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Lipoblastoma and liposarcoma in children: An analysis of 9 cases and a review of the literature
1748
INTERNATIONAL
A 30-month-old boy presented with increased abdominal girth and secondary enuresis. He had undergone removal of a right multicystic dysplastic kidney at age 5 months. Investigation showed a large cyst communicating via the left ejaculatory duct. The cyst was marsupialised, subtotally excised, and cauterized, with complete resolution of symptoms.-M.Iv? de la Hunt
Prospective Study of Outcome in Antenatally Diagnosed Renal Pelvis Dilatation. MS. Jaswon, L. Dibble, S. Puri, et al. Arch Dis Child 80:Fl35-F138, (March), 1999. The authors report a prospective study from an unselected district general hospital population of 104 infants with antenatally detected renal pelvis (anteroposterior diameter of 5 mm or greater). All the infants were investigated postnatally with renal ultrasonography and micturating cystography. Persisting postnatal dilatation was detected in 47 (45%) infants. Of these, the dilatation was believed to be idiopathic in 8 (7.6%), caused by pelviureteric obstruction in 4 (3.8%), and caused by renal dysplasia in 4 (3.8%). Vesico-ureteric retlux (VUR) was detected in 22% of the entire group, but was not related to the degree of renal pelvis dilatation (either antenatally or postnatally), and indeed 61% of those infants had had normal postnatal pelvic ultrasound findings. The finding of renal pelvis dilatation warrants full postnatal investigation. If this is limited to postnatal ultrasonography, significant VUR will be missed.-Mark Davenport
MUSCULOSKELETAL SYSTEM Treatment of Madelung’s Deformity by Lengthening and Relaxation of the Distal Extremity of the Radius by Ilizarov’s Technique. B. de Billy, E Gastaad, M. Repetto, et al. Eur J Pediatr Surg 7296-298, (October), 1997. Madelung’s deformity was first described in 1878. It is characterized by a typical deformity of the carpus; in addition to causing pain, it impedes mobility and esthetic appearance. Surgical correction can be performed during adolescence, the most frequently employed technique being conical osteotomy. The authors present a novel technique for lengthening and aligning the distal radial extremity using Ilizarov’s procedure. Five carpal joints were operated on in 3 13-year-old girls. An improved esthetic effect was obvious in all 3 cases. Mobility improved by 30” in the direction of extension, and pain always subsided immediately after surgery.-Thomas A. Angerpointner
NEOPLASMS Clinical Impact and Prognostic Value of Metaiodobenzylguanidine Imaging in Children With Metastatic Neuroblastoma. E Perel, J. Conway, M. Kletzel, etal. J Pediatr Hematol Oncol21:13-18, (January/Febrnary), 1999. This review of 30 patients with stage IV neuroblastoma from Chicago Memorial Children’s Cancer Service showed that MIBG imaging complements bone scanning in these children. MIBG scanning shows better correlation with outcome in children older than 1 year. They recommend both scans be performed.-Michael f? Hirsh
Detection of Neuroblastoma in the Bone Versus Aspiration. PA. Aronica, VT Pirrotta, Pediatr Hematol Oncol20:330-334, (July/August),
Marrow: Biopsy E.J. Yunis, et al. J 1998.
This review from Children’s Hospital of Pittsburgh of the mode of diagnosis in 57 neuroblastoma patients shows the complementary value of the bone marrow biopsy and aspiration. Together they have the highest yield for detection of metastatic disease, and both should be performed at the time of diagnosis.-Michael I! Hirsh
An Infant With Neuroblastoma Found Through Mass Screening Hisano, et al. J Pediatr Hematol October), 1998.
ABSTRACTS
and MYCN Amplification (MS). I: Taga, N. Okamoto, T. Oncol 20:486-488, (September/
This case report from Japan describes an 8-month-old boy whose left adrenal neuroblastoma was detected by a mass screening program. Usually such patients have a good prognosis, but this tumor underwent cytogenetic studies in which MYCN amplification was detected. The baby had a very bad course thereafter. This is an exception to the 97% survival rate for patients identified via mass screening.-Michael l? Hirsh Successful Treatment of a Patient With Stage IV Rhabdoid Tumor of the Kidney (RTK): Case Report and Review. PE. Waldron, B.M. Rodgers, M.D. Kelly, et al. J Pcdiatr Hematol Oncol 21:53-57, (January/February), 1999. The authors review the clinical course of a 31-month-old boy who presented with stage IV (pulmonary metastases) RTK, a rare form of renal neoplasm (1.8% of all tumors in the National Willms’ Tumor Study [NWTS]). Its overall 5-year survival rate in NWTS III was 25%. This patient underwent intensive multimodal therapy including surgery and chemotherapy. The authors recommend aggressive staging including abdominal lymph node sampling and CNS metastatic workup. The patient is disease-free 60 months after diagnosis.-Michael P Hirsh Lipoblastoma and Liposarcoma in Children: An Analysis of 9 Cases and a Review of the Literature. C.G. Millel; N.L. Yanchas J.E Magee, et al. Can J Surg 41:455-458, (December), 1998. Lipoblastomas reportedly occur primarily in infants and children under 3 years of age. Liposarcomas are most common in the 3rd to 7th decades of life. They are extremely unusual in children younger than 10 years of age. Of the 2,500 cases of liposarcoma reviewed by the United States Armed Forces Institute of Pathology (AFIP), 17 occurred in children under 16 years of age, but only 2 children were younger than 11 years. In this report, the 2 children with liposarcoma were aged 9 and 14 years. Of the 7 children with lipoblastoma, 5 were under 3 years of age. In children, liposarcomas usually occur on an extremity, as in both cases reported here. A truncal location is less common, and retroperitoneal tumors are uncommon. In a large series of lipoblastomas, 70% occurred on an extremity. Others reported a predilection for the trunk. The authors found only I occurring on an extremity. There are 2 types of lipoblastoma. One is well-encapsulated, superficial, and may mimic a lipoma. The other is deeper, with an infiltrative growth pattern. It is sometimes referred to as lipoblastomatosis. Two of the authors’ cases were poorly encapsulated. One liposarcoma in this series was a myxoid variant, which is the most common histological type among children. The other was a round cell type, which is very unusual in children. Lipoblastoma is definitively treated by complete resection. Reported recurrence rates range from 13% to 20%. In this series, all tumors except 1 were completely resected, and there have been no recurrences. Of the 17 pediatric liposarcomas in the AFIP series, there were 3 recurrences (18%) and 1 death (6%). One of the authors’ patients with liposarcoma was lost to follow-up; the other had complete resection and was disease-free at 6 months. The use of adjuvant therapy for childhood liposarcomas is not advocated for most completely resected tumors. The translocation anomaly of the tumor karyotype in case 8 is characteristic for myxoid liposarcoma. This anomaly has not been reported in lipoblastoma. The tumor karyotype of a number of lipoblastomas has been reported, and 6 of 7 (86%) show a breakpoint in chromosome 8, with variable rearrangements of the nuclear material. The authors examined the tumor karyotype in their most recent case of lipoblastoma and found a similar breakpoint in chromosome 8. This
INTERNATIONAL
1749
ABSTRACTS
anomaly has not been Tumor karyotype tumors. Microscopically. myxoid liposarcoma. Traditionally. age has as the authors found,
reported for liposarcoma. plays a significant role in differentiating these lipoblastoma can be indistinguishable from Clinically, the tumors often behave similarly. been used to substantiate the diagnosis. However, age cannot be relied on.-Sigmund H. Ein
Lipoblastoma Presenting as a Mesenteric Mass in an Infant. X Posey, E. Valdiviu, D.L. Persons, et al. J Pediatr Hematol Oncol 20:580-582, (November/December), 1998. Lipoblastoma is a benign fatty tumor of infants and young children. It is extremely rare. It represents 1.6% to 3.1% of all soft tissue tumors in infants. This case report describes a 5-month-old girl who presented with an enlarging abdominal mass that compressed the right kidney, causing hypertension and dilatation of the right-sided collecting system. It stemmed from the transverse colonic mesentery. It was completely resected. With the preoperative CT evaluation showing good encapsulation, the prognosis is excellent.-Michael P. Hirsh Small Bowel Adenocarcinoma 12-Year-Old Girl. K.P. Dunsmore
Metastatic to the Ovaries in a and M.A. Lovell. J Pediatr Hematol
Oncol20:498-501,
(September/October),
1998.
The authors report the case of a 121/2-year-old girl who presented with a 6-week history of crampy abdominal pain, emesis, £ weight loss, and cramps. She had a partial small bowel obstruction and was taken to the OR, where an obstructing jejunal tumor was resected. There was ovarian and peritoneal studding. Pathology showed small bowel adenocarcinoma. This is an extremely rare tumor, particularly in children. It has an association with Crohn’s disease, tropical spme, and familial polyposis. The 5-year survival rate is very poor (0% to 28%). Surgery is the primary treatment modality.-Michael I? Hirsh
High Risk of Leukemia After Short-Term Dose-Intensive Chemotherapy in Young Patients Wiih Solid Tumors. B.H. Kushner; G. Heller; N.I? Cheung, et al. .I Clin Oncol 16:3016-3020, (September), 1998. This review from Sloan Kettering shows a startling increase in the incidence of acute myelogenous leukemia in solid tumor patients treated with repetitive high-dose alkylating agents and etoposide. The incidence was as high as 8%.-Michael I? Hirsh
INTERNATIONAL
A 30-month-old boy presented with increased abdominal girth and secondary enuresis. He had undergone removal of a right multicystic dysplastic kidney at age 5 months. Investigation showed a large cyst communicating via the left ejaculatory duct. The cyst was marsupialised, subtotally excised, and cauterized, with complete resolution of symptoms.-M.Iv? de la Hunt
Prospective Study of Outcome in Antenatally Diagnosed Renal Pelvis Dilatation. MS. Jaswon, L. Dibble, S. Puri, et al. Arch Dis Child 80:Fl35-F138, (March), 1999. The authors report a prospective study from an unselected district general hospital population of 104 infants with antenatally detected renal pelvis (anteroposterior diameter of 5 mm or greater). All the infants were investigated postnatally with renal ultrasonography and micturating cystography. Persisting postnatal dilatation was detected in 47 (45%) infants. Of these, the dilatation was believed to be idiopathic in 8 (7.6%), caused by pelviureteric obstruction in 4 (3.8%), and caused by renal dysplasia in 4 (3.8%). Vesico-ureteric retlux (VUR) was detected in 22% of the entire group, but was not related to the degree of renal pelvis dilatation (either antenatally or postnatally), and indeed 61% of those infants had had normal postnatal pelvic ultrasound findings. The finding of renal pelvis dilatation warrants full postnatal investigation. If this is limited to postnatal ultrasonography, significant VUR will be missed.-Mark Davenport
MUSCULOSKELETAL SYSTEM Treatment of Madelung’s Deformity by Lengthening and Relaxation of the Distal Extremity of the Radius by Ilizarov’s Technique. B. de Billy, E Gastaad, M. Repetto, et al. Eur J Pediatr Surg 7296-298, (October), 1997. Madelung’s deformity was first described in 1878. It is characterized by a typical deformity of the carpus; in addition to causing pain, it impedes mobility and esthetic appearance. Surgical correction can be performed during adolescence, the most frequently employed technique being conical osteotomy. The authors present a novel technique for lengthening and aligning the distal radial extremity using Ilizarov’s procedure. Five carpal joints were operated on in 3 13-year-old girls. An improved esthetic effect was obvious in all 3 cases. Mobility improved by 30” in the direction of extension, and pain always subsided immediately after surgery.-Thomas A. Angerpointner
NEOPLASMS Clinical Impact and Prognostic Value of Metaiodobenzylguanidine Imaging in Children With Metastatic Neuroblastoma. E Perel, J. Conway, M. Kletzel, etal. J Pediatr Hematol Oncol21:13-18, (January/Febrnary), 1999. This review of 30 patients with stage IV neuroblastoma from Chicago Memorial Children’s Cancer Service showed that MIBG imaging complements bone scanning in these children. MIBG scanning shows better correlation with outcome in children older than 1 year. They recommend both scans be performed.-Michael f? Hirsh
Detection of Neuroblastoma in the Bone Versus Aspiration. PA. Aronica, VT Pirrotta, Pediatr Hematol Oncol20:330-334, (July/August),
Marrow: Biopsy E.J. Yunis, et al. J 1998.
This review from Children’s Hospital of Pittsburgh of the mode of diagnosis in 57 neuroblastoma patients shows the complementary value of the bone marrow biopsy and aspiration. Together they have the highest yield for detection of metastatic disease, and both should be performed at the time of diagnosis.-Michael I! Hirsh
An Infant With Neuroblastoma Found Through Mass Screening Hisano, et al. J Pediatr Hematol October), 1998.
ABSTRACTS
and MYCN Amplification (MS). I: Taga, N. Okamoto, T. Oncol 20:486-488, (September/
This case report from Japan describes an 8-month-old boy whose left adrenal neuroblastoma was detected by a mass screening program. Usually such patients have a good prognosis, but this tumor underwent cytogenetic studies in which MYCN amplification was detected. The baby had a very bad course thereafter. This is an exception to the 97% survival rate for patients identified via mass screening.-Michael l? Hirsh Successful Treatment of a Patient With Stage IV Rhabdoid Tumor of the Kidney (RTK): Case Report and Review. PE. Waldron, B.M. Rodgers, M.D. Kelly, et al. J Pcdiatr Hematol Oncol 21:53-57, (January/February), 1999. The authors review the clinical course of a 31-month-old boy who presented with stage IV (pulmonary metastases) RTK, a rare form of renal neoplasm (1.8% of all tumors in the National Willms’ Tumor Study [NWTS]). Its overall 5-year survival rate in NWTS III was 25%. This patient underwent intensive multimodal therapy including surgery and chemotherapy. The authors recommend aggressive staging including abdominal lymph node sampling and CNS metastatic workup. The patient is disease-free 60 months after diagnosis.-Michael P Hirsh Lipoblastoma and Liposarcoma in Children: An Analysis of 9 Cases and a Review of the Literature. C.G. Millel; N.L. Yanchas J.E Magee, et al. Can J Surg 41:455-458, (December), 1998. Lipoblastomas reportedly occur primarily in infants and children under 3 years of age. Liposarcomas are most common in the 3rd to 7th decades of life. They are extremely unusual in children younger than 10 years of age. Of the 2,500 cases of liposarcoma reviewed by the United States Armed Forces Institute of Pathology (AFIP), 17 occurred in children under 16 years of age, but only 2 children were younger than 11 years. In this report, the 2 children with liposarcoma were aged 9 and 14 years. Of the 7 children with lipoblastoma, 5 were under 3 years of age. In children, liposarcomas usually occur on an extremity, as in both cases reported here. A truncal location is less common, and retroperitoneal tumors are uncommon. In a large series of lipoblastomas, 70% occurred on an extremity. Others reported a predilection for the trunk. The authors found only I occurring on an extremity. There are 2 types of lipoblastoma. One is well-encapsulated, superficial, and may mimic a lipoma. The other is deeper, with an infiltrative growth pattern. It is sometimes referred to as lipoblastomatosis. Two of the authors’ cases were poorly encapsulated. One liposarcoma in this series was a myxoid variant, which is the most common histological type among children. The other was a round cell type, which is very unusual in children. Lipoblastoma is definitively treated by complete resection. Reported recurrence rates range from 13% to 20%. In this series, all tumors except 1 were completely resected, and there have been no recurrences. Of the 17 pediatric liposarcomas in the AFIP series, there were 3 recurrences (18%) and 1 death (6%). One of the authors’ patients with liposarcoma was lost to follow-up; the other had complete resection and was disease-free at 6 months. The use of adjuvant therapy for childhood liposarcomas is not advocated for most completely resected tumors. The translocation anomaly of the tumor karyotype in case 8 is characteristic for myxoid liposarcoma. This anomaly has not been reported in lipoblastoma. The tumor karyotype of a number of lipoblastomas has been reported, and 6 of 7 (86%) show a breakpoint in chromosome 8, with variable rearrangements of the nuclear material. The authors examined the tumor karyotype in their most recent case of lipoblastoma and found a similar breakpoint in chromosome 8. This
INTERNATIONAL
1749
ABSTRACTS
anomaly has not been Tumor karyotype tumors. Microscopically. myxoid liposarcoma. Traditionally. age has as the authors found,
reported for liposarcoma. plays a significant role in differentiating these lipoblastoma can be indistinguishable from Clinically, the tumors often behave similarly. been used to substantiate the diagnosis. However, age cannot be relied on.-Sigmund H. Ein
Lipoblastoma Presenting as a Mesenteric Mass in an Infant. X Posey, E. Valdiviu, D.L. Persons, et al. J Pediatr Hematol Oncol 20:580-582, (November/December), 1998. Lipoblastoma is a benign fatty tumor of infants and young children. It is extremely rare. It represents 1.6% to 3.1% of all soft tissue tumors in infants. This case report describes a 5-month-old girl who presented with an enlarging abdominal mass that compressed the right kidney, causing hypertension and dilatation of the right-sided collecting system. It stemmed from the transverse colonic mesentery. It was completely resected. With the preoperative CT evaluation showing good encapsulation, the prognosis is excellent.-Michael P. Hirsh Small Bowel Adenocarcinoma 12-Year-Old Girl. K.P. Dunsmore
Metastatic to the Ovaries in a and M.A. Lovell. J Pediatr Hematol
Oncol20:498-501,
(September/October),
1998.
The authors report the case of a 121/2-year-old girl who presented with a 6-week history of crampy abdominal pain, emesis, £ weight loss, and cramps. She had a partial small bowel obstruction and was taken to the OR, where an obstructing jejunal tumor was resected. There was ovarian and peritoneal studding. Pathology showed small bowel adenocarcinoma. This is an extremely rare tumor, particularly in children. It has an association with Crohn’s disease, tropical spme, and familial polyposis. The 5-year survival rate is very poor (0% to 28%). Surgery is the primary treatment modality.-Michael I? Hirsh
High Risk of Leukemia After Short-Term Dose-Intensive Chemotherapy in Young Patients Wiih Solid Tumors. B.H. Kushner; G. Heller; N.I? Cheung, et al. .I Clin Oncol 16:3016-3020, (September), 1998. This review from Sloan Kettering shows a startling increase in the incidence of acute myelogenous leukemia in solid tumor patients treated with repetitive high-dose alkylating agents and etoposide. The incidence was as high as 8%.-Michael I? Hirsh