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Epithelioid sarcoma in the forearm shows atypical epithelioid cells in sheets associated with tumor necrosis (right portion) (A). The neoplastic cells have nuclei and prominent nucleoli, with eosinophilic glassy cytoplasm (B). (Hematoxylin and Eosin original magnification; A, ×150; B, ×200).
Source publication
Epithelioid sarcoma is an uncommon high-grade sarcoma, mostly involving the extremities.
A 33-year-old man was referred to our institute with a diagnosis of Volkmann's contracture with the symptom of flexion contracture of the fingers associated with swelling in his left forearm. Magnetic resonance imaging (MRI) showed abnormal signal intensity, co...
Citations
... Due to the ulceration differential, diagnosis should include non-healing wounds and warts [20]. ES is also often initially recognized as inflammatory or granulomatous lesions or other benign conditions [21]. At the time of diagnosis, ES tumors are usually small with a diameter below 5 cm [13,22]; however, in some cases, mostly in proximal variant, ES tumors reach over 20 cm dimension [1,23]. ...
Epithelioid sarcoma is a mesenchymal soft tissue sarcoma often arising in the extremities, usually in young adults with a pick of incidence at 35 years of age. Epithelioid sarcoma (ES) is characterized by the loss of SMARCB1/INI1 (integrase interactor 1) or other proteins of the SWI/SNF complex. Two distinct types, proximal and distal, with varying biology and treatment outcomes, are distinguished. ES is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. An optimal long-term management strategy is still to be defined. The best treatment of localized ES is wide surgical resection. Neo-adjuvant or adjuvant radiotherapy may be recommended, as it reduces the local recurrence rate. Sentinel lymph node biopsy should be considered in ES patients. Patients with metastatic ES have a poor prognosis with an expected median overall survival of about a year. Doxorubicin-based regimens are recommended for advanced ES. Tazemetostat, an EZH2 methyltransferase, has shown promising results in ES patients. Novel therapies, including immunotherapy, are still needed.
... To best of our knowledge, there are only 2 case reports of this nature in the English literature. 7,8 The rarity of this condition might reflect the low incidence of epithelioid sarcoma of the flexor muscles of the forearm. 3 In the current case, flexion contracture of the fingers occurred gradually after finger numbness, and ischemic and degenerative changes in the flexor muscles were initially suspected. ...
Epithelioid sarcoma is an uncommon soft tissue sarcoma involving predominantly the distal extremities of adolescents and young adults. Its rarity makes it difficult to diagnose accurately and treat properly in the early stages. We discuss the delayed diagnosis of a 37-year-old man who presented with extrinsic flexor tightness of the wrist and fingers. We initially thought that the lesion resulted from inflamed soft tissue of the flexor muscles causing contracture. However, histological examination of a biopsy specimen revealed nodular proliferation of epithelioid and spindle cells, which were immunoreactive to epithelial and nonepithelial markers, respectively, leading to the final diagnosis of epithelioid sarcoma.
... Ulceration of the overlying skin, numbness, tingling sensation and pain can also occur especially with a concurrent nerve involvement (9). An extensive literature search revealed that there was only single reported case of epithelioid sarcoma associated with Volkman ischaemic contracture similar to the present one (10). ...
... The clinical behavior of this rare sarcoma is unpredictable with some patient showing tumour recurrence or a distant spread while the other may remains disease-free for years (10). Two cases of epithelioid sarcoma of the vulva were described by Hall et al andTjalma et al. in 1980 and1990, respectively (11, 12) in which the tumour showed different behavior with the former patient (11) having tumour recurrence and distant metastasis while the later (12) remained disease-free four years, post surgery. ...
Epithelioid sarcoma is a very infrequent soft tissue sarcoma involving predominantly distal extremities of adolescent and young adult. We hereby report a case of epithelioid sarcoma in a 34-year- old young adult who presented with 1-year history of a painful left upper limb associated subsequently with warm left forearm swelling and a Volkman contracture. He was treated as an inflamed soft tissue condition of the left upper limb. A computed tomography study showed presence of multiple hypodense lesions mainly in the flexor compartment of the left arm as well at the subcutaneous tissue, which measured 1 to 1.7 cm in diameter. Histological examination of the left upper limb mass showed nodular proliferation of epithelioid tumour cells and some with rhabdoid features surrounding a central zone of necrosis and was diagnosed as epithelioid sarcoma. Concurrent presence of epithelioid sarcoma and Volkman ischaemic contracture are rarely seen in clinical practice. The present case highlights the importance of the histology which can be confused with other types of sarcoma or chronic granuloma and even missed at times thereby causing diagnostic problems. Clin Ter 2012; 163(3):215-217.
... In addition, recent studies of fluoro-D-glucose (FDG) positron emission tomography (PET) imaging have shown possible benefits in determining metastasis of epithelioid sarcoma. 7,8 This high-grade sarcoma is reported to have a local recurrence rate as high as 77% and a metastatic rate of up to 45%, with metastases most commonly found in the lung and regional lymph nodes, followed by the scalp and bone. 2,3 Due to the aggressive nature of the tumor, early detection and proper treatment are vital. ...
... These imaging studies were followed by FDG-PET that showed multiple areas of increased uptake, which were confirmed at a later time to be epithelioid sarcoma. 8 This study displays the value of FDG-PET in assisting with early diagnosis and treatment of epithelioid sarcoma metastasis, a value reiterated by this case. ...
Epithelioid sarcoma is rare soft tissue sarcoma first described by Enzinger in 1970. The classic variant often occurs in the deep or superficial soft tissue of the distal upper extremities of individuals between the ages of 10 and 35. Due to the tumor's benign clinical presentation, infrequent occurrence, and histological similarities with other disease processes, diagnosing epithelioid sarcoma in its early stages has become extremely difficult. We report a rare case of epithelioid sarcoma of the toe with bone metastasis, as well as the clinical, pathological, and radiological difficulties in correctly diagnosing epithelioid sarcoma.
... Muscular metastases are uncommon, but occur in many types of malignancy. 130,131 Correlation with the integrated CT or a separate diagnostic CT or MRI may be necessary to identify an anatomic lesion for the diagnosis (Fig. 24). If anatomic imaging modalities do not demonstrate pathology, the uptake is most likely benign in nature, due to physiological, traumatic or inflammatory causes. ...
A routine feature of positron emission tomography/computed tomography (PET/CT) imaging is whole-body acquisition that results in many unexpected findings identified outside of the primary region of abnormality. Furthermore, (18)F-fluorodeoxyglucose (FDG) is a marker of glycolysis and does not specifically accumulate in malignancy. Understanding the physiology and pathophysiology of normal FDG distribution and common incidental findings is therefore essential to the physician interpreting whole-body FDG-PET/CT studies. Whereas many incidental findings are benign and of limited clinical significance, others represent uncommon manifestations of the primary malignancy, second malignancies, or various clinically significant pathologic processes. Patients with a single malignancy are at greater risk of developing synchronous or metachronous second malignancies, possibly related to exposure to shared carcinogenic agents or presence of prooncogenic mutations. The decision of how to pursue an intervention on the basis of an incidental finding is generally left to clinical judgment.
... Muscular metastases are uncommon, but occur in many types of malignancy. 130,131 Correlation with the integrated CT or a separate diagnostic CT or MRI may be necessary to identify an anatomic lesion for the diagnosis (Fig. 24). If anatomic imaging modalities do not demonstrate pathology, the uptake is most likely benign in nature, due to physiological, traumatic or inflammatory causes. ...
A routine feature of positron emission tomography-computed tomography imaging is a whole-body acquisition that results in many unexpected findings identified outside of the primary region of abnormality. Furthermore, 18 F-fluorodeoxyglucose (FDG) is a marker of glycolysis and does not specifically accumulate in malignancy. Understanding the physi-ology and pathophysiology of a normal FDG distribution and common incidental findings is therefore essential to the physician interpreting whole-body FDG positron emission to-mography-computed tomography studies. Whereas many incidental findings are benign and of limited clinical significance, others represent uncommon manifestations of the primary malignancy, second malignancies, or various clinically significant pathologic pro-cesses. Patients with a single malignancy are at greater risk of developing synchronous or metachronous second malignancies, possibly related to exposure to shared carcinogenic agents or presence of prooncogenic mutations. The decision of how to pursue an inter-vention on the basis of an incidental finding is generally left to clinical judgment. Semin Nucl Med xx:xxx © 2010 Published by Elsevier Inc. P ositron emission tomography (PET) has experienced ex-plosive growth as a clinical modality, especially in the realm of oncology. A component of this success has been the introduction of scanners which integrate PET and computed tomography (CT), allowing both studies to be performed sequentially, without need to reposition the patient. 1 The most-commonly used PET radiotracer, 18 F-fluorodeoxyglu-cose (FDG), is a glucose analogue that accumulates in malig-nant tumors exhibiting increased glucose transport and glycolysis. 2 Therefore, FDG-PET is well-established as an im-aging modality in oncology for tumor staging, restaging, sur-veillance of recurrence, and the monitoring of treatment re-sponse. 3-5 A routine feature of PET/CT imaging is whole-body acqui-sition ("eyes to thighs"), which results in many unexpected findings identified outside of the primary region of abnor-mality. Furthermore, FDG is a marker of glycolysis and does not specifically accumulate in malignancy. Various degrees of FDG uptake are therefore present in normal tissues and are noted in benign processes, such as inflammation and infec-tion. Generation of attenuation-corrected PET images is also an intricate multistep procedure; various deviations from ideal will result in artifactual areas of apparent uptake. The accurate interpretation of unexpected findings remains a challenge. Understanding the physiology and pathophysiol-ogy of incidental findings on FDG-PET/CT is essential to the interpreting physician. The purpose of this article is to pro-vide a brief review of common incidental findings, providing insight into their physiology and pathophysiology wherever possible.
Introduction
Epithelioid sarcoma (ES) is a rare histopathological type of soft tissue sarcoma. They are subcutaneous soft tissue masses that tend to arise in extremity sites (the classic type, formerly referred to as distal type) or proximal midline region of the body (the proximal type), such as the perineum, genital tract, and pelvis. The head and neck regions are rarely affected by ES, but the infratemporal fossa (ITF) is extremely rare. ES involving the ITF has not been reported before in literature. In this paper, the imaging features of ES were reviewed in detail, and the anatomical structure and epidemiology of ITF were briefly introduced.
Sources
We performed a systematic search from 3 databases, CNKI(China National Knowledge Internet), FMRS(Foreign Medical Literature Retrieval Service, Shenzhen METSTR Technology CO., Led. China), and PubMed, to obtain literature from January 1970 to July 2020. Epithelioid sarcoma, head and neck regions, infratemporal fossa, diagnostic imaging, anatomy, MRI, and CT were used as keywords for advanced retrieval. A study had to be found eligible for inclusion to be closely related to ES and/or ITF. A total of 129 related pieces of literature were retrieved successfully, of which 37 were closely related to this study. The case report in this article is from the Affiliated Hospital of North Sichuan Medical College. The present study was approved by the institutional review board of the Affiliated Hospital of North Sichuan Medical College and a written informed consent for the publication of the case was obtained from the patient.
Case Presentation
A 33-year-old man accidentally found a slowly growing, painless mass in the left cheek more than a month ago. On plain computed tomography (CT) scan of the outpatient department, an oval slightly low-density mass with well-defined and uniform boundary in the infratemporal fossa was revealed, and on the contrast-enhanced scan, it was homogeneous and moderately enhanced. Initially, the CT appearance favored benign lesions. However, the possibility of a malignant tumor can not be excluded completely. Finally, the patient was referred to the inpatient department a tumor arose from IF and underwent a selective operation. The tumor was completely removed. Histologic findings were compatible with epithelioid sarcoma. Post-operatively, the patient received 2 cycles of radiotherapy, and there was no evidence of recurrence after 6 months follow-up.
Conclusion
The imaging manifestations of ES are various. The anatomic structure of ITF is complex and the pathological types are various. It should be very careful in the qualitative diagnosis of tumors from the ITF, and advanced imaging techniques will be useful in imaging diagnosis.
The epithelioid sarcoma (ES) is a rare, high-grade malignant soft tissue tumor, which shows preference for distal parts of the extremities, particularly the hand. It differ from benign lesions (e.g. ganglion) in ways of that are often non apparent until there is recurrence. Regional lymph node involvement and distant metastases (lungs) are often apparent. Treatment of the ES includes the primary radical surgical excision, sometimes combined with sentinel lymph node biopsy and radiation or chemotherapy. Amputation of the affected fingers is necessary in all cases of recurrence but might be avoided in initial surgery with respect to pediatric patients and maximal function. Consequently, soft tissue defects must be treated following plastic surgical principles. Close oncological follow-ups are inevitable, since there can be tumor recurrence even years after resection of the primary tumor.
As a functional imaging method, positron emission tomography (PET) is widely used in tumor diagnosis, pathological classification, stage assessment, and clinical outcome evaluation. Combination of metabolic information acquired from 18F-fluorodeoxyglucose (18F-FDG) PET with pathological grading results is helpful for further defining the grade of sarcomas and predicting prognosis. 18F-FDG PET appears to be superior to other imaging modalities in detecting bone metastases or pulmonary metastases of sarcoma patients, so it could be used in clinical staging and restaging of sarcoma. Tumor-to-background ratio (TBR) and standardized uptake value (SUV) are two important parameters for quantitative analysis and are helpful for assessing chemotherapy response of tumor. PET imaging offers important information to help maximize the clinical benefit of patients with sarcoma. This article reviews the clinical value of 18F-FDG PET and 18F-FDG PET/CT in diagnosis of sarcoma.
