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Cancers
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Pediatric and AYA Sarcoma and Intermediate Tumors
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Pediatric and AYA Sarcoma and Intermediate Tumors

A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Pediatric Oncology".

Deadline for manuscript submissions: closed (25 March 2024) | Viewed by 4033

Special Issue Editor

Prof. Dr. Yoshihiro Nishida

E-Mail Website
Guest Editor
Department of Rehabilitation Medicine and Orthopedic Surgery, Nagoya University Hospital, Nagoya, Japan
Interests: sarcoma; desmoid; neurofibromatosis type-1 related tumors

Special Issue Information

Dear Colleagues,

Bone and soft tissue sarcoma are a heterogeneous population composed of numerous rare cancers. Among them, there are several tumors that occur frequently in children and the adolescent and young adult (AYA) generation. These sarcomas often include tumors associated with hereditary cancer predisposition syndrome, and genetic counseling is recommended for all AYA patients diagnosed with sarcoma. When genetic abnormalities are responsible for the development of sarcoma (e.g., rhabdomyosarcoma), the type of gene mutation may affect treatment outcome and prognosis.

Recently, new treatments have emerged as multidisciplinary treatments for pediatric and AYA sarcoma patients. They include various drugs, such as molecular-targeted therapeutic agents and radiation therapy using particle beams. While these new therapies have contributed to improving the prognosis of patients, various complications—particularly late complications—have become a problem.

With new technologies such as next-generation sequencing and new treatments, sarcoma practice is advancing rapidly. This Special Issue will highlight pediatric and AYA bone and soft tissue sarcomas including intermediate tumors with these characteristics, and covers diagnostics including genetic mutations, treatments (surgical, medical, radiation), outcomes, and late complications.

Prof. Dr. Yoshihiro Nishida
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cancers is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2900 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • bone and soft tissue sarcoma
  • pediatric
  • AYA
  • treatments
  • outcomes

Published Papers (2 papers)

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Review

14 pages, 480 KiB  
Review
Current Treatment Concepts for Extra-Abdominal Desmoid-Type Fibromatosis: A Narrative Review
by Yong-Suk Lee, Min Wook Joo, Seung-Han Shin, Sungan Hong and Yang-Guk Chung
Cancers 2024, 16(2), 273; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers16020273 - 08 Jan 2024
Cited by 1 | Viewed by 890
Abstract
Extra-abdominal desmoid-type fibromatosis (EADTF) is a rare neoplastic condition of monoclonal fibroblastic proliferation characterized by local aggressiveness with a distinct tendency to recur. Although EADTF is a benign disease entity, these tumors have a tendency to infiltrate surrounding normal tissues, making it difficult [...] Read more.
Extra-abdominal desmoid-type fibromatosis (EADTF) is a rare neoplastic condition of monoclonal fibroblastic proliferation characterized by local aggressiveness with a distinct tendency to recur. Although EADTF is a benign disease entity, these tumors have a tendency to infiltrate surrounding normal tissues, making it difficult to completely eliminate them without adjacent healthy tissue injury. Surgical excision of these locally aggressive tumors without clear resection margins often leads to local recurrence. The aim of this thorough review was to assess the current treatment concepts for these rare tumors. A comprehensive search of articles published in the Cochrane Library, MEDLINE (PubMed), and EMBASE databases between January 2008 and February 2023 was conducted. Surgical intervention is no longer the first-line approach for most cases; instead, strategies like active surveillance or systemic therapies are used as initial treatment options. With the exception of EADTFs situated near vital structures, a minimum of 6–12 months of active surveillance is currently advocated for, during which some disease progression may be considered acceptable. Non-surgical interventions such as radiation or cryoablation may be employed in certain patients to achieve local control. The currently preferred systemic treatment options include tyrosine kinase inhibitors, low-dose chemotherapy, and gamma-secretase inhibitors, while hormone therapy is not advised. Nonsteroidal anti-inflammatory drugs are utilized primarily for pain management. Full article
(This article belongs to the Special Issue Pediatric and AYA Sarcoma and Intermediate Tumors)
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20 pages, 6697 KiB  
Review
Synovial Sarcoma in the Extremity: Diversity of Imaging Features for Diagnosis and Prognosis
by Eun Byul Cho, Seul Ki Lee, Jee-Young Kim and Yuri Kim
Cancers 2023, 15(19), 4860; https://0-doi-org.brum.beds.ac.uk/10.3390/cancers15194860 - 05 Oct 2023
Cited by 4 | Viewed by 2457
Abstract
Synovial sarcomas are rare and highly aggressive soft-tissue sarcomas, primarily affecting adolescents and young adults aged 15–40 years. These tumors typically arise in the deep soft tissues, often near the large joints of the extremities. While the radiological features of these tumors are [...] Read more.
Synovial sarcomas are rare and highly aggressive soft-tissue sarcomas, primarily affecting adolescents and young adults aged 15–40 years. These tumors typically arise in the deep soft tissues, often near the large joints of the extremities. While the radiological features of these tumors are not definitely indicative, the presence of calcification in a soft-tissue mass (occurring in 30% of cases), adjacent to a joint, strongly suggests the diagnosis. Cross-sectional imaging characteristics play a crucial role in diagnosing synovial sarcomas. They often reveal significant characteristics such as multilobulation and pronounced heterogeneity (forming the “triple sign”), in addition to features like hemorrhage and fluid–fluid levels with septa (resulting in the “bowl of grapes” appearance). Nevertheless, the existence of non-aggressive features, such as gradual growth (with an average time to diagnosis of 2–4 years) and small size (initially measuring < 5 cm) with well-defined margins, can lead to an initial misclassification as a benign lesion. Larger size, older age, and higher tumor grade have been established as adverse predictive indicators for both local disease recurrence and the occurrence of metastasis. Recently, the prognostic importance of CT and MRI characteristics for synovial sarcomas was elucidated. These include factors like the absence of calcification, the presence of cystic components, hemorrhage, the bowl of grape sign, the triple sign, and intercompartmental extension. Wide surgical excision remains the established approach for definitive treatment. Gaining insight into and identifying the diverse range of presentations of synovial sarcomas, which correlate with the prognosis, might be helpful in achieving the optimal patient management. Full article
(This article belongs to the Special Issue Pediatric and AYA Sarcoma and Intermediate Tumors)
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