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PubMed2000

[Breast metastases from embryonal rhabdomyosarcoma: apropos 2 cases and a review of the literature].

The presence of breast metastases is uncommon in oncology. In children and young adults, the most common type of primary tumour is rhabdomyosarcoma, and primary breast rhabdomyosarcomas are exceptional. Two cases are reported of breast metastatic rhabdomyosarcoma in two adolescents with several atypical characteristics in their presentation forms: the embryonal variety and the primary location at maxillary sinus. The cases reported in the literature up to now were reviewed, with a special emphasis on epidemiologic, clinical, diagnostic, and therapeutic data of this rare entity.

Adolescent↗
PubMed2003

Metastatic testicular rhabdomyosarcoma--a report of two cases.

Rhabdomyosarcoma is the most common type of soft tissue sarcoma in children. The tumor spreads by local extension, to regional lymph nodes, or by distant metastases. Metastatic spread to the testicle has been rarely described. The authors describe 2 boys who were found to have intratesticular metastases after presenting with primary tumors in their extremities. The first patient, an 11-year-old boy presented with primary disease in his left foot and an enlarged testicle. Rhabdomyosarcoma was found histologically in both the foot and the testicle. A second boy 17 years of age had a primary tumor involving the left upper extremity treated with amputation, chemotherapy, and radiotherapy. A relapse was noted 2 years later in the left testicle and was treated with orchiectomy. The authors discuss the implications and the management of this rare presentation of metastatic rhabdomyosarcoma.

Adolescent↗
PubMed1981

Rhabdomyosarcoma of the female urogenital tract.

During the initial 6 yr (1972-1978) of the Intergroup Rhabdomyosarcoma Study (IRS), 27 patients with rhabdomyosarcoma of the female urogenital tract were treated, and the results of therapy were evaluated on the basis of 3-7 yr of observation. By primary site these tumors were: bladder, 14; vagina, 9; vulva, 2; and cervix uterus, 2. Twenty-three presented with localized tumors; the four remaining patients (1 bladder, 1 vagina, and 2 cervix) had distant metastases at diagnosis. Two patients with bladder tumors underwent pelvic exenteration, local irradiation, and chemotherapy (2 yr), and were without relapse. Ten patients were treated by partial cystectomy and postoperative chemotherapy (with or without local irradiation) and fatal recurrence occurred in three. The remaining seven patients are disease-free for 36-74 mo (mean, 51.8 mo). Six of the eight patients with nondisseminated vaginal tumors were treated by initial pelvic exenteration (2), hysterectomy-vaginectomy (2), or hemivaginectomy (2). All received postoperative chemotherapy and three received local irradiation. Delayed hysterectomy-vaginectomy after primary chemotherapy was performed in the two additional patients with vaginal tumors. Relapse has occurred in one of these eight patients with localized vaginal disease. The remaining seven have been observed for 3-5.5 yr (mean, less than 4 yr). Two patients with vulval lesions treated by a combined therapy regimen are without recurrence. Our conclusion is that chemotherapy has significantly increased survival in females with urogenital tract rhabdomyosarcomas.

Adolescent↗
PubMed1983

Mortality among children with rhabdomyosarcomas of the alveolar histologic subtype.

Of 116 patients with rhabdomyosarcoma of the alveolar histologic subtype who entered the Intergroup Rhabdomyosarcoma Study (IRS) from 1972 to 1978, there were 72 deaths (63%), compared with a mortality of 39% among patients with all nonalveolar types combined. The subgroup with alveolar histology contributed greater than 29% of the total mortality, although it represented 20% of the total patients (p less than .001). This recurrence rate reflects increased local, regional, and distant relapse. Differences were most marked in patients with grossly excised tumors (clinical groups I and II) in which the mortality was 44% in patients with alveolar tumors (48 patients) versus 13.5% in those with embryonal histology (111 patients), and 16% in all patients with nonalveolar tumor types. When survival was influenced by primary tumor site this reflected, in most body areas, an increase in the proportion of patients with the alveolar histology in that site. Patients with unresected or disseminated tumors (clinical groups III and IV) of the alveolar subtype have initial response rates to VAC and radiotherapy which are similar to those of patients with tumors of other cell subtypes (70% for group III and 50% for group IV). In these clinical groups, the differences in survival related to histologic subtype are not significant. The presence of the alveolar histologic subtype represents a rational basis for employing more intensive therapy in the management of patients with rhabdomyosarcomas.

Adolescent↗
PubMed1986

Perianal rhabdomyosarcoma: report of a case in an infant.

Rhabdomyosarcoma is the most common childhood sarcoma, whereas, primary pararectal rhabdomyosarcoma is very rare. To date, four pediatric and eight adult cases have been reported. In children, the tumor presents as a perianal polypoid mass, often initially misdiagnosed. Formerly a very lethal sarcoma, rhabdomyosarcoma is now treated with combined surgery, radiation, and chemotherapy, with encouraging results.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1998

Primary embryonal rhabdomyosarcoma of the breast in an adolescent female: a case report.

Rhabdomyosarcoma is the most common soft tissue sarcoma in children. Primary breast location has been reported rarely in the literature. Most rhabdomyosarcomas encountered in the breast more commonly are metastatic disease from some primary foci in another part of the body. This report addresses the case of an adolescent girl who had primary embryonal rhabdomyosarcoma of the breast with no evidence of local invasion or metastatic disease within the spectrum of Li-Fraumeni syndrome.

Adolescent↗
PubMed1998

The management of paratesticular rhabdomyosarcoma: a single institutional experience with 44 consecutive children.

PURPOSE: We report a single institutional experience with 44 consecutive children treated from 1976 to 1996 for paratesticular rhabdomyosarcoma. MATERIALS AND METHODS: After primary surgical excision, 27 cases were classified as Intergroup Rhabdomyosarcoma Study (IRS) group I, 1 as group II, 8 as group III and 8 as group IV. Except for the first 5 patients, retroperitoneal node dissection was not a required staging procedure and was performed only for radiologically suspicious node metastases. Adjuvant chemotherapy was administered to all patients. Additional radiotherapy was given when nodal involvement or residual disease was documented. RESULTS: The 5-year event-free survival rate was 77% for the entire series of patients, 93% for those in groups I and II, 62.5% in group III and 37.5% in group IV with a median followup of 11 years (range 2 to 20). CONCLUSIONS: Our report confirms the excellent prognosis of localized paratesticular rhabdomyosarcoma. Due to the accuracy of computerized tomography potential morbidity of lymphadenectomy, low rate of retroperitoneal recurrence and presumed efficacy of chemotherapy for controlling microscopic disease, retroperitoneal lymph node dissection seems unnecessary for localized disease.

Adolescent↗
PubMed1990

Nuclear morphometry as a prognostic indicator for genitourinary rhabdomyosarcoma: a preliminary investigation.

Rhabdomyosarcoma of the urogenital tract is a malignant mesenchymal tumor seen primarily in childhood. Multimodal therapy, encompassing surgery, radiotherapy and chemotherapy, has dramatically improved the survival of patients with this disease. However, the quest for markers of tumor aggression is important to decrease the morbidity of treatment given to patients with good prognosis tumors, while at the same time intensifying treatment of tumors with poor prognosis. Using archival tumor specimens from 13 patients with genitourinary rhabdomyosarcoma, a multivariate analysis of multiple nuclear shape descriptors was done with the Hopkins Morphometry System. Three nuclear shape descriptors clearly separated patients with no evidence of disease recurrence or progression from those with recurrent disease, progressive disease or death of disease. These nuclear shape descriptors were standard error of the chain code standard deviation analysis (p = 0.010), range of the feret ellipticity distribution (p = 0.016) and standard error of the chain code range analysis (p = 0.037). With multivariate analysis these shape descriptors taken together separated patients with good and poor prognoses to a level of significance of p = 0.007. Thus, nuclear morphometric analysis may prove to be useful as an individual prognostic indicator in childhood genitourinary rhabdomyosarcoma and warrants further analysis in a much larger, blinded, controlled study.

Cell Nucleus↗
PubMed1990

Paratesticular rhabdomyosarcoma: results of therapy in 18 cases.

The management of paratesticular rhabdomyosarcoma has evolved more slowly than that for testicular germ cell tumors, mainly due to its lower incidence and the lack of standardized treatment protocols. With gradual refinements in staging and therapy the prognosis has improved. Between 1960 and 1988, 18 patients 2 to 18 years old were treated at the Children's Hospital. Management consisted of inguinal orchiectomy, staging evaluation and retroperitoneal lymph node dissection, followed by chemotherapy and radiotherapy according to the stage of the disease. In 11 patients (61%) the disease was confined to the scrotum, while 7 presented with spread to the retroperitoneal lymph nodes, including 3 children with more distant metastases. Histological study revealed predominantly embryonal characteristics except for 1 alveolar cell type. Chemotherapy consisted of actinomycin D in 2 patients, vincristine, actinomycin D and cyclophosphamide in 2, pulse vincristine, actinomycin D and cyclophosphamide in 10, and doxorubicin plus vincristine, actinomycin D and cyclophosphamide in 4. A total of 17 patients remain free of disease (median followup 4 years) with 1 death. The actuarial survival without relapse and over-all survival rate are 89 and 94%, respectively. We conclude that paratesticular rhabdomyosarcoma represents a favorable subgroup of rhabdomyosarcoma.

Actuarial Analysis↗
PubMed1990

Embryonal rhabdomyosarcoma arising in a mature teratoma of the testis: a case report.

We report a case of embryonal rhabdomyosarcoma present in the primary excision of an intratesticular purely mature teratoma. Testicular mature and immature teratomas are usually associated with other germ cell tumors. Nongerminal malignancies that tend to occur in gonadal and extragonadal teratomas are often epithelial. Sarcomas of all types are less frequent but embryonal rhabdomyosarcomas are encountered predominantly. This sarcomatous element can be present in the primary excision or it can appear after chemotherapy in the metastases. Usually, prognosis is dependent on the degree of aggressiveness of the sarcomatous component. A review of the literature reveals that it is unusual for an embryonal rhabdomyosarcoma to develop on purely mature teratoma. We report a case in the testicle. During followup metastases arise rapidly from the purely embryonal rhabdomyosarcomatous component. The accurate diagnosis of this unusual sarcomatous component is of paramount importance because of the implications for therapy.

Adult↗
PubMed1986

Production of basement membrane laminin and type IV collagen by tumors of striated muscle: an immunohistochemical study of rhabdomyosarcomas of different histologic types and a benign vaginal rhabdomyoma.

Immunohistochemical methods were used to demonstrate the distribution of basement membrane laminin and type IV collagen in eight tumors derived from striated muscle (three botryoid, two alveolar, and two adult-type rhabdomyosarcomas; one benign vaginal rhabdomyoma). All of the tumors produced significant amounts of both basement membrane components. Stainings clearly revealed the alveolar nature of the rhabdomyosarcomas, with the alveolar spaces surrounded by distinct basement membranes. Different stages of cellular development were identified in the botryoid sarcomas, with the most immature cells of the cambium layer devoid of external basement membrane around the tumor cells, although the stroma contained finely dispersed basement membrane material and some cells contained intracytoplasmic laminin or type IV collagen, indicative of the synthesis of these proteins. The more mature cells, which had abundant granular cytoplasm, were enveloped by distinct basement membranes and seemed to have coalesced, forming structures resembling myotubes. The adult-type rhabdomyosarcomas were composed of large pleomorphic cells that were surrounded by basement membranes, either individually or in small groups. Some giant cells contained intracytoplasmic laminin. The vaginal rhabdomyoma was composed of round rhabdoblastic cells or elongated strap cells with cross-striations. Cells of both of these types were surrounded by thin but distinct basement membranes. The results suggest that demonstration of basement membranes would be helpful in the diagnosis of tumors derived from striated muscle. The findings concerning different stages of maturation of tumor cells are in accordance with previous in vitro observations of myoblastic cells.

Adult↗
PubMed1986

Extraosseous Ewing's sarcoma versus primitive rhabdomyosarcoma: diagnostic criteria and clinical correlation.

A light and electron microscopic study of 51 cases of Ewing's sarcoma of bone (ESB) and 33 soft tissue sarcomas (carrying a variety of light microscopic diagnoses, including primitive rhabdomyosarcoma) in children and young adults was performed to clarify the similarities and differences among these tumors. Ultrastructural criteria were developed to evaluate the neoplasms. Remarkable ultrastructural uniformity was found in the cases of ESB. In contrast, the soft tissue sarcomas could be divided into two distinct groups on the basis of the ultrastructural criteria: those closely resembling primitive areas of otherwise differentiated rhabdomyosarcomas, and those indistinguishable from ESB. It is proposed that the diagnosis of soft tissue Ewing's sarcoma be reserved for lesions identical to ESB by both light and electron microscopy. The first group of sarcomas may be histogenetically related to rhabdomyosarcoma and should be distinguished from extraosseous Ewing's sarcoma, as their clinical behavior appears to be quite different.

Adult↗
PubMed1990

Sarcoplasmic reticulum (SR) Ca2(+)-ATPase as a marker of muscle cell differentiation: immunohistochemical investigations of rhabdomyosarcomas and enhancement of the immunostaining after sodium methoxide pretreatment.

An immunocytochemical investigation of sarcoplasmic reticulum (SR) Ca2(+)-ATPase (SR-Ca-ATPase) was performed on formalin-fixed paraffin-embedded specimens of different types of rhabdomyosarcomas such as variants of embryonal and pleomorphic forms. Immunostaining frequency of tumours using SR-Ca-ATPase was compared with that of traditionally used muscle specific markers myoglobin, and desmin. Utilizing the possible cleaving of ester bounds sodium methoxide pretreatment was found to be very effective in enhancement of SR-Ca-ATPase immunostaining reaction. In 11 of 15 tissue specimens of 5 cases round shaped and elongated rhabdomyoblasts with definite cytoplasm exhibited positive immunoreactions with all of the polyclonal antibodies tested, using the streptavidin-biotinylated peroxidase complex (S-ABC-method). In formalin-fixed and paraffin-embedded material of 2 cases of undifferentiated rhabdomyosarcomas composed of small round tumour cells with scanty cytoplasm pretreatment with sodium methoxide induced the immunostaining of SR-Ca-ATPase. After that pretreatment a staining of the paranuclear cytoplasm occurred in many of these undifferentiated tumour cells. In these 2 cases, neither myoglobin nor desmin antibodies could react. However, when frozen sections of one of the poorly differentiated tumours were used monoclonal and polyclonal desmin antibodies reacted immunocytochemically in all of the small cells. Sodium methoxide induced or enhanced SR-Ca-ATPase immunocytochemical reaction can be a further addition to the diagnosis of rhabdomyosarcomas in formalin-fixed paraffin-embedded sections, even when desmin antibody fails to react.

Antibodies, Monoclonal↗
PubMed1989

Lectin histochemistry of experimental murine rhabdomyosarcomas.

Methylcholanthrene-induced murine rhabdomyosarcomas and skeletal muscle of 10 and 18 d old murine embryos were investigated by lectin histochemistry (WGA, RCA-I, LCA, Con-A, PSA, UEA-I, PNA) and by immunohistochemistry (vimentin, desmin, myoglobulin). In rhabdomyosarcomas as well as in the developing skeletal muscle a clear trend was visible. A decrease of vimentin positivity and an increase of desmin positivity were associated with a diminution of binding sites for WGA, RCA, and LCA. No binding moieties for these lectin could be demonstrated in myoglobin positive normal and neoplastic rhabdomyomatous cells at all. The homologous expression or absence of markers reflected the cellular variability in rhabdomyosarcomas and may be explained as a phenomenon of different tumor cell maturation. The results show that rhabdomyosarcomatous cells are imitating the normal skeletal muscle development.

Animals↗
PubMed2002

Mixed tumor of paratesticular rhabdomyosarcoma and an adenomatoid tumor in an elderly patient.

We report a case of a mixed tumor of paratesticular rhabdomyosarcoma and an adenomatoid tumor in an elderly patient. Rhabdomyosarcoma is one of the most common childhood tumors; however, it rarely occurs in the elderly. In addition, to our knowledge, paratesticular rhabdomyosarcoma and adenomatoid tumor rarely form mixed tumors. Finally, we consider the pathogenesis of diploblastic tumors.

Aged↗
PubMed1999

Ureteral rhabdomyosarcoma.

Rhabdomyosarcoma is a malignant tumor well known to urologists. These tumors arise from the genitourinary system in 20% to 25% of cases, most commonly from the bladder, prostate, vagina, and paratesticular region. This is the first reported case of a rhabdomyosarcoma arising from the ureter. The radiographic findings and ureteroscopic appearance of this tumor suggested a benign fibroepithelial polyp; however, a ureteroscopic biopsy and subsequent nephroureterectomy revealed an embryonal rhabdomyosarcoma.

Adult↗
PubMed1998

Structural analysis of PAX3 genomic rearrangements in alveolar rhabdomyosarcoma.

In the pediatric cancer alveolar rhabdomyosarcoma, the (2;13)(q35;q14) translocation juxtaposes PAX3 and FKHR to produce a chimeric PAX3-FKHR gene. With the use of Southern blot methodology, genomic rearrangements of PAX3 intron 7 were detected in 23 of 23 fusion-positive alveolar rhabdomyosarcomas and were not detected in 19 fusion-negative embryonal rhabdomyosarcomas. Rearrangements corresponding to the reciprocal FKHR-PAX3 fusion were detected in 21 of 23 PAX3-FKHR-positive cases, though FKHR-PAX3 transcripts were detected in only 15 of 23 cases. Mapping experiments demonstrated that breakpoints occurred throughout this 17.5 kb PAX3 intron and, in 12 of 23 cases, breakpoints clustered within a 4.5-kb region at the 3' end of the intron. Chromatin analysis revealed a prominent DNase I hypersensitive site at the 5' end of the intron but did not indicate any other DNA-protein interactions that might have affected the breakpoint distribution. Sequence analysis identified AT-rich regions within the 3' cluster, as well as alternating purine-pyrimidine and homopyrimidine elements at the borders of this cluster. These finding suggest that translocation breakpoints are constrained to PAX3 intron 7 primarily by functional boundaries related to the flanking exons and may be secondarily affected by sequence features within this intron.

Chromosomes, Human, Pair 13↗
PubMed1999

Trisomy 8 in embryonal rhabdomyosarcoma detected by fluorescence in situ hybridization.

Embryonal rhabdomyosarcoma is the most common malignant soft-tissue tumor in childhood. Cytogenetic studies of this tumor are rare. In one study trisomy 8 was found to be a primary cytogenetic abnormality. In view of the findings of trisomy 8 in a multitude of cancers, we conducted a pilot study to test the hypothesis that a subset of rhabdomyosarcoma also exists with trisomy 8. Accordingly, archival tissues of 12 cases of rhabdomyosarcoma were retrieved and fluorescence in situ hybridization (FISH) using a chromosome 8-specific, alpha-satellite probe was undertaken on formalin-fixed paraffin-embedded tissue sections using the protocol optimized in the Cytogenetics Laboratory at Rhode Island Hospital. The results obtained demonstrated that 6 of 12 tumors showed chromosome 8 trisomy, when a 15% threshold is adopted. In addition, one case was borderline, with 11% of the cells found positive for three fluorescent signals. Future experiments utilizing additional specimens from our centers as well as from other laboratories are needed to confirm and extend the findings of the present study.

Cell Differentiation↗