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D Harms

Publications and source records attributed to D Harms.

At least 55 records · Page 3Linked to original sources
PubMed1999

Dietary polyamines are essential luminal growth factors for small intestinal and colonic mucosal growth and development.

BACKGROUND: Polyamines are essential for cell growth. Dietary and probably gut bacterial derived polyamines contribute significantly to the polyamine body pool. AIMS: To evaluate the influence of dietary, luminal polyamines on growth and development of different gastrointestinal organs in normally growing rats. METHODS: Male suckling Wistar rats were randomly allocated to four treatment groups: polyamine deficient diet (PDD); PDD plus antibiotics (neomycin 2 g/kg and metronidazole 34 mg/kg); PDD plus polyamine supplementation at normal concentrations; or normal standard laboratory chow. After a six month feeding period 7-10 animals/group were sacrificed. RESULTS: No differences in body weight gain, food consumption, or general behaviour could be observed between the four groups of animals. Feeding of PDD alone or PDD plus antibiotics resulted in a highly significant decrease in organ weight, protein content, and DNA content in small intestinal and colonic mucosa whereas no alterations were found in the liver. CONCLUSIONS: Long term feeding of polyamine deficient diets resulted in a significant hypoplasia of small intestinal and colonic mucosa. Dietary, luminal polyamines are important local factors for growth and the development of small intestinal and colonic mucosa.

Animal Nutritional Physiological Phenomena↗
PubMed1999

Results of treatment for soft tissue sarcoma in childhood and adolescence: a final report of the German Cooperative Soft Tissue Sarcoma Study CWS-86.

PURPOSE: The goal of the second German Soft Tissue Sarcoma Study CWS-86 (1985 to 1990) was to improve the prognosis in children and adolescents with soft tissue sarcoma by means of a clinical trial comprising intensive chemotherapy and risk-adapted local therapy. PATIENTS AND METHODS: There were 372 eligible patients. A staging system based on the postsurgical extent of disease was used. Chemotherapy consisted of vincristine, dactinomycin, doxorubicin, and ifosfamide. Radiotherapy was administered early at 10 to 13 weeks simultaneously with the second chemotherapy cycle (32 Gy or 54. 4 Gy). The single dose was reduced to 1.6 Gy and given twice daily (accelerated hyperfractionation). RESULTS: The event-free survival (EFS) and overall survival rates at 5 years were 59% +/- 3% and 69% +/- 3%, respectively. The 5-year EFS rate according to stage was as follows: stage I, 83% +/- 5%; stage II, 69% +/- 6%; stage III, 57% +/- 4%; and stage IV, 19% +/- 6%. The outcome for patients with stage III disease who required radiotherapy was much better in the CWS-86 study compared with the CWS-81 study (5-year EFS, 60% +/- 5% v 44% +/- 6%; P =.053). The most common treatment failure was isolated local relapse, with 14% of patients relapsing at the primary tumor site. CONCLUSION: The improved design of the study incorporating risk-adapted radiotherapy allowed treatment to be reduced for selected groups of patients without compromising survival.

Adolescent↗
PubMed1999

Radiation therapy for intracranial germinoma: results of the German cooperative prospective trials MAKEI 83/86/89.

PURPOSE: A multicenter prospective trial was conducted (Maligue Keimzelltümoren [MAKEI] 83/86/89) to assess outcome in intracranial germinoma after treatment with radiotherapy alone at reduced doses. PATIENTS AND METHODS: Between 1983 and 1993, 60 patients with histologically (n = 58) or cytologically (n = 2) confirmed germinoma were enrolled onto the study. Patients received radiotherapy alone (craniospinal axis/local boost). In the MAKEI 83/86 study (involving 11 patients), the dose to the craniospinal axis was 36 Gy and the dose to the tumor region was 14 Gy. In the MAKEI 89 study (involving 49 patients), doses were 30 and 15 Gy, respectively. RESULTS: Median patient age was 13 years (range, 6 to 31 years). Complete remission was achieved in all patients. The estimated (Kaplan-Meier) 5-year relapse-free survival rate was 91.0% +/- 3.9% at a mean follow-up of 59.5 months (range, 3 to 180 months); the estimated overall survival rate was 93.7% +/- 3.6%. Relapse occurred in five patients 10 to 33 months (mean, 18.4 months) after diagnosis (one patient developed a spinal canal metastasis and underwent salvage radiotherapy and chemotherapy; four patients had metastases outside the CNS and underwent salvage chemotherapy alone). Four patients died: one died from disease, two died from therapy-related complications, and one committed suicide. Acute complications with long-lasting sequelae were tumor or surgery related (three cases of blindness, six of reduced vision, two of hemiparesis). Psychosocial development was normal in the majority of patients. CONCLUSION: Radiotherapy directed toward the craniospinal axis or tumor site alone at decreased dose levels is effective. To reduce the risk of late side effects, further attempts to decrease total doses are justified. In cases of recurrent disease, chemotherapy administered outside the CNS is the treatment of choice.

Adolescent↗
PubMed1999

German military medicine: missions and innovations.

The German military medical service's foremost task in peacetime is to train and prepare medical personnel for their tasks in wartime. To this end, six medical subservices have been formed. They provide inpatient and outpatient care for the assigned military formations. Medical support for the reaction forces follows the principles of "operational medicine," which is provided in three qualitatively coordinated medical support levels. These support levels from the "lifesaving chain" for Bundeswehr soldiers deployed abroad, who are entitled to receive medical care that corresponds to the medical standard in Germany.

Diffusion of Innovation↗
PubMed1998

[Self-mutilating hand and finger injuries among physicians suspected of insurance fraud].

27 cases of questionable self-mutilation among physicians (concerning finger and hand injuries) were analyzed retrospectively. The evaluation based on files of the insurance companies, reconstructive examinations and experimental investigations. Three typical cases are described in detail, the "victims" were suspected to have committed a special kind of fraud in order to claim financial compensation from their accident-insurance companies; the contract included special invalidity payments for finger injures (so-called "Gliedertaxe"). Many of these physicians had worked in the surgical field: apart from one 38-year-old female all of them were males, aged between 41 and 66 years. The mutilating instruments were often circular saws, sometimes chain saws and a great variety of other tools, for example hatchet, big scissors and other sharp instruments. By means of forensic-medical expertise and criminalistic investigations the insurance companies were able to demonstrate clearly that the amputation was voluntarily self-inflicted. The forensic-medical argumentation concerning intentional self-mutilation included the so-called execution position of the finger, the injury pattern, the investigations of blood stains and ergonometric aspects.

Adult↗
PubMed1998

Second malignancies after treatment for Ewing's sarcoma: a report of the CESS-studies.

PURPOSE: During recent years, more intensified systemic and local treatment regimens have increased the 5-year survival figures in localized Ewing's sarcoma to more than 60%. There is, however, concern about the risk of second malignancies (SM) in long-term survivors. We have analyzed the second malignancies in patients treated in the German Ewing's Sarcoma Studies CESS 81 and CESS 86. MATERIALS AND METHODS: From January 1981 through June 1991, 674 patients were registered in the two sequential multicentric Ewing's sarcoma trials CESS 81 (recruitment period 1981-1985) and CESS 86 (1986-1991). The systemic treatment in both studies consisted of a four-drug-regimen (VACA = vincristine, actinomycin D, cyclophosphamide, and adriamycin; or VAIA = vincristine, actinomycin D, ifosfamide, and adriamycin) and a total number of four courses, each lasting nine weeks, was recommended by the protocol. Local therapy in curative patients was either complete surgery (n = 162), surgery plus postoperative radiotherapy with 36-46Gy (n = 274), or definitive radiotherapy with 46-60Gy (n = 212). The median follow-up at the time of this analysis was 5.1 years, the maximum follow-up 16.5 years. RESULTS: The overall survival of all patients including metastatic patients was 55% after 5 years, 48% after 10 years, and 37% after 15 years. Eight out of 674 patients (1.2%) developed a SM. Five of these were acute myelogenic leukemias (n = 4) or MDS (n = 1), and three were sarcomas. The interval between diagnosis of Ewing's sarcoma and the diagnosis of the SM was 17-78 months for the four AMLs, 96 months for the MDS and 82-136 months for the three sarcomas. The cumulative risk of an SM was 0.7% after 5 years, 2.9% after 10 years, and 4.7% after 15 years. Out of five patients with AML/MDS, three died of rapid AML-progression, and two are living with disease. Local therapy (surgery vs. surgery plus postoperative irradiation vs. definitive radiotherapy) had no impact on the frequency of AML/MDS, but local therapy did influence the risk of secondary sarcomas. All three patients with secondary sarcomas had received radiotherapy; however, all three sarcomas were salvaged by subsequent treatment and are in clinical remission with a follow-up of 1 month, 4.3 years, and 7.5 years after the diagnosis of the secondary sarcoma. Thus far, SM contributed to less than 1 % (3/328) of all deaths in the CESS-studies. CONCLUSIONS: The risk of leukemia after treatment for Ewing's sarcoma is probably in the range of 2%. The risk of solid tumors also seems to be low within the first 10 years after treatment and remains in the range of 5 % after 15 years. In the CESS-studies, less than 1% of all deaths within the first 10 years after diagnosis were caused by SM. Effective salvage therapy for secondary sarcomas is feasible.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1998

TEL-AML1 fusion transcript in relapsed childhood acute lymphoblastic leukemia. The Berlin-Frankfurt-Münster Study Group.

The cryptic translocation t(12;21)(p13;q22) has been recently recognized as the most common genetic rearrangement in B-lineage childhood acute lymphoblastic leukemia (ALL). The resulting fusion transcript, termed TEL-AML1, has been associated with an excellent prognosis at initial ALL diagnosis. Hence, we postulated that the incidence of TEL-AML1 fusion should be lower in patients with ALL relapse. To address this assumption and to investigate the prognostic significance of TEL-AML1 expression in relapsed childhood ALL, bone marrow samples of 146 children were analyzed by reverse-transcriptase (RT)-polymerase chain reaction (PCR). All children were treated according to Berlin-Frankfurt-Münster (BFM) ALL relapse trial protocols (ALL-REZ BFM 90-96). Their clinical features and outcome were compared with those of 262 patients who could not be tested due to lack of bone marrow samples. Thirty-two of 146 children with relapsed ALL were TEL-AML1-positive. Four of the negative patients had T-lineage and nine Philadelphia chromosome (Ph)-positive leukemia. Thus, the incidence of TEL-AML1 in relapsed Ph1-negative, B-cell precursor ALL is 32 of 133 (24%). The 32 TEL-AML1-positive and 101 negative patients differed significantly with respect to duration of last remission (42.5 v 27 months; P = . 0001) and age at initial diagnosis (53.5 v 74 months; P = .0269). At a median follow-up time of 21.5 months, children positive for TEL-AML1 had a significantly (P = .0011) higher probability of event-free survival (EFS; 0.79 v 0.33). The predominant majority of patients had been treated for initial ALL according to German multicenter BFM (108 of 133) or Cooperative ALL study group (CoALL) (19 of 133) frontline protocols. The comparison of tested and not-tested (N = 262) patients showed no significant difference. TEL-AML1 positivity predicted a favorable short-term outcome; long-term results are unknown. Screening for TEL-AML1 should become routine at relapse diagnosis and might be used for therapy stratification in future trials.

Adolescent↗
PubMed1998

Teratomas in infancy and childhood.

BACKGROUND AND PROCEDURE: Outcomes in children with teratomas collected between October 1982 and December 1995 in cooperative protocols of the German Society of Pediatric Oncology and Hematology (GPOH) were analyzed. Teratomas were diagnosed in 329 (42%) of 780 registered patients with germ cell tumors. The annual incidence was 0.24/100,000. Main primary sites were coccygeal (n = 132, 2.2:1 female predominance), ovary (n = 81), testis (n = 40) and brain (n = 15, 2.8:1 male predominance). RESULTS: Two hundred seventy cases of extracranial non-testicular teratoma were evaluated: In mature teratomas (n = 154) the observed relapse rate was 10%. Incomplete resection was the main risk factor for relapse. After complete resection, the relapse-free survival (RFS, Kaplan-Meier-estimation) was 0.96 +/- 0.01 (n = 126, observation time 18-155 months) in comparison to an RFS of 0.56 +/- 0.09 in incompletely resected teratomas grade 0 (n = 28, observation time 28-94 months) (P < 0.01). Im-mature teratomas were treated by surgery alone in 76 cases and by surgery and adjuvant chemotherapy in 40 cases. The observed relapse risk was 18%. Main risk factors for relapse were incomplete tumor resection (n = 38) as well as immaturity in incompletely resected teratomas. Fifteen of 29 relapsing patients presented with malignant tissue in the recurrent tumor (mainly yolk sac tumor); in contrast, seven of 40 patients with immature teratoma relapsed despite adjuvant chemotherapy without showing malignant components (P = 0.014). Nine of 36 (25%) relapsing patients died of disease. Eleven of the 27 (41%) surviving children suffered from mutilation after repeated surgery. COMMENTS: It is suggested that an international randomized trial for patients with incompletely resected high risk teratoma be initiated to evaluate the effect of adjuvant chemotherapy on specific end-points: 1) influence on relapse rate in general; 2) reduction of the proportion of malignant relapses; 3) avoidance of mutilating surgery.

Adolescent↗
PubMed1998

Novel WT1 mutation, 11p LOH, and t(7;12) (p22;q22) chromosomal translocation identified in a Wilms' tumor case.

About 5-10% of sporadic Wilms' tumors (WT) are associated with mutations in the Wilms' tumor 1 gene (WT1). More than 90% of patients with Denys-Drash syndrome (DDS; characterized by renal nephropathy, gonadal anomaly, and predisposition to WT) show constitutional intragenic WT1 mutations. We describe a novel WT1 stop-mutation in exon 2. This heterozygous germline mutation was detected in a one-year-old girl who was bilaterally affected with Wilms' tumor but without any other clinical manifestations of DDS. The C-to-A transversion is predicted to result in a polypeptide comprising only the first 165 amino acids of the WT1 protein. Loss of heterozygosity (LOH) studies comparing tumor DNA with lymphocyte DNA revealed LOH for the entire short arm of chromosome 11 in tumor tissue. In addition to the chromosome 11 lesions, the tumor showed a seemingly balanced chromosomal translocation t(7;12) (p22;q22) as the only visible cytogenetic aberration.

Chromosomes, Human, Pair 11↗
PubMed1998

Immunophenotype, proliferation, DNA ploidy, and biological behavior of gastrointestinal stromal tumors: a multivariate clinicopathologic study.

To determine the prognostic impact of clinical, immunohistochemical, and biological parameters, we examined 52 gastrointestinal stromal tumors (GIST) by conventional light microscopy and immunohistochemistry. DNA ploidy was analyzed by image cytometry on cytospin preparation. The proliferative activity was determined by mitosis counting and assessment of Ki-67 reactivity by means of monoclonal antibody Ki-S5. A histopathologic grade was assigned to each tumor according to the French Federation of Cancer Centers (FNCLCC) grading system. Next to vimentin, CD34 was the most prevalent antigen, followed by markers of neural and muscular differentiation. Many tumors exhibited a mixed phenotype. Twenty-one tumors were diploid, eight hypodiploid, and 23 aneuploid. In univariate analysis, tumor grade, Ki-S5 labeling index, mitotic count, atypical mitoses, cellularity, and sex were predictive of both mortality and metastasis risk. DNA ploidy only correlated with overall survival, whereas the tumor location affected the occurrence of metastases. Multivariate analysis selected Ki-S5 scores (P < .0001) and atypical mitoses (P=.012) as independent prognosticators for overall survival, and tumor grade (P=.0036) and size (P=.0055) as predictors of metastatic spread. We conclude that GIST are primitive mesenchymal tumors capable of divergent differentiation, which does not influence their prognosis. The latter appears to be best predicted by histopathologic grading and the Ki-67 labeling index.

Adult↗
PubMed1998

Whole blood clot lysis in newborns and adults after adding different concentrations of recombinant tissue plasminogen activator (Rt-PA).

Optimal treatment of newborns with thromboembolic complications likely differs from that for adults because of ontogenetic features of both coagulation and fibrinolysis that affect the thrombotic processes and the response to thrombolytic agents. Although there are data on plasma clot lysis in newborns, the potential for dissolution of whole blood clots has not been explored. We investigated whether there is a difference between newborns and adults in sensitivity of whole blood clots to lysis with recombinant tissue plasminogen activator (rt-PA). Blood was obtained from 15 newborns and from 13 adults and anticoagulated with sodium citrate. Whole blood clots were generated by addition of thrombin and calcium. After 3 hours of retraction the clots were put into tubes containing 1.45 mL plasma of the same patient. After 1 hour of incubation, rt-PA was added to result in final concentrations of 3, 1, 0.3, 0.1, and 0 microg/mL. Clots were weighed after 0.5, 1, 2, and 3 hours. At any time point measured, whole blood clot lysis was more efficient in newborns than it was in adults. This was true for spontaneous clot lysis (p <0.05 at 1, 2, and 3 hours) as well as for all concentrations of rt-PA tested (p <0.05 at 1 hour). Whole blood clot lysis in new-borns was most efficient at 1 microg/mL rt-PA, whereas adults showed best lysis at the highest concentration tested (3 microg/mL rt-PA). The rate of plasminogen consumption was similar in newborns and adults. Recommendations for antithrombotic therapy in new-borns have been loosely extrapolated from recommendations for adults. Our data can be helpful in establishing guidelines for thrombolytic therapy in the neonatal period. Retracted whole blood clots mimic better the in vivo situation than previously reported in studies of lysis of nonretracted plasma clots. Based on our data, we think that despite low levels and slower activation kinetics of fetal plasminogen, the dosage of rt-PA should be lower in newborns than in adults.

Adult↗
PubMed1998

Giant cell collagenoma: a benign dermal tumor with distinctive multinucleate cells.

We present five cases of a hitherto unreported cutaneous neoplasm. The tumors appeared as solitary slow-growing flesh-colored nodules arising in young and middle-aged adults. They were located on the trunk, the upper extremities, and the face, and did not recur after complete excision. Clinically, they were diagnosed as dermal nevus, Spitz's nevus, fibroma, or neurofibroma. Histology revealed polypoid flat-dome-shaped lesions with a sharply demarcated matrix consisting of coarse hyalinized collagen bundles arranged in a prominent storiform pattern and separated by mucin-containing clefts. Despite a low overall cellularity, the tumors contained numerous, occasionally bizarre-shaped, multinucleate giant cells with crowded vesicular nuclei and a pale staining foamy cytoplasm, as well as plump fibroblastlike cells with analogous nuclear morphology. Atypical nuclei or mitotic figures were not observed. The cells were strongly positive for vimentin but negative for cytokeratin, smooth muscle actin, desmin, S-100 protein, CD34, factor XIIIa, and the macrophage markers KP1, Mac 387, and Ki-M1p, suggesting a fibroblastic origin. Based on the overall architecture, we conclude that these tumors probably represent a distinctive variant of solitary circumscribed storiform collagenoma (sclerotic fibroma) and propose the designation of giant cell collagenoma.

Adult↗
PubMed1998

Prognosis of children born to mothers with HELLP-syndrome.

In literature there have been differences in the assessment of the outcome of children born to mothers with HELLP syndrome. In a retrospective study we investigated six annual groups (1989-1994) at the Perinatal Center in Erlangen (11,235 births, 68 children of mothers with HELLP syndrome), 53 children were treated in our neonatal intensive care unit (NICU). The control group (n = 219) consisted of a complete age group in our NICU. The gestational age (mean 33 weeks, p < 0.003) and the birth weight (mean 1671 g, p < 0.001) were significantly lower in the HELLP group. No significant differences were detected with respect to the frequency of leucocytopenia (p = 0.518) and thrombocytopenia (p = 0.215). Despite a relatively high rate (37.7%) of RDS there was only a significant tendency to the disadvantage of HELLP children (p = 0.075). There was no difference in frequency of intracranial hemorrhage (ICH) (p = 0.566). Infections were diagnosed less frequently in HELLP children (p = 0.042). Mortality in the control group was higher only as a tendency (p = 0.07). The follow-up examinations of the neurological development covered 31 of the 53 treated children. After 6-72 months (median 24 months), 90.3% of these children showed normal development or only minor disabilities. The prognosis of children of mothers with HELLP syndrome is not as bad as has been assumed so far.

Female↗
PubMed1998

[Rhabdomyosarcoma and extraosseous Ewing's sarcoma].

Rhabdomyosarcomas (RMS) and extraosseous Ewing's sarcomas (EOE) including malignant peripheral neuroectodermal tumors (MPNT) are the most frequent soft tissue malignancies of childhood. They account for 60.2% of 2.350 cases collected in the files of the Kiel Pediatric Tumor Registry. RMS: It is absolutely necessary to distinguish between embryonal (e) and alveolar (a) RMS, since these are two distinct tumor entities with significant differences in clinical presentation, morphology, molecular biology, cytogenetics and prognosis (Botryoid and spindle cell RMS are special variants of eRMS). The overall proportion of eRMS: aRMS is 2.4:1. Most cases of eRMS develop in the first 10 years of life (77.3%) while the age distribution of aRMS is almost constant in childhood and adolescence. Embryonal RMS exhibit a significantly higher proportion of male patients than aRMS (m:f = 1.72:1 vs. 1.06:1). A higher percentage of aRMS cases (25%) shows metastatic disease at the time of diagnosis than eRMS (8%), and the overall survival rates of aRMS are significantly lower even in localised disease (stage I-III) than in eRMS (59% vs. 76%; p < 0.002) (Data from the Cooperative Soft Tissue Sarcoma Study CWS). EOE: Despite proven histogenetic relationship (identical chromosomal rearrangements and fusion genes) the members of the Ewing's sarcoma (ES) family, classic ES and MPNT, whether osseous or extraosseous display significant differences in location, morphology and prognosis. Morphologically, MPNT and classic ES can be considered to be the extremes of a spectrum with overt neurodifferentiation in the former and lack of neural differentiation in the latter. Matched-pairs analysis of CWS EOE and MPNT cases show dramatically more unfavorable overall survival rates in MPNT than in EOE (45% vs. 67%).

Adolescent↗
PubMed1997

Prognostic factors and staging systems in childhood hepatoblastoma.

The objectives of this study were to investigate clinical and patho-histological characteristics of childhood hepatoblastoma on their value as prognostic factors, and to evaluate the predictive impact of different staging systems for liver tumors on 72 patients treated in the German Pediatric Liver-Tumor Study HB89. Statistical analysis was performed by comparing patients' disease-free survival with characteristics and stages. Multivariate analysis was done by the Cox proportional-hazards model, the recursive partitioning and amalgamation model (RECPAM) and the model of clustering by response (CBR). The following characteristics were significantly related with prognosis: tumor involvement of one vs. both liver lobes, multifocal disseminated vs. unifocal growth pattern in the liver, distant metastases, vascular invasion, fetal vs. embryonal differentiation, and serum alpha-fetoprotein; patients with values of < or = 100 ng/ml or > or = 1,000,000 ng/ml had a worse outcome than those with 100 to 1,000,000 ng/ml. Multivariate analysis with the 3 models revealed that tumor-growth pattern, serum alpha-fetoprotein and, in the Cox and CBR models, vascular invasion also are independent prognostic factors, permitting the allocation of hepatoblastoma patients to 1 of 2 prognostic groups for differential therapy. Post-surgical staging and the conventional TNM system for liver carcinoma had a high predictive value, in contrast to a TNM system proposed by the UICC for testing on childhood liver tumors. We therefore propose that the TNM system for liver carcinoma be applied for comparison of treatment results in hepatoblastoma.

Child↗
PubMed1997

Correlation of germ-line mutations and two-hit inactivation of the WT1 gene with Wilms tumors of stromal-predominant histology.

The WT1 gene, located on chromosome 11p13, is mutated in a low number of Wilms tumors (WTs). Germ-line mutations in the WT1 gene are found in patients with bilateral WT and/or associated genital tract malformations (GU). We have identified 19 hemizygous WT1 gene mutations/deletions in 64 patient samples. The histology of the tumors with mutations was stromal-predominant in 13, triphasic in 3, blastemal-predominant in 1, and unknown in 2 cases. Thirteen of 21 patients with stromal-predominant tumors had WT1 mutations and 10 of these were present in the germ line. Of the patients with germ-line alterations, six had GU and a unilateral tumor, two had a bilateral tumor and normal GU tracts, and two had a unilateral tumor and normal GU. Three mutations were tumor-specific and were found in patients with unilateral tumors without GU. These data demonstrate a correlation of WT1 mutations with stromal-predominant histology, suggesting that a germ-line mutation in WT1 predisposes to the development of tumors with this histology. Twelve mutations are nonsense mutations resulting in truncations at different positions in the WT1 protein and only two are missense mutations. Of the stromal-predominant tumors, 67% showed loss of heterozygosity, and in one tumor a different somatic mutation in addition to the germ-line mutation was identified. These data show that in a large proportion of a histopathologically distinct subset of WTs the classical two-hit inactivation model, with loss of a functional WT1 protein, is the underlying cause of tumor development.

DNA-Binding Proteins↗
PubMed1997

Giant cell tumor of the larynx: case report and review of the literature.

BACKGROUND: Primary manifestation of a giant cell tumor (GCT) in the larynx is exceedingly rare. Until now, only 17 cases have been reported in the literature. Characterization of a GCT can be difficult partly because of similarities with the giant cell subtype of malignant fibrous histiocytoma (MFH). METHODS: The case of a 35-year-old man with a laryngeal GCT is presented. The patient underwent laryngectomy and has been free of recurrence since then (90 months). The literature is reviewed, and previously reported cases are presented in a table and compared with the presented case. The difficulties in the differential diagnosis are discussed. CONCLUSIONS: When a laryngeal GCT is diagnosed, further differential diagnostic considerations should follow. One of these considerations is the MFH, which displays considerably more cellular atypia than GCT. Because most cases of giant cell MFH are high-grade sarcomas, the distinction between GCT and MFH is necessary.

Adult↗