Case report 28

A 12-year-old boy with severe back pain and blast-like cells in the CSF

Flow-cytometric DNA analysis of intracranial tumors in children.

The objective of this study was to investigate flow-cytometric DNA values of pediatric intracranial tumors, and to establish DNA analysis as a potential prognostic parameter. Twenty-nine brain tumor specimens from 26 pediatric patients were cryo-preserved within a 3-year period. The DNA content was measured by flow cytometry. Six of the tumor specimens had aneuploid DNA patterns. The median of the proliferation index was lower in the survivor group compared with the non-survivor group (36.4% and 47.5%, respectively). Ten of the 26 patients are still alive, eight were lost to follow up, and eight died. Flow-cytometric DNA analysis may be a helpful tool for examining brain tumors in children.…

Lack of association between HLA specificities and Wilms' tumour.

HLA antigen distribution was determined in thirty patients with Wilms' tumour, and their frequencies compared with those of an ethnically matched control population. No statistically significant association was found between any single HLA antigen and Wilms' tumour disease. The value of prospective HLA typing studies, with special respect to genetic aspects, histopathological subgrouping and survival rate of Wilm's tumour patients is discussed.

Fetal rhabdomyomatous nephroblastoma. Pathologic histology and special clinical and biologic features.

Fetal rhabdomyomatous nephroblastoma is a very rare variant of Wilms' tumor. The special clinical and histologic features of this variant are presented on the basis of a case seen in a boy who was 13 months old at nephrectomy 8 months ago and has remained healthy since then. The tumor chiefly consists of fetal striated muscle; it occurs predominantly in infancy and early childhood and is often bilateral. Angiography shows a relative paucity of vessels. The volume of the tumor (determined by ultrasonography) decreases only slightly after preoperative radio- and chemotherapy because of a predominance of mesenchymal structures. The finding of nodular renal blastema in renal parenchyma with an …

Rhabdomyosarcoma of the bladder, prostate or vagina: the role of surgery

Rhabdomyosarcoma of pelvic organs is not common enough for many people to develop large series. However, the authors from Mainz retrospectively analysed 107 children with this condition, and suggest that primary chemotherapy followed by radical surgery yields excellent cure rates. OBJECTIVE To retrospectively analyse the outcome of children with rhabdomyosarcoma (RMS) of the bladder, prostate or vagina who were treated with chemotherapy, with or without radical surgery or additional radiotherapy, at our institution since 1968. PATIENTS AND METHODS From a total of 107 children with RMS seen between 1968 and December 2001, 22 (mean age 5.9, range 0.5–18) had RMS of bladder/prostate or vagina.…

Obesity after childhood craniopharyngioma--German multicenter study on pre-operative risk factors and quality of life.

Craniopharyngiomas are tumorous embryogenic malformations. As the survival rate after craniopharyngioma is high (92 %), prognosis and quality of life (QoL) in survivors mainly depend on adverse late effects such as obesity.We analyzed 214 children and adolescents with craniopharyngioma. The records of 185 patients (86 %) were available for retrospective analysis of weight profiles and risk factors for obesity. Quality of life (QoL) was measured in 145 patients by the Fertigkeitenskala Münster/Heidelberg score (FMH) and in 77 patients by PEDQOL questionnaire.Eighty-two of 185 patients (44 %) developed severe obesity (body mass index [BMI]3 SD). Obese patients were compared with 79 patients (…

Confluens-sinuum-Obstruktion durch Langerhanszell-Histiozytose

Die Langerhanszell-Histiozytose, bis vor wenigen Jahren als Histiozytose X bezeichnet, umfast 3 verschiedene Syndrome, namlich das eosinophile Granulom, die Hand-Schuller-Christian-Krankheit sowie die Abt-Letterer-Siwe-Krankheit, und gehort zu den seltenen Erkrankungen des Kindesalters. Wir berichten uber einen 10 jahrigen Jungen, der mit einer Schwellung am Hinterkopf und beginnender Hirndrucksymptomatik zur stationaren Aufnahme kam. Klinik, konventionelle radiologische Diagnostik und Computertomographie liesen differentialdiagnostisch ein eosinophiles Granulom als wahrscheinlich erscheinen. MRT und MRT-Angiographie zeigten jedoch die akute Bedrohung des Patienten durch das intrakraniale W…

Postoperative sonographische Verlaufsuntersuchungen von Hirntumoren im Kindesalter

Postoperative neurosonography was performed in 69 babies and children after osteoclastic neurosurgery for primary cerebral tumors. Astrocytoma of the posterior fossa (26 cases) and medulloblastoma (25 cases) were frequent histological findings. Early postoperative bed-side examinations revealed reliably complications such as cerebral hemorrhage, edema or hygroma. Postoperative baseline studies were performed and reliability of sonographic criteria for tumor recurrence, such as high echogenicity and dense echotexture, were established. Regressive tissue changes under the usual postoperative cerebral radiation therapy and chemotherapy included transient increase in perifocal edema followed by…

Evoked otoacoustic emissions and pure tone threshold audiometry in patients receiving cisplatinum therapy.

Eight children and young adults with cancer were evaluated serially using pure tone audiometry as well as registration of click-evoked otoacoustic emissions (EOAE) 1 day prior to therapy as well as after various numbers of doses of cisplatinum. A reduction of EOAE-amplitudes following cisplatinum therapy was observed in all patients. This reduction tended to recover after the end of cisplatinum administration. Since EOAE are believed to result from cochlear bio-mechanical processes, the reduced emissions are interpreted as signs of cochlear dysfunction. We conclude, that EOAE testing may be a simple, non-invasive method that may detect early, transient functional impairment of hearing due t…

Long-term follow-up of children with magnetic resonance imaging and ultrasound after treatment of brain tumors

This paper compares the results of MRI and US follow-up examinations of 46 children who had undergone surgery for brain tumors. The cases included 42 posterior fossa tumors, 3 supratentorial tumors and 1 upper cervical spinal cord tumor. US examination proved to be less specific and sensitive than MRI. However, when a "bone window" is available, US is, because of the ease of its application, better suited for frequent routine examinations. Long-term follow-up should, therefore, consist of frequent regular US examinations combined with yearly MRI examinations.

Extent of Surgery in Rhabdomyosarcoma of Urogenital Structures

After high inguinal semicastration in group-1 paratesticular rhabdomyosarcoma (RMS), the patient having undergone chemotherapy can be followed closely by CT scanning without retroperitoneal lymphadenectomy. In contrast, retroperitoneal RMS should be operated on as radically as possible after downstaging the tumor mass. In RMS of the female genitalia locally limited organ-preserving surgery is the method of choice. The prognosis is excellent with adjuvant chemotherapy. Only 20% of all bladder RMS arise from the bladder dome or the movable part of the bladder, where primary partial resection including a safety margin of 3 cm of healthy tissue is possible. The majority, however, arising from t…

Behandlung eines extraneural metastasierten Medulloblastoms

We report a patient with a history of a previously treated medulloblastoma, now presenting with generalized osseous metastases. Currently there is a complete remission under continuous therapy with VP-16. Since the frequency of systemic metastases in patients with medulloblastoma is approximately 5%, we recommend a more extensive initial evaluation in these patients.

EEG-Befunde bei ALL-Patienten 10 Jahre nach Bestrahlung und Zytostatikatherapie

35 patients with acute lymphoblastic leukemia were studied electroencephalographically during a period of 5 to 10 years (median 9 years) after diagnosis. During this time 51% of the patients intermittently showed changes in the background activity, which may indicate discrete CNS-lesions. All but one patient, suffering from an intracerebral hemorrhage, had normal EEGs at the end of the individual observation period.

Early Auditory Evoked Potentials (EAEP) in Neurosurgery — A New Method for Diagnosis and Localization of Posterior Fossa Tumors in Childhood

Auditory stimuli of suprathreshold intensity (above 60 dBHL) evoke about 15 waves: an early series (EAEP) during the initial 10 milliseconds (ms), a middle latency sequence (8 to 50 ms) and the longer latency cortical potentials (50 – 300 ms). PICTON et al. (1974) made a survey of all three potential groups. Only the EAEP (waves I to IV) are generated in the infratentorial part of the brain and reflect progressive activation of the auditory tracts and nuclei (Fig. 1): Wave I is assumed to originate at the distal part of the acoustic nerve, wave II in the medulla, wave III in the caudal and wave IV in the rostral pons and wave V in the midbrain (STARR and ACHOR, 1975; STOCKARD and ROSSITER, …

Male fertility in long-term survivors of childhood ALL.

A study of fertility was conducted in postpubertal male patients who had been treated for acute lymphoblastic leukemia (ALL) during childhood or adolescence between 1970 and 1980. Thirteen men (age 18 to 35 years) participated on a volunteer basis. Their age at diagnosis was between 2 and 15 years. Therapy followed the protocol ''Memphis VII (Pinkel)." Interview, physical examination, andrological studies (ejaculate), and hormone status (luteinizing hormone, follicle-stimulating hormone, and testosterone) were performed at least 5 years after completion of therapy. No normozoospermia was achieved; 10 patients were identified with asthenozoospermia and 3 patients with azoospermia. With respe…

Surgery in rhabdomyosarcoma of the bladder, prostate and vagina

The treatment of bladder and prostate rhabdomyosarcoma (RMS) is highly controversial. Aside from chemotherapy, treatment modalities include conservative surgery, radical surgery, and pre-, intra-, or postoperative irradiation. Between 1968 and 1993, 78 children with RMS were treated at our institution. In all, 22 tumors were located in the urogenital tract (bladder/prostate, 13; paratesticular, 5; vaginal, 2; others, 2). Altogether, 6 patients had stage II disease; 7, stage III disease; and 2, stage IV disease. All 15 patients with RMS of the bladder, prostate, or vagina received chemotherapy, and 4 had additional radiotherapy. Surgery was also done in 10 patients; parents refused an operat…

Paediatric urological malignancies

DETECTING TUBERCULIN SENSITIVITY

Malignant peripheral neuroectodermal tumors in urology.

During the past few years, a new tumor type has emerged in the pediatric and adolescent group of cancer patients, which has been designated malignant peripheral neuroectodermal tumor (MPNT). This tumor has some clinical and pathological signs in common with either soft-tissue sarcomas or classic Ewing's sarcoma, but is defined as a distinct entity because of its immunohistological characteristics. The tumor expresses neuronal markers, but the pattern varies: chromogranin, neuron-specific enolase, synaptophysin, protein S-100 and others. MPNT can occur in the urogenital region. The differential diagnosis on clinical grounds must include Ewing's and soft tissue sarcomas, and also Wilms' tumor…

Wilms tumours diagnosed by routine physical examinations.

Sir: The prognosis of stage I and -II Wilms tumours with favourable histology is good, reaching 97% (stage I) and 92% (stage II) 5 year disease-free survival rates [1]. With these figures in mind the question arises whether the overall prognosis of Wilms tumours could be improved by presymptomatic routine physical examinations in an attempt to avoid stage III to V primary diagnoses. In the Federal Republic of Germany six routine physical examinations (RPE) are offered to infants, and two additional ones to children between 12 and 48 months. The 1980-1987 German Wilms tumour study [1] listed 313 patients, 29 of whom (9%) were diagnosed by such routine examinations. As shown in Table 1, the R…

Retroperitoneal neuroblastomas - a single institution experience in 90 patients

Purpose Nowadays neuroblastomas are detected incidentally by ultrasound in early stages. Primary surgery, chemotherapy and/or radiotherapy are treatment options. However, the approach in babies is controversial. A surveillance strategy is propagated by some groups. In this retrospective study, we evaluated age and stage at diagnosis, therapy and outcome of patients treated at our institutions since 1970. Material and methods 90 patients with retroperitoneal neuroblastomas were treated. They were divided into 2 groups according to age at time of diagnosis:  1 year. Results 40 patients were  1 year (1- 18 years). Detection of an asymptomatic tumour:  1 year 10%. Tumor stage at diagnosis > 1 y…

Increasing rates of DNA single-strand breaks in lymphocytes of clinical personnel handling cytostatic drugs

A total of 27 persons, working in cancer stations with exposure to cytostatics, and 40 healthy control persons were examined for DNA single-strand breaks in peripheral lymphocytes. Non-smoking personnel from cancer stations were found to have an increased rate of DNA single-strand breaks compared to the non-smoking control subjects. In the case of smokers an increased rate of DNA single-strand breaks could be recorded for those working in cancer stations as well as with the controls. DNA single-strand breaks indicate reversible damage to DNA. As DNA repair is not perfect in every case, an increased number of DNA single-strand breaks leads to irreversible DNA damage.

Wilms' tumor ? treatment and prognosis of 373 children

Between June 1980 and June 1988, 373 patients with Wilms' tumors were diagnosed and treated (34.5%, stage I; 34.5%, stage II; 14.8%, stage III; 10.7%, stage IV; and 5.4%, stage V). In all, 11% of the patients were diagnosed as a result of routine checkup examinations. The tumor volume was 400 ml in 52.7%. Overall, 78.7% of the patients had a standard-type Wilms' tumor, 7% had a low-grade malignancy lesion and 14.3% had a high-grade-malignancy tumor. Aside from radical tumor nephrectomy, treatment encompassed chemotherapy in all cases and radiotherapy in half of the patients according to the study protocol. In all, 32% of the children received preoperative treatment and 68% underwent primary…

von Hippel-Lindau disease

Congenital intracranial tumors

Tumors that are diagnosed already at birth or during the first or second week of life are called congenital tumors. They represent a special challenge to pediatric oncologists and to surgeons, and are relatively rare. From 1956 to August 1983 in the Pediatric Department of the University Hospital of Mainz, 1.027 children with tumors were observed and treated, 16 of them with congenital tumors. Among 479 tumor patients from 1956 to 1972 there was only one child with a congenital tumor; but from 1973 to 1983 there were 15 cases. Possibly, the absolute number of congenital tumors is increasing!

Secondary thyroid carcinoma after treatment for childhood cancer

Background Second malignant neoplasms (SMNs) have become a primary concern in evaluating long-term effects of treatment in pediatric oncology. Thyroid carcinoma has proven to be a common SMN. Methods. In a multicenter study involving 58 hospitals in Germany, Austria and Switzerland, 18 of 239 (7.5%) SMNs documented following first malignant neoplasm (FMN) in childhood were thyroid carcinoma. Results. The age at diagnosis of FMN ranged from 1 to 15 years. Eleven patients were female. Six children had survived Hodgkin disease, seven acute leukemia, two Ewing sarcoma and three various other tumors. Fifteen of the 18 patients had been treated with radiotherapy to the head and neck region. The t…

CLINICAL AND NEUROPHYSIOLOGICAL FINDINGS IN 47 LONG-TERM SURVIVORS OF CHILDHOOD MALIGNANCIES TREATED WITH VARIOUS DOSES OF VINCRISTINE

ABSTRACT 96 long-term survivors of cancer in childhood were reexamined after succesful therapy. In 49 who had not been treated with VINCRISTINE (VCR) reflex status and motor conduction velocities were normal. In contrast, 47-after treatment with VCR-had loss of deep tendon reflexes in a high rate and a prolongation of sensory CV independent of the 1 total cumulative dose of VCR, 2 application mode according to different treatment regimen, 3 time interval since the last injections of the drug, 4 age of children at onset of therapy.

MRI studies after treatment of brain tumors in childhood and adolescence

Forty-seven children and adolescents with brain tumors were examined by magnetic resonance imaging (MRI) after tumor resection. The typical changes and complications after surgery and chemotherapy, as well as the corresponding MRI findings, are discussed. Typical examples of boundary-layer lesions, tumor recurrences, hydrocephalus, porencephalic cysts, and hygromas are given.

Clinical aspects and prognosis of ependymoma in infants and children

Thirty-two patients (22 boys and 10 girls) with a histologically confirmed diagnosis of ependymoma were treated between 1972 and 1999. A total macroscopic resection was achieved in 16 of these patients, whereas 15 resections were classified by the surgeon as subtotal. In 1 patient a ventriculostomy was created as part of a palliative strategy. All children over 3 years old were treated with postoperative radiotherapy. Chemotherapy consisted of procarbazine, ifosfamide, etoposide, methotrexate, cisplatin and cytosine arabinoside. There was 1 perioperative death. Twenty children developed a relapse of disease within 2 months to 13 years and 1 month after the initial therapy. A maximal number …

Orbital rhabdomyosarcoma in childhood

Background: Rhabdomyosarcoma is the most common malignant orbital tumor in children. Treatment modalities (individualized therapy or study protocol) have been changed radically. Surgery was supplemented by radiation therapy and chemotherapy. The objective of our retrospective analysis was to define the prognosis in correlation to changes of treatment in an unselected patient group of a single institution. Patients and methods: Between 1954 and 1995, 18 patients (age at presentation 1 month to 17 years, 11 male and 7 females) with orbital rhabdomyosarcoma were diagnosed and treated at our institution. Results: The 5-year survival rate was 76 %. Primary exenteration was replaced by tumor rese…

Angioma-Like Pseudometamorphosis in Wilms' Tumors Subjected to Preoperative Radio- and Chemotherapy

Six Wilms' tumors (stage III-V), which had undergone preoperative irradiation and chemotherapy, were examined histologically. While mesenchymal and differentiated epithelial tumor elements were preserved to a large extent, blastemic tumor structures were almost completely lacking. Instead, pseudoangioma-like structures similar to cavernous hemangioma prevailed. As demonstrated by transitional pictures, the pseudoangioma-like pattern is a result of the fact that blastemic tumor elements are replaced by blood after therapy-induced necrosis and lysis. The clinical advantages of preoperative treatment are briefly discussed, especially for patients with advanced tumors.

Early diffuse leptomeningeal primitive neuroectodermal tumors can escape detection by magnetic resonance imaging.

Primitive neuroectodermal tumors are easily detected by neuroradiologic imaging, as a rule. We report on two patients with early diffuse leptomeningeal primitive neuroectodermal tumors which escaped detection by contrast-enhanced magnetic resonance imaging.

Unusual pattern of gastric and hepatic infiltration in an infant with acute monocytic leukemia

A 10-month-old girl with acute monocytic leukemia is presented. Ultrasound of the abdomen on admission showed an unusual distribution of leukemia infiltrates in the stomach and liver. The leukemic infiltrates in the wall of the stomach presented as intraluminal polyps, while the leukemic infiltration of the liver was strictly confined to the portal vessels.

Radical surgery and different types of urinary diversion in patients with rhabdomyosarcoma of bladder or prostate--a single institution experience.

Abstract Purpose In a retrospective study we analyzed the outcome of patients treated for rhabdomyosarcoma (RMS) of the bladder/prostate with special attention to radical surgery. Methods In 25 patients with genitourinary RMS (15 bladder/10 prostate) the median age at diagnosis was 4 years [1] , [2] , [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] , [17] , [18] , and 8 patients had a stage II RMS, 12 stage III and 5 stage IV. In 19/25 (12 bladder/7 prostate), radical surgery and urinary diversion were performed. Urinary diversion comprised 2 continent anal diversions, 11 continent cutaneous diversions, 4 colon conduits and 2 urethral diversions (2 +…

Acid Dnase Activities In Peripheral, Mononuclear Blood Cells: A Possible Parameter To Detect Proliferating Cell Populations

After electrophoresis in DNA -containing polyacrylamide gels, two acid DNase activities can be detected in peripheral, mononuclear cells of the human blood. One of these acid DNase activities correlates with cell proliferation; its isoelectrical point is at pI 7.4. By means of this DNase activity, a quantity of less than 1% leukemic cells can be detected. The increased acid DNase activity can indicate the proliferation of malignant cell populations and possibly the proliferation of cell populations during immunological reactions

210. Das Ewing-Sarkom ? Erfahrungen mit der gliedmassenerhaltenden, chirurgischen, radiologischen und chemotherapeutischen Kombinationstherapie

Seit 1981 wird im Mainzer Universitatsklinikum vor allem bei peripher lokalisierten Ewing-Sarkomen eine Kombinationsbehandlung durchgefuhrt, die ohne erhohtes Uberlebensrisiko eine bleibende Funktionstuchtigkeit der betroffenen Extremitat ermoglicht. Es handelt sich hierbei um eine zeitlich genau aufeinander abgestimmte Anwendung 4 verschiedener Chemotherapeutica, einer lokalen Resektion des tumortragenden Knochenabschnittes und einer von 60 auf 36 Gy reduzierten Rontgennachbestrahlung, die strahlenbedingte Spatschaden weitgehend vermeidet und zur Rezidivprophylaxe ausreicht. Eine osteoplastische Wiederherstellung ist ebenfalls moglich. Mehrere nach diesen Vorgehen behandelte Beispiele bele…

Immunological investigations in two brothers with ataxia telangiectasia Louis-Bar

Two of three brothers with the classical signs of ataxia telangiectasia were investigated for their immunological disorders at the ages of 13 and 16 years, respectively. The elder brother also suffers from autoimmune hemolytic anemia, a complication which has not yet been described in the course of ataxia telangiectasia. Immunological investigations made in both brothers showed a reduction in the number and function of T lymphocytes. The number of B lymphocytes was normal, among which there were cells staining for IgA, although serum IgA was absent. It seems possible that this phenomenon is caused by a disturbance in the process of maturation of lymphoid cells with a lack of differentiation…

Testicular lymphoblastic leukemia/lymphoma

Acute lymphoblastic leukemia is by far the most frequent malignant disease in children. In all, 5% of the boys affected will develop testicular disease either at initial presentation or during the disease course or as the first site of relapse. Modern treatment regimens have reduced the occurrence of testicular relapses, which was more frequent in the 1970s. There is no place for preventive measures for early recognition of testicular leukemia; routine biopsies have been abandoned, and prophylactic irradiation is not justified. In gross overt disease, orchiectomy is justified (1) in cases of huge bulky testicular disease, (2) if unilateral disease is probable, and (3) if radiation of the te…

Progress and controversies in modern treatment of Wilms' tumors.

During the past 25 years the treatment of Wilms' tumors has been refined owing to a series of thoroughly performed clinical studies. The overall prognosis is currently 80% (long-term cures), but different therapeutic issues are still discussed controversially, especially the question of preoperative treatment. A number of different drugs have proved effective in the treatment of Wilms' tumors. It remains to be determined as to which drug combinations are the most effective for which histologic subtype and which stage of disease. It seems that these questions can be answered only if further studies consequently implicate analyses of early and long-term toxicities. The same is true for analys…

Pediatric urological cancer.

Acute leukemia following anticancer treatment

Radical Surgery in Children and Adolescents with Rhabdomyosarcoma of the Lower Urinary Tract Using Orthotopic Bladder Substitution and Heterotopic Urinary Diversion – A Single Institution Experience

Abstract Purpose Children with rhabdomyosarcoma(RMS)of the bladder/prostate can be treated by radical or organ sparing surgery both in combination with chemotherapy ± radiation therapy. In this retrospective study we analyzed the outcome with special attention to radical surgery and urinary diversion (UD). Material and Methods 24/32 patients with RMS of the genitourinary tract had RMS of the bladder (n=14) or prostate (n=10). 8 patients were stage II, 11 stage III and 5 stage IV. In 18/24 with RMS of the bladder/prostate, radical surgery and urinary diversion was performed. 3 had chemotherapy only, 2 in combination with radiotherapy. One patient underwent partial cystectomy. For UD: uretero…