Adult-Type Soft Tissue Sarcomas in Pediatric-Age Patients: Experience at the Istituto Nazionale Tumori in Milan

Purpose Nonrhabdomyosarcoma soft tissue sarcomas are a heterogeneous group of tumors for which optimal treatment remains controversial. We report on a large group of 182 patients younger than 18 years old treated at a single institution over a 25-year period. Patients and Methods In this relatively homogeneous subgroup of adult-type histotypes, surgery was the mainstay of treatment; radiotherapy was administered to 73 patients, and chemotherapy was administered to 114 patients (70 received chemotherapy as adjuvant therapy). Results Overall survival at 5 years was 89% in patients who underwent complete resection at diagnosis, 79% in patients who had marginal resection, 52% in initially unresected patients, and 17% in patients with metastases at onset. Outcome was unsatisfactory in patients with large and high-grade tumors, even after gross resection; adjuvant chemotherapy seemed to improve the results in this group. Initially unresected patients who responded well to chemotherapy and subsequently underwent complete resection had an event-free survival rate of approximately 70%. The rate of response to chemotherapy was 58%. Conclusion The identification of prognostic variables should enable risk-adapted therapies to be planned. Patients with initially unresectable disease and patients with resected large and high-grade tumors are at high risk of metastases and treatment failure. Although the limits of this retrospective analysis are self-evident, our data would suggest that intensive chemotherapy (with an ifosfamide-doxorubicin regimen) might have a more significant role in these patients than what is generally assumed.

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A non-comparative phase II study of dose intensive chemotherapy with doxorubicin and ifosfamide followed by high dose ICE consolidation with PBSCT in non-resectable, high grade, adult type soft tissue sarcomas

Investigational New Drugs ◽

10.1007/s10637-013-0027-8 ◽

2013 ◽

Vol 31 (6) ◽

pp. 1592-1601 ◽

Cited By ~ 3

Author(s):

Jörg Thomas Hartmann ◽

M. Horger ◽

T. Kluba ◽

A. Königsrainer ◽

P. de Zwart ◽

...

Keyword(s):

Soft Tissue ◽

Phase Ii ◽

Phase Ii Study ◽

Soft Tissue Sarcomas ◽

High Dose ◽

High Grade ◽

Intensive Chemotherapy ◽

Adult Type

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Long-term results of a prospective randomized trial of adjuvant brachytherapy in soft tissue sarcoma.

Journal of Clinical Oncology ◽

10.1200/jco.1996.14.3.859 ◽

1996 ◽

Vol 14 (3) ◽

pp. 859-868 ◽

Cited By ~ 676

Author(s):

P W Pisters ◽

L B Harrison ◽

D H Leung ◽

J M Woodruff ◽

E S Casper ◽

...

Keyword(s):

Soft Tissue ◽

Soft Tissue Sarcoma ◽

Local Control ◽

Distant Metastasis ◽

Soft Tissue Sarcomas ◽

Complete Resection ◽

High Grade ◽

Disease Specific Survival ◽

Recurrence Rates ◽

Disease Specific

PURPOSE This trial was performed to evaluate the impact of adjuvant brachytherapy on local and systemic recurrence rates in patients with soft tissue sarcoma. PATIENTS AND METHODS In a single-institution prospective randomized trial, 164 patients were randomized intraoperatively to receive either adjuvant brachytherapy (BRT) or no further therapy (no BRT) after complete resection of soft tissue sarcomas of the extremity or superficial trunk. The adjuvant radiation was administered by iridium-192 implant, which delivered 42 to 45 Gy over 4 to 6 days. The two study groups had comparable distributions of patient and tumor factors, including age, sex, tumor site, tumor size, and histologic type and grade. RESULTS With a median follow-up time of 76 months, the 5-year actuarial local control rates were 82% and 69% in the BRT and no BRT groups (P = .04), respectively. Patients with high-grade lesions had local control rates of 89% (BRT) and 66% (no BRT) (P = .0025). BRT had no impact on local control in patients with low-grade lesions (P = .49). The 5-year freedom-from-distant-recurrence rates were 83% and 76% in the BRT and no BRT groups (P = .60), respectively. Analysis by histologic grade did not demonstrate an impact of BRT on the development of distant metastasis, despite the improvement in local control noted in patients with high-grade lesions. The 5-year disease-specific survival rates for the BRT and no BRT groups were 84% and 81% (P = .65), respectively, with no impact of BRT regardless of tumor grade. CONCLUSION Adjuvant brachytherapy improves local control after complete resection of soft tissue sarcomas. This improvement in local control is limited to patients with high-grade histopathology. The reduction in local recurrence in patients with high-grade lesions is not associated with a significant reduction in distant metastasis or improvement in disease-specific survival.

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The Effect of Resection Margin on Local Recurrence and Survival in High Grade Soft Tissue Sarcoma of the Extremities: How Far Is Far Enough?

Cancers ◽

10.3390/cancers12092560 ◽

2020 ◽

Vol 12 (9) ◽

pp. 2560

Author(s):

Annika Bilgeri ◽

Alexander Klein ◽

Lars H. Lindner ◽

Silke Nachbichler ◽

Thomas Knösel ◽

...

Keyword(s):

Overall Survival ◽

Soft Tissue ◽

Local Recurrence ◽

Resection Margin ◽

Soft Tissue Sarcomas ◽

High Grade ◽

Recurrence Free Survival ◽

Free Survival ◽

Positive Margins ◽

Local Recurrence Free Survival

Background: The significance of surgical margins after resection of soft tissue sarcomas in respect to local-recurrence-free survival and overall survival is evaluated. Methods: A total of 305 patients with deep-seated, G2/3 soft tissue sarcomas (STS) of the extremity, the trunk wall, or the pelvis were reviewed. The margin was defined according to the Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) classification system (R0-2), the Union Internationale Contre le Cancer (UICC) classification (R + 1 mm) for which a margin <1 mm is included into the R1 group, and in groups of <1 mm, 1–5 mm, >5 mm, or >10 mm. Results: Of these patients, 31 (10.2%) had a contaminated margin, 64 (21%) a margin of <1 mm, 123 (40.3%) a margin of 1–5 mm, 47 (15.4%) a margin of >5 mm, and 40 (13.1%) a margin of >10 mm. The 5-year local recurrence-free survival (LRFS) was 81.6%. Overall survival (OS) at 5 years was 65.9%. Positive margins worsened LRFS and OS. A margin of >10 mm did not improve LRFS and OS as compared to one of >5 mm. Conclusions: A resection margin of <1 mm showed a trend but not significantly better LRFS or OS compared to a contaminated margin. This finding supports use of the UICC classification. A margin of more than 10 mm did not improve LRFS or OS.

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Clinical Significance of Additional Wide Resection for Unplanned Resection of High Grade Soft Tissue Sarcoma

The Open Orthopaedics Journal ◽

10.2174/1874325000802010126 ◽

2008 ◽

Vol 2 (1) ◽

pp. 126-129 ◽

Cited By ~ 14

Author(s):

T Morii ◽

H Yabe ◽

H Morioka ◽

U Anazawa ◽

Y Suzuki ◽

...

Keyword(s):

Soft Tissue ◽

Soft Tissue Sarcoma ◽

Soft Tissue Sarcomas ◽

Wide Resection ◽

High Grade ◽

Event Free Survival ◽

Free Survival ◽

Musculoskeletal Sarcoma ◽

Oncological Outcomes ◽

Local Recurrence Free Survival

Purpose: Unplanned resection of musculoskeletal sarcoma involves tumor excision without any suspicion of malignancy or regard for the necessity of defining adequate margins. For orthopaedic oncologists, many opportunities arise for management of unplanned resections initially performed by non-specialist surgeons. The puropose of this study is to assess the clinical outcomes and the problems of the patients with unplanned resection of high-grade soft tissue sarcoma. Methods: 77 consecutive patients were retrospectively reviewed. Oncological outcomes together with validity and problems of additional treatments were analyzed. Results: Five-year local recurrence-free survival, metastasis-free survival, event-free survival and total survival were 71.55%, 73.2%, 57.5% and 85.9%, respectively. Among adjuvant therapy including additional wide resection, radiotherapy and systemic chemotherapy, only additional wide resection significantly improved oncological outcomes. Conclusion: Additional wide resection appears to be effective in the treatment of high-grade soft tissue sarcomas following primary resection with compromised margins of resection.

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Efficacy and Safety of Hypofractionated Preoperative Radiotherapy for Primary Locally Advanced Soft Tissue Sarcomas of Limbs or Trunk Wall

Cancers ◽

10.3390/cancers13122981 ◽

2021 ◽

Vol 13 (12) ◽

pp. 2981

Author(s):

Hanna Koseła-Paterczyk ◽

Paweł Teterycz ◽

Mateusz J. Spałek ◽

Aneta Borkowska ◽

Anna Zawadzka ◽

...

Keyword(s):

Soft Tissue ◽

Multimodal Therapy ◽

Preoperative Radiotherapy ◽

Locally Advanced ◽

Soft Tissue Sarcomas ◽

High Grade ◽

Free Survival ◽

Phase 2 Trial ◽

Median Tumor Size ◽

Good Local Control

Background: The use of adjuvant radiotherapy (RT) shows a significantly decreased incidence of local recurrence (LR) in soft tissue sarcomas (STS). This study aimed to assess the treatment scheme’s effect in patients with primary STS treated at one institution. Methods: In this phase 2 trial, 311 patients aged ≥18 years with primary, locally advanced STS of the extremity or trunk wall were assigned to multimodal therapy conducted at one institution. The preoperative RT scheme consisted of 5 Gy per fraction for a total dose of 25 Gy. Surgery was performed within 2–4 days from the last day of RT. The primary endpoint was LR-free survival (LRFS). Adverse events of the treatment were assessed. Results: We included 311 patients with primary locally advanced STS. The median tumor size was 11 cm. In total, 258 patients (83%) had high-grade tumors. In 260 patients (83.6%), clear surgical margins (R0) were obtained. Ninety-six patients (30.8%) had at least one type of treatment adverse event. LR was observed in 13.8% patients. The 5-year overall survival was 63%. Conclusion: In this group, with a significant percentage of patients with extensive, high-grade STS, hypofractionated preoperative RT was associated with good local control and tolerance.

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Myxofibrosarcoma: clinical and prognostic value of MRI features

Current Medical Imaging Formerly Current Medical Imaging Reviews ◽

10.2174/1573405616999200729152135 ◽

2020 ◽

Vol 16 ◽

Cited By ~ 1

Author(s):

Paolo Spinnato ◽

Andrea Sambri ◽

Tomohiro Fujiwara ◽

Luca Ceccarelli ◽

Roberta Clinca ◽

...

Keyword(s):

Soft Tissue ◽

Local Recurrence ◽

Contrast Enhancement ◽

Imaging Modality ◽

Soft Tissue Sarcomas ◽

The Elderly ◽

High Rate ◽

Imaging Features ◽

High Grade ◽

Mri Features

: Myxofibrosarcoma is one of the most common soft tissue sarcomas in the elderly. It is characterized by an extremely high rate of local recurrence, higher than other soft tissue tumors, and a relatively low risk of distant metastases.Magnetic resonance imaging (MRI) is the imaging modality of choice for the assessment of myxofibrosarcoma and plays a key role in the preoperative setting of these patients.MRI features associated with high risk of local recurrence are: high myxoid matrix content (water-like appearance of the lesions), high grade of contrast enhancement, presence of an infiltrative pattern (“tail sign”). On the other hand, MRI features associated with worse sarcoma specific survival are: large size of the lesion, deep location, high grade of contrast enhancement. Recognizing the above-mentioned imaging features of myxofibrosarcoma may be helpful to stratify the risk for local recurrence and disease-specific survival. Moreover, the surgical planning should be adjusted according to the MRI features

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Functional and psychosocial effects of multimodality limb-sparing therapy in patients with soft tissue sarcomas.

Journal of Clinical Oncology ◽

10.1200/jco.1989.7.9.1217 ◽

1989 ◽

Vol 7 (9) ◽

pp. 1217-1228 ◽

Cited By ~ 28

Author(s):

A E Chang ◽

S M Steinberg ◽

M Culnane ◽

M H Lampert ◽

A J Reggia ◽

...

Keyword(s):

Radiation Therapy ◽

Soft Tissue ◽

Sexual Activity ◽

Standardized Test ◽

Soft Tissue Sarcomas ◽

High Grade ◽

Postoperative Radiation ◽

Limb Function ◽

Postoperative Radiation Therapy ◽

Global Quality Of Life

We have documented functional and psychosocial changes in patients with extremity soft tissue sarcomas who have undergone multimodality limb-sparing treatments. In 88 patients, parameters related to economic status, sexual activity, pain, limb function, and global quality of life (QOL) were recorded prior to surgery and every 6 months postoperatively. Changes from the preoperative assessment for every parameter were analyzed in each patient. Six months after surgery, there was a decrease in employment status, sexual activity, and in limb function in a significant number of patients. At 12 months, these decreases were still evident. Despite these changes, global QOL measured by a standardized test showed at least some improvement in a significant proportion of patients at 12 months. These findings highlight the difficulty in defining QOL. It could not be ascertained if radiation therapy and/or chemotherapy were causative factors in specific changes because of the small numbers of patients in each subgroup. However, among 60 patients with high-grade sarcomas, significant wound problems developed in 10 of 33 who received postoperative radiation therapy in combination with adjuvant doxorubicin and cyclophosphamide chemotherapy compared with one of 27 patients who received adjuvant chemotherapy alone (P = .016). Also, among high-grade sarcoma patients with 12-month follow-up, six of 19 patients who received radiation therapy and chemotherapy developed joint contractures compared with zero of 15 patients who received chemotherapy alone (P less than .04). The combination of postoperative radiation therapy and chemotherapy appeared to be associated with significantly more tissue-related injury in patients with high-grade sarcomas compared with chemotherapy alone.

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