Background Malignant peripheral nerve sheath tumor (MPNST) is normally biologically an

Background Malignant peripheral nerve sheath tumor (MPNST) is normally biologically an intense tumor that the treating choice is the surgery. and disease free survival were 58% and 35% respectively. In univariate analysis, sex (p = 0.05), tumor depth (p < 0.03), and cellular differentiation (p < 0.002) were shown to be adverse prognostic factors for disease free survival and sex (p = 0.04), cellular differentiation (p < 0.0004), and tumor grade (p = 0.05) for overall survival. However, in multivariate analysis, cellular differentiation (p < 0.005) and tumor grade (p < 0.01) emerged as independent prognostic factors for both disease free and buy 527-95-7 overall survival, respectively. Postoperative radiotherapy (RT) has shown a definite role in both disease free and overall survival in this study. Conclusion MPNSTs constituted a significant proportion (12%) of soft tissue sarcoma in our medical center. Heterogeneous differentiation and multifocality of the tumor were few distinct features of MPNST. Sex and cellular differentiation were noticed as the new adverse prognostic factors and adjuvant radiotherapy has been proved to be a significant treatment tool in the current series. Background Malignant peripheral nerve sheath tumor (MPNST) is usually a rare variety of soft tissue sarcoma of ectomesenchymal origin [1,2]. World Health organization (WHO) coined the term MPNST replacing previous heterogeneous and often confusing terminology, such as malignant schwannoma, malignant neurilemmoma, and neurofibrosarcoma, for tumors of neurogenic origin and similar biological behavior [3,4]. These tumors often create diagnostic problems because of their cellular origin and histopathological similarities with other spindle cell sarcomas like monophasic synovial sarcoma, leiomyosarcoma and fibrosarcoma [5]. They arise from a major or minor peripheral nerve branches [6] or sheath of peripheral nerve fibers [7,8]. These tumors may arise spontaneously in adult patients, although 5% to 42% of MPNST have an association with multiple neurofibromatosis Type-I [9-12]. Thus, a combination of gross, histopathological, and immunohistochemical studies are Rabbit polyclonal to PCSK5 used for diagnosing these tumors. Another interesting clinical feature of this tumor is usually multifocality and development of second primary tumors of same histology [5]. Surgery is the main stay of treatment of this tumor though they are biologically aggressive in nature [5,12,13]. In this article, we reviewed the case records of the patients with malignant buy 527-95-7 peripheral nerve sheath tumors to investigate their clinico-pathological features, treatment outcome, survival, and prognostic factors. Patients and methods A retrospective analysis of MPNST patients treated in January 1994 to December 2002 in the Surgical Oncology unit at All India Institute of Medical Sciences, New Delhi was performed. The source of the data was a soft tissue sarcoma database. Twenty-four out of 200 soft tissue sarcoma patients had MPNST. The clinical details, including the presence or absence of VRHD [14], the histopathology, treatment details, and follow-up were analyzed. Pretreatment evaluation included core needle biopsy or incision and/or excision biopsy for the tissue diagnosis. Extent and stage of the disease was evaluated by the contrast enhanced computed tomography (CT) or magnetic resonance imaging (MRI). Patients buy 527-95-7 with small (<5 cm) and subcutaneously located tumors underwent no imaging. All patients underwent surgical excision as the primary therapy and histopathological examination and immunohistochemical staining confirmed the final diagnosis. Adjuvant radiotherapy and chemotherapy was decided on the basis of the size (>5 cm), depth (deep seated), grade (high), and recurrence of the tumor. After completion of therapy all patients were followed-up every 3 monthly on regular basis. Survival was calculated from the date of diagnosis to the date of last follow-up or death. The details of local buy 527-95-7 and systemic recurrences and second primaries were also analyzed. Development of a new sarcoma of same histology in a different anatomical.