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      Neoplastic diseases of the spermatic cord: an overview of pathological features, evaluation, and management

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          Abstract

          Extracellular tumors found with the spermatic cord, known as neoplasms, are usually identified to be benign. However, the accurate and timely diagnosis of spermatic cord masses is highly crucial, especially when most results are often overlooked or unclear. In this review, we discuss the anatomy and embryology of the spermatic cord. Upon rooting these fundamental concepts, we discuss an array of benign and malignant neoplastic tumors, including their origin, pathological features, clinical evaluation and management, as well as other case-specific characteristics of unique presentation. Many of these neoplasms are based on local neurological, vascular, muscular, bone, soft tissue, or lymphatic origin, while others have metastasized from particular areas of the body.

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          Most cited references76

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          Malignant schwannoma--clinical characteristics, survival, and response to therapy.

          One hundred and sixty-five cases of malignant schwannoma were reviewed. Sixty-five (40%) of the patients had evidence of disseminated neurofibromatosis. Patients with neurofibromatosis were younger, had malignant schwannomas that were centrally rather than peripherally located, and had a shorter five-year survival (23%) than patients with solitary malignant schwannomas (47%). Histologically, tumors developing in patients with neurofibromatosis had a collagenous appearance, while tumors in patients without neurofibromatosis were undifferentiated and highly cellular. The clinical course of both groups of patients tended to be that of multiple local recurrences, although local recurrence had a more ominous prognosis in patients with neurofibromatosis. Chemotherapy responses in all these patients were extremely poor; however, the results of adjuvant therapy after surgery appeared encouraging. Fourteen patients (8.5%) had a malignant schwannoma in an area of prior radiation therapy and died of disease a median of 14 months after diagnosis. Malignant schwannoma should be considered in the differential diagnosis of tumors developing in areas previously treated with radiation.
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            Management of spermatic cord tumors: a rare urologic malignancy.

            Primary spermatic cord tumors are rare yet clinically significant urologic lesions that affect patients of all ages. They are the most common tumors of the paratesticular region and generally present as asymptomatic, slow growing, firm, palpable paratesticular masses. Although most of them are benign comprised primarily of lipomas, approximately 25% are potentially life-threatening malignant tumors. The most common reported malignant histological types include liposarcomas, leiomyosarcomas, rhabdomyosarcomas, malignant fibrous histiocytoma, and fibrosarcomas. Management of these malignant tumors has been difficult because of their rarity, therefore there is little consensus regarding optimal surgical and adjunctive treatment strategies. It is recommended that radiological techniques such as scrotal ultrasound (US), computed tomography, and magnetic resonance be used to evaluate these masses before surgery. The curative treatment of choice is radical orchiectomy with high cord ligation and wide excision of surrounding soft tissue structures within the inguinal canal. Patients with inadequately resected disease should undergo a reoperative procedure for wide inguinal re-resection. Retroperitoneal lymphadenectomy is recommended when there is preoperative evidence of retroperitoneal lymph node metastasis and as an adjuvant treatment for rhabdomyosarcomas since they have a higher propensity for lymphatic spread. Adjuvant treatments, such as radiotherapy and chemotherapy, have shown little efficacy, except in the management of patients with local recurrence or high-grade rhabdomyosarcomas. Long-term follow up is recommended given their high recurrence rates.
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              Myofibroblastic malignancies.

              Malignant tumors composed of myofibroblasts are increasingly being recognized, but their existence remains controversial. Currently accepted examples within this category represent spindle cell or pleomorphic neoplasms of the soft tissues with a spectrum of histological grades. Low- and intermediate-grade myofibrosarcomas are fascicular spindle cell neoplasms resembling fibrosarcoma or leiomyosarcoma. They infiltrate deep soft tissue with disproportionate involvement of head and neck sites and can recur locally but infrequently metastasize. They variably express myoid immunohistochemical markers, and their differential diagnosis includes benign myofibroblastic proliferations such as fasciitis and fibromatosis as well as other types of spindle cell sarcoma. High-grade (pleomorphic) myofibrosarcomas are an ultrastructurally defined subset of malignant fibrous histiocytoma, which they resemble in morphology and behavior. Inflammatory myofibroblastic tumor and infantile fibrosarcoma are neoplasms that have myofibroblastic features and have been included in this category, but they have distinctive genetic findings. This article reviews the concept of myofibrosarcoma and describes its variants.
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                Author and article information

                Journal
                Transl Androl Urol
                Transl Androl Urol
                TAU
                Translational Andrology and Urology
                AME Publishing Company
                2223-4691
                February 2017
                February 2017
                : 6
                : 1
                : 101-110
                Affiliations
                [1 ]Department of Physiology and Biophysics, Stony Brook University School of Medicine, Stony Brook, NY, USA;
                [2 ]Foley Plaza Medical, New York, NY, USA;
                [3 ]Department of Urology, Stony Brook University School of Medicine, Stony Brook, NY, USA
                Author notes

                Contributions: (I) Conception and design: NL Smith, SA Khan; (II) Administrative support: None; (III) Provision of study material or patients: None; (IV) Collection and assembly of data: None; (V) Data analysis and interpretation: None; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors.

                Correspondence to: Sardar Ali Khan, MD, FRCS, FACS. Department of Urology, Health Sciences Center T9-040, Stony Brook University School of Medicine, 101 Nicolls Road, Stony Brook, NY 11794-8093, USA. Email: skysalik@ 123456gmail.com .
                Article
                tau-06-01-101
                10.21037/tau.2017.01.04
                5313312
                28217455
                ddccaeb8-8843-4151-bfc8-16a19ec00de1
                2017 Translational Andrology and Urology. All rights reserved.
                History
                : 05 November 2016
                : 11 November 2016
                Categories
                Review Article

                spermatic cord,spindle cells,myofibroblastic tumor,malignancies,neoplasm,aggressive angiomyxoma,myositis ossificans,scrotum

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