Clinicopathological Features And Prognosis of Primary Pulmonary Rhabdomyosarcoma In The Middle-Aged And Elderly: A Case Report And Literature Review

Background: Primary pulmonary rhabdomyosarcoma（PPRMS）in the middle-aged and elderly is a very rare event with only a few cases published. To date, only thirty-five cases of PPRMS in the middle-aged and elderly have been published. However, the analyses on clinicopathological characteristics and prognosis of PPRMS in the middle-aged and elderly have not been performed.Case presentation: We report an additional case of PPRMS in the middle-aged and elderly.Conclusions: PPRMS in the middle-aged and elderly was a highly malignant soft tissue tumor with obvious gender characteristics and significant age distribution. Pleomorphic rhabdomyosarcoma (RMS) was the most common subtype, with poor prognosis and significantly prolonged survival time after surgical resection.

Pleomorphic lipoma: A rare entity

Pleomorphic lipoma is a rare neoplasm that is considered as a variant of spindle cell lipoma. It predominantly occurs in the dermis or subcutis of the posterior neck, upper back, and shoulders. Pleomorphic lipoma may clinically present as a slow-growing and well-circumscribed subcutaneous mass. Though it is a benign tumour it may contain atypical cells for which it may mimick sarcoma or other malignant soft tissue tumours. So histopathological diagnosis is vital for preventing unnecessary surgery. Here we report a case of a pleomorphic lipoma on upper back in a 55yr old patient.

Embryonal Rhabdomyosarcoma with Posttherapy Cytodifferentiation and Aggressive Clinical Course

Rhabdomyosarcoma is the most common soft tissue sarcoma in children and adolescents. Embryonal rhabdomyosarcoma (ERMS), its most common subtype, is a malignant soft tissue tumor with morphologic and immunophenotypic features of embryonic skeletal muscle. The histologic findings in ERMS typically include a range of differentiation in rhabdomyoblasts from primitive to terminally differentiated forms, and the latter become more prominent after chemotherapy-induced cytodifferentiation. Several reports have shown therapy-related cytodifferentiation to portend a good prognosis in ERMS. We discuss the case of a pediatric patient who presented with ERMS of the orbit. Although her tumor showed extensive posttreatment cytodifferentiation and several other good prognostic clinicopathologic factors, it pursued an aggressive course, resulting in early metastasis and death. This case represents an unusual course and may be instructive as to the clinicopathologic features impacting prognostication, and ultimately the biology, of this aggressive family of tumors.

Clinical characteristics of patients with undergoing unplanned excisions of malignant soft tissue tumors

Introduction Malignant soft tissue tumors are rare tumors representing <1% of all malignancies. As these tumors are rare, it is not uncommon that malignant soft tissue tumor excision is performed without the required preoperative imaging, staging, or wide resection margins for sarcomas. The purpose of this study was to investigate the characteristics of patients with undergoing unplanned excisions. Risk factors for tumor recurrence and mortality in patients treated with unplanned excisions were also analyzed. Methods Forty-nine patients who underwent unplanned excision at other hospitals and additional wide excision at our hospital between January 2002 and December 2018 were identified. Among them, 42 patients with follow-up for more than 1 year were included in this retrospective study. The relationships between sex, age, tumor depth, histological grade, location, size, surgical margin at additional wide excision, residual tumor, reconstruction, kind of hospital where the primary excision was done (sarcoma vs non-sarcoma center), preoperative examination, chemotherapy, radiation therapy, and oncological outcomes were statistically analyzed. Results Mean patient age was 57.3 years (15–85 years) and the mean observation period was 72.5 months (14–181 months). This analysis showed 53.8% tumors that underwent unplanned excisions were small (<5 cm) and 70.7% tumors were superficial. Multivariate analysis revealed that a positive margin during additional wide excision was significantly associated with a lower 5-year LRFS ( p < 0.01). Conclusion Most of the tumors underwent unplanned excisions were small (<5 cm) and superficial. Surgeons should be aware that a positive margin during additional wide excision is an independent risk factor for local recurrence.

Radiomics of diffusion-weighted MRI compared to conventional measurement of apparent diffusion-coefficient for differentiation between benign and malignant soft tissue tumors

Diffusion-weighted imaging (DWI) is proven useful to differentiate benign and malignant soft tissue tumors (STTs). Radiomics utilizing a vast array of extracted imaging features has a potential to uncover disease characteristics. We aim to assess radiomics using DWI can outperform the conventional DWI for STT differentiation. In 151 patients with 80 benign and 71 malignant tumors, ADCmean and ADCmin were measured on solid portion within the mass by two different readers. For radiomics approach, tumors were segmented and 100 original radiomic features were extracted on ADC map. Eight radiomics models were built with training set (n = 105), using combinations of 2 different algorithms—multivariate logistic regression (MLR) and random forest (RF)—and 4 different inputs: radiomics features (R), R + ADCmin (I), R + ADCmean (E), R + ADCmin and ADCmean (A). All models were validated with test set (n = 46), and AUCs of ADCmean, ADCmin, MLR-R, RF-R, MLR-I, RF-I, MLR-E, RF-E, MLR-A and RF-A models were 0.729, 0.753 0.698, 0.700, 0.773, 0.807, 0.762, 0.744, 0.773 and 0.807, respectively, without statistically significant difference. In conclusion, radiomics approach did not add diagnostic value to conventional ADC measurement for differentiating benign and malignant STTs.

Protein Arginine Methyltransferase (PRMT) Inhibitors—AMI-1 and SAH Are Effective in Attenuating Rhabdomyosarcoma Growth and Proliferation in Cell Cultures

Rhabdomyosarcoma (RMS) is a malignant soft tissue cancer that develops mostly in children and young adults. With regard to histopathology, four rhabdomyosarcoma types are distinguishable: embryonal, alveolar, pleomorphic and spindle/sclerosing. Currently, increased amounts of evidence indicate that not only gene mutations, but also epigenetic modifications may be involved in the development of RMS. Epigenomic changes regulate the chromatin architecture and affect the interaction between DNA strands, histones and chromatin binding proteins, thus, are able to control gene expression. The main aim of the study was to assess the role of protein arginine methyltransferases (PRMT) in the cellular biology of rhabdomyosarcoma. In the study we used two pan-inhibitors of PRMT, called AMI-1 and SAH, and evaluated their effects on proliferation and apoptosis of RMS cells. We observed that AMI-1 and SAH reduce the invasive phenotype of rhabdomyosarcoma cells by decreasing their proliferation rate, cell viability and ability to form cell colonies. In addition, microarray analysis revealed that these inhibitors attenuate the activity of the PI3K-Akt signaling pathway and affect expression of genes related to it.