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PubMed1990

Flow and image cytometric DNA analysis in rhabdomyosarcoma.

Rhabdomyosarcoma is the most common malignant soft-tissue tumor in childhood, with an overall 3-year disease-free survival of 73%. DNA content is known to correlate with prognosis and therapy response in many cancers. To determine the role of DNA content in rhabdomyosarcoma, 23 tumor samples were studied retrospectively: 18 primary tumors and 5 post-chemotherapy recurrences or specimens obtained at second-look surgeries. The DNA analysis was performed on disaggregated paraffin-embedded tissue nuclei by flow and image cytometry and correlated with the histology and clinical history. Of the primary tumors 4 were diploid, 4 polyploid, and 10 aneuploid (9 with a single aneuploid G0G1 peak and 1 multiploid) by flow cytometry. The concordance rate between flow and image cytometry was 19 of 23 (83%); one case did not have flow cytometry available. Most embryonal rhabdomyosarcomas were aneuploid (10 of 12; 83%), and they had a high incidence of recurrence in Stages III and IV (4 of 12; 33%). Although aneuploidy in pediatric cancers may predict a therapeutic response and good prognosis, this was not supported by our findings in rhabdomyosarcoma. The tumor DNA content correlated with the clinical stage but not with the patient's clinical course or tumor histopathological type. DNA content did not appear to be as important a prognostic tool as tumor stage.

Adolescent↗
PubMed1985

[Combination chemotherapy with cis-diamminedichloroplatinum, vinblastine and bleomycin for a rhabdomyosarcoma of the prostate in a child: report of a case].

A case of prostatic rhabdomyosarcoma in a 5-year-old boy is reported. He was brought to our clinic on Apr. 1, 1982 with complaints of pollakisuria and urethral pain. X-ray examinations revealed a huge intrapelvic tumor, and it was histopathologically diagnosed as embryonal rhabdomyosarcoma with a specimen of transrectal needle biopsy. Since the tumor was too huge to resect completely, he was initially treated with combination chemotherapy regimen of vincristine, actinomycin D and cyclophosphamide (VAC therapy), and resulted in failure. Then another combination chemotherapy consisting of cis-diamminedichloroplatinum, vinblastine and bleomycin (PVB therapy) was tried, and the tumor showed reduction in size. On Oct. 15, 1982, total cystectomy with ileal conduit urinary diversion was performed. Histopathologically, degenerative change and partial necrosis of the tumor cell were recognized. After the operation, he was treated with radiation therapy and prophylactic VAC therapy. But six months later, multiple pulmonary metastases occurred and gradually increased in size and number. They did not respond to any other chemotherapy. He died on July 13, 1983. We discussed the chemotherapy for rhabdomyosarcoma, and emphasized that the PVB therapy should be tried on rhabdomyosarcoma as an initial chemotherapy.

Age Factors↗
PubMed1989

[Histopathologic and immunohistochemical study of rhabdomyosarcoma].

Histopathologic and immunohistochemical study of 120 cases of soft tissue malignancy diagnosed as rhabdomyosarcoma is reported. There were 65 males and 55 females including 62 adults (greater than or equal to 15 years) and 58 children (less than 15 years). In the adult group, the tumor was common in the limbs and the trunk, whereas in the children group, the urogenital system and regions in the head and neck. Histologically, they were divided into three types: 1. embryonic type (65 cases), 2. alveolar type (28 cases) and 3. polymorphic type (27 cases). Fifty-two cases of proved rhabdomyosarcoma and 21 cases of misdiagnosed rhabdomyosarcoma were stained immunohistochemically with myoglobin antiserum. Forty seven of those 52 cases were positive to various degrees, whereas all the misdiagnosed cases were negative. It was evident that these doubted cases were misdiagnosed in previous histopathologic interpretation. Our study suggests that the immunohistochemical myoglobin stain be an important method for the differentiation of rhabdomyosarcoma. The cause of histopathologic misdiagnosis and the significance of myoglobin antiserum in the histopathologic diagnosis and differential diagnosis of this tumor are discussed.

Adolescent↗
PubMed1986

Genital rhabdomyosarcoma: current management and review of the literature.

Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and 20 per cent of these cancers involve the pelvis or genitourinary system. Radical pelvic surgery, such as exenteration, was considered at one time to be the standard treatment for this cancer which carried a very poor prognosis. Over the past 15 years, a combined modality approach to treating rhabdomyosarcoma, using chemotherapy, radiotherapy, and less radical surgery, has evolved and survival rates have improved. This paper presents a case of rhabdomyosarcoma involving the perineum which was treated by combination chemotherapy of Vincristine, Actinomycin-D, and Cytoxan, followed by wide local excision, interstitial and external beam radiotherapy, and postoperative chemotherapy. The literature on pelvic rhabdomyosarcoma is reviewed and the current approach to treating this cancer using multimodal therapy is discussed.

Adolescent↗
PubMed1987

[Two cases of paratesticular rhabdomyosarcoma].

Two cases of paratesticular rhabdomyosarcoma are reported. A 15-year-old boy was admitted in July, 1983, with a 3-week history of gradually increasing painless scrotal swelling on the right side. With the suspicion of testicular cancer, right radical orchiectomy was performed and pathological examination revealed an embryonal rhabdomyosarcoma of the paratesticular region. Chemotherapy and retroperitoneal lymphadenectomy were performed, and the latter revealed lymph node metastasis at the bifurcation of the aorta. Radiotherapy was not done because of adherent ileus. About 8 months after the orchiectomy, he died of recurrence at pelvic cavity and brain metastasis. A 6-year-old boy presented in March, 1985, with a complaint of right scrotal swelling that was painless and gradually increasing for about a month. Transinguinal exploration revealed a paratesticular neoplastic lesion and right radical orchiectomy was performed. Pathological diagnosis was paratesticular embryonal rhabdomyosarcoma. No evidence of metastasis was found by chest X-ray, excretory urogram, abdominal CT-scan or lymphangiography. As post-operative treatment, only chemotherapy was performed, and 9 months after the orchiectomy, the patient was asymptomatic. Ninety four cases of paratesticular rhabdomyosarcoma found in the Japanese literature are reviewed and mainly the policy of treatment is discussed.

Adolescent↗
PubMed1986

Monoclonal antibodies to titin in conjunction with antibodies to desmin separate rhabdomyosarcomas from other tumor types.

Titin is a major constituent protein of sarcomeric muscles and is thought to give rise to an elastic filament component underlying the myofibrillar organization. Monoclonal antibodies to titin have been characterized on normal and pathological human material and on human cell lines in culture. A positive immunocytochemical reaction was restricted to sarcomeric muscles and did not occur on visceral or vascular smooth muscles or on various nonmuscle tissues. When different tumor types were examined titin antibodies reacted solely with rhabdomyosarcomas and did not react with leiomyosarcoma or leiomyoma, or with the nonmuscle tumor types tested. In rhabdomyosarcomas a noticeably smaller population of cells were positive with antibodies to titin than with antibodies to desmin showing that individual cells within a rhabdomyosarcoma achieve different degrees of myogenic differentiation. The results reinforce the use of desmin as a marker for muscle sarcomas and show that a positive identification of rhabdomyosarcoma can be achieved by immunocytochemistry with the parallel use of desmin and titin antibodies.

Animals↗
PubMed1985

[A case of embryonal rhabdomyosarcoma of the kidney].

A case of embryonal rhabdomyosarcoma of the left kidney is reported. A 16-year-old boy was admitted with the complaint of left abdominal pain and fever on January 6, 1983. Radiological examination showed a tumor of the left kidney; and, nephrectomy was performed. Histopathologically the entire tumor was composed of undifferentiated round cells. Diagnosis of embryonal rhabdomyosarcoma was made on the basis of special stains including immunohistochemical study with nervous tissue specific enolase. Although radiation and chemotherapy were performed postoperatively, the tumor recurred and the patient died on October 22, 1983. The problems of differential diagnosis of embryonal rhabdomyosarcoma from sarcomatous types of nephroblastoma, particularly rhabdoid tumor and other undifferentiated renal tumors were discussed. Fifteen rhabdomyosarcoma of the kidney including our case have been reported in the Japanese literature.

Adolescent↗
PubMed1979

Primary rhabdomyosarcoma of the tentorium with peculiar angiographic findings.

Primary intracranial rhabdomyosarcoma is extremely rare, only nine cases have been reported in the world literature. To differentiate it from medullomyoblastoma, rhabdomyosarcoma should be devoid of neuroblastic elements. A case of rhabdomyosarcoma of the tentorial region in a 17-year-old boy is reported. Carotid angiograms demonstrated a peculiar neovascularity. Multiple cytologic preparations of CSF repeatedly contained rhabdomyosarcoma cells and CSF dissemination in the spinal subarachnoid space was documented at autopsy. The literature is reviewed.

Adolescent↗
PubMed1982

[Rhabdomyosarcoma of the urinary bladder: complete remission induced by vinblastine, cis-platinum, and bleomycin].

Combination therapy consisting of vincristine, actinomycin-D, and cyclophosphamide with or without adriamycin is the most common chemotherapy for rhabdomyosarcoma in childhood. But the effective chemotherapy for rhabdomyosarcoma resistant to these four drugs has not been established. We report a case with rhabdomyosarcoma, which was resistant to these four drugs but responded completely to three drug combination chemotherapy consisting of vinblastine, cis-platinum, and bleomycin (VPB therapy). A 11-months-old boy was referred to us because of giant abdominal tumor. Postoperative diagnosis was Group III embryonal rhabdomyosarcoma of the urinary bladder. Partial resection was followed by vincristine, actinomycin-D, cyclophosphamide, and adriamycin, but his residual tumor was growing. Then VPB therapy was administered and the first course of the chemotherapy reduced the size of tumor. After three courses of VPB therapy the second-look operation was performed. At operation no residual tumor was found and a complete remission was confirmed. During the course of VPB therapy no severe adverse effect was detectable.

Bleomycin↗
PubMed1983

[Labial embryonal rhabdomyosarcoma. Value of cell culture and electron microscopy, histogenesis].

The authors report the case of a labial embryonal rhabdomyosarcoma of a baby. This appears to be an exceptional localization. The study of the first biopsy which was too superficial leads to the thought of a capillary angioma. At the age of 6 months the lesion is 35 mm in diameter, a lobulated formation, typically botryoid. The excision after a frozen section control was in healthy tissue. At the age of 10 months, there is a local relapse with a nodule of 18 mm with left submandibular lymph nodes. A second surgical operation allows the culture of the tumoral tissue. The conventional histopathological examinations shows the aspect of an embryonal rhabdomyosarcoma. On the ultrastructural examination one can visualize in some cells an intracytoplasmic filamentous material. The cellular proliferation in culture, after May Grunwald Giemsa coloration is monomorphous, spindle shaped and of fibroblastic aspect. The ultrastructural study of this cellular proliferation after trypsinization on the 7th day shows some cells including an intracytoplasmic filamentous material. On the data of the cellular culture, the ultrastructural studies and the review of the literature, the authors discuss the possibility of the relationship between the cells of embryonal rhabdomyosarcomas and the fibroblasts and myofibroblasts. Embryonal sarcoma seems to be a better denomination than embryonal rhabdomyosarcoma, as regards the histogenesis.

Age Factors↗
PubMed1983

Immunohistochemical study of alveolar and embryonal rhabdomyosarcoma.

Paraffin-embedded sections of 11 alveolar and 12 embryonal rhabdomyosarcomas, 12 lymphomas, five neuroblastomas, five extraskeletal neoplasms resembling Ewing's sarcoma, and six epithelial tumors were tested for immunoreactivity against myosin, myoglobin, and isozymes BB and MM of creatine kinase with a peroxidase-antiperoxidase method. Of the 23 cases of rhabdomyosarcomas 17 were positive for at least three of the antigenic determinants. In contrast, the other investigated tumors were consistently negative for all markers, with the exception of breast and prostatic carcinomas. Our results establish that the presence of three or four of the above markers in a tumor is strongly suggestive of a rhabdomyosarcoma and helpful in the distinction of alveolar and embryonal rhabdomyosarcomas from lymphomas, neuroblastomas, and extraskeletal neoplasms resembling Ewing's sarcoma.

Adolescent↗
PubMed1984

The ultrastructure of alveolar and embryonal rhabdomyosarcoma. A correlative light and electron microscopic study of 17 cases.

An ultrastructural study is presented of 8 alveolar rhabdomyosarcomas and 8 embryonal rhabdomyosarcomas, and one mesenchymoma with predominantly rhabdomyoblastic differentiation. Thin and thick myofilaments and Z-lines or Z-like densities were found in all. Intermediate filaments were found in 4 alveolar and 4 embryonal rhabdomyosarcomas. A correlation was noted between the light- and electron microscopic appearance of the tumours regarding the degree of differentiation. A wide differentiation spectrum of the tumour cells was observed which could be correlated partly with normal fetal myogenesis. Ultrastructural differences were demonstrated in the alveolar and embryonal types. Electron microscopy is considered to play an important role in the characterization and diagnosis of rhabdomyosarcoma and is a valuable tool in the differential diagnosis.

Adult↗
PubMed1995

[The initial clinical manifestations of rhabdomyosarcoma].

Unilateral proptosis, dislocation of the globe and impairment of motility in childhood are known to be typical presenting signs of rhabdomyosarcoma of the orbit. In a retrospective analysis of 17 patients with rhabdomyosarcoma (1974-1993) only 8 patients presented with typical signs. 9 patients did never show the typical signs which initially lead to a wrong diagnosis in many cases. A 5-year-old boy complained of a conjunctival cyst in the lower fornix of the left eye. Two weeks later the cyst was replaced by a solid tumor. The tumor was not completely resectable, histology revealed an embryonal rhabdomyosarcoma. Presenting signs and symptoms of orbital rhabdomyosarcoma show a sometimes misleading variety including conjunctival cyst formation. In any recent onset of unilateral rapidly progressing alterations of the lid, the conjunctiva or the caruncle in childhood the physician should consider a rhabsomyosarcoma of the orbit.

Adolescent↗
PubMedDate not supplied

Meningeal rhabdomyosarcoma. Report of a case with cytologic, immunohistologic and ultrastructural studies.

Primary rhabdomyosarcoma of the meninges, a very rare brain tumor, is reported. Cytologic findings by squash preparation were useful as an adjunct to frozen section diagnosis during surgery. The cytologic features of rhabdomyosarcoma without cross-striation have some similarities to those of gemistocytic astrocytomas and anaplastic meningiomas, but the cytoplasmic filaments of rhabdomyosarcoma are different from those of gemistocytic astrocytoma and anaplastic meningioma. Histologically the tumor was embryonal rhabdomyosarcoma with a partially botryoid pattern. The tumor cells have no cross-striations but react positively to antimyoglobin serum on immunoperoxidase staining. In this case, intracytoplasmic filaments resembling poorly formed myofibrils were found on electron microscopic study. The histologic and immunohistologic findings suggest that this rare mesenchymal malignancy might arise from primitive totipotential cells.

Adult↗
PubMed1994

Rhabdomyosarcoma in adults.

Rhabdomyosarcomas occur in adults in one of two forms; as sporadic cases of the juvenile histological types and more commonly, if controversially, as the major adult form, pleomorphic rhabdomyosarcoma. This review documents the historical background to the fluctuating diagnostic popularity of pleomorphic rhabdomyosarcoma, together with a presentation of its defining clinicopathologic characteristics, and presents the clinicopathologic features of the juvenile types as they occur in adults. The concept of pleomorphic rhabdomyosarcoma as a distinct clinicopathologic entity, presenting as an aggressive, predominantly spindle-cell tumor arising in the skeletal musculature of middle-aged adults and defined by the presence of large, pleomorphic tumor cells, which show, at least focally, immunophenotypic or ultrastructural sarcomeric muscle differentiation, is discussed and supported.

Adult↗
PubMed1993

Actin, tropomyosin and alpha-actinin as markers of differentiation in human rhabdomyosarcoma cell lines induced with dimethyl sulfoxide.

Most rhabdomyosarcomas are poorly differentiated malignant tumors. Dimethyl sulfoxide has been shown to modulate cell differentiation in cultured human cells. We induced differentiation in human rhabdomyosarcoma cell lines A-673, RD and A-204 with 1.25% dimethyl sulfoxide, and used desmin, the protein most frequently used as a marker of muscle cell differentiation, to trace this process. As alternative markers of the degree of differentiation, we quantified the expression of the proteins actin, tropomyosin and alpha-actinin in these cell lines, and followed the changes in expression of these proteins after induction for 8, 12, 24, 48 and 72 hrs. In the process of differentiation, protein expression in both the cytoplasm and cytoskeleton was significantly increased by treatments lasting 12 hrs. (alpha-actinin) and 24 hrs. (actin). On the basis of our results, alpha-actinin can be considered as an earlier marker of differentiation than actin in human rhabdomyosarcoma cell lines. However, the earliest indication of differentiation was a modification in desmin expression (8 hrs.). Because changes in tropomyosin expression were less marked, we consider this protein as a poor marker of rhabdomyosarcoma cell differentiation.

Actinin↗
PubMed1996

Expression of transduced carcinoembryonic antigen gene in human rhabdomyosarcoma inhibits metastasis.

Carcinoembryonic antigen (CEA) is a highly glycosylated cell surface glycoprotein belonging to the immunoglobulin superfamily. CEA has been involved in vitro in adhesion mechanisms, but little is known about the function of this glycoprotein in vivo in normal tissue differentiation and malignancy. With regard to the relationship between CEA expression and tissue differentiation, it has been reported that transfection of the CEA gene in rat L6 myoblasts results in a complete block of myogenic differentiation. To extend investigations to the transformed myogenic counterpart and examine CEA effects on differentiation and malignancy outside the colon system, we have transfected the human CEA gene in human rhabdomyosarcoma cells. Human rhabdomyosarcoma cells transfected with the CEA gene correctly expressed membrane CEA anchored via glycosylphosphatidylinositol and secreted CEA in the medium. CEA gene transfer in human rhabdomyosarcoma cells, which display a limited differentiation ability, does not further inhibit myogenic differentiation or alter in vitro proliferation or natural killer sensitivity. CEA transfection does not affect s.c. growth in nude mice, but the ectopic expression of CEA in human rhabdomyosarcoma cells can strongly inhibit their metastatic ability to lungs and adrenals after i.v. injection. The impairment of metastatic potential correlates with a reduction in the homotypic adhesion properties of the cells. These data suggest that CEA, in some systems, can interfere with intercellular adhesion and, at least for cells not metastatic to the liver, can act as an anti-metastatic molecule.

Adrenal Gland Neoplasms↗
PubMedDate not supplied

Rhabdomyosarcoma of the head and neck in adults: MR and CT findings.

PURPOSE: To evaluate imaging findings of rhabdomyosarcoma of the head and neck in adults. METHODS: We examined 11 patients (seven men and four women; 17 to 73 years old) with pathologically proved rhabdomyosarcoma of the head and neck. The tumors originated in the paranasal sinuses (n = 6), cheek (n = 3), nasal cavity (n = 1), and infratemporal fossa (n = 1). Eight of the rhabdomyosarcomas were of the embryonal type, two were pleomorphic, and one was alveolar. Necrosis was seen in four patients, but calcification or intratumoral hemorrhage was not found. Two tumors had nodal extension. Contrast-enhanced CT was performed in 10 patients, and two patients had contrast-enhanced MR imaging. RESULTS: On CT scans, the masses enhanced to the same degree as adjacent muscle. The masses showed a homogeneous pattern in six cases and a heterogeneous pattern in four cases. The tumor margins were poorly defined in eight cases. On MR images, the masses were homogeneously isointense with muscle on T1-weighted studies and were hyperintense relative to muscle on T2-weighted studies. On both CT and MR images, 10 of 11 cases showed poorly defined, homogeneous masses destroying adjacent bony structures. CONCLUSIONS: MR imaging seems to be better than CT for initial and follow-up examination of patients with rhabdomyosarcoma because of its multiplanar capability and because it more precisely defines the extent of tumor.

Adolescent↗