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At least 1,207 records · Page 67Linked to original sources
PubMed1977

[Ultrastructural study on formal pathogenesis of experimentally induced rhabdomyosarcomas (author's transl)].

UNLABELLED: During oncogenesis the rhabdomyosarcomas pass different stages of differentiation. In experimentally induced rhabdomyosarcomas the chronological sequence of cytoplasmatic changes can be analysed. MATERIAL AND METHODS: 10 mg 9,10-dimethyl-1,2-benz-anthracene were injected subcutaneously or intramuscularly in the hind-paw of Wistar rats with 100 gms weight. 30, 60, 105, and 150 days later the animals were killed and specimens withdrawn. The soft-tissue was prepared for light- and electron-microscopy. RESULTS: In the soft-tissue tumors, experimentally produced with DMBA, 4 different cell types can be observed, which--depending on exposition time--dominate in the histological picture: (1) The myofibrillar cells. These cells are similar to rhabdomyocytes and contain numerous cytoplasmatic fibrillae with sarcomere-like structure. (2) The myofilamentous cells. These cells contain the fibrillar structure, but without sarcomere-like arrangement. (3) The undifferentiated sarcoma cells. These cells show a pathologically changed ergastoplasm and chondrioma. (4) The matured fibrosarcoma cells are nearly similar to fibrocytes. Under the cytoplasmatic changes especially the hyperplasia of the chondrioma can be observed, in which dystrophic megamitochondriae are found. These possibly reflect the abnormal glycolysis in cancer tissue. Also mitochondrial antibodies are discussed at the form pathogenesis of this change in organelles. Regarding the change of the endoplasmatic reticulu, the annulatae lamellae can be noticed which are typical for tumor cells. Furthermore megacisterns are found, in which protein-containing material is condensed as a consequence of a disturbed synthesis and secretion.

Animals↗
PubMed1997

[Report on a case of adult rhabdomyosarcoma subjected to tumorectomy and lobectomy].

Rhabdomyosarcoma (RMS) rarely occurs in adult and intra thoracic activity. This is a report on a case we experienced of adult RMS, which was subjected to tumorectomy and lobectomy. The patient was a 23 years old male, who first consulted a hospital in his neighborhood due to anterior chest pain suffered since May 1995. Since the examination showed an abnormal shadow in right anterior mediastinum, the patient was referred to our department. By biopsy under CT, the patient was diagnosed as having embryonal type of RMS and underwent surgery. The outcome was good and the patient is currently in the course of chemotherapy using IRS (Inter-group Rhabdomyosarcoma study)-III.

Adult↗
PubMed1997

[Rhabdomyosarcoma of the orbit].

The authors evaluated 9 years' experience with the diagnosis and treatment of embryonic rhabdomyosarcoma of the orbit in children. They evaluated in detail a group of 5 children treated and followed up for 1-9 years. Due to comprehensive surgical, radiation and chemotherapeutic treatment all patients survive and in 80% the visual function is preserved. For differential diagnostic problems, with regard to the variable manifestation of rhabdomyosarcoma which may imitate orbitocellulitis, chalaseon, epibulbar lipodermoid or papilloma, the authors emphasize the importance of rapid primary diagnosis by NMR and biopsy. In the treatment they appreciate greatly a combination of radiotherapy and chemotherapy which is a modern trend and can eradicate the tumour without radical surgery.

Child↗
PubMedDate not supplied

Adult embryonal rhabdomyosarcoma showing marked response to radiotherapy: a case report.

A case of embryonal rhabdomyosarcoma (RMS) arising from adult lower proximal extremity is described. Rhabdomyosarcoma (RMS) is most common among children, but adult embryonal RMS is rare. The patient was a 44-year-old man with a large tumor of the left extremity invading to the pelvis. The histological diagnosis was embryonal RMS. Radiation therapy was delivered a total dose of 50 Gy to the tumor. Although adult RMS, usually pleomorphic type, is considered to be radioresistant, the tumor showed marked response to radiotherapy and local control was achieved easily in this case.

Adult↗
PubMed1997

Family with Graves disease, multinodular goiter, nonmedullary thyroid carcinoma, and alveolar rhabdomyosarcoma.

Benign thyroid disease is a risk factor for nonmedullary thyroid carcinoma [Houlston and Stratton: Q J Med 88:685-693, 1995]. We report on a family with 7 members with benign and/or malignant thyroid neoplasia; one affected female died of a paravertebral alveolar rhabdomyosarcoma at age 20. The occurrence of thyroid nodular hyperplasia, nonmedullary thyroid cancer, and rhabdomyosarcoma in the same family may be due to chance, common environmental factors, or, most likely, genetic predisposition.

Adolescent↗
PubMed1997

[Clinical diagnosis of orbital rhabdomyosarcoma in a child].

The authors present data on problems associated with the diagnosis of orbital rhabdomyosarcoma in children. They analyze a ten-year period during which they verified in a department of child ophthalmology the disease in four children. The presence of the tumour is one per 60,000 out-patient examinations and during the follow-up period it had a seven times rarer incidence than retinoblastoma. The authors emphasize the varied clinical picture of the tumour and its differential diagnostic pitfalls and the possibility of mistaking temporarily the tumour for inflammatory or post-traumatic orbital changes. The authors remind of the necessity to consider the possible presence of rhabdomyosarcoma in all rapidly growing tumourous orbital processes, in particular in young children.

Child↗
PubMed1976

Rhabdomyosarcoma of the urinary bladder with intraepithelial spread in an adult.

Rhabdomyosarcoma of the urinary bladder in adults is exceedingly rare. Reported cases have been of the embryonal botryoid type, as seen in children. We will describe a case of pleomorphic rhabdomyosarcoma occurring in the urinary bladder of an adult. In this case, we noted striking intraepidermal migration of tumor cells, resembling the epidermotropic growth characteristic of tumor cells in Paget disease of the breast. The pathological features of this apparently unique case form the subject of this report.

Aged↗
PubMed1997

[Treatment results for rhabdomyosarcoma in children].

The results of treatment in 45 children with rhabdomyosarcoma was presented. Two-years survival was achieved in 63% and five-year in 45%. The importance of location and group of clinical progression discussed was importance prognostic factors in rhabdomyosarcoma.

Adolescent↗
PubMed1998

[Paratesticular rhabdomyosarcoma. A case report].

The authors report a case of paratesticular rhabdomyosarcoma in a 21-year-old patient. The clinical features were dominated by left loin pain, inguinoscrotal pain and scrotal swelling. Transillumination was negative. Laboratory markers (alpha-foetoprotein, beta HCG) were normal. Testicular ultrasound showed an enlarged testis, comprising heterogeneous nodules with infiltration of the spermatic cord. Abdominal ultrasonography and CT scan showed a latero-aortic retroperitoneal lymph node mass and a large left kidney. Chest x-ray was normal. Treatment consisted of radical orchidectomy. Histological examination of the operative specimen confirmed the diagnosis of paratesticular rhabdomyosarcoma. The patient was treated by chemotherapy and subsequently developed recurrence with metastases.

Adult↗
PubMedDate not supplied

Novel anticancer function of inositol hexaphosphate: inhibition of human rhabdomyosarcoma in vitro and in vivo.

Inositol hexaphosphate (IP6) is a naturally occurring polyphosphorylated carbohydrate that has been shown to suppress the growth of epithelial cancers, including those of breast and colon. The objective of this study was to investigate whether IP6 inhibits growth of rhabdomyosarcoma (RMS), a tumor of mesenchymal origin, which is the most common soft tissue sarcoma in children. We performed both in vitro and in vivo studies to evaluate the effect of IP6 on human RD cells growth. Our results show that IP6 suppresses growth of rhabdomyosarcoma cell line (RD) in vitro in a dose-dependent fashion. A 50% inhibition of cell growth (IC50) was induced by < 1.0 mM IP6. However, the removal of IP6 from the media, after 72 hours of treatment, allowed cells to recover their logarithmic growth. Exposure of RD cells to IP6 led to differentiation; cells became larger with abundant cytoplasm, expressing higher levels of muscle-specific actin. Consistent with in vitro observation, IP6 suppressed RD cell growth in vivo, in a xenografted nude mice model. When compared to controls, IP6-treated mice produced a 25 fold smaller tumors (p = 0.008), as observed after a two weeks treatment. In a second experiment, wherein the treatment period was extended to five weeks, a 49 fold (p = 0.001) reduction in tumor size was observed in mice treated with IP6. Histologically no evidence of tumor cell necrosis was observed. These data suggest a potential usefulness of this cytostatic, and non-cytotoxic, compound in novel therapeutic strategies for these types of tumor.

Animals↗
PubMed1998

[Leiomyosarcoma and rhabdomyosarcoma: rare tumors of the kidney].

Renal sarcoma represents 2.8% of all renal malignant tumours. The most common kind is leiomyosarcoma while rhabdomyosarcoma is the most unusual. Clinical presentation and diagnosis of both types of tumours are unspecific. Treatment is controversial and the overall prognosis poor. The present paper presents two cases, one leiomyosarcoma and one rhabdomyosarcoma which were treated surgically and were followed by chemo- and radiotherapy. A revision of the criteria for grading these as primary tumours of the kidney is also included.

Adult↗
PubMed1998

[Rhabdomyosarcoma of the prostate in childhood: a case report].

A case of prostatic rhabdomyosarcoma in an 8-year-old boy is presented. He was referred to Kurobe City Hospital with chief complaints of urinary retention and fever. Radiologic examinations revealed a huge prostatic tumor. Prostatic needle biopsy was performed and the pathological diagnosis was embryonal rhabdomyosarcoma of the prostate. He was referred to our hospital and treated with chemotherapy consisting of cisplatin, vincristine, cyclophosphamide, adriamycin, actinomycin-D and radiotherapy based on the regimen of IRS III. Total prostatectomy was performed 6 months after the start of therapy. Viable tumor cells were found in the prostate and the left obturator lymph nodes. After the operation, we continued chemotherapy. No recurrence was observed 8 months after the operation. However, local recurrence occurred in the pelvis 10 months after the operation and he died 2 months after the recurrence.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1998

Rhabdomyosarcoma in children--a ten year review.

PURPOSE: The purpose of this study is to retrospectively analyze all pediatric patients with Rhabdomyosarcoma (RMS) of various anatomic sites, treated in our department over a 10-year period, for treatment results. Anatomical site, group, and gender are individually analyzed as prognostic indicators of overall survival. MATERIALS AND METHODS: Sixteen rhabdomyosarcoma patients diagnosed by biopsy or surgical resection were reviewed. All patients were treated according to assigned IRS protocols except one. Age ranged from 1 to 19 years with a median age of 4 years. Ten patients were male and 6 were female, 14 were white and 2 black. Anatomic sites included six from the head and neck region, seven in the trunk and three in the extremities. Embryonal RMS was present in all but one which was classified as undifferentiated. All patients had surgery (biopsy-5, partial-1 or complete resection-10) and chemotherapy (VA, VAC, VAC plus Adriamycin, or VAC plus Adriamycin, CIS Platinum and VP-16). Ten patients received irradiation consisting of 3060 cGy to 5850 cGy using shrinking fields with 1.8 to 2.0 Gy/day/5 day/wk. RESULTS: Patients tolerated the treatment well and there were no late complications. Only one patient had a recurrence in the primary site with a median follow-up of 61 months (range 5-118 months) for the whole group. The 5-year disease free survival and actuarial survival for all patients treated were 73% and 87% respectively, with four patients developing metastasis and three of those dead of disease. CONCLUSION: This study represents a heterogeneous group of patients with RMS treated over a 10-year-period. The results correlate with those found in the most recent published IRS data for embryonal histology. From experience gained from earlier studies, newer IRS protocols have tailored treatment protocols to specific site with more intense therapy used for difficult treatment sites.

Adolescent↗
PubMed1976

[A propos a case of alveolar rhabdomyosarcoma (author's transl)].

This is a case of alveolar rhabdomyosarcoma with a rare clinical evolution. A first metastasis causes paraplegia; a second causes obstructive jaundice; a third subcutaneous metastasis is resected; the primitive tumor is discovered accidentally in the right calf, 8 weeks after the beginning of the disease. The literature is reviewed. Diagnosis of the alveolar rhabdomyosarcoma is often difficult because of confusion with a lymphoma, another type of sarcoma, a melanoma or even an epithelial tumor. The surgeon who removes a superficial node, obviously malignant, in a young subject, should think of this type of tumor. A multidisciplinary treatment associating radical resection, radio- and chemotherapy improves the very poor prognosis of this sarcoma.

Adult↗
PubMed1990

Soft tissue sarcoma of the perineal region in childhood. A report from the Intergroup Rhabdomyosarcoma Studies I and II, 1972 through 1984.

Thirty-six previously untreated patients younger than 21 years of age with sarcoma arising in the perineal region were entered on the Intergroup Rhabdomyosarcoma Studies (IRS) I and II from 1972 through 1984. The tumor histologic subtype was alveolar rhabdomyosarcoma (RMS) in 20 patients (56%), embryonal RMS in 11 patients (30%), and other types of sarcoma in 5 patients (14%). Fifteen children had grossly complete surgical excision (Clinical Groups I and II), and 15 had localized gross residual tumor (Group III) after initial operative management. Six patients had distant metastases (Group IV) at diagnosis. Twelve patients without distant metastases underwent regional lymph node biopsy; tumor involvement was found in six. Subsequent treatment consisted of chemotherapy with vincristine (VCR) and dactinomycin (AMD) for all patients; patients in Groups III and IV also received cyclophosphamide (CYC) with or without Adriamycin (ADR) (doxorubicin; Adria Laboratories, Columbus, OH). Radiation therapy (RT) was administered to patients in Clinical Groups II, III, and IV. Overall, 28 (78%) patients achieved a complete clinical response. The 3-year disease-free survival rate for these patients was 42%, compared with 52% for all other patients in the combined IRS I and IRS II series (P = 0.44). The overall 3-year survival rate was 59%, compared with 64% for all other patients in IRS I and IRS II (P = 0.48). Aggressive treatment is needed in children with perineal sarcoma to improve their prognosis.

Adolescent↗
PubMed1976

[Ultrastructure and formal pathogenesis of embryonal rhabdomyosarcoma (author's transl)].

An embryonal rhabdomyosarcoma of the nasopharynx of a 10 year old boy is analysed with light and electron microscopy. With regard to cell shape and cytoplasmic features the following four tumour cell types could be distinguished: 1. Undifferentiated mesenchymal cells with a big loosely packed nucleus and a small cytoplasmic rim with only few cell organelles; 2. Undifferentiated tumour cells with a broad cytoplasmic body which contains a dense network of nonspecific intermediate filaments with a diameter of about 100 A; 3. Immature rhabdomyoblasts with randomly orientated specific myofilaments; 4. Fully differentiated rhabdomyoblasts with well developed myofibrils often showing a sarcomeric pattern. Glycogen deposits which were seen in great masses in many tumour cells were regarded to result from degenerative processes within the tumor. The cellular stages in the development of rhabdomyoblasts are basically identical to those known from the embryogenesis and regeneration of striated muscle. From these observations the two following developmental pathways are suggested: 1. Origin of the tumour from an undifferentiated mesenchymal cell; 2. Atypical regeneration of striated muscle which terminates in malignant progressive tumour growth. At present, the body of information about rhabdomyosarcomas supports the assumption of an origin from immature mesenchymal cells. Nevertheless, the second theory cannot be totally excluded.

Cell Differentiation↗
PubMed1979

Paratesticular rhabdomyosarcomas and leiomyosarcomas: a clinicopathological review.

One case of embryonal paratesticular sarcoma and 2 cases of leiomyosarcomas are reported. The 13-year-old boy with embryonal sarcoma is well after an orchiectomy and high ligation of the spermatic cord followed by radiotherapy and chemotherapy. A 66-year-old man has been doing well after orchiectomy and hemiscrotectomy for a paratesticular leiomyosarcoma. The third patient had a highly pleomorphic leiomyosarcoma and died 2 months postoperatively. The clinical and histological diagnosis of paratesticular rhabdomyosarcomas and leiomyosarcomas is reviewed and the various therapeutic approaches to these neoplasms are discussed. Retroperitoneal lymph node dissection, radiotherapy and chemotherapy are important adjuncts to orchiectomy in the management of rhabdomyosarcomas. On the contrary, retroperitoneal lymph node dissection and radiotherapy are not indicated in leiomyosarcomas, since these neoplasms tend to metastasize by the hematogenous route and are radioresistant. At the present time we are unable to evaluate chemotherapy in the management of paratesticular leiomyosarcomas.

Aged↗