Cancer care in Romania: challenges and pitfalls of children's and adolescents' multifaceted involvement.

Communication about diagnosis and medical treatment for children suffering from life-threatening illnesses is complex. It is a primary step in involving underage patients and families in care and lays the foundation for obtaining parental permission and patient assent for treatment. In practice child participation in care is often difficult to obtain due to patients' different and sometimes fluctuating preferences, but also parents' protective strategies. Physicians may be susceptible to parental wishes to limit information and feel uncomfortable discussing issues related to uncertainty of cure with patients. A qualitative study in Romanian paediatric oncology units was conducted to explore children's involvement from the perspectives of parents and oncologists. Interviews with participants discussed 18 patient cases. Data were transcribed and thematic analysis was used to interpret and mine patients' involvement during treatment. Different facets of patient participation were identified: restricting, widening and enhancing involvement. A fourth category, unintentional involvement, occurred for all patients due to children's observations during long-term hospitalisations and access to Internet. Uncertainty overarched parental attitudes regarding the extent to which children should be included. Physicians usually complied with parental wishes to limit involvement, but together with parents involved patients at least in a practical way. Adults' protective attitude may backfire, as adolescents' online searches often expose patients to worse-case scenarios. Further research should acknowledge the hazards of restricted diagnosis disclosure and develop clinician tools to support families in communicating with patients. This should be paralleled by physician efforts to elicit patients' needs regarding participation.

Parents' Challenges and Physicians' Tasks in Disclosing Cancer to Children. A Qualitative Interview Study and Reflections on Professional Duties in Pediatric Oncology.

BACKGROUND: Professional guidelines encourage physicians to provide children with as much information regarding their health as deemed developmentally and emotionally appropriate. However, empirical research indicates that in clinical practice, an open discussion with children about cancer is often lacking. This study explores impeding factors to and possible strategies for open communication of cancer diagnosis to children from the perspectives of parents and physicians. PROCEDURE: Semi-structured interviews were conducted with 18 parents of children with cancer and 10 treating oncologists. The patient sample was obtained from three pediatric units in Romania. Interviews were transcribed verbatim and interpreted using thematic analysis. Inductive open-coding procedures identified participants' accounts regarding their experiences with cancer diagnosis and treatment. Final themes were selected by grouping codes that formed a pattern in the data. RESULTS: An interplay of mainly three different factors-information overload and emotional turmoil, lack of knowledge and skills for disclosing the diagnosis, and assumptions about burdening the child when discussing cancer-restricted parent-patient communication and subsequently affected physician-patient exchanges. Oncologists recommended open communication at diagnosis, but left the final decision to the parents. They adapted their communication style with patients to parents' preference. CONCLUSIONS: Although physicians need to respect the wishes of children's legal representatives, they also have a duty to promote patients' best interests. We recommend that physicians employ a proactive stance in ensuring that children with cancer are appropriately informed about their diagnosis. In case of parents' arduous objections to full disclosure, an ethical consultation should be considered.

Chemotherapy-related toxicity in childhood neoplasia.

PURPOSE: To evaluate the incidence and time of occurrence of chemotherapy-related toxic events in 100 children admitted to the Hematology-Oncology Ward of "Sfanta Maria" Children's Emergency Hospital in Iasi, Romania, over a 4-year period. METHODS: An analytical, descriptive and comparative, retrospective and prospective study covering a 4-year period on the incidence of chemotherapy side effects, was performed on 100 children admitted for solid tumors or hematologic malignancies. The probability of each adverse event to appear and the time period from chemotherapy initiation to the moment of side effect appearance were assessed. RESULTS: The most frequent toxicity was alopecia (79.5%), followed by medullary aplasia (71.1%), oral candidiasis (65.3%), diarrhea and emesis (64% each), toxic hepatitis (61%), and Cushing's syndrome (21.5%). Oral herpes and thrush were less frequent (13.2% and 12.2%, respectively). Remissions of the underlying disease were achieved in 69.9% of the cases. Alopecia, medullary aplasia and oral candidiasis developed during the first 14 months of treatment. Mucositis, emetic syndrome and toxic hepatitis were diagnosed within the first 12 months of treatment. Diarrhea and oral herpes or thrush appeared during the first 15 months, while Cushing's syndrome developed during the first 6 months. All remissions were obtained during the first 4 months of treatment. CONCLUSIONS: While alopecia and medullary aplasia were the most frequent side effects of chemotherapy in our study group, the earliest were Cushing's syndrome, emetic syndrome and toxic hepatitis.

Childhood rhabdomyosarcoma. Anatomo-clinical and therapeutic study on 25 cases. Surgical implications.

UNLABELLED: Rhabdomyosarcomas (RMS) are the most frequent soft tissue sarcomas of childhood. Despite advances in knowledge about biological pathways of tumorigenesis, risk stratification and multimodal treatment, the immediate and long-term prognosis of these lesions in many countries with limited resources is still poor. PATIENTS AND METHODS: Twenty-five histologically confirmed pediatric RMS were recorded during the period of study. Demography, clinical presentation, diagnostic means, pretreatment staging and post-surgical grouping, histological type, therapy and outcome were evaluated. RESULTS: The mean age was 6.7 years; the group included 12 boys and 13 girls. Twelve lesions were localized in the genitourinary tract, eight in the trunk and extremities, two cases each in head and neck and retroperitoneum and one case in biliary tract. Primary surgical attempt was performed in 15 patients but only in nine of them underwent complete resection (three with free margins) other six cases achieving removal with residual disease. In 10 cases, solely biopsy was possible. Twenty-four patients received chemotherapy but only four cases performed radiation therapy. Overall survival rate was only 36% (nine cases). CONCLUSIONS: As mean feature children from our series had late presentation with locally extended (bulky and node positive) lesions and unfavorable sites. Improved multimodal management of RMS in recent years will probably lead to better survival curves in an increasing number of cases and an outstanding outcome in children with locally advanced disease.

[Children's Langerhans cell histiocytosis, diagnostic problems]. / Probleme de diagnostic în histiocitoza langerhans la copil.

Langerhans cell histiocytosis is a group of idiopathic disorders characterized by the proliferation of cells of the mononuclear phagocyte system and the dendritic cell system. From the clinical point of view there are 3 forms of histiocytosis encountered in child pathology: acute, disseminated called Abt-Letterer-Siwe (it usually affects children under 2 years of age), eosinophilic granuloma and the intermediate clinical form called Hand-Schuller-Christian disease. We present the case of a premature born child that had, even from the day she was born, a rash disseminated on the entire surface of the body associated with enlarged lymph nodes, hepatosplenomegaly and thrombocytopenia. The diagnosis of Langerhans cell Histiocytosis is difficult to establish and this case is an example that confirms it. Therefore, we present the difficulties in diagnosing this case and its particular evolution.

[Diamond-Blackfan anemia. Case report]. / Anemia diamond-blackfan. Cazuri clinice.

Diamond-blakfan anemia (DBA) is a rare condition in pediatric pathology, with more than 500 cases described in literature. It is a congenital eritroblastopenia, with AD/AR/ X-linkage inheritance, 90% were diagnosed by one year of age. Physical appearances were abnormal only in 29%. The current recommendations are corticosteroids, with suportive treatment and bone marrow transplantation. In general, DBA remains a single cytopenia. Long term prognosis remains uncertain, DBA is a preleukemia condition. We present two cases that we took care of in our clinics.

[Statistical evaluation of clinical characteristics and therapeutic management of Hodgkin disease in children over a 10 year period]. / Studiu statistic clinico-terapeutic si evolutiv pe 10 ani al bolii Hodgkin la copil.

UNLABELLED: Hodgkin disease represents the third malign disease like frequency registered to children. Generally, the most cases have been observed to children between 10 and 15 years old. MATERIAL AND METHOD: In our study the patients have been investigated on Pediatric Hemato-Oncologic Department from Clinical and Emergencies Hospital for Children "Sf. Maria" Iasi in a period between 1995 and 2004. RESULTS: The age of patients was between 2 and 17 years, from this group 31 were boys (54.38%) and 21 girls (45.62%). The medium incidence of disease was 5.7 cases per year. Histological analysis indicates 2 cases with lymphocyte prevalence (3.50%), 18 with nodular sclerosis (31.58%), 32 with mixed cellularity (56.14%) and 5 patients with lymphoid depletion (8.78%). Ann-Arbor classification of disease indicates high frequency of stage II on the beginning (38 cases), with primary localization to neck (52 cases) and mediastinum (29 cases). 35 patients have presented biochemical syndrome (stage b). After therapeutically intervention on 55 patients the recovery has been observed, 42 patients didn't present relapse symptoms, 5 patients presented precocious relapse (under 12 months), respectively 6 patients presented tardy relapse (after 12 months). CONCLUSION: The factors for favorable prognosis were: histology with lymphocyte prevalence, stage I or II without systemic signs (FFS 96%), primary localization of disease on one or two groups of ganglions, without hilar and mediastinal zones.

[Kostmann disease in children]. / Boala kostmann la copil.

Kostmann's disease (KD) is a severe congenital neutropenia, a rare autosomal recessive disorder of neutrophil number. Complete blood count with differential reveals an ANC less than 500/mm3 as seen in classic cases, monocytosis and eosinophilia. Bone marrow aspiration or biopsy reveals an arrest of neutrophil precursor maturation at the promielocyte or myelocyte level. KD results in an increased susceptibility to frequent bacterial infections. In the absence of medical intervention with granulocyte colony-stimulating factor (G-CSF), bone marrow transplantation, the mortality rate is 70% within the first year of life. We present three cases to illustrate this rare entity, the difficulty of diagnosis, but also to underline the importance of correct treatment.

[Clinical features and therapeutics in Hodgkin disease of children]. / Particularitati evolutive si terapeutice ale bolii Hodgkin la copilul mic.

AIM: To investigate the incidence and evolution of Hodgkin disease to children under 5 years age. MATERIAL AND METHOD: The patients have been investigate to Pediatric Hemato-Oncologic Department from Clinical and Emergencies Hospital for Children "Sf. Maria" Iasi in a period between January 1980 and December 2004. In this period 129 patients with Hogkin disease have been hospitalised, and among these 48 patients was under 5 years age (37.20%). RESULTS: From our patients 41 were boys (85.41%) respectively 7 girls (14.59%). Anatomopathologic examination of samples from affected ganglions indicate: 7 patients with lymphocyte prevalence (14.58%), 6 with nodular sclerosis (12.50%), 31 with mixed cellularity (64.58%) and 4 patients with lymphoid depletion (8.34%). Ann-Arbor classification of disease indicate a high incidence of stage II (27 cases--56.25%) in the beginning period than stage I (15 cases--31.25%), III (3 cases--6.25%) and IV (3 cases--6.25%). In 9 cases (18.75%) biochemical syndrome has been observed. Radiotherapy (35 cases--72.91%) and chemotherapy (27.09%) have been used. Failure Free Survival after 5 years for patients with lymphocyte prevalence was 96% for stage I A and II A, 48% for stage III respectively 18% for stage IV. Complications observed were: precocious--medullary toxicity (3 cases), metabolic disorders (4 cases), and late--lung fibrosis with restrictive respiratory dysfunction (2 cases), cardiac dysfunctions (1 case).

[Digestive disease in the immunocompromised patient with acute lymphoblastic leukemia. Experience of the IVth Pediatric Clinic--Oncology Department of the Iasi Sfânta Maria Children's Hospital]. / Patologia digestiva la pacientul imunocompromis prin leucemie acuta limfoblastica in experienta clinicii a IV-a pediatrie--sectia himato-oncologie a spitalului de copii Sfânta Maria" Iasi.

UNLABELLED: The goals of this paper were to study the various types of digestive disease in acute lymphoblastic leukemia (ALL), to characterize the children in the study group by age, sex and environment, by presence of liver and spleen enlargement, levels of GOT, GPT, vomiting, to evaluate methotrexate (MTX) serum levels al 24 hours and 48 hours after administration, and to analyze the correlation between MTX levels and MTX liver and blood toxicity. MATERIAL AND METHOD: We studied 39 immunocompromised children hospitalized in the IV-th Pediatric Clinic-Oncology Ward, between 1983-2005, with acute lymphoblastic leukemia (ALL); most of them exhibited defects of humoral immunity such as transitory hypogammaglobulinemia, and defects of the cellular immunity that accompanied hepatomegaly, hepatic cytolysis and biliary obstruction. RESULTS: The diagnostic of ALL was sustained by: medullar biopsy, lumbar punction, cytochemical reactions, blood cell count, flow-cytometry, methotrexate level determination. Hepatic damage was measured by: GOT, GPT, bilirubin, LDH, GGT, FA, HBS antigen, anti HCV antibodies, anti HVA antibodies, anti toxoplasmosis antibodies, anti CMV antibodies, serum protein levels, TQ, inflammation markers. A slight increase in the number of diagnosed ALL cases during the past two decades was noticed and ALL incidence was higher in boys than girls. Hematologic toxicity of MTX is a real problem, causing neutropenia. MTX also caused oral lesions (in 69.36% of children), vomiting (in 69.2% of children) and liver toxicity (in 51.3% of children). MTX serum level 24 hours after administration is significantly different from the serum level at 48 hours after administration. Thus, the use of calcium folinate is proved to be very effective. We have also demonstrated that vomiting had some other causes besides MTX administration.

[Statistical study of the evolution over ten years of the clinical and therapeutic approach in childhood soft tissue sarcoma]. / Studiul statistic clinico-terapeutic si evolutiv pe 10 ani in sarcoamele de tesuturi moi la copii si tineri.

UNLABELLED: Soft tissue sarcoma has a primitive mesenchymal origin and represents a heterogeneous group of malignant entities with a continuous rising frequency in the age range below 18. Rhabdomyosarcoma (RMS) constitutes 5.8% from the whole amount of pediatric solid tumors, taking the fourth place after CNS tumors, neuroblastoma, and Wilms tumors. The other category of non-rhabdomyosarcoma tumors in children and teenagers represents 3% of the solid malignancies under 18 years old. Our study looks at the particularities of the pathological, clinical, therapeutic aspects and the statistical correlation with prognosis. We included in the study a well-known category of soft tissue tumors called of uncertain malignancy. MATERIAL AND METHOD: The study was made between January 1995 and July 2005 in the Pediatric Department of Hematology and Oncology of the "Sf. Maria" Clinical Emergency Hospital Iasi on a group of 58 de patients ranging between 0 and 18 years old. RESULTS: Positive diagnostic confirmation was established on pathological grounds in optic microscopy and immuno-histo-enzymology performed on bioptic samples in Department of Pediatric Surgery and analyzed in Pathology Laboratory of "Sf. Maria" Clinical Emergency Hospital Iasi. Based on histological examination 19 cases (32.75%) were of rhabdomyosarcoma type with following subtypes: alveolar--7 patients, embryonic-- 9 cases, fusiform - 2 cases, bothrioid--1 case), 8 cases were undifferentiated soft tissue sarcomas and one patient had a tumor of pleiomorphic type; 13 children (22.41%) had non-rhabdomyosarcoma soft tissue sarcomas: 6 fibrosarcomas, 2 synovial sarcomas, 1 leiomyosarcoma, 1 Kaposi sarcoma, 1 case of malignant peripheral nerve sheath tumor, 1 case of angioma tumor, one liposarcoma; 16 cases were included in soft tissue tumors of uncertain origin (fibromatosis--6 cases, fibrous histiocytoma--4 cases, hamartoma--cases, myoblastoma--1 case, fibro-xanthoma--1 case, hemangioendothelioma--1 case); 1 PPNET (Askin tumor). CONCLUSIONS: The continuously augmented incidence of soft tissue sarcoma in young ages and the advanced stages initial presentations of tumors require a much more serious and rhythmic survey at general practitioner level. An improvement of diagnostic means is useful, taking into account the limits of optic microscopy. Therapy must be stage and histology adapted; lowering risk toxicity of good prognosis cases and enhancing doses when required are the only reasonable options to avoid over treatment and to have a much better survival rate.

[The global survival rate calculated by Kaplan-Meier method in children with malignant lymphomas]. / Supravietuirea globala în limfoame maligne la copil apreciata prin metoda Kaplan-Meier.

The Kaplan Meier method is being used in oncology in order to calculate the survival rate during/at the end of the study. The purpose of this study is the assessment of the survival period referred to the clinical and histopathological state, laboratory findings, the diagnostic and treatment time. The material of the study is a LIMCO group (n=308 cases) hospitalized in the Oncology Department of the Children's Clinical and Emergency Hospital "Sf. Maria", Iasi between 1980-1995. The LIMCO group was divided in 2 smaller groups: LMH group (110 cases) and a LMNH group (198 cases). The results statistical analysis was made by chi2, Long-Rank and Breslow test. The results established a series of favorable LIMCO prognostic factors referred to the clinical state I-II, the histopathological type, normal or pathological laboratory findings at the first admission and the quality of the remission after the multimodal treatment.

[Metabolic abnormalities with hematologic manifestation]. / Anomalii metabolice cu expresie hematologica.

These are unusual diseases in pediatric pathology. We present the morphologic aspects of peripheral blood smear and bone marrow smear of nine storage disease cases.

[Global survival rate calculated by Kaplan-Mayer method in children with malignant lymphomas]. / Supravietuirea globala în limfoame maligne la copil apreciata prin metoda Kaplan Maier.

The Kaplan Maier method is being used in oncology in order to calculate the survival rate during/at the end of the study. The purpose of this study is the assessment of the survival period referred to the clinical and histopathological state, laboratory findings, the diagnostic and treatment time. The material of the study is a LIMCO group (n = 308 cases) hospitalized in the Oncology Department of the Children's Clinical and Emergency Hospital "Sf. Maria", Iasi between 1980-1995. The LIMCO group was divided in 2 smaller groups: LMH group (110 cases) and a LMNH group (198 cases). The results statistical analysis was made by chi 2, Long-Rank and Breslow test. The results established a series of favorable LIMCO prognostic factors referred to the clinical state I-II, the histopathological type, normal or pathological laboratory findings at the first admission and the quality of the remission after the multimodal treatment.

[Hemangioendothelioma in children]. / Hemangioendoteliom la copil.

The hemangioendothelium is a vascular tumor rarely seen in the pediatric practice. This case underlines the difficulty that lies in establishing a differential diagnosis with a localized adenopathy when this vascular tumor is developing inside a ganglionic region. The surgical treatment followed by interferon therapy determined a favorable evolution in this case because the hemangioendothelium is a vascular borderline tumor. Still, there are cases that recur, metastasize and have an evolution towards death.