Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Perineal sagittal approaches (posterior sagittal anorectoplasty and anterior and posterior sagittal transanorectal approaches) allow complete anatomic exposure of the perineum and lower pelvis. Moreover, they reduce the risk of damaging important structures because the incision is led in the midline. Therefore, many surgeons have used these approaches to treat diseases other than anorectal malformations (ARM), including intestinal dysganglionosis, trauma, pseudohermaphroditism, presacral mass, and rectal duplication. The aim of this study was to describe a small series of patients operated on via these approaches at Gaslini Children's Hospital over a 5-year period. We retrospectively evaluated 10 patients consecutively operated on via a perineal sagittal approach, with or without sphincteric structure involvement, between January 1997 and December 2001. All of these patients were without ARM. Indications included retrorectal abscesses (two), iatrogenic anal canal stenosis (one), postinflammatory anal canal stenosis (one), internal anal sphincter neurogenic achalasia (one), female pseudohermaphroditism (one), benign sacrococcygeal teratomas (two), malignant sacrococcygeal teratoma (one), and perineal rhabdomyosarcoma (one). Protective colostomy was used in four patients. The parameters that we analysed included technical details, possible complications, perineal cosmetic appearance, and outcome. No complications were experienced. The postoperative cosmetic perineal appearance was excellent in all patients, and continence, when assessed, was always considered satisfactory. All tumours underwent complete gross resection. However, one patient with malignant sacrococcygeal teratoma died as a result of the malignant process 2 years after surgery. Although our study was carried out on a small series of patients, it confirmed that perineal sagittal approaches can be used not only for ARM but also for other conditions involving perirectal pouches, presacral space, and urogenital structures, as these approaches are safe and provide excellent cosmetic results as well as satisfactory functional outcome. Although tumours can be treated via these approaches, outcome remains related to the nature and malignancy of the disease itself.
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PMID:Feasibility of perineal sagittal approaches in patients without anorectal malformations. 1551 90

A total of 15 cases of prepubertal testicular tumours were reported by the department of pathology, Government Medical College, Srinagar, Kashmir over a period of 15 years, from January 1984 to December 1998. The cases included yolk sac tumour (10 cases; 67%), teratoma [(mature) 2 cases (13%)], rhabdomyosarcoma [(paratesticular) 2 cases (13%)] and NHL-Burkitt's lymphoma [one case (7%)]. The youngest patient was 10 months old and the oldest was of 14 years age. Ten cases (67%) occurred at or below the age of 4 years. The youngest patient had yolk sac tumour and oldest had rhabdomyosarcoma. In 2 cases both the testes were involved with one of these two cases having bilateral undescended testes.
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PMID:Prepubertal testicular tumours in Kashmir--a histopathological report of 15 cases. 1586 71

Two years after testicular resection was carried out in a 40-year-old man that revealed mixed germ cell tumor of more than one histological type (seminoma, embryonal cell carcinoma, and yolk sac tumor), he presented with an asymptomatic pulmonary nodule in his left lower lobe. Video-assisted thoracoscopic partial resection of the tumor revealed a 24 x 20 mm teratoma with somatic-type malignancy in which pleomorphic rhabdomyosarcoma was a major element. One year later, asymptomatic tumor recurrence occurred at both edges of the stapler line as 22 x 20 mm and 10 x 5 mm nodules composed only of pleomorphic rhabdomyosarcoma. Throughout the course there was no abdominal lymph node swelling detected by computed tomography (CT) and tumor markers were normal. Adjuvant chemotherapy was started after the tumor recurrence. Currently, the patient is still undergoing chemotherapy 5 months after the tumor recurrence. In conclusion, despite the fact that primary pulmonary rhabdromyosarcoma is a rare neoplasm, metastatic pulmonary germ cell tumor with somatic-type malignancy showing predominantly rhabdomyosarcomatous differentiation should be considered in the differential diagnosis of such lesions of the lung.
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PMID:Metastatic germ cell tumor of the lung masquerading as primary rhabdomyosarcoma. 1618 96

Testicular and even more paratesticular tumours in children are rare. The aim of the study is to characterise the spectrum of these lesions with focus on the feasibility and effectiveness of testis sparing surgery. Twenty-four boys treated between 1980 and 2004 at the University Leipzig Medical Centre were evaluated. At presentation patients were between 5 months and 18 years old (median 23 months). Generally a high rate of malignant or potentially malignant tumours was observed. The majority of these tumours occurred in the first three years of age. The spectrum of testicular tumours comprised 13 germ cell tumours (6 yolk sac tumours, 6 teratomas, 1 embryonal carcinoma) and 4 sex cord stromal tumours (2 Leydig's cell, Sertoli's cell, granulosa cell). Both Leydig's cell tumours were endocrine active. Further on, we observed 3 boys with paratesticular rhabdomyosarcoma (RMS), and three with testicular and paratesticular metastases (Wilms' tumour, neuroblastoma, leukaemia). Serum alpha1-fetoprotein (AFP) was clearly elevated in 5 of 6 yolk sac tumours but remained within normal limits concerning the other entities. Human chorionic gonadotrophin was normal in all cases tested. During the observation period high inguinal orchidectomy was the surgical standard method. Dependent on tumour histology, stage and the recommended treatment schedule postoperative chemotherapy was added. Testis sparing surgery was performed in 3 boys with primary testicular tumours (2 Leydig's cell, mature cystic teratoma). Local relapses were not observed. Systemic relapses occurred in 3 cases (2 RMS, leukaemia). During a median follow up of 5 years all patients with primary testicular tumours survived event free. Meta-analysis of the recent literature revealed that testis sparing surgery is feasible and save in prepubertal boys after exclusion of a malignant tumour. If a testis sparing approach is planned, the following criteria are essential: 1. The presence of a well defined circumscribed nodule confirmed by imaging. 2. Normal levels of serum AFP and hCG. 3. The presence of sufficient healthy testicular parenchyma. However, the high rate of malignant or potentially malignant tumours suggests that high inguinal orchidectomy should remain the surgical standard of therapy.
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PMID:Surgery in infants and children with testicular and paratesticular tumours: a single centre experience over a 25-year-period. 1752 8

In this review we examine the diagnosis and treatment of pediatric liver tumors- both malignant and benign. The two most common malignant tumors are hepatoblastoma and hepatocellular carcinoma. Hepatoblastoma is seen in younger children, hepatocellular carcinoma in older children. Other malignant liver tumors are quite rare and include biliary rhabdomyosarcoma, angiosarcoma, rhabdoid tumor, and undifferentiated sarcoma. The commonly seen benign liver tumors in children are infantile hemangioma, mesenchymal hamartoma, and focal nodular hyperplasia. Rare benign tumors are hepatic adenoma, which is occasionally seen in teenage girls, and teratoma which is a very rare liver tumor in infants.
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PMID:Tumors of the liver in children. 1771 39

We present the interesting case of a 38-year-old man with a primary malignant tumor of the right testis that metachronously metastasized to the urinary bladder and the stomach. Histologically, the testicular tumor was a mixed germ cell tumor composed of teratoma and embryonal carcinoma, but it also contained a sarcoma component of somatic type malignancy. Metastases showed rhabdomyoblastic differentiation histologically identical to the sarcoma component of the testicular tumor that was diagnosed as rhabdomyosarcoma. By applying fluorescence in situ hybridization (FISH) to the cytogenetic examination of cells taken from the periventricular lymph node metastases, we demonstrated a structural chromosomal aberration characteristic of testicular neoplasms, i.e. the presence of isochromosome 12p (i(12p)). Additionally, the diagnosis was supported by immunohistochemistry.
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PMID:A case of primary testicular germ cell tumor with rhabdomyosarcoma metastases as an example of applying the FISH method to diagnostic pathology. 1809 64

Primary ovarian rhabdomyosarcoma with heterologous elements is a rare ovarian neoplasm. Most cases reported in the English literature were associated either with teratomas or with mixed mullerian tumors. Pure primary rhabdomyosarcoma is extremely uncommon. We present hereby a case of primary pure embryonal rhabdomyosarcoma in a 25-year-old woman, showing heterologous elements, but associated neither with teratoma nor with mixed mullerian tumors.
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PMID:Primary ovarian rhabdomyosarcoma with heterologous elements: a case report. 1858 Mar 18

46 XY gonadal dysgenesis patients often develop gonadal tumors, including gonadoblastoma and other types of germ cell tumors. We report a case of a 16-year-old female adolescent with primary amenorrhea and a right adnexal mass. Subsequent study revealed that she is a 46 XY phenotypic female adolescent with complete gonadal dysgenesis and with no alterations of the sex-determining region Y gene. Microscopic examination of the gonads revealed bilateral gonadoblastoma mixed with dysgerminoma and mature teratoma. The tumor in the right gonad was also mixed with yolk sac tumor and immature teratoma with rhabdomyoblastic components, mimicking adult rhabdomyoma and rhabdomyosarcoma. No metastasis in the regional lymph nodes was identified and the patient is disease free 15 months postsurgery. The complexity of the tumorigenesis in this case indicates that the gonadal cells in gonadal dysgenesis are extremely unstable and highly tumorigenic. This tumorigenesis is not necessarily associated with sex-determining region Y gene alterations; therefore, it reinforces the recommendation of gonadectomy for gonadal dysgenesis patients, regardless of the sex-determining region Y gene status.
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PMID:A 46 XY phenotypic female adolescent with bilateral gonadal tumors consisting of five different components. 1858 Mar 19

To gain insight concerning prognosis, we investigated seven cases of post-chemotherapy retroperitoneal lymph-node dissections from patients with testicular germ-cell tumors that contained sizable nodules of differentiated skeletal muscle, but that lacked both a primitive cellular component and mitotic activity. The patients were 18-28 years old at the time of retroperitoneal lymph-node dissection. All had a previous non-seminomatous germ-cell tumor of the testis, five of which had a teratoma component. In one the testicular tumor had foci of embryonal rhabdomyosarcoma. The retroperitoneal lymph-node dissections were performed 0.2-4.7 years after orchiectomy, all following cisplatin-based chemotherapy, and contained rhabdomyomatous tumors that ranged from 0.8-5 cm. These consisted of nodular to diffuse aggregates of fetal-type rhabdomyocytes with central to peripheral nuclei and abundant, eosinophilic, fibrillary cytoplasm with occasional cross striations. Elongated myotubes with multiple nuclei in a common sarcoplasm occurred at least focally in all cases. Mild to moderate nuclear atypia, including nuclear enlargement and nucleolar prominence, was present, but mitotic activity, necrosis, and a primitive cellular component were absent. All but one retroperitoneal lymph-node dissection also contained other teratomatous elements. Follow-up in six patients showed three were disease free at 2.2-3.4 years; two developed recurrent teratoma at 1.3-3.7 years; and a sixth developed recurrent teratoma at 0.5 and 2 years, followed at 17 years by progressive tumor with elevated alpha-fetoprotein. No patient with available follow-up developed progressive sarcoma. We conclude that rhabdomyomatous tumors in retroperitoneal lymph-node dissection specimens after chemotherapy for metastatic testicular germ-cell tumors show clinical behavior similar to teratoma rather than rhabdomyosarcoma. We believe the most likely explanation for the finding of pure rhabdomyomatous tumors in this setting, a phenomenon sometimes termed 'cytodifferentiation,' is selective persistence of differentiated tumor cells because of chemotherapy.
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PMID:Differentiated rhabdomyomatous tumors after chemotherapy for metastatic testicular germ-cell tumors: a clinicopathological study of seven cases mandating separation from rhabdomyosarcoma. 1963 44

The development of an angiosarcomatous component in germ cell tumors is rare. Here we studied 12 cases of mediastinal germ cell tumors with an angiosarcomatous component. All patients were men with a mean age of 34 years (range, 24-49 years). No patient had a documented testicular germ cell tumor. The mean size of mediastinal tumors was 12.9 cm (range, 5.5-16.0 cm). Grossly, the tumors were cystic with variegated hemorrhagic, mucinous, and fleshy solid areas. Microscopically, all tumors were composed of germ cell tumor. The most common germ cell tumor component was teratoma (n = 10); and other germ cell tumor components included seminoma (n = 3), yolk sac tumor (n = 3), embryonal carcinoma (n = 2), and choriocarcinoma (n = 1). The angiosarcomatous component was present in primary mediastinal tumors (n = 6), metastasis (n = 3), or both primary mediastinal tumor and metastasis (n = 3). The angiosarcomatous component accounted for an average of 30% (range, 5%-95%) of the primary mediastinal tumor. In addition, other non-germ cell components, including rhabdomyosarcoma (n = 3), leiomyosarcoma (n = 1), and poorly differentiated carcinoma (n = 1), were also present in the tumors. Of the 10 patients with follow-up available, all patients developed metastasis (n = 8) or local recurrence (n = 2); 7 died of disease at a mean of 33 months (range, 21-75 months), and 3 patients were alive at a mean of 75 months (range, 5-120 months). Our findings suggest that the presence of an angiosarcomatous component in mediastinal germ cell tumor, even in a small amount, is associated with a poor clinical outcome.
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PMID:Mediastinal germ cell tumors with an angiosarcomatous component: a report of 12 cases. 2015 8


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