Low-grade fibromyxoid sarcoma,a deceptively benign tumor in a 5-year-old child

Low-grade fibromyxoid sarcoma (LGFMS) is very uncommon in the pediatric population with only 20% of reported cases under the age of 18. The youngest reported case to date has been in a 4-year-old child. Lesions are usually slow growing and asymptomatic, and locations described in children have included paravertebral, thigh and intrathoracic. Although benign in appearance, these lesions can behave aggressively, with local recurrence and distant metastases primarily to lungs. These lesions can be resistant to the usual chemotherapy and radiotherapy with surgical resection being the treatment of choice. We report a case of a 5-year-old boy who presented with a mass in the left buttock.     

METASTATIC OSTEOSARCOMA TO THE LIVER AFTER TREATMENT FOR SYNOVIAL SARCOMA: A Case Report

Metastatic osteosarcoma most commonly affects the lungs and other bones. Hepatic metastasis at the time of diagnosis is extremely rare. A 14-year-old boy with synovial sarcoma of the left popliteal fossa was treated with surgical resection, radiotherapy for microscopic residual disease, and 1 year of chemotherapy (vincristine, cyclophosphamide, dactinomycin, and doxorubicin). Approximately 10 years after the initial diagnosis, a secondary osteosarcoma developed in the left proximal tibia. Computed tomography at presentation showed bilateral pulmonary metastases and large ossified nodules in the liver that demonstrated abnormal avidity on ^99m TcMDP bone scan indicating hepatic metastasis. Despite chemotherapy (cisplatin, ifosfamide, high-dose methotrexate, and dacarbazine), the patient died of progressive disease 4 months after the diagnosis of the second cancer. Hepatic metastasis was found at the time of diagnosis of a secondary osteosarcoma and manifested as ossified nodules. The risk of radiation-induced osteosarcoma should always be considered in decisions about treatment for soft-tissue sarcoma.     

Tumour-induced rickets: A case report and review of the literature

Hypophosphataemic rickets was diagnosed in a 6-year-old boy with a negative family history. After 16 years of medical treatment he developed a malignant sarcoma of the right distal thigh. Removal of the tumour by high amputation of the leg resulted in disappearance of the phosphate leak. In spite of surgery and chemotherapy, the patient died due to extensive lung metastases. Retrospective analysis of the initial X-ray films showed a benign lesion on the lateral side of the right distal femur. This lesion is believed to be at the origin of the rickets. This is the first paediatric case reported with malignant degeneration of a benign tumour causing rickets.Conclusion Patients with the classical hallmarks of X-linked, familial hypophosphataemic rickets but no affected family members should have a careful periodic search for a tumour, even years after onset of the disorder.     

Late recurrent metastasis in Wilms' tumour

Recurrence of Wilms' tumour after 5 years of disease-free survival is rare. We present the case of a 26-year-old man who had been diagnosed of Wilms' tumour at the age of 6 years treated by surgery, chemotherapy, and radiotherapy and who remained disease free for 8 years, after which lung metastases were detected. Second complete remission was attained with surgery and chemotherapy and 20 years after initial diagnosis he again presented with lung metastases, similarly achieving complete remission with surgery and secondline chemotherapy. The clinical and biological aspects of late metastasis in this neoplasm are discussed. © 1994 Wiley-Liss, Inc.     

Rhabdomyosarcoma arising within congenital cystic adenomatoid malformation

Rhabdomyosarcoma arising within a congenital cystic adenomatoid malformation (CCAM) is an unusual entity. The patient underwent a lobectomy of his right lower lobe of lung due to a CCAM at the age of two. One year later, he developed a solid embryonal rhabdomyosarcoma at the same location. He received 1-year period chemotherapy and when the tumor reduced to a resectable size, surgical excision was done. The tumor cells appeared more differentiated after chemotherapy. The patient remains disease free to date, 16 months after surgery.     

Low toxicity and efficacy of (153)samarium-EDTMP and melphalan as a conditioning regimen for secondary acute myelogenous leukemia

We report the case of a 15-yr-old girl who developed secondary acute myelogenous leukemia (AML) 4 yr after completion of therapy for metastatic Ewing sarcoma (primary right acetabulum with metastatic disease to the lungs). Peripheral blood stem cells were collected after the second cycle of chemotherapy with the plan for future consolidation with high-dose chemotherapy and autologous stem cell rescue; however, because of the patient's excellent response to chemotherapy and surgery, therapy was completed without the need for high-dose chemotherapy. No human leukocyte antigen (HLA)-matched related donor was available for a bone marrow transplant. Because of previous lung radiation, high-dose samarium [30 mCi/kg of samarium-153 ethylenediaminetetramethylenephosphonate ((153)Sm-EDTMP) day -14] and melphalan (140 mg/m(2) day -2) were chosen as the conditioning regimen to avoid potential lung complications. The patient received an infusion of 6.1 x 10(8)/kg mononuclear autologous cells on day 0. She achieved engraftment on day +23. Three years after transplantation, she continues to have complete remission. Samarium and melphalan constitute a well-tolerated regimen with potential antileukemic activity.     

Metastatic osteosarcoma to the liver after treatment for synovial sarcoma: a case report

Metastatic osteosarcoma most commonly affects the lungs and other bones. Hepatic metastasis at the time of diagnosis is extremely rare. A 14-year-old boy with synovial sarcoma of the left popliteal fossa was treated with surgical resection, radiotherapy for microscopic residual disease, and 1 year of chemotherapy (vincristine, cyclophosphamide, dactinomycin, and doxorubicin). Approximately 10 years after the initial diagnosis, a secondary osteosarcoma developed in the left proximal tibia. Computed tomography at presentation showed bilateral pulmonary metastases and large ossified nodules in the liver that demonstrated abnormal avidity on ^99m TcMDP bone scan indicating hepatic metastasis. Despite chemotherapy (cisplatin, ifosfamide, high-dose methotrexate, and dacarbazine), the patient died of progressive disease 4 months after the diagnosis of the second cancer. Hepatic metastasis was found at the time of diagnosis of a secondary osteosarcoma and manifested as ossified nodules. The risk of radiation-induced osteosarcoma should always be considered in decisions about treatment for soft-tissue sarcoma.     

Interleukin‐8‐producing primary cardiac undifferentiated sarcoma in a child with sustained fever

We report the case of a 12‐year‐old boy with primary undifferentiated sarcoma of the left atrium. He had sustained fever during the clinical course and multiple lung and brain metastases. Chemotherapy and irradiation were ineffective; he died 41 days after hospitalization. On retrospective analysis, interleukin‐8 (IL‐8) was elevated; this was supported by immunohistochemistry and gene expression analysis of tumor samples. IL‐8 continued to increase with tumor progression accompanied by elevated neutrophil count and C‐reactive protein. IL‐8 is involved in malignant tumor proliferation, migration, and angiogenesis and may have been related to the clinical condition and prognosis in the present case.     

Concordant rhabdoid tumor of the kidney in a set of identical twins with discordant outcomes

We report identical twin boys who each had stage IV rhabdoid tumor of the left kidney at the age of 5 months and 2 years, respectively. The 5-month-old boy, despite receiving chemotherapy, died of progressive disease at the age of 12 months. Following resection of the tumor, his twin brother was treated with 6 cycles of combination chemotherapy consisting of cisplatinum, doxorubicin, vincristine, cyclophosphamide, and actinomycin-D alternating with ifosfamide and etoposide. After complete regression of lung and brain metastases, he received high-dose thiotepa, etoposide, and cyclophosphamide, followed by autologous peripheral stem cell rescue. The patient is presently alive and free of disease 6 years posttransplant. High-dose chemotherapy followed by autologous stem cell transplant may be an effective front-line therapeutic approach for patients with metastatic rhabdoid tumor of the kidney.     

Ewing sarcoma: phalangeal primary with fatal cardiac metastases

A 39-year-old man had pain and swelling of the terminal phalanx of a finger. Radiograph was interpreted as osteomyelitis, and amputation through the mid-phalanx was performed. Histology revealed Ewing sarcoma. Lung metastases rapidly developed. Right lung irradiation and systemic chemotherapy, including doxorubicin, were instituted. He developed progressive severe right ventricular failure which was attributed to effects of large pulmonary metastases. Autopsy showed massive right ventricular metastases, the primary pathological cause of the heart failure, without evidence of doxorubicin cardiomyopathy.     

A Pediatric Case of Metastatic Conventional Parosteal Osteosarcoma Treated With Multidrug Chemotherapy

Parosteal osteosarcoma (POS) is conventionally a low‐grade sarcoma with limited metastatic potential; however, the tumor occasionally transforms into a high‐grade dedifferentiated POS, which commonly metastasizes to distant organs. The present report describes a rare pediatric case of conventional POS with no dedifferentiated component yet had multiple pulmonary metastases at initial diagnosis. Following limb‐sparing surgery and osteosarcoma‐oriented neoadjuvant chemotherapy, the patient received total resection of pulmonary metastases. Despite no treatment for pulmonary recurrence 1 year after adjuvant chemotherapy, the patient is alive with stable disease 4 years and 6 months after the initial diagnosis.     

Primary Malignant Fibrous Histiocytoma of the Lung in a Child: A Case Report and Review of Literature

Malignant fibrous histiocytoma (MFH), an aggressive high-grade soft tissue sarcoma, usually occurs in the elderly during the fifth to seventh decade of life. It commonly arises in the retroperitoneum, extremities, and head and neck region. Primary pulmonary MFH is extremely rare and is frequently fatal. We present the youngest known case, a 9-year-old boy with a primary left lung grade II inflammatory MFH, stage II. He underwent a left upper lobectomy for tumor resection. After completing radiation therapy, he was started on vincristine, actino-mycin D, and cyclophosphamide alternating with vincristine, doxorubicin, and cyclophosphamide every 3 weeks. After five such cycles, he had a histologically proven local recurrence. He then received chemotherapy consisting of ifosfamide (2 g/m²) and etoposide (VP-16) (100 mg/m²) given daily for 3 days every 3 weeks. The patient attained complete remission (CR) after five such cycles and completed treatment without any major complications. He received a total of 16 courses and is continuing in CR 36 months off treatment. Ifosfamide and etoposide (VP-16), known for their usefulness in treatment of adult soft tissue sarcomas, can be used as salvage chemotherapy for patients with MFH who fail the front-line conventional chemotherapy.     

Squamous cell carcinoma of the lung in an autistic child who has never smoked

Primary bronchogenic carcinoma of the lung is extremely rare in childhood, particularly the squamous cell type. Only 13 cases have been reported in the literature. We report a case of squamous cell carcinoma in an autistic, 16-year-old boy who presented with a productive cough. Interestingly, he was a never-smoker, but had been exposed to environmental tobacco smoking by his father for 13 years. The diagnosis was delayed by approximately 1 month due to his young age. He was diagnosed with squamous cell carcinoma of the lung by video-assisted thoracoscopic surgery, and chemotherapy was arranged. Considering his age, autism, and good performance status, a combined chemotherapeutic regimen with gemcitabine plus carboplatin was planned. After the second cycle of chemotherapy, the cough resolved and a computed tomography scan showed a partial response of the central conglomerated mass with the absence of the malignant pleural effusion.     

Rituximab as potential therapy for paraneoplastic cerebellar degeneration in pediatric Hodgkin disease

Paraneoplastic cerebellar degeneration (PCD) is a rare neurological syndrome associated with lung cancer, breast adenocarcinoma,ovarian adenocarcinoma, and Hodgkin disease. It is rarely seen in pediatrics. We report a case of a 10-year-old boy with a 2-year prodrome that led to a diagnosis of PCD in association with stage IV Hodgkin disease. He received radiation and chemotherapy for his Hodgkin disease with resolution of his lymphoma. Based on promising data in adults on the efficacy of rituximab over other immuno suppressive agents in paraneoplastic disorders, he was treated with rituximab with marked improvement of the cerebellar syndrome.     

Leiomyosarcoma of the kidney: report of a patient with favorable response to doxorubicin and cisplatin suspended in a lipid contrast medium and cyclophosphamide

A man who had unresectable leiomyosarcoma of the left kidney with skin and lung metastases was treated with intra-arterial infusion chemotherapy consisting of cisplatin and doxorubicin suspended in lipid contrast medium, lipiodol, and oral administration of cyclophosphamide. The tumor responded well to this treatment three times. He is alive and well in remission, the renal tumor decreased in size, and lung metastases became unclear more than 50 weeks after treatment was completed.     

Placental site trophoblastic tumor (PSTT) in mother and child: first report of PSTT in infancy

BACKGROUND: To the authors' knowledge, placental site trophoblastic tumors occurring simultaneously in mother and infant have not previously been reported. PROCEDURE: The clinicopathologic features of metastatic placental site trophoblastic tumor in a mother and her 4-month-old son are described. RESULTS: The disease in the infant was aggressive, and he died in multiorgan failure within 5 weeks of hospital admission. Autopsy showed widespread metastases to liver, lungs, pleura, kidney, mesentery and lymph nodes. The mother, who had a uterine tumor and lung metastases, was treated with chemotherapy and hysterectomy and has no evidence of disease 26 months post-treatment. CONCLUSIONS: This report shows that placental site trophoblastic tumors can metastasize in both mother and child.     

A case of Langerhans cell histiocytosis presented with pneumothorax

Pneumothorax (PTX) is an unusual complication of Langerhans cell histiocytosis (LCH) in childhood. Spontaneous PTX is rare in childhood, and it is very rare in infancy. There are no specific recommendations for the treatment of PTX from LCH described in the literature. We are presenting a 19-month-old boy, who suddenly developed left-sided PTX with infiltrations in both lungs. He presented with PTX and skin lesions. He had a prolonged cardiac arrest, and although resuscitation was successful he required continuing ventilatory support (intermittent positive-pressure ventilation). Because he suddenly developed right-sided PTX and died on the second day of the admission, his LCH diagnosis was made only postmortem. So, he did not receive chemotherapy. It is likely that intermittent positive-pressure ventilation during the operation induced the development of much more multiple lung bullae, which subsequently ruptured, and/or it facilitated the development of the right-sided PTX. The patients with PTX and skin lesions, including babies, most likely have LCH and specific chemotherapy should be started in emergency, even before the final diagnosis is achieved.     

Extraneural metastasis of a nongerminomatous germ cell tumor of the central nervous system in a pediatric patient with a ventriculoperitoneal shunt: a case report and review of the literature

We describe the case of a 7-year-old white boy who presented with a mixed malignant germ cell tumor with predominant embryonal carcinoma component. The patient underwent right ventriculoperitoneal (VP) shunt placement for hydrocephalus at the time of diagnosis. He received multiagent chemotherapy followed by second-look surgery. Despite an initial response to chemotherapy, the patient had metastatic progression of disease within the craniospinal axis. He received craniospinal radiation and high-dose chemotherapy. Although, he had resolution of central nervous system (CNS) disease, follow-up off treatment revealed extra-abdominal metastases. This is a rare case to discuss abdominal metastasis of a CNS germ cell tumor in a patient with a VP shunt. The influence of VP shunt placement on treatment and management decisions of patients with CNS tumors will be discussed.     

Ewing sarcoma/PNET: 27 years of experience in Slovenia

The study includes 91 patients treated for Ewing sarcoma between 1972 and 2002. Forty were younger and 51 were older than 16. The overall 5-year survival was 36%: 53.5% in the younger and 22.5% in the older age group. Among patients with localized disease, 5-year survival was 58% in younger and 25.5% in the older. Survival was similar whether patients received three treatment modalities or chemotherapy combined with either surgery or radiotherapy. Recurrence was noted in 47 out of 75 patients with localized disease, most commonly as local recurrence or lung metastases. Among 18 evaluated patients, 14 had minor physical defects and 4 had severe late treatment effects (sterility in 3 and secondary osteogenic sarcoma in 1).     

REFRACTORINESS TO RITUXIMAB MONOTHERAPY IN A CHILD WITH RELAPSED/REFRACTORY BURKITT NON-HODGKIN LYMPHOMA

The authors describe a 6-year-old boy diagnosed with mediastinal Burkitt lymphoma with tumor invasion into bone marrow and both kidneys. After receiving chemotherapy according to NHL BFM-95 protocol for the high-risk disseminated lymphoma, the patient reached complete remission. He relapsed in the mediastinum at 5 months from the diagnosis. He underwent thoracotomy and tumor mass was removed by inferior lobectomy of right lung. Residual tumor progressed rapidly. Autologous stem cell transplantation could not be performed because of unresponsiveness to cytoreductive chemotherapy. Twenty-three days after the last chemotherapy course, he received rituximab at a dose of 375 mg/m ² by intravenous infusion weekly, for a total of 8 dose. However, multiple intra-abdominal metastatic lesions were detected at the end of the therapy. Palliative radiotherapy was applied to these sites. He died because of disease progression, 11 months after the diagnosis.