Clinical characteristics of sarcoma patients: a population-based data analysis from a German clinical cancer registry

Abstract

Purpose Sarcomas are a heterogeneous group of malignant neoplasms with a wide range of histological types and occur in almost any anatomic site and side. This study evaluated the prognostic factors in sarcoma patients based on German clinical cancer registry data. Methods The German clinical cancer register of Saxony-Anhalt was used for all data analyses. Sarcoma cases of all clinical or pathological T-stages (T1a–T4c), all N-stages (N0-3) and M-stages (0–1b) corresponding to the Union for International Cancer Control (UICC) stages I to IVB were considered. In our analyses, 787 cases diagnosed between 2005 and 2022 were included. Further, we assessed the association of cancer-related parameters with mortality and hazard ratios (HR) from the Cox proportional hazard models. We included sex, age at diagnosis, histological grade, T-, N- and M-stages, tumor size, tumor localization and tumor side as parameters in our regression models. Results The majority of sarcoma patients were diagnosed with leiomyosarcoma (12%), liposarcoma (11%), angiosarcoma (5.3%) and myxofibrosarcoma (2.7%). In our univariate regression models, tumors localized in more than one location, head, face and neck region as well as the pelvis and lower extremity were associated with increased mortality risk (more than one location: HR 7.10, 95% CI 2.20–22.9; head, face and neck: HR 1.35, 95% CI 0.89–2.06; pelvis: HR 1.27, 95% CI 0.86–1.89; lower extremity: HR 1.44, 95% CI 1.05–1.96). Higher histological grades, UICC-grades and TNM-stages were related to a higher mortality risk. Differing histological subtypes had significant influence on overall survival and progression-free survival. Patients diagnosed with fibromyxoid sarcoma, rhabdomyosarcoma and angiosarcoma were related to higher mortality risk compared to other histological subtypes (fibromyxoid sarcoma: HR 5.2, 95% CI 0.71–38.1; rhabdomyosarcoma: HR 2.93, 95% CI 1.44–6.00; angiosarcoma: HR 1.07, 95% CI 0.53–2.18). Conclusions Histological grade, tumor size, nodal and distant metastasis, tumor localization and histological subtype were determined as prognostic factors in terms of survival.

Soft Tissue Sarcomas with Chromosomal Alterations in the 12q13-15 Region: Differential Diagnosis and Therapeutic Implications

“…Despite their rarity, sarcomas carry a large disease burden in the pediatric and adult populations and are a significant cause of cancer deaths during the first 20 years of life [2]. There are numerous genetic syndromes that increase the risk of developing sarcomas, including neurofibromatosis type 1, Maffucci's syndrome, Li-Fraumeni syndrome, and McCune-Albright syndrome [2][3][4][5]. In addition, exposure to radiation is an established risk factor for sarcomas, and immune suppression has also been implicated in the development of sarcomas [2][3][4][5].…”

“…There are numerous genetic syndromes that increase the risk of developing sarcomas, including neurofibromatosis type 1, Maffucci's syndrome, Li-Fraumeni syndrome, and McCune-Albright syndrome [2][3][4][5]. In addition, exposure to radiation is an established risk factor for sarcomas, and immune suppression has also been implicated in the development of sarcomas [2][3][4][5]. Multiple environmental risk factors for sarcoma development exist, but drawing definitive conclusions regarding the association between risk factors and sarcomas have proven to be challenging [3][4][5].…”

“…In addition, exposure to radiation is an established risk factor for sarcomas, and immune suppression has also been implicated in the development of sarcomas [2][3][4][5]. Multiple environmental risk factors for sarcoma development exist, but drawing definitive conclusions regarding the association between risk factors and sarcomas have proven to be challenging [3][4][5]. Soft tissue sarcomas may arise in extremities, abdomen/retroperitoneum, trunk, head, and neck area [2][3][4][5].…”

“…Multiple environmental risk factors for sarcoma development exist, but drawing definitive conclusions regarding the association between risk factors and sarcomas have proven to be challenging [3][4][5]. Soft tissue sarcomas may arise in extremities, abdomen/retroperitoneum, trunk, head, and neck area [2][3][4][5]. The majority of patients with soft tissue sarcomas present with a painless mass, although pain is noted at presentation in up to a third of cases [2][3][4][5].…”

“…Soft tissue sarcomas may arise in extremities, abdomen/retroperitoneum, trunk, head, and neck area [2][3][4][5]. The majority of patients with soft tissue sarcomas present with a painless mass, although pain is noted at presentation in up to a third of cases [2][3][4][5]. Delay in the diagnosis of sarcomas is common, with the most common incorrect diagnosis for extremity and trunk lesions being hematoma, cystic, or benign adipocytic tumor [3][4][5].…”