Clinicopathologic correlates of solitary fibrous tumors.

Solitary fibrous tumor (SFT) is a rare tumor of mesenchymal origin that account for less than 2% of all soft tissue masses. Initially identified in the pleura, SFT has been identified in multiple anatomic locations and can arise anywhere in the body. The varying histologic features along with non-specific means of identification have led SFT to be associated with several different names. Over the last several decades, sustained advances through research and technology have led to more reliable methods for differentiating this distinct soft tissue tumor. Advances specifically in immunohistochemistry and molecular diagnostics have identified CD34 as the most consistent marker in SFT, however even this lacks specificity to conclusively narrow down the broad differential for exact identification. More recently the discovery of the NAB2-STAT6 fusion gene has led to more precise diagnosis of SFT. Like many other soft tissue tumors, surgical management is the mainstay of treatment for SFT with emphasis on obtaining tumor-negative margins. Radiation therapy and chemotherapy regimens have not demonstrated global effectiveness, and thus no standardized treatments have been identified. Given the rarity of SFT and current supportive evidence for therapies, management should be focused on tumor extirpation. Nonetheless, individualized therapy, determined within a multidisciplinary setting should be considered.

Solitary fibrous tumor

The immune cytokine tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) has attracted rapidly evolving attention as a cancer treatment modality because of its competence to selectively eliminate tumor cells without instigating toxicity in vivo. TRAIL has revealed encouraging promise in preclinical reports in animal models as a cancer treatment option; however, the foremost constraint of the TRAIL therapy is the advancement of TRAIL resistance through a myriad of mechanisms in tumor cells. Investigations have documented that improvement of the expression of anti-apoptotic proteins and survival or proliferation involved signaling pathways concurrently suppressing the expression of pro-apoptotic proteins along with down-regulation of expression of TRAILR1 and TRAILR2, also known as death receptor 4 and 5 (DR4/5) are reliable for tumor cells resistance to TRAIL. Therefore, it seems that the development of a therapeutic approach for overcoming TRAIL resistance is of paramount importance. Studies currently have shown that combined treatment with anti-tumor agents, ranging from synthetic agents to natural products, and TRAIL could result in induction of apoptosis in TRAIL-resistant cells. Also, human mesenchymal stem/stromal cells (MSCs) engineered to generate and deliver TRAIL can provide both targeted and continued delivery of this apoptosis-inducing cytokine. Similarly, nanoparticle (NPs)-based TRAIL delivery offers novel platforms to defeat barricades to TRAIL therapeutic delivery. In the current review, we will focus on underlying mechanisms contributed to inducing resistance to TRAIL in tumor cells, and also discuss recent findings concerning the therapeutic efficacy of combined treatment of TRAIL with other antitumor compounds, and also TRAIL-delivery using human MSCs and NPs to overcome tumor cells resistance to TRAIL.

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Harnessing TRAIL-Induced Apoptosis Pathway for Cancer Immunotherapy and Associated Challenges.

Benefits and challenges of antibody drug conjugates as novel form of chemotherapy

Antibody-based therapeutics, which induce apoptosis of malignant cells by selectively binding to their receptors, hold tremendous promise for clinical cancer therapy. Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) has received considerable interest due to its favorable capability of activating apoptosis in cancer cells by interacting with death receptors (DRs). However, cancer stem-like cells (CSCs) show deficient or lower DR and are highly resistant to TRAIL-mediated apoptosis limiting the therapeutic efficacy. Here, we report a liposome-mediated acclimatization strategy to overcome the CSC-emanated TRAIL resistance. The liposomal assemblies coencapsulating plasmid DNA encoding TRAIL and salinomycin enable cancer cells as protein generators to express TRAIL, and more importantly, can acclimatize resistant CSCs to be sensitized to the TRAIL-triggered apoptosis by salinomycin-induced upregulation of DR expression on CSCs. This programmable liposome-based drug codelivery system shows the potential to efficiently eliminate CSCs and inhibit CSC-enriched tumor growth in the orthotopic colon tumor mouse model.

Combating Cancer Stem-Like Cell-Derived Resistance to Anticancer Protein by Liposome-Mediated Acclimatization Strategy.

Proliferative fasciitis is an extremely rare benign myofibroblastic proliferation that typically presents as a rapidly growing subcutaneous mass. Precise histopathological interpretation is required to obtain a proper diagnosis. Due to a symptomatology overlap, discrimination from soft tissue sarcomas is crucial in the prevention of unnecessary excessive treatment that could be potentially harmful to the patients. Here, we present a rare case of atypical localization of proliferative fasciitis. The lesion was predominantly localized in the groin with the invasion of the scrotum and clinically mimicked soft tissue sarcoma. However, according to a proper histopathologic analysis, the diagnosis of proliferative fasciitis was concluded. With a large number of pseudosarcomatous lesions, there is a rising urge to introduce these rare but benign processes to physicians in order to prevent misdiagnosing patients.

Rare Pseudosarcomatous Lesions Posing Diagnostic Challenges: Histopathologic Examination as a Dominant Tool Preventing Misdiagnosis of Proliferative Fasciitis

Summary: The latissimus dorsi (LD) myocutaneous flap is heavily used in reconstructive plastic surgery as either a local or distant flap, and mostly for coverage of large defects. To date, only a limited number of studies have described the use of an LD flap for functional reconstruction. Restoration of the extensor mechanism remains an unexplored area, and several issues remain to be addressed. First, generally accepted recommendations for the surgical technique do not include specific steps to achieve functional qualities of the upper extremity after complete removal of the triceps brachii muscle. Second, to date, it has not been clarified whether elbow extension requires correction because the movement can be naturally compensated for by gravity. To contribute to the current knowledge base in this field, the authors describe a technique for the reconstruction of an extensor mechanism of the elbow by transferring a pedicled functional LD flap while maintaining an intact insertion. Reconstruction was performed in a patient who experienced a second recurrence of a malignant peripheral nerve sheath tumor and underwent complete excision of the triceps brachii muscle. After excision, a meshed epidermal graft was used for wound closure. The patient’s postoperative course was uneventful. His elbow was immobilized for 3 weeks using an elbow splint, followed by intensive rehabilitation. The functional result was excellent, with an Enneking limb function score of 26. During the 16-month follow-up, no signs of local recurrence or systemic spread were observed.

Successful Restoration of Elbow Extension Using the Latissimus Dorsi Flap: Case Report

Introduction Solitary fibrous tumor (SFT) is an extremely rare disease with a high misdiagnosis rate and a potentially malignant biologic nature. We have collected and analyzed data from 18 SFT patients to provide a deeper insight into this uncommon disease entity. Methods In our study, 18 patients who had undergone surgery between April 2014 and December 2021 for the diagnosis of SFT were evaluated. The collected data for each patient included the location of the SFT, the preoperative diagnosis, the definitive histological diagnosis, the presence of postoperative complications, the time of recurrence, the time of systemic progression, the type of treatment, and the survival rate. The median follow-up was 36 months. Results In three patients, the preoperative diagnosis did not correlate with the definitive histology of SFT. In patients with the limb location of SFT, no signs of recurrence nor distant metastases were seen within the study period. In total, 50% of the postsurgical complications were associated with the abdominal location of the SFT. In newly diagnosed SFT patients, two patients (20%) developed local recurrence, and the median time until recurrence was 22.5 months. Out of patients that were admitted and operated on for recurrent SFT, 67% relapsed, and the median time to relapse was 9.5 months. The systemic progression of the disease was observed in 33% of patients treated for recurrent SFT. Conclusion In our study, the misdiagnosis rate was high and correlated with previously published studies. Postsurgical complications were associated with the extrathoracic location of SFT. The mainstay of SFT treatment remains radical surgery, although radiotherapy alone can significantly improve overall survival. Clinical trials are urgently needed to evaluate the potential effect of other treatment modalities, such as immunotherapy and targeted therapy, in SFT patients.

Diagnostic challenges and treatment options in patients with solitary fibrous tumor: A single-center observational study

Composite haemangioendothelioma is a rare vascular neoplasm with indolent to intermediate malignant potential. Diagnosis of this disease relays on histopathological identification of at least two different morphologically distinctive vascular components in proper clinical settings. Exceedingly rare cases of this neoplasm can exhibit areas resembling high-grade angiosarcoma, which does not change the biological behaviour. Such lesions tend to occur in the setting of chronic lymphoedema and thus, can mimic Stewart-Treves syndrome, which has a much worse clinical outcome and prognosis.We present a case of 49 years old male suffering from chronic lymphoedema of the left lower extremity who had developed a composite haemangioendothelioma with high grade angiosarcoma-like areas mimicking the Stewart-Treves syndrome. Given the multifocality of the disease, the only potentially curable surgical treatment would be hemipelvectomy, which was refused by the patient. The patient has been followed-up, with no signs of local progression of the remaining disease, nor a distant spread outside the involved extremity for two years.Composite haemangioendothelioma represents a rare malignant vascular tumour, with significantly more favourable biological behaviour than angiosarcoma, even in cases where angiosarcoma-like areas are present. For that reason, composite haemangioendothelioma can be easily misdiagnosed as true angiosarcoma. The rarity of this disease unfortunately hampers the development of clinical practice guidelines and the implementation of treatment recommendations. Most of the patients with localized tumour are treated by wide surgical resection, without neo- or adjuvant radiotherapy or chemotherapy. However, in the case of this diagnosis, the watch-and-wait approach is better than mutilating procedure, highlighting the necessity of establishing of the correct diagnosis. 

https://diagnosticpathology.biomedcentral.com/counter/pdf/10.1186/s13000-023-01365-1

Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report

Andrej Ozaniak

Papers

Rare Pseudosarcomatous Lesions Posing Diagnostic Challenges: Histopathologic Examination as a Dominant Tool Preventing Misdiagnosis of Proliferative Fasciitis

Successful Restoration of Elbow Extension Using the Latissimus Dorsi Flap: Case Report

Diagnostic challenges and treatment options in patients with solitary fibrous tumor: A single-center observational study

Patient with composite haemangioendothelioma containing angiosarcoma-like areas in the setting of congenital lymphoedema mimicking Stewart-Treves syndrome: a case report

100P Combinatorial treatment strategies for overcoming the immunotherapy resistance in soft tissue sarcomas