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Botryoid rhabdomyosarcoma of the prostate is a rare tumour in children, mostly below 6 years of age. A case of 5 year old male child presenting with acute retention of urine due to botryoid rhabdomyosarcoma of the prostate is described.
A case of botryoid-type embryonal rhabdomyosarcoma of the renal pelvis in a 49-year-old woman is reported. The tumor led to hydronephrosis. The surgical resection specimen disclosed a translucent, polypoid mass attached to the wall of the renal pelvis by thin stalk. Light-microscopic examination revealed a large exophytic polypoid tumor with intact surface epithelium, which was negative for dysplasia or carcinoma in situ. There was a condensation of epithelioid to spindle cells underneath the basement membrane, forming a cambium layer. The core of the lesion contained interspersed epithelioid to spindle cells with myxoid change and edema. Cells of the cambium layer as well as interspersed cells in the core exhibited marked cytologic atypia with mitotic figures. Immunohistochemical stains for cytokeratin, S-100 and myoglobin were negative, stains for desmin and actin were positive. Although botryoid-type embryonal rhabdomyosarcomas have been reported to occur at various sites in the genital tract and lower urinary tract, to our knowledge, this is the first reported case of the tumor within the renal pelvis. Also, the occurrence of these tumors in adults is quite rare.
We report a case of intrascrotal rhabdomyosarcoma in a 59-year-old-male. The patient with a mass in the right scrotum was at first diagnosed with epididymitis and treated with antibiotics. The mass however, grew gradually and right orchiectomy revealed intrascrotal alveolar rhabdomyosarcoma. Para-aortic lymph node metastasis was pointed out by abdominal computed tomography. He received multidrug chemotherapy consisting of vincristine, actinomycin-D, cyclophosphamide, adriamycin, and cisplatin (IRS-III regimen 35). A complete response was obtained 3 months after the start of the chemotherapy. The patient is alive without recurrence 2 years after the surgery.
The authors report a case of rhabdomyosarcoma in a 17 year old boy which recurred after combination chemotherapy and radical prostatectomy, followed by death of the patient due to haematological complications of second-line chemotherapy. The diagnosis of these rhabdomyosarcomas is facilitated by the various immunohistochemical stains now available. Chemotherapy is undeniably effective and, combined with surgery and radiotherapy, ensures a 5-year survival of 47% to 93% depending on the stage. The first-line chemotherapy avoids the need for radiotherapy in the early stages and allows less mutilating surgical procedures, while maintaining the survival rate.
Significant progress has been made in the 1980s in early-stage vulvovaginal rhabdomyosarcoma, diethylstilbestrol (DES) clear cell adenocarcinoma of the vagina, and unilateral germ cell tumors of the ovary. In an early state of vulvovaginal rhabdomyosarcoma, systemic vincristine, dactinomycin, and cyclophosphamide (VAC) chemotherapy followed by local excision or local radiation results in a high cure rate with retention of future fertility. Similarly, early-stage DES-related adenocarcinoma of the vagina treated by wide local excision and localized vaginal radiation also results in retention of fertility and a high cure rate. Finally, significant progress has been made in unilateral germ cell tumors of the ovary in which surgical treatment by unilateral salpingo-oophorectomy followed by cisplatin, etoposide, and bleomycin results in not only high cure rates and retention of fertility but will probably be standard therapy for all germ cell tumors of the ovary, including dysgerminoma, a disease most frequently treated in the past by radiation therapy with loss of subsequent fertility.
Intestinal localization of rhabdomyosarcoma is exceptional, this case is the first to be published in the world literature. A 35-year-old patient with abdominal pain, fever, was found to have an infiltrative white-grey tumour, involving 20 cm the jejunoileal wall and also the surrounding mesenterium up to the origin of upper mesenterical vessels and lymph nodes. Histologic examination showed an alveolar type of rhabdomyosarcoma intricated with solid undifferentiated tumoral cells. The presence of multinucleated giant cells and the positivity of protein S 100 reaction was important for differential diagnosis, given the alveolar soft part sarcoma, malignant mesothelioma, malignant melanoma or papillary carcinoma.
Primary rhabdomyosarcoma can arise in the skin, but there are few reports on this common childhood malignancy in the dermatological literature. We report on a male infant with a cutaneous tumour growing on the right nasal bridge since his 10th week of life. Clinically the tumour mimicked pilomatrixoma. Histological and immunohistological examination of the skin tumour and of subsequent lymph node metastases revealed rhabdomyosarcoma of the alveolar growth pattern. Our patient died at the age of 4 years of disseminated organ metastases.
We report the case of a 6-week-old girl who presented with a pedunculated embryonal rhabdomyosarcoma arising in a giant congenital melanocytic nevus (GCMN) on her lower back. There was no associated leptomeningeal involvement. The patient underwent surgical resection of the rhabdomyosarcoma at age 2 months, with subsequent chemotherapy consisting of actinomycin D and vincristine. No recurrences or metastases of tumor have been noted at 5 months of age.
Human tumor cell lines that do not express O6-methylguanine-DNA methyltransferase (MGMT) in detectable quantities (Mer-) are hypersensitive to the effects of O6-guanine-alkylating agents. Because the Mer- phenotype enhances tumor response to such agents, we investigated possible mechanisms involved in regulation of MGMT expression in a panel of Mer+ and Mer- pediatric rhabdomyosarcoma xenograft and cell lines. All Mer- cell and xenograft lines lacked not only MGMT activity but also the protein and mRNA as well, suggesting that its expression is transcriptionally regulated. Transfection of Mer+ and Mer- rhabdomyosarcoma cell lines with MGMT gene promoter-CAT constructs yielded similar levels of CAT expression, indicating that Mer- cells possessed the necessary factors to support transcription. Methylation in the 5'-untranslated region of the MGMT gene was assayed by Southern analysis using methylation-sensitive restriction enzymes. Digestion with HpaII and its methylation-insensitive isoschizomer, MspI, revealed little overall correlation between methylation and MGMT expression. However, methylation in a single SmaI site at position-69 was observed in all MGMT deficient lines but not in any MGMT expressing lines. These results suggest that methylation of specific cytosines in the MGMT promoter may play a role in suppressing its expression, as well as being a potentially useful marker for the Mer- phenotype.
OBJECTIVE: To study the expression of fragile histidine triad (FHIT) protein in rhabdomyosarcoma(RMS) and the possible mechanisms of its effect on tumor. METHODS: Immunohistochemical technique(SP) was used to detect the expression of FHIT in 44 cases of RMS and 20 cases of normal skeletal muscles. RESULTS: Reduced expression of FHIT was 68.2% (30/44) in the cases of RMS, and was 35% (7/20) in the cases of normal skeletal muscles; a significant difference was seen between the two groups(P < 0.05). There was no significant relationship of the expression of FHIT with the sex of the patient and the histological type and grade of the tumor(P > 0.05). However, a significant relationship was observed between the expression of FHIT and the prognosis of the tumor in respect to relapse and metastasis of tumor(P < 0.05). CONCLUSION: The reduced expression of FHIT may play an important role in the development and progression of rhabdomyosarcoma, and thus may become a new prognostic marker in RMS.
Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood, but it is rare in young adults. It usually occurs in the pelvis or genito-urinary tract. We report a case of abdominal embryonal rhabdomyosarcoma who received a combined treatment modality with radical pelvic surgery and combination chemotherapy of vincristine, actinomycin-D and cyclophosphamide (VAC). She is well with no evidence of tumour recurrence for 3 years after primary surgery.
We report the case of a special form of rhabdomyosarcoma called "lymphadenopathic" which was reported only two times in the literature. This lymphadenopathic alveolar RMS shows lymph nodes as clinical manifestation mimicking lymphoma with an unknown primary neoplasm. In the case we report, clinical examination, and radiological studies revealed superficial and deep located lymph nodes in association with bilateral pleural effusion. Conventional histological examination showed lymph node infiltration by a metastatic tumour having alveolar pattern. Some of tumour cells were round with multilobed off-centred nucleus and eosinophilic cytoplasm containing fibril material with longitudinal and transverse striation indicating a muscle nature of the tumour proliferation. The immunohistochemical study showed intense and diffuse immunoreactivity of tumour cells for vimentin and a discreet immunostaining by desmin. These findings were compatible with the diagnosis of lymph node metastasis by alveolar rhabdomyosarcoma.
OBJECTIVE: To study the clinicopathological, immunohistochemical and electron microscopic characteristics of pediatric rhabdomyosarcomas (RMS). METHODS: One hundred and forty-five cases of pediatric rhabdomyosarcomas were studied by routine histological, immunohistochemical and electron microscopic studies. RESULTS: There were 97 male and 48 female patients with ages ranging from 4 months to 13 years and a mean of 4.2 years. The follow-up period of 100 patients was from 1 year to 20 years with a mean of 5 years after diagnosis. All cases were subtyped into the following histological categories: embryonal RMS, botryoid RMS, spindle cell RMS, alveolar RMS and solid RMS. Histopathological subtypes, tumor site and tumor stage correlated significantly with the patients' 5 years survival. The best prognosis was observed in spindle cell and botryoid RMS. Embryonal RMS carried an intermediate prognosis. Patients with alveolar RMS and solid RMS had the worst prognosis. Tumors involving bladder, head and neck carried a favorable clinical outcome. Patients with tumors involving trunk extremities retroperitoneum and pelvis did poorly. Immunohistochemically, all cases were positive for Vimentin. The positive staining rates for desmin, SMA and myoglobin were 78%, 75% and 37%, respectively. All tumors were negative for NSE, CD99 and LCA. Electron microscopy study showed features of myofilament and sarcomere in 10 of 15 cases. CONCLUSIONS: RMS is the most common soft tissue sarcoma of childhood. Immunohistochemistry and electron microscopy are helpful in diagnosis and classification of RMS.
The case of nasopharyngeal rhabdomyosarcoma was presented. 58-years old woman complained of sore throat, dysphagia and temporary hoarseness was treated in Department of Otolaryngology Medical Academy in Warsaw. The nasopharyngeal tumor extended between Rosenmüller's fossa level to base of the tongue, 3x2 cm in size. Because biopsy specimen result was not definite, the tumor was surgical removed wholly. Histopathological diagnosis was: rhabdomyosarcoma embryonic. Chemotherapy and radiotherapy followed surgery. At the present moment, four years after treatment, the patient is without local recurrence and metastases. There was two important reasons to present this case: 1) the tumors originate from embryonal tissues are the most frequent of childhood, they are rare in patient over 30 year, 2) the most common sites in head and neck are the orbit, nasopharynx, middle ear, nasal cavity and paranasal sinuses. The pathology of the tumor, clinical presentation, diagnosis and treatment has been discussed after literature review.
Pleomorphic rhabdomyosarcoma of the diaphragm represents fewer than 0.0001% of all cancers. One case is reported, and a total of eight cases to date are reviewed. It is an adult disease of both sexes, the lowest reported age being 14 years. The tumor presents at a late stage, and the average symptom-death interval is only a few months. Early diagnosis and wide excision are essential along with radiotherapy and chemotherapy before any survival--much less worthwhile survival--can be hoped for. There is evidence of host immune response against rhabdomyosarcomas. Immunotherapy, therefore, should be considered. For assessment of the biological behavior of the tumor and the treatment, standardization of staging and grading are essential, although there is some evidence that the prognosis of primary and recurrent tumors treated radically are similar.
The authors present the results obtained in 14 children affected with orbital rhabdomyosarcoma, who were studied between 1979 and 1991 at the I Dept. of Radiology and Radiotherapy of the II Policlinico, Naples, Italy. The patients (age range: 14 months to 15 years) had received radiotherapy and chemotherapy: the dose was 6000 cGy to the orbital region (one case was given 4500 cGy and then interrupted radiotherapy for personal reasons). Twelve children underwent a standard protocol, that is biopsy followed by immediate irradiation and chemotherapy; surgery was performed in 2 cases. 90% survival at 2 years was obtained in our series. All 14 patients are alive and free of detectable disease, after a minimum of 7 to a maximum of 126 months from diagnosis. In 3 children a locoregional relapse was observed 12, 15, and 16 months after clinical diagnosis. They have received a new cycle of radiation therapy and are now free of detectable disease. In all children, ocular structures have been spared, and the complications observed so far have been few. Therefore, the authors suggest that the combination of immediate radiotherapy and chemotherapy might represent an optimal tool for the treatment of orbital rhabdomyosarcomas.
BACKGROUND: Transforming growth factor-beta (TGF-beta) can inhibit the growth of most epithelial and endothelial cells. The growth regulative role of TGF-beta on soft tissue sarcoma was seldom reported. Here we examined TGF-beta1 effects on the growth of human rhabdomyosarcoma cell RD and searched the relative molecular mechanism. METHODS: The viability of RD was examined by [(3)H]-thymidine incorporation and [3-(4,5-dimethylthiazol-z-yl)-2,5-diphenyl tetrazolium bromide] (MTT) assay. RD cell cycle was analysed by flow cytometry. The protein and mRNA of cell cycle regulative factors in RD were detected by Western blot and reverse transcription-polymerase chain reaction (RT-PCR), respectively. The kinase activity of cdk2 or cdk4 was examined by immunoprecipitation and kinase assay. Immunofluorescent staining was used to detect the location of cell cycle regulative factors in RD by laser scanning confocal microscope. RESULTS: TGF-beta1 inhibits RD proliferation by G1-arrest in cell cycle progression. TGF-beta1 can prominently up-regulate P27 of RD, then augment P27 to bind cyclinE-cdk2 complexes, which effectively suppress cdk2 kinase activity. P21 increased and c-myc decreased in RD due to TGF-beta1. Both P15 and cdk4 have not been involved in the growth inhibitory event. TGF-beta1 treatment induced P27 to congregate around nucleus. P21 pervaded from nucleus to both nucleus and cytoplasm by TGF-beta1 treatment. CONCLUSION: TGF-beta1 inhibits the proliferation of human rhabdomyosarcoma cell line RD and induces RD G1-arrest. This course is accomplished by TGF-beta1 up-regulating P27 to suppress cdk2 kinase activity. The induction of P21 and down-regulation of C-myc might participate in the growth-arrest event.
Overall five-year survival of Childhood rhabdomyosarcoma (RMS) is reported to be 70% in the Intergroup Rhabdomyosarcoma Study Group (IRSG), however, the figure in Japan is almost 15% lower than that of IRSG. Treatment regimen of RMS essentially depends on the histology of the tumor, site, preoperative staging and postoperative grouping that leads to the risk classification. VAC is a standard chemotherapeutic regimen for low and intermediate risk group. Clinical trial with CPT-11 or high dose chemotherapy are underway for high risk RMS.