Soft tissue sarcoma (STS) accounts for 1% of all malignant neoplasms in adults. Their diagnosis and management constitute a challenging target. They originate from the mesenchyme, and 50 subtypes with various cytogenetic profiles concerning soft tissue and bones have been recognized. These tumors mainly affect middle-aged adults but may be present at any age. Half of the patients have metastatic disease at the time of diagnosis and require systemic therapy. Tumors above 3-5 cm in size must be suspected of potential malignancy. A thorough history, clinical examination and imaging that must precede biopsy are necessary. Modern imaging techniques include ultrasound, computed tomography (CT), new magnetic resonance imaging (MRI), and positron emission tomography/CT. MRI findings may distinguish low-grade from high-grade STS based on a diagnostic score (tumor heterogeneity, intratumoral and peritumoral enhancement). A score ≥ 2 indicates a high-grade lesion, and a score ≤ 1 indicates a low-grade lesion. For disease staging, abdominal imaging is recommended to detect early abdominal or retroperitoneal metastases. Liquid biopsy by detecting genomic material in serum is a novel diagnostic tool. A preoperative biopsy is necessary for diagnosis, prognosis and optimal planning of surgical intervention. Core needle biopsy is the most indicative and effective. Its correct performance influences surgical management. An unsuccessful biopsy means the dissemination of cancer cells into healthy anatomical structures that ultimately affect resectability and survival. Complete therapeutic excision (R0) with an acceptable resection margin of 1 cm is the method of choice. However, near significant structures, i.e., vessels, nerves, an R2 resection (macroscopic margin involvement) preserving functionality but having a risk of local recurrence can be an acceptable choice, after informing the patient, to prevent an unavoidable amputation. For borderline resectability of the tumor, neoadjuvant chemo/radiotherapy has a place. Likewise, after surgical excision, adjuvant therapy is indicated, but chemotherapy in nonmetastatic disease is still debatable. The five-year survival rate reaches up to 55%. Reresection is considered after positive or uncertain resection margins. Current strategies are based on novel chemotherapeutic agents, improved radiotherapy applications to limit local side effects and targeted biological therapy or immunotherapy, including vaccines. Young age is a risk factor for distant metastasis within 6 mo following primary tumor resection. Neoadjuvant radiotherapy lasting 5-6 wk and surgical resection are indicated for high-grade STS (grade 2 or 3). Wide surgical excision alone may be acceptable for patients older than 70 years. However, locally advanced disease requires a multidisciplinary task of decision-making for amputation or limb salvage.

New trends in the surgical management of soft tissue sarcoma: The role of preoperative biopsy

Surgery is the cornerstone of treatment for retroperitoneal sarcoma (RPS). Surgery should be performed by a surgical oncologist with sub-specialization in this disease and in the context of a multidisciplinary team of sarcoma specialists. For primary RPS, the goal of surgery is to achieve the complete en bloc resection of the tumor along with involved organs and structures to maximize the clearance of the disease. The extent of resection also needs to consider the risk of complications. Unfortunately, the overarching challenge in primary RPS treatment is that even with optimal surgery, tumor recurrence occurs frequently. The pattern of recurrence after surgery (e.g., local versus distant) is strongly associated with the specific histologic type of RPS. Radiation and systemic therapy may improve outcomes in RPS and there is emerging data studying the benefit of non-surgical treatments in primary disease. Topics in need of further investigation include criteria for unresectability and management of locally recurrent disease. Moving forward, global collaboration among RPS specialists will be key for continuing to advance our understanding of this disease and find more effective treatments.

/pdf/surgical-management-of-retroperitoneal-sarcoma-16gzh8fw.pdf

Surgical Management of Retroperitoneal Sarcoma

Under the umbrella of cutaneous sarcomas (CS) we include a heterogeneous group of rare, malignant, mesenchymal neoplasia, such as dermatofibrosarcoma protuberans, atypical fibroxanthoma, cutaneous undifferentiated pleomorphic sarcoma, cutaneous angiosarcoma and leiomyosarcoma. Clinical presentation and histopathological examination are the cornerstone of CS diagnosis and classification. There are scarce data in the literature in regards to the clinical and dermatoscopic characteristics of CS and the role of dermatoscopy in their early identification. We performed a literature review, aiming to summarize current data on the clinical and dermatoscopic presentation of the most common types of cutaneous sarcomas that may facilitate early diagnosis and prompt management. Based on the available published data, CS are characterized by mostly unspecific dermatoscopic patterns. Dermatofibrosarcoma protuberans, Kaposi’s sarcoma, and in a lesser degree, cutaneous angiosarcoma, may display distinct dermatoscopic features, facilitating their early clinical recognition. In conclusion, dermatoscopy, in conjunction with the overall clinical context, may aid towards suspicion of CS.

https://doi.org/10.3390/diagnostics13101822

Clinical and Dermoscopic Characteristics of Cutaneous Sarcomas: A Literature Review

The pelvic anatomy poses great challenges to orthopedic surgeons. Sarcomas are often large in size and typically enclosed in the narrow confines of the pelvis with the close proximity of vital structures. The aim of this study is to report a systematic planned multidisciplinary surgical approach to treat pelvic sarcomas. Seventeen patients affected by bone and soft tissue sarcomas of the pelvis, treated using a planned multidisciplinary surgical approach, combining the expertise of orthopedic oncology and other surgeons (colleagues from urology, vascular surgery, abdominal surgery, gynecology and plastic surgery), were included. Seven patients were treated with hindquarter amputation; 10 patients underwent excision of the tumor. Reconstruction of bone defects was conducted in six patients with a custom-made 3D-printed pelvic prosthesis. Thirteen patients experienced at least one complication. Well-organized multidisciplinary collaborations between each subspecialty are the cornerstone for the management of patients affected by pelvic sarcomas, which should be conducted in specialized centers. A multidisciplinary surgical approach is of paramount importance in order to obtain the best successful surgical results and adequate margins for achieving acceptable outcomes.

/pdf/a-planned-multidisciplinary-surgical-approach-to-treat-23eb00jp.pdf

A Planned Multidisciplinary Surgical Approach to Treat Primary Pelvic Malignancies

Limited information exists regarding the epidemiology, metastasis, and survival of dermatofibrosarcoma protuberans (DFSP). 

The Epidemiology of Dermatofibrosarcoma Protuberans Incidence, Metastasis, and Death Among Various Population Groups: A SEER Database Analysis

Retroperitoneal and pelvis sarcomas are uncommon tumors for which complete surgical resection is the mainstay of treatment. However, achieving complete gross resection with microscopically negative margins is challenging, and local recurrence rates can be high. Patients often succumb to uncontrolled local disease. Radiation therapy offers a potential means for sterilizing microscopic residual disease, although its use continues to be controversial. Chemotherapy alone or in combination with radiation continues to be investigated as an adjunct to surgery, along with immunotherapy and targeted therapies. In this review, we discuss the current management of retroperitoneal and pelvis sarcomas, focusing on studies of surgery and radiation therapy to maximize local control. 

Treatment for local control of retroperitoneal and pelvis sarcomas: A review of the literature.

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally aggressive cutaneous malignancy. Complete resection is the primary treatment but there is debate over the optimal method. Wide local excision was traditionally the standard of care; however, National Comprehensive Cancer Network guidelines now recommend Mohs micrographic surgery as the preferred approach. Medical therapy with imatinib can be used in advanced or unresectable disease. This review will discuss the current management of DFSP, focusing on optimal surgical approach.

Surgical management of dermatofibrosarcoma protuberans

Rust, Dylan BS1; Hirano, Michio MD2; Martinez, Mercedes MD3; Kato, Tomoaki MD4; Weiner, Joshua MD4 Author Information 

119: Multi-visceral transplantation for the treatment of mitochondrial neurogastrointestinal encephalomyopathy (MNGIE)

Clinical TransplantationVolume 38, Issue 1 e15230 LETTER TO THE EDITOR Multivisceral transplantation for the treatment of mitochondrial neurogastrointestinal encephalomyopathy Dylan Rust, Dylan Rust Vagelos College of Physicians and Surgeons, Columbia University, New York, New York, USASearch for more papers by this authorMercedes Martinez, Mercedes Martinez orcid.org/0000-0001-6128-0155 Division of Abdominal Organ Transplant, Columbia University Irving Medical Center, New York, New York, USA Department of Pediatrics, Columbia University Irving Medical Center, New York, New York, USASearch for more papers by this authorStephen Lagana, Stephen Lagana Department of Pathology, Columbia University, New York, New York, USASearch for more papers by this authorMichio Hirano, Michio Hirano Department of Neurology, Columbia University Irving Medical Center, New York, New York, USASearch for more papers by this authorTomoaki Kato, Tomoaki Kato Division of Abdominal Organ Transplant, Columbia University Irving Medical Center, New York, New York, USASearch for more papers by this authorJoshua Weiner, Corresponding Author Joshua Weiner [email protected] orcid.org/0000-0003-3780-1875 Division of Abdominal Organ Transplant, Columbia University Irving Medical Center, New York, New York, USA Correspondence Joshua Weiner, Division of Abdominal Organ Transplant, Columbia University Irving Medical Center, 622 West 168th Street, PH14-105, New York, NY 10032, USA. Email: [email protected]Search for more papers by this author Dylan Rust, Dylan Rust Vagelos College of Physicians and Surgeons, Columbia University, New York, New York, USASearch for more papers by this authorMercedes Martinez, Mercedes Martinez orcid.org/0000-0001-6128-0155 Division of Abdominal Organ Transplant, Columbia University Irving Medical Center, New York, New York, USA Department of Pediatrics, Columbia University Irving Medical Center, New York, New York, USASearch for more papers by this authorStephen Lagana, Stephen Lagana Department of Pathology, Columbia University, New York, New York, USASearch for more papers by this authorMichio Hirano, Michio Hirano Department of Neurology, Columbia University Irving Medical Center, New York, New York, USASearch for more papers by this authorTomoaki Kato, Tomoaki Kato Division of Abdominal Organ Transplant, Columbia University Irving Medical Center, New York, New York, USASearch for more papers by this authorJoshua Weiner, Corresponding Author Joshua Weiner [email protected] orcid.org/0000-0003-3780-1875 Division of Abdominal Organ Transplant, Columbia University Irving Medical Center, New York, New York, USA Correspondence Joshua Weiner, Division of Abdominal Organ Transplant, Columbia University Irving Medical Center, 622 West 168th Street, PH14-105, New York, NY 10032, USA. Email: [email protected]Search for more papers by this author First published: 27 January 2024 https://doi.org/10.1111/ctr.15230Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. REFERENCES 1Hirano M, Silvestri G, Blake DM, et al. Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE): clinical, biochemical, and genetic features of an autosomal recessive mitochondrial disorder. Neurology. 1994; 44(4): 721-727. 10.1212/WNL.44.4.721 PubMedWeb of Science®Google Scholar 2Yadak R, Breur M, Bugiani M. Gastrointestinal dysmotility in MNGIE: from thymidine phosphorylase enzyme deficiency to altered interstitial cells of Cajal. Orphanet J Rare Dis. 2019; 14(1): 33. 10.1186/s13023-019-1016-6 PubMedWeb of Science®Google Scholar 3De Giorgio R, Pironi L, Rinaldi R, et al. Liver transplantation for mitochondrial neurogastrointestinal encephalomyopathy. Ann Neurol. 2016; 80(3): 448-455. 10.1002/ana.24724 CASPubMedWeb of Science®Google Scholar 4D'Angelo R, Boschetti E, Amore G, et al. Liver transplantation in mitochondrial neurogastrointestinal encephalomyopathy (MNGIE): clinical long-term follow-up and pathogenic implications. J Neurol. 2020; 267(12): 3702-3710. 10.1007/s00415-020-10051-x PubMedWeb of Science®Google Scholar 5Kripps K, Nakayuenyongsuk W, Shayota BJ, et al. Successful liver transplantation in mitochondrial neurogastrointestinal encephalomyopathy (MNGIE). Mol Genet Metab. 2020; 130(1): 58-64. 10.1016/j.ymgme.2020.03.001 PubMedWeb of Science®Google Scholar Volume38, Issue1January 2024e15230 ReferencesRelatedInformation 

Multivisceral transplantation for the treatment of mitochondrial neurogastrointestinal encephalomyopathy.

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Dylan J Rust

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Treatment for local control of retroperitoneal and pelvis sarcomas: A review of the literature.

Surgical management of dermatofibrosarcoma protuberans

119: Multi-visceral transplantation for the treatment of mitochondrial neurogastrointestinal encephalomyopathy (MNGIE)

Multivisceral transplantation for the treatment of mitochondrial neurogastrointestinal encephalomyopathy.