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At least 775 records · Page 43Linked to original sources
PubMed2001

Integrated diagnostic imaging of primary thoracic rhabdomyosarcoma.

We report a rare case of primary thoracic rhabdomyosarcoma in a girl who was referred with acute chest pain, hacking cough, and wheezing. A chest X-ray revealed a complete opacity of the right hemithorax. Ultrasound revealed a right-sided pleural effusion and a solid mass above the liver dome, suggesting a neoplastic disease, which quickly led to further specific examination. Use of CT and MRI together with bone scintigraphy completed the investigation. The biopsy specimen showed a pattern of alveolar rhabdomyosarcoma. This case was reported to emphasize the role of US in the evaluation of a child with hemithorax opacity.

Chest Pain↗
PubMed2001

Metastatic rhabdomyosarcoma to the breast.

Rhabdomyosarcoma is a common extramammary primary malignancy in childhood that rarely metastasises to the breast. We present a patient with primary sinonasal rhabdomyosarcoma who was in remission when she developed breast metastases. We describe particular imaging findings of this disease, using ultrasound and MR imaging. To our knowledge, MR findings have only been described in one previous case report in the literature.

Breast Neoplasms↗
PubMed1998

Laryngeal embryonal rhabdomyosarcoma in a child: MRI appearance.

We report a rare case of a young girl with an embryonal rhabdomyosarcoma of the right aryepiglottic fold. Local disease recurrence, occurring 7 years after subtotal resection and adjuvant chemotherapy, was studied with plain radiography, CT and MRI. To the best of our knowledge, this is the first report illustrating the MRI characteristics of a laryngeal embryonal rhabdomyosarcoma in a child.

Child↗
PubMed1997

[Rhabdomyosarcoma of the orbits 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 resection (microsurgery), radiation therapy and chemotherapy. Favorable outcome in the precytostatic era (with permanent loss of function) was not significantly improved by polychemotherapy. Microsurgery seems to increase the risk of recurrence. CONCLUSION: Considering the high risk of recurrence, primary exenteration in selected patients with expanded primary tumor is still a therapeutic option.

Adolescent↗
PubMed2005

Congenital pineoblastoma and parameningeal rhabdomyosarcoma: concurrent two embryonal tumors in a young infant.

BACKGROUND: Pineoblastomas are very rare brain tumors in fetus and neonates, comprising only 0.9% of congenital brain tumors. The occurrence of multiple tumors of different histopathologic types in the same individual is a rare event, most often encountered in hereditary cancer syndromes. CASE REPORT: We report a female fetus presented with a congenital pineoblastoma at the 32nd week of gestation, with hydrocephalus and concurrent parameningeal embryonal rhabdomyosarcoma in early infancy. RESULTS: Cytogenetic analysis showed normal karyotype in the peripheral blood of the patient, and p53 mutational analysis revealed no germ line mutations. DISCUSSION: This is the first case with concurrent congenital pineoblastoma and parameningeal embryonal rhabdomyosarcoma in early infancy. We suggest that concurrence of these tumors could be due to mutations in other tumor suppressor genes or secondary to exposure to unknown in utero factors.

Brain Neoplasms↗
PubMed2005

Rhabdomyosarcoma relapse in an unusual site.

Lower genitourinary tract rhabdomyosarcoma is the most common soft tissue sarcoma in children. Recurrence is common but usually occurs at the original site. A case of a young boy with recurrent rhabdomyosarcoma at an unusual site (glans penis) is presented.

Child, Preschool↗
PubMed2006

Transpubic radical prostatectomy: a novel approach for rhabdomyosarcoma of the prostate in children.

Rhabdomyosarcoma is the most common soft tissue sarcoma, representing 10-15% of all malignant solid tumours in children and can occur in any site. The prognosis for rhabdomyosarcoma of the prostate has improved significantly in the last few decades. The management involves a combination of chemotherapy, radiotherapy and surgery. Several approaches to resect the prostate in adults have been described. These include radical retropubic, transperineal, posterior sagittal as well as the transpubic approach. In the child, the transpubic approach allows direct access to the prostate and proximal urethra whilst obviating problems noted in adults. We describe this approach to the prostate in a 3-year-old boy.

Anastomosis, Surgical↗
PubMed1996

Intraventricular rhabdomyosarcoma after resection of hyperplastic choroid plexus.

Primary intracranial rhabdomyosarcoma is extremely rare. Here, a case that is most consistent with a botryoid rhabdomyosarcoma is described in a 4.5-year-old boy. The case is unique because it was preceded by a congenital Dandy-Walker malformation, choroid plexus hyperplasia and an enormous oversecretion of cerebrospinal fluid during infancy.

Brain Diseases↗
PubMed2005

A case of primary botryoid conjunctival rhabdomyosarcoma.

BACKGROUND: Rhabdomyosarcoma is the most common childhood primary orbital malignancy, but the occurrence of this tumour in the conjunctiva is rare. METHODS: A 14-year-old girl presented with a visible case of conjunctival mass in the left eye. The year before, the patient was referred to another hospital for a biopsy of a conjunctival mesenchymal tumour in the same site, which was periodically checked after the excision. We intervened by partially removing the conjunctival mass. RESULTS: The histopathology revealed a botryoid rhabdomyosarcoma. The patient underwent chemotherapy and fractionated radiotherapy. The follow-up after three years revealed that the patient remained clinically tumor-free, with no ocular complication from the radiotherapy. CONCLUSION: It is evident that by not excising the tumour in its entirety it is therefore possible to preserve the underlying rectus muscles and not interfere with ocular motility. Subsequent radiotherapy and chemotherapy helped to destroy any residual tumour. Alternatively, surgical excision of the mass alone could lead to recurrence of the tumour after some months or years.

Adolescent↗
PubMed2006

Spindle cell rhabdomyosarcoma in adults: clinicopathological and immunohistochemical analysis of seven new cases.

Rhabdomyosarcoma (RMS) is currently classified into embryonal RMS, including its botryoid and spindle cell variants, alveolar RMS, including a solid variant, and pleomorphic RMS. In children and adolescents embryonal RMS occurs in a younger age group than alveolar RMS, and pleomorphic RMS is almost always seen in older adults. Most recently rare spindle cell and sclerosing, pseudovascular RMS have been reported in adults as well. We analysed the clinicopathological and immunohistochemical features of seven new cases of spindle cell RMS arising in adult patients. Five patients were male and two were female and the age of the patients ranged from 38 to 76 years. Four neoplasms arose on the lower extremities and one case each on the forearm, the lateral aspect of the neck and the penis. Five neoplasms were completely excised, in one incompletely excised neoplasm additional chemotherapy was given, and in one patient a biopsy was done only so far. All neoplasms arose in subcutaneous and deep soft tissues with dermal involvement in one case, and the size of the neoplasms ranged from 4 to 19 cm in largest diameter. Histologically, a plump or diffuse infiltration was seen, and all neoplasms were mainly composed of cellular bands and fascicles of atypical spindle-shaped tumour cells containing enlarged and atypical nuclei associated with a variable number of rhabdomyoblasts. In addition, focal areas reminiscent of sclerosing, pseudovascular RMS were noted in three cases, and in two cases each small solid areas with pleomorphic tumour cells as well as scattered round tumour cells were present. Proliferative activity ranged from 1 to 60 mitoses in 10 high-power fields and tumour necrosis was evident in four cases. Immunohistochemically, all neoplasms tested stained variably positive for desmin, myf-4, WT1 and CD 99, whereas fast myosin was positive in only two out of seven cases. In addition, five out of seven cases tested stained focally positive for alpha-smooth muscle actin. The remaining antibodies (h-caldesmon, S-100 protein, CD 34, pancytokeratin and epithelial membrane antigen) were all negative. Follow-up information was available in five patients (range from 10 to 48 months) and revealed lung metastases in two patients who died of disease within a short period. In summary, spindle cell rhabdomyosarcoma represents a rare neoplasm in adulthood characterized clinically by a rather poor prognosis, and shows a broad morphological spectrum including most likely the sclerosing, pseudovascular variant. Immunohistochemically, tumour cells in RMS stain positively for CD 99 and WT1 as well, which is of importance in the differential diagnosis to other mesenchymal neoplasms, whereas fast myosin does not represent a reliable marker for RMS in adults.

12E7 Antigen↗
PubMed1997

Characteristics of rhabdomyosarcoma cell lines derived from uterine carcinosarcomas.

Rhabdomyosarcoma (RMS) is occasionally found in the female genital tract, and mostly appears as one of the heterologous mesenchymal components in uterine carcinosarcoma designated as malignant mixed mullerian tumour (MMMT). We examined the biological properties of a pure rhabdomyosarcoma (RMS) cell line designated FU-MMT-3, which was newly established from a surgical specimen taken from a patient with uterine MMMT. We also evaluated c-myc and MYCN gene amplification in three RMS cell lines (including FU-MMT-3) derived from three MMMTs by Southern blot analysis. FU-MMT-3 cells were propagated continuously for 57 serial passages over a 2-year period in vitro. FU-MMT-3 was able to produce tumours demonstrating pure RMS in athymic nude mice. Cytogenetically, FU-MMT-3 showed a triploidy pattern, with complex karyotypic abnormalities including trisomy of chromosome 8. All three RMS cell lines, including FU-MMT-3, showed amplification of the c-myc gene (approximately fourfold to eightfold), while no cell lines demonstrated MYCN gene amplification. FU-MMT-3 is considered to provide a useful system for the study of the biological behaviour of RMS in MMMTs. Extra copies of chromosome 8 and c-myc gene amplification may be associated with the rhabdomyoblastic differentiation in MMMT.

Animals↗
PubMed2005

Embryonal rhabdomyosarcoma of the common bile duct mimicking choledochal cyst.

Embryonal rhabdomyosarcoma of the common bile duct is a very rare malignancy of childhood. The radiological appearance of the lesion is similar to that of congenital choledochal cyst if there is no local invasion to the adjacent tissues. The authors present a case of embryonal rhabdomyosarcoma of the common bile duct which was considered to be a congenital choledochal cyst preoperatively, and they discuss this very rare childhood tumor, with a brief survey of the literature. In conclusion, it is important to know that this rare tumor can simulate congenital choledochal cyst; it should be considered in the differential diagnosis of obstructive jaundice in children.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2007

Primary rhabdomyosarcoma of the breast in a 13-year-old girl: report of a case.

We report a case of primary alveolar rhabdomyosarcoma of the breast in a 13-year-old Japanese girl. The patient initially presented with a 13 x 8-cm mass in her left breast, which was diagnosed as alveolar rhabdomyosarcoma after an excisional biopsy. Genetic expression of the tumor revealed t(2;13)(q35-37;q14). She underwent modified radical mastectomy (Bt + Ax) and nine lymph nodes were found to be involved. Systemic examinations showed multiple bone and lung metastases 2 weeks after her operation. Despite chemotherapy with doxorubicin, ifosfamide, and actinomycin D, which resulted in remission for 6 months, she died of the disease 8 months after surgery.

Adolescent↗
PubMed2000

Rapid growth of a retroperitoneal rhabdomyosarcoma following right hemicolectomy for ascending colon cancer: report of a case.

We report herein the case of a 69-year-old man in whom rapid growth of a retroperitoneal rhabdomyosarcoma occurred following hemicolectomy for ascending colon cancer. On his first admission for surgery, a small lesion, 1.5 cm in diameter, was detected adjacent to the inner side of the left kidney by abdominal axial computed tomography (CT), which was initially suspected to be a benign lesion; however, a postoperative follow-up CT scan done 5 months later revealed that the lesion had enlarged remarkably to 8 cm in diameter. Thus, total resection was performed under the presumed diagnosis of a malignant retroperitoneal tumor. The tumor was found to be adjacent to the inner portion of the left kidney and covered by Gerota's fascia. As it involved the ileolumbar muscle and had a metastatic lymph node, complete resection was performed. The resected specimen was 8.5 x 6.5 x 5 cm in size and was histologically confirmed as a retroperitoneal rhabdomyosarcoma of embryonal type. Two courses of adjuvant chemotherapy with adriamycin, vincristine, and cyclophosphamide were given, and the patient has shown no signs of recurrence for 2 years since his second operation.

Adenocarcinoma↗
PubMedDate not supplied

Association of alveolar rhabdomyosarcoma with the Beckwith-Wiedemann syndrome.

Rhabdomyosarcoma (RMS) is a soft tissue tumor of childhood frequently diagnosed between the first and fifth year of life. Children with the Beckwith-Wiedemann syndrome (BWS), a congenital overgrowth syndrome characterized by exomphalos, macroglossia, and macrosomia, have an increased risk of developing childhood tumors including Wilms tumor, hepatoblastoma, neuroblastoma, and RMS. Although an association between RMS and the BWS is well accepted, only four cases have been reported to date, and of these, three were reported as embryonal RMS. Based on these data, an association between BWS and embryonal RMS has been proposed. We report three additional cases of BWS with RMS and review the clinical data for each patient as well as the pathology of their tumors. All three cases of BWS had histology consistent with alveolar RMS and were diagnosed at 6 weeks and 5 and 13 years of age. In two of these BWS cases, constitutional defects of 11p15 imprinting were demonstrated. Furthermore, cytogenetic analysis of the tumors did not detect the t(2;13) or t(1;13) translocations that generate the PAX3- or PAX7-FKHR fusion proteins common to alveolar RMS. These observations suggest that the development of alveolar RMS tumors in BWS may occur without the chromosomal rearrangement producing the PAX-FKHR fusion protein. In summary, we present three new cases of RMS demonstrating a new association between BWS and an uncommon subtype of alveolar RMS. The absence of the translocations commonly associated with alveolar rhabdomyosarcoma suggests a common 11p15 pathway for alveolar RMS and BWS.

Adolescent↗
PubMedDate not supplied

Early history of pathology studies by the Intergroup Rhabdomyosarcoma Study Group.

This review chronicles the series of publications that were the result of the willingness of pathologists and clinicians in the United States to share their pathologic materials and clinical data on patients who were placed on treatment protocols for rhabdomyosarcoma and related tumors over an extended period of time. The availability of this database enabled pathologists and clinicians to study a tumor type that is rare in individual institutions, but occurs in large enough numbers to produce valid conclusions not otherwise possible. Furthermore, young investigators were challenged by this opportunity and were able to spend the necessary time to make new observations that, in retrospect, helped direct protocol designs that produced significant improvement in patient survival. The key factor in this process is the surrender of individual scientific prerogatives to a small number of investigators. It is also important to recognize that the pathologist component of these series of contributions is only a part of the entire effort. It takes an organization of gifted, dedicated experts in many disciplines working together. The investigators who served on the Intergroup Rhabdomyosarcoma Group over a 25-year period eminently fulfilled this.

Child↗
PubMed2004

Paratesticular pleomorphic rhabdomyosarcoma in an adult.

Rhabdomyosarcoma (RMS) is the commonest variety of soft tissue sarcoma in children and young adults. It represents 6.5% of all malignant tumours in paediatrics with an annual incidence of 4 to 7 cases/million children. The primary paratesticular site is considered to have a good prognosis in comparison with other rhabdomyosarcomas, despite the frequency of retroperitoneal lymph node invasion. This superficial site allows rapid diagnosis and consequently often complete resection of the tumor. The multi-disciplinary treatment of paratesticular RMS has improved control of the disease with a 2-year survival of 80%. We herein report a case with paratesticular pleomorphic RMS, a rare and prognostically unfavorable variant of RMS, who is disease free for 60 months after management with multidisciplinary approach.

Adolescent↗
PubMed2005

Primary cardiac rhabdomyosarcoma following a uterine leiomyosarcoma: double primary sarcomas.

Primary cardiac rhabdomyosarcoma is rare. A dismal prognosis is usually given due to its high propensity for distant metastases, with survival rarely exceeding 2 years. We report a case that was initially considered to be a cardiac metastatic tumor from a primary uterine leiomyosarcoma, but after morphological and immunohistochemical examinations was shown to be a primary cardiac rhabdomyosarcoma. The right atrium was filled with a mass that oscillated between the right atrium and ventricle through the tricuspid valve. To reduce the risk of sudden death from tumor embolism into the pulmonary arteries, the tumor was resected. Even though the surgery was not curative, it eliminated the risk of sudden death due to tumor embolism into the pulmonary arteries and improved short-term survival.

Fatal Outcome↗