scholarly journals Dermatofibrosarcoma protuberans of the breast: A rare diagnosis requiring aggressive initial surgical therapy

2018 ◽  
Vol 4 (2) ◽  
pp. 25
Author(s):  
Navin Sookar ◽  
Jacinta Bisnath ◽  
Jameel Ali
Keyword(s):  
Soft Tissue ◽  
Local Recurrence ◽  
Surgical Therapy ◽  
Soft Tissue Sarcomas ◽  
Dermatofibrosarcoma Protuberans ◽  
High Rate ◽  
Incisional Biopsy ◽  
Positive Margins ◽  
Dermal Layer ◽  
Rare Diagnosis

Dermatofibrosarcoma protuberans (DFSP) is an extremely rare cutaneous neoplasm of the dermal layer of the skin and is histologically classified as a sarcoma. DFSP itself has an incidence of 0.8 cases per million annually. It accounts for 1% of all soft tissue sarcomas and less than 0.1% of all malignancies. DFSP has a high rate of local recurrence especially if there are positive margins on excision, but only up to 4% develop metastasis. Most reported cases are located in the trunk, extremities and head especially scalp. However, DFSP of the breast is very rare with very few reported in the literature. We report a case of a 33-year-old woman who presented with a histological diagnosis of DFSP of the breast, based on incisional biopsy. We report this case which highlights the important aspects in evaluating DFSP in the breast as well as its treatment.

Myxofibrosarcoma: clinical and prognostic value of MRI features

2020 ◽  
Vol 16 ◽  
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

scholarly journals Sarcomas of the extremities and the pelvis: comparing local recurrence after incisional and after core-needle biopsy

2022 ◽  
Vol 20 (1) ◽  
Author(s):  
Alexander Klein ◽  
Christof Birkenmaier ◽  
Julian Fromm ◽  
Thomas Knösel ◽  
Dorit Di Gioia ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Local Recurrence ◽  
Core Needle Biopsy ◽  
Needle Biopsy ◽  
Tumor Resection ◽  
Soft Tissue Sarcomas ◽  
Wide Resection ◽  
Incisional Biopsy ◽  
Core Needle

Abstract Background The degree of contamination of healthy tissue with tumor cells during a biopsy in bone or soft tissue sarcomas is clearly dependant on the type of biopsy. Some studies have confirmed a clinically relevant contamination of the biopsy tract after incisional biopsies, as opposed to core-needle biopsies. The aim of our prospective study was to evaluate the risk of local recurrence depending on the biopsy type in extremity and pelvis sarcomas. Methods We included 162 patients with a minimum follow-up of 6 months after wide resection of extremity sarcomas. All diagnostic and therapeutic procedures were performed at a single, dedicated sarcoma center. The excision of the biopsy tract after an incisional biopsy was performed as a standard with all tumor resections. All patients received their follow-up after the conclusion of therapy at our center by means of regional MRI studies and, at a minimum, CT of the thorax to rule out pulmonary metastatic disease. The aim of the study was the evaluation of the influence of the biopsy type and of several other clinical factors on the rate of local recurrence and on the time of local recurrence-free survival. Results One hundred sixty-two patients with bone or soft tissue tumors of the extremities and the pelvis underwent either an incisional or a core-needle biopsy of their tumor, with 70 sarcomas (43.2%) being located in the bone. 84.6% of all biopsies were performed as core-needle biopsies. The median follow-up time was 55.6 months, and 22 patients (13.6%) developed a local recurrence after a median time of 22.4 months. There were no significant differences between incisional and core-needle biopsy regarding the risk of local recurrence in our subgroup analysis with differentiation by kind of tissue, grading of the sarcoma, and perioperative multimodal therapy. Conclusions In a large and homogenous cohort of extremity and pelvic sarcomas, we did not find significant differences between the groups of incisional and core-needle biopsy regarding the risk of local recurrence. The excision of the biopsy tract after incisional biopsy in the context of the definitive tumor resection seems to be the decisive factor for this result.

Status of Surgical Margins and Prognosis in Adult Soft Tissue Sarcomas of the Extremities: A Series of Patients Treated at a Single Institution

2005 ◽  
Vol 23 (1) ◽  
pp. 96-104 ◽  
Author(s):  
A. Gronchi ◽  
P.G. Casali ◽  
L. Mariani ◽  
R. Miceli ◽  
M. Fiore ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Local Recurrence ◽  
Soft Tissue Sarcomas ◽  
Hazard Ratio ◽  
Surgical Margins ◽  
Prognostic Impact ◽  
Positive Surgical Margins ◽  
Positive Margins ◽  
Prognostic Effect ◽  
Marginal Status

Purpose To explore the prognostic effect of microscopic marginal status after surgery for extremity soft tissue sarcomas. Patients and Methods We analyzed 911 consecutive patients surgically treated throughout a 20-year span at a single referral center. Six hundred forty-two were first seen with a primary tumor, and 269, with a locally recurrent tumor. All patients underwent macroscopically complete resection. Microscopic marginal status was negative (tumor size > 1 mm) in 748 patients and positive (≤ 1 mm) in 163 patients. Median follow-up was 107 months. Results Patients with primary disease had a lower disease-specific mortality in comparison to those first examined for recurrence (25% v 37%, respectively, at 10 years). Size, malignancy grade, depth, histotype, and local recurrence had a statistically significant prognostic effect at multivariable analysis, while microscopically positive surgical margins had not, though a trend in favor of negative margins was observed. However, an extra risk was observed for patients with positive margins after 3 to 5 years (hazard ratio, 1.8 after 5 years v 0.8 before 5 years). In patients treated for a local recurrence, the prognostic impact of positive margins was higher (hazard ratio, 1.6). Conclusion Positive surgical margins had a weak adverse prognostic effect, which was more pronounced for those patients escaping an early relapse. This would seem to justify a policy of surgical adequacy in adult soft tissue sarcomas, though clinical decision making in borderline presentations for conservative surgery might be reasonably flexible and shared with the patient. Once a local relapse has occurred, the impact of local treatments seems more critical.

scholarly journals Popliteal Fossa Sarcomas

2021 ◽  
Author(s):  
Ozgur Erdogan ◽  
Aykut Çelik ◽  
Ertuğrul Tekçe ◽  
Güray Altun ◽  
Korhan Ozkan ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Local Recurrence ◽  
Soft Tissue Sarcomas ◽  
Popliteal Fossa ◽  
Tumor Type ◽  
Fusiform Cell ◽  
Positive Margins ◽  
Large Patient ◽  
The Mean

Abstract Background and Aim: Soft tissue sarcomas of the popliteal fossa are extremely rare tumors of mesenchymal origin accounting for 3%–5% of all extremity sarcomas. However, data regarding the tumor type, neurovascular involvement, and administration of radiation therapy before or after resection are limited. This study aimed to report on popliteal fossa sarcomas analyzing data from two institutions based on a relatively large patient sample.Materials and Method: Twenty-four patients (80%; 9 men and 15 women) with a popliteal fossa soft tissue sarcoma were included in this study. The reviewed patient data included sex, age, duration of complaints, interval to diagnosis, radiology, pre- and postoperative biopsy, tumor histology, surgery type, complications, and pre- and postoperative oncologic and functional outcomes.Results: The mean age of the patients was 48 ± 21.23 (range 3–72) years at the time of diagnosis. The most common histological diagnoses were synovial sarcoma (6 patients), hemangiopericytoma (2 patients), soft tissue osteosarcoma (2 patients), fusiform cell sarcoma (2 patients), and myxofibrosarcoma (2 patients). Local recurrence after limb salvage was observed in six patients (25%). At the latest follow-up, 2 patients died of the disease, 2 patients were still alive with progressive lung disease and soft tissue metastasis, and the remaining 20 patients were free from the disease.Conclusions: Microscopically positive margins may not be an absolute indication for amputation. Also, negative margins do not provide a guarantee that local recurrence will not occur. Lymph node or distant metastasis may be predictive factors for local recurrence rather than positive margins.

scholarly journals 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 ◽  
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.

scholarly journals The Therapeutic Approach of Soft – Tissue Sarcomas. A 12 Cases Analysis

2018 ◽  
Vol 1 (Supplement) ◽  
pp. 58
Author(s):  
V. Georgeanu ◽  
T. Atasiei ◽  
D. Gartonea ◽  
B. Shazam ◽  
G. Goleşteanu ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Local Recurrence ◽  
Synovial Sarcoma ◽  
Therapeutic Approach ◽  
Clear Cell ◽  
Postoperative Radiotherapy ◽  
Soft Tissue Sarcomas ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma ◽  
Special Cases

Abstract Introduction. Soft Tissue Sarcomas (STS) is a group of rare malignant tumors with mesenchymal tissue origin. At present, over 50 histopathological types with typical chromosome changes are described. Treatment is multidisciplinary, centered on the surgical approach. Method. Between 2014 and 2016, 12 STS cases were treated in our clinic: 3 liposarcomas, 2 synovial sarcomas, 1 angiosarcoma, 2 rhabdomyosarcomas, 2 myxofibrosarcomas, 1 fibroblast sarcoma and 1 clear cell sarcoma. With the exception of angiosarcoma that benefited only from chemotherapy, the other cases were surgically approached, followed by radiotherapy in 7 cases, and chemotherapy in one case. Results. Patient follow-up was for a minimum of 2 years. In 3 cases (fibroblast sarcoma, clear-cell sarcoma, synovial sarcoma), a local recurrence occurred between 3 and 9 months. In 2 cases (fibroblastic sarcoma, synovial sarcoma), pulmonary metastases occurred between 6 and 18 months and the patients died at 8 months and 2 years respectively. The rest of the cases were clinically and imagistically assessed (local MRI, lung CT) at 6 months for at least 2 years and showed no signs of local recurrence or metastasis. Conclusions. The evolution of STS depends on the histological type and to the stage of diagnosis (local extension, degree of differentiation and presence of metastases). Therapeutic approach should be centered on an accurate resection, within the limits of oncological safety even with re-resection. Postoperative radiotherapy is used in most of the cases, and chemotherapy is reserved for special cases with unfavorable local evolution or metastasis.

Treatment-Induced Pathologic Necrosis: A Predictor of Local Recurrence and Survival in Patients Receiving Neoadjuvant Therapy for High-Grade Extremity Soft Tissue Sarcomas

2001 ◽  
Vol 19 (13) ◽  
pp. 3203-3209 ◽  
Author(s):  
Fritz C. Eilber ◽  
Gerald Rosen ◽  
Jeffery Eckardt ◽  
Charles Forscher ◽  
Scott D. Nelson ◽  
...  
Keyword(s):  
Overall Survival ◽  
Soft Tissue ◽  
Local Recurrence ◽  
Neoadjuvant Therapy ◽  
Independent Predictor ◽  
Survival Rates ◽  
Soft Tissue Sarcomas ◽  
High Grade ◽  
Recurrence Rates ◽  
Pathologic Assessment

PURPOSE: To determine whether treatment-induced pathologic necrosis correlates with local recurrence and overall survival in patients who receive neoadjuvant therapy for high-grade extremity soft tissue sarcomas. PATIENTS AND METHODS: Four hundred ninety-six patients with intermediate- to high-grade extremity soft tissue sarcomas received protocol neoadjuvant therapy. All patients underwent surgical resection after neoadjuvant therapy and had pathologic assessment of tumor necrosis in the resected specimens. RESULTS: The 5- and 10-year local recurrence rates for patients with ≥ 95% pathologic necrosis were significantly lower (6% and 11%, respectively) than the local recurrence rates for patients with less than 95% pathologic necrosis (17% and 23%, respectively). The 5- and 10-year survival rates for the patients with ≥ 95% pathologic necrosis were significantly higher (80% and 71%, respectively) than the survival rates for the patients with less than 95% pathologic necrosis (62% and 55%, respectively). Patients with less than 95% pathologic necrosis were 2.51 times more likely to develop a local recurrence and 1.86 times more likely to die of their disease as compared with patients with ≥ 95% pathologic necrosis. The percentage of patients who achieved ≥ 95% pathologic necrosis increased to 48% with the addition of ifosfamide as compared with 13% of the patients in all the other protocols combined. CONCLUSION: Treatment-induced pathologic necrosis is an independent predictor of both local recurrence and overall survival in patients who receive neoadjuvant therapy for high-grade extremity soft tissue sarcomas. A complete pathologic response (≥ 95% pathologic necrosis) correlated with a significantly lower rate of local recurrence and improved overall survival.

scholarly journals Analysis of Margin Classification Systems for Assessing the Risk of Local Recurrence After Soft Tissue Sarcoma Resection

2018 ◽  
Vol 36 (7) ◽  
pp. 704-709 ◽  
Author(s):  
Kenneth R. Gundle ◽  
Lisa Kafchinski ◽  
Sanjay Gupta ◽  
Anthony M. Griffin ◽  
Brendan C. Dickson ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Local Recurrence ◽  
Soft Tissue Sarcoma ◽  
Multidisciplinary Treatment ◽  
Residual Tumor ◽  
Classification Systems ◽  
Positive Margins ◽  
Risk Levels ◽  
R Classification ◽  
Margin Classification

Purpose To compare the ability of margin classification systems to determine local recurrence (LR) risk after soft tissue sarcoma (STS) resection. Methods Two thousand two hundred seventeen patients with nonmetastatic extremity and truncal STS treated with surgical resection and multidisciplinary consideration of perioperative radiotherapy were retrospectively reviewed. Margins were coded by residual tumor (R) classification (in which microscopic tumor at inked margin defines R1), the R+1mm classification (in which microscopic tumor within 1 mm of ink defines R1), and the Toronto Margin Context Classification (TMCC; in which positive margins are separated into planned close but positive at critical structures, positive after whoops re-excision, and inadvertent positive margins). Multivariate competing risk regression models were created. Results By R classification, LR rates at 10-year follow-up were 8%, 21%, and 44% in R0, R1, and R2, respectively. R+1mm classification resulted in increased R1 margins (726 v 278, P < .001), but led to decreased LR for R1 margins without changing R0 LR; for R0, the 10-year LR rate was 8% (range, 7% to 10%); for R1, the 10-year LR rate was 12% (10% to 15%) . The TMCC also showed various LR rates among its tiers ( P < .001). LR rates for positive margins on critical structures were not different from R0 at 10 years (11% v 8%, P = .18), whereas inadvertent positive margins had high LR (5-year, 28% [95% CI, 19% to 37%]; 10-year, 35% [95% CI, 25% to 46%]; P < .001). Conclusion The R classification identified three distinct risk levels for LR in STS. An R+1mm classification reduced LR differences between R1 and R0, suggesting that a negative but < 1-mm margin may be adequate with multidisciplinary treatment. The TMCC provides additional stratification of positive margins that may aid in surgical planning and patient education.

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