Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Aug;12(15):16254-16263.
doi: 10.1002/cam4.6289. Epub 2023 Jun 27.

Multidisciplinary management of adolescents and young adults (AYA) sarcoma: A successful effort of an adult high-volume cancer center

Alexia Francesca Bertuzzi  1 , Maria Susanna Grimaudo  2 , Alice Laffi  1 , Laura Giordano  3 , Nicolò Gennaro  4 , Umberto Cariboni  5 , Licia Vanessa Siracusano  1 , Vittorio Quagliuolo  5 , Piergiuseppe Colombo  2   6 , D'Orazio Federico  7 , Salvatore Lorenzo Renne  2   6 , Cristina Specchia  8 , Ferdinando Cananzi  2   5 , Andrea Marrari  9 , Pierina Navarria  10 , Primo Andrea Daolio  11 , Stefano Bastoni  11 , Armando Santoro  1   2
Affiliations

Multidisciplinary management of adolescents and young adults (AYA) sarcoma: A successful effort of an adult high-volume cancer center

Alexia Francesca Bertuzzi et al. Cancer Med. 2023 Aug.

Abstract

Introduction: The aim of this retrospective study was to investigate the clinicopathological characteristics of AYA sarcomas and their clinical outcomes at a high-volume single center.

Methods: Demographic, clinicopathological data on the diagnosis, treatment and follow-up of all sarcoma patients aged 16-39 years (ys) observed at our Institute between January 2010 and December 2021 were retrospectively collected, including diagnostic (TTD) and treatment delay(TTT), clinical outcomes (OS and PFS), and late-treatment effects.

Results: We identified 228 AYA patients, median age 30 years, 29% ≤ 25 years, 57% males, 88% soft tissue sarcomas (STS), and 12% bone sarcomas (BS). Among STSs, 13% were small round cell tumors (SRCT), 52% intermediate-high-grade, 24% low-grade STSs. Among BS, 32% were high-grade. Median TTD and TTT were 120 (0-8255) and 7 days (0-83), respectively. Surgery was performed in 83%, radiotherapy in 29%, and systemic therapy in 27%. Median follow-up was 72.9 months(1.6-145), 5-year and 10-year OS were 78.5% and 62%, respectively. Kaplan-Meyer analysis showed a significantly better 5-year OS and PFS for patients with >92 days of TTD (OS 85.7% vs. 66.7%, p = 0.001, PFS 50.2% vs. 24.9%, p = 0.009). According to age (≤25 years vs. > 25 years), 5-year OS was 69.8% versus 82.2%, respectively (p = 0.047).

Conclusion: Our analysis confirmed previous data on sarcoma AYA patients followed in a referral center. Unexpectedly, diagnostic delay was not associated with poor OS and PFS. Patients <25 years showed a poorer prognosis due to the higher incidence of SRCT.

Keywords: AYA; adolescent; oncology; sarcoma; young adult.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

FIGURE 1
FIGURE 1
Different histologies among the sarcoma AYA patients. ASPS, alveolar soft part sarcoma; BSs, bone sarcomas; CCS, clear cells sarcoma; DFS, dermatofibrosarcoma protuberans; FD, desmoid fibromatosis; GCT, giant cell tumor; GIST, gastrointestinal stromal tumor; MPNST, malignant peripheral nerve sheath tumor; SRCTs, small round cells tumors; SS, synovial sarcoma; STSs, soft tissue sarcomas; UPS, undifferentiated pleomorphic sarcoma.
FIGURE 2
FIGURE 2
AYA patients described according to the site of sarcoma origin. (A) The whole study population according to the site of origin; (B) Soft tissue sarcomas, (C) Bone sarcomas, (D) Ewing's sarcomas, and (E) Desmoid fibromatosis by sites of origin.
FIGURE 3
FIGURE 3
(A) AYA patients according to the sarcoma stage at diagnosis, (B) localized/locally advanced, and (C) metastatic.
FIGURE 4
FIGURE 4
Overall survival stratified by time to diagnosis.
FIGURE 5
FIGURE 5
Trend of the overall survival (OS) and progression‐free survival (PFS). (A) OS in the whole study population; (B) PFS in the whole study population; (C) OS according to the sarcoma grade; (D) PFS according to the sarcoma grade. I‐H, intermediate/high‐grade sarcomas, L, low‐grade sarcomas.
FIGURE 6
FIGURE 6
Overall survival in the study population according to age.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

References

    1. Reed DR, Hayashi M, Wagner L, et al. Treatment pathway of bone sarcoma in children, adolescents, and young adults. Cancer. 2017;123:2206‐2218. doi:10.1002/cncr.30589 - DOI - PMC - PubMed
    1. Papworth KE, Arroyo VM, Styring E, Zaikova O, Melin BS, Lupo PJ. Soft‐tissue sarcoma in adolescents and young adults compared with older adults: a report among 5000 patients from the Scandinavian sarcoma group central register. Cancer. 2019;125:3595‐3602. doi:10.1002/cncr.32367 - DOI - PubMed
    1. Van Gaal JC, De Bont ESJM, Kaal SEJ, Versleijen‐Jonkers Y, van der Graaf WTA. Building the bridge between rhabdomyosarcoma in children, adolescents and young adults: the road ahead. Crit Rev Oncol Hematol. 2012;82:259‐279. doi:10.1016/j.critrevonc.2011.06.005 - DOI - PubMed
    1. van der Graaf WTA, Orbach D, Judson IR, Ferrari A. Soft tissue sarcomas in adolescents and young adults: a comparison with their paediatric and adult counterparts. Lancet Oncol. 2017;18:e166‐e175. doi:10.1016/S1470-2045(17)30099-2 - DOI - PubMed
    1. Komdeur R, Klunder J, van der Graaf WTA, et al. Multidrug resistance proteins in rhabdomyosarcomas: comparison between children and adults. Cancer. 2003;97:1999‐2005. doi:10.1002/cncr.11259 - DOI - PubMed

Publication types