Liquid extradural hematoma with congenital fibrinogen deficiency. Case report.
A case of extradural hematoma in a child with congenital fibrinogen deficiency is reported. Although the hematoma was 3 days old, it was purely liquid.
Afibrinogenemia↗
Biomedical subjects
T Kutluk
Publications and source records attributed to T Kutluk.
Urticaria pigmentosa associated with Wilms tumor.
Urticaria pigmentosa is the most common manifestation of mastocytosis, with the majority of cases undergoing spontaneous resolution, especially in children. Several reports have documented hematologic malignancies developing in patients with urticaria pigmentosa. We present a 4.5-year-old boy with urticaria pigmentosa who developed Wilms tumor. To our knowledge, coexisting urticaria pigmentosa and Wilms tumor have not previously been described.
Biopsy, Needle↗
DNA content and proliferative activity in children with neuroblastoma.
Many studies have reported that neuroblastoma patients with aneuploid DNA content and a low cellular proliferative activity have better outcome; other studies have reported contradictory results. Formalin-fixed, paraffin-embedded archival pretreatment specimens of 56 neuroblastomas were studied. Thick sections from paraffin blocks were deparaffinized, and rehydrated. Following enzymatic digestion and filtration, cellular suspensions were analyzed by flow cytometry. Six tumors were aneuploid (13.3%) and 39 samples were diploid (86.7%). S-phase fraction (SPF) ranged from 1 to 78% with a median of 31%. DNA ploidy and proliferative activity results showed no correlation with the prognostic variables. There was no significant difference between the 5-year overall and event-free survival rates of the aneuploid and the diploid neuroblastomas or between the neuroblastomas with a high and low proliferative activity. The results revealed the prognostic significance of neither DNA ploidy nor the cellular proliferative activity in neuroblastoma in contrast to other studies.
Aneuploidy↗
Nasal-paranasal-oronasopharyngeal lymphomas in childhood: the role of staging system on prognosis.
Most of the patients with nasal-paranasal and oropharyngeal-nasopharyngeal (NPONP) lymphomas had early-stage disease according to the Murphy system. But the survival rates are not correlated with the stages. Treatment results were analyzed to see the effects of the staging in NPONP lymphomas. Fifty-five children (median age 8 years, M/F: 4.5) with NPONP lymphoma were included in this study. The Murphy staging system was used at diagnosis and all cases were restaged according to the TNM system: primary tumor, regional lymph node, and metastasis. The survival rates were analyzed by grouping the patients according to the treatment and stages. The disease was located in Waldeyer's ring, the sinonasal region, and the nasopharynx in 45.4, 27.3, and 27.3% of patients, respectively. Thirty-nine patients had stage I or II disease according to the Murphy system. When the TNM system was used, 92% of these patients were upstaged to stage III-IV. Five-year overall and event-free survival rates were 52.1 and 50.4%, respectively, for the whole group. Five-year event-free survival rates for Murphy stage I, II, and III disease were 66.7, 56.9, and 45.4%, respectively. The rates for TNM stage III and IV patients were 64.3 and 43.8%. Treatment protocols were intensified in most of the early-stage disease treated with modified LSA2-L2 regimen and better survival rates were obtained in these patients. The intensification of the treatment by using intrathecal treatment and doxorubicin in patients with early-stage disease at NPONP location seems effective. In conclusion, the Murphy staging system is not suitable for the staging of NPONP lymphomas. It should be revised to predict the prognosis and decision-making for treatment.
Adolescent↗
Peripheral neuropathy during alpha-interferon therapy in a child with Hodgkin's disease.
Peripheral neuropathy is one of the rarely reported neurological complications of interferon therapy. The authors report such a case in a 15-year-old boy during alpha-interferon therapy for Hodgkin's disease. He received alpha-interferon at a dose of 1.8 million units/day 5 times a week by subcutaneous injections as adjuvant immunotherapy post autologous stem cell transplant. Twenty months after the initiation of therapy, he complained of severe pain in his lower distal extremities. Neurological examination revealed the absence of deep tendon reflexes. A nerve conduction study showed a sensorial, polyneuropathic involvement in the lower extremities. Within 4 weeks after the stopping of interferon, his pain improved, and recovery was also seen by nerve conduction studies. Symptoms reappeared at the resumption of interferon treatment. This study suggests that peripheral neuropathy may rarely occur in patients given long-term interferon treatment at high cumulative dosage.
Adolescent↗
Serum CA 125 levels in children with non-Hodgkin's lymphoma.
To evaluate the role of serum CA 125 levels for the diagnosis, follow-up, and prognosis in childhood non-Hodgkin's lymphomas (NHL), 44 children (35 newly diagnosed patients and 9 patients with relapsed or progressive disease, median age 6.5 years, M/F ratio 2.1) with NHL were included in this study. CA 125 levels in serum and/or ascites and effusions were measured by radioimmunoassay. The upper limit of normal was 35 IU/mL. The correlation of CA 125 levels with tumoral location and treatment results were investigated. A total of 27 (61.4%) patients had increased and 17 patients had normal serum CA 125 levels. Fifteen patients with increased CA 125 levels had malignant ascites, pleural effusion, or both, although none with normal CA 125 levels had any serous membrane involvement. The mean CA 125 levels were 72.5 IU/mL in patients with no serosal involvement (ascites or effusion) and, 144.0, 273.7, 324.3 IU/mL in patients with pleural effusion, ascites, and ascites + effusion, respectively. The mean CA 125 levels in ascites and pleural effusion were 202.1 and 156.2 IU/mL, respectively, and were similar to the corresponding serum levels. The increased CA 125 levels returned to normal in 18 patients whose diseases were in remission during the follow-up. One-year survival rate for newly diagnosed patients was 80.2 and 87.5% in patients with increased and normal serum CA 125 levels, respectively. In conclusion, CA 125 levels were significantly higher in patients with serous membrane involvement and there was a correlation between treatment response and marker levels. Little is known about the expression of CA 125 in NHLs. This is the first report suggesting that serum CA 125 levels can be a useful marker for the follow-up of childhood NHL. CA 125 seems to be a promising tumor marker in the assessment of prognosis and therapeutic response in non-Hodgkin's lymphomas.
Adolescent↗
Hodgkin's disease in Turkish children: clinical characteristics and treatment results of 210 patients.
Although Hodgkin's disease (HD) is one of the common malignancies in childhood, there is limited information from developing countries in English literature. The aim of this study is to give epidemiologic features and treatment results of 210 previously untreated children with HD from a developing country. Between 1 June 1984 and 31 December 1992, all children seen who were younger than 18 years old with newly diagnosed, untreated, biopsy-proven Hodgkin's disease were included in this study. A clinical staging system was used to determine the dissemination of the disease. While patients with stage I-II disease received canapé treatment protocol (three cycles COPP [cyclophosphamide, vincristine, procarbazine, prednisolone] or ABVD [doxorubicin, bleomycine, vinblastine, dacarbazine] plus low-dose involved-field radiotherapy), patients with stage III-IV disease were treated by sandwich protocol (six cycles COPP plus low-dose involved-field radiotherapy). A total of 210 patients with a median age of 8 years were eligible for this study. Male to female ratio was 3:1 and 37 (17.6%) were less than 5 years of age. The major histologic subtype was mixed cellularity (69.6%). Overall survival rates were 91.5 and 87.7%, and event-free survival rates were 71.5 and 70.5% at 5 and 10 years, respectively. No secondary malignancy has been observed so far. The prevalence of Hodgkin's disease in young children is higher and the distribution of histologic subtypes is also different from many Western countries. Canapé and sandwich treatment protocols could be used safely in clinically staged childhood HD with tolerable toxicity.
Adolescent↗
Malignant hypertension and paraganglioma in a 14-year-old girl.
A 14-year-old girl presented with malignant hypertension. Physical examination and abdominal computerized tomography revealed a paraaortic mass. Urinary catecholamines, serum renin, and aldesterone levels were high. The mass was totally excised and a paraganglioma of the organ of Zuckerkandl was confirmed. The hypertension resolved after surgery.
Adolescent↗
Malignant ovarian tumors in children: 22 years of experience at a single institution.
PURPOSE: Malignant ovarian tumors of childhood are relatively rare and thus, management is still unclear. We reviewed our experience with these tumors to evaluate their histopathologic characteristics, treatment, and outcome. PATIENTS AND METHODS: From January 1975 to December 1997, 56 patients had their malignant ovarian tumors diagnosed, treated, and followed-up in our institution. All tumors were completely excised when possible; otherwise, biopsy was performed. Staging was made according to Federation Internationale de Gynecologie Oncologique classification. Chemotherapy was recommended for all patients. Twelve cases were treated with vincristine, actinomycin, cyclophosphamide (VAC) before 1986; 12 with cisplatin, vinblastine, and bleomycin (PVB) from 1986 to 1989; and 23 with the bleomycin, etoposide, and cisplatin (BEP) regimen from 1989 to present. The Kaplan-Meier survival method was used to calculate the survival. The log-rank test was used to compare groups with respect to survival. RESULTS: Age range was 0 to 16 years (median 11 yrs; average 9.8 yrs). Only two patients were younger than 1 year. The most common presenting symptom was abdominal pain, occurring in 27 patients (48.2%). Thirty-three patients (60%) had total one-sided salpingo-oophorectomy and three patients had bilateral salpingo-oophorectomy. Nineteen patients had stage I, 15 had stage II, 19 had stage III, and 3 had stage IV disease. Dysgerminoma was the most common type. Overall survival (OAS) and event-free survival were 68% (median follow-up time: 71 mos) and 57%, respectively, after 22 years. Histopathology was not correlated with survival. Two important predictors for survival are age (P < 0.0001) and treatment protocol (P = 0.013). The BEP protocol was superior to the other regimens. The OAS was 74.6% in BEP, 55% in PVB, and 63.6% in VAC regimens. CONCLUSION: Although age at diagnosis and treatment with BEP regimen have major roles in determining prognosis of the ovarian tumors in childhood, for patients with advanced ovarian germ cell tumors, intensification of chemotherapy or the development of new approaches is necessary.
Adolescent↗
Veno-occlusive disease-like hepatotoxicity in two children receiving chemotherapy for Wilms' tumor and clear cell sarcoma of kidney.
We report two pediatric cases who developed veno-occlusive disease-like hepatotoxicity while receiving chemotherapy for Wilms' tumor and clear cell sarcoma of kidney. The chemotherapeutics, including vincristine, actinomycin D, and epirubicin in case 1 and vincristine and actinomycin D in case 2, were given before the hepatotoxicity developed. Other possible causes of hepatotoxicity were excluded. Recovery was observed with supportive therapy after 2 and 1 weeks, respectively. After recovery, the children tolerated continued chemotherapy without any decrease in the doses of drugs. We conclude that vincristine and actinomycin D were the cause of this rare from of hepatotoxicity and that chemotherapy for the underlying malignant disease could be given safely after clinical recovery.
Antineoplastic Combined Chemotherapy Protocols↗
Turkish experience with rhabdomyosarcoma: an analysis of 255 patients for 20 years.
Two hundred and fifty-five previously untreated patients (pts) with rhabdomyosarcoma (RMS) (age range 15 days to 17 years, median 5 years) were evaluated and treated in our institution. Head and neck primaries were seen in 125 patients (49%), abdominopelvic in 73 (29%), trunk and lung in 20 (5%) and extremity lesions in 37 (15%). The histology was: embryonal 137; alveolar 42; botryoid 18; pleomorphic 14. Forty-four patients could not be subclassified. The stage of the patients were as follows: 15 in state I, 74 in stage II, 139 in stage III and 27 in stage IV, according to the IRS grouping system. Patients were treated with a combination of surgery and radiation to doses of 35-55 Gy according to the patient's age and stage. All the patients received chemotherapy according to VAC or pulse-VAC (before 1988) and modified AVAC (after 1988) protocol. Survival curves were calculated by the Kaplan-Meier method. The statistical significance of each variable was tested by the log-rank test. Overall survival was 42 percent at 10 years. Three important predictors for survival time were clinical group (p < 0.001), age (p < 0.001) and primary site (p = 0.005). The best results involved clinical group I-II, age one to five years and orbital and genitourinary primary sites. An important predictor of survival time was also detected between those treated during the first ten years (1972-82) and last 10 years (1982-92), p < 0.005. Of the 96 deaths, 37 were from progressive disease, 24 from infection, 4 during postoperative period (first 7 days), 18 from unknown causes and 13 from other causes.
Adolescent↗
Hodgkin's disease and renal paraneoplastic syndromes in childhood.
The purpose of this study was to investigate children followed as having both Hodgkin's disease (HD) and nephropathy and discuss the factors which might play roles in the pathogenesis of this association by reviewing the pertinent literature. Our experience among 661 children with HD revealed ten cases (1.5%) with nephropathy; eight of these were biopsy proven. Tissue diagnoses were amyloidosis (AA type) in four cases, and membranoproliferative glomerulonephritis and minimal change glomerulopathy in two cases each. Sex distribution was equal. There was a predominance of the mixed cellular (MC) histologic type in our patients with HD. Nephropathy was shown to antedate the diagnosis of HD in two cases and to herald a relapse in one. In brief, the development of a nephropathy in a patient with HD can be considered as a paraneoplastic phenomenon. Renal amyloidosis may already be present at the time of diagnosis of HD and must be kept in mind as a cause of proteinuria due to preexisting nephropathy. Developing renal paraneoplastic syndrome, even in early-staged HD, in children, may be a poor prognostic factor.
Adolescent↗
Scleroderma in a child after chemotherapy for cancer.
The association of malignancy with scleroderma is very rare in childhood. A 13-year-old girl was diagnosed as having thymic carcinoma and received systemic chemotherapy. She presented with symptoms of Raynaud's phenomenon 9 months after the cessation of chemotherapy. She also had difficulty in swallowing. Based on the presence of Raynaud's phenomenon, characteristic skin changes over the face and hands, oesophageal involvement and pulmonary restrictive defect demonstrated by pulmonary function tests, the diagnosis of generalised scleroderma was established. There was no evidence of tumor recurrence. Although she was treated with penicillamine and prednisolone, no significant improvement was achieved in her condition during the 14-month follow up.
Adolescent↗
Monotherapy with meropenem versus combination therapy with piperacillin plus amikacin as empiric therapy for neutropenic fever in children with lymphoma and solid tumors.
The purpose of this study was to compare meropenem monotherapy with combination therapy for empirical treatment of neutropenic fever in children with lymphoma and solid tumors. Ninety episodes of neutropenic fever in children (0.7-16.0; mean age 7.7 years) with solid tumors in a single center were randomized to receive either meropenem (50 mg/kg/dose-maximum 1 g, every 8 hours) or piperacillin (200 mg/kg/dose, every 6 hours) plus amikacin (15 mg/kg daily). Failure was defined as treatment modification. Non-Hodgkin's lymphoma (NHL) accounted for 62.2 percent of all episodes, and solid tumors (37.8%) for the rest. Blood cultures were positive in 23 percent of all episodes. Sixty-seven percent of all isolated microorganisms stained Gram-positive. Overall success was 70.0 percent (63/90). The success with meropenem was comparable to that seen with piperacillin plus amikacin: 76.6 versus 64.6 percent (p = 0.25). The failure rate was 33 percent with Gram-positive culture and 78 percent with Gram-negative or mixed cultures. The solid tumor group had significantly less bacteremia (4/34 versus 17/56; p < 0.05) and treatment failure (3/34 versus 24/56; p < 0.001) than the NHL group. No serious drug-related adverse event was noticed. Meropenem monotherapy was as effective as piperacillin plus amikacin combination in the empirical treatment of neutropenic fever in children with lymphoma and solid tumors.
Adolescent↗
Intraperitoneal involvement in rhabdomyosarcoma CT findings in a child.
Intraperitoneal neoplastic involvement in rhabdomyosarcoma is rare and its incidence and imaging characteristics need to be further described. We present the computerized tomography (CT) findings of a case with pelvic rhabdomyosarcoma and intraperitoneal neoplastic involvement. Enhanced peritoneal and retroperitoneal masses were seen around the liver, spleen, in the paracolic gutters, and in the lesser sac without evidence of ascites, mesenteric nodules or omental caking. Our case also showed that absence of ascites does not preclude the presence of peritoneal involvement. Progression in the peritoneal disease was also well demonstrated by CT.
Adolescent↗
Multiple primary malignancy: a report on Langerhans cell histiocytosis associated with Hodgkin's disease.
Since multiple primary malignant tumors are rare in children, their presence can be a diagnostic and therapeutic problem. In this report, we present a six-year-old boy with Langerhans cell histiocytosis and Hodgkin's disease. On admission, the patient had lytic lesions and a periosteal reaction on the left trochanter major from which an open bone marrow biopsy was performed. The biopsy revealed Langerhans cell histiocytosis. Eight months later, the child presented with enlarged left cervical lymph nodes and the biopsy demonstrated Hodgkin's disease. Although there was an eight-month interval between the two histopathological diagnoses, the diffuse pulmonary parenchymal infiltration observed on the first admission, was later confirmed by an open-lung biopsy as Hodgkin's disease. The patient was said to have two concurrent lymphoreticular malignancies. To our knowledge, this is the youngest case reported with this association in the English language literature.
Biopsy↗
A case of the cerebro-costo-mandibular syndrome.
Explore the source record for details and available documents.
Brain↗
Methotrexate-induced leukoencephalopathy. A case report.
A six-year-old girl with non-Hodgkin's lymphoma who was treated with both intravenous (IV) and intrathecal (IT) methotrexate and developed brain damage secondary to the cytostatic drug is described. This patient displayed hypertension, hypothermia/hyperthermia, lethargy, deterioration and coma as clinical findings, and bilateral, focal white matter hyperintensities in the occipital lobes were seen in her magnetic resonance imaging (MRI). Treatment-related leukoencephalopathy is one such adverse effect of IT methotrexate administration on the central nervous system and usually appears in a generalized form.
Antimetabolites, Antineoplastic↗