Diagnosis and Management of Genitourinary Rhabdomyosarcoma

2019 ◽  
Author(s):  
Ahmed Abdelhalim ◽  
Zhan Tao (Peter) Wang ◽  
Ali Nael ◽  
Antoine E Khoury

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Genitourinary RMS accounts for 15 to 25% of all RMSs and is a heterogeneous group of soft tissue tumors that vary in presentation, distribution, and prognosis. This article reviews the pathophysiology and tumor biology of RMS. It will also describe the initial approach to its diagnosis and current tumor surveillance protocols. Furthermore, this article presents the evidence behind a number of different staging and risk stratification systems currently used to guide treatment. Lastly, this article reviews future developments of investigational studies and risk stratification under investigation by a number of large international collaborative study groups. This review contains 17 figures, 7 tables, and 68 references. Keywords:  Rhabdomyosarcoma, genitourinary, staging, diagnosis, paratesticular, bladder, prostate, RMS

2020 ◽  
Vol 24 (06) ◽  
pp. 645-666
Author(s):  
Amit Shah ◽  
Kethesparan Paramesparan ◽  
Philip Robinson ◽  
Winston J. Rennie

AbstractClinicians are commonly faced with patients presenting with a solitary palpable soft tissue mass. Most soft tissue lesions are benign, and not every mass is due to a neoplastic process. Many pathologies can mimic a malignant tumor. Despite appropriate clinicoradiologic assessment, these lesions can be mistaken for a soft tissue sarcoma and can lead to multiple investigations or an intervention, inconveniencing patients and leading to an increased health care cost. With the relevant clinical history, clinical examination, and specific imaging characteristics, the diagnosis can be narrowed. We present a pictorial review of soft tissue sarcoma mimics with guidance on appropriate differential diagnoses.


2020 ◽  
Vol 2 ◽  
pp. 82-86
Author(s):  
Vikas Batra ◽  
Nafisa Shakir Batta ◽  
Ankur Gupta

Glomangiomas (glomus tumors) are benign vascular tumors commonly located at the distal extremities, are usually subungual lesions, and account for 2% of all soft-tissue tumors. Patients with digital glomus tumors present with hypersensitivity to cold, paroxysmal severe pain, and point tenderness. These tumors are infrequent in the knee area, and when seen are superficial, usually have a diameter of less than 1 cm, which make their radiological diagnosis arduous. We report a noteworthy, unusual case of a large glomus tumor in the popliteal fossa showing biceps femoris infiltration, in a 51-year-old female patient who experienced severe intermittent posterior knee pain for the past 2 years. Magnetic resonance imaging revealed a large popliteal inhomogeneous soft-tissue lesion with irregular margins insinuating the posterolateral musculature mimicking soft-tissue sarcoma. Histopathology revealed a glomus tumor.


Author(s):  
Peter Hohenberger ◽  
Bernd Kasper ◽  
Kamran Ahrar

Soft tissue sarcomas describe a very heterogeneous group of soft tissue tumors mainly arising in the lower extremities. If diagnosed at an early stage and a complete resection of the primary tumor is achieved, the patients' prognosis is excellent. However, metastatic tumor spread is common with only limited treatment possibilities. Despite an improved insight into tumor biology of sarcomas, no notable improvement has been gained in the last 20 years regarding prognosis of patients. Metastatic lung disease has long been the preserve of systemic treatments, local treatments being considered in a purely palliative intention. Several studies have objectified benefit to the local treatment of metastases, especially in an oligometastatic state. The development of techniques for stereotactic radiotherapy on the one hand and the refusal or contraindication for surgery on the other hand inaugurated studies in this direction. Besides surgery and radiotherapy, other local modalities have been investigated in the last few years such as thermal therapy (radiofrequency and laser ablation) or combined modalities (isolated limb perfusion and deep-wave hyperthermia plus chemotherapy) to help patients with metastatic soft tissue sarcoma. Minimally invasive, image-guided therapies such as thermal ablation should be considered particularly in patients who are not suitable surgical candidates or may have exhausted all other viable surgical options. Some of these techniques will be reviewed in this article, and their value for the patients will be evaluated in the light of indication from tumor biology and technical feasibility. These highly selected and specific procedures should only be performed after decision making in an interdisciplinary sarcoma-board.


2012 ◽  
Vol 111 (3) ◽  
pp. 337-345 ◽  
Author(s):  
Martin Benesch ◽  
André O. von Bueren ◽  
Tobias Dantonello ◽  
Katja von Hoff ◽  
Torsten Pietsch ◽  
...  

Author(s):  
D. C. Swartzendruber ◽  
Norma L. Idoyaga-Vargas

The radionuclide gallium-67 (67Ga) localizes preferentially but not specifically in many human and experimental soft-tissue tumors. Because of this localization, 67Ga is used in clinical trials to detect humar. cancers by external scintiscanning methods. However, the fact that 67Ga does not localize specifically in tumors requires for its eventual clinical usefulness a fuller understanding of the mechanisms that control its deposition in both malignant and normal cells. We have previously reported that 67Ga localizes in lysosomal-like bodies, notably, although not exclusively, in macrophages of the spocytaneous AKR thymoma. Further studies on the uptake of 67Ga by macrophages are needed to determine whether there are factors related to malignancy that might alter the localization of 67Ga in these cells and thus provide clues to discovering the mechanism of 67Ga localization in tumor tissue.


Author(s):  
J. P. Brunschwig ◽  
R. M. McCombs ◽  
R. Mirkovic ◽  
M. Benyesh-Melnick

A new virus, established as a member of the herpesvirus group by electron microscopy, was isolated from spontaneously degenerating cell cultures derived from the kidneys and lungs of two normal tree shrews. The virus was found to replicate best in cells derived from the homologous species. The cells used were a tree shrew cell line, T-23, which was derived from a spontaneous soft tissue sarcoma. The virus did not multiply or did so poorly for a limited number of passages in human, monkey, rodent, rabbit or chick embryo cells. In the T-23 cells, the virus behaved as members of the subgroup B of herpesvirus, in that the virus remained primarily cell associated.


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