Pediatric rhabdomyosarcoma incidence and survival in the United States: An assessment of 5656 cases, 2001-2017.

BACKGROUND: While rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents, past epidemiology studies of this malignancy used data that covered <30% of the US population. Therefore, we evaluated RMS incidence using data from U.S. Cancer Statistics (USCS) and survival trends using the National Program of Cancer Registries (NPCR), which covers 100% and 94% of the U.S. population, respectively. METHODS: Incidence and survival were assessed for pediatric patients diagnosed with RMS during 2003-2017 and 2001-2016, respectively. Both demographic and clinical variables were evaluated. Age-adjusted incidence rates, average annual percent change (AAPC), and 5-year relative survival (RS) were calculated, all with corresponding 95% confidence intervals (CIs). Cox regression models were used to evaluate the impact of demographic and clinical variables on survival. RESULTS: We identified 5656 primary RMS cases in USCS during 2003-2017. The age-adjusted incidence rate was 4.58 per 1 million (95% CI: 4.46-4.70) with an AAPC of 0.3% (95% CI: -0.7 to 1.2%). In NPCR, 5-year RS for all cases was 68.0% (95% CI: 66.6-69.3%). In multivariable analyses, non-Hispanic (NH) Black cases had worse survival compared with NH White cases (hazard ratio [HR] = 1.16, 95% CI: 1.01-1.33). CONCLUSION: The incidence and survival rates were stable in the largest and most comprehensive population-based analysis for pediatric RMS cases in the U.S. Additionally, we observed a survival disparity among NH Black cases. Findings from this study could inform interventions to address disparities, risk stratification strategies, and clinical trial design.

Embryonal and Alveolar Rhabdomyosarcoma in Adults: Real-Life Data From a Tertiary Sarcoma Centre.

AIMS: Embryonal and alveolar rhabdomyosarcoma (ERMS, ARMS) are subtypes of RMS that mainly occur in children, with relatively good outcomes. The incidence in adults is extremely low and survival is significantly worse compared with children. Data are scarce and literature generally combines all RMS subtypes, including pleomorphic RMS, which primarily occurs in adults and behaves more like undifferentiated pleomorphic sarcoma. The aim of this study was to evaluate patient and tumour characteristics, outcome and prognostic factors in adult patients with ERMS and ARMS. MATERIALS AND METHODS: All adult (18 years or older) ERMS and ARMS patients (presenting 1990-2016) were identified from a prospectively maintained database and were included in this analysis. RESULTS: Overall, 66 patients were included (42 men, 24 women). The median age at presentation was 28 years (range 18-71). The median overall survival for all ARMS (n = 42) and ERMS (n = 24) patients was 18 months, with a 5-year overall survival rate of 27%. Patients presenting with localised disease (n = 38, 58%) and metastatic disease (n = 25, 42%), had a 5-year overall survival rate of 36% and 11%, respectively. In univariate analysis we found alveolar subtype, fusion gene positivity, infiltrative tumour and metastatic presentation to be negative prognostic factors. CONCLUSION: Survival in adult ERMS and ARMS patients is poor and the current data may be useful in the design of trials with novel agents. Ideally, paediatric and adult oncologists should set up trials together to get a better understanding of biological, genetic and clinically relevant factors in this disease.

A review of pediatric middle ear tumors and analysis of the demographics, management, and survival of pediatric rhabdomyosarcomas of the middle ear.

OBJECTIVE: To examine the types of pediatric middle ear tumors and review the demographics, management, and survival of pediatric patients with rhabdomyosarcoma (RMS) of the middle ear. METHODS: Pediatric patients in the Surveillance, Epidemiology, and End Results (SEER) database were included from 1973 to 2014 based on a diagnosis of middle ear tumors using the ICD O-3 code: C30.1: Middle ear primary site. Patients were included from ages 0-18 years. RESULTS: Forty pediatric middle ear tumor cases were identified. Twenty patients were female (50%). Twenty-seven (67.5%) cases were rhabdomyosarcomas (RMS). Pediatric RMS patients tended to be diagnosed in early childhood (mean age 5.30 years, standard deviation 2.9, range 1.00-13.00, 59.3% of patients were ages 5 or below). Most pediatric RMS patients received chemotherapy and radiation therapy as part of the treatment regimen (88.8%). Finally, the 5-year overall and disease-specific survival rates were 59% and 63% respectively. CONCLUSIONS: Pediatric middle ear tumors are rare. Females and male pediatric patients are both at risk for middle ear tumors. RMS is the most common malignant middle ear tumor affecting pediatric patients. Despite the use of multimodality therapies, survival rates for pediatric patients with RMS of the middle ear are low. Physicians may consider including middle ear tumors on the differential diagnosis for pediatric patients with symptoms presenting similarly to non-resolving otitis media.

Outcome of 449 adult patients with rhabdomyosarcoma: an observational ambispective nationwide study.

Five-year overall survival (OS) of localized RMS exceeds 70% in children (<18) but is very poor in adult patients. We analyzed the outcome and prognostic factors (PF) of a national series of adult patients with RMS in a large study. The study population consisted of two different cohorts: a retrospective cohort (157 adult patients treated in 13 reference centers between 05/1981 and 02/2010) and the prospective cohort (292 patients with RMS diagnosed and treated between 01/2010 and 12/2014 in France) included in the NetSarc database. A descriptive analysis of patients' characteristics and prognostic factors was conducted on both series which were compared. In the retrospective series, histological subtypes were embryonal (E-RMS) for 21% of patients, alveolar (A-RMS) for 35% of patients, and "adult-type" P-RMS (pleomorphic, spindle cell RMS, not otherwise specified) (P) for 44% patients. This distribution significantly differed in the prospective cohort: A-RMS: 18%; E-RMS: 17%; and P-RMS 65%. With a median follow-up of 8.5 years, 5-year OS for localized RMS and advanced RMS (with nodes and/or metastases) was 43% and 5%, respectively, (P < 0.0001), and median OS was 51, 33, and 16 months for E-RMS, A-RMS, and P-RMS, respectively, in the retrospective cohort. The median OS was less than 40 months for the prospective nationwide cohort for the entire population. In a multivariate analysis of the retrospective study, independent prognostic factors for OS were A-RMS, R0 resection, and adjuvant radiotherapy (RT). For localized RMS, age and use of pediatric chemotherapy (CT) regimen are independent prognostic factors. Adult patients with RMS have a poorer overall survival than pediatric patients, and survival varies considerably across histological subtypes.

Does fibroids surgery by endoscopy or laparotomy represent a malignancy threat?

BACKGROUND: The aim of this study was to 1) estimate the incidence of unsuspected uterine sarcoma in patients after myomectomies and hysterectomies for benign uterine fibroids; 2) to assess the incidence of uterine sarcomas among patient who had uterine fibroids or a suspicion of uterine sarcoma before undergoing the surgical procedures. METHODS: Retrospective study in single center university hospital. Patient's records with uterine fibroids and uterine sarcoma from 2003 to 2015 collected. Data presented as mean and standard deviation for continuous variable and percentage for categorical variables. The exact Clopper-Pearson interval was used to calculate 95% confidence interval. RESULTS: The total number of women with uterine fibroids was 2269 and uterine sarcomas 7. Of these seven, four were uterine leiomyosarcomas (ULMS), one endometrial sarcoma (ESS), one high-grade undifferentiated uterine sarcoma (HGUS) and one embryonal rhabdomyosarcoma (ERMS). Six of these patients underwent direct open conventional cancer treatment. The mean age of the patients with ULMS was 73.7±3.9 years. Immunohistochemistry results indicated SMA(+), CD 10(+), desmin(+) and h-caldesmon(+). The two patients with HGUS and ERMS were also preoperatively diagnosed with suspected malignancy and operated upon according. However, only one woman 48 years old primarily diagnosed with benign uterine fibroids underwent a laparoscopic subtotal hysterectomy (LSH) procedure; however, the histological results detected an ESS. Immunohistochemistry showed CD10(+), desmin(+), smooth muscle actin (SMA)(-), Aktin(-) and 5% Ki67(+).Two weeks after the initial surgery, an open cervical stump resection with bilateral sapping-oophorectomy and an omentectomy was performed. CONCLUSIONS: The frequency of unsuspected ESS was 1/2269 among the women who underwent myomectomies and hysterectomies for the treatment of benign uterine fibroids. The total incidence of uterine sarcoma was 7/2275 among the patients who had presumed uterine fibroids or a suspicion of uterine sarcoma prior to undergoing the surgical procedures.

Childhood rhabdomyosarcoma in Kano, Nigeria: a retrospective analysis of 52 cases.

BACKGROUND: Rhabdomyosarcoma is the most common soft tissue sarcoma in children ≤ 15 years of age. There is, however, a paucity of reports on the pattern of its occurrence in Nigeria and other parts of Africa. OBJECTIVE: The aim of this review is to highlight the age and sex distribution, anatomical location and morphological characteristics of rhabdomyosarcoma among children in Kano, northwestern Nigeria. PATIENTS AND METHOD: This is a 14 year retrospective study of all cases of rhabdomyosarcomas occurring in children diagnosed at the Pathology Department of Aminu Kano Teaching Hospital, Kano, Nigeria. Clinical data obtained included sex, age, tumour site and histologic subtype. RESULTS: Fifty two children were seen with a male to female ratio of 1 : 1.3. The ages of these Patients ranged from 4 months to 15 years with a mean (SD) age of 6.99 (4.1) years and peak age frequency in the 0 to 5 years age group. The embryonal variant was the most common histological subtype (69%). The others were alveolar (27%) and pleomorphic (4%) variants. The head and neck was the most affected region (44%) and the most common primary site was the orbit (19.2%). CONCLUSION: Rhabdomyosarcoma is the most common childhood soft tissue sarcoma in Kano. It occurs in females more frequently than males and the most common histologic subtype is embryonal rhabdomyosarcoma affecting predominantly the orbit.

Malignant tumors of the upper aerodigestive tract as seen in a Nigerian tertiary health institution.

BACKGROUND: Cancers of the upper aerodigestive tract constitute a diverse heterogeneous group of malignant neoplasms with unique epidemiological, pathological, and treatment considerations. Only few studies have been conducted so far on these tumors in Nigeria. This study aims to study in greater detail, the pathological features of these cancers in Nigerian patients. MATERIALS AND METHODS: The surgical specimens of patients diagnosed with malignant tumors of the upper aerodigestive tracts in the Department of Morbid Anatomy and Forensic Medicine, Obafemi Awolowo University Teaching Hospital Complex (OAUTHC) in Ile-Ife, Nigeria, over a 10-year period, formed the basis of this study. Analysis was done for differences in proportion using the Chi-square test (P is significant at < 0.05) by SPSS version 15. RESULTS: There were a total of 62 cases. The overall mean age was 50.7 years, while the age range was from 3 years to 90 years. The male to female ratio was 3.1:1. A majority of the patients (67.7%) were older than 40 years. About 30.6, 27.4, and 16.1% of cases occurred in the larynx, nasopharynx, and nasal cavity, respectively, while 93.5% of the tumors were carcinomas. Squamous cell carcinoma was the most common histological variety. Nonepithelial tumors were not seen below the age of 20 years. CONCLUSION: This study shows that malignant upper aerodigestive tract tumors seen in our environment are mainly diseases of adulthood that tend to occur about seven to nine years earlier than in other populations. Squamous cell carcinoma is the predominant histological variety. Although the larynx is the most frequent anatomic site, the nasopharynx and nasal cavity are more commonly affected than the oral cavity unlike in other populations. Nonepithelial tumors are extremely rare below the age of 20 years.

Cytogenetic and single nucleotide polymorphism array findings in soft tissue tumors in infants.

Soft tissue tumors in children under one year of age (infants) are rare. The etiology is usually unknown, with external factors or congenital birth defects and hereditary syndromes being recognized in only a small proportion of the cases. We ascertained the cytogenetic findings in 16 infants from whom tumor tissue had been obtained during a 25-year period. In eight of them, single nucleotide polymorphism (SNP) array analyses could also be performed. No constitutional chromosome aberrations were detected, and assessment of clinical files did not reveal any congenital or later anatomical defects. Three tumors--one infantile fibrosarcoma, one embryonal rhabdomyosarcoma, and one angiomatoid fibrous histiocytoma (AFH)--had abnormal karyotypes. As the AFH had an exchange between chromosome arms 12p and 15q, additional fluorescence in situ hybridization and reverse transcription-polymerase chain reaction analyses were performed, unexpectedly revealing an ETV6/NTRK3 fusion. Three of the eight tumors, including the AFH with an abnormal karyotype, analyzed by SNP array showed aberrations (loss of heterozygosity or imbalances). The present series suggests that the addition of array-based technologies is valuable for detecting underlying pathogenetic mechanisms.

Adult rhabdomyosarcoma survival improved with treatment on multimodality protocols.

PURPOSE: Rhabdomyosarcoma (RMS) is a pediatric sarcoma rarely occurring in adults. For unknown reasons, adults with RMS have worse outcomes than do children. METHODS AND MATERIALS: We analyzed data from all patients who presented to Memorial Sloan-Kettering Cancer Center between 1990 and 2011 with RMS diagnosed at age 16 or older. One hundred forty-eight patients met the study criteria. Ten were excluded for lack of adequate data. RESULTS: The median age was 28 years. The histologic diagnoses were as follows: embryonal 54%, alveolar 33%, pleomorphic 12%, and not otherwise specified 2%. The tumor site was unfavorable in 67% of patients. Thirty-three patients (24%) were at low risk, 61 (44%) at intermediate risk, and 44 (32%) at high risk. Forty-six percent were treated on or according to a prospective RMS protocol. The 5-year rate of overall survival (OS) was 45% for patients with nonmetastatic disease. The failure rates at 5 years for patients with nonmetastatic disease were 34% for local failure and 42% for distant failure. Among patients with nonmetastatic disease (n=94), significant factors associated with OS were histologic diagnosis, site, risk group, age, and protocol treatment. On multivariate analysis, risk group and protocol treatment were significant after adjustment for age. The 5-year OS was 54% for protocol patients versus 36% for nonprotocol patients. CONCLUSIONS: Survival in adult patients with nonmetastatic disease was significantly improved for those treated on RMS protocols, most of which are now open to adults.

Head and neck rhabdomyosarcoma: a critical analysis of population-based incidence and survival data.

OBJECTIVE: To evaluate trends in incidence, survival, and treatment of rhabdomyosarcoma (RMS) of the head and neck. STUDY DESIGN: Retrospective review of a national database. SETTING: Tertiary medical center. SUBJECT AND METHODS: Incidence and survival trends were examined for head and neck RMS diagnosed between 1973 and 2007 using the Surveillance, Epidemiology, and End Results Program. Frequencies, incidence rates, and relative survival curves were calculated for various RMS subtypes and primary sites. RESULTS: Between 1973 and 2007, the incidence of RMS of the head and neck increased significantly, with an annual percentage change of 1.16%. Relative 5-year survival was statistically unchanged during the study period at 62.8% ± 2.3%. When analyzed by univariate analysis, overall survival was found to be dependent on sex, age, primary site, extent of disease, and histology. When evaluated by stage, most orbital tumors (60.6%) presented with localized disease, while most parameningeal tumors presented with either regional (53.2%) or distant (28.1%) spread. Multivariate analysis found that age less than 10 years at diagnosis and tumors with localized or regional spread were associated with improved overall survival. Relative survival was found to be largely dependent on extent of disease rather than primary site. CONCLUSIONS: Despite reported advances in overall and disease-free survival for patients with RMS, population-based analysis shows no substantial improvement during the past 30+ years. The prognosis of these patients is largely dependent on extent of disease at diagnosis.

Childhood rhabdomyosarcoma: a review of 35 cases and literature.

UNLABELLED: BCKGROUND: Rhabdomyosarcoma is one of the most frequent soft tissue sarcomas in children. It constitutes a unique group of soft tissue sarcomas found in children, primarily infants, toddlers and preschool pupils. OBJECTIVE: The purpose of this review is to examine all the cases of histologically confirmed childhood rhabdomyosarcomas; to re-examine the review pattern, and review recent advances in the biology of this neoplasm. MATERIALS AND METHODS: the sample consisted of 35 histologically confirmed rhabdomyosarcomas between January, 1996 and 2005. The specimens consisted of excision, incision and tru-cut biopsies. Fresh sections were cut from paraffin bedded tissue blocks and stained with haematoxylin and eosin. The slides were reviewed by three pathologists. The sites of biopsies were noted from the referral forms. RESULTS: Thirty five cases of paediatric rhabdomyosarcomas were recorded out of a total of 55 cases during the period of study. This represents 63.6% of all cases of rhabdomyosarcomas. The commonest anatomical sites were head and neck regions which accounted for 21 cases of the 35, while retroperitoneal, vagina and testis accounted for 8, 3 and 3 respectively. The highest proportion of cases occurred in ages 6-15 years. The embryonal subtype accounted for highest proportion, followed by alveolar, pleomorphic and spindle types respectively. CONCLUSION: This study shows that childhood rhabdomyosarcomas are prevalent in this region, and that the most prevalent of the variants is embryonal rhabdomyosarcoma. The commonest anatomical sites were head and neck regions and the highest proportion of cases occurred in ages 6-15 years.

Birth characteristics and the risk of childhood rhabdomyosarcoma based on histological subtype.

BACKGROUND: Little is known about risk factors for childhood rhabdomyosarcoma (RMS) and the histology-specific details are rare. METHODS: Case-control studies formed by linking cancer and birth registries of California, Minnesota, New York, Texas and Washington, which included 583 RMS cases (363 embryonal and 85 alveolar RMS) and 57 966 randomly selected control subjects, were analysed using logistic regression. The associations of RMS (overall, and based on embryonal or alveolar histology) with birth weight across five 500 g categories (from 2000 to 4500 g) were examined using normal birth weight (2500-3999 g) as a reference. Large (>90th percentile) and small (<10th percentile) size for gestational age were calculated based on birth weight distributions in controls and were similarly examined. RESULTS: High birth weight increased the risk of embryonal RMS and RMS overall. Each 500 g increase in birth weight increased the risk of embryonal RMS (odds ratio (OR)=1.27, 95% confidence interval (CI)=1.14-1.42) and RMS overall (OR=1.18, 95% CI=1.09-1.29). Large size for gestational age also significantly increased the risk of embryonal RMS (OR=1.42, 95% CI=1.03-1.96). CONCLUSIONS: These data suggest a positive association between accelerated in utero growth and embryonal RMS, but not alveolar RMS. These results warrant cautious interpretation owing to the small number of alveolar RMS cases.

Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975-2005.

BACKGROUND: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents aged<20 years; its etiology remains largely unknown. It is believed that embryonal (ERMS) and alveolar rhabdomyosarcoma (ARMS), the most common subtypes, arise through distinct biologic mechanisms. The authors of this report evaluated incidence and survival trends by RMS demographic subgroups to inform future etiologic hypotheses. METHODS: Incidence and survival trends in RMS among children and adolescents aged<20 years were analyzed using data from the Surveillance, Epidemiology, and End Results Program. Frequencies, age-adjusted incidence and survival rates, and joinpoint regression results, including annual percentage change (APC) and 95% confidence interval (CI), were calculated. RESULTS: Between 1975 and 2005, the incidence of ERMS was stable, whereas a significant increase in the incidence of ARMS was observed (APC, 4.20%; 95% CI, 2.60%-5.82%). This trend may have been attributable in part to shifts in diagnosis, because a significant negative trend in RMS, not otherwise specified was observed concurrently. A bimodal age peak for ERMS was observed, with the second, smaller peak in adolescence noted for males only; ARMS incidence did not vary by age or sex. Five-year survival rates for RMS and ERMS increased during the period from 1976 to 1980 (52.7% and 60.9%, respectively) to the period from 1996 to 2000 (61.8% and 73.4%, respectively), whereas there was little improvement for ARMS (40.1% and 47.8%, respectively). CONCLUSIONS: Observed differences in incidence and survival for 2 major RMS subtypes across sex and age subgroups further supported the hypothesis that there are unique underlying etiologies for these tumors. Exploration of these differences presents an opportunity to increase current knowledge of RMS.

Childhood rhabdomyosarcoma in Ibadan, Nigeria: 1984-2003.

BACKGROUND: Rhabdomyosarcoma is the most common soft tissue sarcoma in children under 15 years of age. There is, however, a paucity of reports on the pattern of its occurrence in Nigeria and other parts of Africa. AIM: To describe the pattern of childhood rhabdomyosarcoma in Ibadan with respect to demography, morphology and tumour site. METHODS: This retrospective study was based on data obtained from the Ibadan cancer registry, College of Medicine, University of Ibadan. All histologically confirmed cases of rhabdomyosarcoma in children under 15 years of age seen at the University College Hospital Ibadan between 1984 and 2003 were included. Information obtained includes age, gender, morphology and sites of the tumours. RESULTS: Ninety-one children were seen with a male:female ratio of 1.5:1. Ages ranged from <1 to 14 years with a mean (SD) of 6.2 (4.1) years. The embryonal variant was the most common histological subtype (61.5%). Other subtypes were alveolar (13.2%), pleomorphic (4.4%) and rhabdomyosarcoma 'not otherwise specified' (20.9%). The majority (50.6%) of tumours were in the head and neck region and the common primary sites were soft tissue of the head, face (24.2%) and orbit (14.3%). Other sites included soft tissue of the pelvis (11.0%), genito-urinary tract (9.9%) and abdomen (9.9%). CONCLUSION: The pattern of rhabdomyosarcoma in Nigeria is similar to that in the United States and Europe, except for the rarity of parameningeal sites and extremities. There is a need for larger descriptive studies on childhood rhabdomyosarcoma in Africa.

Juvenile rhabdomyosarcomas in Port Harcourt, Nigeria: A twelve year review.

BACKGROUND: Juvenile rhabdomyosarcoma (JRMS) though rare, is the most common soft tissue malignancy of childhood that exhibits bimodal age distribution pattern. OBJECTIVE: Review the pattern of JRMS in Port Harcourt based on sex, age, tumor sites and histologic types. DESIGN: A retrospective descriptive study. SETTING: The study was conducted in the University of Port Harcourt Teaching Hospital (UPTH), Port Harcourt, Nigeria over 12 years. METHODOLOGY: We studied 21 juvenile rhabdomyosarcomas during the period under review. The hematoxylin and eosin stained histological slides were retrieved and reviewed to confirm previous diagnosis and histologically typed for the study. The sex, age and tumor sites were retrieved from the histology consultation forms, RESULT: These tumors were diagnosed from patients aged 0 - 20 years with the highest frequencies (38.1% each) occurring at 0 - 5 and 16 - 20 years age group. Males are more affected with a ratio 3.2:1. The trunk is the most common site of occurrence (47.7%) of which the genitourinary system is the most affected (23.8%) in this study. The most common histologic type is the embryonal rhabdomyosarcoma (71.5%). Alveolar rhabdomyosarcoma accounted for (19%) and the sarcoma botryoides (9.5%). CONCLUSION: The age of presentation and anatomic sites of the tumor are important in the diagnosis of these tumors. If a tumor histologically shows as small round blue cells, rhabdomyosarcoma should be considered as a differential diagnosis.

Orbital embryonal rhabdomyosarcoma in Karachi (1998-2002).

OBJECTIVE: To study the epidemiology of ocular Rhabdomyosarcoma (ORMS) in Karachi. METHODS: Incident ORMS cases resident of Karachi, registered at Karachi Cancer Registry (KCR) during 1st January 1998 to 31st December 2002 were included in the study. The data were classified using ICD-O2; computerized with Canreg-3, and analyzed using SPSS 10.0. RESULTS: Ten cases of ORMS were reported to KCR during 1998-2002. RMS originated in the orbit in eight cases, conjunctiva in one and eyelid in one. Nine cases presented with proptosis, associated with conjunctivitis in four cases. One case presented with eyelid swelling. The crude annual incidence rate was 0.13/100,000, the age standardized rate was 0.3/100,000. The mean age of childhood cases was 10.4 years (95% CI 4.0; 16.7); and adult cases was 24.8 years (95% CI 12.8; 36.7). At presentation, eight patients were older than 10 years and three were older than 20 years. Five cases were categorized as childhood malignancies. Tumors were a TNM stage III disease at presentation in eight cases; survival at the end of one year was 70%, and at the end of two years 20%. There were no survivors at the end of three years. CONCLUSION: ORMS in Karachi is a disease with a dismal survival. It may reflect a late presentation, or shorter adult ORMS survival or a manifestation of a different genetic pattern, associated with rapid evolution and poor prognosis. Health education for the population, especially parents and health providers is essential for early ORMS diagnosis. Pediatricians, ophthalmologists and health professionals, can play a vital role. Healthcare planning should focus on capacity building for ophthalmologic screening. Cytogenetic studies are advised to determine the genetic pattern.

[Soft tissue malignancies in childhood and adolescence. Pathology and clinical relevance based on data from the kiel pediatric tumor registry]. / Weichteilmalignome bei Kindern und Jugendlichen -- Pathologische Anatomie und ihre klinische Relevanz. Mit Daten aus dem Kieler Kindertumorregister.

Soft tissue malignancies in childhood and adolescence encompass a wide variety of histologically and genetically different tumor entities. In the files of the Kiel Pediatric Tumor Registry, 4,272 soft tissue malignancies were collected since 1977. Rhabdomyosarcomas are by far the most frequent sarcomas (44.6 % of the cases), followed in decreasing order of frequency by the family of Ewing tumors (peripheral primitive neuroectodermal tumors and extraosseous Ewing's sarcomas; altogether 22.3 %), malignant peripheral nerve sheath tumors (8.1 %), synovial sarcomas (5.0 %), leiomyosarcomas (3.2 %), fibrosarcomas (2.4 %), extrarenal malignant rhabdoid tumors (2.0 %), and alveolar soft tissue sarcomas (1.1 %). A further group (11.3 %) includes rare tumors, intermediate fibrohistiocytic tumors, and unclassified sarcomas. Embryonal rhabdomyosarcomas are 2.5 times more frequent than the alveolar rhabdomyosarcomas, which are prognostically unfavorable and located predominantly in the extremities and the trunk. With regard to clinical findings, histology, molecular biology and prognosis, embryonal and alveolar rhabdomyosarcomas have to be considered as two different tumor types. The family of Ewing tumors includes extraosseous Ewing's sarcoma and peripheral primitive neuroectodermal tumors (synonym: malignant peripheral neuroectodermal tumors), the former tumors without and the latter with neural differentiation. Many cases of infantile malignant peripheral nerve sheath tumors and infantile fibrosarcomas are low-grade malignancies and prognostically more favorable than their "adult" counterparts.

Treatment strategies for perianal rhabdomyosarcoma: report of two cases.

Intensive chemotherapy and high-dose radiation with complete excision of the tumor are the treatment of choice for rhabdomyosarcoma (RMS). However, because there are so few reports of perianal RMS, a mainstream treatment has not been established. We report two cases of perianal RMS and review 13 cases documented in Japan since 1990. Case 1 was a 7-year-old boy first treated by tumor excision followed by chemotherapy and external irradiation. He has since been tumor-free but suffered severe perianal erosion and ulceration. Case 2 was a 17-year-old girl first treated by preoperative chemotherapy to reduce the tumor size, after which the tumor was completely excised with anatomical guidance using three-dimensional computed tomography via the posterior sagittal approach, followed by open intraoperative irradiation. She has since been tumor-free with preserved anal function. These case reports show the importance of cogitated treatments for preservation of anal function and optimal therapeutic outcome in patients with perianal RMS.

Paediatric rhabdomyosarcoma of the ear and temporal bone.

The objective of the present study was to review the presentation, management, outcome and morbidity of paediatric patients presenting to a single centre with rhabdomyosarcoma of the ear and temporal region. All patients diagnosed with rhabdomyosarcoma of the ear and temporal region between 1980 and 2000 were entered into this retrospective study. Fourteen patients were identified. The median age at presentation was 4.5 years with a mean time of onset of symptoms to diagnosis of 21 weeks. In many patients, the presentation mimicked that of chronic otitis media, delaying diagnosis. Histological subtype was embryonal in 13 patients and alveolar in 1. All patients underwent multimodality treatment. The 5-year disease-free survival rate was 81%. Regional post-treatment morbidity included chronic aural discharge (6/14), facial palsy (8/14), growth disturbance (4/14) and maxillo-facial deformity occurring in four children. From the results, we conclude that these patients should usually present to an ENT surgeon who should keep the diagnosis in mind when dealing with children with chronic otitis media as early diagnosis with referral to a specialist multidisciplinary team will optimize the chance of survival. Discharge, hearing loss and aural polyp, although commonly because of chronic otitis media, should prompt urgent investigation and biopsy, particularly if associated with facial palsy, lymphadenopathy or an obvious mass.

Adult embryonal rhabdomyosarcoma in axilla.

A case of embryonal rhabdomyosarcoma (RMS) arising from an axillary space is described. RMS is one of the most common soft-tissue neoplasms in children, but adult embryonal RMS is rare. The patient was a 55-year-old man with a tumor of the left axillary area. RMS arising from an axillary area is also uncommon. The histological diagnosis was embryonal RMS. He received irradiation combined with chemotherapy (VAC: VCR, act-D, CPA) for the primary and metastatic lesions, obtained complete remission, and has remained disease-free for over four years as of this report.