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      Rare retroperitoneal conditions that mimic uterine myoma

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          Abstract

          The most frequent tumoral condition of the uterus is represented by uterine myoma. The diagnosis, in most cases, is established by clinical examination and ultrasound scan. Nevertheless, there are rare cases, in which the surgical findings reveal a retroperitoneal tumor instead of a uterine myoma. These could be represented by schwannomas or Castleman disease. The schwannomas are rarely malignant and arise from the Schwann cells of nerve fibers. These tumors are frequently found at the level of the head, neck and mediastinum and rarely in the pelvis. Generally, schwannomas localized at retroperitoneal level are asymptomatic and with a very slow growth rate. The treatment consists in complete surgical resection. The recurrence rate is low and, generally, the prognosis is good. The Castleman disease is considered a rare entity, but it should be always taken into consideration when it comes to a differential diagnosis in a young patient who presents a retroperitoneal mass at imagery exams. The condition affects the lymphatic system and is characterized by a hyperplasia of the lymph nodes, sometimes associated with herpes virus infection. The clinical picture is often non-specific; the pain may be the only symptom. The imaging methods are not always conclusive for the final positive diagnosis and the histopathological examination is always necessary. Pelvic Castleman disease can be misdiagnosed as myoma or an adnexal tumor. In this article, we review the present knowledge regarding the pathogenesis, pathology and management of these rare retroperitoneal tumors. Both conditions, when located in pelvis must be taken into consideration in the differential diagnosis of uterine myomas, especially in the pedunculated form.

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          Most cited references49

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          Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems.

          Peripheral nerve sheath tumors are common neoplasms, with classic identifiable features, but on occasion, they are diagnostically challenging. Although well-defined subtypes of peripheral nerve sheath tumors were described early in the history of surgical pathology, controversies regarding the classification and grading of these tumors persist. Advances in molecular biology have provided new insights into the nature of the various peripheral nerve sheath tumors, and have begun to suggest novel targeted therapeutic approaches. In this review, we discuss current concepts and problematic areas in the pathology of peripheral nerve sheath tumors. Diagnostic criteria and differential diagnosis for the major categories of nerve sheath tumors are proposed, including neurofibroma, schwannoma, and perineurioma. Diagnostically challenging variants, including plexiform, cellular and melanotic schwannomas are highlighted. A subset of these affects the childhood population, and has historically been interpreted as malignant, although current evidence and outcome data suggest they represent benign entities. The growing current literature and the author's experience with difficult to classify borderline or "hybrid tumors" are discussed and illustrated. Some of these classification gray zones occur with frequency in the gastrointestinal tract, an anatomical compartment that must always be entertained when examining these neoplasms. Other growing recent areas of interest include the heterogeneous group of pseudoneoplastic lesions involving peripheral nerve composed of mature adipose tissue and/or skeletal muscle, such as the enigmatic neuromuscular choristoma. Malignant peripheral nerve sheath tumors (MPNST) represent a diagnostically controversial group; difficulties in grading and guidelines to separate "atypical neurofibroma" from MPNST are provided. There is an increasing literature of MPNST mimics which neuropathologists must be aware of, including synovial sarcoma and ossifying fibromyxoid tumor. Finally, we discuss entities that are lacking from the section on cranial and paraspinal nerves in the current WHO classification, and that may warrant inclusion in future classifications. In summary, although the diagnosis and classification of most conventional peripheral nerve sheath tumors are relatively straightforward for the experienced observer, yet borderline and difficult-to-classify neoplasms continue to be problematic. In the current review, we attempt to provide some useful guidelines for the surgical neuropathologist to help navigate these persistent, challenging problems.
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            Idiopathic multicentric Castleman's disease: a systematic literature review

            Multicentric Castleman's disease describes a group of poorly understood lymphoproliferative disorders driven by proinflammatory hypercytokinaemia. Patients have heterogeneous clinical features, characteristic lymph node histopathology, and often deadly multiple organ dysfunction. Human herpesvirus 8 (HHV8) causes multicentric Castleman's disease in immunosuppressed patients. The cause of HHV8-negative multicentric Castleman's disease is idiopathic; such cases are called idiopathic multicentric Castleman's disease. An absence of centralised information about idiopathic multicentric Castleman's disease represents a major challenge for clinicians and researchers. We aimed to characterise clinical features of, treatments for, and outcomes of idiopathic multicentric Castleman's disease.
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              Surgery in Castleman's disease: a systematic review of 404 published cases.

              We undertook a systematic review of 404 published cases of Castleman's disease to identify the role of the surgeon beyond assistance in tissue-based diagnosis. Castleman's disease is a rare primary disease of the lymph node caused by infection with herpesviridae. Little is known about the role of surgery in this condition. Medline, Embase, Cochrane Database of Systematic Reviews, ISI Thompson Web of Knowledge, and hand search of articles' bibliography. Of the 1791 citations identified through the initial electronic search and screened for possible inclusion, 488 articles were retained after title and abstract reviews. Of these, 239 were accepted for this review. A complete dataset containing age, gender, centricity (unicentric vs multicentric), histopathologic type (hyaline vascular [HV] vs plasma cell [PC]), anatomical location of the only focus in unicentric Castleman's disease (UCD) or the dominant focus in multicentric Castleman's disease (MCD), nature of the surgical approach (resective vs diagnostic), and outcome (disease-free survival [DFS] vs death due to disease) was extracted. A resective or debulking surgical approach was described in 77.0% of all patients, but was far more common in unicentric (262/278; 94.2%) than multicentric (49/126; 38.9%) disease (χ² 146.8; P < 0.0001). Unicentric disease had a significantly higher overall survival (95.3% vs 61.1%; χ² 55.7; P < 0.0001), 3 year DFS (89.7% vs 55.6%; χ² 27.8; P < 0.0001), and 5 year DFS (81.2% vs 34.4%; χ² 28.6; P < 0.0001) than multicentric disease. Failure to treat unicentric disease by resective surgery resulted in a significant mortality (17.6% vs 3.8% χ²; P < 0.05). In multicentric disease, outcomes are comparable between debulking surgery alone, immunochemotherapy alone, or a combination of both (28.0% vs 28.9% vs 50.0%; P = nonsignificant). Surgery is the gold standard for treatment of unicentric Castelman's disease. The role of debulking surgery in human immunodeficiency virus (-) MCD needs to be evaluated in prospective studies.
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                Author and article information

                Journal
                Rom J Morphol Embryol
                Rom J Morphol Embryol
                RJME
                Romanian Journal of Morphology and Embryology
                Academy of Medical Sciences, Romanian Academy Publishing House, Bucharest
                1220-0522
                2066-8279
                Jan-Mar 2020
                07 May 2020
                : 61
                : 1
                : 7-14
                Affiliations
                [1 ]Department of Obstetrics and Gynecology, Faculty of Medicine, Grigore T. Popa University of Medicine and Pharmacy, Iaşi, Romania
                [2 ]Department of Ophthalmology, Faculty of Medicine, Grigore T. Popa University of Medicine and Pharmacy, Iaşi, Romania
                [3 ]Department of Immunology, Faculty of Medicine, Grigore T. Popa University of Medicine and Pharmacy, Iaşi, Romania
                [4 ]Department of General Surgery, Faculty of Medicine, Grigore T. Popa University of Medicine and Pharmacy, Iaşi, Romania
                [5 ]Cuza Vodă Hospital of Obstetrics and Gynecology, Iaşi, Romania
                Author notes
                Corresponding Author: Claudia Florida Costea Associate Professor, MD, PhD, Department of OphthalmologyFaculty of Medicine Grigore T. Popa University of Medicine and Pharmacy 16 University Street700115 IaşiRomania+40744–972 648 costea10@ 123456yahoo.com
                Corresponding Author: Alexandru Cărăuleanu Senior Lecturer, MD, PhD, Department of Obstetrics and GynecologyFaculty of Medicine Grigore T. Popa University of Medicine and Pharmacy 16 University Street700115 IaşiRomania+40744 517 106 acarauleanu@ 123456yahoo.com
                Article
                610120007014
                10.47162/RJME.61.1.01
                7728116
                32747890
                647c1343-3099-4075-947e-c56b68b5fd25
                Copyright © 2020, Academy of Medical Sciences, Romanian Academy Publishing House, Bucharest

                This is an open-access article distributed under the terms of a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International Public License, which permits unrestricted use, adaptation, distribution and reproduction in any medium, non-commercially, provided the new creations are licensed under identical terms as the original work and the original work is properly cited.

                History
                : 31 January 2020
                : 07 May 2020
                Categories
                Review

                uterine myoma,schwannoma,castleman disease,retroperitoneal tumors

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