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

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

We report 2 cases of rhabdomyosarcoma of the paratesticular tissues. Differentiation from chronic epididymal infection, such as tuberculosis, may be difficult but it must not delay operation. Lymphography is of limited value in delineating lymph node metastases. In both cases secondary spread was suggested but not confirmed histologically. Lymphography, because of a definite mortality rate of 0.1 per cent and morbidity rate of 1 per cent, would appear not to be indicated.
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PMID:Rhabdomyosarcoma of the paratesticular tissues. 728 56

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

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

The extratesticular scrotal contents consist of the epididymis, spermatic cord, and fascia derived from the embryologic descent of the testis through the abdominal wall. As opposed to intratesticular masses, most extratesticular masses are benign. Cystic masses (including hydroceles, epididymal cysts, and varicoceles) are easily diagnosed with ultrasonography (US) and are benign. Epididymitis is a common extratesticular lesion as well as the most frequent cause of an acute scrotum. It may be either acute or chronic and can be potentially complicated by epididymo-orchitis or scrotal abscess. Findings include epididymal enlargement, skin thickening, hydroceles, and hyperemia. The epididymis can also be affected by sarcoidosis, a noninfectious granulomatous disorder. The most common extratesticular neoplasms are lipomas (most often arising from the spermatic cord) and adenomatoid tumors (most often found in the epididymis). Despite their relative rarity, malignant neoplasms do occur and include rhabdomyosarcoma, liposarcoma, leiomyosarcoma, malignant fibrous histiocytoma, mesothelioma, and lymphoma. These tumors are often large at the time of presentation. The US findings of solid masses are often nonspecific. Magnetic resonance imaging can be very helpful in the evaluation of some of these disorders, allowing for a more specific diagnosis in cases of lipoma, fibrous pseudotumor, and polyorchidism.
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PMID:From the archives of the AFIP: extratesticular scrotal masses: radiologic-pathologic correlation. 1502 90

Paratesticular rhabdomyosarcoma (RMS) is an extremely rare malignancy in adults, accounting for 7% of all RMS cases and 6% of all non-germinal intrascrotal tumors. The clinical signs are similar to those of a hydrocele or testicular tumor, typically presenting as a unilateral, painless mass in the inguinal canal or scrotum. No specific serum markers are currently available for this tumor. RMS of the epididymis is extremely rare. Particularly when it is associated with epididymitis, this malignancy is usually overlooked. We herein present a case of epididymal embryonal RMS, manifesting an painful scrotal edema, misdiagnosed as epididymitis. The patient received 3 cycles of adjuvant chemotherapy postoperatively and remained disease-free after 4 years of follow-up.
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PMID:Embryonal rhabdomyosarcoma of the epididymis presenting as epididymitis: A case report. 2707 79

Herein we describe the first reported case of epididymal inflammatory pseudotumor (IPT) with incidental sperm granuloma in an adolescent. IPTs of epididymal origin are very rare, with a differential diagnosis including benign and malignant processes. Rhabdomyosarcomas and inflammatory myofibroblastic tumors are important diagnostic considerations that display pathologic similarities to IPTs. These lesions were excluded from diagnosis in this case and the important pathologic features allowing for exclusion are detailed within. Lastly, sperm granulomas are extremely rare pathologic findings in pediatric patients and their presence in this situation is likely the result of downstream tumor obstruction.
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PMID:Epididymal Inflammatory Pseudotumor With Downstream Sperm Granuloma in an Adolescent Patient: A Case Report and Review of the Literature. 2729 65