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)

A 5-year-old boy with intrascrotal rhabdomyosarcoma arising from the epididymis is described. The patient was submitted to radical orchidectomy via the inguinal approach and high ligation of the spermatic cord. The anatomopathological examination revealed germ cell rhabdomyosarcoma of the epididymis with no testicular or spermatic cord involvement, which corresponded to stage I of the IRS classification (Intergroup Rhabdomyosarcoma Study). Its clinical and histological features and treatment are discussed.
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PMID:[Rhabdomyosarcoma of the epididymis]. 151 Apr 84

This is a case report of embryonal rhabdomyosarcoma of the epididymal appendage in a 15-month old infant. It is a rare tumor with a good prognosis when treated promptly. The ultrasound examination showed the testicle to be enlarged but hypo-echoic in comparison to the epididymis which was infiltrated with tumor. An inflammatory mass or enlargement of a testicle demands careful diagnosis and appropriate therapy with immediate referral to a urologist if the normal clinical course deviates. Representative images are included. Rhabdomyosarcoma of the testicle or extra testicular scrotal structures is rare. A report in 1983 by Kage et al from Japan revealed two cases of the tumor, however, both were in adult males. This case report is an embryonal rhabdomyosarcoma in a fifteen month old infant which presented with a confusing clinical picture. This case is presented in an effort to alert the clinician to the possibility of a rare, non-germ cell tumor presenting as an inflammatory lesion.
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PMID:Rhabdomyosarcoma of the testicular appendage in an infant: case report. 219 21

A case of rhabdomyosarcoma of the spermatic cord in a 6-year-old boy is reported. On February 26, 1986, he visited Nakamura Hospital with the chief complaint of enlargement of the left scrotal content. The contralateral scrotum was normal. The left scrotal content was a hard thumb-head-sized tumor. The left testis and epididymis were not distinguishable from the tumor. On the same day, left high inguinal orchiectomy was performed. The tumor was 3.5 by 2.5 by 2.5 cm in size and was distinguishable from the testis, epididymis and tunica vaginalis. Histopathological findings were embryonal rhabdomyosarcoma and it appeared to have originated from the spermatic cord. Two years after operation, the boy is living without metastasis. Including our experience, 101 cases of the paratesticular rhabdomyosarcoma found in Japanese literature are reviewed and briefly discussed.
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PMID:[A case of embryonal rhabdomyosarcoma of the spermatic cord]. 269 46

Although the greatest incidence of testicular neoplasms is in the age group 20 to 35.9% of all intrascrotal tumors occur in males over 60. They may be classified into four major groups based on the tissue origin of the tumor. The most common group comprising 50% are lymphoreticular neoplasms or lymphomas. They occur in both blacks and whites, result in a diffuse enlargement of the testis and commonly involve the epididymis and cord. Prognosis is poor and survival is usually less than two years. Germ cell tumors comprise 25% and the tumors are usually large. Most of them are the classical seminoma which has a good prognosis following orchiectomy and retroperitoneal radiation. A few are the spermatocytic seminoma which is usually benign. Three percent are teratocarcinoma with embryonal elements which is highly malignant and survival less than two years. Ten percent are tumors of gonadal stromal origin. There are two types, the Leydig cell and the more rare Sertoli cell. About one-fourth of these patients develop gynecomastia and some a decrease in libido. The prognosis is good as less than 10% of these tumors are malignant. Neoplasms of supportive and paratesticular structures comprise a heterogeneous group of benign and malignant lesions and comprise about 15% of intrascrotal tumors. Most of them have the same features as tumors of similar tissues encountered throughout the body. They are the mesothelioma or adenomatoid tumor, fibroma, rhabdomyosarcoma, leiomyoma, lipoma, liposarcoma, mucinous cystadenocarcinoma, and leiomyosarcoma. As in all intrascrotal tumors, the diagnosis, treatment, and prognosis are based on the microscopic findings after removal of the tumor.
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PMID:Intrascrotal tumors in the older male. 372 56

A 17-year-old Japanese boy was admitted with the complaint of painless, progressive enlargement of the left scrotal contents during the preceding two months. The tumor was firm and nontender without transillumination. Orchitectomy with high ligatation of the spermatic cord was performed. The tumor, weighing 13.1 g, was gray, solid, located at the distal part of the spermatic cord, and separated from the testis and epididymis. The pathological diagnosis showed left spermatic cord rhabdomyosarcoma of the combined embryonal and alveolar type. All of the retroperitoneal lymph nodes dissected were negative for metastasis. The patient was treated with actinomycin D and vincristin postoperatively, and is well three years after the operation. Ninety-two cases of intrascrotal rhabdomyosarcoma reported in Japan are reviewed.
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PMID:[An operative case of spermatic cord rhabdomyosarcoma--with a review of similar cases in Japan]. 407 66

This is a report of prospective study of eight patients with testicular tumours seen at the Urology Unit of the Lagos University Teaching Hospital over a five-year period (1979-1983). The mean age was 32.7 years. Four patients (50%) had germ cell tumours including embryonal carcinoma 25%, seminoma 12.5% and malignant teratoma undifferentiated (MTU) 12.5%. The seminoma in this group originated from a testis which was previously undescended but brought into the scrotum at six years of age. The other four patients (50%), had non germ cell tumours. Two of these patients (25%) had paratesticular tumours including rhabdomyosarcoma of paratesticular adnexae and liposarcoma. One (12.5%) had adenomatoid tumour of the epididymis while the last patient (12.5%) had malignant fibrous mesothelioma of the tunica vaginalis. This study reaffirms the fact that testicular tumours are rare in blacks and that Nigeria has the lowest incidence reported at 0.1 per 100,000 per annum.
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PMID:Testicular cancer in Nigerians. 749 39

Paratesticular tumors are uncommon tumors, most being found incidentally at autopsy. The most common benign paratesticular tumor is the adenomatoid tumor. A somewhat less frequent benign paratesticular tumor, papillary cystadenoma, accounts for approximately 33 percent of all the primary epididymal tumors and is frequently seen in patients with Von Hippel-Lindau disease. Malignant tumors are rare. Of these, rhabdomyosarcoma is the most frequent. Seen almost exclusively in the pediatric population, paratesticular rhabdomyosarcomas account for 7 percent of childhood rhabdomyosarcomas. We describe two cases of paratesticular tumors in adults: a papillary cystadenoma of the epididymis in a 72-year old male, and an incidental rhabdomyosarcoma in a 49-year old male.
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PMID:Adult paratesticular tumors: report of two cases. 801 95

Twenty two patients with tumours or tumour-like conditions of the para-testicular region (PTR) were diagnosed over a 5 year period. Of these, 16 (73%) were benign with only one true neoplasm (papillary cystadenoma). The tumour-like conditions were comprised of 6 cases of adenomatous hyperplasia of epididymis, 4 cases of spermatic granuloma, 2 cases each of spermatocele and nodular-fibrous proliferation and one mesothelial cyst. Three patients with spermatic granuloma gave a history of trauma. No case of adenomatoid tumour was encountered. Six malignant neoplasms were diagnosed (27%) all of which were sarcomas. Five were primary to the PTR and one was a metastatic deposit from a primary leiomyoblastoma of the mesentery. Among the 5 primary sarcomas were 2 cases each of rhabdomyosarcoma and leiomyosarcoma, and 1 case of liposarcoma. The patient with leiomyoblastoma presented first with a measenteric mass which was followed a month later by a rapidly growing mass in the scrotum. Immunohistochemical staining (IHC) and Electron microscopy (EM) were done in this case. Intra-cytoplasmic myofilaments as well as desmosomes were observed. The patients ranged from 14 to 65 years of age and most presented with painless mass in the scrotum. Follow-up was not possible due to practical difficulties.
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PMID:Tumours and tumour-like conditions of the para-testicular region--a study of morphological features. 980 50

This is a review of literature data on rarely met with intrascrotal non-testicular tumors still not well enough clarified in clinical practice. Histologically these tumors derive from: 1. Epididymis. 2. Funiculus spermaticus and/or tunica vaginalis. 3. Tissues within the scrotum (fatty, fibrous, muscular, lymphatic, nervous). 4. Metastases. The individual types of neoplasms, some of them representing casuistic rarity, so far described as sporadic observations in the pertinent literature, are discussed. Adenomatoid tumor of epididymis and rhabdomyosarcoma are the most frequently encountered. Clinically paratesticular tumors do not lend themselves to differentiation from the ones involving the testis regardless of the fact that for some of them data on specific laboratory and serum or tissue tumor markers have been duely reported. Diagnosis is made histologically following operative management--orchiepididymectomy. According to histological pattern, if necessary the treatment proceeds with radio- or chemotherapy. Finally, two personal observations are described.
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PMID:[Intrascrotal nontesticular tumors]. 1083 21

We investigated 115 testicular and 3 epididymal tumors and 6 cases of the complete androgen insensitivity syndrome (AIS) for the expression of inhibin-alpha, CD99, HEA125, PLAP, and chromogranin, using monoclonal antibodies and standard immunhistochemical techniques. Ihibin-alpha was detected in the neoplastic cells in 27 of 27 primary Leydig cell tumors (LCTs), 1 of 1 metastatic LCT, 6 of 20 Sertoli cell tumors (SCTs), 4 of 5 juvenile granulosa cell tumors (GCTs), and 2 of 5 unclassified sex cord-stromal tumors (USCSTs). Except for 2 choriocarcinomas, the choriocarcinomatous component of 1 mixed germ cell tumor, and a small focus of inhibin-positive syncytiotrophoblast in 1 embryonal carcinoma, inhibin-a immunoreactivity was not present in the neoplastic cells of the 38 remaining testicular germ cell tumors; 11 B-cell and 1 T-cell lymphomas; 1 granulocytic sarcoma; and 1 rhabdomyosarcoma of the testis; 1 adenoma of the rete testis, and 3 adenomatoid tumors of the epididymis. Inhibin-alpha immunoreactivity was present in the Sertoli cells and Leydig cells in 5 testicular hamartomas and in 1 Sertoli cell adenoma in 6 cases of AIS; both Sertoli and Leydig cells were also positive in the extranodular testicular parenchyma present in 2 of these cases. CD99 was detected in 10 of 15 primary LCTs, 1 of 7 SCTs, 3 of 5 JGCTs, and in 1 of 5 USCSTs but was not found in any tumor outside the sex cord-stromal category. HEA125 immunostaining was not detected in sex cord-stromal tumors; however, 3 of 12 seminomas, 3 of 12 embryonal carcinomas, 6 of 8 yolk sac tumors, and 1 of 2 teratomas were HEA125 positive. PLAP was not detected in sex cord-stromal tumors except for 4 of 15 primary LCTs but was present in most germ cell tumors. Chromogranin immunostaining was present in the sex cord-like element in 1 of 5 USCSTs, 1 of 8 YSTs, 1 of 2 teratomas, and in 1 of 1 rete adenoma, and in normal adjacent rete testis. In conclusion, although inhibin-alpha and PLAP, and, to a somewhat lesser extent, CD99 and HEA125 immunostaining are helpful in the differential diagnosis of certain testicular neoplasms that are difficult to distinguish on morphologic grounds, chromogranin is far less helpful in this context.
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PMID:Inhibin-alpha CD99, HEA125, PLAP, and chromogranin immunoreactivity in testicular neoplasms and the androgen insensitivity syndrome. 1101 71


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