Unique Case of Giant Adult Paratesticular Spindle Cell Rhabdomyosarcoma
Università degli Studi di Bari Aldo Moro, Bari, Apulia, ItalyUrology (Impact Factor: 2.19). 12/2008; 73(3):500-2. DOI: 10.1016/j.urology.2008.09.025
A 50-year-old unmarried rural man was referred for a giant painless scrotal lump which had developed over a period of about six years. At the admission physical examination disclosed a large swelling scrotum and palpable left supraclavicular lymph nodes. Blood counts, renal and hepatic function tests, lactate dehydrogenase, beta-HCG, and alpha-fetoprotein were normal. Computed tomographic scan of thorax and abdomen revealed supraclavicular, mediastinal, and retroperitoneal adenopathies. The patient underwent right-sided radical orchiectomy. The final pathological diagnosis was paratesticular spindle cell rhabdomyosarcoma. Unfortunately, few days after surgery, patient presented a clinical and laboratory picture of disseminated intravascular coagulation followed by exitus.
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ABSTRACT: Primary cardiac tumors are rare. Of all primary heart neoplasms, sarcomas account for around 10%, and of these, rhabdomyosarcomas (RMS) constitute a minority. A case of primary left atrial RMS, reminiscent of leiomyosarcoma, is reported herein. Histologically, the tumor was composed of tightly-packed spindle-shaped cells arranged in long fascicles. At immunohistochemistry, the tumor cells stained strongly and diffusely with vimentin, muscle-specific actin, desmin and myogenin, and focally with fast-myosin and sarcomeric actin. WT-1 showed diffuse and intense cytoplasmic staining. Staining for calponin was weak. Staining for alpha-SMA, H-caldesmon, CD34, epithelial membrane antigen (EMA), keratin wide-spectrum (CK w.s.), and S100 protein was negative. Electron microscopy revealed poorly differentiated spindle cells, containing contractile filaments with a "Z-band-like" appearance. The final diagnosis was embryonal RMS, spindle cell variant. Conclusively, spindle cell RMS is a well-recognized variant of embryonal RMS, typically occurring in soft tissue, with only rare cases described in visceral organs. This is the first case of primary cardiac spindle cell RMS ever described.
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ABSTRACT: The diverse embryological origins of the contents of the scrotum create an environment that fosters a wide variety of unusual pathologies. Most scrotal pathologies are discovered by the patient and are initially evaluated by a thorough physical examination and scrotal ultrasonography. Scrotal lesions can be broadly grouped by the anatomical location in which they develop; the clinician must consider a wide differential diagnosis based on this location. Solid testicular masses are considered germ cell tumors until proven otherwise, but numerous other possible pathologies exist, including ectopic tissue, metastasis, and other neoplastic growths. Rete testis lesions are classified as developmental, benign or malignant. Cystic lesions of the epididymis are most commonly benign, but malignant neoplasms can also be present. The paratesticular region has the broadest differential diagnosis, as it contains numerous distinct structures and is a common location for ectopic tissue and metastatic disease; a narrower range of lesions develop in the scrotal wall because of its simpler anatomy. Treatment options range from conservative observation to wide surgical excision and should be considered carefully; the aim of therapy is to remove malignant or potentially malignant tissue while minimizing effects on fertility and function.
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