Malignant Fibrous Histiocytoma Research Articles

Pancreatic metastasis from undifferentiated pleomorphic sarcoma. An extremely rare entity

Undifferentiated pleomorphic sarcoma (UPS) is a very aggressive malignant tumor that originates in bone or soft tissues, being its most frequent location in the extremities. We present the case of a 71-year-old man who, two years of follow-up after a lower right limb UPS excision surgery, presented a single metastasis of sarcomatoid origin in the pancreas that was treated by cephalic pancreaticoduodenectomy. Sarcomatoid metastases to the pancreas are extremely rare. Resection of the metastasis is the only therapeutic alternative that has reported benefit in terms of the survival of these patients.

Undifferentiated Pleomorphic Sarcoma

Undifferentiated Pleomorphic Sarcoma

Cancer Radiomic and Perfusion Imaging Automated Framework: Validation on Musculoskeletal Tumors.

Limitations from commercial software applications prevent the implementation of a robust and cost-efficient high-throughput cancer imaging radiomic feature extraction and perfusion analysis workflow. This study aimed to develop and validate a cancer research computational solution using open-source software for vendor- and sequence-neutral high-throughput image processing and feature extraction. The Cancer Radiomic and Perfusion Imaging (CARPI) automated framework is a Python-based software application that is vendor- and sequence-neutral. CARPI uses contour files generated using an application of the user's choice and performs automated radiomic feature extraction and perfusion analysis. This workflow solution was validated using two clinical data sets, one consisted of 40 pelvic chondrosarcomas and 42 sacral chordomas with a total of 82 patients, and a second data set consisted of 26 patients with undifferentiated pleomorphic sarcoma (UPS) imaged at multiple points during presurgical treatment. Three hundred sixteen volumetric contour files were processed using CARPI. The application automatically extracted 107 radiomic features from multiple magnetic resonance imaging sequences and seven semiquantitative perfusion parameters from time-intensity curves. Statistically significant differences (P < .00047) were found in 18 of 107 radiomic features in chordoma versus chondrosarcoma, including six first-order and 12 high-order features. In UPS postradiation, the apparent diffusion coefficient mean increased 41% in good responders (P = .0017), while firstorder_10Percentile (P = .0312) was statistically significant between good and partial/nonresponders. The CARPI processing of two clinical validation data sets confirmed the software application's ability to differentiate between different types of tumors and help predict patient response to treatment on the basis of radiomic features. Benchmark comparison with five similar open-source solutions demonstrated the advantages of CARPI in the automated perfusion feature extraction, relational database generation, and graphic report export features, although lacking a user-friendly graphical user interface and predictive model building.

Superficial dedifferentiated liposarcoma: A clinicopathologic study

IntroductionDedifferentiation occurs in approximately 10% of atypical lipomatous tumors/well-differentiated liposarcomas (ALT/WDLPS), primarily in retroperitoneal or deep-seated tumors, conferring metastatic potential. Superficial dedifferentiated liposarcoma (sDDLPS) is rare, and its progression and natural history are poorly documented. MethodsWe performed a 15-year retrospective review of our pathology database to identify cases of DDLPS in the skin or subcutaneous tissue. Diagnosis of primary sDDLPS required evidence of non-lipogenic sarcoma in the skin or subcutis, with concurrent ALT/WDLPS and/or MDM2 amplification. ResultsWe identified 14 cases of DDLPS involving skin or subcutis: 7 primary sDDLPS and 7 secondary lesions (3 from recurrent deep DDLPS and 4 from metastasis). Primary sDDLPS cases (4 females, 3 males; median age: 74) mainly presented as undifferentiated spindle cell or pleomorphic sarcoma. Tumor grades were grade 2 (5 cases) and grade 3 (2 cases), with three cases also showing grade 1 areas. MDM2 amplification was confirmed in 6 sDDLPSs for which FISH was successfully performed. Follow-up available for 6 sDDLPS patients showed 2 local recurrences, treated with re-excision and radiation therapy, with all disease-free at last follow-up (5–126 months). Of the 7 secondary cases, 2 had ongoing disease after multiple recurrences, 1 was disease-free, and all 4 with cutaneous metastasis died of disease (follow-up range: 24–263 months). ConclusionThese findings emphasize the importance of distinguishing between primary sDDLPS and secondary lesions due to their distinct prognoses. Metastasis or superficial extensions from deep DDLP correlate with a considerably worse prognosis than those originating in superficial tissues.

DDIT3-amplified or low-polysomic pleomorphic sarcomas without MDM2 amplification: Clinicopathological review and immunohistochemical profile of nine cases

Several high-grade pleomorphic sarcoma cases that cannot be classified into any existing established categories have been reported. These cases were provisionally classified into undifferentiated pleomorphic sarcoma (UPS). Some dedifferentiated liposarcoma (DDLS) cases may also have been classified into the UPS category due to the absence of MDM2 amplification or an atypical lipomatous tumor/well-differentiated liposarcoma component. We retrieved and reviewed 77 high-grade pleomorphic sarcoma cases, initially diagnosed as UPS in 66 cases and DDLS in 11 cases. Fluorescence in situ hybridization (FISH) analyses of DDIT3 and MDM2 were performed for available cases. Of the cases successfully subjected to DDIT3 FISH (n = 56), nine (7 UPS and 2 DDLS) showed DDIT3 amplification but no MDM2 amplification. Two UPS cases showed both telomeric (5′) and centromeric (3′) amplification of DDIT3 or low polysomy of chromosome 12, whereas 5 UPS and 2 DDLS cases showed 5′-predominant DDIT3 amplification. Histopathologically, all cases showed UPS-like proliferation of atypical pleomorphic tumor cells. Immunohistochemically, only one case showed focal nuclear positivity for DDIT3, supporting the previous finding that DDIT3 expression was not correlated with DDIT3 amplification. All three cases with focal MDM2 expression involved 5′-predominant amplification, two of which showed DDLS-like histological features. The majority of cases (7/9) showed decreased expression in p53 staining, suggesting that DDIT3 amplification regulates the expression of TP53 like MDM2. From a clinicopathological perspective, we hypothesize that DDIT3-amplified sarcoma, especially with 5′-predominant amplification, can be reclassified out of the UPS category.

Metastatic lesions of the uterus, fallopian tubes and ovaries in undifferentiated pleomorphic sarcoma of the left tibia (clinical case)

Undifferentiated pleomorphic osteosarcoma belongs to the group of rarely occurring tumors. Despite the treatment, the disease progresses in 30–40 % of patients with osteosarcomas. The main route of metastasis of bone tissue sarcomas is hematogenous, while lymphogenic spread is observed less frequently. As a rule, secondary metastatic changes occur in the lungs. Less often there is a secondary lesion of the bones of the skeleton and brain. Metastatic lesion of uterus, fallopian tubes and ovaries in malignant undifferentiated pleomorphic sarcoma is extremely rare. Therefore, we found it interesting to describe a clinical case of such a rare metastatic lesion. Patient K. underwent amputation of the left limb at the level of the lower third of the femur for undifferentiated pleomorphic sarcoma of the left tibia in 2019, and 4 courses of adjuvant polychemotherapy were performed. In 20 months after completion of complex treatment of the primary tumor, complaints of lower abdominal pain, increased body temperature up to 37.8 °C in the evenings appeared. According to the results of follow-up examination, a voluminous, multinodular, solid mass of merging character was detected in the pelvis, with total dimensions of up to 11 cm, and a cavitary mass of up to 5 cm was detected in the posterior vault. A trepan-biopsy of the mass in the projection of the right ovary was performed. The morphological picture in the volume of trepan biopsy specimens is characteristic of spindle cell sarcoma. Metastasis of undifferentiated pleomorphic bone sarcoma (malignant fibrous histiocytoma) is most likely. Due to metastatic lesions of the uterus, fallopian tubes, ovaries, omentum, mesentery and serous membrane of the colon loops, peritoneum of the bladder, surgical intervention in the volume of removal of the distal part of the sigmoid colon, rectosigmoid, upper ampullary parts of the rectum, uterus with fallopian tubes and ovaries, appendix was performed. Immunohistochemical study of the postoperative material revealed that the immunophenotype of tumor cells confirmed the morphological picture typical for undifferentiated pleomorphic bone sarcoma. The patient was further prescribed antitumor drug therapy. This clinical case demonstrates a rare, atypical metastasis of undifferentiated pleomorphic osteosarcoma, which allows to expand the knowledge about the flow of malignant diseases of this localization.

Demographic and clinical profile of 1106 adult soft tissue sarcoma patients: A single institutional prospective database experience from India.

Adult soft tissue sarcomas (STS) are rare and diverse. Current management is based on limited literature from the West. Therefore, data from different geographical regions is required, including the low-middle-income countries. This is our experience managing adult sarcomas in the tertiary cancer center of North India. This is a retrospective analysis of the structured sarcoma database of patients treated in the surgical oncology department between 1992 and 2020. The descriptive analysis includes demography, site distribution, diagnosis, histopathology variations, prior surgical interventions, and stage. A total of 1106 soft tissue sarcoma patients were treated in three decades. Age distribution was 13%, 43%, 31%, and 11% in <20, 21-40, and 41-60 and >60 years, respectively. The male-to-female ratio was 1.73. The anatomical distribution was 17%, 42%, 23%, 7%, 7%, and 3% in upper extremity, lower extremity, trunk, retroperitoneum, head and neck, and viscera, respectively. Overall, 49% of patients had undergone prior suboptimal surgeries at community hospitals. Common histology subtypes were synovial sarcoma (18%), undifferentiated pleomorphic sarcoma (UPS) (13%), dermatofibrosarcoma protuberans (12%), and liposarcoma (9%). A pathological discordance of 13% was identified between the initial and the final histologies. Overall, 61% of tumors were high-grade. Memorial Sloan Kettering Stages II and III were present in 33% and 35% of patients, respectively. This is one of the largest single institutional experiences of STS from the Asian population. Mostly young adults were affected with male preponderance. The lower extremity and trunk were common subsites. Frequent histologies were synovial sarcoma and UPS. A high rate of suboptimal surgical intervention at the community level and pathological discordance was noted. This study highlights the need to establish prospective structured databases for capturing quality information related to rare malignancies and providing insights for future research.

Pan-cancer Genomic Analysis of AXL Mutations Reveals a Novel, Recurrent, Functionally Activating AXL W451C Alteration Specific to Myxofibrosarcoma.

Myxofibrosarcoma (MFS) is a common soft tissue sarcoma of the elderly that typically shows low tumor mutational burden, with mutations in TP53 and in genes associated with cell cycle checkpoints ( RB1 , CDKN2A ). Unfortunately, no alterations or markers specific to MFS have been identified and, as a consequence, there are no effective targeted therapies. The receptor tyrosine kinase AXL, which drives cellular proliferation, is targetable by new antibody-based therapeutics. Expression of AXL messenger RNA is elevated in a variety of sarcoma types, with the highest levels reported in MFS, but the pathogenic significance of this finding remains unknown. To assess a role for AXL abnormalities in MFS, we undertook a search for AXL genomic alterations in a comprehensive genomic profiling database of 463,546 unique tumors (including 19,879 sarcomas, of which 315 were MFS) interrogated by targeted next-generation DNA and/or RNA sequencing. Notably, the only genomic alterations recurrent in a specific sarcoma subtype were AXL W451C (n = 8) and AXL W450C (n = 2) mutations. The tumors involved predominantly older adults (age: 44 to 81 [median: 72] y) and histologically showed epithelioid and spindle-shaped cells in a variably myxoid stroma, with 6 cases diagnosed as MFS, 3 as undifferentiated pleomorphic sarcoma (UPS), and 1 as low-grade sarcoma. The AXL W451C mutation was not identified in any non-sarcoma malignancy. A review of publicly available data sets revealed a single AXL W451C-mutant case of UPS that clustered with MFS/UPS by methylation profiling. Functional studies revealed a novel activation mechanism: the W451C mutation causes abnormal unregulated dimerization of the AXL receptor tyrosine kinase through disulfide bond formation between pairs of mutant proteins expressing ectopic cysteine residues. This dimerization triggers AXL autophosphorylation and activation of downstream ERK signaling. We further report sarcomas of diverse histologic subtypes with AXL gene amplifications, with the highest frequency of amplification identified in MFS cases without the W451C mutation. In summary, the activating AXL W451C mutation appears highly specific to MFS, with a novel mechanism to drive unregulated signaling. Moreover, AXL gene amplifications and messenger RNA overexpression are far more frequent in MFS than in other sarcoma subtypes. We conclude that these aberrations in AXL are distinct features of MFS and may aid diagnosis, as well as the selection of available targeted therapies.

FGF23 Expression Is a Promising Immunohistochemical Diagnostic Marker for Undifferentiated Pleomorphic Sarcoma of Bone (UPSb).

Background: Undifferentiated pleomorphic sarcoma of bone (UPSb) is a rare primary bone sarcoma that lacks a specific line of differentiation. Distinguishing between UPSb and other malignant bone sarcomas, including dedifferentiated chondrosarcoma and osteosarcoma, is challenging due to their overlapping features. We have previously identified that UPSb tumours have elevated mRNA levels of Fibroblast Growth Factor 23 (FGF23) transcripts compared to other sarcomas including osteosarcoma. In the present study, we evaluated the specificity and practicality of FGF23 immunoreactivity as a specific diagnostic tool to differentiate UPSb tumours from osteosarcomas and dedifferentiated chondrosarcomas. Methods: A total of 10 UPSb, 10 osteosarcoma, and 10 dedifferentiated chondrosarcoma cases (all high-grade), were retrieved and immunohistochemistry for FGF23 was performed. Results: FGF23 protein was expressed at high levels in 80-90% of undifferentiated pleomorphic sarcoma of the bone cases, whereas it was expressed at significantly lower levels in dedifferentiated chondrosarcoma and osteosarcoma cases. A semiquantitative analysis, considering the intensity of immunoreactivity, confirmed significantly elevated FGF23 expression levels in UPSb tissues compared to those observed in osteosarcoma and dedifferentiated chondrosarcoma tissues. Conclusions: The results we present here suggest that FGF23 immunohistochemistry may be a useful tool to aid in differentiating UPSb from morphologically similar malignant bone sarcomas, especially in situations where sampling is restricted and there is limited clinical information available.

A randomized, non-comparative phase 2 study of neoadjuvant immune-checkpoint blockade in retroperitoneal dedifferentiated liposarcoma and extremity/truncal undifferentiated pleomorphic sarcoma.

Based on the demonstrated clinical activity of immune-checkpoint blockade (ICB) in advanced dedifferentiated liposarcoma (DDLPS) and undifferentiated pleomorphic sarcoma (UPS), we conducted a randomized, non-comparative phase 2 trial ( NCT03307616 ) of neoadjuvant nivolumab or nivolumab/ipilimumab in patients with resectable retroperitoneal DDLPS (n = 17) and extremity/truncal UPS (+ concurrent nivolumab/radiation therapy; n = 10). The primary end point of pathologic response (percent hyalinization) was a median of 8.8% in DDLPS and 89% in UPS. Secondary end points were the changes in immune infiltrate, radiographic response, 12- and 24-month relapse-free survival and overall survival. Lower densities of regulatory T cells before treatment were associated with a major pathologic response (hyalinization > 30%). Tumor infiltration by B cells was increased following neoadjuvant treatment and was associated with overall survival in DDLPS. B cell infiltration was associated with higher densities of regulatory T cells before treatment, which was lost upon ICB treatment. Our data demonstrate that neoadjuvant ICB is associated with complex immune changes within the tumor microenvironment in DDLPS and UPS and that neoadjuvant ICB with concurrent radiotherapy has significant efficacy in UPS.

PTEN pathogenic variants are associated with poor prognosis in patients with advanced soft tissue sarcoma

BackgroundWe aimed to examine whether PTEN pathogenic variants (mutPTEN) were associated with overall survival (OS) in patients with advanced soft tissue sarcoma (STS) with the presence of one or more of the most common genomic alterations including p53, CDKN2A, RB1, and ATRX pathogenic variants.MethodsThis study included patients from Kaiser Permanente Northern California and Stanford Cancer Center with grade 2 or higher locally advanced and metastatic STS.ResultsA total of 174 patients had leiomyosarcoma (LMS), 136 had undifferentiated pleomorphic sarcoma (UPS), 78 had Liposarcoma (LPS), and 214 had other histology subtypes (Others). Among all patients with STS, OS was worse for those with mutPTEN versus wild-type PTEN (wtPTEN, adjusted HR [aHR] = 1.58 [95% CI, 1.11–2.23]), mutCDKN2A vs wtCDKN2A (aHR = 1.33 [95% CI .99–1.80]), and mutRB1 vs wtRB1 (aHR = 1.26 [95% CI 0.93–1.70[), while OS was similar for mutp53 vs wtp53 and mutATRX vs wtATRX. MutPTEN versus wtPTEN was consistently associated with worse OS in histologic subtypes including LMS and UPS and molecular subgroups.ConclusionMutPTEN vs wtPTEN was associated with worse OS in advanced STS. If confirmed, our findings could be helpful for prognostic stratification in clinical practice and for further understanding the molecular mechanisms of STS.

Glycoprotein Nonmetastatic Melanoma Protein B (GPNMB) Immunohistochemistry Can Be a Useful Ancillary Tool to Identify Perivascular Epithelioid Cell Tumor

Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors that express smooth muscle and melanocytic makers. Diagnosis of PEComas can be challenging due to focal or lost expression of traditional immunohistochemical markers, limited availability of molecular testing, and morphological overlap with much more common smooth muscle tumors. This study evaluates the use of glycoprotein nonmetastatic melanoma protein B (GPNMB) immunohistochemical staining as a surrogate marker for TSC1/2/MTOR alteration or TFE3 rearrangement to differentiate PEComas from other mesenchymal tumors. Cathepsin K was also assessed for comparison. A total of 399 tumors, including PEComas, alveolar soft part sarcomas, and other histologic PEComa mimics, were analyzed using GPNMB and cathepsin K immunohistochemistry. GPNMB expression was seen in all PEComas and alveolar soft part sarcomas with the majority showing diffuse and moderate-to-strong labeling, whereas other sarcomas were negative or showed focal labeling. When a cutoff of diffuse and at least moderate staining was used, GPNMB demonstrated 95% sensitivity and 97% specificity in distinguishing PEComas from leiomyosarcoma, well-differentiated/dedifferentiated liposarcomas, and undifferentiated pleomorphic sarcomas. Cathepsin K with a cutoff of any labeling had lower sensitivity (78%) and similar specificity (94%) to GPNMB. This study highlights GPNMB as a highly sensitive marker for PEComas and suggests its potential use as an ancillary tool within a panel of markers for accurate classification of these tumors.

Management of head & neck sarcomas in adults: A retrospective study

The research purpose is to review the surgical approach and evaluate the results in adult patients with head and neck sarcomas.The histopathology varied, including two leiomyosarcomas, six malignant fibrous histiocytomas, two malignant peripheral nerve sheath tumors, four dermatofibrosarcomas protuberans, three osteosarcomas, two angiosarcomas, one liposarcoma, one Ewing sarcoma, one synovial sarcoma, two unclassified/non-differentiated sarcomas and one solitary fibrous tumor. Surgical resection included maxillectomy, mandibulectomy, craniectomy, parotidectomy, scalp resection, face skin resection and laminectomy. The reconstruction was performed with one rectus abdominis flap, four radial forearm flaps, two latissimus dorsi flaps, two vascularized fibula flaps, two pectoralis major myocutaneous flaps, two trapezius flaps, two temporalis flaps, seven scalp flaps and two nasolabial flaps.The total patient number was 24. The hospitalization was uncomplicated, followed by postoperative radiotherapy in the majority of cases. In a mean 15-year follow-up period, 11 patients are still alive and disease-free. There were four recurrences treated with palliative radiotherapy.The surgical approach for head and neck sarcomas, including the achievement of a functionally acceptable result by organ sparing techniques, remains challenging. Wide resection combined with the appropriate reconstruction, particularly with microsurgical techniques, and followed by adjuvant radiotherapy or chemotherapy offer improved prognosis and quality of life.

Dedifferentiated Leiomyosarcoma of the Uterine Corpus with Heterologous Component: Clinicopathological Analysis of Five Consecutive Cases from a Single Institution and Comprehensive Literature Review.

Dedifferentiation is a very rare phenomenon in uterine leiomyosarcoma (LMS). The aim of this study was to comprehensively analyze the clinicopathological characteristics of uterine dedifferentiated LMS (DDLMS). We reviewed electronic medical records and pathology slides from five patients with uterine DDLMS and performed immunostaining. The mean age of the patients was 56 years. Two patients presented with abdominal discomfort, while in three cases the uterine tumors were detected on routine medical examination. The mean size of the tumors was 17.0 cm. Four patients underwent hysterectomy. The initial stages were distributed as IB (2/5), IIIC (2/5), and IVC (1/5). Post-operative concurrent chemoradiation therapy, radiation therapy, and chemotherapy were administered in one, one, and two patients, respectively. Despite post-operative treatment, three patients developed metastatic recurrences in the abdominal and pelvic organs. Recurrence-free survival time ranged between 4 and 30 months. Histologically, the differentiated areas demonstrated the classic morphology of malignant smooth muscle differentiation, whereas the dedifferentiated areas resembled undifferentiated pleomorphic sarcoma and were characterized by large pleomorphic tumor cells admixed with haphazardly arranged atypical cells with marked nuclear pleomorphism. All cases also exhibited heterologous components, including chondrosarcoma (CSA; 3/5) and rhabdomyosarcoma (2/5). In two cases, the heterologous components were initially detected in primary tumors. In three cases, the primary tumors did not exhibit any dedifferentiated or heterologous components. Instead, more than half of the recurrent tumors consisted of heterologous components. Three cases showed a sharp demarcation between the LMS and CSA components, while in two cases the dedifferentiated area imperceptibly merged with the differentiated component. Immunostaining revealed that the dedifferentiated components exhibited a lack of desmin immunoreactivity in three of the four examined cases. A subset of uterine LMS represents various amounts and types of dedifferentiation and heterologous components in both primary and recurrent tumors. Routine recognition of DDLMS and distinction from its mimickers are required for accurate diagnosis and further characterization of these rare tumors.

Diagnostic Importance of Clinic-Pathologic Features and p16, CD34, MDM2 Expression in Differential Diagnosis of Tumors

Background: The large majority of soft tissue tumors are benign. Malignant mesenchymal neoplasm amount toless than 1% of the overall human burden of malignant tumors but they are life threatening and may pose asignificant diagnostic and therapeutic challenge since there are more than 50 histological subtypes of STS, whichare often associated with unique clinical, prognostic and therapeutic features.Methods: The study was undertaken in department of Pathology, King George’s Medical University, Lucknow.The Study Design was Retrospective and prospective study carried over a period of two year from September2018 to august 2020 including 70 cases. Results. Most common diagnosis of malignant cases was Synovial saroma(21.4%) followed by Leiomyosarcoma (19.0%) and Undifferentiated pleomorphic sarcoma &amp; Fibromyxoid sarcoma(11.9% each). Less common diagnosis were Ewing’s sarcoma, Liposarcoma and Rhabdomyosarcoma (9.5% each),1 (2.4%) case each was diagnosed as Chondrosarcoma, MPNST and Myxoid liposarcoma.Conclusions: Genetic alterations involving the 12q13-15 chromosomal region are common in musculoskeletalsarcomas, and many bone and soft-tissue malignant tumors showed amplification of various genes located in thisregion.

Orbital pleomorphic sarcoma: A case report

Aims/Purpose: To report a case involving an undifferentiated pleomorphic sarcoma localized in the orbit.Methods: Case report and literature review.Results: Undifferentiated pleomorphic sarcomas (UPS) are one of the least common types within all malignant sarcomas, with their incidence reaching 5–10% of the cases. Within this group of UPS, tumours involving the head and neck have an even lower incidence (approximately 3 to 5%). We report the case of an 86 year old male with a history of cardiovascular disease, presenting with a painless lesion in his right eye. The orbit CT reported a soft tissue lesion in contact with the right ocular globe, lacrimal gland and extraconal fat. At examination the patient registered best corrected visual acuity right eye (OD) no light perception right eye and left eye (OS) 20/50. At slit lamp examination OD is not evaluable due to corneal compromise. OS anterior segment and posterior pole with no alterations. Immunohistochemistry was negative for P40, desmine, CD34, SOX‐10 and cytokeratin cocktail. S100 had weak and patchy staining. These results led to a giant cell pleomorphic sarcoma diagnosis, histological grade 3. The patient underwent urgent orbital exenteration, with an adequate postsurgical evolution and oncology follow up.Conclusions: The diagnosis of an UPS is based on exclusion, due to their heterogeneous nature, with histological characteristics that overlap with other malignant mesenchymal neoplasms, mainly with metastatic sarcomatoid carcinoma and melanoma. These tumours are characterized by their rapid growth and potential for metastasis, especially in the lungs. Treatment includes surgical resection, sometimes combined with adjuvant chemotherapy. Strict periodic checkups are essential, since local recurrence is frequent (between 19 to 31%), with most cases occurring within the first 24 months.

A case of embolic stroke associated with undifferentiated pleomorphic sarcoma

A case of embolic stroke associated with undifferentiated pleomorphic sarcoma

Undifferentiated Pleomorphic Sarcoma with Focal Myogenic Differentiation A Rare Case of Testicular Sarcoma

Undifferentiated Pleomorphic Sarcoma with Focal Myogenic Differentiation A Rare Case of Testicular Sarcoma

Anterior Thigh Myxofibrosarcoma with Management of Wide Excision and Modified Anterior Thigh Compartment Resection : A Case Report

Introduction: Myxofibrosarcoma (MFS) is a type of malignancy from the group of malignant fibrous histiocytoma. Myfxofibrosarcoma is a type of soft tissue neoplasm that is aggressive. The clinical symptoms are not pathognomonic and the histological picture is very heterogeneous, often receiving delayed treatment and causing misdiagnosis. Complementary histochemical and immunohistochemical staining is mandatory to confirm the diagnosis of MFS. Extensive surgical treatment and followed by radiotherapy is the first choice of myxofibrosarcoma treatment.&#x0D; Case Presentation: A 54-years old woman complained of a lump on her left thigh that had been getting bigger for the past 2 years. The lump initially looked the size of a marble, then grew to the size of a tennis ball in the last 7 months. On physical examination, a mass was found in the left thigh area, a hard, fixed mass, the size of a tennis ball. Fine needle aspiration examination showed suspicious results for myxofibrosarcoma. The patient underwent a Magnetic Resonance Imaging examination in the left lower extremity area and underwent wide excision and anterior thigh compartment resection surgery.&#x0D; Discussion: Wide resection is the standard treatment for MFS [4]. The choice of procedure for each patient is different and should be based on tumor size, location, stage, surrounding neurovascular and bony elements, as well as functional and cosmetic considerations [4]. The minimum resection margin in MFS is at least 1 cm which aims to minimize the risk of local recurrence [4]. The recommended resection margin is at least 2 cm for MFS resection [4]. We had plan a 2 cm margin of the entire preoperative MRI enhancement area. It should be noted that the local recurrence rate for MFS in margin-negative resections is relatively high compared with other STS subtypes.&#x0D; Conclusion: We recommend Wide Excison and Modified Anterior Thigh compartment Resection is recommended procedure for anterior thigh myxofibrosarcoma. This procedure involves preserving uncontaminated thigh neurovascular, and only resecting one of the sarcoma-infiltrated muscle heads and preserving the other quadriceps muscle head of the thigh to maintain knee extension function. This paper is the first report on the successful treatment of anterior thigh myxofibrosarcoma without weakness of knee extension complication.

The Effect of Histopathological Subtype on 5 Year and 10 Year Overall Survival in Primary Malign Tumors of the Chest Wall

Primary tumors of the chest wall are rare tumors comprising 5% of thoracic neoplasms. The current study was designed to evaluate the effect of histopathological subtype on the 5-year and 10-year overall survival and regional recurrence in primary malignant tumors of the chest wall. From December 2007 to December 2019, the data of 20 patients were analyzed. Overall survival rates, median overall survival, regional tumor recurrence and median disease-free survival rates were compared retrospectively between histopathological subtypes. Chondrosarcoma (n=7), desmoid tumor (n=6), undifferentiated pleomorphic sarcoma [(UPS), (n=4)], Ewing's sarcoma (n=1), malignant peripheral nerve sheath tumor (n=1), and liposarcoma (n=1) were the subtypes identified. A lower median overall survival was detected in the UPS histopathological subtype (p = 0.004). The 5 and 10-year overall survival rates in the chondrosarcoma group were 71% and 37%, respectively; desmoid tumors were found in 83% and 42%, respectively. There was no patient with 5-year survival in UPS. A shorter median overall survival was found in lateral wall masses compared to anterior and posterior wall masses (p = 0.001). During the follow-up, 10 patients (50%) died. It was observed that the median disease-free survival in the UPS subtype was shorter compared to the other subtypes (p = 0.002). The regional recurrence rate was 57%, 17% and 75% in chondrosarcoma, desmoid tumor and UPS, respectively. In UPS, a primary malignant tumor of the chest wall, 5-year and 10-year overall survival rates and median overall survival; it was found to be significantly shorter than the chondrosarcoma and desmoid tumor groups.