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Introduction: Heterotopic ossification (HO), acquired or hereditary, is a diverse pathological condition defined by the production of extraskeletal bone in muscles, soft tissues, and connective tissues. Acquired HO is relatively prevalent and develops mostly in response to trauma, although its etiology is unknown. Genetic forms provide insight into the pathobiological mechanisms of this disorder. Fibrodysplasia ossificans progressiva (FOP) is a rare hereditary form of HO that can have a significant impact on affected individuals. FOP steadily weakens affected subjects and increases their risk of death. Areas covered: The U.S. Food and Drug Administration has recently approved the retinoid palovarotene as the first compound to treat heterotopic ossification in patients with FOP. This review provides a comprehensive overview of current and potential future pharmacotherapeutic options and their modes of action. The online databases PubMed, Cochrane Library, Web of Science, and ClinicalTrials.gov were searched using the terms 'heterotopic ossification' and 'fibrodysplasia ossificans progressiva' or synonyms, with a special focus over the last 5 years of publications. Expert opinion: Approval of palovarotene, as the first retinoid indicated for reduction in the volume of new HO, may revolutionize the therapeutic landscape. However, long-term safety and efficacy data for palovarotene are currently lacking. [ABSTRACT FROM AUTHOR]

Congenital fibrous soft tissue tumors in the eyelids are exceptionally rare, with only seven cases of neonatal myofibromas reported. Myopericytoma, a variant with pericytic differentiation, has not previously been described in neonatal eyelid tumors. This report details a case of a massive congenital myopericytoma in a newborn’s lower eyelid. The lesion had a broad area of adhesion to the anterior lamella of the lid. After resection, when the child was 11 days old, the resulting defect was successfully reconstructed with a V-Y type perforator-based flap. [ABSTRACT FROM AUTHOR]

Objectives: This systematic review and meta-analysis evaluates the combined accuracy of ultrasonic imaging in diagnosing soft tissue tumors and pseudotumors and identifies factors contributing to variability in its diagnostic accuracy. Methods: A literature search in significant databases used specific keywords and inclusion/exclusion criteria. Two authors independently performed cohort characterization and data analysis, with a third author resolving disagreements. A multivariate random effects model estimated pooled sensitivity, specificity, predictive values, and overall accuracy. Results: Ultrasound imaging demonstrated high pooled sensitivity and specificity, indicating effectiveness in identifying lesions. Variability in accuracy was attributed to factors such as tumor type and location, operator experience, and clinic location. Conclusions: Ultrasonic imaging is a reliable diagnostic tool for soft tissue tumors and pseudotumors, providing high sensitivity and specificity. However, variability in accuracy underscores the need to consider operator experience and tumor characteristics. Further research should explore the impact of technological advancements and include non-English studies to enhance understanding of ultrasound imaging's diagnostic validity. [ABSTRACT FROM AUTHOR]

A previously healthy adult male presented with a slowly enlarging orbital mass associated with 5 mm of non-pulsatile proptosis. On imaging, a soft tissue lesion with avid contrast enhancement and associated bony hyperostosis was noted. The lesion and hyperostotic bone were surgically debulked, and significant arterial bleeding was noted intraoperatively consistent with an arteriovenous malformation. Histopathologic analysis revealed a vascular malformation with enhanced microvasculature infiltrating the periosteum. While vascular lesions elsewhere in the body can be associated with skeletal changes, bony hyperostosis is a rare feature of orbital vascular malformations. [ABSTRACT FROM AUTHOR]

Background and importance: Chordomas are centrally located, expansile soft tissue neoplasms that arise from the remnants of the embryological notochord. Hemorrhagic presentation is exceedingly rare and can resemble pituitary apoplexy. Moreover, a purely intrasellar location of a chordoma is extremely uncommon. We report a case of a hemorrhagic intrasellar chordoma in an adult male, which presented similarly to pituitary apoplexy and was resolved with surgical resection. Clinical presentation: A 69-year-old male presented with a 4 week history of acute onset headache and concurrent diplopia, with significantly reduced testosterone and slightly reduced cortisol. His left eye demonstrated a sixth cranial nerve palsy. Magnetic resonance imaging of the brain showed a large hemorrhagic mass in the pituitary region with significant compression of the left cavernous sinus and superior displacement of the pituitary gland. The patient underwent an endoscopic endonasal transsphenoidal approach for the resection of the lesion. Near total resection was achieved. Final pathology revealed chordoma with evidence of intratumoral hemorrhage, further confirmed by immunopositive stain for bra-chyury. Post-operatively, the patient had improved diplopia and was discharged home on low dose hydrocortisone. At 3-month follow-up, his diplopia was resolved and new MRI showed stable small residual disease. Conclusions: Apoplectic chordomas are uncommon given chordoma's characteristic lack of intralesional vascularity and represent a diagnostic challenge in the sellar region. Our unique case demonstrates that despite our initial impression of pituitary apoplexy, this was ultimately a case of apoplectic chordoma that responded well to endoscopic endonasal surgery. [ABSTRACT FROM AUTHOR]

Our patient presented with an elastic soft mass of his left index finger. Hematoxylin and eosin staining showed a high cellular density with spindle-shaped cells in a storiform pattern. Immunohistochemical staining was positive for CD68, factor XIIIa and α-smooth muscle actin, and negative for CD34, STAT6, S100 protein, and desmin. [ABSTRACT FROM AUTHOR]

Soft tissue sarcomas are rare malignant tumors. Traditionally, treatment is guided by patient and tumor characteristics. Data on the influence of patient characteristics, particularly nutritional status, on clinical outcomes are scarce. Body composition and its changes during treatment play an essential role in predicting toxicity, clinical outcomes, and mortality. This analysis aimed to investigate the relationship between treatment toxicity and body composition. Patients diagnosed with sarcoma who underwent first-line palliative chemotherapy between October 2017 and January 2020 were included. Baseline and follow-up computed tomographic scans at the third lumbar vertebra, available from diagnostic purposes, were analyzed using SliceOmatic software. Treatment toxicity was defined as a composite score of the Common Terminology Criteria for Adverse Events. Nutritional Risk Screening (NRS) 2002 score, psoas muscle thickness to height ratio, and comorbidity showed a significant association with overall toxicity, while skeletal muscle index and age showed a strong trend. In summary, the NRS 2002 tool must be routinely implemented in inpatient and outpatient settings for cancer patients, and nutritional therapy needs to become a fixed component of multimodal cancer treatment. Furthermore, validated standardized procedures for the quantification of muscle mass are needed to individualize and optimize cancer treatment. [ABSTRACT FROM AUTHOR]

Acinetobacter baumannii–calcoaceticus complex is emerging as one of the most common causes of hospital-acquired infection globally. We present a case of orbital cellulitis caused by the A. baumannii–calcoaceticus complex. A 73-year-old Chinese woman with a history of rheumatoid arthritis presented with subacute onset of right upper eyelid swelling for 1 week. Computer tomography revealed a post-septal soft tissue lesion located at the right superior orbit that was enhanced with contrast, compressing on the superior aspect of the globe. Anterior orbitotomy with incisional biopsy of the right superior orbital lesion was performed, and histopathological examination was consistent with nonspecific inflammatory mass. The microbiological culture of the specimen yielded A. calcoaceticus and A. baumannii complex, which was sensitive to ciprofloxacin, ampicillin, and gentamicin. The infection resolved after a 1-week course of intravenous augmentin. Ophthalmologists should be alert to the possibility of patients having A. baumannii and A. calcoaceticus in periorbital cellulitis. [ABSTRACT FROM AUTHOR]

Introduction: NTRK-rearranged spindled cell tumors have been increasingly recognized with the widespread use of molecular studies. We describe a pediatric spindle cell neoplasm with MTAP-RAF1 gene fusion that fits into this group. Case report: An 8-year-old girl presented with mandibular mass. Histopathologically, it was a moderate to increased cellular spindle cell tumor with mild-to-moderate nuclear pleomorphism, focal perivascular keloid-like collagen, that was positive for S-100 and CD34. MTAP-RAF1 fusion was detected by next generation sequencing, confirming a low-grade sarcoma with MTAP-RAF1 fusion that is presently included in the category of NTRK-rearranged spindled cell tumors. Discussion: MTAP-RAF1 fusion, in the spectrum of spindle cell neoplasms with kinase gene rearrangements, can occur in the pediatric age group. [ABSTRACT FROM AUTHOR]

Due to the poor cell compatibility, capsular contracture is usually accompanied by the implantation of Silicone Rubber (SR). In this study, synthetical carboxyl grafted polyhedral oligomeric silsesquioxane (POSS-COOH) was incorporated into SR to prepare a composite filling material, namely POSS-COOH/SR. in vivo implantation showed that the occurrence of capsular formation around POSS-COOH/SR was inhibited, in vitro experiment showed cell improved proliferation and adhesion capability on POSS-COOH/SR. Through proteomics analysis, the extracellular matrix (ECM) regulatory protein in the cells on the surface of POSS-COOH/SR changed significantly. In summary, POSS-COOH/SR could be a better biomaterial to scaffold for soft tissue filling. [ABSTRACT FROM AUTHOR]

Background: Lipofibromatosis is a relatively new entity, considered in the differential diagnosis of soft tissue tumors in children, involving mainly the extremities. Most cases can be completely resected without recurrence. Atypical forms have been described and their highly infiltrative capability can lead to severe clinical impairment. Case report: We report an infant with rapidly growing posterior cervicothoracic tumor, extending from the bulbo-medullary junction to T6 vertebra, leading to loss of spontaneous breathing, quadriparesis and devastating long-term consequences. Spinal tomography scan at 35 days suggested myelomeningocele. Prenatal and early postnatal ultrasounds were reviewed and no central nervous system involvement or fusion defects were present. Magnetic resonance at 40 days showed infiltrative mass, later confirmed by pathology as lipofibromatosis. Two resections failed to completely remove the tumor. Discussion: Lipofibromatosis can involve critical structures in the central nervous system and is difficult to treat surgically in those circumstances. [ABSTRACT FROM AUTHOR]

Neoadjuvant chemotherapy and radiotherapy for the management of soft tissue sarcomas (STS) are still preferably delivered sequentially, with or without concurrent hyperthermia. Concurrent delivery of chemo-, radio- and thermotherapy may produce synergistic effects and reduce chemotherapy-free intervals. The few available studies suggest that concurrent chemoradiation (CRT) has a greater local effect. Data on the efficacy and toxicity of adding hyperthermia to CRT (CRTH) are sparse. A cohort of 101 patients with STS of the extremities and trunk who received CRT (n = 33) or CRTH (n = 68) before resection of macroscopic tumor (CRT: n = 19, CRTH: n = 49) or re-resection following a non-oncological resection, so called 'whoops procedure', (CRT: n = 14, CRTH: n = 19) were included in this retrospective study. CRT consisted of two cycles of doxorubicine (50 mg/m2 on d2) plus ifosfamide (1500 mg/m2 on d1-5, q28) plus radiation doses of up to 60 Gy. Hyperthermia was delivered in two sessions per week. All patients received the minimum dose of 50 Gy. Median doses of ifosfamide and doxorubicin were comparable between CRT (75%/95%) and CRTH (78%/97%). The median number of hyperthermia sessions was seven. There were no differences in acute toxicities. Major wound complications occurred in 15% (CRT) vs. 25% (CRTH) (p = 0.19). In patients with macroscopic disease, the addition of hyperthermia resulted in a tendency toward improved remission: regression ≥90% occurred in 21/48 (CRTH) vs. 4/18 (CRT) patients (p = 0.197). With a median postoperative follow-up of 72 months, 6-year local control and overall survival rates for CRTH vs. CRT alone were 85 vs. 78% (p = 0.938) and 79 vs. 71% (p = 0.215). Both CRT and CRTH are well tolerated with an expected rate of wound complications. The results suggest that adding hyperthermia may improve tumor response. [ABSTRACT FROM AUTHOR]

The article discusses a case of periorbital nodular fasciitis in a 35-year-old woman presenting with unilateral S-shaped ptosis. The patient underwent surgical excision of the lesion, which was found to be a benign mass consistent with nodular fasciitis. The article emphasizes the importance of intraoperative assessment in confirming the diagnosis and guiding treatment. The study was conducted at the Barretos Cancer Hospital in Brazil, highlighting the global nature of medical research and collaboration. [Extracted from the article]

Regardless of age at presentation, many soft tissue neoplasms have overlapping histopathologic and immunophenotypic features to serve as a diagnostic challenge. We reported a case of a spindle cell neoplasm in an infant, which was initially considered a vascular anomaly clinically and an eventual biopsy revealed marked inflammation with a spindle cell component that was resolved as an infantile fibrosarcoma with an ETV6 break-apart. The context of this case lead to a further consideration of various other spindle cell neoplasms arising predominantly in the soft tissues during the infancy period as defined by the first two years of age. Though sharing similar morphologic features, these tumors can be categorized into several molecular genetic groups, which have provided both diagnostic and pathogenetic insights as well as treatment options in some cases. [ABSTRACT FROM AUTHOR]

Introduction: One-third of fetal soft tissue tumors are malignant and include congenital fibrosarcoma (CF). We report two fetal CFs arising in the posterior mediastinum. Case Presentation: In case 1, the CF resulted in a mediastinal shift, extensive infiltration of the tumor around adjacent structures, pulmonary hypoplasia, pleural effusion, and rapid growth. The pregnancy was terminated. Case 2 had multiple intrathoracic masses, thoracic hypoplasia, pleural effusion, and fetal death. Both were diagnosed as fibrosarcoma at fetopsy. Discussion: Although congenital CF tends to be locally aggressive with a low metastatic rate, it tends to grow rapidly and the tumor location can affect fetal survival. In Case 1, the tumor demonstrated locally aggressive behavior whereas multiple distant metastases such as lung, liver, adrenals, and left eye were detected in Case 2. The tumor was directly responsible for intrauterine fetal demise in the second case. [ABSTRACT FROM AUTHOR]

Benign benign vascular tumors (e.g., hemangiomas) and malformations are commonly encountered lesions in all ages of life, especially in infancy and childhood. Hemangiomas are considered to be proliferative vascular lesions while malformations are defects of embryonal vascular morphogenesis. Less than 1% of hemangiomas within the body occur in skeletal muscle and of these approximately 15% have been reported to occur in the head and neck musculature (e.g. masseter, trapezius, sternocleidomastoid, mylohyoid, temporalis muscles) Intramuscular angioma (the preferred term for lesions formerly known as intramuscular hemangiomas by WHO Tumors of Soft Tissue and Bone Classification, 5th edition 2020) (IA) occurring in the extraocular muscles or palpebral muscles (orbicularis oculi) are extremely rare with only a few case reports in the English literature. To date, all the extraocular muscles have reportedly been involved. With the case reported herein, the medial rectus muscle appears to be the most common extraocular muscle involved. [ABSTRACT FROM AUTHOR]

A 28-year-old male presented with gradually progressive swelling of the right lower eyelid along with a prominence of the eye for 6 months. He had received oral steroids and intraorbital triamcinolone acetonide injection in the inferior quadrant for active thyroid eye disease. External examination revealed right eye proptosis and swelling along the inferior orbital region. Magnetic resonance imaging showed an ill-defined soft tissue lesion in the inferior extraconal space and a bulky right inferior rectus. Histopathology of the biopsied material revealed inflammation with septate fungal filaments, identified as Aspergillus flavus on culture. He responded well to oral voriconazole despite a recurrence during the course of treatment. Intraorbital steroids are given for idiopathic and thyroid-associated orbital inflammation. This is a report of a rare complication of fungal orbital abscess following intraorbital corticosteroid injection in an immunocompetent young patient. [ABSTRACT FROM AUTHOR]

Background Infantile myofibromas (IM) are benign soft tissue lesions of childhood and represent a significant portion of the benign spectrum of fibroblastic-myofibroblastic tumors. Cytological diagnosis of these tumors can be challenging because of overlapping morphology and limited case report descriptions. We describe the cytological features and the cytological differential diagnoses. Case report We describe cytological features of two IMs. The main features were the presence of loose clusters and dispersed bland myofibroblasts in varying stages of maturation with traversing blood vessels and myxoid stroma. The cells typically lacked features of atypia, mitoses and significant pleomorphism. Conclusion Diagnosis of IM on the basis of cytology alone can be tricky and definitive diagnosis should be made only after correlating the cytological features with histology. However, bland morphology of differentiating myofibroblasts can aid in cytological diagnosis and help to exclude other malignant spindle cells neoplasms needing preoperative chemotherapy. [ABSTRACT FROM AUTHOR]

A 71-year-old male with a 4-month history of bulging, tearing, and redness in the right eye presented with vision loss, proptosis, conjunctival hyperemia, and chemosis. Magnetic resonance imaging showed a right intraconal solid mass with extraconal extension, hyper-intense in T2 sequences with heterogeneous contrast enhancement. Complete excision of the mass was performed through a lateral orbitotomy. Histological analysis revealed a neoplasm with high vessel density, solid growth of oval cells, a concentric proliferation of the wall of small vessels, and a weak and patchy positivity for smooth muscle actin. These findings were consistent with the diagnosis of myopericytoma. After surgery, visual acuity improved in the affected eye and after 18 months of follow-up there have been no signs of recurrence. [ABSTRACT FROM AUTHOR]

This study aims to evaluate whether sarcopenia, measured by chest computed tomography (CT), affects survival outcomes and postoperative complications in soft tissue sarcoma (STS) patients undergoing surgery. In this retrospective study, CT scans of 79 patients were reviewed to measure pectoralis and T12 vertebra muscle area. Both were then adjusted for height (cm2/m2) as pectoralis muscle index (PMI) and T12 vertebra muscle index (TMI). Analyses were performed by dichotomizing muscle indices at gender-specific 50th percentile; PMI and TMI < 50th percentile were defined as low, and ≥50th percentile as high. Overall postsurgical complication rate (PCR) was 16%. Median length of hospital stay (LOHS) was 10 days (3–90). PMI and TMI were significantly lower in women (p = 0.02, p = 0.04). Median body mass index was significantly higher in high PMI and TMI groups (p = 0.01 for both). PCR and LOHS were similar between low and high PMI and TMI groups. Median follow-up was 29 months, 37 patients had recurrence and 23 died. No significant difference was noted between low and high PMI and TMI groups, in terms of disease-free or overall survival. PMI and TMI as measured by chest CT had no impact on survival outcomes or postoperative complications in localized STS. [ABSTRACT FROM AUTHOR]

Myxoid liposarcoma (MLPS) has different patterns that are often difficult to distinguish from other soft tissue lesions. MLPS is characterized by a reciprocal translocation involving the DNA Damage Inducible Transcript 3 gene (DDIT3) that can be detected using fluorescent in situ hybridization (FISH). Recently, the marker for cancer testis antigen 1b (CTAG1B) was found to be expressed in MLPS. The aim of the present study was to assess the potential use immunohistochemistry (IHC) for CTAG1B expression and DDIT3 rearrangement to diagnose MLPS and distinguish it from similar lesions. Out of 29 cases including MLPS and its mimics, CTAG1B was expressed in 92.86% of cases of MLPS and 20% of its mimics. DDIT3 rearrangement was 100% sensitive and 92.86% specific in distinguishing MLPS from its mimics. The DDIT3 rearrangement was found to be more sensitive but less specific than cytoplasmic expression of CTAG1B marker. DDIT3 polysomy and amplification were detected in some cases. Therefore, both CTAG1B expression and FISH for DDIT3 gene can be used to distinguish MLPS from similar tumors. The use of both immunohistochemistry for CTAG1B in addition to DDIT3 gene rearrangement detection by FISH was more specific than using either of them alone. However, the DDIT3 gene rearrangement alone was the most sensitive test for distinguishing MLPS from its mimics. [ABSTRACT FROM AUTHOR]

Introduction: Infantile fibrosarcoma (IFS) usually arises in the extremities during the first 12 months of life and responds well to surgery. It is unusual in the oropharynx or the prenatal period. Case report: A giant solid mass was first detected in the oropharynx and anterior neck at 24 weeks of gestation by ultrasound and fetal MRI. An EXIT procedure with intrapartum intubation with appropriate supportive therapy was successful. The diagnosis of IFS was made postpartum, and the lesion responded to neoadjuvant chemotherapy. Conclusion: IFS may arise as early as 24 weeks of gestation. In this case, an EXIT procedure allowed postpartum diagnosis with subsequent treatment. [ABSTRACT FROM AUTHOR]

Proliferative fasciitis (PF) is a benign fibroblastic reaction with histological and clinical characteristics that overlap with those of malignant soft tissue tumors; thus, it is referred to as a pseudosarcomatous reaction. It continues to be an important cause of diagnostic error and overtreatment. The childhood PF subtype has some distinct histological and immunohistochemical characteristics that make differential diagnosis with malignant tumors even harder, especially with sarcoma. These proliferations generally occur in the lower limbs, and the periorbital region is a rare location of appearance. Here, we describe a case of childhood subtype PF in a 16-year-old girl located in the periorbital area. To the best of our knowledge, this is the first reported case of childhood subtype PF in the periorbital area, and the third case if PF subtypes are not taken into account. [ABSTRACT FROM AUTHOR]

Plamotamab (XmAb SP ® sp 13676) is a humanized bsAb that binds both CD3 and the tumor antigen CD20 in order to recruit cytotoxic T cells to kill CD20+ tumor cells. As shown in Figure 2(C), the patient's right inner thigh EMWM tumor that completely disappeared after 2 cycles of XmAb SP ® sp 13676 had a dense CD8+ T cell infiltrate whereas the left anterolateral thigh EMWM tumor that grew in size after 2 cycles of XmAb SP ® sp 13676 had a scattered CD8+ T cell infiltrate (Figure 2(G)). (E) PET-CT images of left anterolateral thigh soft tissue tumor after two cycles of XmAb®13676.(F) PET-CT images of right inner thigh soft tissue tumors after four cycles of XmAb®13676. Patients having nonsense CXCR4 mutations have lower ORR to ibrutinib compared to frameshift mutations and wild type (55%, 79% and 85%, respectively; I p i = < 0-001) as well as lower PFS; 43 months vs. not reached for patients with frameshift mutations and wildtype CXCR4 mutations ( I p i = 0.01) [[7]]. [Extracted from the article]

We aimed to compare the oncological outcomes of patients who underwent re-excision following unplanned surgery and those who underwent planned surgery. Patients who were referred to our hospital after a mass was inappropriately resected and reported to have a malignant pathology with a positive surgical margin, as well as patients diagnosed with malignant soft tissue tumors, and operated on following a multidisciplinary assessment and staging between 2012 and 2018. All patients were followed up at least 6 months. Our study included a total of 125 patients. Forty percent (n = 50) underwent unplanned excision and sixty percent (n = 75) underwent planned excision.There was no statistically significant difference in the survival curves between the two groups (p = 0.248). Tumor size was larger, and the rate of deep-localized tumors was higher in patients undergoing planned surgery than in the unplanned surgery group (p = 0.001). The rate of tumors localized in the upper extremities was significantly higher in the unplanned surgery group than in the planned surgery (p = 0.033). MRI examinations could detect residual tumors with an accuracy of 80%. Age (>48 years), tumor size (>8 cm), tumor grade (grade 3), and distant organ metastasis at follow-up, which were among risk factors found to have a significant effect on mortality. Patients undergoing re-excision after an inappropriate resection and undergoing planned resection had a similar prognosis. Superficial, upper extremity-localized, and relatively small-size tumors are more prone to inadequate surgical resection. MRI can be used to detect residual tumors at a high rate in patients who have undergone unplanned surgery. Distant organ metastasis is the most important factor affecting survival. [ABSTRACT FROM AUTHOR]

Lipoma is a benign soft tissue tumor that is mostly found in the trunk, however, lipomas of the thumb are rarely reported, around 1% of cases. We report a case of a Saudi male aged 32-year-old who presented with a progressive left thumb swelling for a long time. The aim is to add more cases to the literature and to consider it as a differential. [ABSTRACT FROM AUTHOR]

Preauricular lesions such as cysts, pits, fissures are congenital malformations of the preauricular soft tissues. These lesions are common and seen in the paediatric population, located near the anterior aspect of the ear. Surgical excision of the lesion would usually be performed if the lesion is infected to avoid multiple episodes of infection. We report a case of a 9-year-old boy who presented with a lesion of the preauricular area. It was excised due to enlargement of the lesion over time. Histopathological examination revealed the lesion to be a rare myopericytoma of the preauricular sinus. In this report, we discuss regarding this rare soft tissue lesion with regards to the head and neck region. [ABSTRACT FROM AUTHOR]

Rhabdomyosarcoma (RMS) is a heterogeneous soft tissue neoplasm most frequently found in children and adolescents. As the prognosis for recurrent and metastatic RMS remains poor, immunotherapies are hoped to improve quality of life and survival. CD137 is a member of tumor necrosis factor receptor family and a T cell costimulatory molecule which induces potent cellular immune responses that are able to eliminate malignant cells. Therefore, it was puzzling to find expression of CD137 on an RMS tissue microarray by multiplex staining. CD137 is not only expressed by infiltrating T cells but also by malignant RMS cells. Functional in vitro experiments demonstrate that CD137 on RMS cells is being transferred to adjacent antigen-presenting cells by trogocytosis, where it downregulates CD137 ligand, and thereby reduces T cell costimulation which results in reduced killing of RMS cells. The transfer of CD137 and the subsequent downregulation of CD137 ligand is a physiological negative feedback mechanism that is likely usurped by RMS, and may facilitate its escape from immune surveillance. In addition, CD137 signals into RMS cells and induces IL-6 and IL-8 secretion, which are linked to RMS metastasis and poor prognosis. However, the ectopic expression of CD137 on RMS cells is an Achilles' heel that may be utilized for immunotherapy. Natural killer cells expressing an anti-CD137 chimeric antigen receptor specifically kill CD137-expressing RMS cells. Our study implicates ectopic CD137 expression as a pathogenesis mechanism in RMS, and it demonstrates that CD137 may be a novel target for immunotherapy of RMS. [ABSTRACT FROM AUTHOR]

Benign giant renal schwannoma is rarely found and the diagnosis difficulty only depends on physical and imagological examination. Pathological examination is essential to confirm the diagnosis of renal schwannoma. Nephrectomy and tumorectomy are primary treatments for renal schwannoma. Although most of the reported patients present satisfactory outcome, however, there is still not sufficient evidence to reveal the biological characteristics and post-operation recurrence rate of renal schwannoma. Herein, we report a rare case of giant and complicated renal schwannoma. A 56-years-old female patient was admitted to the urology department due to left lower back pain for approximately 5 days. No positive signs and other special abnormalities were found. CT scan presented a soft tissue tumor with inhomogeneous enhanced in the renal hilum. Surgery was performed to excise the tumor and left renal. Renal schwannoma was confirmed by pathological examination. At the 6-month follow-up, no evidence of recurrence was found. Our present report could provide more material for further study. [ABSTRACT FROM AUTHOR]

Perineurioma is a rare benign soft tissue tumor arising from the peripheral nerve sheath. To date only two cases of orbital perineurioma have been reported in literature. A 45-year-old, with no comorbidities, presented with protrusion of the right eye since one-year. Examination revealed 8mm proptosis of the right eye. Dilated fundoscopy of the right eye showed a pale optic disc with choroidal folds temporal to it. Computerised Tomogram (CT) of the orbit showed a well-defined intraconal mass in the inferotemporal part of the orbit. A trans conjunctival orbitotomy with lateral canthotomy was performed and the tumor was removed en bloc. On immunohistochemistry, the tumour was negative for S-100 and showed patchy weak Epithelial Membrane Antigen positivity which was suggestive of a soft tissue perineurioma. [ABSTRACT FROM AUTHOR]

We describe a novel technique for endonasal endoscopic orbital surgery. Previously described strategies for minimally invasive orbital surgery include mobilization of the medial rectus muscle and cryosurgical retraction of the pathologic lesion. Herein we describe endoscopic trans-septal suture retraction of an orbital cavernous hemangioma to facilitate intra-orbital dissection from an external approach. A 36-year-old male presented with blurred vision in his left eye for several years, along with several months of progressive left proptosis and decreasing visual acuity. Imaging revealed a 2.5 cm orbital mass consistent with a cavernous hemangioma. The patient underwent a combined transconjunctival and endoscopic approach for resection. Following left medial orbital wall and floor decompression, endoscopic trans-septal suture retraction facilitated dissection of the lesion from intraorbital structures. A complete extracapsular resection of the lesion was achieved. The inferomedial orbit was reconstructed with a synthetic implant. Trans-septal suture retraction is an effective adjunctive technique for endonasal endoscopic orbital tumor surgery. This technique facilitates safe retraction and dissection of soft tissue lesions from critical intraorbital structures. [ABSTRACT FROM AUTHOR]

Rhabdomyosarcoma (RMS) is a malignant soft tissue tumor of the pediatric population which is  rarely seen in adults. Metastatic rhabdomyosarcoma is even rarer. We present an unusual case of a 49 year old female presenting with palpitations and uterine bleeding. An Echo-cardiogram revealed a large oval mass on the posterior mitral leaflet and a Computerized Tomography (CT) scan of the abdomen revealed a uterine growth. Surgical excision of the cardiac mass was done and histological analysis of cardiac lesion confirmed it to be rhabdomyosarcoma with a primary source in the uterus. The patient became asymptomatic from a cardiac standpoint after excision of the mass and was scheduled for chemo/radiation therapy for the primary uterine malignancy. Metastatic cardiac rhabdomyosarcoma can be confused with a myxoma or any other primary or secondary cardiac tumors resulting in delayed diagnosis. However, its aggressive nature makes it a life-threatening tumor that requires an early diagnosis to prevent fatal consequences. [ABSTRACT FROM AUTHOR]

Background: Understanding resting energy expenditure (REE) is important for determining energy requirements; REE might be altered in individuals with cancer. The objective of this study was to characterize determinants of REE in patients with stages II–IV colorectal cancer (CRC). Methods: REE was measured via indirect calorimetry in patients with newly diagnosed CRC. Computerized tomography images from medical records ascertained skeletal muscle and total adipose tissue cross-sectional areas, which were then transformed to lean soft tissue (LST) and fat mass (FM) values (in kg). Linear regression assessed determinants of REE. Results: 86 patients were included (n = 55, 64.0% male; 60 ± 12 years old; median body mass index: 27.6, interquartile range: 24.3–31.2 kg/m2), with most (n = 40) having stage III disease. Age, sex, and weight were significant predictors of REE [R2 = 0.829, standard error of the estimate (SEE): 128 kcal/day, P < 0.001]. Replacing weight with LST and FM yielded a similar model, with age, sex, LST, and FM predictive of REE (R2 = 0.820, SEE: 129 kcal/day, p < 0.001). Conclusion: Age, sex, weight, LST, and FM were the main contributors to REE. Further investigation of REE changes over time and its relationship to total energy expenditure, dietary intake, and clinical outcomes should be explored. [ABSTRACT FROM AUTHOR]

Intraductal tubulopapillary neoplasm (ITPN) is a premalignant epithelial tumor of the pancreas. We report a case of ITPN detected incidentally during histopathology examination (HPE) from a specimen taken at the time of endosonography in a patient with pancreatitis. A 53-year-old man presented with severe recurrent epigastric pain, associated with episodic nonbilious vomiting, with a weight loss of about 7 Kg since 15 days. He underwent Magnetic resonance cholangiopancreatography which showed an ovoid soft tissue lesion in the Pancreatic duct (PD) reported as possible intraductal neoplasm. Patient underwent Endoscopic Ultrasound, whereby the PD was dilated and a large hypoechoic lesion was noted in the PD (head region) causing upstream dilatation. Lesion did not show any vascularity on Doppler. Fine needle biopsy was sent from the intraductal lesion for histopathology examination (HPE). Patient then underwent an ERCP. After performing a pancreatic sphincterotomy, pancreaticoscopy was done using SPYGLASS DS system which showed a nodular intraluminal lesion in the head region with irregular and abnormal vascularity and papillary fronds. Biopsies were taken from this lesion using SpyBite biopsy forceps. To our surprise this HPE report came out to be ITPN. Patient was then referred for surgery. There is often a difference of opinion in distinguishing and classifying such tumors when compared to the subtypes of mucinous neoplasm. Identification of this neoplasm is often challenging for pathologists as well as radiologists. We present this case since it is infrequent and atypical in routine medical practice. [ABSTRACT FROM AUTHOR]

Introduction: Soft tissue sarcomas (STS) encompass a group of rare and heterogeneous cancers. Rapid evolution in the field justifies a focused critical review of the clinical literature.Areas covered: Important advances, and associated controversies, in managing patients with STS are reviewed by treatment setting, histopathological subtype, and general management considerations. Despite many advances, opportunities remain to improve care to benefit patients. Areas of sarcoma research showing early promise indicate that new treatment development requires a deeper understanding of the biological characteristics of unique histotypes, with a focus on targeting sarcoma-relevant oncogenic driver mechanisms.Expert opinion: Mandatory treatment of sarcoma patients in reference centers represents a simple and cost-effective means of improving survival as the knowledge generated through international collaboration can be implemented sooner to improve patient outcomes. Sarcoma complexity complicates clinical trial designs in both peri-operative and advanced treatment settings. Future trials in front-line therapy in advanced situations should enroll patients based on the goal of therapy - tumor shrinkage or palliation - using appropriate endpoints to evaluate benefit. Global collaboration remains key to identifying the most appropriate patients and concepts to test in well-designed and accessible clinical trials. The sarcoma community shares a common desire that standard treatments will be established based on molecular profiling within the next five to 10 years. [ABSTRACT FROM AUTHOR]

Introduction: Although rapid evolution over the past few years in advanced soft tissue sarcoma (STS) management has not been without its challenges, it has brought clarity in several areas.Areas covered: This article summarizes the proceedings of the third edition of the Soft Tissue Sarcoma: Evidence and Experience symposium held March 2019 in Madrid, Spain. An update is provided of current approaches to advanced STS management. Case studies illustrate the role of trabectedin in advanced STS management.Expert opinion: First-line treatment of advanced STS requires distinct therapeutic strategies depending on goal: tumor shrinkage or tumor control. Since all sarcoma patients benefit from active treatment irrespective of age or line of therapy, oncologists have a duty to offer active systemic therapies unless the patient is unfit for treatment or chooses to end active treatment. Beyond the first line, histology becomes increasingly relevant for treatment selection. Agents with activity in specific sarcoma subtypes have been identified. Rare tumors represent a substantial medical need requiring strong international collaboration between research groups, pharmaceutical companies, regulatory agencies, and patients to identify active drugs per subtype. Multidisciplinary care in an expert sarcoma center is the primary means of reducing morbidity and mortality in patients with sarcoma. [ABSTRACT FROM AUTHOR]

Background: Given the rapid evolution in the management of soft tissue sarcoma (STS), it is essential to revisit the evidence regularly. This review examines topics of interest for early management of STS: the impact of molecular genetics on sarcoma classification; the importance of a correct diagnosis and strategy in the surgical management of STS; current status on use of radiotherapy in STS.Areas covered: Accurate diagnosis of STS combines histomorphology, immunochemistry, and molecular genetics, although morphology is the mainstay of therapeutic planning. As diagnosis of STS is challenging, it is best conducted within a multidisciplinary environment. Expert surgery in STS takes into account multiple parameters including biopsy, imaging, pathological knowledge, technical issues, and a multidisciplinary approach. The sum of these factors informs decisions about whether or not to perform surgery and the choice of surgical technique. Advances in radiotherapy are challenging the paradigm of applying the same dose and treatment schedule to all STS patients irrespective of subtype. Preoperative radiotherapy of specific histotypes appears to be the future although more research is required to address uncertainties such as fraction size, total dose, combined modality regimens, and individual sensitivity to radiotherapy.Expert opinion: STS should be managed in a reference center. [ABSTRACT FROM AUTHOR]

Introduction: The aim of this case series was to assess sports activity levels in long-term survivors of soft-tissue sarcomas after multimodal treatment including limb salvaging surgical resection and radio-chemotherapy. Methods: Thirty-two patients (17 f/15 m) with a mean age of 29 (range 10–44) years at the time of diagnosis and a mean follow-up time of 9 (range 3–21) years following sarcoma were included. Ten patients had been diagnosed with liposarcoma, seven with synovial sarcoma, four with fibrosarcoma, three with undifferentiated pleomorphic sarcomas and the remaining eight patients with different soft-tissue sarcoma entities. Sarcoma location of twenty-four (75%) patients was in the thigh, eight (25%) in the lower leg. Eleven (34%) tumors were located epifascial, 21 (66%) in deeper tissue layers. Sports activity was measured by the University of California, Los Angeles (UCLA) Activity Score. Results: One year before treatment 30 patients (94%) and >3 years post-treatment 29 patients (91%)had been pursuing athletic activity regularly. They were performing 5.5 h/week prior and 4.2 h/week >3 years post surgery. The mean UCLA score in case of subfascial sarcomas was 8.0 and thereby lower (p < 0.05) than those of epifascial sarcomas, which were 9.3. Furthermore, the mean UCLA score after subfascial sarcoma resection 3 years postoperative was still below preoperative UCLA levels (p < 0.05), whereas patients after epifascial tumor resection showed no loss of sports activity. No relation between complications and postoperative sports activity level was found. Conclusions: Healthy long-term survivors can achieve high levels of sports activity following limb salvage after soft-tissue sarcomas. The concerned muscle groups may influence the functional and sports outcome and draw attention to this topic in future rehabilitation. This knowledge may be of high value for patients counseling, physicians and orthopedists treating patients, as well as for patients inquiring information regarding post-treatment activity levels. Sport is possible after soft tissue sarcoma treatment and longer-term rehabilitation which is usually three years Rehabilitation is best undertaken in specialized centers It is important to maintain contact with surgeons in order to optimize mobilization High impact sports may be affected by deep muscle resections. [ABSTRACT FROM AUTHOR]

Ewing´s Sarcoma Family of Tumors (ESFT) are clinically aggressive bone and soft tissue tumors in children and young adults. Analysis of the immune tumor microenvironment (TME) provides insight into tumor evolution and novel treatment options. So far, the scarcity of immune cells in ESFT has hindered a comprehensive analysis of rare subtypes. We determined the relative fraction of 22 immune cell types using 197 microarray gene expression datasets of primary ESFT tumor samples by using CIBERSORT, a deconvolution algorithm enumerating infiltrating leucocytes in bulk tumor tissue. The most abundant cells were macrophages (mean 43% of total tumor-infiltrating leukocytes, TILs), predominantly immunosuppressive M2 type macrophages, followed by T cells (mean 23% of TILs). Increased neutrophils, albeit at low number, were associated with a poor overall survival (OS) (p =.038) and increased M2 macrophages predicted a shorter event-free survival (EFS) (p =.033). High frequency of T cells and activated NK cells correlated with prolonged OS (p =.044 and p =.007, respectively). A small patient population (9/32) with combined low infiltrating M2 macrophages, low neutrophils, and high total T cells was identified with favorable outcome. This finding was confirmed in a validation cohort of patients with follow up (11/38). When comparing the immune TME with expression of known stemness genes, hypoxia-inducible factor 1 α (HIF1α) correlated with high abundance of macrophages and neutrophils and decreased T cell levels. The immune TME in ESFTs shows a distinct composition including rare immune cell subsets that in part may be due to expression of HIF1α. [ABSTRACT FROM AUTHOR]

Introduction: Lipofibromatosis is a benign pediatric soft tissue tumor arising preferentially in the distal extremities. Histologically, the tumor shows abundant adipose tissue admixed with a spindle cell component, often concentrated in septal and perimysial locations. The index case is being presented to discuss the histopathological and immunohistochemical clues to differentiate it from other fibrofatty tumors of childhood. Case report: An 11-month-old male child presented with a slowly growing mass on the upper back. MRI findings were suggestive of an adipocytic tumor. Microscopy revealed a lesion composed of mature adipocytes and intervening fibrous bands with infiltration into the adjacent skeletal muscle, features of lipofibromatosis. Conclusion: Lipofibromatosis should be considered in the differential diagnosis of a pediatric fibrofatty tumor. Accurate diagnosis is essential for proper patient management as incomplete removal of the tumor may result in recurrence. [ABSTRACT FROM AUTHOR]

We report a case of a 2-year-old female who presented with bilateral progressive proptosis, visual loss, nasal obstruction, and breathing difficulty. Magnetic resonance imaging revealed a large sino-orbital mass that was extending to the orbital apex and skull base. An initial diagnosis of rhabdomyosarcoma was made elsewhere on the basis of the presence of round and spindle cell tumor. Subsequent biopsy with immunohistochemical staining was positive for nuclear staining with β-catenin, shifting the diagnosis to a myofibroblastic tumor, favoring desmoid-type fibromatosis. With image guidance, near complete excision of tumor was performed by a multidisciplinary team, while respecting danger zones such as the skull base and the optic nerve. Following a recurrence over 2 months, additional excision was performed with a 6-month treatment of methotrexate and vinblastine. Desmoid tumor is a rare form of soft tissue tumor uncommonly seen in the orbital area. Although benign, it is known to be recurrent and infiltrative. Few data are known and further information will aid in the management of these tumors. [ABSTRACT FROM AUTHOR]

Introduction: The development of precision medicine and targeted therapies have revolutionized cancer treatment. Historically, treatment was chosen based on the tumor-histology, but can now be tailored to patient-specific biomarkers. Investigations have shown up to 40% of cancer patients who undergo molecular testing have an actionable biomarker with a drug currently available, and that patients benefit from these drugs. In 2018, larotrectinib became the first drug developed and approved exclusively as a tumor-agnostic therapy. Because of advancement in precision medicine, biomarker testing has become a standard of care in a variety of tumors and its use is increasing.Areas covered: We discuss the landscape of biomarker testing in the context of soft tissue sarcomas and thyroid cancer, two rare diseases with historically high unmet needs in their subpopulations. We summarize historical data and contemporary applications.Expert opinion: The paradigm shift in oncology treatment toward precision medicine, including tumor-agnostic agents, is experiencing substantial momentum but still facing challenges. A gap exists between guideline recommendations for biomarker testing and clinical application, resulting in compromised access and suboptimal outcomes. Future progress will require routine access to testing and expanding treatment options coupled with awareness, predictability, and strategies to address resistance mechanisms. [ABSTRACT FROM AUTHOR]

The purpose of this study was to explore the experiences of parents and co-parents where one parent was diagnosed with cancer with specific interest in the impact of the disease on their role as a parent and the supports that were found to be useful in managing the ongoing care of their children. A qualitative research method using semi-structured interviews and thematic network analysis techniques was used. Participants included adult English-speaking parents receiving treatment for a new diagnosis of cancer at a major tertiary oncology research and treatment centre, with prior social work involvement. Participants reported multiple, complex layers of need. Four salient themes were evident: (a) emotional impact of a cancer diagnosis on the patient, (b) accessing professional supports, (c) children's understanding, and (d) meeting children's needs. Parents described difficulties in managing emotionally, physically, and existentially. Accessing supports within the healthcare service and community was paramount, as was guidance around communicating with children. Receiving a proactive service from a social worker was highly regarded. The results demonstrate that it is essential to respond to the needs of parents with cancer in order to minimise prolonged emotional distress. Parents diagnosed with cancer have unique needs including concern about the impact on their children and families. Social workers should be proactive in identifying patient needs and ensuring services and supports are provided in a timely way. [ABSTRACT FROM AUTHOR]

Calcifying fibrous pseudotumor (CFP) is a rare, benign soft tissue tumor that may uncommonly arise in the pleura. These tumors can show multifocal dissemination across the pleural surface, but the mechanism underlying this dissemination is unclear. Review of previously reported cases of pleural CFP demonstrates a strong predilection for basal and diaphragmatic pleural surfaces, and a significantly higher rate of multifocality compared with other locations. We present a 59-year-old male with multiple CFP of the pleura. Reactive-appearing adhesions spanning the pleural surfaces were present, and by electron microscopy, were involved by tumor. We suggest this is the likely mode of dissemination across the pleural surfaces. [ABSTRACT FROM AUTHOR]

Angiomyofibroblastoma (AMFB) is a very rare mesenchymal tumour of the lower female genital tract particularly originated from the vulva (Fletcher et al. 1992). Discussion Angiomyofibroblastomas (AMFBs) are a member of genital stromal mesenchymal tumours along with cellular angiofibroma, superficial myofibroblastoma, fibroepithelial stromal polyp, mammary-type myofibroblastoma and aggressive angiomyxoma (Schoolmeester and Fritchie 2015). [Extracted from the article]

Background: Recurrence after wide excision or residual tumor after an unplanned excision of a malignant soft tissue sarcoma (STS) is a complex problem, due to a higher recurrence rate and poorer survival rate compared with primary resection. Regional hyperthermia was used, with the expectation that it will enhance the anti-tumor effects of chemotherapy and radiotherapy. This study aimed to assess the efficacy of neoadjuvant concomitant radiotherapy, hyperthermia, and chemotherapy (RHC) for salvage of recurrent or residual malignant STS. Methods: We identified 64 patients with recurrent or residual STS treated between 1994 and 2013. After excluding those with low-grade malignancy, with recurrent bone tumor in the soft tissues, with truncal STS, and who declined to participate, 23 patients (7 with recurrence and 16 with residual tumor) underwent RHC. The histologic diagnoses were undifferentiated pleomorphic sarcoma (n = 11), synovial sarcoma (n = 3), leiomyosarcoma and myxoid liposarcoma (n = 2 each), and other histologic types. As primary outcomes, the 5-year overall survival (OS), distant metastasis-free survival (D-MFS), and local control (LC) rates were evaluated by Kaplan-Meier analysis. Results: The median follow-up period was 112.3 months. The 5-year OS, D-MFS, and LC were 86.4%, 77.4%, and 86.7%, respectively. In the univariate analysis, tumor depth was considered as a negative prognostic factor for OS and D-MFS, and a positive margin was also a negative prognostic factor for OS, D-MFS LC with retained on Cox proportional hazards model in OS, and D-MFS. Conclusion: RHC is an effective option for salvage treatment of recurrent and residual STS. [ABSTRACT FROM AUTHOR]

We present two cases of malignant soft tissue tumor around the elbow joint treated with en bloc resection, in which reconstruction using a frozen autograft technique in combination with a free anterolateral thigh flap offered reliable method for limb salvage and good recovery of elbow function. [ABSTRACT FROM AUTHOR]

Purpose: Radiotherapy before or after resection is one of the pillars of treatment for localised high risk soft tissue sarcomas. Treatment intensification has been described with concurrent chemotherapy and hyperthermia. The aim of this study is to assess local control after multimodal treatment, focussing on the treatment of local recurrences after surgery only. Patients and methods: Of 42 patients treated in a prospective protocol with radiotherapy and hyperthermia, nine were treated for isolated local recurrences without metastatic spread. Most patients were treated with trimodal therapy including chemotherapy with ifosfamide and underwent resection whenever possible. Median follow-up was 1.4 years. Results: The treatment was well tolerated. Estimated disease free survival, distant metastases free survival and local control for the whole cohort after 1.5 years were 66, 73 and 88%, respectively. Neoadjuvant vs. adjuvant treatment influenced local control with a trend to statistical significance. Resection status did not influence local control. The cohort of patients treated for local recurrence after surgery alone had a significantly impaired local control compared to multimodal treatment at primary diagnosis (100 vs. 52%, p < 0.001). Conclusions: With multimodal therapy including radiotherapy and hyperthermia local tumour control is achievable even in locally recurrent tumours. The clear-cut difference of the treatment of local recurrence in contrast to primary diagnosis might either reflect difficulties in diagnosis and treatment of local recurrences or biological aggressiveness of recurrent tumours. However, we recommend to consider multimodal treatment at primary diagnosis of high risk soft tissue sarcomas. [ABSTRACT FROM AUTHOR]

Granular cell tumors (GCTs) are rare soft tissue neoplasms which may be multicentric. The vast majority are benign, however approximately 100 malignant GCTs have been reported, with only 8 originating in the vulva. Malignant GCTs are very aggressive with very poor survival rates. As the diagnosis of malignant GCT carries an extremely poor prognosis, the utilization of EM ensures that the most accurate diagnosis possible can be rendered. [ABSTRACT FROM AUTHOR]

Therapies targeting programmed death 1-(PD-1) or its ligand (PD-L1), promoting antitumor T-cell activity have been successfully introduced into clinical practice. Clinical response correlates with PD-L1 expression by tumor cells or immune cells within the tumor microenvironment. The PD-L1/PD-1 axis and tumor microenvironment has been rarely studied in high-grade sarcomas of soft tissue (hSTS), a group of rare, genetically heterogenous and clinically aggressive tumors. We examined PD-L1 protein and CD274/PD-L1 gene copy number variations in 128 primary resected, therapy-naive hSTS using immunohistochemistry and fluorescence-in-situ hybridization. Frequency of tumoral PD-L1 expression varied widely in different disease subentities, with highest rates of positivity (40%) seen in undifferentiated pleomorphic sarcomas (UPS) and rare positivity detected in synovial sarcomas (6%). Amplification of the CD274/PD-L1 gene occurred in 14% of UPS and was rare in other subtypes. PD-L1 protein expression was significantly more frequent in CD274/PD-L1 amplified cases (p D 0.015). The subgroup of UPS was further characterized regarding the interaction between PD-L1 and the immunologic tumor microenvironment. High density of CD3C and CD8C tumor infiltrating lymphocytes (TILs) was significantly correlated with the presence of PD-L1 expression and seen more frequently in tumors with lower TNM stage (p D 0.024). Both, PD-L1 expression and high density lymphocytic infiltration were independent prognostic factors for a favorable overall (p D 0.001, HR 6.105 (2.041-8.258)), disease-specific (p D 0.003, HR 10.536 (2.186-50.774)) and disease-free survival (p D 0.020, HR 3.317 (1.209-9.106); values for CD8) in this particular subgroup of hSTS, whereas PD-L1 expression in TILs or CD274/PD-L1 gene amplification were not associated with outcome. These findings represent novel insights into the immune landscape of soft tissue sarcomas, in particular UPS and strengthen the rationale for immunotherapy, including targeting the PD-1/PD-L1 axis in these tumors. [ABSTRACT FROM AUTHOR]