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J Thorac Dis 2021 Apr;13(4):2649-2660

Mainz University Thoracic Center, Thoracic Surgery and Pulmonary Medicine, Mainz, Germany.

Background: Soft tissue sarcoma (STS) tend to metastasis to the lungs. Pulmonary metastasectomy seems to be a common practice always when plausible. The objective of this article was to review systematically the results of a literature search on pulmonary metastasectomy for STSs published in the last ten years and to offer a brief overview about the current practice as well. Read More

Medicina (Kaunas) 2021 Feb 18;57(2). Epub 2021 Feb 18.

Department of Orthopedic Surgery, Kindai University Hospital, Osaka-Sayama City, Osaka 589-8511, Japan.

Undifferentiated pleomorphic sarcoma (UPS) is major type of soft tissue sarcomas. UPS presenting with inflammation is rare, and its pathophysiology remains unclear. Herein, we report a rare case of UPS with prolonged fever. Read More

BMC Musculoskelet Disord 2021 Feb 15;22(1):189. Epub 2021 Feb 15.

Department of Radiology, The Third Hospital of Hebei Medical University, 139 Ziqiang Road, Shijiazhuang, 050051, Hebei Province, China.

Background: To investigate the clinical, imaging and pathological features of dedifferentiated chondrosarcoma for better diagnosis.

Methods: Patients who had been confirmed to have dedifferentiated chondrosarcoma were enrolled in this study and analyzed in the clinical, imaging and pathological data.

Results: Twenty-five patients had pathologically confirmed dedifferentiated chondrosarcoma including 15 males and 10 females with an age range of 24-74 (median 58, interquartile range 49-65). Read More

Spec Care Dentist 2021 May 5;41(3):417-422. Epub 2021 Feb 5.

ASST Santi Paolo e Carlo, Presidio Ospedaliero San Paolo, Odontostomatologia II Clinical Unit, Milan, Italy.

Undifferentiated pleomorphic sarcoma (UPS), also defined malignant fibrous histiocytoma (MFH), is one of the most common sarcomas affecting soft tissues in adults, but it can also arise as a primary tumor in bone. UPS is extremely uncommon in the lower jaw and presents very aggressive clinical behavior with high rate of local recurrences and frequent metastases. Secondary UPS are even rarer than primary forms, and they are usually closely associated with local trauma or radiotherapy. Read More

BMC Cancer 2021 Jan 22;21(1):93. Epub 2021 Jan 22.

Department of Diagnostic and Interventional Radiology, School of Medicine & Klinikum rechts der Isar, Technical University of Munich, Ismaninger Str. 22, 81675, Munich, Germany.

Background: Small soft tissue masses are often falsely assumed to be benign and resected with failure to achieve tumor-free margins. Therefore, this study retrospectively investigated the distribution of histopathologic diagnosis to be encountered in small soft tissue tumors (≤ 5 cm) in a large series of a tertiary referral center.

Methods: Patients with a soft tissue mass (STM) with a maximum diameter of 5 cm presenting at our institution over a period of 10 years, who had undergone preoperative Magnetic resonance imaging and consequent biopsy or/and surgical resection, were included in this study. Read More

Gan To Kagaku Ryoho 2020 Dec;47(13):2180-2182

Dept. of Digestive Surgery, Kohsei Chuo General Hospital.

Undifferentiated pleomorphic sarcoma develops in adult soft tissues and has a poor prognosis. It often recurs in the limbs and trunk, but is rare in the mesentery. Complete resection of the tumor is the first-line treatment, and there are previously reported cases of the usefulness of chemotherapy and radiation therapy; however, several factors remain to be clarified. Read More

Gan To Kagaku Ryoho 2020 Dec;47(13):2168-2170

Dept. of Surgery, PL General Hospital.

The patient was a 73-year-old woman who had undergone breast-conserving surgery followed by irradiation (50 Gy/25 Fr)to the residual breast for left breast cancer 4 years before. Computed tomography for routine examination revealed a soft tissue mass on her left chest wall. Ultrasonography showed a hypoechoic mass with heterogeneous internal echo, 3. Read More

Jt Dis Relat Surg 2021 6;32(1):253-257. Epub 2021 Jan 6.

Koç Üniversitesi Hastanesi Ortopedi ve Travmatoloji Bölümü, 34010 Topkapı, Istanbul, Türkiye.

Undifferentiated pleomorphic sarcoma (UPS) of the skin is a rare soft tissue sarcoma subtype with a high risk of metastasis and local recurrence. Ultraviolet exposure plays a prominent role in its etiology. Herein, we present two rare cases of UPS of the skin with an occurrence in non-ultraviolet exposed locations and discuss the need of wide resection and the identification of the depth of the tumor. Read More

J Cancer Res Ther 2020 Oct-Dec;16(6):1524-1527

Department of Radiation Oncology, Proton Medical Research Center, University of Tsukuba Hospital, Tsukuba, Ibaraki, Japan.

Li-Fraumeni syndrome (LFS) is a genetic disease that is hypersensitive to radiotherapy. Proton therapy (PT) was strongly recommended for pediatric and radiation-sensitive tumors. However, there is little information on PT for LFS. Read More

J Plast Reconstr Aesthet Surg 2021 Apr 8;74(4):755-767. Epub 2020 Nov 8.

Plastic and Reconstructive Microsurgery, Careggi University Hospital, Florence, Italy.

Background And Objectives: Upper limb preservation after soft tissue sarcoma (STS) surgical excision is now the accepted gold standard and it often requires reconstruction with free flaps. The purpose of this review is to summarize current literature on upper limb reconstruction with free flaps after STS resection.

Methods: A systematic review was performed in July 2019 in PubMed and MedLine Ovid databases according to the PRISMA guidelines. Read More

Cancer Med 2020 12 16;9(24):9282-9292. Epub 2020 Oct 16.

Toronto Sarcoma Program at Mount Sinai Hospital, Toronto, Canada.

Non-osteogenic, non-Ewing soft-tissue sarcoma (NONE-STS) of bone is a rare presentation of primary bone cancers. Optimal treatments and outcomes for this heterogenous group are poorly described. We evaluated the factors associated with long-term outcomes in patients with this disease. Read More

Anticancer Res 2020 Oct;40(10):5793-5800

Department of Orthopaedics and Sports Orthopaedics, Technical University of Munich, Klinikum rechts der Isar, Munich, Germany

Background/aim: Myxofibrosarcoma (MFS) is characterized by an infiltrative growth pattern. This study aimed to determine the correlation between overall survival (OS) and morphological features of MFS as well as examine the reproducibility of these findings on preoperative magnetic resonance imaging (MRI).

Patients And Methods: Fifty-eight MFS patients underwent preoperative MR imaging with the following features analysed: i) tumour size, ii) localization, iii) margins, iv) morphology, v) signal characteristics, vi) contrast enhancement, vii) presence and extent of perilesional oedema, and viii) presence of the tail sign. Read More

Neurosurgery 2020 12;88(1):E114-E122

Department of Neurologic Surgery, Mayo Clinic, Jacksonville, Florida.

Background And Importance: In the setting of intracranial neoplasms, EWSR1-cAMP Response Element-Binding Protein (CREB) transcription factor family fusions have been described in myxoid mesenchymal tumors, extremely rare entities with a close histopathologic and immunologic resemblance to myxoid subtype angiomatoid fibrous histiocytomas (AFH). Controversy exists on whether these central nervous system lesions are a subtype of myxoid AFH or a completely separate entity, which entitles a distinct clinical behavior and, consequently, a different approach to management. Upon review of the literature, only 14 cases of intracranial tumors harboring an EWSR1-CREB family fusion were identified, with only 3 cases presenting in middle-aged adults, none of which reported an EWSR1-CREM fusion mutation. Read More

Mod Pathol 2021 02 28;34(2):396-407. Epub 2020 Aug 28.

Diagnostic and Research Institute of Pathology, Comprehensive Cancer Centre Graz, Medical University of Graz, Graz, Austria.

Fusions involving NTRK1, NTRK2, and NTRK3 are oncogenic drivers occurring in a spectrum of mesenchymal neoplasms ranging from benign to highly malignant tumors. To gain further insights into the staining profile with the pan-TRK assay, we analyzed a large number of soft tissue sarcomas and correlated our findings with molecular testing. Additionally, we expand the spectrum of NTRK-fusion tumors by reporting a mesenchymal lesion in the lung as well as a mesenchymal skin lesion in the spectrum of benign fibrous histiocytoma with NTRK-fusion. Read More

J Korean Assoc Oral Maxillofac Surg 2020 Aug;46(4):282-287

Department of Oral Medicine, Hospital Sírio Libanês, São Paulo, Brazil.

Undifferentiated pleomorphic sarcoma (UPS) is a high-grade neoplasm that is usually located in the extremities and retroperitoneum. In the past, UPS was considered the most common soft tissue sarcoma in adults; due to improvements in diagnostic techniques, most cases have been reclassified as other lineage-specific tumors. Gnathic bones are rarely affected, and the clinicopathological characteristics of this neoplasm when diagnosed in the jaw remain to be better described. Read More

World Neurosurg 2020 11 7;143:398-404. Epub 2020 Aug 7.

Department of Neurosurgery, National Neuroscience Institute, King Fahad Medical City, Riyadh, Saudi Arabia.

Background: Angiomatoid fibrous histiocytoma (AFH) is a rare, fibrohistiocytic, soft-tissue neoplasm. Intracranial AFH is extremely rare. Here we present 2 pediatric cases of intracranial AFH and perform a literature review on this disease entity. Read More

Am J Surg Pathol 2020 11;44(11):1535-1540

Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania.

Primary pulmonary myxoid sarcoma (PPMS) is a recently reported, exceedingly rare low-grade lung neoplasm characterized by reticular/lace-like growth of spindle to epithelioid cells embedded in an abundant myxoid matrix. Morphologically, it overlaps with a myxoid variant of angiomatoid fibrous histiocytoma (AFH) of the soft tissue. Genetically, they were both reported to harbor EWSR1-CREB1 fusion, while EWSR1-ATF1 has only been reported in AFH thus far. Read More

Mod Pathol 2020 11 7;33(11):2233-2243. Epub 2020 Aug 7.

Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY, USA.

Gene fusions constitute pivotal driver mutations often encoding aberrant chimeric transcription factors. However, an increasing number of gene fusion events have been shown not to be histotype specific and shared among different tumor types, otherwise completely unrelated clinically or phenotypically. One such remarkable example of chromosomal translocation promiscuity is represented by fusions between EWSR1 or FUS with genes encoding for CREB-transcription factors family (ATF1, CREB1, and CREM), driving the pathogenesis of various tumor types spanning mesenchymal, neuroectodermal, and epithelial lineages. Read More

J Am Acad Dermatol 2020 12 15;83(6):e429-e430. Epub 2020 Jul 15.

Division of Dermatology, Department of Medicine, Washington University in St. Louis, St Louis, Missouri. Electronic address:

Dermatol Online J 2020 May 15;26(5). Epub 2020 May 15.

San Diego Family Dermatology, National City, CA Touro University California College of Osteopathic Medicine, Vallejo, CA Scripps Family Medicine Residency, Scripps Mercy Hospital Chula Vista, Chula Vista, CA Family Medicine Residency, Family Health Centers of San Diego, San Diego, CA.

Pleomorphic dermal sarcoma is a cutaneous soft tissue sarcoma that presents as a rapidly enlarging tumor, typically on a sun-exposed location of elderly individuals. The neoplasm shares many similar features - clinical, pathologic, immunohistochemical and genomic - with atypical fibroxanthoma. However, adverse histologic characteristics (deep subcutaneous invasion, tumor necrosis, lymphovascular invasion, and/or perineural invasion) differentiate pleomorphic dermal sarcoma from atypical fibroxanthoma and may account for the more aggressive biologic behavior of pleomorphic dermal sarcoma: local recurrence and metastases. Read More

Dermatol Online J 2020 Apr 15;26(4). Epub 2020 Apr 15.

Department of Dermatology and Venereology, Centro Hospitalar Universitário de São João EPE, Porto.

Plexiform fibrohistiocytic tumor is an uncommon soft tissue neoplasm of intermediate malignancy, most frequently occurring as a painless, slow-growing nodule that shows a distinct predilection for children and young adults. We report a healthy 11-year-old boy presenting with a 1-year history of an asymptomatic cutaneous nodule on his left shoulder. Histopathological and immunohistochemical analysis confirmed a diagnosis of plexiform fibrohistiocytic tumor. Read More

Sci Rep 2020 06 10;10(1):9414. Epub 2020 Jun 10.

Department of Orthopaedic Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.

Infiltrative tumor growth into adjacent soft tissues is a major cause of the frequent recurrence and tumor-related death of myxofibrosarcoma (MFS), but no useful biomarkers reflecting tumor burden and infiltrative growth are available. While emerging evidence suggests a diagnostic and functional role of extracellular/circulating microRNA (miRNA) in various malignant diseases, their significance in MFS patients remains unknown. Global miRNA profiling identified four upregulated miRNAs in MFS patient sera and culture media of MFS cells. Read More

Cureus 2020 Apr 23;12(4):e7801. Epub 2020 Apr 23.

Plastic, Reconstructive and Hand Surgery, Northwell Health/Donald and Barbara Zucker School of Medicine, Staten Island University Hospital, Staten Island, USA.

Malignant fibrous histiocytoma (MFH) is an undifferentiated high-grade pleomorphic sarcoma and is considered the most common primary soft tissue sarcoma in adults. MFH is known to arise in the trunk, extremities and retroperitoneum although it can arise anywhere in the body.MFH of the skin is uncommon and even less frequent is the involvement of the scalp, especially with skull invasion. Read More

Vet Pathol 2020 07 29;57(4):490-496. Epub 2020 Apr 29.

Michigan State University, Lansing, MI, USA.

Multinucleated giant cells (MGCs) are a prominent histological feature of various mesenchymal neoplasms and are often considered a criterion of malignancy. Mesenchymal neoplasms with MGCs for which the cell lineage is unclear generally are referred to as giant cell sarcomas. Here we characterize the gross, histologic, and immunohistochemical features of 90 giant cell sarcomas in domestic pet rabbits. Read More

Am J Dermatopathol 2020 Sep;42(9):689-693

Cellular Pathology, St George's University Hospital, London, United Kingdom.

Angiomatoid fibrous histiocytoma (AFH) is a relatively rare soft tissue tumor of intermediate malignant potential, occurring most commonly in young adults, with a recognized propensity for local recurrence and occasional metastasis. A case of AFH occurring on the finger of a 60-year-old man is described in which the unusual location and age group for this entity raised the original wrong diagnosis of an aneurysmal and cellular fibrous histiocytoma. Further workup demonstrated an EWSR1-CREB1 translocation, confirming the correct diagnosis of AFH. Read More

Hum Pathol 2020 05 1;99:107-115. Epub 2020 Apr 1.

Department of Pathology, Ospedale Pediatrico Bambino Gesú, Rome, 00165, Italy.

Fibrous histiocytoma (FH) or dermatofibroma is a common cutaneous lesion mostly seen in adults and rare in the first two years of life. Two hundred sixty-seven patients younger than 18 years with a diagnosis of FH or dermatomyofibroma, a lesion with morphologic overlap with FH, were identified from the files of a single institution, with only 13 (4.8%) occurring in patients younger than 5 years. Read More

Ann R Coll Surg Engl 2020 May 1;102(5):e105-e106. Epub 2020 Apr 1.

Hull Royal Infirmary, Hull and East Yorkshire Hospitals NHS Trust, Hull, UK.

Primary malignant fibrous histiocytoma, now classified as pleomorphic undifferentiated sarcoma, is the most common soft-tissue sarcoma in adult life. Primary splenic pleomorphic undifferentiated sarcoma is extremely rare and aggressive, and is associated with a poor prognosis; only 14 cases of splenic pleomorphic undifferentiated sarcoma have been documented in the English literature. We discuss a case of a 56-year-old woman with iron-deficiency anaemia, early satiety and left upper-quadrant pain, who was preoperatively diagnosed with a large splenic cyst following thorough investigation. Read More

Cancer Med 2020 05 17;9(10):3344-3352. Epub 2020 Mar 17.

Department of Medical Oncology, Zhongshan Hospital, Fudan University, Shanghai, China.

Background: Anlotinib is a novel, orally administered, multitarget receptor tyrosine kinase inhibitor. It functions by inhibiting tumor angiogenesis and proliferative signaling pathways. In this study, we aimed to investigate the efficacy and safety of anlotinib plus epirubicin in a sarcoma patient-derived xenografts (PDX) model. Read More

Gan To Kagaku Ryoho 2019 Dec;46(13):2568-2570

Dept. of Surgery, Kohsei Chuo General Hospital.

The patient was a 49-year-old man with persistent fever since the introduction of hemodialysis(HD). Vomiting and abdominal swelling appeared 4 months after initiating hemodialysis. Computed tomography(CT)scan revealed a tumor measuring 9 cm, and disorders of passage from the jejunum. Read More