Ewing's sarcoma of the parotid gland: A rare entity with review of the literature.

Background: Ewing's sarcoma is a rare malignant entity. Extraosseous Ewing's sarcoma (EES) of the head and neck region is a rare occurrence, and Ewing's sarcoma of the parotid gland is even rarer. To the best of our knowledge, we reported the first case of extraskeletal ES originating from the parotid gland in the Tunisian literature. Case report: We report a rare case of EES of the parotid gland in a 35-year-old female. She presented with left parotid tumefaction. Physical examination revealed solid and fixed mass associated with facial paralysis. Magnetic resonance imaging illustrated a left intra-parotid process occupying the entire gland measuring 42 mm infiltrating the masseter and pterygoid muscles. The patient had a total left parotidectomy with ipsilateral triangular lymph node dissection. The definitive pathological examination and the immunohistochemical staining confirmed a primary peripheral neuroectodermal tumor or PNET with the presence of a specific EWING/PNET-type translocation in 60% of the tumor cells. She had an adjuvant chemotherapy (four cycles of vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide and etoposide) followed by external radiotherapy. Conclusion: A clinical and radiological follow-up by cervical MRI was done every 3 months and The 10-month follow-up showed no locoregional and distant recurrence.

Introduction: Le sarcome d'Ewing est une entité maligne rare. La localisation extra osseuse en particulier la région de la tête et du cou est caractérisée par son agressivité locorégionale. Nous rapportons le cas d'un sarcome d'Ewing de la parotide. A notre connaissance, il s'agit du premier cas rapporté dans la littérature tunisienne. Presentation du cas: Il s'agit d'une femme âgée de 35 ans qui a consulté initialement pour une tuméfaction au niveau de la glande parotide gauche. L'examen clinique a révélé une masse sous angulomandibulaire associée à une paralysie faciale périphérique gauche. Une IRM parotidienne a objectivé un processus intra-parotidien gauche occupant l'entièreté de la glande mesurant 42 mm mal limitée infiltrant modérément le muscle masséter et ptérygoïdiens. Le bilan d'extension était sans anomalie. La patiente a eu une parotidectomie gauche large avec un curage triangulaire homolatéral. L'examen anatomopathologique définitif et l'étude par hybridation in situ en fluorescence (FISH) ont confirmé la présence d'une translocation spécifique type EWING/PNET dans 60% des cellules tumorales. La patiente a été traitée par une chimiothérapie type VDC/IE (vincristine, doxorubicine, cyclophosphamide en alternance avec ifosfamide, et etoposide) suivie d'une radiothérapie externe. Un suivi clinique et radiologique trimestrielle n'a montré aucune récidive locorégionale ni à distance à ce jour soit à 10 mois de recul. Conclusion: Une surveillance clinique et radiologique trimestrielle a été faite et le contrôle à 10 mois n'a pas montré de récidive locorégionale ou à distance.

18 F-Fluorodeoxyglucose Uptake in Bilateral Diaphragmatic Crura: A Relatively Uncommon Benign Variant Noted in a Treated Case of Extraosseous Paraspinal Ewing's Sarcoma.

A toddler was diagnosed with extraosseous Ewing's sarcoma, primary large epidural paraspinal soft tissue in the lumbar region encasing the cord and neural foramen from D12-L1 to L4-L5. After eight cycles of induction chemotherapy with vincristine, doxorubicin, and cyclophosphamide alternating with etoposide and ifosfamide, 18 F-FDG positron emission tomography/computed tomography ( 18 F-FDG-PET/CT) scan confirmed no active disease. Later external beam radiotherapy (EBRT) at D10-L5 was completed. At 3 months follow-up, 18 F-FDG-PET/CT reconfirmed no residual/active disease; however, a new incidental finding of diffuse benign bilateral diaphragmatic 18 F-FDG uptake was noted in the clinically asymptomatic patient, which remained unexplained.

Primary intradural Extraosseous Ewing's sarcoma of the cauda equina: a Case Report and Literature Review.

Intradural Extraosseous Ewing sarcoma (IEES) is an infrequent occurrence. We report a case of a 66-year-old male who presented with a 2-month history of low back pain and bilateral S1 sciatica, with acute sphincter dysfunction. Imaging studies revealed an intradural extramedullary lesion in the cauda equina spanning from level L4 to S1. The patient underwent partial removal of the intradural lesion. Histopathological examination showed the presence of small round cells, which were consistent with Ewing Sarcoma. The patient was then treated with targeted radiation therapy and chemotherapy. The rarity of IEES in this specific location underscores the significance of evaluating and managing patients with intradural spinal tumors with careful consideration of this diagnosis. To further investigate this condition, we conducted a thorough review of the literature on IEES involving the lumbar spine and cauda equina. Our analysis revealed that patients with this condition frequently exhibit rapidly progressive neurological symptoms likely attributed to hemorrhagic transformation. This characteristic may serve as a distinguishing factor from other lesion types, particularly benign ones. Our study provides a comprehensive summary that can offer direction for clinical management in comparable uncommon and novel cases.

Upper Urothelial Tract Extraosseous Bone Formation: An Unexpected Finding and Differential Diagnostic Considerations.

Extraosseous bone formation of the upper urothelial tract is an unusual phenomenon with limited documentation in the uropathology literature, reported in only 2 clinical series of patients undergoing percutaneous nephrolithotomy for the management of renal stones. While speculations regarding the pathogenesis of this occurrence have been published, heterotopic ossification is still poorly understood. We report the finding of extraosseous bone formation in the renal pelvis of a 30-year-old male patient with a history of kidney stones. Histologic sections of the ureter and renal pelvis showed submucosal nodules of woven bone. Ancillary fluorescence in-situ hybridization studies were negative for MDM2 amplification and USP6 rearrangement.

Natural history of bone-only metastasis in renal cell carcinoma.

BACKGROUND: Bone metastasis (BM) is considered a poor prognostic factor of renal cell carcinoma (RCC). Confusion exists regarding how to deal with RCC patients with bone-only metastasis. PATIENTS AND METHODS: The clinical data of consecutive RCC patients with bone-only metastasis at Peking University Cancer Hospital between 2006 and 2018 were retrospectively collected and analyzed. RESULTS: Fifty-four eligible patients were screened from an RCC database of 1,878 metastatic patients. After a median follow-up of 43.6 m, 61.1% of the patients were presented with progression of prior BM or new BM. The progression-free survival (PFS) and overall survival (OS) was 16.2 m (95%CI: 11.4-21.0) and 65.2 m, respectively. For the 30 patients with oligo-metastasis (≤3 loci) and 24 ones with multiple-metastasis (>3 loci), the median OS was not reached and 42.0m (95%CI: 12.7-71.2) with statistical difference (P < 0.001). In the oligo-metastasis group, the median PFS of the 15 patients treated with local therapy and of the 13 patients treated with systemic therapy was 14.2 m (95%CI: 5.3-23.3) and 18.0 m (95%CI:15.4-20.6), respectively. In the multiple-metastasis group, the median PFS and OS of the 18 patients treated with systemic therapy was 16.6 m (95%CI: 7.5-25.7) and 63.9 m (95%CI: 21.8-106.0), respectively. Univariate analysis and multivariate analysis showed that the number of metastatic sites (oligo/multiple) and International Metastatic Renal Cell Carcinoma Database Consortium (IMDC) score, RCC pathological subtype were significantly associated with prognosis (P < 0.05). CONCLUSION: RCC patients with bone-only metastases have a favorable prognosis. The number of metastatic sites, IMDC, RCC pathological subtype could serve as survival predictors, which might provide clue of treatment modality.

Unveiling the Uncommon: A Case of Metastatic Ewing Sarcoma of the Kidney.

Primary Ewing sarcoma of the kidney (ESK) is a rare and aggressive entity, with a poor prognosis. It often presents as metastatic disease with the lungs being the most common site. In adults, the occurrence of these tumors is uncommon, with patients exhibiting non-specific symptoms such as weight loss, flank pain, hematuria, and an abdominal mass. The combination of these vague clinical symptoms and the rarity of these tumors often results in a delayed diagnosis, leading to poorer outcomes for these patients. We present a case of a 38-year-old female with metastatic ESK. The patient initially presented with abdominal pain, vomiting, and a four-day history of constipation. The diagnosis was confirmed through computed tomography scans, ultrasound-guided biopsy of the lesion, and fluorescence in situ hybridization that revealed translocation of the EWS gene on chromosome 22q12. She was managed with chemotherapy regimens and palliative care; however, the disease progressed and she passed away six months after her initial diagnosis.

A unique case of a recurrent extraosseous ossifying fibroma of the orbit: A diagnostic dilemma.

The aim is to present a unique clinical case of a 62-year-old male with an ossifying fibroma (OF) in the left orbit. He presented with a slow-growing mass with bone consistency which easily shifted with palpation. After removal, it was given the histopathological diagnosis of osteoma. However, the lesion grew back rapidly and it became even larger 4 months later. After a computed tomography scan with a contrast medium, another surgery was performed. The histopathological diagnosis of the recurrent lesion was an epidermal cyst with secondary degenerative changes. As both diagnoses did not fit the clinical picture, we sent the material from both surgeries for a secondary assessment. The final histopathological diagnosis was OF. OF should be included in the differential diagnosis of benign lesions in the orbit with aggressive behavior.

Ewing's Sarcoma of the Hand: An Unusual Presentation in a Young Hispanic Male.

Ewing's sarcoma is a neuroectodermal malignancy classically associated with innocuous and chronic symptomatology. Although tumors typically involve the axial skeleton, some malignancies may be confined to extraosseous tissue only. This report presents the case of a 15-year-old Hispanic male with a tender, slow-growing mass of seven months in the subcutaneous tissue of the right hand. Core needle biopsy and fine needle aspiration confirmed the diagnosis of high-grade extraosseous Ewing's sarcoma and the patient was treated via surgical resection and chemotherapy. Nonspecific findings of Ewing's sarcoma may mimic infection or trauma and contribute to a delay in diagnosis. However, social and economic influences including limited English proficiency and insurance status also critically affect the timing of presentation.

Extraosseous Ewing sarcoma in the fossa jugular: A rare case report.

Background: Extraosseous Ewing sarcoma (EES) is a rare case that accounts for 20% of Ewing sarcoma cases. EES is the second most prevalent pediatric malignancy after peripheral primitive neuroectodermal tumors. EES mostly arise from soft tissue and extra-skeletal. Computed tomography (CT) and magnetic resonance imaging (MRI) are primary modalities for determining tumor location, characteristics, type, and extent of tumors. In addition, for presurgical management, radio intervention with arterial embolization is needed as a preoperative. Case Description: We present a case of a 15-year-old boy diagnosed with EES. He had a "horn-like" tumor that grew progressively on his right ear over 5 months. Head CT scan and MRI were conducted to assess the extent. Embolization was performed before surgery. The surgery was conducted to excise the tumor radically. The histology pathology examination showed EES. Conclusion: EES rarely occurs in the head and neck. This may manifest as a solid mass with bleeding components that destroy the nearby bones, with exophytic mass. Imaging is important for early finding and detecting complications of EES.

A prognostic nomogram and risk classification system of elderly patients with extraosseous plasmacytoma: a SEER database analysis.

BACKGROUND: The survival trends and prognostic factors of patients with extraosseous plasmacytoma (EOP) or extramedullary plasmacytoma (EMP) have not been reported in recent years. The objective of this study was to develop a novel nomogram and risk stratification system for predicting the overall survival (OS) of elderly patients with EOP based on the Surveillance, Epidemiology, and End Results (SEER) database. METHODS: The demographic characteristics of 900 patients aged 60 years and above, diagnosed with EOP between 2000 and 2019, were extracted from the SEER database. The patient population was randomly divided into a training cohort and an internal validation cohort in a ratio of 7:3. Univariate and multivariate Cox regression analyses were conducted to identify independent predictors of prognosis in elderly EOP patients, followed by developing a nomogram for prognostic assessment. The performance of the model was evaluated through receiver-operating characteristic (ROC) curves, C-index, calibration curves for calibration accuracy assessment, and decision curve analysis (DCA) to assess its clinical utility. All elderly EOP patients were stratified into three risk subgroups by cutoff value utilizing X-tile software based on their total OS scores for comparative analysis purposes. Kaplan-Meier (K-M) survival curve analysis was employed to validate any observed differences in OS among these three risk groups. RESULTS: Six factors including age, year of diagnosis, marital status, primary site, surgery, and prior tumor history were identified to be independently predictive of the OS of elderly patients with EOP, and these predictors were included in the construction of the nomogram. The 1-, 3-, and 5-year area under the curves (AUCs) for OS were 0.717, 0.754, and 0.734 in the training cohort and 0.740, 0.730, and 0.765 in the validation cohort, respectively. The C-index values in the two cohorts were 0.695 and 0.690. The calibration curves and DCA exhibit commendable consistency and validity, respectively, thereby demonstrating their robust performance. The training set was stratified into low-, medium-, and high-risk subgroups based on the optimal cutoff points (167.8 and 264.8) identified. The K-M curve and cumulative risk curve exhibited statistically significant disparities in survival rates among the groups. CONCLUSIONS: We developed a nomogram and risk classification system, which can serve as an intuitive and effective tool for clinicians to enhance the prediction of OS in elderly EOP patients, thereby facilitating the formulation of more rational and personalized treatment strategies.

Case Report: Primary osteosarcoma of the kidney.

Extraosseous osteosarcoma is a rare malignant tumor, most commonly occurring in the thigh, upper limbs, and retroperitoneum. However, there are only a few reported cases of renal osteosarcoma. Herein, we present the case of a 54-year-old woman with malignant extraosseous osteosarcoma of the left kidney. CT and MR imaging revealed a soft tissue mass originating from the left kidney.

A case of a dog with mandibular extraskeletal osteosarcoma after long-term puncture extirpation of the salivary gland cyst in the mandible.

Background: Extraskeletal osteosarcoma, unlike skeletal osteosarcoma, is a rare malignant mesenchymal tumor with a soft tissue primary that has been reported to occur in a variety of soft tissues. Case Description: The case is a 14-year-old, unneutered male Miniature Pinscher, weighing 6.7 kg, who had been treated medically for more than 5 years with a management strategy of puncture extirpation of a salivary gland cyst in the mandible; 1 month earlier, the fluid retention could not be removed, and after a computerized tomography scan showed no lesion in the mandible adjacent to the mass lesion, surgical resection was performed. Conclusion: Previous reports of extraskeletal osteosarcoma from the salivary glands in dogs have been rare. However, treatment of a salivary gland cyst in the mandible by long-term puncture extirpation may be a potential predisposing factor for the development of extraskeletal osteosarcoma around the mandible.

Extra-skeletal Ewing's sarcoma of the leg with multiple skeletal and pulmonary metastases: A rare pediatric case report.

Being the second most common malignant bone tumor in children and young adults, Ewing's sarcoma can also occur as a primary soft-tissue tumor called extraosseous or extra-skeletal Ewing's sarcoma. It is a rare entity, especially in the pediatric population. We report the case of an adolescent who presented to our department for lower extremity magnetic resonance imaging to explore leg swelling. It revealed an extra-skeletal Ewing's sarcoma with multiple bone metastases. By reporting this case, we also review the literature on this rare abnormality.

Clinicodemographic characteristics of extraosseous ewing sarcoma: A comparative meta-analysis of pediatric and adult patients.

Background: Evidence suggests different presentation patterns and prognosis of extraosseous Ewing Sarcoma (EES) based on age. Thus, we carried out this study to test the difference between children and adult EES cases regarding clinicodemographic characteristics and prognosis. Methods: A total of 4 databases were explored yielding 18 relevant studies for data synthesis. Outcomes included the comparison of demographic and clinical characteristics as well as prognosis between children and adults with EES. Log odds ratio (logOR) and its 95% confidence interval (CI) were pooled across studies. Statistical models/methods were selected based on heterogeneity. Results: Our analysis included a total of 1261 children and 1256 adults. When we compared these two age categories, we did not observe a significant difference in the risk of developing EES [logOR = -0.13; 95% CI: -0.65: 0.39; I2 = 88.42%]. No significant differences regarding gender, tumor location, and size (≤5 vs. >5 cm), EWSR1 positivity, or management modality. We did not observe significant difference regarding clinical outcomes, such as 5-year overall survival and event-free survival, recurrence, mortality, no evidence of disease, and secondary metastasis. Conclusions: Our findings highlight the absence of an association between the age category of patients and the incidence of EES, as well as its clinical outcomes.

Survival Differences of Patients with Resected Extraskeletal Osteosarcoma Receiving Two Different (Neo)Adjuvant Chemotherapy Regimens: A Systematic Review and Meta-analysis.

AIMS: Extraskeletal osteosarcoma (ESOS) is a malignant tumour developing in soft tissues, characterised by the production of osteoid or bone matrix by tumour cells. The standard treatment for localised ESOS is wide resection. Radiotherapy and chemotherapy are usually incorporated into the management of patients. Two types of chemotherapy regimen are mostly used: an osteosarcoma-type chemotherapy, based on cisplatin, and a soft-tissue sarcoma (STS)-type chemotherapy, using the combination of doxorubicin and ifosfamide. To investigate the difference in survival between these two chemotherapy regimens, a systematic review of studies reporting the 5-year disease-free survival (DFS) rates among patients with ESOS submitted to surgery and who received (neo)adjuvant chemotherapy with osteosarcoma-type or STS-type chemotherapy was carried out. MATERIALS AND METHODS: Of the 401 articles identified by systematically searching the PubMed, Embase and Cochrane Central Register of Controlled Trials databases, six retrospective studies were included in the final analysis. In total, 319 patients with localised/resected ESOS were included in the study. RESULTS: Our meta-analysis showed a benefit in 5-year DFS favouring the use of osteosarcoma-type chemotherapy (relative risk = 1.32, 95% confidence interval 1.03-1.69; P = 0.54); I2 heterogeneity was 0%. The 5-year DFS rate was 56.3% (95% confidence interval 48.3-64.3) with osteosarcoma-type chemotherapy and 45.2% (95% confidence interval 34.5-55.9) with STS-type chemotherapy, with I2 heterogeneity of 27% and 0%, respectively. CONCLUSIONS: Our analysis suggests that there may be a difference regarding the type of (neo)adjuvant chemotherapy regimen used in the treatment of patients with resected ESOS in favour of osteosarcoma-type chemotherapy. Future studies evaluating the role of this treatment modality in this scenario need to consider the type of chemotherapy regimen when comparing with an arm of surgery with/without radiotherapy alone.

Solitary Extraosseous Plasmacytoma of Submandibular Gland - Report of a Rare Case.

BACKGROUND: Plasmacytomas are tumors involving plasma cells that can be either osseous or extraosseous. Solitary extraosseous plasmacytomas are rarely found in the head and neck region. Various pathological, immuno-histochemistry, and radiological investigations must be done to distinguish it from multiple myeloma. CASE: A 41-year-old female presented with a progressively increasing mass in right submandibular region for 1 year. She also had complaints of dysphagia and dyspnea for 3 months. Histopathological examinations showed lymphoplasmacytic infiltrate and possibility of plasma cell neoplasm was suspected. On further investigations like immunohistochemistry, bone marrow biopsy, quantitative immunoglobulin, immunoelectrophoresis, and urine analysis, diagnosis of extraosseous plasmacytoma was made. Positron emission tomography-computed tomography (PET-CT) scan did not show any distant foci of the disease. The patient received definitive radiotherapy to the primary region and tolerated well. The patient is asymptomatic after 1 month of radiotherapy and is on regular follow-up. CONCLUSIONS: Extraosseous plasmacytoma of the submandibular region is very rare but radiosensitive tumor. Multiple myeloma must be ruled out prior to start of any treatment. Long term follow ups are required to comment on disease progression, dissemination, or recurrence.

Epidemiology and survival of primary extraosseous plasmacytoma: insights from a population-based study with a 20-year follow-up.

Primary extraosseous plasmacytoma (PEP) is a rare and localized form of plasmacytoma that is not well understood. This study aimed to investigate the clinical features and prognostic factors associated with PEP. Using the Surveillance, Epidemiology, and End Results (SEER) database, a total of 1044 patients diagnosed with PEP between 2000 and 2019 were identified. The average age was 60.3 ± 15.2 years, with 64.3% being male (male: female = 1.8:1) and 53.8% being over 60-year old. The survival outcome of patients with PEP depends on several factors including age, race, marital status, and treatment options such as chemotherapy, radiotherapy, and surgery, which were also identified as independent predictors of overall survival for PEP. Patients who were younger, Asian or Pacific Islander, American Indian or Native American, and received radiotherapy or surgery had a more favorable prognosis, while those who underwent chemotherapy had poorer outcomes.

Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma.

With the exception of well-differentiated liposarcoma, dedifferentiated liposarcoma, leiomyosarcoma, solitary fibrous tumour, malignant peripheral nerve sheath tumour, and undifferentiated pleomorphic sarcoma, the majority of the ≈70 histologic subtypes of retroperitoneal sarcoma are defined as 'ultra-rare' sarcomas, with an incidence of ≤1-5/1,000,000 persons/year. For most of these ultra-rare RPS subtypes, diagnosis and treatment follows international guidelines for the management of more common RPS histologies, with en bloc surgical resection as the mainstay of curative treatment, and enrolment in clinical trials where possible. Because the treatment of RPS is heavily driven by histology, the surgeon must be familiar with specific issues related to the diagnosis and management of ultra-rare sarcoma subtypes. Expert radiological and surgeon reviews are required to differentiate similarly presenting tumours where surgery can be avoided (e.g., angiomyolipoma), or where upfront systemic therapy is indicated (e.g., extraosseous Ewing's sarcoma). Thus, the management of all retroperitoneal sarcomas should occur at a sarcoma referral centre, with a multidisciplinary team of experts dedicated to the surgical and medical management of these rare tumours. In this focused review, we highlight how diagnosis and management of the ultra-rare primary RPS histologies of malignant perivascular epithelioid cell tumour (PEComa), extraosseous Ewing sarcoma (EES), extraosseous osteosarcoma (EOS), and rhabdomyosarcoma (RMS) critically diverge from the management of more common RPS subtypes.

Prospective, Long-Term Functional Outcomes of Extra-Osseous Talotarsal Stabilization (EOTTS) Using HyProCure in Adult Patients with Talotarsal Joint Instability: Assessment of Physical Activity and Patient Satisfaction.

BACKGROUND: The partial dislocation of the talus from the calcaneus and navicular bones is a primary factor leading to a prolonged overpronation during weightbearing. This study aimed to assess the possibility of returning to physical activity and long-term patient satisfaction after an extra-osseous talotarsal stabilization (EOTTS) procedure with a HyProCure sinus tarsi implant for partial talotarsal joint dislocation (TTJ). METHODS: A total of 41 adult patients (61 feet), with an average age of 46.41, were included and treated surgically with EOTTS as a stand-alone surgery. Physical activity and functional scores were assessed pre- and post-operatively using questionnaires-the UCLA Activity Score, Symptom-Related Ankle Activity Scale (SAAS), Sports Frequency Score (SFS), Lower Extremity Functional Scale (LEFS), and VAS scale. Satisfaction was assessed on a ten-point scale. The follow-up period was on average 8.61 years (from 7.33 to 10.31). RESULTS: EOTTS had a positive impact on physical activity, and a high rate of patient satisfaction (8.95 ± 1.9) was noted. The treatment led to a reduction in foot pain, as well as an increase in SAAS and LEFS scores (15,6% and 19,3%, respectively, p <0.01). The VAS pain score decreased by 18,6% (p <0.001). SFS and UCLA scores showed a small increase, but it was not statistically significant. A positive correlation was noted between patient satisfaction and time of physical activity per week (R = 0.33, p =0.04), and also between patient satisfaction and SAAS scores (R = 0.43, p =0.005). Pain from other joints (knee, hip) was eliminated or reduced in 40% of patients after surgery. CONCLUSIONS: EOTTS with a HyProCure implant is an effective long-term treatment option for partial talotarsal joint dislocation, leading to a reduction in foot pain and increased patient satisfaction, and allowing for a return to physical activity.