Neurogenic Tumors of Soft Tissue

2012 ◽  
Vol 15 (1_suppl) ◽  
pp. 62-107 ◽  
Author(s):  
Justin M.M. Cates ◽  
Cheryl M. Coffin

Neurogenic tumors are an uncommon yet important category of soft tissue tumors in children and adolescents because of their frequent association with various genetic syndromes. The heterogeneous cellular composition of the peripheral nerve and the wide metaplastic capacity of the neural crest and its derivatives generate a variety of neoplasms with neurogenic differentiation. This article reviews the clinicopathologic features and differential diagnosis of neurogenic tumors in the first two decades of life, and highlights use of selected ancillary methods for diagnosis.

2019 ◽  
Vol 23 (01) ◽  
pp. 076-084 ◽  
Author(s):  
Amanda Isaac ◽  
Bianca Bignotti ◽  
Federica Rossi ◽  
Federico Zaottini ◽  
Carlo Martinoli ◽  
...  

AbstractNerve tumors are rare and heterogeneous soft tissue tumors arising from a peripheral nerve or showing nerve sheath differentiation. In a radiologic setting it is necessary to recognize soft tissue lesions that are of neural origin, their association with a peripheral nerve, and whether they are a true tumor or a so-called pseudotumor such as a neuroma, fibrolipoma, or peripheral nerve sheath ganglion. Ultrasound (US) and magnetic resonance imaging are the best modalities to characterize these lesions. US can be used to guide biopsy in difficult and uncertain cases when the lesion is either indeterminate or possibly malignant. At present, no single imaging feature or reproducible criteria, or a combination, can differentiate reliably between a neurofibroma and a schwannoma or discriminate with certainty between benign and malignant neurogenic tumors. Adequate imaging and consultation with a nerve tumors/sarcoma unit is advised.


2012 ◽  
Vol 15 (1_suppl) ◽  
pp. 267-305 ◽  
Author(s):  
Rita Alaggio ◽  
Cheryl M. Coffin ◽  
Sara O. Vargas

Many soft tissue tumors of childhood lack obvious differentiation toward a defined mesenchymal tissue type or have a phenotype that does not correspond to any defined normal tissue. These challenging tumors are currently regarded as neoplasms of uncertain differentiation. Nonetheless, there have been great strides in the understanding of their pathologic and genetic features and biologic underpinnings. The application of new genetic information to the pathologic diagnosis among this group of tumors is an emerging area in diagnostic pediatric pathology. This article reviews the clinicopathologic features of tumors of uncertain and/or miscellaneous origin, with an emphasis on the unique aspects of these neoplasms in children and adolescents, use of diagnostic adjuncts, and differential diagnosis.


2012 ◽  
Vol 15 (1_suppl) ◽  
pp. 11-25 ◽  
Author(s):  
Cheryl M. Coffin ◽  
Rita Alaggio ◽  
Louis P. Dehner

Soft tissue tumors in children and adolescents are an important group of neoplasms, pseudoneoplasms, and tumefactive malformations with some distinctive clinicopathologic, genetic, syndromic, and therapeutic implications. In addition to the basic pathologic examination, there is the availability of diagnostic adjuncts in various settings based upon the histopathologic features that facilitate and/or corroborate a diagnosis. Immunohistochemistry, cytogenetics, molecular genetics, and an ever-increasing array of new technologies are available to address specific diagnostic questions and even potential therapeutic strategies. This review focuses upon some of the unique aspects of soft tissue tumors in children, including the classification, approach to the diagnosis, grading, clinical and pathologic staging, therapy-related changes, pathogenesis, and risk factors.


2016 ◽  
Vol 63 (12) ◽  
pp. 2252-2253 ◽  
Author(s):  
Israel Fernandez-Pineda ◽  
J. J. Jenkins ◽  
T. C. Santiago ◽  
H. J. Prajapati ◽  
A. S. Pappo

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Hyun Sung Kim ◽  
Suk Kim ◽  
Kyungbin Kim ◽  
Kyung Un Choi ◽  
Joo Youn Kim

Myolipomas are very rare benign lipomatous soft tissue tumors which are usually located in retroperitoneum, abdominal and pelvic cavity, and the abdominal wall. They can be diagnosed histologically by the presence of irregularly admixed mature adipose tissue and smooth muscle fibers. The correct diagnosis of myolipoma is important, because it should be considered in the differential diagnosis of fat-containing lesions of the soft tissue and should follow a benign clinical course despite its frequently large size and deep location. We report here a case of myolipoma arising in the mesentery of the jejunum.


PLoS ONE ◽  
2022 ◽  
Vol 17 (1) ◽  
pp. e0262272
Author(s):  
Joanna Przybyl ◽  
Lien Spans ◽  
Kristen Ganjoo ◽  
Nam Bui ◽  
David Mohler ◽  
...  

High-level amplification of MDM2 and other genes in the 12q13–15 locus is a hallmark genetic feature of well-differentiated and dedifferentiated liposarcomas (WDLPS and DDLPS, respectively). Detection of this genomic aberration in plasma cell-free DNA may be a clinically useful assay for non-invasive distinction between these liposarcomas and other retroperitoneal tumors in differential diagnosis, and might be useful for the early detection of disease recurrence. In this study, we performed shallow whole genome sequencing of cell-free DNA extracted from 10 plasma samples from 3 patients with DDLPS and 1 patient with WDLPS. In addition, we studied 31 plasma samples from 11 patients with other types of soft tissue tumors. We detected MDM2 amplification in cell-free DNA of 2 of 3 patients with DDLPS. By applying a genome-wide approach to the analysis of cell-free DNA, we also detected amplification of other genes that are known to be recurrently affected in DDLPS. Based on the analysis of one patient with DDLPS with longitudinal plasma samples available, we show that tracking MDM2 amplification in cell-free DNA may be potentially useful for evaluation of response to treatment. The patient with WDLPS and patients with other soft tissue tumors in differential diagnosis were negative for the MDM2 amplification in cell-free DNA. In summary, we demonstrate the feasibility of detecting amplification of MDM2 and other DDLPS-associated genes in plasma cell-free DNA using technology that is already routinely applied for other clinical indications. Our results may have clinical implications for improved diagnosis and surveillance of patients with retroperitoneal tumors.


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