Desmoplastic small round cell tumor of the mandible in a child with unusual plantar metastasis.

SUMMARY: Desmoplastic small round-cell tumor (DSRCT) is a rare and aggressive tumor that usually affects young males. Abdominal or pelvic disease is generally present in patients with DSRCT. Despite multimodality treatment, it still remains highly aggressive and has poor prognosis. We report a 16-year-old male with DSRCT in the mandible, an unusual site of the disease, treated with chemo-radiotherapy but recurred in an unexpected site 5 months after the completion of treatment.

Infantile myofibromatosis in a three-year-old boy presented as subdural hematoma.

Infantile myofibromatosis, mostly developing at birth or in early infancy, is a rare clinical disorder characterized by myofibroblastic lesions. We report clinical, radiological and pathological features of a three-year-old boy who was diagnosed with infantile myofibromatosis originating from the occipital region contrary to radiological findings in advanced diagnostic studies.

Successful treatment of retroperitoneal giant cell-type malignant fibrous histiocytoma in a 5-year-old boy.

Malignant fibrous histiocytoma, usually seen in patients older than 10 years, is an aggressive soft-tissue sarcoma occurring mostly in the extremities and the trunk, but it is extremely rare in children. We report the clinical, radiological and pathologic features of a five-year-old boy who was diagnosed as a retroperitoneally originated malignant fibrous histiocytoma. The patient with unresectable mass was successfully treated with multidisciplinary approach, with chemotherapy, surgery and radiotherapy, by using combined chemotherapy consisting of vincristine, cisplatinum, adriamycin, cyclophosphamide, actinomycin D and dacarbazine.

Second neoplasms in pediatric patients treated for cancer: a center's 30-year experience.

To investigate the incidence and outcome of secondary neoplasms in pediatric patients treated for childhood cancer. Between December 1971 and January 2000, a total of 5859 patients younger than age 17 were diagnosed and treated for childhood cancers in our center. Of this group, 1511 (36%) patients were followed for more than 36 months. These long-term survivors were included in this analysis. Twenty-six patients developed a secondary malignancy with an overall risk of 1.7% in this cohort. The male:female ratio was 17:10, with a median age of 7.66 at diagnosis (range, 2 to 16 y). Four patients (14.8%) with Hodgkin lymphoma; 3 each (11.1%) with retinoblastoma and rhabdomyosarcoma; 2 each (7.4%) with Wilms tumor, Ewing sarcoma, medulloblastoma, ganglioneuroblastoma, and non-Hodgkin lymphoma; and 1 each (3.7%) with ependymoma, nasopharyngeal carcinoma, osteosarcoma, astrocytoma had a secondary malignant disease during the long-term follow-up period. Secondary malignant diseases were osteosarcoma in 6 patients, acute lymphoblastic leukemia in 2, acute myelogenous leukemia in 2, and rare malignant disease in others. Four patients with osteosarcoma developed disease within the radiation field. Osteosarcoma was the most frequently occurring secondary neoplasm. Less toxic treatment modalities should be used to decrease the risk of secondary malignant diseases.

Effective treatment of multifocal aggressive fibromatosis with low-dose chemotherapy.

Desmoid tumor (aggressive fibromatosis), as a member of a group of borderline neoplasms, is a rare tumor of fibroblastic origin that remains difficult to treat. Treatments with surgery, radiotherapy and different medical protocols including interferon (IFN)-alpha, hormonal agents such as tamoxifen (anti-estrogen) as well as non-steroidal anti-inflammatory drugs and low-dose antineoplastic agents have been reported. In this report we describe a new patient with multifocal aggressive fibromatosis who was successfully treated with low-dose chemotherapy consisting of methotrexate and vinblastine.

Anaplastic large cell lymphoma in a child presenting with cutaneous nodules and blisters.

A 13-year-old girl was admitted to our hospital with a one-month history of bullous skin lesions. Physical examination revealed ulcerated and nonulcerated cutaneous plaques, bullae, enlarged cervical and supraclavicular lymph nodes and hepatomegaly. In another hospital, histopathological diagnosis of a skin biopsy was reported to be consistent with tuberculosis and she was treated with antimycobacterial drugs. Since no response was obtained, she was referred to our center after a new lymph node biopsy was obtained. At our center, histopathological diagnosis was anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma (ALCL). We started LMT chemotherapy regimen and initial response was complete. Eight months after initial admisision, she experienced cutaneous recurrence of disease while on maintenance protocol. Chemotherapy was changed to LSA4 regimen. She is still on chemotherapy and has been in complete remission for nine months. Clinicians should be aware of this uncommon presentation of ALCIL, which can be confused with other diseases clinically or histologically.

Leptin and neuropeptide Y plasma levels in children with cancer.

This study investigated leptin and neuropeptide Y levels in children with cancer, the relationship of those levels to cachexia, and their usefulness as prognostic indicators. Twenty-three newly diagnosed children with cancer were included in the study. The median age at diagnosis was 8 years (range 1.5-14), and the male to female ratio was 13:10. Body mass index, serum leptin and neuropeptide Y levels were measured at diagnosis and at each cycle of chemotherapy. The mean neuropeptide Y level was 211.1 pmol/l at diagnosis and decreased to 92.8 pmol/l at the fifth cycle of chemotherapy. In contrast, the mean leptin level was 3.9 ng/ml at diagnosis and increased to 13.0 ng/ml at the fifth cycle of chemotherapy. Thus, levels of these factors are influenced by treatment status and disease progression. The mean neuropeptide Y level at diagnosis was 82.32 pmol/l in children with complete remission and 430.16 pmol/l in those who died with disease during the follow-up period. The mean leptin level at diagnosis was 6.60 ng/ml in children with complete remission and 0.192 ng/ml in patients who died with disease during the follow-up period. The neuropeptide Y and leptin levels seem to be related to prognosis and could be used as prognostic indicators in the follow-up of children with cancer.

Synchronous multicentric giant cell tumor in a 16-year-old boy.

Synchronous multicentric giant cell tumor of the bone is a rare variant of a lesion appearing during childhood. The authors report clinical, radiological, and pathological features of a 16-year-old boy who was diagnosed with synchronous multicentric giant cell tumor, which originated in the right distal femur and the left fibula.

Use of recombinant factor VIIa for bleeding in children with Glanzmann thrombasthenia.

Glanzmann thrombasthenia is a very rare inherited platelet function disorder in which bleeding may be extremely difficult to stop. Recombinant factor VIIa is one of the alternative treatments for bleeding. The authors report here their experience with the use of factor VIIa, which may be useful for arresting bleeding in Glanzmann thrombasthenia.

Acute lymphoblastic leukemia as a second malignant neoplasm in a child with medulloblastoma.

Second malignant neoplasm in childhood is increasing due to advances in therapy modalities. Acute lymphoblastic leukemia as a second malignancy following the treatment of medulloblastoma is a very rare condition. A 13-year-old boy was diagnosed as acute lymphoblastic leukemia following radiotherapy and chemotherapy for treatment of medulloblastoma.

The comparison of antibody response to different hepatitis b vaccines with and without pre-S2 antigen in children with cancer.

Children with cancer are at an increased risk of hepatitis B infection and chronic liver disease. Since hepatitis B vaccines containing pre-S2 antigen has been recently reported as being more efficient in providing immunization in healthy individuals, the authors compared antibody response to pre-S2-containing vaccine with no-pre-S2-containing hepatitis B vaccine, when given in double doses to 100 children receiving chemotherapy. Patients, aged 1 to 16 years with negative HBV serology, were vaccinated with 2 different types of HBV vaccines between 1997 and 1999. Group 1 received Gen Hevac B containing pre-S2 (n = 41) in a dose of 20 microg for patients younger than 10 years old and 40 microg for older patients. Group 2 was vaccinated at the same dose with hepatitis B vaccines not containing pre-S2 antigen. All vaccinations were repeated at 0, 1, and 6 months. Serum samples were drawn for determination of anti-HBs titers at 1, 3, 6, and 8 months. After the third dose of vaccine, the seroconversion rate was 72% in group 1 and 62% in group 2. The anti-HBs levels were higher in the group receiving pre-S2-containing hepatitis B vaccine. However, the difference between groups was not statistically significant (p > .05). The administration of pre-S2-containing hepatitis B vaccines may give a better seroconversion and higher antibody response to vaccination in children with cancer. But a further large-scale study is needed to confirm this finding.