Indication and surgical approach for reconstruction with endoprosthesis in bone-associated soft tissue sarcomas:Appropriate case management is vital

It is important for surgeons performing sarcoma surgery to know that bone resection and tumor prosthesis applications in soft tissue sarcomas(STS)have unique features in terms of indication,surgical approach and follow-up,in terms of the management of these cases.Some STS are associated with bone and major neurovascular structures.Bone-associated STS are generally relatively large and relatively deep-seated.Additionally,the tendency for metastasis is high.In some cases,the decision about which structures to resect is difficult.These cases are often accompanied by poor oncological and surgical outcomes.Management of cases should be done by a multidisciplinary team in advanced centers specialized in this field.The surgical team must have sufficient knowledge and experience in the field of limb-sparing surgery.Preoperative evaluation and especially good planning of bone and soft tissue reconstruction are vital.

Adjuvant therapy for orbital non-rhabdomyosarcoma soft tissue sarcoma:comparison of long-term outcome between radiotherapy and chemotherapy

AIM:To illustrate clinicopathological features of orbital non-rhabdomyosarcoma soft tissue sarcoma(NRSTS),and to compare the treatment outcome between postoperative radiotherapy(RT) and chemotherapy in a retrospective analysis nearly 20y.METHODS:A retrospective cohort study of 56 patients with orbital NRSTS were reviewed,34 of whom received postoperative RT,and 22 received postoperative chemotherapy.The clinicopathological features,local recurrence,metastases,and survival data were recorded.Survival analysis was performed using the Kaplan-Meier method.RESULTS:During follow-up(111.8mo,ranged 8-233mo) for 56 patients,19 patients of them developed local recurrence,and 7 patients developed distant metastases.Fifteen patients died during follow-up period.Overall survival rates considering the whole study group was 78.57% at 5y,and 72.16% at 10y after the initial diagnosis.Compared with chemotherapy,RT was associated with lower risk of local recurrence [hazard ratio for RT vs chemotherapy,0.263,95% confidence interval(CI),0.095-0.728,P=0.0015];with lower risk of distant metastasis(hazard ratio for RT vs chemotherapy,0.073,95%CI,0.015-0.364,P=0.0014);and with lower risk of death from disease(hazard ratio for RT vs chemotherapy,0.066,95%CI,0.022-0.200,P<0.0001).The 5-year survival rate in RT group was 97.06% compared to 50% in chemotherapy group.CONCLUSION:In patients with orbital NRSTS,postoperative RT provides better control of local recurrence,distant metastasis,and death from disease than chemotherapy.RT is the more preferrable adjuvant therapy compared to chemotherapy possibly.

New trends in the surgical management of soft tissue sarcoma: The role of preoperative biopsy

Soft tissue sarcoma(STS)accounts for 1%of all malignant neoplasms in adults.Their diagnosis and management constitute a challenging target.They originate from the mesenchyme,and 50 subtypes with various cytogenetic profiles concerning soft tissue and bones have been recognized.These tumors mainly affect middle-aged adults but may be present at any age.Half of the patients have metastatic disease at the time of diagnosis and require systemic therapy.Tumors above 3-5 cm in size must be suspected of potential malignancy.A thorough history,clinical examination and imaging that must precede biopsy are necessary.Modern imaging techniques include ultrasound,computed tomography(CT),new magnetic resonance imaging(MRI),and positron emission tomography/CT.MRI findings may distinguish low-grade from high-grade STS based on a diagnostic score(tumor heterogeneity,intratumoral and peritumoral enhancement).A score≥2 indicates a high-grade lesion,and a score≤1 indicates a lowgrade lesion.For disease staging,abdominal imaging is recommended to detect early abdominal or retroperitoneal metastases.Liquid biopsy by detecting genomic material in serum is a novel diagnostic tool.A preoperative biopsy is necessary for diagnosis,prognosis and optimal planning of surgical intervention.Core needle biopsy is the most indicative and effective.Its correct performance influences surgical management.An unsuccessful biopsy means the dissemination of cancer cells into healthy anatomical structures that ultimately affect resectability and survival.Complete therapeutic excision(R0)with an acceptable resection margin of 1 cm is the method of choice.However,near significant structures,i.e.,vessels,nerves,an R2 resection(macroscopic margin involvement)preserving functionality but having a risk of local recurrence can be an acceptable choice,after informing the patient,to prevent an unavoidable amputation.For borderline resectability of the tumor,neoadjuvant chemo/radiotherapy has a place.Likewise,after surgical excision,adjuvant therapy is indicated,but chemotherapy in nonmetastatic disease is still debatable.The five-year survival rate reaches up to 55%.Reresection is considered after positive or uncertain resection margins.Current strategies are based on novel chemotherapeutic agents,improved radiotherapy applications to limit local side effects and targeted biological therapy or immunotherapy,including vaccines.Young age is a risk factor for distant metastasis within 6 mo following primary tumor resection.Neoadjuvant radiotherapy lasting 5-6 wk and surgical resection are indicated for highgrade STS(grade 2 or 3).Wide surgical excision alone may be acceptable for patients older than 70 years.However,locally advanced disease requires a multidisciplinary task of decision-making for amputation or limb salvage.

Soft Tissue Sarcomas: Descriptive Study of 232 Cases Collected, over a Period of 10 Years, at the Hospital of Oncology, Department of the Hassan II Hospital FEZ

Soft-tissue sarcomas are uncommon tumors that have traditionally been managed by wide excisional surgery and radiotherapy;the use of chemotherapy has been reserved for advanced disease. Advances in multidisciplinary care have improved the evaluation and care of patients with this disease. Limb-conserving surgical paradigms, superior radiotherapy delivery, and novel adjuvant agents for specific tumors are now available. The objective of this study is to report the epidemiological, clinical, histological, therapeutic and evolutionary characteristics of soft tissue sarcomas at the oncology hospital: Chu Hassan II in FEZ, and to define the factors influencing patient survival. This is a retrospective study of 232 cases of soft tissue sarcoma, collected between January 2010 and June 2020. The eligibility criteria were an age greater than 16 years, and histological evidence of a soft tissue sarcoma excluding gastrointestinal stromal tumors (GIST). Items collected were: epidemiological, clinical, histological, radiological, and therapeutic. These are 232 cases, 120 Men and 112 Women, the mean age was 48.89 years (Extreme = 18 - 76 years). The tumor was localized to the extremities at (58.72%). The predominant histological type was Leiomyosarcoma in 61 cases (26.29%). The tumor stage was localized in (17.67%) of cases, locally advanced in 34.05% and metastatic in 44.08% of patients, all localized cases were treated surgically including (84%) conservative surgery and (16%) radical surgery. Radiation therapy was performed in 32.75% of patients. Chemotherapy was performed in 74.14% of patients. Age and tumor stage are prognostic factors influencing the survival of soft tissue sarcomas.

HIGH DOSE IFOSFAMIDE, DOXORUBICIN, DACARBAZINE AND G-CSF FOR PATIENTS WITH METASTATIC OR LOCALLYADVANCED SOFT TISSUE SARCOMA

Objective: A pilot study to test the feasibility and efficacy of high dose IFO and standard dose ADR and DTIC with G-CSF support in treatment of advanced soft tissue sarcoma (STS). Methods: 35 patients of no prior chemotherapy with metastatic or locally advanced unresectable STS were treated by this regimen, including 18 rhabdomyosarcomas, 7 malignant fibrous histiocytomas, 2 neurofibrosarcomas, 2 fibrosarcomas, 2 leiomyosarcomas, 2 synoviosarcomas, and 2 malignant hemangiopericytomas. IFO dose was 2 g/m2 on day 1–5 (with mesna uroprotection), ADR 50mg/m2 on day 1 and DTIC 250 mg/m2 on day 1–5. G-CSF (2 μg/kg/d) was administered on day 6 to 15 or until recovery of leukocytes account. The cycles were repeated every 3 weeks. Result: There were five complete responses (CR including pathologic CR) and eleven partial responses for overall 46% objective response rate. Most responses were observed within two cycles. The median survival was 15 months. Following CR, two patients remain disease free at 45 and 28 months, respectively. 6/120 (5%) cycles were complicated by grade IV neutropenia, 46/120 (38%) cycles had grade III neutropenia. No patients had treatment-related deaths. Nonhematologic toxicity consisted predominantly of anorexia and vomiting. No other severe toxicities were seen, especially no severe cardiotoxicity. Conclusion: This regimen is well tolerated and has substantial benefits for patients with advanced soft tissue sarcomas.

Fascia Lata Donor Site Foreign Body Granuloma: An Unusual Presentation of a Rare Mimic of Soft Tissue Sarcoma

Background: Foreign body granuloma is a rare late complication of fascia lata graft donor site with few reported cases in the literature. It can mimic soft tissue sarcoma. Clinical and radiological findings may not be enough to solve the puzzle and histology remains the mainstay of diagnosis. Aim: Our aim is to highlight the unusual long interval between initial surgery and presentation of foreign body granuloma. Case Presentation: A 65-year-old man who presented 48 years after initial surgery with progressive painless fascia lata donor site soft tissue swelling. The management and histology findings were highlighted. Conclusion: The occurrence of foreign body granuloma remains an important differential following a history of past surgical procedure regardless of the time interval between the surgical procedure and the clinical presentation.

Abdominal metastases of primary extremity soft tissue sarcoma: A systematic review

BACKGROUND Despite the fact that about one third of patients with primary localized extremity soft tissue sarcoma(e STS)will develop metastatic disease,abdominal metastases(AM)and retroperitoneal metastases(RM)constitute rare events.There is no clear consensus on how to achieve follow-up on patients with primary localized e STS following curative resection,especially regarding the surveillance of potential AM/RM.AIM To systematically analyse incidence,diagnosis,treatment and outcome of AM/RM in e STS patients.METHODS In this systematic review,899 studies available in Pub Med and published between 2000 and 2018 were screened,identifying 17 original articles focused on AM or RM in e STS.Article selection was based on the PRISMA guidelines,using the search terms(abdominal metastasis AND soft tissue sarcoma)and(soft tissue sarcoma metastasis abdomen).All studies published between January 1,2000 and December 31,2018 were screened.Further articles were identified by crosssearching article references,with the final search date being February 18,2019.Due to limited data and the different reporting techniques used,the present review focused on descriptive analysis of the included studies.RESULTS Of the 17 studies included,six original articles reported on incidence±diagnosis,therapy and outcome in AM and RM,whilst three original and eight case reports focused on diagnostic pathway,therapeutic procedures or outcomes without allowing conclusions regarding incidence of AM and RM.According to the former six studies,incidence of AM ranged from 0.9%-5.6%in patients with miscellaneous histological subtypes,and up to 12.1%in patients with myxoid liposarcoma.The most common histological subtypes that developed AM or RM were(myxoid)liposarcoma and leiomyosarcoma,but also rare subtypes such asepithelioid sarcoma,myxofibrosarcoma,synovial sarcoma,and malignant peripheral nerve sheath tumour had been reported to develop AM/RM.Surgery for AM/RM was performed in five of eight case-reports(62.5%)and in 20.8%-100.0%of original articles.In particular,patients with hepatic metastases undergoing metastasectomy had a survival benefit compared to patients treated with chemotherapy or best supportive care(>3 years vs<6 mo).CONCLUSION Patients with e STS should undergo surveillance with abdominal ultrasonography/computed tomography,or even whole-body-magnetic resonance imaging to detect AM/RM at an early stage.

Clinical analysis of 251 cases of soft tissue sarcomas

Objective To explore the therapeutic principles and prognostic factors of soft tissue sarcoma. Methods Two hundred and fifty-one patients with soft tissue sarcoma (STS) treated at Shanghai Cancer Hospital during 1986 -1990 were reviewed retrospectively. Results The 1-, 3 - , 5 - , 10 - year tumor-free survival rates were 67. 74% , 57. 16% , 52, 41% , 38. 60% , respectively.The overall survival rates for 1,3,5 and 10 years were 81. 01 % ,67.75% ,60.79% ,and 49.23% respectively. Log-rank test showed that the patients with different pathological findings, histological grades,mass location and size, anatomical depth, and surgical margin showed different outcomes. Whether the sarcomas invaded the vessels or metastasized would influence the survival rates. The patients who underwent different interventions or operations also had different outcomes. The prognosis of STS was associated with age,histological type,histological grade, tumor size, surgical margin and metastasis according to the Cox regression

Analysis of soft tissue sarcomas in 1118 cases

Background It is important to analyze and compare soft tissue sarcomas periodically so as to update the incidence, the clinical diagnosis, the treatment, and the ongoing research. The present study was conducted to determine the relative frequency of each type of soft tissue sarcoma.Methods A total of 1118 cases of primary soft tissue sarcomas treated between January 1993 and December 2006 were evaluated in a retrospective analysis.Results According to the pathologic grouping, the diseases with the highest proportion were malignant fibrous histiocytomas （35.24%）, synovial sarcomas （17.08%）, liposarcomas （16.28%）, and rhabdomyosarcomas （12.61%）. Soft tissue sarcomas were detected in every age group and occurred in all parts of the body. The number of cases increased gradually over the years.Conclusions Malignant fibrous histiocytomas had the highest frequency among the soft tissue sarcomas. The number of cases increased gradually over the years.

Undifferentiated high-grade pleomorphic sarcoma of the common bile duct:A case report and review of literature

BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a rare malignant mesenchymal tumor with a poor prognosis.It mainly occurs in the extremities,trunk,head and neck,and retroperitoneum regions.Owing to the lack of specific clinical manifestations and imaging features,UPS diagnosis mainly depends on pathological and immunohistochemical examinations for exclusive diagnosis.Here we report an extremely rare case of high-grade UPS in the common bile duct(CBD).There are limited available data on such cases.CASE SUMMARY A 70-year-old woman was admitted to our department with yellow eyes and urine accompanied by upper abdominal distending pain for 2 wk.Her laboratory data suggested significantly elevated hepatorenal function levels.The imaging data revealed calculous cholecystitis,intrahepatic and extrahepatic bile duct dilation with extrahepatic bile duct calculi,and a space-occupying lesion at the distal CBD.After endoscopic biliary stenting and symptomatic support therapy,CBD exploration and biopsy were performed.The frozen section indicated malignant spindle cell tumor of the CBD mass,and further radical pancreaticoduodenectomy was performed.Finally,the neoplasm was diagnosed as a high-grade UPS combined with the light-microscopic morphology and immunohistochemical results.CONCLUSION This extremely rare case highlighted the need for increasing physicians'vigilance,reducing the odds of misdiagnosis,and providing appropriate treatment strategies.

Clear cell sarcoma of the pancreas,an unusual cancer with unusual metastatic site or unusual primary site?

Clear cell sarcoma(CCS)is a type of malignant tumor that can arise from tendons and aponeuroses.This malignant proliferation of cells with melanocytic lineage normally occurs in young patients,and it is normally identified in extremities.However,different sites including gastrointestinal organs are also described.Due difficulties in the molecular and histopathology evaluation,the diagnosis is often confused with malignant melanoma.Most cases are treated with surgical resection,but overall,the prognosis is poor.In this editorial,we will discuss a very interesting case of CCS identified in the pancreas.We will discuss the literature and controversies in the management of this type of cancer.Furthermore,we will address molecular strategies to be incorporated in those cases to better understand the primary location of the tumor.Finally,future perspectives of the field and new strategies of treatment will be described.

INVESTIGATION OF RELATIONSHIPS BETWEEN KI－67SCORE，DNA INDEX，AND HISTOLOGIC GRADEIN SOFT TISSUE SARCOMAS

Proliferative activity of soft tissue sarcomas(STS)in 31 cases was estimated by histologic grading, mitotic count, DNA analysis by flow cytometry, and immunohistochemical procedures with monoclonal antibody Ki-67.Aneuploid was found in 12 of 16 cases(75.0%) with Grade 3, and in 4 of 15 cases(26.7%) with Grade 1, 2(P=0.0121).Tumors with more than 100 Ki-67 positive tumor cells per 10 high power fields(HPF) had a higher rate of aneuploid(81.3%) than those with less than 100 Ki-67 per 10 HPF (26.7%)(P=0.0038). There were significant correlations between Grade and DI (r=0.4901, P=0.0051), Grade and Ki-67(r=0.4636, P=0.0086), Ki-67 and DI(r=0-6368, P=0.0001). The results indicate that DI and reactivity of tumor cells to Ki-67 may reflect Proliferative activity and be helpful for clinicians to judge the biological behaviour of tumors more accurately and objectively. Supplementary to the grading of STS, DI and Ki-67 score could be useful as Prognostic parameters for clinical investigation of multimodality therapy for individual Patients.

THE CLINIC－PATHOLOGIC ANALYSIS OF 66 CASES WITH SOFT TISSUE SARCOMA OF ABDOMEN

We accepted 66 cases with soft tissue sarcoma of abdomen testified by the Pathologists. The ratio of male to female was 1.5 to 1, but the aged-groups in female were more. The male incidences increased as the ages did. The mean age was 46.79 ±17. 04. The neoplasms arises more often in the abdominal wall below 30 years old, and it does mainly in the abdominal viscera and in the retroperitoneum over 30 years old. Fibrosarcoma Protuberance was the main type in the abdominal wall,and leiomyosarcoma was the main type in the abdominal viscera, and in the retroperitoneum, the most was liposarcoma, the following was leiomyosarcoma. Among the soft tissue sarcoma of the abdomen. the most was in retroperitoneum, and the following was in abdominal viscera. Therefore, it was difficult to diagnose early. The keys to the question were B-mode ultrasonic examining,computed tomography (CT) scanning and magnetic resonance imaging (MRI). Surgery was the primary therapeutic modality. The resectability was the major prognostic factor Radiotherapy and chemotherapy indicated a clear beneficial effect on soft tissue sarcoma of the abdomen and they can slightly decrease the recurrences and the metastases, but they had no obvious influence on the survival. In 5-year survival, the lesion of abdomen was the best, and those both of abdominal viscera and of retroperitoneum were the bad. To increase the 5-year survival, what counts are early diagnosis and the resectability.

Germline predisposition to soft tissue sarcoma

Soft tissue sarcoma(STS)most often occurs sporadically,but can also arise in the setting of a germline cancer predisposition syndrome(CPS).There is significant diversity amongst STS diagnoses as these tumors exhibit a variety of histologies,occur in all age groups,and can occur in any location in the body.This diversity is also reflected in the many known associated germline cancer predisposition associations.Some STS diagnoses,such as anaplastic rhabdomyosarcoma,are associated with high heritability and other STS,such as Ewing sarcoma,are notably absent from known CPS.Recognizing when a STS is more likely to be hereditary can influence clinical management.Individuals diagnosed with STS due to CPS may be at risk for other malignancies and should undergo additional surveillance for early detection.Additionally,family members should undergo genetic testing as they also may be at risk to develop STS and other CPS-associated malignancies.Some underlying cancer predisposition diagnoses may have implications for the treatment of a concurrent malignancy as in the case of PARP inhibitor therapy in the setting of homologous recombination deficiency.This review summarizes current knowledge of selected STS and their associations with CPS.

Immune checkpoint inhibitor resistance in soft tissue sarcoma

The emergence of immunotherapy as a cancer therapy has dramatically changed the treatment paradigm of systemic cancer therapy.There have been several trials evaluating immune checkpoint blockade(ICI)in soft tissue sarcoma.While there is generally a limited response in sarcoma,a subset of patients has durable responses to immunotherapy.This is attributable to a variety of factors including histologic subtype,tumor-infiltrating lymphocytes,and the tumor microenvironment among others.There is ongoing translational and clinical research evaluating ICI resistance in sarcoma and identifying therapeutic strategies to overcome this resistance.Herein,we provide a review of the current data,proposed mechanisms of resistance,and potential approaches to overcome this resistance.

The infl uence of national guidelines on soft tissue sarcoma patient outcome: a single center experience

Aim:The aim was to study the impact of nationwide clinical practice guidelines for soft tissue sarcomas(STS),introduced in 1994 and again in 2005 in North Savo,Finland.Methods:We retrospectively reviewed all the patients whose sarcoma was treated by a multidisciplinary team between the years 2000 and 2009 with mean follow-up time of 68 months.The patients were divided into 2 groups according to years:Group A(2000–2005,72 patients)and Group B(2006–2009,64 patients).Primary outcomes were local recurrence,metastases,and overall survival.Results:Fifty-five percent were men with an average age of 59 years.The most common sarcomas were pleomorphic sarcoma(37%)and liposarcoma(26%).Although there were significantly less amputations in Group B(A:15%,B:3%),there were more metastases(A:10%,B:23%)with an overall lower overall survival rate(A:70%,B:58%)than in Group A.Conversely,Group A had a higher 1st year survival rate(A:100%,B:87%).We found that upper limb sarcomas were more likely to be diagnosed with incisional biopsies,but there was no correlation between incisional biopsy and recurrence,metastases or survival.Conclusion:Due to nonadherence of the 2005 national treatment recommendations,there has been no improvement either in management or survival.The importance of educating guidelines to doctors referring patients to specialized units cannot be overemphasized to affect successful management in the treatment of STS.

Primary intracranial extraskeletal myxoid chondrosarcoma:A case report and review of literature

BACKGROUND Primary intracranial extraskeletal myxoid chondrosarcoma(EMC)is an extremely rare low-to intermediate-grade malignant soft tissue sarcoma,and only 15 cases have been reported in the literature.Due to its rarity,clinical data and research on this tumor type are extremely limited,the pathogenesis and histological origin are still unclear,and the diagnostic and standard clinical treatment strategies for intracranial EMC remain controversial and undefined.CASE SUMMARY We reported a case of a 52-year-old male who was admitted to the hospital with headache and dizziness for 1 mo,and his health status deteriorated during the last week.CT of the head showed a well-defined low-density lesion situated in the left cavernous sinus.Brain magnetic resonance imaging(MRI)showed a 3.4 cm×3.0 cm sized,well-defined,round-shaped and heterogeneously enhanced lesion located in the left cavernous sinus.The entire lesion was removed via supratentorial craniotomy and microsurgery.Postoperative pathological diagnosis indicated primary intracranial EMC.Subsequently,the patient underwent 45 Gy/15 F stereotactic radiotherapy after discharge.At present,it is 12 mo after surgery,with regular postoperative follow-up and regular MRI examinations,that there are no clinical symptoms and radiographic evidence indicating the recurrence of the tumor,and the patient has returned to normal life.CONCLUSION Currently,the most beneficial treatment for primary intracranial EMC is gross total resection combined with postoperative radiotherapy.Long-term follow-up is also necessary for patients.

National Comprehensive Cancer Network guidelines compliance of a sarcoma service:A retrospective review

BACKGROUND Clinical workup and treatment guidelines have been published by the National Comprehensive Cancer Network(NCCN)to ensure patients are treated uniformly and appropriately.This study sought to retrospectively review patients with a new diagnosis of sarcoma who were treated in a National Cancer Institute(NCI)designated center and determine compliance rates with guidelines for sarcoma.AIM To evaluate our compliance of NCCN sarcoma guidelines at a major NCI designated center and to report instances of deviation that could be used for future studies to improve patient care.METHODS Data was collected retrospectively as an internal review and quality assessment of 35 newly diagnosed and treated patients.Demographic data were recorded and information concerning whether patients had appropriate imaging,biopsy and management.Variables of interest were expressed as raw numbers and percentages.RESULTS Primary site imaging was obtained in 100%of cases.Chest and full-body imaging were obtained in 97%and 100%of indicated cases,respectively.Tissue was obtained preoperatively in 97%of cases.Imaging was reviewed at multidisciplinary Treatment Planning Conference(TPC)in 97%of cases.Pathology was reviewed in 94%of cases in TPC.Both tumor,node,metastasis staging and plan of care were reviewed in 100%of cases in TPC.Treatment guidelines were followed in 94%of cases reviewed.CONCLUSION This study evaluated the workup and treatment provided by a single NCI designated sarcoma service to a series of patients with pathologies defined with the NCCN sarcoma treatment guidelines.Although adherence to NCCN was reported to be very high future prospective studies are required to investigate whether NCCN guidelines impact patient outcomes.

Analysis of Sentinel Lymph Node Biopsy on Clear Cell Sarcoma Treatment

Clear Cell Sarcoma (CCS) is a rare soft tissue sarcoma that corresponds to a less than 1% of all sarcomas and is a high degree neoplasm with propensity to disseminate to regional lymph nodes. Regional lymphadenectomies have a controversial impact on sarcomas treatment and survival, even though those patients have higher local recurrence and lower survival. So, sentinel lymph node biopsy must be one option for better regional staging to STS with higher propensity to lymph node commitment. The authors demonstrate two cases of CCS submitted to surgery through compartmental resection associated with the sentinel lymph node biopsy. A literature review about soft tissue sarcoma and lymph node sentinel biopsy is shown as an initial experience of two cases.

Dermatofibrosarcoma Protuberans of the Neck: A Case Report

Background: Dermatofibrosarcoma protuberans (DFSP) is rare soft tissue sarcoma but has a locally aggressive nature. Although most cases are of low grade and have a rare metastasis rate, all DFSP variants have a tendency to show local recurrence. Wide excision with negative margins is the treatment of choice. Aim: To report a case of DFSP who presented with an asymptomatic slow growing tumor similar to etiologies such as hypertrophic scars or other benign soft tissue tumors. Case Presentation: A 68-year-old male presented with a large soft tumor located at the left posterior neck. Local excision was done under the preoperative impression of a benign tumor such as lipoma or sebaceous cyst. However the diagnosis of DFSP was made upon histological examination and the patient underwent another surgery to achieve free resection margins under general anesthesia as well as adjuvant radiotherapy. Conclusion: DFSP is a malignant tumor that is diagnosed histopathologically. Due to the low incidence rate, slow-growing nature, and non-alarming initial presentation features, diagnostic delay or even misdiagnosis is not uncommon.