Unusual Soft Tissue Uptake of Tc-99m MDP in Radiation-induced Sarcoma: Diagnostic Conundrum.

Tc-99m methylene diphosphonate (MDP) is a bone imaging agent used for skeletal staging, but it can also be localized in extraosseous calcifying lesions. We report a case of an 84-year-old woman with breast carcinoma who underwent surgery followed by radiotherapy 10 years ago and now presented with a right axillary mass referred for Tc-99m MDP to exclude bone metastasis. Tc-99m MDP shows intense tracer uptake in the right thoracic region corresponding to the site of calcified soft tissue mass in the right lateral chest wall. Subsequent ultrasonography revealed an ill-defined lesion containing coarse calcifications. Biopsy showed radiation-induced sarcoma. Extra osseous Tc-99m MDP uptake may provide important diagnostic information that may alter patient management.

An unusual presentation of peripheral ameloblastoma in the maxilla.

Peripheral ameloblastoma (PA) is believed to be the rarest variant of ameloblastoma and only has been described in isolated case reports. PA is usually confined to the soft tissues surrounding the supporting tissues of the teeth. Although it manifests nonaggressive behavior and can be treated with complete removal by local surgical excision, long term follow up is mandatory to prevent future recurrence and possible malignant transformation.

Extraskeletal ewing's sarcoma on hard palate biltaerally: A rare case report.

Ewing Sarcoma belongs to the category of undifferentiated blue small round cell tumour and its origin has been traced to be that from inside of the bone, but can also arise in soft tissues (extraosseous form). These lesions belong to the category of round cell tumours, which includes a varied range of tumours. This category, although found in other extremities and thoracic regions, head and neck region have been reported to have less number of tumours, in addition to that the soft tissue counterparts are even scarcely reported. Thereby, this case reports represents a soft tissue counterpart of Ewings Sarcoma on the hard palate, which not only extends unilaterally but extends bilaterally.

Peripheral Dentinogenic Ghost Cell Tumor- Diagnostic Challenge in a Gingival Epulis: Report of a Case with Update of all Dentinogenic Ghost Cell Cases Reported in English Literature.

Dentinogenic ghost cell tumor (DGCT), a variant of the calcifying odontogenic cyst, is considered to be a benign epithelial and mesenchymal neoplasm containing aberrant epithelial keratinization, ghost cells, and spherical calcifications. While there can be peripheral and central variants of this entity, the extraosseous type is rarer and usually innocuous in its clinical presentation. The aim of this article is to report a case of peripheral DGCT on the maxillary anterior region in a 14-year-old female evolving for 5 years and to emphasize the importance of histopathologic examination of gingival growths to avoid diagnostic pitfalls. A compilation of all reported cases in the English literature till date with details on the site, size, age sex, symptoms radiographic features, treatment follow-up, and recurrences has been attempted for better understanding of the biologic nature of this rare neoplasm.

Rare Presentation of Primary Pleural Ewing Sarcoma With a Mass Extending Into the Right Ventricle: A Case Report.

Primary pleural Ewing sarcoma is a rare type of Ewing sarcoma with only a few case reports identified in the literature. The condition is challenging to diagnose with deceiving symptoms and wide differential diagnosis. Diagnosis is confirmed with a combination of radiological and pathological assessment. Treatment is similar to other types of Ewing sarcoma with chemotherapy and surgery being the mainstay of treatment. We identify an unusual presentation of pleural Ewing sarcoma in a 31-year-old male with a mass extending into the right ventricular outlet causing rapid deterioration of the patient.

Primary Ewing's sarcoma of the uterine cervix: a case report and review of the literature.

BACKGROUND: Ewing's sarcoma (ES) is an aggressive cancer of bone and soft tissue, most of which tend to occur in the bone. Extraosseous Ewing's sarcoma (EES) of the cervix is extremely rare. CASE PRESENTATION: In the present work, we reported a 39-year-old cervical EES patient with a 2.5*2.1*1.8 cm tumor mass. According to previous literatures, our case is the smallest tumor found in primary cervical ES ever. The patient initially came to our hospital due to vaginal bleeding, and then the gynecological examination found a neoplasm between the cervical canal and partially in the external cervical orifice. The diagnosis of EES was confirmed below: Hematoxylin & Eosin staining (H&E) revealed small round blue malignant cells in biopsy specimens. Immunohistochemistry (IHC) showed the positive staining for CD99, NKX2.2, and FLI1. Disruption of EWSR1 gene was found by fluorescence in situ hybridization (FISH), and the EWSR1-FLI1 gene fusion was determined by next-generation sequencing (NGS). The patient received laparoscopic wide hysterectomy, bilateral adnexectomy, pelvic lymphadenectomy, and postoperative adjuvant chemotherapy and remained disease free with regular follow-up for 1 year. CONCLUSIONS: Through a systematic review of previously reported cervical ES and this case, we highlighted the importance of FISH and NGS for the accuracy of ESS diagnosis, which could assist on the optimal treatment strategy. However, due to the rarity of the disease, there is no standard treatment schemes. Investigation on molecular pathological diagnosis and standardization of treatment regimens for cervical ES are critical to patients' prognosis.

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.

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.

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.

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.

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.

Emerging Role of Non-collagenous Bone Proteins as Osteokines in Extraosseous Tissues.

Bone is a unique tissue, composed of various types of cells embedded in a calcified extracellular matrix (ECM), whose dynamic structure consists of organic and inorganic compounds produced by bone cells. The main inorganic component is represented by hydroxyapatite, whilst the organic ECM is primarily made up of type I collagen and non-collagenous proteins. These proteins play an important role in bone homeostasis, calcium regulation, and maintenance of the hematopoietic niche. Recent advances in bone biology have highlighted the importance of specific bone proteins, named "osteokines", possessing endocrine functions and exerting effects on nonosseous tissues. Accordingly, osteokines have been found to act as growth factors, cell receptors, and adhesion molecules, thus modifying the view of bone from a static tissue fulfilling mobility to an endocrine organ itself. Since bone is involved in a paracrine and endocrine cross-talk with other tissues, a better understanding of bone secretome and the systemic roles of osteokines is expected to provide benefits in multiple topics: such as identification of novel biomarkers and the development of new therapeutic strategies. The present review discusses in detail the known osseous and extraosseous effects of these proteins and the possible respective clinical and therapeutic significance.

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.

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.

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.

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.