| A 6-year-old male presented with headache, vomiting, visual obscuration, and papilledema. Clinical presentation, initial laboratory data, and radiologic evaluation suggested a diagnosis of pseudotumor cerebri. The development of lower cranial nerve palsies after transient resolution of symptoms prompted reassessment of the diagnosis, which revealed retropharyngeal rhabdomyosarcoma involving the right jugular vein. cerebral angiography demonstrated the complete venous occlusion at the jugular foramen. Careful neurologic follow-up is essential in a patient with pseudotumor cerebri or idiopathic intracranial hypertension. ( info) |
12/140. Analysis of p53 and K-ras genes and their proteins in a sarcoma botryoides of the uterine cervix. Several data indicate that the activation of oncogenes and growth factors as well as inactivation of the tumor suppressor genes are implicated in the development of human neoplasms, including sarcomas. In the present study we described a case of the extremely rare, but highly malignant neoplasm of the female genital tract known as sarcoma botryoides of the uterine cervix and assessed, using molecular and an immunohistochemical analysis, p53 and K-ras alterations in the tumor. A point mutation in exon 6 of the p53 tumor suppressor gene was found but no K-ras gene point mutations at codons 12, 13 and 61 were detected using molecular analysis. p53 protein was overexpressed in more than half of the neoplastic cells, however, ras p21 protein expression was not immunohistochemically detected. Our data indicate that p53, but not K-ras gene alterations may play a role in the development and progression of sarcoma botryoides of the uterine cervix. ( info) |
| Primary rhabdomyosarcoma of the diaphragm presenting as an epigastric mass is extremely rare, with only three published cases. We highlight the unique imaging features in a 2(1)/(2)-year-old boy which predicted the correct anatomical site preoperatively. awareness of this rare tumour and its imaging characteristics should help in differentiating it from other more common tumours in this location, especially primary hepatic tumours. ( info) |
| We report a case of rhabdomyosarcoma of the bladder in an adult. A 73-year-old male was referred to our hospital due to macrohematuria. ultrasonography, CT and MRI revealed a bladder tumor, and cystoscopic biopsy of the tumor pathologically revealed rhabdomyosarcoma. Total cystectomy with ileal conduit urinary diversion was performed. copyright copyright 1999 S. Karger AG, Basel ( info) |
15/140. Embryonal rhabdomyosarcoma of the uterine corpus in a 76-year-old patient. BACKGROUND: Primary embryonal rhabdomyosarcoma of the uterine corpus is an extremely rare tumor. A case of rhabdomyosarcoma originating in the uterine myoma is presented with a review of the literature. CASE: A 76-year-old female presented with a half-year history of abdominal swelling and fever of unknown origin. A 15 x 15 x 17 cm myoma-like tumor was noted on the anterior wall of the uterine myometrium. The patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. The histology and immunohistochemistry aided in the final diagnosis of a pure embryonal rhabdomyosarcoma of the uterine corpus. This patient received three courses of CYVADIC chemotherapy consisting of cyclophosphamide, vincristine, doxorubicin, and dacarbazine after the surgery and is now alive with no evidence of disease 10 months from her surgery. CONCLUSION: This case is extremely rare with respect to the uterine corpus origin, especially the myoma nodule and the age of the patient. ( info) |
| Intra-oral masses in neonates can seriously compromise the airway, potentially causing hypoxia and death if not recognized and managed appropriately. We report a case in which an intra-oral mass was diagnosed on antenatal ultrasound scan. Preparation for delivery involved a multidisciplinary team approach, with a strategy for management at delivery. The child was delivered by elective Caesarean section and had a patent airway. A tracheostomy was performed immediately after delivery. The infant underwent a debulking procedure 3 weeks after birth. A histological diagnosis of embryonal rhabdomyosarcoma was made and a course of chemotherapy commenced. The child had a partial response to treatment with considerable shrinkage of the tongue mass. We discuss the management options in neonates with intra-oral masses to provide an adequate airway and maintain fetal oxygenation. The differential diagnosis of fetal oral masses is reviewed. ( info) |
17/140. Rabdomyosarcoma of the biliary tree. Rabdomyosarcoma of the biliary tree is one of the rare causes of biliary tract obstruction in childhood. Nevertheless it is the most common cause of obstructive jaundice due to neoplastic biliary obstruction. We present a two-year-old child with obstructive jaundice secondary to an embryonal rhabdomyosarcoma of the biliary tree. She underwent surgery and, after total excision of the mass, a hepaticojejunostomy and Roux-en-Y anastomosis were performed. She was referred to the Pediatric Oncology Group for follow-up. rhabdomyosarcoma of the bilary tree, although rare, must be considered in the etiology of obstructive jaundice in children. ( info) |
18/140. Rapid growth of a retroperitoneal rhabdomyosarcoma following right hemicolectomy for ascending colon cancer: report of a case. We report herein the case of a 69-year-old man in whom rapid growth of a retroperitoneal rhabdomyosarcoma occurred following hemicolectomy for ascending colon cancer. On his first admission for surgery, a small lesion, 1.5 cm in diameter, was detected adjacent to the inner side of the left kidney by abdominal axial computed tomography (CT), which was initially suspected to be a benign lesion; however, a postoperative follow-up CT scan done 5 months later revealed that the lesion had enlarged remarkably to 8 cm in diameter. Thus, total resection was performed under the presumed diagnosis of a malignant retroperitoneal tumor. The tumor was found to be adjacent to the inner portion of the left kidney and covered by Gerota's fascia. As it involved the ileolumbar muscle and had a metastatic lymph node, complete resection was performed. The resected specimen was 8.5 x 6.5 x 5 cm in size and was histologically confirmed as a retroperitoneal rhabdomyosarcoma of embryonal type. Two courses of adjuvant chemotherapy with adriamycin, vincristine, and cyclophosphamide were given, and the patient has shown no signs of recurrence for 2 years since his second operation. ( info) |
19/140. Embryonal rhabdomyosarcoma and chromosomal breakage in a newborn infant with possible Dubowitz syndrome. We report on a newborn girl with Dubowitz syndrome (DS) and embryonal rhabdomyosarcoma (ERMS), with multiple chromosomal breakage (MCB). The tumor was resected but recurred in a few months, resulting in the infant's death. Malignancy and chromosomal breakage have been reported previously in DS. However, ERMS has not been reported among the malignant tumors diagnosed in DS. To our knowledge, concurrence of DS, ERMS, and MCB has not been reported previously. This is the first observation of DS in the Arab ethnic group. ( info) |
20/140. The vulvar mass in the prepubertal child. The differential diagnosis of the vulvar mass in the prepubertal patient is extensive and reported cases of a vulvar hamartoma in the literature are limited. The case presented in this work and review of the literature demonstrate that when considering the differential diagnosis of vulvar masses in the prepubertal female, hamartoma should be included. This review outlines the differential diagnosis of the vulvar mass and gives a comprehensive review of benign masses arising from embryonic remnants, those of mesenchymal origin as well as sarcoma botryoides-embryonal rhabdomyosarcoma and the embryonal sinus tumor. ( info) |