Fibrolamellar hepatocellular carcinoma with skeletal metastases.

Skeletal metastases is relatively rare in hepatocellular carcinoma and accounts for 4-16% of extrahepatic metastases. The authors report a 13-year-old girl with fibrolamellar hepatocellular carcinoma, who rejected further systemic chemotherapy following hepatic lobectomy and experienced sternal and vertebral painful metastases nearly 5 years after the operation. The sternal metastatic lesion was removed surgically, whereas external irradiation was delivered to the lumbar vertebral lesion. The patient received no systemic treatment following metastases and died with widespread disease. Despite metastatic disease, the patient survived 6.5 years following the initial diagnosis.

Management of cutaneous hemangiomas: a retrospective analysis of 1109 cases and comparison of conventional dose prednisolone with high-dose methylprednisolone therapy.

The effectiveness of the different pharmacological agents and different doses of systemic corticosteroids was analyzed. A total of 1109 patients (median age 8 months; F/M: 2.3) with hemangioma, followed up in our unit for 23 years, were evaluated retrospectively. Forty-five of them received systemic corticosteroids. Two different pharmacological agents, prednisolone (in 26 patients) and methyl prednisolone (in 19 patients), had been used in three different regimens. Groups were compared according to the final results and rebound regrowth. Response was considered good or excellent in 16 patients (36%). Then were no differences in response to therapy among the three regimens. No difference was found in response to therapy between prednisolone and methylprednisolone and the two different doses of the methylprednisolone. Rebound regrowth was significantly higher in methyl prednisolone than in the prednisolone group (p = .045). In multivariate analysis the dimension of the lesion (p = .0065) and age at initiation of treatment (p = .0041) were the most important factors affecting the response. In conclusion, the systemic corticosteroids are effective in 36% of patients, independent of dosage and pharmacological agents and duration of the therapy. The dimension of the lesion and age at initiation of treatment are the most important factors affecting the response to treatment.

Congenital infantile fibrosarcoma of the thigh in a newborn.

Congenital/infantile fibrosarcoma occurs frequently in the first year of life and differs from fibrosarcoma in adulthood. The clinical course of congenital/infantile fibrosarcoma is more favorable and metastatic spread is rare. While adult fibrosarcomas are common in the thigh, congenital/infantile fibrosarcomas affect chiefly the distal portions of the extremities. Standard treatment is primarily wide surgical excision. Chemotherapy may be given as neoadjuvant or adjuvant treatment in order to avoid the morbidity associated with wide excision. In this case report we present our experience of a newborn affected by congenital/infantile fibrosarcoma of the left thigh.

Pleuropulmonary blastoma in a child presenting with pneumothorax.

Pleuropulmonary blastoma is an uncommon primary malignant tumor of the lung. Surgery is the most important part of the treatment. Despite the use of chemotherapy with or without radiotherapy, the prognosis is poor. Here we report on the case of a 2.5 year-old boy presenting with pneumothorax who turned out to have a right hemithoracic mass. The patient underwent surgical resection of the mass. Pathologic diagnosis was type II pleuropulmonary blastoma. Postoperative thoracic CT showed nodular residual densities so the patient was given adjuvant combination chemotherapy with cisplatin and etoposide. He has been disease free for 12 months.

Paraneoplastic cerebellar degeneration and Horner syndrome: association of two uncommon findings in a child with Hodgkin disease.

An 11-year-old boy admitted with a right cervical mass was found to have Hodgkin disease. On admission, he also had right Homer syndrome and severe cerebellar ataxia. Cranial MRI revealed marked cerebellar atrophy. He was treated with chemotherapy consisting of doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD), in addition to radiotherapy. Three months after initiation of therapy, he had a partial remission of tumor. Neurologic symptoms improved dramatically after chemotherapy started. Hodgkin disease should be included in the differential diagnosis of children with cerebellar findings and Horner syndrome.

Primary intracranial germ cell tumors in children: a report of eight cases and review of the literature.

This study was conducted to evaluate the signs and symptoms on admission, diagnosis, localization, therapy, and survival of patients with primary intracranial germ cell tumors (PICGCT). Eight patients with surgically confirmed PICGCTs were treated and followed up at Hacettepe University's Department of Pediatric Oncology between 1974 and 1995. While one patient was admitted with a second recurrence of her disease, the others were admitted or referred primarily to our institution. In this period, 357 germ cell tumor and 684 primary intracranial malignant tumors were diagnosed and treated at our institution. Thus, PICGCTs comprised 1.1 percent of the primary intracranial malignant tumors and 2.2 percent of the germ cell tumors. There were four females and four males and the median age was eight years (13 months to 12 years). On admission, the most common symptoms were diabetes insipidus (3/8) and vomiting (3/8). One patient also and Down's syndrome. Locations of the tumors were suprasellar in three, in the third ventricle in two, and in the cerebral parenchyma, and pineal and hypothalamic regions in the remainder. There were germinomas, three malignant teratomas, and two mixed germ cell tumors. Only two patients could be treated with appropriate and adequate chemotherapy and radiotherapy. Three patients died: one in the postsurgical period, one after the third surgical approach and one 11 months after the diagnosis of progressive disease; three were lost to follow-up. The remaining two patients (with second recurrence and disseminated disease) are alive and without disease. Our experience with these patients demonstrated that appropriate and adequate chemotherapy is as effective a treatment as radiotherapy, even with recurrence of the disease.

Childhood cancer and hypercalcemia: report of a case treated with pamidronate.

Hypercalcemia in a 4-year-old boy with non-Hodgkin lymphoma treated with pamidronate is presented. The child had relapsed disease with bone metastasis. Hypercalcemia is rare in children, and bisphosphonates are relatively new agents for the treatment of hypercalcemia. Information concerning their use in the treatment of hypercalcemia in childhood is limited. We found that pamidronate is effective and has no significant side effects in a child.

Benign vascular tumors and vascular malformations in childhood: a retrospective analysis of 1127 cases.

Vascular lesions in childhood are classified as vascular malformations and hemangiomas. Vascular malformations are congenital abnormalities thought to arise from defects during embryological development of vascular tissue. Hemangiomas are benign tumors of vascular endothelium and can spontaneously become involuted in almost all cases. One thousand one hundred and twenty-seven patients with vascular lesions were followed by the Department of Pediatric Oncology, Hacettepe University, for 19 years. Diagnosis was based mainly on history, clinical condition and follow-up data in 98.2 percent of cases. The distribution of the vascular lesions was as follows: 969 patients had hemangiomas, 120 had lymphatic malformations, 18 had combined vascular malformations, 11 had venous malformations, six had port-wine stain, and three had angiokeratoma. Except for ten cases with Klippel-Trenaunay Weber syndrome, vascular malformations were not accompanied by any syndrome.

Renal lymphoma. An unusual presentation in a child.

An unusual case of non-Hodgkin's lymphoma with primary renal involvement in a four-year-old boy is presented. The diagnosis was established by renal biopsy. The findings of an exploratory laparotomy and an extensive staging procedure showed no other primary site. The patient has been followed up for 14 months and no evidence of recurrence has been observed.

Cutaneous anthrax.

The case of a 9-year-old boy diagnosed and treated for cutaneous anthrax is presented. We discuss the clinical features and course of this disease, encountered today only rarely in less developed regions where humans are in contact with infected animals.

Primary hypertrophic osteoarthropathy.

In this article a family with five members suffering from primary hypertrophic osteoarthropathy (PHO) is reported. Our cases are characterized by swollen and painful joints and clubbing of distal phalanges of hands and feet. The diagnosis made upon on radiological and clinical findings.

Short-time and low-dose intravenous acyclovir therapy in varicella zoster infections with malignant disease in children receiving combined chemotherapy.

The effects of low-dose and short-time acyclovir therapy in 14 children with malignant disease of ages 4-18 years who had developed varicella zoster virus infections while receiving aggressive chemo-/+radiotherapy are reported. Ten of them had chickenpox and 4 herpes zoster. Acyclovir 5 mg/kg was infused IV every 12 h in 9 patients and every 8 h in 5 patients for a median of 4 days' duration. We resumed the primary therapy when the patients' lesions had dried out and became crusted and new lesions had not reappeared. The period of initiation of the acyclovir therapy to the resumption of oncological treatment was 8.4 +/- 2.7 days for chickenpox and 12.0 +/- 3.4 days for herpes zoster patients. After restarting the oncological therapy, no adverse effects of acyclovir or complication of infection were observed. The efficiency of early, short-term, and relatively low dose acyclovir therapy is discussed and compared to the results in the relevant literature.