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Abstract Background Undifferentiated pleomorphic sarcoma, previously called malignant fibrous histiocytoma, is a type of malignant mesenchymal tumor (sarcoma) of soft tissue and sometimes bone. It is uncommon in the oral cavity and very sporadic in the maxillary sinus. Microscopic diagnosis of this malignancy in the maxillary sinus can be very challenging, because there is a range of features that may overlap with other benign and malignant tumors. Case presentation In this paper, we report a case of undifferentiated pleomorphic sarcoma in the maxillary sinus of a 61-year-old Iranian man who was referred to the maxillofacial surgery ward due to pain and swelling of the upper jaw and visual problems in the right eye. In the initial incisional biopsy, peripheral giant cell granuloma was diagnosed in the hospital service. Yet, on request of the surgeon, during reviewing the slides in the oral pathology service of the School of Dentistry, and using an immunohistochemical method, undifferentiated pleomorphic sarcoma was diagnosed. In this paper, a case of undifferentiated pleomorphic sarcoma in the maxillary sinus is reported, with an emphasis on the management of its problems and diagnostic errors. Conclusion This study reviews the challenges and histopathological diagnostic errors of this uncommon tumor in this rare location. This lesion may be similar to other malignant tumors, and its correct diagnosis requires a detailed and complete examination.

Soft tissue sarcomas (STS) are a group of rare malignant tumors arising from mesenchymal stem cells. There are more than 60 different types of neoplasms that fall under the umbrella of STS, including tumors that originate from cartilage, adipose tissue, skeletal muscle, or connective tissue, among many other tissue types. One particular type of high-grade aggressive STS is the undifferentiated pleomorphic sarcoma (UPS), formerly known as the malignant fibrous histiocytoma (MFH). There has historically been much debate about the classification and differentiation of UPS from other types of sarcomas, which has only recently been characterized by novel methods of immunohistochemistry markers and sophisticated cytogenetics. This has left much of the literature regarding UPS largely irrelevant in context of our current classification standards.A recent retrospective analysis of 100 UPS cases revealed an incidence of only 9% for UPS originating in the retroperitoneum. We present a case of UPS originating in the retroperitoneum. In this case, a patient suffering from a large right-sided intra-abdominal mass underwent complete surgical resection. We also demonstrate some of the complexities involved in the diagnosis and treatment of a rare retroperitoneal form of UPS.

Undifferentiated pleomorphic sarcoma (UPS) is an aggressive soft tissue sarcoma with a poor prognosis. The patients are usually found to have metastasis when the primary tumor is diagnosed. Eccrine syringofibroadenoma (ESFA) is a rare cutaneous adnexal lesion of eccrine duct origin. There are five subtypes, one of which is reactive ESFA, known to occur in reaction to an inflammatory or neoplastic process. In this article, we report a case of the co-existence of both UPS and ESFA in a 70-year-old male patient, presenting with a painless, erythematous, irregular surface nodule with a peripherally extended brownish hyperkeratotic plaque on the right palm. The histologic findings revealed an ill-defined dermal tumor of atypical epithelioid and spindle-shaped cells with large pleomorphic hyperchromatic nuclei and abundant eosinophilic cytoplasm. Some of those cells were multinucleated giant cells in the stroma with vascular proliferation and mixed inflammatory cell infiltrate. The tumor cells, which were only positive for vimentin, supported the diagnosis of undifferentiated pleomorphic sarcoma (UPS). Meanwhile, the overlying epidermis demonstrated hyperkeratosis, papillated epidermal hyperplasia, and proliferation of anastomosing slender cords and strands of cuboid cells within loose fibrovascular stroma. These findings are the characteristics of eccrine syringofibroadenoma (ESFA). We describe here a patient in whom reactive ESFA occurred on and surrounded the UPS tumor.

Undifferentiated pleomorphic cardiac sarcomas are extremely rare, highly malignant mesenchymal cardiac neoplasms typically presenting in the sixth decade of life. Here, we have reported a rare case of undifferentiated pleomorphic cardiac sarcoma presenting with dyspnea in a young male. On further evaluations, a large, ill-defined, multilobulated mass was observed in left atrium (LA) with encasement of left circumflex artery, attachment to mitral valve annulus, and invasion through LA wall and pericardium.

Undifferentiated pleomorphic sarcoma (UPS) formerly known as malignant fibrous histiocytoma is a high-grade aggressive soft-tissue sarcoma. UPS is a soft-tissue sarcoma without a definable line of differentiation. Men between the ages of 50 and 70 are frequently affected by it. In adults, the lower extremity and retroperitoneum are the most affected sites, while the paranasal sinus is affected in younger individuals. A 16-year-old male patient reported with swelling in his left posterior maxilla for 2 months. The swelling began quickly and gradually expanded to its current size. On examination, an ill-defined, deep-seated exophytic, ovoid growth was seen on the left maxillary alveolus. Histopathological findings indicated UPS. UPS is a rare malignancy that typically originates in the body’s soft tissues. The treatment for UPS is determined on the site of the cancer, but it typically includes surgery and radiation.

Undifferentiated pleomorphic sarcoma (UPS) is a rare type of tumor, and UPS originating in the adrenal gland is even rarer. Up to now, there have been no reports in English literature of UPS originating from the adrenal gland. This case report presents a 44-year-old female patient with UPS of the adrenal gland, who has shown no signs of recurrence or metastasis half a year after undergoing resection of a left adrenal tumor. A retrospective analysis of the patient’s diagnosis and treatment process is conducted, with the aim of providing a reference for the diagnosis and treatment of adrenal UPS.

Undifferentiated pleomorphic sarcoma is an extremely rare malignant thyroid tumor. Thyroid sarcoma differs from common malignant thyroid tumors, such as thyroid follicular cell carcinoma. It is usually highly malignant, progresses rapidly, and is prone to remote metastasis. Currently, there is no standard protocol for the treatment of thyroid sarcomas, and most treatment effects are unsatisfactory. Argon-helium cryoablation is an important method of local treatment that is widely used in patients with unresectable advanced tumors. However, owing to the low incidence of thyroid sarcomas, there are no relevant literature reports on the treatment of thyroid sarcomas using cryoablation in China. This study reports the case of a patient with undifferentiated pleomorphic sarcoma of the thyroid gland who was treated with argon-helium cryoablation, and the immediate outcome was good. Based on a review of relevant literature, we discussed the effectiveness and safety of argon-helium cryoablation treatment to provide clinical guidance and references for the treatment of patients with thyroid sarcoma.

Primary laryngeal sarcomas are rare. Their nomenclature and classification are similar to soft tissue counterparts; however, there are notable differences between clinical presentation, behavior, treatment, and follow-up. There is sparse information regarding the clinical features, biologic behavior, and treatment modalities of laryngeal sarcomas. To increase our understanding about these tumors, we describe herein an additional series of four cases of different pathologic types of laryngeal sarcomas, including low-grade chondrosarcoma, leiomyosarcoma, well-differentiated liposarcoma, and undifferentiated pleomorphic sarcoma. Our main aim is to upsurge awareness about the morphologic variations of laryngeal sarcomas, to avoid potential pitfalls during histopathologic examination. It is essential to ensure that correct diagnosis, subclassification, and grading are achieved for proper guidance of treatment and clinical follow-up at multidisciplinary team meetings.

Abstract Background Undifferentiated pleomorphic sarcoma is an uncommon sarcoma and its presence in the spleen is even rarer, with only a handful of cases reported in English literature. It is typically only diagnosed following histological analysis. Its rarity also means that there is little consensus over ideal management. Case presentation This report presents a case of a 40-year-old Caucasian male who was found to have a splenic mass after presenting with non-specific abdominal pain and generalized malaise. Numerous imaging modalities were used which demonstrated a large partially solid and partially cystic lesion in spleen with no evidence of metastasis. As core biopsies were undiagnostic, he was planned for a diagnostic and therapeutic splenectomy. However, despite magnetic resonance imaging 11 days prior to his operation showed no evidence of liver metastasis, a massive splenic tumour with hepatic metastases was identified intraoperatively. An open splenectomy, distal pancreatectomy and liver metastasectomy was hence carried out. Histological analysis confirmed liver metastasis secondary to a splenic undifferentiated pleomorphic sarcoma. The patient recovered well and was discharged home. He presented again three weeks after his operation with lower back pain, abdominal pain and fever. Computed tomography demonstrated extensive recurrent disease burden in the peritoneum and liver. The patient passed away a month after surgery. Conclusion Splenic undifferentiated pleomorphic sarcoma is a rare tumour which may pose a significant diagnostic challenge on both clinical and histopathological grounds. Following diagnosis and treatment, its aggressive nature often results in a poor prognosis. Current literature fails to delineate any superior management strategy to increase survival.

A 52-year-old male had pain in the right back and right hypochondrium, and an abdominal CT scan revealed a 49-mm tumor in the right upper perirenal space. Additional MRI and PET-CT suggested that the tumor may be a primary adrenal carcinoma and could invade the liver and diaphragmatic leg. The tumor was completely removed by laparotomy and histopathologically diagnosed as retroperitoneal primary undifferentiated pleomorphic sarcoma. The patient has remained recurrence-free for 1.5 years after the surgery.

Positive regulatory domain member (PRDM) proteins play a critical role in the transmission of signals that control cell proliferation and differentiation, and neoplastic transformation. Positive regulatory domain member 10 (tristanin) is a poorly studied member of PRDM protein family. Gene fusion transcripts containing PRDM10 were recently identified in low-grade undifferentiated pleomorphic sarcomas (UPS), and associated with pleomorphic morphology and low mitotic index. The aim of this study was to investigate the immunohistochemical staining of PRDM10 in a larger sample of soft tissue sarcomas. Therefore, the study included 118 soft tissue sarcomas from different classes, and PRDM10 antibody was applied to all of them. Immuno-histochemically, staining was observed in 22 (19%) cases, while 96 (81%) showed no staining. When PRDM10 expression was compared with clinico-pathological features, there was a statistically significant correlation between PRDM10 expression and myxoid changes, multi-nucleated giant cells, and surgical margin (p = 0.017, p = 0.034, p = 0.032, respectively). No statistically significant association was found between PRDM10 expression and other parameters. Based on the obtained data, it can be said that PRDM10-positive-stained tumors (tumors with PDRM10 expression) are mostly myxoid, containing multi-nucleated giant cells, and can be removed with well-circumscribed margins.

Abstract Background Undifferentiated pleomorphic sarcoma (UPS) in the esophagus is extremely rare. Therefore, there are few reports of UPS in the esophagus (UPSE). We present a case of UPSE after multiple cancer treatments. Case presentation A 73-year-old man with a history of cancer treatment, including distal gastrectomy, transverse colectomy, and chemoradiotherapy, was diagnosed with an elevated lesion such as a submucosal tumor in the lower esophagus by regular endoscopy. A boring biopsy was performed, and the specimen showed features of sarcoma. The patient underwent a partial esophagectomy without lymph node dissection. Histopathological findings confirmed an undifferentiated pleomorphic sarcoma. Adjuvant therapy was not administered, and the patient survived without recurrence 1 year after surgery. Conclusions Currently, complete resection is the only treatment option for UPSE. An optimal treatment strategy using chemotherapy or radiotherapy should be established.

Abstract Background Undifferentiated pleomorphic sarcoma (UPS) is considered one of the most common types of soft tissue sarcoma (STS). Current studies have shown that the prognosis of UPS is related to some of its clinical characteristics, but no survival prediction model for the overall survival (OS) of UPS patients has been reported. The purpose of this study is to construct and validate a nomogram for predicting OS in UPS patients at 3, 5 years after the diagnosis. Methods According to the inclusion and exclusion criteria, 1079 patients with UPS were screened from the SEER database and randomly divided into the training cohort (n = 755) and the validation cohort (n = 324). Patient demographic and clinicopathological characteristics were first described, and the correlation between the two groups was compared, using the Kaplan–Meier method and Cox regression analysis to determine independent prognostic factors. Based on the identified independent prognostic factors, a nomogram for OS in UPS patients was established using R language. The nomogram’s performance was then validated using multiple indicators, including the area under the receiver operating characteristic curve (AUC), consistency index (C-index), calibration curve, and decision curve analysis (DCA). Results Both the C-index of the OS nomogram in the training cohort and the validation cohort were greater than 0 .75, and both the values of AUC were greater than 0.78. These four values were higher than their corresponding values in the TNM staging system, respectively. The calibration curves of the Nomogram prediction model and the TNM staging system were well fitted with the 45° line. Decision curve analysis showed that both the nomogram model and the TNM staging system had clinical net benefits over a wide range of threshold probabilities, and the nomogram had higher clinical net benefits than the TNM staging system as a whole. Conclusion With good discrimination, accuracy, and clinical practicability, the nomogram can individualize the prediction of 3-year and 5-year OS in patients with UPS, which can provide a reference for clinicians and patients to make better clinical decisions.

Introduction Retroperitoneal tumors with endocrine abnormalities are suspected to be functional adrenal tumors. Retroperitoneal soft tissue sarcomas are rare tumors, without endocrine potential. Case presentation A 60‐year‐old male was referred for a 15 cm mass in the left suprarenal space. His plasma cortisol and catecholamine levels were elevated. He underwent open left adrenalectomy with radical nephrectomy and his endocrinological abnormalities were improved. Pathological findings suggested that it had originated from the retroperitoneal fat tissue, and a diagnosis of undifferentiated pleomorphic sarcoma was made based on the results of immunohistochemical analysis and fluorescence in situ hybridization. Interestingly, neither cortisol nor catecholamine was elevated when, 6 months after surgery, local recurrence developed. Conclusion This is the first reported case of undifferentiated pleomorphic sarcoma accompanied by high levels of cortisol and catecholamine. We should keep in mind the possibility of tumors like retroperitoneal soft tissue sarcomas inducing endocrine abnormalities.

Abstract Background Undifferentiated pleomorphic sarcoma (UPS), also known as malignant fibrous histiocytoma (MFH), hardly originates from the colorectum. Case presentation We reported a 65-year-old female presented with UPS in the descending colon. Computed tomography (CT) revealed an irregularly thickened descending colon. On colonoscopy examination, an ulcerative tumour was identified. The patient received radical resection of the left colon and partial enterectomy. The resected tumor was ulcerative, 10 cm × 8 cm × 5 cm in size, and infiltrated the serosa layer. Postsurgical pathology showed that the tumor was high-graded UPS in the colon with large amounts of necrotic tissues. Conclusions UPS in the large intestine is a rare malignant tumor with a poor prognosis and unknown pathogenesis. The main treatment for UPS is early complete resection. Postsurgery adjuvant radiotherapy or chemotherapy can be attempted.

Undifferentiated pleomorphic sarcoma (UPS) is a rare and aggressive soft tissue tumor with a high degree of malignancy and rapid progression, usually occurring in the extremities, retroperitoneum, and abdomen, whereas it rarely arises in the mediastinum, and is treated mainly by surgical resection. The prognosis of patients with advanced sarcoma is poor, and doxorubicin monotherapy is the standard first-line chemotherapy for most advanced soft tissue sarcomas (STS), but the prognosis is generally unsatisfactory. Immune checkpoint inhibitors (ICIs) have been established as therapies for many solid cancers in recent years; however, evidence on the efficacy of ICIs in undifferentiated sarcoma is scarce, mostly consisting of small studies, and no ICIs are currently approved for use in sarcomas. We report a case of a middle-aged man with primary mediastinal UPS with high PD-L1 expression (TPS was approximately 80%) and TLS positive. The patient was treated with sequential tislelizumab monotherapy maintenance after 6 cycles of tislelizumab combined with epirubicin, efficacy evaluation was partial remission (PR), progression-free survival (PFS) was 8.5 months, and grade 1 fatigue was identified as an adverse event.

Abstract Background Primary undifferentiated pleomorphic sarcoma (UPS) of the pancreas is an exceedingly rare malignant tumor, with only 15 cases have been reported in the medical literature. At present, clinicians have poor recognition of the tumor, the epidemiology, diagnosis, and treatment of this disease have yet not been established. Case presentation In this report, we depict the clinical and imaging characteristics of a 37-year-old man presenting with a primarily cystic UPS. The patient complained of epigastric pain and distention over 20 days. Abdominal CT and pancreatic magnetic resonance imaging revealed cystic and cystic solid masses in the pancreatic body and tail. An abdominal ultrasound echogram revealed the mass in the body of the pancreas to be cystic with separation echo inside, and the wall was thick, not smooth. Besides, a hypoechoic mass was seen in the tail area of the pancreas with an inhomogeneous echoic pattern, containing small patches of no echo zone in the central. Microscopically, spindle fibroblast-like cells are arranged in a characteristic storiform pattern with pleomorphic and multinucleated cells. Immunohistochemically, tumor cells were positive for CD68 and vimentin. Seven months postoperatively, he was diagnosed with pulmonary lymph node metastasis and died 5 months later. Combined with this case report, we also reviewed the literature regarding UPS of the pancreas. Conclusions As we know, this is the first report on ultrasonography findings of pancreatic UPS. Despite there are no distinctive manifestation of UPS, a solid cystic lesion on ultrasonography or a hypodense area in the lesion on T2-weighted imaging, should be considered for differential diagnosis with pancreatic UPS. We believe this article may add some ideas into the diagnosis and therapy of patients with this tumor.

Undifferentiated pleomorphic sarcoma (UPS), once termed as malignant fibrous histiocytoma, has always been diagnosed exclusively in clinical practice because it lacks any defined resemblance to normal mesenchymal tissue. Although myxofibrosarcoma (MFS) has been separated from UPS due to its fibroblastic differentiation with myxoid stroma, UPS and MFS are still identified as a sarcoma group in terms of molecular landscapes. In this review article, we will describe the associated genes and signaling pathways involved in the process of sarcoma genesis and make a summary of conventional management, targeted therapy, immunotherapy, and some novel potential treatments of UPS/MFS. With the progressive advancements in medical technology and a better understanding about the pathogenic mechanism of UPS/MFS in the coming decades, new lights will be shed on the successful management of UPS/MFS.

IntroductionSoft tissue sarcomas (STS) are highly metastatic, connective-tissue lineage solid cancers. Immunologically, sarcomas are frequently characterized by a paucity of tumor infiltrating lymphocytes and an immune suppressive microenvironment. Activation of the STING pathway can induce potent immune-driven anti-tumor responses within immunogenic solid tumors; however, this strategy has not been evaluated in immunologically cold sarcomas. Herein, we assessed the therapeutic response of intratumoral STING activation in an immunologically cold murine model of undifferentiated pleomorphic sarcoma (UPS).Materials and ResultsA single intratumoral injection of the murine STING agonist, DMXAA resulted in durable cure in up to 60% of UPS-bearing mice. In mice with synchronous lung metastases, STING activation within hindlimb tumors resulted in 50% cure in both anatomic sites. Surviving mice all rejected UPS re-challenge in the hindlimb and lung. Therapeutic efficacy of STING was inhibited by lymphocyte deficiency but unaffected by macrophage deficiency. Immune phenotyping demonstrated enrichment of lymphocytic responses in tumors at multiple timepoints following treatment. Immune checkpoint blockade enhanced survival following STING activation.DiscussionThese data suggest intratumoral activation of the STING pathway elicits local and systemic anti-tumor immune responses in a lymphocyte poor sarcoma model and deserves further evaluation as an adjunctive local and systemic treatment for sarcomas.

Jiaqiang Wang,1 Shuping Dong,1 Jianpo Zhang,2 Shilei Gao,1 Zhehuang Li,1 Po Li,1 Junhui Yuan,3 Zhichao Tian1 1Department of Bone and Soft Tissue, the Affiliated Cancer Hospital of Zhengzhou University and Henan Cancer Hospital, Zhengzhou, Henan Province, 450008, People’s Republic of China; 2Pathology Department, the Affiliated Cancer Hospital of Zhengzhou University and Henan Cancer Hospital, Zhengzhou, Henan Province, 450008, People’s Republic of China; 3Medical Imaging Department, the Affiliated Cancer Hospital of Zhengzhou University and Henan Cancer Hospital, Zhengzhou, Henan Province, 450008, People’s Republic of ChinaCorrespondence: Zhichao TianDepartment of Bone and Soft Tissue, the Affiliated Cancer Hospital of Zhengzhou University and Henan Cancer Hospital, Dongming Road, Zhengzhou, Henan Province, 450008, People’s Republic of ChinaTel +86-13592413731Fax +86-371-65961505Email tianzhichaoyy@163.comBackground: Although the annual incidence of undifferentiated pleomorphic sarcoma (UPS) is extremely low, it can be subdivided into different subtypes. UPS with fever of unknown origin (also known as neoplastic fever) is a specific subtype of UPS, which shows certain clinical features that differentiate it from other UPS subtypes. However, no studies have focused on this rare UPS subtype. This study retrospectively analyzed the clinical data of patients with UPS to provide a reference for the diagnosis and treatment of UPS with neoplastic fever.Methods: This study included patients with UPS who were diagnosed and treated between June 2012 and June 2018. We examined whether these patients had a history of neoplastic fever. The characteristics of patients with UPS with neoplastic fever were summarized and analyzed.Results: We reviewed the medical records of 183 patients with UPS. Seven (3.83%) of these patients had neoplastic fever. In patients with UPS with neoplastic fever, the primary lesions were located in the extremities and across the muscle space. In these patients, magnetic resonance imaging showed necrosis within the tumor body and extensive soft tissue edema around the tumor body. Patients with UPS with neoplastic fever had a lower metastasis rate (14.29% vs 44.94%) and a higher 3-year survival rate (85.71% vs 59.55%) than those without neoplastic fever.Conclusion: UPS with neoplastic fever is characterized by intratumoral necrosis and extensive edema of the surrounding soft tissues. Patients with UPS with neoplastic fever may have a better prognosis than those without neoplastic fever.Keywords: undifferentiated pleomorphic sarcoma, neoplastic fever, fever of unknown origin, sarcoma

Background: Chemoimmunotherapy is safe and efficacious in treating many types of malignant tumors. However, clinical data demonstrating the effect of this combination treatment in patients with metastatic soft tissue sarcoma (STS) are currently limited. This study evaluated the safety and efficacy of a programmed cell death protein 1 (PD-1) inhibitor plus doxorubicin in patients with advanced STS who failed previous systemic therapy.Methods: This was a single-center, single-arm, open-label phase II trial. Patients with unresectable or metastatic STS who had previously failed systemic therapy were enrolled. Patients received up to six cycles of doxorubicin and sintilimab (a PD-1 inhibitor), while sintilimab treatment continued for up to 2 years. Primary outcomes were objective response rate (ORR) and safety. Univariate Cox proportional hazards model was used to analyze the relationship between clinicopathological parameters and progression-free survival (PFS).Results: A total of 38 patients (20 men and 18 women) were enrolled in this study. The overall ORR was 39.5%, disease control rate was 71.1%, and the median PFS was 4.5 months [95% confidence interval (CI), 3.0–8.5 months]. The adverse events (AEs) associated with the combined treatment were mild, manageable, and well-tolerated. The most common grade 3 or higher AEs were hematologic, including leukopenia (21.1%), anemia (18.4%), and thrombocytopenia (18.4%). Patients with undifferentiated pleomorphic sarcoma (UPS) or dedifferentiated liposarcoma had a significantly longer PFS than those with other pathological subtypes [hazard ratio (HR) = 0.42, 95% CI 0.21–0.83; p = 0.013]. There was no significant difference in the median PFS between patients who had previously received anthracycline-based chemotherapy and those who had not (HR = 0.74, 95% CI 0.34–1.58, p = 0.43).Conclusion: Sintilimab plus doxorubicin is a safe and promising treatment for patients with advanced STS who have failed previous systemic therapy (including anthracycline-based chemotherapy). The efficacy of this combination therapy in UPS and dedifferentiated liposarcoma is superior to that in other sarcomas.Clinical Trial Registration:https://www.chictr.org.cn, registration number: ChiCTR1900027009.

Undifferentiated pleomorphic sarcoma (UPS), a rare soft tissue sarcoma subtype, mainly occurs in the deep parts of the limbs and trunk, observed as rapidly growing painless lumps, rarely located under the skin or protrudes from the skin surface. The risk of recurrence and metastasis is associated with multiple factors. Mutation of tumor gene, tumor occurrence, location and depth of invasion, and tumor size have great influence on prognosis. In this study, we described a case of UPS with KRAS/NF1 co-mutation. This case had undergone UPS extended resection for four times combined with chemotherapy in another hospital. The resection area was more than 3 cm, and tumor relapsed after all operations. This time, the tumor protruded from the left lateral surface with ulceration and infection. Due to multiple surgeries, the anatomy of the lateral femoral vessels has been seriously damaged. We performed expanded tumor resection and adjacent flap transfer repair; meanwhile, vacuum sealing drainage (VSD)-negative pressure closed the drainage, and the patient recovered well after surgery. After surgery, the patient was transferred to the Department of Oncology for chemotherapy. There was no recurrence after 6 months of follow-up. Gene mutation plays an important role in UPS recurrence and metastasis. At the same time, occurrence and location, depth, and size of UPS have great influence on the prognosis. Deeper exploration and expanded resection are important for the prognosis of UPS.

IntroductionUndifferentiated pleomorphic sarcoma (UPS) can be associated with a relatively dense immune infiltration. Immune checkpoint inhibitors (anti-PD1, anti-PDL1, and anti-CTLA4) are effective in 20% of UPS patients. We characterize the immune microenvironment of UPS and its association with oncologic outcomes.Material and methodsSurgically resected UPS samples were stained by immunohistochemistry (IHC) for the following: tumor-associated immune cells (CD3, CD8, CD163, CD20), immune checkpoints (stimulatory: OX40, ICOS; inhibitory: PD-L1, LAG3, IDO1, PD1), and the adenosine pathway (CD73, CD39). Sections were reviewed for the presence of lymphoid aggregates (LA). Clinical data were retrospectively obtained for all samples. The Wilcoxon rank-sum and Kruskal-Wallis tests were used to compare distributions. Correlations between biomarkers were measured by Spearman correlation. Univariate and multivariate Cox models were used to identify biomarkers associated with overall survival (OS) and disease-free survival (DFS). Unsupervised clustering was performed, and Kaplan-Meier curves and log-rank tests used for comparison of OS and DFS between immune clusters.ResultsSamples analyzed (n=105) included 46 primary tumors, 34 local recurrences, and 25 metastases. LA were found in 23% (n=10/43), 17% (n=4/24), and 30% (n=7/23) of primary, recurrent, and metastatic samples, respectively. In primary UPS, CD73 expression was significantly higher after preoperative radiation therapy (p=0.009). CD39 expression was significantly correlated with PD1 expression (primary: p=0.002, recurrent: p=0.004, metastatic: p=0.001), PD-L1 expression (primary: p=0.009), and CD3+ cell densities (primary: p=0.016, recurrent: p=0.043, metastatic: p=0.028). In recurrent tumors, there was a strong correlation between CD39 and CD73 (p=0.015), and both were also correlated with CD163+ cell densities (CD39 p=0.013; CD73 p

Background: Primary cardiac undifferentiated pleomorphic sarcomas (UPS) are very rare entities with a myriad of potential presentations. Multiorgan embolism can result in significant morbidity and mortality in the absence of early recognition and timely intervention. This case report describes a patient who received treatment for the abovementioned pathology and a literature review of available evidence on primary cardiac neoplasms was performed. Case presentation: This case is a 62-year-old female with cardioembolic stroke due to a left atrial mass. On hospital day 10 prior to operative intervention, the patient developed renovisceral and bilateral common iliac artery thromboemboli leading to acute limb ischemia and underwent bilateral femoral cutdown with aortoiliac thromboembolectomy. There was no mesenteric ischemia. Histopathological assessment of the emboli revealed primary cardiac undifferentiated pleomorphic sarcoma. She developed a catastrophic hemorrhagic stroke while on therapeutic anticoagulation which led to withdrawal of care. Conclusion: The literature review and case report demonstrate the need for timely intervention, judicial use of anticoagulation and histological assessment of emboli in cases of cardioembolic phenomena from an underlying cardiac neoplasm.

Abstract Background Chordoma is a rare bone tumor that is typically resistant to chemotherapy and is associated with genetic abnormalities of the T-box transcription factor T (TBXT) gene, which encodes the transcription factor brachyury. Brachyury is felt to be a major contributor to the development of chordomas. Case presentation We describe a 67-year-old woman who developed an undifferentiated pleomorphic sarcoma in her thigh. Despite treatment with standard chemotherapy regimens, she had a rapidly progressive course of disease with pulmonary metastases and passed away 8 months from diagnosis with pulmonary complications. Her medical history was remarkable in that she had a spheno-occipital chordoma at age 39 and later developed multiple other tumors throughout her life including Hodgkin lymphoma and squamous cell carcinoma and basal cell carcinoma of the skin. She had a family history of chordoma and her family underwent extensive genetic study in the past and were found to have a duplication of the TBXT gene. Conclusions Brachyury has been found to associate with tumor progression, treatment resistance, and metastasis in various epithelial cancers, and it might play roles in tumorigenesis and aggressiveness in this patient with multiple rare tumors and germ line duplication of the TBXT gene. Targeting this molecule may be useful for some malignancies.

Abstract Undifferentiated pleomorphic sarcoma (UPS) is a new disease in the World Health Organization's classification of tumors of soft tissue and bone published in 2013. Primary mediastinal UPS is rare, especially with pleural effusion. Herein, we describe the pathological findings of pleural effusion followed by mediastinal UPS, which was initially misdiagnosed as epithelial malignant pleural mesothelioma (MPM). The cytopathological findings of the pleural effusion cell block often contribute to the diagnosis of various malignant tumors. However, these findings may lead to misdiagnosis of highly invasive mediastinal tumors such as UPS. A biopsy for primary mediastinal lesions should be performed because MPM rarely mimics mediastinal tumors with pleural effusion.

Pleomorphic sarcoma of the larynx is a rare variant of laryngeal cancer. We present the case of a 59-year-old male patient who has been smoking for 40 years. He presented with signs and symptoms of an obstructive glottic mass. The diagnostic workup pointed to a malignant pathology; the histopathology report confirmed the diagnosis of glottic undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma).

BackgroundUndifferentiated pleomorphic sarcoma (UPS) is a malignant tumor that originates in the mesenchymal tissue and is common in the extremities and retroperitoneum. Primary UPS of the duodenal papilla is rare and a distinct clinical entity.Case presentationIn this report, a 48-year-old Chinese man was admitted to our hospital with symptoms of melena. The patient underwent choledochectomy and choledochaljejunostomy for obstructive jaundice 8 years before admission. Endoscopic examination after admission confirmed a mass located at the duodenal papilla. Then, the duodenal papilla and tumor resection were performed, and the histopathology report confirmed the diagnosis of UPS. The patient refused further treatment and died 2 months later due to local recurrence and intrahepatic metastasis.ConclusionsIt is rare that the mass in the duodenal papilla is diagnosed as UPS. The unpredicted behavior of these tumors warrants a careful plan considering their indolent nature and possible recurrence and metastasis. The prognosis was poor despite the early complete resection.

Aortic spindle cell sarcoma is a rare neoplasm with poor prognosis that is often found incidentally due to its adverse effects. CT and MRI with contrast are useful imaging modalities, but a tissue biopsy is the gold standard for diagnosis. Tumor resection is the ultimate treatment followed by chemotherapy. Our case was an adult female who presented mainly for shortness of breath, and further imaging workup demonstrated a soft tumor juxtaposed to a major vein with compressive effect. The patient’s tumor was resected, and the pathology result confirmed undifferentiated aortic sarcoma. The patient’s condition improved and she was discharged with outpatient oncology follow-up and possible treatment.

Introduction Prostate sarcoma is an extremely rare disease with a poor prognosis. Undifferentiated pleomorphic sarcoma has never been described in the prostate. Case presentation A 27‐year‐old man complained of frequent urination and dysuria for several years. Various examinations were suggestive of prostate sarcoma. The pathological diagnosis was confirmed as prostate sarcoma via ultrasound‐guided transrectal needle biopsy. Because the location of the tumor in the prostate was confirmed by magnetic resonance imaging, we performed robot‐assisted radical prostatectomy. The final pathological diagnosis was undifferentiated pleomorphic sarcoma. Local recurrence occurred at the front of the rectum 2 months after surgery. Although chemotherapy and radiotherapy were initially effective, he died 18 months after surgery. Conclusion Undifferentiated pleomorphic sarcoma of the prostate is believed to have a poor prognosis. When selecting the surgical procedure, functionality should be considered for individual cases with complete resection.

Abstract This report describes a patient who presented with a large thyroid nodule and compressive symptoms. Immunohistochemical staining showed diffuse marked reactivity for vimentin and focal reactivity for CD68 and Ki‐67 that is compatible with primary undifferentiated pleomorphic sarcoma of the thyroid. This report emphasizes and discusses extremely rare thyroid cancer type.

Objective: Undifferentiated pleomorphic sarcoma (UPS) is a highly malignant, aggressive, and pleomorphic subtype of soft tissue sarcoma in adults. However, UPS is difficult to be diagnosed due to the lack of specific morphological and immunophenotypic features. Here, we aimed to identify new biomarkers for the diagnosis of UPS.Methods: The mRNA and protein expression of neurofibromin 1 (NF1) in 68 pairs of UPS and adjacent normal tissues were detected by qRT-PCR and immunohistochemistry, and the correlation between the NF1 protein expression and clinicopathological characteristics was analyzed. Then, differentially expressed microRNAs (DE miRNAs) were identified between the UPS tumor tissue and matched adjacent normal tissue using Hisep sequencing, Gene Ontology (GO), and Kyoto Encyclopedia of Genes and Genomes (KEGG). The DE miRNAs of the regulating NF1 gene were also identified using the TargetScan and miRanda databases and validated by qRT-PCR.Results: Compared with the adjacent normal tissue, both mRNA and protein expressions of NF1 in the UPS tumor tissue were significantly decreased, and the positive rate of NF1 protein was associated with the tumor size, metastasis, and recurrence. A total of 125 known DE miRNAs were identified from the screened miRNAs based on | log2(Fold Change) ≥5 and p-value < 0.05 (A total of 82 upregulated and 43 downregulated DE miRNAs in the UPS tissue). Target genes regulated by the DE miRNAs were enriched in pathways of metabolisms, RNA degradation, PI3K-Akt, and Rap1 pathway. In total, 11 miRNAs which were predicted to regulate the NF1 gene were screened. After verification, the relative expressions of hsa-miR-199a-3p and hsa-miR-34a-5p were increased and decreased in the UPS tumor tissue compared with those in the adjacent normal tissue, respectively.Conclusion: NF1 and NF1-related microRNAs including hsa-miR-199a-3p and hsa-miR-34a-5p may be novel biomarkers in the diagnosis of undifferentiated pleomorphic sarcoma (UPS).

PurposeOur previously study showed that recombinant human endostatin (Endostar) combined with chemotherapy had significant activity to increase the mPFS in patients with advanced sarcomas with tolerable side effects. However, the small cohort size and short follow-up time made it difficult to screen sensitive sarcoma subtypes and determine whether there is an overall survival benefit. With the largest sarcoma cohort to our knowledge, we try to confirm the efficacy and safety of chemotherapy combined with Endostar in stage IV sarcomas, with the specific purpose of finding out the sensitive sarcoma types for this combined treatment.MethodsAfter the exclusion of ineligible patients, 156 patients with stage IV bone and soft tissue sarcomas were included in this study according to the inclusion criteria.ResultsBy the end of follow-up, the ORR was 10.7% (9/84) vs 1.4% (1/72) (p=0.041), the DCR was 26.2% (22/84) vs 5.6% (4/72) (p=0.001) in the combined group and chemotherapy group, respectively. The mPFS of combined group was significantly longer than the chemotherapy group (10.42 vs 6.87 months, p=0.003). The mOS were 26.84 months and 23.56 months, without significant difference (p= 0.481). In osteogenic sarcoma, there was no statistically significant difference in the mPFS between the two groups (p=0.59), while in the soft tissue sarcoma, the mPFS in the combined group was significantly higher than that of the chemotherapy group (11.27 vs 8.05 months, p=0.004). Specifically, undifferentiated polymorphic sarcoma (UPS) was the possible sarcoma subtypes that benefited from the combined therapy. For the 38 UPS patients (28 patients in the combined group and 10 patients in the chemotherapy group), the mPFS in the combined group was up to 14.88 months, while it was only 7.1 months in the chemotherapy group, with a significant difference (p=0.006). The most common adverse events in the combined group were myelosuppression, gastrointestinal reactions and abnormal liver function, without significant difference in two groups.ConclusionChemotherapy plus Endostar could prolong mPFS and improve ORR and DCR in patients with stage IV soft tissue sarcoma, suggesting that the combined therapy could improve the patient prognosis in soft tissue sarcomas, especially the UPS patients.

Pleomorphic sarcomas are extremely rare in the oral cavity, representing only 5% of all soft tissue sarcomas in the extremities of adults. They form a group of highly undifferentiated pleomorphic malignant tumors. A 36-year-old male reported to the OP with a chief complaint of a tooth ache in the right lower quadrant for the past 6 months. The patient gave a history of extraction of a mobile tooth in that region. A radiograph of the region was advised. An incisional biopsy was also done and sent for histopathological examination. The microscopy revealed the presence of spindle-shaped cells showing dysplastic features. A panel of immunohistochemical markers were performed to identify the tissue of origin of the lesion. The article details the clinical, radiographic, and microscopic features of a pleomorphic soft tissue lesion along with the array of immunohistochemical markers, leading to the final diagnosis of such enigmatic lesions.

Abstract Local control for the treatment of primary bone tumors is generally delayed following neoadjuvant chemotherapy. This was born out of the historical need to manufacture custom implants when performing limb-salvage resection. There is increasing reason to reconsider the timing of local control in the setting of primary bone tumors. In this report, we describe two cases in which upfront surgery was utilized and review rationale, prior literature, and potential benefits of this approach.

Abstract Primary UPS of the lung, so‐called MFH, is a rare aggressive neoplasm and known to be a high risk of recurrence and metastasis. Surgical resection without residual tumor is the main option of treatment and the best chance for long‐term survival.

Shiqi Chen,1,2 Wending Huang,1,2 Peng Luo,1,2 Weiluo Cai,1,2 Lingge Yang,1,2 Zhengwang Sun,1,2 Biqiang Zheng,1,2 Wangjun Yan,1,2 Chunmeng Wang1,2 1Department of Musculoskeletal Oncology, Fudan University Shanghai Cancer Center, Shanghai 200032, People’s Republic of China; 2Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, People’s Republic of ChinaCorrespondence: Chunmeng Wang; Wangjun YanDepartment of Musculoskeletal Oncology, Fudan University Shanghai Cancer Center, 270 Dong’an Road, Xuhui District, Shanghai 200032, People’s Republic of ChinaEmail cmwang1975@163.com; yanwj@fudan.edu.cnPurpose: Our study aimed to describe the clinical features of undifferentiated pleomorphic sarcoma (UPS) and identify the predictors of poor outcomes.Patients and methods: The clinicopathological variables and treatment strategies of 100 UPS patients who underwent surgical resections at a single institution between November 2004 and July 2016 were reviewed. Kaplan–Meier and Cox regression method were conducted for survival analysis.Results: The median follow-up time was 94 months (range, 1.5–154 months). R0 resection was applied for 72 cases, and the median tumor size was 5.75cm (range, 1–30cm). Tumor grades of 45 patients were intermediate grade (G2), and 54 patients were with advanced stage (stage III/IV). Twenty-seven patients presented with tumors involving important structures, in which the nerve was the most frequently invaded structure (n=12). During the follow-up, 40 patients suffered from postoperative local recurrence, and distant metastasis was observed in 25 patients which mainly metastasized to the lung (n=14). The 5-year OS rate, 5-year LRFS rate, and 5-year MFS rate was 53%, 55%, and 70%, respectively. Multivariate analysis revealed that tumor presentation, tumor size, and important structures involved (p=0.033, p=0.004, and p=0.033, respectively) were independent prognostic factors associated with OS. Meanwhile, age, resection quality and tumor grade were independent prognostic factors for LRFS (p=0.033, p=0.045, and p=0.007, respectively) and tumor depth was significantly associated with MFS (p=0.050) in multivariate analysis.Conclusion: Primary treatment of UPS should be conducted by experts in large sarcoma center. Wide surgical margin provides sufficient control of the disease recurrence.Keywords: undifferentiated pleomorphic sarcoma, Asian, long-term follow-up, prognostic factor

Background: Primary chest wall sarcoma is a rare disease with limited reports of surgical resection. Methods: This retrospective review included 41 patients with primary chest wall sarcoma who underwent chest wall resection and reconstruction from 2001 to 2015. The clinical, histologic, and surgical variables were collected and analyzed by univariate and multivariate Cox regression analyses for overall survival (OS) and re-currence-free survival (RFS). Results: The OS rates at 5 and 10 years were 73% and 61%, respectively. The RFS rate at 10 years was 57.1%. Multivariate Cox regression analysis revealed old age (hazard ratio [HR], 5.16; 95% confidence interval [CI], 1.71–15.48) as a significant risk factor for death. A surgical resection margin distance of less than 1.5 cm (HR, 15.759; 95% CI, 1.78–139.46) and histologic grade III (HR, 28.36; 95% CI, 2.76–290.87) were independent risk factors for recurrence. Conclusion: Long-term OS and RFS after the surgical resection of primary chest wall sarcoma were clinically acceptable.

Abstract Background Primary sarcoma of the lung is a very rare malignant tumor accounting for less than 0.5% of all lung tumors and presenting diagnostic and treatment challenge. We describe a case of a patient diagnosed with primary lung undifferentiated pleomorphic sarcoma developing subsequent peritoneal and small bowel metastases, which stand for highly unusual disease presentation. Case presentation A 57-year-old male presented with extensive partially necrotic tumor in the left upper lobe (LUL) of the lung that involved LUL bronchus and extended to the visceral pleura. There was no evidence of nodal or visceral dissemination. After initial presentation, the patient was admitted to the hospital’s pulmonology department for further workup. The most likely diagnosis based on biopsy specimen was poorly differentiated sarcoma. Left pneumonectomy with mediastinal lymph node dissection was performed. The final pathohistological diagnosis (PHD) was undifferentiated pleomorphic sarcoma (UPS). Three months after lung surgery, a follow-up CT scan was done which showed a 60-mm obstructive metastatic intraabdominal lesion with small bowel infiltration and further separate peritoneal deposits. Unfortunately, an urgent surgery had to be performed as the patient developed signs of acute abdomen due to bowel perforation. Only 2 months later, the patient passed away at home. Conclusions Treatment options of UPS are based on algorithms used in treatment of extremity lesions with well-established role of surgery. However, the role of perioperative chemotherapy remains equivocal with no strong evidence-based data due to the rarity of the disease. Small bowel is an unexpected metastatic site, but of significant clinical relevance.

Abstract Background Undifferentiated pleomorphic sarcoma (UPS) as a primary cardiac tumor is rare, with extremely poor prognosis owing to high recurrence and invasion. We encountered a patient who presented with a primary cardiac tumor incarcerating the mitral valve and who was in a shock state. Case presentation A 41-year-old man was transported emergently to our hospital owing to acute respiratory distress and hemoptysis. He was diagnosed with acute left heart failure caused by a large mass in the left atrium (LA) that obstructed cardiac blood flow, as revealed by imaging study findings, and he underwent an emergency open-heart surgery for tumor resection. He was pathologically diagnosed with UPS invading the muscle layer of the LA. However, after receiving combined therapy for local recurrence and distant metastasis, including proton beam radiotherapy and chemotherapy with molecularly targeted drugs, he could return to work for 2 more years after surgery. Conclusion In this study, we reported the case of a patient who was in a state of shock state owing to the presence of UPS in the LA. The patient underwent an emergency surgery and received combined therapy. He survived for 2 more years after an initial diagnosis, without active local recurrence and distant metastasis.

Abstract Background Undifferentiated pleomorphic sarcoma is a very rare and aggressive type of primary cardiac tumors. Most cardiac sarcomas result in rapid growth and quick death. According to different sources the median survival is typically 6 to 12 months. We are presenting a case of primary cardiac sarcoma with 26 months disease free survival following cytoreductive surgery and chemotherapy. Case presentation A 48-year-old woman with progressing symptoms of dyspnea and palpitations for over 2 months was referred to a cardiologist. With the help of echocardiography and cardiovascular magnetic resonance cardiac sarcoma was suspected. Open biopsy and cytoreductive surgery were performed, complete resection of the tumor was not possible. Histology revealed undifferentiated pleomorphic sarcoma. Seven cycles of chemotherapy with Doxorubicine and Ifosfamide were completed. Cardiovascular magnetic resonance revealed a complete response – only signs of fibrosis without any signs of tumor were visible. Follow ups with echocardiography, cardiovascular magnetic resonance and chest, abdomen and pelvic computed tomography is performed every 3 months. Twenty-six months from initial diagnosis the patient is still free of recurrence of tumor with no compromises of the quality of life. Conclusion Standard chemotherapy together with cytoreductive surgery can have a complete response effect in undifferentiated pleomorphic sarcoma with unusual long-term survival.

We retrospectively analyzed the diagnosis and treatment process of one patient with recurrent undifferentiated pleomorphic sarcoma (UPS) of infratemporal fossa and made a definite diagnosis by combining the imaging and pathological examination results. After treatment failure with 2 cycles of chemotherapy and several surgeries, UPS was eventually treated by surgery + carbon ion radiotherapy, and MRI reexamination showed no relapse. Head and neck UPS is located deeply, easily recurs after operation, and difficult to be resected completely by surgery, with a gradually shortened interval of relapse over the number of surgeries, which becomes a treatment challenge. After the last surgery, the patient received carbon ion radiotherapy, with a good therapeutic effect, and no sign of relapse just before sending this article. Based on the above advantages, we have concluded that surgery + carbon ion radiotherapy is a new effective pathway to treat head and neck UPS.

Cardiac undifferentiated pleomorphic sarcoma (UPS) associated with fever and inflammatory response is an extremely rare condition. Herein, we report a rare case of cardiac UPS with unusual clinical presentation and inflammatory response. A 67-year-old male complaining of progressive dyspnea and intermittent fever of unknown cause was referred to our hospital for surgical resection of a left atrial mass. Laboratory analysis showed leukocytosis (26 × 103/μL) and high C-reactive protein (CRP) levels (155.4 mg/L). Hemoculture tests and urine analysis were negative for infection. A contrast chest computed tomography revealed a mass measuring 5.5 × 4.5 cm, occupying the left atrium cavity. The patient underwent surgical excision of the mass, however, surgical margin of the resected tumor could not be evaluated, due to the multifragmented nature of the resection specimen. Postoperative CRP and leukocyte levels normalized, highlighting the relationship between the tumor and the inflammatory status. Early diagnosis is crucial for a proper management and favorable outcome, enabling patients to undergo chemotherapy and achieve complete surgical resection.

Introduction: Head and neck sarcomas result in high mortality rates. A lot of new cases of sarcomas are diagnosed every year constituting about 1 % of all head and neck malignancies. Undifferentiated pleomorphic sarcomas (UPS) are high grade soft tissue malignant tumors which occur primarily in limbs and retroperitoneal cavities. These tumors can often metastasize to the central nervous system. However, in rare instances, soft tissue sarcomas may develop as a primary lesion within the intracranial compartments. Case description: A young male presented to the clinic with occipital headache and blurring of vision. Initial workup included brain contrast enhanced computed tomography (CECT) and magnetic resonance imaging (MRI). The CECT suggested that there was an extra-axial mass present which was pressing against the adjacent left frontal lobe. Overlying frontal bone of the left side showed remodeling effect and associated mild periosteal reaction. MRI scan showed intracranial extra-axial lobulated mass with T1 intermediate to low signal intensity and intermediate to high signals on T2 sequences. Heterogeneous enhancement on post-contrast sequences was also seen. The lesion had a broad-based attachment with dura mater and was closely applied to the orbital roof without orbital invasion. Staging Positron emission tomography–computed tomography (PET/CT) scan showed a solitary site of disease in an intracranial location. Final diagnosis was confirmed by histopathology following excision of mass as undifferentiated pleomorphic sarcoma. Post-surgery MRI brain showed satisfactory postoperative appearance without any residual disease. Patient remained asymptomatic for 2 years and 6 months following the resection of the tumor. Practical implications: Most of the extra-axial intracranial soft tissue tumors arise from the meninges with meningiomas making the substantial bulk; however, possibility of other relatively rare tumors of meningeal origin must not be ignored. Intracranial soft tissue sarcomas mostly arise from meninges thus require a good understanding of clinical presentation as well as acquaintance with morphological features on radiological imaging to differentiate from other tumors. These can be treated with excision and radiotherapy along with sequential follow-ups to look for recurrence. Tissue sampling is mandatory followed by complete staging scan in case of sarcomas to rule out possible primary or secondary disease.

Abstract Background High-grade spindle cell sarcomas are a subtype of rare, undifferentiated pleomorphic sarcomas (UPSs) for which diagnosis is difficult and no specific treatment strategies have been established. The limited published data on UPSs suggest an aggressive clinical course, high rates of local recurrence and distant metastasis, and poor prognosis. Case presentation Here we present the unusual case of a 45-year-old male patient with a lumbosacral UPS extending into the sacrum. An initial diagnosis of a low-grade malignant spindle cell tumor was based on a tumor core biopsy. After complete extensive resection, the diagnosis of an UPS of the lumbosacral region was confirmed by excluding other types of cancers. Despite treatment with neoadjuvant radiotherapy, extensive resection, and adjuvant chemotherapy, the patient presented with multiple pulmonary metastases 3 months after surgery. The patient then began treatment with crizotinib at an oral dose of 450 mg per day, based on the detection of a LMNA-NTRK1 fusion gene in the tumor by next-generation sequencing. Over 18 months of follow-up through July 2018, the patient maintained a near-complete clinical response to crizotinib. Conclusions The LMNA-NTRK1 fusion was likely the molecular driver of tumorigenesis and metastasis in this patient, and the observed effectiveness of crizotinib treatment provides clinical validation of this molecular target. Molecular and cytogenetic evaluations are critical to accurate prognosis and treatment planning in cases of UPS, especially when treatment options are limited or otherwise exhausted. Molecularly targeted therapy of these rare but aggressive lesions represents a novel treatment option that may lead to fewer toxic side effects and better clinical outcomes.

An undifferentiated pleomorphic sarcoma (UPS), also known as a malignant fibrous histiocytoma in the past, commonly involves the soft tissue of the extremities and the retroperitoneum. However, a primary UPS of the colon mesentery is very rare. A 69-year-old male patient visited our outpatient department for treatment of an enlarged, palpable mass in the right lower quadrant (RLQ). Computed tomography showed a 15-cm multilobulated, heterogeneous, enhanced mass in the RLQ, which we suspected originated from the colon. He underwent a right hemicolectomy, and the pathologic result was a colon mesenteric UPS. We report a rare case of a primary UPS of the colon mesentery and discuss the characteristics of this neoplasm in reference to the literature.

Immune checkpoint inhibitors (ICIs) such as PD1/PD-L1 blockers are an established treatment for many solid cancers. There are currently no approved ICIs for sarcomas, but satisfactory results have been seen in some patients with disseminated disease in certain histological types. Most studies on PD-L1 in sarcoma have used small specimens and there are no clear cutoff values for scoring. We investigated PD-L1 immunoreactivity in high-grade chondrosarcomas (CS), abdominal liposarcoma (LS) and undifferentiated pleomorphic sarcomas (UPS). In total, 230 tumors were stained with SP142 and SP263 assays and evaluated by two clinical pathologists. Immunoreactivity in tumor and immune cells was correlated with clinical outcome. Overall, ≥1% PD-L1 immunoreactivity in tumor cells was found in 11 CS, 26 LS and 59 UPS (SP142 assay) and in 10 CS, 26 LS and 77 UPS (SP263 assay). Most tumors exhibited ≤10% PD-L1 immunoreactivity, but a subset across all three subtypes had >50%. Kaplan–Meier survival analysis showed no significant difference in metastasis-free or overall survival in relation to PD-L1 immunoreactivity in tumor or immune cells for any subtype. As there is a lack of clinical data regarding PD-L1/PD-1 status and therapy response, it is not currently possible to establish clear cutoff values. Patients with high (>50%) PD-L1 immunoreactivity in tumor cells (TC) with the SP263 assay would be a logical group to investigate for potentially beneficial PD1/PD-L1-targeted treatment.