Proceedings of the Society of Britsh Neurological Surgeons. The authors reported on 20 patients aged less than 16 yr admitted to the Regional Neurosurgical Unit over a period of
10 yr. In eight cases an arteriovenous malformation was found, in four an aneurysm, and in one, hemophilia. No cause was found in seven cases. Overall, the mortality rate was 40% but only 25% for children with a vascular lesion. Surgical removal was attempted in only three, two of whom survived with severe disability. The authors conclude that in the absence of a vascular lesion, subcortical hematomata should be removed through a craniotomy relatively early, but deeply placed hematomata should be treated conservatively.--R. J. Brereton
NEOPLASMS Wilms" Tumor: Its Morphology in Patients Under One Year of Age. Nancy Ugarte, F. Gonzalez-Crussi, and Wet Hsueh.
Cancer 48:346-353, (July), 1981. The entity, "congenital mesoblastic nephroma," as a common tumor of infancy, has been recognized for a decade. The National Wilms' Tumor Study has identified an additional group of unusual tumors found in infants and previously included in the category of Wilm's' Tumor. This report describes a large institutional series (1955 1979, at Chicago Memorial Hospital) emphasizing that such unusual tumors are the rule, rather than the exception among infants with renal neoplasms discovered during the first year of life. Of 32 patients in this category, only I 1 had a neoplasm which could be described as typical Wilms" Tumors, and two of these were bilateral. Included in this series were eight patients with typical congenital mesoblastic nephroma. The other groups included: (a) six of the rhabdomyomatous (not rhabdoid) forms of Wilms' Tumors, three of which were bilateral, (h) two cases of "cystic" nephroblastoma, (c) two renal sarcomas, including one "clear cell" type, and one rhabdoid. No case which could bc described as a typical Wilms' Tumor was found in a patient less than 6 me. Of the six rhabdomyomatous nepfiroblastomas, there have been two tumor deaths, and one therapy death. The patient with a "clear cell" sarcoma is alive with hepatic metastasis, and the infant with a rhabdoid sarcoma is alive 3 me since surgery. Daniel M. Hays Congenital Mesoblastic Nephroma: Relationship to Other Renal Tumors of Infancy, H. M. Snyder, I11, E. E. Lack, A.
Chetty-Baktavizian, S. B. Bauer, A. H. Colodny. and A, B. Retik. J. Urol. 126:513-516, (October), 1981. Although nephroblastomas (Wilms' tumor) do occur in the newborn, most of the solid renal tumors in this age group are found to be congenital mesoblastic nephromas, a distinctly different pathologic entity. While a more cellular histologic variant of congenital mesoblastic nephroma has been recognized recently, which occasionally may follow a malignant course, the tumor with conventional histology has never been reported to metastasize and follows a benign course when treated by adequate local excision alone. The authors report on their 50 years of experience with 11 patients with this tumor (nine cases with conventional histology and two with cellular variant histology). Typically, the tumor presented in the newborn as a large asymptomatic mass. Local invasion was never seen and the tumors were resected without difficulty. Grossly, the tumors usually resembled a uterine "fibroid" and, unlike nephroblastomas, rarely exhibited hemorrhage or
ABSTRACTS
necrosis. Microscopically, the conventional histologic congenital mesoblastic nephroma was composed of sheets of spindle shaped cells. The cellular variant of congenital mesoblastic nephroma exhibited increased cellularity and a higher mitotic index. The relationship of congenital mesoblastic nephromas to other neonatal renal tumors is discussed and a unifying schema of neoplasia in infantile renal tumors is proposed.--George Holcomb, Jr.
Childhood Rhabdomyosarcoma: A Retrospective Study of 7 0 C a s e s . M. Car/i, B. DeBernardi, E. Madon, G. Paolucci,
M. Castello and C. Messina. Med Surg Ped 1,497-504, 1979. A retrospective study of 70 children with rhabdomyosarcoma treated in different Italian pediatric centers from 1968 to 1978, has been carried out to evaluate frequency, outcome and prognostic factors. The primary tumor was in the head and neck (24), orbit (10), trunk (14), retroperitoneum (5), perineum-anus (3), extremities (3), biliary tract (1). The embryonal ( 5 4 ) a n d alveolar (13) histologic subtypes accounted for the majority of cases; 13 patients were at stage I, 12 at stage II, 37 at stage III, and 8 at stage IV. Fifty three patients received coordinated therapy including surgery, radiotherapy and combination chemotherapy with vincristine and actinomycin-D (4), vincristine, actynomycin-D and cyclophosphamide and adriamycin (32), peptichemio, vincristine, actinimycin-D, cyclophosphamide and adriamycin (9). Seventeen patients treated before 1974 received less radical therapy and overall received inadequate chemotherapy. Thirty six of 70 cases are alive at a followup of 18-89 me after diagnosis. Successful treatment failed in 39 of 70 patients. Twenty two patients did not reach complete response; one died of complication and sixteen developed local or local and generalised recurrence of tumor. Correlation of survival with the primary site, extent of disease, histologic subtype of the tumor and chemotherapy are discussed.--Paolo Magillo
of the Gallbladder. S. Mihara, H. Yano, H. Matsurnoto, et al. J Jpn Soc Pediatr Surg 17:895903, (August), 1981. Rhabdomyosarcoma
Rhabdomyosarcoma of the gallbladder seen in a 6 yr old girl was reported. The patient was explored for jaundice, fever, and epigastralgia. The gallbladder and the common bile duct were filled with numerous botryoid masses. Cholecystectomy, removal of botryoid masses through choledochotomy, and T-tube drainage were carried out. Histology was embryonal rhabdomyosarcoma and excision of the common bile duct and hepatic duct were carried out 14 days after the initial operation. The patient was treated with combined radiotherapy and chemotherapy (Vincristine, Actinomycin-D, and Cyclophosphamide) and is doing well 1 yr after the operation.--H. Suzuki
219
Non-Hodgkin lymphoma in Children. Results in 26 Patients treated with IGG-74 Protocol. A. Cornelli, B. DeBernardi, G.
Dini, C. Cozzutto, A. Rizzo, G. Ricci, P. Tom&, P. G. Mori, and L. Massimo. Med Surg Ped 1:477-486, 1979. This paper presents the results of a new protocol, labeled L N H - I G G - 7 4 , which was applied to 26 children diagnosed in the period September 74-November 79 at Giannina Gaslini Children's Hospital. Treatment consisted of multichemotherapy combinations administered continuously for 1-2 yr, in relation to extension of the disease at onset. Twenty children achieved complete remission (CR) before starting maintenance; however most of them responded to therapy dramatically acquiring CR in 1-3 wk. None of the six patients bearing abdominal unresectable lymphoma acquired CR; they died in 1 to 6 me from diagnosis. Two patients relapsed at 18 and 27 me respectively; both are alive at 9 and 4 me from relapse, respectively; the remaining patients are alive free of disease from 2 to 62 me. Ten of them are off-therapy from 7 to 37 me. No patients died because of therapy. No case of meningeal relapse has been registered. Abdominal nonlocalised lymphoma unfortunately still represents a group at poor prognosis.--Paolo Magillo Histiocytosis X: Oncologic Aspects. G. Dini, A. Cornel/i, B.
De Berhardi, F. Gatto, N. Tontoli, G. Ricci, P. Torni~, M. Guarino, P. Magillo, L. Massirno. Gaslini, 12, 172, 1980. Histiocytosis X (HX) encompasses a large spectrum of clinical patterns characterized by different severity and evolution. The tendency to admit patients with monostatic lesion in orthopedic departments and those with more than one lesion, or disseminated disease, to medical departments has determined artificial distinctions and allowed conflicting ideas about the natural history of HX. In this paper a series of 23 children is reported, 13 with one lesion, and 10 with multiple lesions, or disseminated disease. All patients belonging to the first group are presently alive and well, although six of them underwent one or more relapse episodes; ten patients belonging to the second group are alive (seven without evidence of disease) while one died after multiple relapses 4 yr from diagnosis. The authors report the impression that adjuvant treatment did not influence the course of the disease in the first group, while appeared to modify the evolution of the disease in patients of the second group.-Paolo Magillo Management of Children with Unilateral Ovarian Malignancies. M. S. Piver. Pediatr Ann 10:46-52, (May), 1981.
The author gives a review of the various malignancies seen in children with recommendations for their treatment. In summary, unilateral salpingo-oophorectomy is the Preferred treatment for most ovarian malignancies in children. Nonconservative surgery--that is, bilateral salpingo-oophorectomy--should b e reserved for most cases of dysgerminoma, gonadoblastoma, and invasive serous, mucinous, clear cell and endometroid adenocarcinoma of the ovary.--David L. Collins
ABSTRACTS
sis. The authors developed a technique of measuring epiphyseal extrusion beyond the confines of the acetabulum. The addition of an index of abnormal extrusion was found to be statistically very useful when added to the Catterall Classification in prognosticating the course of the involved femoral head. Anthony H. Alter Lengthening of the Lower Extremity by the Wagner Method: a Review of the Boston Childrens" Hospital Experiencce.
R. W. Ferd, D. J. Riseborough. J Bone Joint Surg 63:11221131, (September), 1981. Thirty eight patients who underwent forty leg lengthenings were studied. This included twenty four femoral and sixteen tibial lengthenings. The surgical technique was described using the Wagner instrumentation. Thirty five complications occurred in thirty eight patients, all of which were regarded as minor. The most common were loosening of fixation, minor pin tract infection, and various fractures through the segment a n d / o r fixation devices. The authors conclude that, although the complications are numerous and of low morbidity, the Wagner technique remains the procedure of choice for limb lengthening in cooperative patients for whom the severity of limb length inequality merits major surgical i n t e r v e n t i o n . Anthony H. Alter Congenital Pseudarthrosis of the Tibia. R. T. Morrissy, E. J.
Riseborough, J. E. Hall. J Bone Joint Surg 63B:367-375, (August), 198 I. In a review of 40 cases of congenital pseudarthrosis of the tibia, the methods of treatment and its results were assessed in an effort to find the most effective treatment and indications of when further treatment would be best avoided in favour of amputation. The surgical procedure of composite skin and bone pedicle grafting from the other leg was the superior technique. Poor results in terms of union or shortening were likely if grafting was still needed in older childreen beyond 6 yr or in those with rapid absorption of grafts. In these amputation yielded a more acceptable result, The presence of neurofibrornatosis in half of the patients did not affect the result but of the 20 so affected, gliomata of the central nervous system developed in five.--M. G. H. Smith. A Histochemical Study of Muscle in Club Foot. D. H. Gray, J. M. Katz. J Bone Joint Surg 63B:417-423, (August), 1981.
In a study of muscle biopsy material from 62 patients with club foot, evidence is produced that in soleus (but not other muscles) changes in the composition of the muscle and reduction in the number of fibers may have been caused by defective innervation in the developing leg.--M. G. H. Smith.
NERVOUS SYSTEM Spontaneous Intracranial Hemorrhage. P. Woo, C. B. Sed-
zimir, R. V. Jeffreys, and M. D. M. Shaw. J Neurol Neurosurg Psychiart 44:373, (April), 1981 Proceedings of the Society of Britsh Neurological Surgeons. The authors reported on 20 patients aged less than 16 yr admitted to the Regional Neurosurgical Unit over a period of
10 yr. In eight cases an arteriovenous malformation was found, in four an aneurysm, and in one, hemophilia. No cause was found in seven cases. Overall, the mortality rate was 40% but only 25% for children with a vascular lesion. Surgical removal was attempted in only three, two of whom survived with severe disability. The authors conclude that in the absence of a vascular lesion, subcortical hematomata should be removed through a craniotomy relatively early, but deeply placed hematomata should be treated conservatively.--R. J. Brereton
NEOPLASMS Wilms" Tumor: Its Morphology in Patients Under One Year of Age. Nancy Ugarte, F. Gonzalez-Crussi, and Wet Hsueh.
Cancer 48:346-353, (July), 1981. The entity, "congenital mesoblastic nephroma," as a common tumor of infancy, has been recognized for a decade. The National Wilms' Tumor Study has identified an additional group of unusual tumors found in infants and previously included in the category of Wilm's' Tumor. This report describes a large institutional series (1955 1979, at Chicago Memorial Hospital) emphasizing that such unusual tumors are the rule, rather than the exception among infants with renal neoplasms discovered during the first year of life. Of 32 patients in this category, only I 1 had a neoplasm which could be described as typical Wilms" Tumors, and two of these were bilateral. Included in this series were eight patients with typical congenital mesoblastic nephroma. The other groups included: (a) six of the rhabdomyomatous (not rhabdoid) forms of Wilms' Tumors, three of which were bilateral, (h) two cases of "cystic" nephroblastoma, (c) two renal sarcomas, including one "clear cell" type, and one rhabdoid. No case which could bc described as a typical Wilms' Tumor was found in a patient less than 6 me. Of the six rhabdomyomatous nepfiroblastomas, there have been two tumor deaths, and one therapy death. The patient with a "clear cell" sarcoma is alive with hepatic metastasis, and the infant with a rhabdoid sarcoma is alive 3 me since surgery. Daniel M. Hays Congenital Mesoblastic Nephroma: Relationship to Other Renal Tumors of Infancy, H. M. Snyder, I11, E. E. Lack, A.
Chetty-Baktavizian, S. B. Bauer, A. H. Colodny. and A, B. Retik. J. Urol. 126:513-516, (October), 1981. Although nephroblastomas (Wilms' tumor) do occur in the newborn, most of the solid renal tumors in this age group are found to be congenital mesoblastic nephromas, a distinctly different pathologic entity. While a more cellular histologic variant of congenital mesoblastic nephroma has been recognized recently, which occasionally may follow a malignant course, the tumor with conventional histology has never been reported to metastasize and follows a benign course when treated by adequate local excision alone. The authors report on their 50 years of experience with 11 patients with this tumor (nine cases with conventional histology and two with cellular variant histology). Typically, the tumor presented in the newborn as a large asymptomatic mass. Local invasion was never seen and the tumors were resected without difficulty. Grossly, the tumors usually resembled a uterine "fibroid" and, unlike nephroblastomas, rarely exhibited hemorrhage or
ABSTRACTS
necrosis. Microscopically, the conventional histologic congenital mesoblastic nephroma was composed of sheets of spindle shaped cells. The cellular variant of congenital mesoblastic nephroma exhibited increased cellularity and a higher mitotic index. The relationship of congenital mesoblastic nephromas to other neonatal renal tumors is discussed and a unifying schema of neoplasia in infantile renal tumors is proposed.--George Holcomb, Jr.
Childhood Rhabdomyosarcoma: A Retrospective Study of 7 0 C a s e s . M. Car/i, B. DeBernardi, E. Madon, G. Paolucci,
M. Castello and C. Messina. Med Surg Ped 1,497-504, 1979. A retrospective study of 70 children with rhabdomyosarcoma treated in different Italian pediatric centers from 1968 to 1978, has been carried out to evaluate frequency, outcome and prognostic factors. The primary tumor was in the head and neck (24), orbit (10), trunk (14), retroperitoneum (5), perineum-anus (3), extremities (3), biliary tract (1). The embryonal ( 5 4 ) a n d alveolar (13) histologic subtypes accounted for the majority of cases; 13 patients were at stage I, 12 at stage II, 37 at stage III, and 8 at stage IV. Fifty three patients received coordinated therapy including surgery, radiotherapy and combination chemotherapy with vincristine and actinomycin-D (4), vincristine, actynomycin-D and cyclophosphamide and adriamycin (32), peptichemio, vincristine, actinimycin-D, cyclophosphamide and adriamycin (9). Seventeen patients treated before 1974 received less radical therapy and overall received inadequate chemotherapy. Thirty six of 70 cases are alive at a followup of 18-89 me after diagnosis. Successful treatment failed in 39 of 70 patients. Twenty two patients did not reach complete response; one died of complication and sixteen developed local or local and generalised recurrence of tumor. Correlation of survival with the primary site, extent of disease, histologic subtype of the tumor and chemotherapy are discussed.--Paolo Magillo
of the Gallbladder. S. Mihara, H. Yano, H. Matsurnoto, et al. J Jpn Soc Pediatr Surg 17:895903, (August), 1981. Rhabdomyosarcoma
Rhabdomyosarcoma of the gallbladder seen in a 6 yr old girl was reported. The patient was explored for jaundice, fever, and epigastralgia. The gallbladder and the common bile duct were filled with numerous botryoid masses. Cholecystectomy, removal of botryoid masses through choledochotomy, and T-tube drainage were carried out. Histology was embryonal rhabdomyosarcoma and excision of the common bile duct and hepatic duct were carried out 14 days after the initial operation. The patient was treated with combined radiotherapy and chemotherapy (Vincristine, Actinomycin-D, and Cyclophosphamide) and is doing well 1 yr after the operation.--H. Suzuki
219
Non-Hodgkin lymphoma in Children. Results in 26 Patients treated with IGG-74 Protocol. A. Cornelli, B. DeBernardi, G.
Dini, C. Cozzutto, A. Rizzo, G. Ricci, P. Tom&, P. G. Mori, and L. Massimo. Med Surg Ped 1:477-486, 1979. This paper presents the results of a new protocol, labeled L N H - I G G - 7 4 , which was applied to 26 children diagnosed in the period September 74-November 79 at Giannina Gaslini Children's Hospital. Treatment consisted of multichemotherapy combinations administered continuously for 1-2 yr, in relation to extension of the disease at onset. Twenty children achieved complete remission (CR) before starting maintenance; however most of them responded to therapy dramatically acquiring CR in 1-3 wk. None of the six patients bearing abdominal unresectable lymphoma acquired CR; they died in 1 to 6 me from diagnosis. Two patients relapsed at 18 and 27 me respectively; both are alive at 9 and 4 me from relapse, respectively; the remaining patients are alive free of disease from 2 to 62 me. Ten of them are off-therapy from 7 to 37 me. No patients died because of therapy. No case of meningeal relapse has been registered. Abdominal nonlocalised lymphoma unfortunately still represents a group at poor prognosis.--Paolo Magillo Histiocytosis X: Oncologic Aspects. G. Dini, A. Cornel/i, B.
De Berhardi, F. Gatto, N. Tontoli, G. Ricci, P. Torni~, M. Guarino, P. Magillo, L. Massirno. Gaslini, 12, 172, 1980. Histiocytosis X (HX) encompasses a large spectrum of clinical patterns characterized by different severity and evolution. The tendency to admit patients with monostatic lesion in orthopedic departments and those with more than one lesion, or disseminated disease, to medical departments has determined artificial distinctions and allowed conflicting ideas about the natural history of HX. In this paper a series of 23 children is reported, 13 with one lesion, and 10 with multiple lesions, or disseminated disease. All patients belonging to the first group are presently alive and well, although six of them underwent one or more relapse episodes; ten patients belonging to the second group are alive (seven without evidence of disease) while one died after multiple relapses 4 yr from diagnosis. The authors report the impression that adjuvant treatment did not influence the course of the disease in the first group, while appeared to modify the evolution of the disease in patients of the second group.-Paolo Magillo Management of Children with Unilateral Ovarian Malignancies. M. S. Piver. Pediatr Ann 10:46-52, (May), 1981.
The author gives a review of the various malignancies seen in children with recommendations for their treatment. In summary, unilateral salpingo-oophorectomy is the Preferred treatment for most ovarian malignancies in children. Nonconservative surgery--that is, bilateral salpingo-oophorectomy--should b e reserved for most cases of dysgerminoma, gonadoblastoma, and invasive serous, mucinous, clear cell and endometroid adenocarcinoma of the ovary.--David L. Collins