1/140. Embryonal rhabdomyosarcoma with only numerical chromosome changes. Case report and review of the literature. An embryonal rhabdomyosarcoma, presenting as a retroperitoneal mass in a 15-year-old girl, is reported. The histological and immunohistochemical picture was typical, except for the presence of focal chondroid differentiation. Interestingly, expression of the "muscle markers" desmin and alpha-sarcomeric actin was present in the latter areas. cytogenetic analysis showed a hyperdiploid karyotype without structural chromosome changes. The pertinent literature on the subject is reviewed. Hyperdiploidy of the clonal type seems to occur frequently, but no characteristic karyotype is so far emerging. ( info) |
| The purpose of this paper is to present a child with embryonal rhabdomyosarcoma involving the left middle ear, who initially presented with unilateral facial paralysis. A 5-year-old boy presented with a 4-week history of left-sided facial weakness, associated with persistent otitis media on that side. Examination revealed complete left lower motor neuron facial weakness and hearing loss. A myringotomy revealed a soft tissue mass behind the tympanic membrane. biopsy and oncologic assessment confirmed a stage II, group III left middle ear embryonal rhabdomyosarcoma. Despite debulking surgery, local irradiation and multiple chemotherapeutic courses the child deteriorated quickly. He developed carcinomatous meningitis and died 9 months after his initial presentation. In conclusion, middle ear tumors should be considered in the differential diagnosis of unresolving otitis media, particularly when associated with persistent ipsilateral facial paralysis. An ear mass, discharge, facial swelling, or systemic symptoms may be initially absent despite the presence of this aggressive malignancy. Careful examination of the middle ear is recommended in children with facial weakness. A myringotomy incision may be necessary including a complete assessment of the middle ear cavity, particularly when there is no fluid return. ( info) |
3/140. Cervical sarcoma botryoides. A case report. BACKGROUND: Sarcoma botryoides is a rare gynecologic malignancy. Reported treatment protocols range from localized resection of the tumor to radical surgery and adjuvant chemotherapy. Recently, limited excisional surgery combined with chemotherapy has contributed to excellent survival rates and preserved bladder and rectal function. CASE: We report a case of cervical sarcoma botryoides with minimal invasion. The patient had primary surgical treatment with complete resection of the tumor without adjuvant chemotherapy. Four months after surgery she presented with extensive metastatic retroperitoneal disease and died two weeks later. CONCLUSION: The clinical course of this patient suggests that complete surgical resection in cases of localized disease is not adequate therapy for sarcoma botryoides. Adjuvant chemotherapy should always be used, even at a very early stage. ( info) |
4/140. Prenatal sonographic features of embryonal rhabdomyosarcoma. We describe a case of fetal rhabdomyosarcoma detected during the third trimester of pregnancy by prenatal sonography. At 33 weeks' gestation, sonography performed because of suspected polyhydramnios showed a solid mass of 120 x 54 mm arising from the anterior wall of the fetal thoracic cage. Another mass within the left maxillary area which originated from the left orbital floor was also detected. In the abdomen, there were multiple round masses in and around the liver. As the previous scan at 28 weeks had appeared normal, the multiple masses which became visible and enlarged rapidly in different locations led us to believe that there was fetal cancer. The most likely diagnosis was rhabdomyosarcoma (which was later confirmed), because it is the most prevalent soft-tissue tumor in children and may develop within or outside muscle anywhere in the body and at any age. Two other reported cases which were detected by prenatal ultrasound examination are also discussed. ( info) |
5/140. rhabdomyosarcoma (RMS) of the uterine cervix in two Ethiopian patients: case report. Most rhabdomyosarcomas (RMS) of the female genital tract occur in infants and young children as sarcoma botryoides. To our knowledge, RMS of the cervix has never been reported in ethiopia. Two patients with RMS of cervix in their second and third decades of life were seen in a short period of time span at Tikur Anbessa Hospital in the first half of 1996. Difficulties were encountered in reaching a diagnosis and appropriately managing the patients which necessitated the revision of previous biopsy result especially in the first patient. Both presented clinically with bloody vaginal discharge and cervical mass. RMS of the cervix deserves attention in a young patient with cervical mass and bloody vaginal discharge. The excised tissue should be subjected to histopathologic study. Treatment with polychemotherapy and/or radiation in addition to aggressive surgical approach should be made available to obtain good results. ( info) |
| A 10-month-old boy presented with a 6-week history of abdominal pain. The pain was due to a large, stage IV embryonal rhabdomyosarcoma of the urinary bladder. The rhabdomyosarcoma was found in association with neurofibromatosis 1 (NF1) manifesting multiple cafe au lait spots and bowing of the right calf. The diagnosis of NF1 had not been made before presentation. This case report is intended to heighten the awareness of the manifestations of NF1 and the possibility of developing a nonneuroectodermal tumor as a concomitant of NF1, and to emphasize the importance of timely diagnosis and treatment of such an NF1-associated malignancy. Reports of the epidemiologic evidence for rhabdomyosarcoma in children with NF1 are reviewed. ( info) |
7/140. Sarcoma botryoides of the cervix. Report of a case with cytopathologic findings. BACKGROUND: Cytologic findings of sarcoma botryoides were still equivocal because sarcoma botryoides of the uterine cervix is an extremely rare neoplasm, and few cases have been reported to date. CASE: A 17-year-old female was diagnosed with sarcoma botryoides of the uterine cervix. The entire vaginal canal was occupied with polypoid masses, which arose from the anterior lip of the uterine cervix, and the tumor was classified as group I (Intergroup rhabdomyosarcoma Study). After wedge resection and six courses of combination chemotherapy, the tumor recurred in the same location of the cervix as the primary lesion. touch smear of the polypoid mass formed loose clusters and also showed short spindle cells in a necrotic background. The nucleus of the tumor cells had a thin nuclear membrane, fine chromatin pattern and partly clear nucleolus, showing mild nuclear atypia. Immunohistochemically, some of the tumor cells showed positive staining for myoglobin and desmin. CONCLUSION: The cytologic findings of sarcoma botryoides of the female genital tract are typical features of nonepithelial malignant tumor. Immunohistochemical study is useful for the diagnosis of rhabdomyosarcoma. ( info) |
8/140. Conservative treatment followed by chemotherapy with doxorubicin and ifosfamide for cervical sarcoma botryoides in young females. Sarcoma botryoides of the cervix is an extremely rare tumour and seems to be associated with a better prognosis than its vaginal counterpart. Recent studies have suggested that it is possible to limit surgery to local excision in stage I cases. We report three cases of young subjects treated successfully with polypectomy or diathermy loop excision followed by adjuvant chemotherapy. One patient had a local recurrence which was treated with further local excision. All subjects remain alive without evidence of recurrence and with normal menstrual function 36, 38 and 38 months following initial diagnosis. A conservative surgical approach to early cervical sarcoma botryoides is possible. The efficacy of adjuvant chemotherapy and the regimen of choice still need to be investigated. ( info) |
| rhabdomyosarcoma is an uncommon neoplasm in the adult population. Sporadic cases of primary rhabdomyosarcoma arising in the abdomen have been reported, but these cases are limited almost exclusively to the pediatric population. We report a well-documented case of primary intra-abdominal rhabdomyosarcoma in a 57-year-old woman. The patient presented with a pelvic mass and an elevated serum CA 125 and was referred to gynecologic oncologists at our institution for a presumed primary gynecologic malignancy. Intraoperatively, amorphous gelatinous tumor comprised a large portion of the peritoneal cavity. Surgical exploration of the abdomen failed to implicate any specific organ as the site of origin of the tumor. The overall histologic pattern of the resected tumor was most consistent with embryonal type rhabdomyosarcoma. To our knowledge this is the first well-documented case report of non-hepatobiliary, adult, intra-abdominal embryonal rhabdomyosarcoma in the English language literature. The presentation of a rare adult sarcoma mimicking a gynecologic malignancy was an unusual feature that complicated the diagnosis in this case. ( info) |
| We report on a case of embryonal rhabdomyosarcoma in an 11-yr-old boy investigated for mild chest pain after trauma. Chest radiography showed a massive right pleural effusion. Cytological analysis of the pleural fluid demonstrated the presence of malignant small undifferentiated cells. The rhabdomyoblastic nature of the cells was confirmed by positive immunostains of HHf35 actin, desmin, and skeletal muscle myosin; histological examination of a core biopsy confirmed the diagnosis of rhabdomyosarcoma. Computed tomography and gallium scan revealed the presence of an extensive anterior and lower chest wall mass involving the mediastinum, as well as retroperitoneal lymphadenopathy. Massive pleural effusion is a frequent presentation in malignant disease, but is rare in rhabdomyosarcoma. The immunochemical stain studies performed on cytological smears in this case proved to be very useful for making the definitive and accurate diagnosis. Diagn. Cytopathol. 1999;21:125-128. ( info) |