1/5. rhabdomyosarcoma in a child with massive pleural effusion: cytological diagnosis from pleural fluid.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.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/5. Embryonal rhabdomyosarcoma of the uterus in a postmenopausal woman. Case report and review of the literature.BACKGROUND: Embryonal rhabdomyosarcoma is a rare sarcoma which characteristically occurs in non genitourinary sites in children. CASE: We present a case of uterine embryonal rhabdomyosarcoma in a postmenopausal patient who presented with increasing abdominal girth, early satiety, weight loss, and pelvic pain. CONCLUSION: Embryonal rhabdomyosarcoma does not commonly originate from the uterine corpus, and it is rarely seen in postmenopausal patients. A review of the literature confirms the unique nature of this case.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/5. Phacomatosis pigmentokeratotica associated with hemihypertrophy and a rhabdomyosarcoma of the abdominal wall.Phacomatosis pigmentokeratotica (PPK) represents a specific "twin nevus" syndrome in which a speckled lentiginous nevus (SLN) is associated with an organoid nevus with sebaceous differentiation. A boy with a large nevus sebaceus on the left face and upper part of the trunk, a giant segmental SLN extending from the abdomen to the feet bilaterally, and right hemihypertrophy developed an embryonal rhabdomyosarcoma of the right abdominal wall at age 6 months. A variety of musculoskeletal, neurologic, and ocular anomalies have been observed in patients with PPK, reflecting the individual manifestations of both SLN and Schimmelpenning syndromes. This report adds hemihypertrophy to the spectrum of extracutaneous manifestations of PPK and, to our knowledge, represents the first observation of a rhabdomyosarcoma arising in contiguity with an SLN in a patient with PPK. The development of a rhabdomyosarcoma in our patient likely reflects both increased propensity for growth (as evidenced by the hemihypertrophy) and the pluripotent nature of neural-crest derived cells within the field defect that underlies an SLN.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/5. Two different manifestations of botryoid sarcoma (embryonal rhabdomyosarcoma) of the biliary tree.A 16-month-old girl and a 5-year-old boy with botryoid sarcoma of the biliary tree are presented. The girl's tumor infiltrated the liver, whereas the boy's tumor was confined to the extrahepatic biliary tree. Computed tomography not only enabled the evaluation of the tumor-surrounding structures, but also indicated the tumorous nature of the lesion following intravenous administration of a contrast agent. In cholangiograms, the exact extension of the intraluminal tumors could be differentiated by the typical bizarre filling defects.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/5. Perineal and perirectal sarcomas: report of two cases.PURPOSE: Perineal sarcomas are rare tumors that are typically of an extensive nature by the time of diagnosis. In this article, two case reports are followed by a brief review of different types of sarcomas that may occur in the perineal and perirectal region. STUDY patients: This study consists of two cases that are representative of the many types of perineal/perirectal sarcomas. PRINCIPLE CONCLUSIONS: Perineal and perirectal sarcomas are generally of poor prognosis mainly because of delayed diagnosis. Computed tomography and magnetic resonance imaging can be extremely useful to help assess these tumors. It is well known that the most important criterion for diagnosing leiomyosarcomas appears to be the presence of mitotic activity. Anorectal stromal tumors having five or more mitoses per 50 high powered fields are considered to be malignant. The mainstay of treatment is surgical excision with wide margins. Currently, chemotherapeutic and radiotherapy trials are under way.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |