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      Adult primary paratesticular mesenchymal tumors with emphasis on a case presentation and discussion of spermatic cord leiomyosarcoma

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          Abstract

          Background

          The aim of this report is related to adult primary paratesticular mesenchymal tumors with emphasis on a case presentation and discussion of the spermatic cord leiomyosarcoma. Primary paratesticular tumors are rare, only accounting for 7% to 10% of all intrascrotal tumors. In adults, more than 75% of these lesions arise from the spermatic cord, 20% being leiomyosarcoma. Tumor grade, stage, histologic type, and lymph node involvement are independently predictive of prognosis.

          Findings

          The case report concerns a 81-year-old man presented with a 3-year history of painless lump in the right hemiscrotum. Scrotal examination demonstrated a 5.1-cm, firm-to-hard mass attached to the spermatic cord. Scrotal ultrasound scan revealed a heterogeneous mass separate from the testis. He was treated with an radical orchi-funicolectomy. Histologically the lesion is composed of spindled cells with often elongated, blunt-ended nuclei and variably eosinophilic cytoplasm. Areas with pleomorphic morphology are present. The level of mitotic activity is equal to 3/10 HPF in the areas with spindle cell morphology and to 12/10 HPF in the areas with pleomorphic morphology. The final diagnosis was that a leiomyosarcoma of the spermatic cord, with grade 1 and grade 2 areas, stage pT2b cN0 and cM0. The patient has been followed up for 3 months with CT scans and shows no signs of recurrence.

          Conclusions

          Spermatic cord leiomyosarcoma, although rare, should be one of the first differential diagnoses for a firm-to-hard lump in the cord. Apart from radical orchi-funicolectomy, there has been added benefit of adjuvant radiotherapy to prevent any loco-regional lymph node recurrence.

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          The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1613030331125632

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          Most cited references33

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          Dedifferentiated liposarcoma: a clinicopathological analysis of 155 cases with a proposal for an expanded definition of dedifferentiation.

          We studied 155 cases of dedifferentiated liposarcoma to define its clinicopathologic features and behavior, in particular how the extent and grade of dedifferentiation affected outcome. Tumors occurred in late adult life (median, 61.5 years: range, 21-92 years), most commonly in the retroperitoneum (106 cases), extremities and trunk (32 cases), and scrotum/spermatic cord (13 cases). The majority of dedifferentiated liposarcomas presented as de novo lesions, whereas the remainder developed as a late complication of a preexisting well-differentiated liposarcoma after an average interval of 7.7 years. At the time of presentation, most of the dedifferentiated liposarcomas displayed extensive areas of high-grade dedifferentiation resembling malignant fibrous histiocytoma or high-grade fibrosarcoma, whereas a minority contained only areas of low-grade dedifferentiation resembling fibromatosis or well-differentiated fibrosarcoma. Divergent myosarcomatous or osteosarcomatous differentiation was observed focally in six cases. The behavior of dedifferentiated liposarcomas was that of a high-grade sarcoma with a local recurrence rate of 41%, a metastatic rate of 17%, and disease-related mortality of 28%. The most important prognostic factor was location in that retroperitoneal tumors had significantly worse survival than those in other sites. Tumors were divided into those having less than or those with more than 25% dedifferentiation, and dedifferentiated zones were classified into low grade or high grade. Neither low-grade dedifferentiation nor a low percentage of dedifferentiation was associated with an improved outcome for the tumors examined in this study; however, in no cases was the absolute size of the dedifferentiated focus <2 cm. Therefore, this study did not determine a minimum, or threshold, amount of dedifferentiation below which outcome was more favorable. The behavior of liposarcomas in which the dedifferentiated component was entirely low grade was more similar to that of traditional dedifferentiated liposarcoma than to that of well-dedifferentiated liposarcoma. Our study supports the expansion of the definition of dedifferentiated liposarcoma to include tumors with low-grade dedifferentiation and also suggests that low-grade dedifferentiation represents a precursor lesion of high-grade dedifferentiation.
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            Adult spermatic cord sarcomas: management and results.

            We present a 20-year surgical experience with spermatic cord sarcoma, describe prognostic features, and evaluate the results of surgical and adjunctive treatments. Forty-seven patients were identified from an institutional database. Disease-free survival was calculated, and prognostic factors such as tumor grade, size, extent of operation, and adjuvant therapy were analyzed. The median patient age was 58 years (range, 16-83 years), and the median follow-up was 51 months (range,.5-226 months). The most common tumor types included liposarcoma (51%), leiomyosarcoma (19%), embryonal rhabdomyosarcoma (13%), and malignant fibrous histiocytoma (11%). Twenty-nine (62%) patients had high-grade tumors, 21 (45%) were treated with adjuvant radiation, and 9 (19%) received chemotherapy. The overall 5- and 10-year disease-specific survival was 75% and 55%, respectively. No specific prognostic factors were identified for recurrence or disease-free survival. In 21 patients who underwent reoperative wide resection after a prior incomplete resection, a trend toward improved disease-free survival was noted (P <.059). Of these, six (29%) had residual viable sarcoma. We could not demonstrate a therapeutic effect of adjuvant radiation or chemotherapy. We demonstrated that aggressive surgical strategies, including reoperative wide resection, significantly decrease local recurrence and may improve disease free-survival in select patients with spermatic cord sarcoma.
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              The management of spermatic cord sarcoma.

              Between April 1963 and July 1991, 18 patients were treated for spermatic cord sarcoma. The histologic subtype distribution was: 7 leiomyosarcoma, 7 liposarcoma, 2 malignant fibrous histiocytoma, and 1 mesothelioma. All patients underwent surgical resection: 16 radical orchiectomy and local excision. Nine were treated with orchiectomy alone, and 9 received adjuvant radiation. The radiation fields encompassed the ipsilateral iliac and inguinal lymph nodes, vas deferens, and hemiscrotum in 7 patients, and iliac and inguinal lymph nodes in 2 patients. The actuarial 5 and 8-year disease free survivals for the 18 patients were 77% and 58%, with an overall survival of 78% and 70%, respectively. The 5 and 8-year locoregional control rates were 82% and 61%. Five of 9 patients treated with surgery alone developed locoregional recurrence while none of the nine who had adjuvant radiation relapsed. The median follow-up for the irradiated group, however, was shorter (123 vs 63 months) and staging studies more complete. These potential biases are discussed. In this series, relapse was common after orchiectomy alone. Adjuvant radiation therapy may reduce the incidence of locoregional failure.
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                Author and article information

                Contributors
                Journal
                Diagn Pathol
                Diagn Pathol
                Diagnostic Pathology
                BioMed Central
                1746-1596
                2014
                6 May 2014
                : 9
                : 90
                Affiliations
                [1 ]Urology Division, “Augusto Murri” General Hospital, Fermo, Italy
                [2 ]Section of Pathological Anatomy, Polytechnic University of the Marche Region, School of Medicine, United Hospitals, Via Conca 71, I - 60126 Torrette, Ancona, Italy
                [3 ]Unit of Anatomic Pathology, Department of Surgery, Faculty of Medicine, Cordoba, Spain
                [4 ]Fundação Champalimaud, Lisbon, Portugal
                [5 ]Oncology Division, “Augusto Murri” General Hospital, Fermo, Italy
                [6 ]Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA
                Article
                1746-1596-9-90
                10.1186/1746-1596-9-90
                4039061
                24885500
                5c79238b-0ac1-4b57-8f2e-504c6f04f74b
                Copyright © 2014 Galosi et al.; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 31 March 2014
                : 15 April 2014
                Categories
                Short Report

                Pathology
                leiomyosarcoma of the spermatic cord,mesenchymal tumors of the scrotum,spermatic cord,liposarcoma,malignant fibrous histiocytomas,rhabdomyosarcoma

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