A pictographic guide for decision making in surgery for pelvic bone sarcoma.

Pelvic bone sarcoma surgery is challenging due to complex anatomy, proximity to major neurovascular structures, and, more importantly, the potential for complications. Decision-making is vital in offering patients the best oncological and functional outcomes after surgery. Multidisciplinary teams involved from the stage of diagnosis and treatment planning, followed by surgery by experienced teams have proven to be beneficial. Tumour-free margin clearance is essential, and surgical planning must be tailored to achieve the same. The choice of reconstruction needs to be decided based on the amount of bone resected and the available expertise and resources. Lesions isolated only to PI or PIII region may not need reconstruction. Though pedestal cups and Custom-made prosthesis are useful in reconstruction after periacetabular tumour resections, hip transposition surgery is also widely practiced by surgeons with favourable outcomes particularly after neo-adjuvant radiotherapy/proton beam therapy. Navigation has shown promise in achieving tumour-negative margins and disease-free progression particularly in chondrosarcoma. A flap-based approach can be considered for hindquarter amputations; however, patients need to be counseled regarding the complications following this surgery. This article, with proposed flowcharts, is aimed at providing practicing surgeons with a guide toward decision-making while planning pelvic bone sarcoma surgery.

Improved production of class I phosphatidylinositol 4,5-bisphosphate 3-kinase.

Phosphatidylinositol 4,5-bisphosphate 3-kinases (PI3K) are a family of kinases whose activity affects pathways needed for basic cell functions. As a result, PI3K is one of the most mutated genes in all human cancers and serves as an ideal therapeutic target for cancer treatment. Expanding on work done by other groups we improved protein yield to produce stable and pure protein using a variety of modifications including improved solubility tag, novel expression modalities, and optimized purification protocol and buffer. By these means, we achieved a 40-fold increase in yield for p110α/p85α and a 3-fold increase in p110α. We also used these protocols to produce comparable constructs of the ß and δ isoforms of PI3K. Increased yield enhanced the efficiency of our downstream high throughput drug discovery efforts on the PIK3 family of kinases.

Targeting hypoxia-inducible factors in malignancies caused by Kaposis sarcoma associated herpesvirus.

In this editorial, we highlight the potential use of inhibitors of hypoxia-inducible factors (HIFs) for the use in Kaposi's sarcoma associated herpesvirus (KSHV) (also known as human herpesvirus-8) related malignancies. The past 20 years has accumulated detailed knowledge of the role of these factors in ensuring the maintenance of the KSHV in infected cells, in aiding the growth of the virus infected cells and aiding in the spread of virus from infected cells by inducing lytic reactivation. Today, a wide range of inhibitors for HIFs are currently being clinically evaluated for use in treating a variety of cancers. We discuss the current state of this research area as it relates to KSHV malignancies and describe pre-clinical and clinical evidence of drugs that target HIF to back up the idea that these inhibitors could be a novel way to treat KSHV related diseases.

A preliminary study of primary retroperitoneal sarcoma at a tertiary University Hospital in Bangkok, Thailand: a retrospective observational study.

Background: Retroperitoneal sarcoma (RPS) is rare and difficult to treat with a high recurrent rate. Very little data regarding primary RPS exists in Thailand. Objectives: To study the outcome of treatment of primary RPS at a tertiary University Hospital in Bangkok, Thailand. Methods: All patients who had RPS undergoing the first surgical resection at King Chulalongkorn Memorial Hospital from June 2003 to December 2019 were retrospectively enrolled in the study. Perioperative management, results of treatment, postoperative complications, and outcome were analyzed. Results: Thirty-eight patients entered the study. Large abdominal mass was the most common presentation (90%). Liposarcoma was the most common histology (58%). Twenty patients (53%) had preoperative core needle biopsy and 21 (55%) underwent preoperative radiotherapy (RT). The tumor size ranged from 3 cm to 48 cm (median 22 cm). Five patients (13%) had total mass removal only while 33 (87%) had complete gross resection with ≥1 visceral organ resection. Surgical margins classified as R0, R1, and R2 were 61%, 34%, and 5%, respectively. Five patients (16%) had postoperative complications. There was no 30-day postoperative mortality. The local recurrence rate was 34%. Survival analysis revealed a 5-year overall survival rate of 37% and 5-year disease-free survival rate of 29%. The 5-year and 10-year recurrent rates were 71% and 95%, respectively. Multivariate analysis showed that preoperative radiation was the only factor reducing recurrence (19% vs. 53%, OR: 0.21, P = 0.011). Conclusion: The preliminary study of outcome of the treatment of primary RPS at our institution showed a fair prognosis of this rare malignancy despite our aggressive surgical approaches. Preoperative radiation may help reduce recurrence in selected primary RPS patients.

Shared decision making in primary malignant bone tumour surgery around the knee in children and young adults: protocol for a prospective study.

BACKGROUND: Children and young adults needing surgery for a primary malignant bone tumour around the knee face a difficult, life-changing decision. A previous study showed that this population wants to be involved more in the decision-making process and that more involvement leads to less decisional stress and regret. Therefore, a well-designed and standardized decision-making process based on the principles of shared decision-making needs to be designed, implemented, and evaluated. METHODS: We developed a shared decision-making (SDM) model for this patient population, including an online decision aid (DA). This model has been implemented in the standard care of patients with a primary malignant bone tumour around the knee. Following implementation, we will analyse its effect on the decision-making process and the impact on patient experiences using questionnaires and interviews. Moreover, potential areas for improvement will be identified. DISCUSSION: Given the importance of involving patients and parents in surgical decision-making, particularly in life-changing surgery such as malignant bone tumour surgery, and given the lack of SDM models applicable for this purpose, we want to share our model with the international community, including our study protocol for evaluating and optimising the model. This study will generate valuable knowledge to facilitate the optimisation of current patient care for local treatment. The sharing of our implementation and study protocol can serve as an example for other centres interested in implementing SDM methods in an era characterized by more empowered patients and parents who desire autonomy and reliable and realistic information.

Undifferentiated embryonal sarcoma of the liver masquerading as a cystadenoma in a young adult: a case report.

BACKGROUND: Undifferentiated embryonal sarcoma of the liver is an infrequent hepatic malignancy, primarily observed in the pediatric population. This neoplasm is exceedingly rare among adults. Despite its scarcity in adult cases, it remains imperative to accurately discern undifferentiated embryonal sarcoma of the liver utilizing diverse imaging modalities to prevent misdiagnosis with more prevalent benign and malignant hepatic masses. In this case, a comprehensive imaging examination was conducted, encompassing ultrasound, computed tomography, magnetic resonance imaging, and positron emission tomography-computed tomography scans. Notably, the positron emission tomography-computed tomography scan revealed 18F-fluorodeoxyglucose uptake characteristics indicative of malignancy, providing a pivotal clue for prompt diagnosis of undifferentiated embryonal sarcoma of the liver. CASE PRESENTATION: This report presents the diagnostic procedure employed for a 37-year-old female patient of Han ethnicity in China diagnosed with undifferentiated embryonal sarcoma of the liver. The patient was admitted with a 2-day history of mid-upper quadrant abdominal pain. The patient's temperature and inflammatory markers, such as white blood cell count and hypersensitive reactive protein, were slightly elevated. Ultrasound showed a cystic-solid nodule in the liver. The computed tomography revealed a cystic mass in the right lobe of the liver, characterized by a low-density shadow and a lack of significant enhancement during contrast-enhanced scanning, initially suggesting cystadenoma. Enhanced magnetic resonance imaging revealed a block-like abnormal signal shadow in the right hepatic lobe, suggestive of various etiologies, including benign lesions, cystadenoma with hemorrhage, or hemangioma with hemorrhage. However, the positron emission tomography-computed tomography showed increased 18F-fluorodeoxyglucose consumption within both the cystic wall and lesion, raising suspicion of malignancy. Surgical resection of the posterior hepatic lobe was performed under general anesthesia after comprehensive preoperative preparations. During the procedure, a space-occupying lesion was identified in the right posterior hepatic lobe, adhering to the diaphragm. Surface liver parenchyma overlying the tumor exhibited rupture, with several blood clots visible. The tumor was successfully and completely excised. Pathological examination revealed a fusiform cell tumor, necrosis, hemorrhage, cystic changes, cellular atypia, mitotic images, and eosinophilic globules suggestive of undifferentiated embryonal sarcoma of the liver. Immunohistochemical staining indicated CK (+), vimentin (Vim; +), Desmin (+), actin (-), α1-AT (+), GPC-3 (+), PDGFRa (+), MDM2 (+), P16 (+), Ki-67 (+) 30-35%, and PAS (+). The patient underwent five cycles of combination chemotherapy with ifosfamide and epirubicin, administered at 21-day intervals at our hospital. Following 3 years of postoperative surveillance, the patient remained stable, with no evidence of hepatic tumor recurrence. CONCLUSION: On the basis of this case and a comprehensive literature review, we recommend that clinicians consider the possibility of undifferentiated embryonal sarcoma of the liver in patients presenting with non-specific clinical and serological markers, particularly when there is inconsistency between ultrasound and computed tomography imaging findings, along with elevated 18F-fluorodeoxyglucose uptake observed in both the cystic wall and lesion on positron emission tomography-computed tomography examination. Given the rarity and high-grade malignancy of undifferentiated embryonal sarcoma of the liver, heightened clinical awareness and recognition are crucial for early diagnosis and successful therapy.

Successful treatment of GEMOX regimen combined with nimotuzumab in the pancreatic cancer with wild KRAS and mutant BRCA: a report of two cases.

Background: Pancreatic cancer is characterized by chemoresistance. In recent years, more potential therapeutic molecular targets for pancreatic cancer have been developed, and thus increasing attention has been paid to precise chemotherapy to improve the prognosis of patients with advanced pancreatic cancer. Case Description: In this study, we reported two rare cases of advanced pancreatic cancer. One patient was diagnosed with retroperitoneal lymph node metastasis after radical resection of pancreatic ductal adenocarcinoma. The diagnosis of another patient was pancreatic ductal adenocarcinoma with liver metastasis. The whole genome sequencing of their tumor tissues detected both wild-type Kirsten rat sarcoma viral oncogene homolog (KRAS) and mutant breast cancer susceptibility gene (BRCA). And immunohistochemistry showed their tumor tissue was negative for epidermal growth factor receptor. We used the combined chemotherapy of gemcitabine (1,000 mg/m2) + oxaliplatin (135 mg/m2) + nimotuzumab (400 mg). After nine times of chemotherapy, the imaging examinations including positron emission tomography-computed tomography showed that both cases achieved complete remission. And there were no serious side effects during chemotherapy. Then, the patients were treated with oral olaparide (600 mg/day) for one year, and survived without tumor progress for more than 1.5 years. Conclusions: These two cases achieved excellent effects of precise chemotherapy, which provided an important reference for the treatment of pancreatic cancer patients with wild KRAS and mutant BRCA.

HyperArc radiotherapy for recurrent synovial sarcoma of infratemporal fossa: a rare case report and review of the literature.

Background: Synovial sarcoma (SS), a malignant and uncommon soft tissue sarcoma, typically manifests in the extremities and trunk. However, its occurrence in the infratemporal fossa (ITF) of the head and neck is exceedingly rare. Patients afflicted with SS in this anatomical region pose considerable challenges, as radical surgery is often difficult to undertake, leading to a high rate of postoperative recurrence. Moreover, it is often difficult to effectively control the tumor when the cancer relapses. Much of our understanding regarding SS of ITF stems from limited case reports, with a lack of established clinical guidelines for its management. There exists a significant clinical need for effective therapeutic approaches. Case Description: This case report documents a patient with SS of ITF, experiencing repeated recurrences despite undergoing multiple surgeries and chemotherapy treatments. The patient underwent HyperArc (HA) radiotherapy (RT) in conjunction with concurrent chemotherapy utilizing cisplatin, resulting in a remarkable 3-year follow-up period devoid of recurrence. Conclusions: The primary objective of this case report is to disseminate knowledge regarding this rare manifestation of SS of ITF, detailing the successful treatment strategy employed. In this case, we employed a comprehensive treatment strategy involving concurrent chemoradiotherapy based on HA. Our findings demonstrate that this approach was effective in achieving disease control and improving patient outcomes.

The efficacy and applicability of chimeric antigen receptor (CAR) T cell-based regimens for primary bone tumors: A comprehensive review of current evidence.

Primary bone tumors (PBT), although rare, could pose significant mortality and morbidity risks due to their high incidence of lung metastasis. Survival rates of patients with PBTs may vary based on the tumor type, therapeutic interventions, and the time of diagnosis. Despite advances in the management of patients with these tumors over the past four decades, the survival rates seem not to have improved significantly, implicating the need for novel therapeutic interventions. Surgical resection with wide margins, radiotherapy, and systemic chemotherapy are the main lines of treatment for PBTs. Neoadjuvant and adjuvant chemotherapy, along with emerging immunotherapeutic approaches such as chimeric antigen receptor (CAR)-T cell therapy, have the potential to improve the treatment outcomes for patients with PBTs. CAR-T cell therapy has been introduced as an option in hematologic malignancies, with FDA approval for several CD19-targeting CAR-T cell products. This review aims to highlight the potential of immunotherapeutic strategies, specifically CAR T cell therapy, in managing PBTs.

Theranostic strategies in sarcoma: preliminary clinical evidence.

INTRODUCTION: Sarcomas encompass a highly diverse range of malignancies, characterized by varied morphological and molecular profiles. Treatment options in case of therapy-refractory or advanced disease are limited. In this context, theranostics emerges as an innovative platform seamlessly integrating diagnosis and therapy, offering promising prospects. AREAS COVERED: This special report delves into the initial clinical applications of theranostic-based approaches in sarcomas. Specifically, it examines various strategies targeting biomarkers associated with sarcomas, including fibroblast activation protein (FAP), prostate-specific membrane antigen (PSMA), C-X-C chemokine receptor type 4 (CXCR4) and somatostatin receptor 2 (SSTR2). EXPERT OPINION: The heterogeneous uptake of the CXCR4-targeted radioligand in lesions, along with its poor correlation with immunohistochemistry data, diminishes the attractiveness of this theranostic approach in the sarcoma oncological setting. SSTR2-targeted approaches in sarcoma, although potentially effective, are limited to a single case. Early experiences with FAP inhibitors in sarcoma patients have shown particularly promising outcomes, indicating effective disease control with minimal toxicity. While PSMA presents an enticing target for theranostic approaches in sarcomas, its utilization remains anecdotal and requires further investigation. Prospective and well-designed clinical trials are imperative to delineate the potential impact of FAPI- and PSMA-based approaches on sarcoma therapeutic landscapes, offering innovative and personalized treatment options.

Primary Ewing's Sarcoma of the Sternum in an Adult Male: A Rare Case Report.

Ewing's sarcoma, a rare primary bone malignancy primarily affecting adolescents and young adults, typically manifests in the pelvic bones and femur. Primary Ewing's sarcoma of the sternum is exceptionally rare, constituting less than 1% of cases. We present a case of a 34-year-old man with a 2-month history of anterior chest wall pain initially attributed to muscular spasm. Subsequently, the patient developed a palpable mass and imaging demonstrated a mid-lower sternal lesion with cortical destruction and soft tissue involvement, confirmed as Ewing's sarcoma on biopsy. In addition, a suspicious lesion was identified in the left distal tibia, which was histologically confirmed as a metastasis from the primary sternal sarcoma. Neoadjuvant chemotherapy preceded partial sternotomy with rib resection and reconstruction, achieving clear surgical margins. Postoperative evaluation showed shrinkage in the sternal lesion and near-resolution of the tibial metastasis. Subsequent chemotherapy cycles resulted in no evidence of the disease on the follow-up positron emission tomography scan. This case underscores the diagnostic challenges of primary sternal Ewing's sarcoma and emphasizes the importance of early recognition and comprehensive evaluation in managing such rare presentations.

Surgical Management of a Rare Case of Low-Grade Myofibroblastic Sarcoma of the Larynx.

Low-grade myofibroblastic sarcoma (LGMS) of the larynx is an extremely rare entity. We report a case of LGMS in a 59-year-old man presenting with progressive hoarseness and a right laryngeal mass and there was no recurrence during the 6-month follow-up after the total laryngectomy.

Biphenotypic Sinonasal Sarcoma- A case Series with a Review of Literature.

Biphenotypic sinonasal sarcoma (BSNS) is a rare malignant tumor that affects the upper nasal cavity and ethmoid sinuses. It is more commonly found in middle-aged women and is characterized by the infiltration and hypercellular proliferation of spindle cells. These cells exhibit specific immunoreactivity. We add seven cases diagnosed as BSNS, to the handful of cases already available in the literature.BSNS is a malignant disease of the sinonasal tract that requires prompt and accurate diagnosis, followed by surgical resection and consideration of radiotherapy. Our analysis of seven cases supports previous research that confirms the aggressive nature of the disease but also shows that it is treatable with the right approach.

Myofibroma with Atypical Features can Mimic Low-Grade Myofibroblastic Sarcoma: Two Paediatric Cases.

Myofibroma is a rare benign mesenchymal tumor that frequently affects the pediatric population with a predilection for the head and neck region. About 10% of myofibroma cases, presenting atypical features, can be misinterpreted as low-grade myofibroblastic sarcoma (LGMS), with therapeutic and prognostic impact. Here, we report two pediatric cases of benign myofibroblastic tumors, one of them showing typical characteristics of myofibroma, the other was an atypical myofibroma, which initially mimicked low-grade myofibroblastic sarcoma. Atypical myofibromas, despite its distinctive characteristics, follow a benign course, similar with typical myofibroma. It is necessary to distinguish atypical myofibroma from low-grade myofibroblastic sarcoma and avoid unnecessary invasive therapy.

Ewing's Sarcoma of Maxillary Sinus.

Ewing's sarcoma is a small round cell tumor commonly involving long bones. Head and neck involvement is seen less frequently with maxillary sinus being the rarest site. Here we report a case of a12-year-old female patient presented with left side facial swelling for 3 weeks duration which was diffuse and hard on palpation. CT and MRI of Nose & para nasal sinus revealed a mass lesion in the left maxillary sinus infiltrating and eroding the orbital wall and extending intracranially. Biopsy from the lesion revealed Ewing's sarcoma with strong positivity for CD-99 marker. Patient was referred to radiotherapy due to extensive disease at younger age. The CT and MRI along with HPE and IHC markers enables early diagnosis and prompt treatment that leads to better survival and prognosis, as unlike Ewing's sarcoma of long bones, that metastasize early. The combined chemoradiotherapy is now the standard line of treatment along with close follow up for at least 2 years.

OSteosarcoma of Ethmoid Sinus-A Rare Entity.

Osteogenic sarcoma of the Craniofacial region are uncommon.They have been mainly reported in the mandible and the maxilla, Primary osteosarcoma arising from ethmoid sinus is an extremely rare condition. We report a case of 34 year old male, presented with gradually increasing swelling over the right medial canthus with history of double vision for one year. Clinically we thought it was a mucous retention cyst in the ethmoid area. After doing Contrast enhanced CT, we proceeded with transnasal endoscopic removal of the cyst. Histopathological examination of the specimen surprisingly turned out to be a low grade Osteogenic sarcoma. Patient was treated with adjuvant chemotherapy.

Chronic Invasive Fungal Sinusitis Mimicking Malignancy Post-Radiotherapy: A Case Report.

Invasive fungal sinusitis is a life-threatening form of fungal rhinosinusitis. Due to the aggressive clinical presentation and radiological appearance, there is diagnostic difficulty in differentiating invasive fungal sinusitis from a malignant process. This is even more challenging in oncological patients who have undergone previous head and neck radiotherapy, due to possibility of a recurrence of primary malignancy and radiation-induced neoplasms. We report a rare case of invasive fungal sinusitis mimicking a malignancy in a post-radiotherapy patient. Our patient was a 68-year-old male, 25-years post-radiotherapy for nasopharyngeal carcinoma. He presented with a 3-month history of purulent sputum and right facial paraesthesia. Magnetic resonance imaging showed an irregular destructive enhancing mass of the greater wing of right sphenoid and pterygoid bone with extensive extension into nearby structures. In view of extensive local and bony invasion, and a history of radiotherapy, initial suspicions were that of primary malignancy, specifically radiation-induced sarcoma, and recurrence of nasopharyngeal carcinoma. He underwent transpterygoid biopsy of the lesion, and histopathology demonstrated Aspergillus species, with no malignancy identified. Our report highlights the diagnostic difficulties in the post-radiotherapy cancer patient presenting with symptoms suggestive of aggressive sino-nasal disease. Invasive fungal sinusitis closely mimics the clinical and radiological findings of several neoplastic processes. We discuss the clinical and radiological characteristics of pathologies that may mimic invasive fungal sinusitis. Histological examination remains the gold standard for diagnosis, and early fungal staining is crucial. Furthermore, one should not presume the initial histopathological diagnosis to be confirmatory of isolated fungal disease. Repeat radiological investigations for disease resolution and histopathologic re-evaluation if required should be performed, keeping in mind possibility of coexisting malignancy.

Spindle Cell Sarcoma of the Maxilla: A Rare Entity, Case Report, and Review of the Literature.

Spindle cell sarcoma (SCS) is a malignancy, with the most recent Surveillance, Epidemiology, and End Results (SEER) data citing a total of 250 reported cases occurring in the head and neck. Of these cases, none originated in the maxillofacial hard tissue. The diagnosis of SCS requires its differentiation from other sarcomas and spindle cell neoplasms. Therefore, a comprehensive review to reinforce its inclusion in oral and maxillofacial surgeons' differential diagnosis for osseous neoplastic pathology is desired. In the present case report, we have described a recurrent case of Maxillary Undifferentiated Spindle cell sarcoma in a 11 year old male child post chemoradiotherapy which was excised intoto successfully .

Computed tomography radiomics models of tumor differentiation in canine small intestinal tumors.

Radiomics models have been widely exploited in oncology for the investigation of tumor classification, as well as for predicting tumor response to treatment and genomic sequence; however, their performance in veterinary gastrointestinal tumors remains unexplored. Here, we sought to investigate and compare the performance of radiomics models in various settings for differentiating among canine small intestinal adenocarcinoma, lymphoma, and spindle cell sarcoma. Forty-two small intestinal tumors were contoured using four different segmentation methods: pre- or post-contrast, each with or without the inclusion of intraluminal gas. The mesenteric lymph nodes of pre- and post-contrast images were also contoured. The bin settings included bin count and bin width of 16, 32, 64, 128, and 256. Multinomial logistic regression, random forest, and support vector machine models were used to construct radiomics models. Using features from both primary tumors and lymph nodes showed significantly better performance than modeling using only the radiomics features of primary tumors, which indicated that the inclusion of mesenteric lymph nodes aids model performance. The support vector machine model exhibited significantly superior performance compared with the multinomial logistic regression and random forest models. Combining radiologic findings with radiomics features improved performance compared to using only radiomics features, highlighting the importance of radiologic findings in model building. A support vector machine model consisting of radiologic findings, primary tumors, and lymph node radiomics features with bin count 16 in post-contrast images with the exclusion of intraluminal gas showed the best performance among the various models tested. In conclusion, this study suggests that mesenteric lymph node segmentation and radiological findings should be integrated to build a potent radiomics model capable of differentiating among small intestinal tumors.

Case report: A nodular lesion in the ventral region of the neck in the rat as a starting point for considerations on differential diagnosis.

The purpose of this case report is to present a poorly differentiated sarcoma in a pet rat. A veterinarian detected a small-sized nodular lesion in the ventral region of the neck during a follow-up visit related to another ailment. The anatomical structures found in the neck region in the rat and the differential diagnosis when deformities are palpated in this body part are discussed in detail. The patient underwent a total of four surgical procedures, as well as radiotherapy and chemotherapy. The rat survived in good condition for 144 days after finding the tumor.