Malignant Soft Tissue Tumors Research Articles

Does the apparent diffusion coefficient from diffusion-weighted MRI imaging aid in the characterization of malignant soft tissue tumors and sarcomas.

To identify trends in apparent diffusion coefficient (ADC) measurements from diffusion-weighted imaging (DWI) with respect to tumor type classification and other tumor characteristics whether common malignant soft tissue tumors can be distinguished. A consecutive series of extremity malignant soft tissue tumors and soft tissue sarcomas (STS) among 78 adult patients with conventional MRI and DWI were included. Each case was evaluated with respect to T1/T2 signal alterations and heterogeneity, presence of peritumoral edema, necrosis, cystic changes, internal hemorrhage, and maximum longitudinal dimension blinded to the histology. The ADC mean and minimum were obtained using a free-hand region of interest of the whole tumor and the darkest (lowest signal area) ADC area of the tumor. Kruskal-Wallis and Wilcoxon Rank-Sum Tests were used to determine associations and significance between tumor subtypes. Intraclass correlation (ICC) and kappa calculations were utilized to assess inter-reader agreements for ADC values and reader diagnosis. Liposarcomas showed more heterogenous T1W images with hyperintense T1W signal when compared to tumors not classified as liposarcoma (P = 0.046 and P = 0.010, respectively). Liposarcomas were relatively consistent in demonstrating an absence of hemorrhage (81.8%) while undifferentiated pleomorphic sarcomas consistently showed intralesional hemorrhage (90%). When comparing individual tumor classifications against the rest of the samples, lymphomas registered lower mean and minimum ADC values in the whole tumor and in themost hypointense area of the tumor for both readers (P < 0.05). The interobserver agreement between the two readers was good to excellent for all four ADC measurements (ICC = 0.65-0.98). Diffusion-weighted imaging generated ADC measurements are reproducible but currently offer limited insight in being able to differentiate among different malignant soft tissue tumor and sarcoma histologies.T1W and T2W signal characteristics also offer limited insight indifferentiating between soft tissue malignancies.

Establishment of predictive nomogram and web-based survival risk calculator for desmoplastic small round cell tumor: A propensity score-adjusted, population-based study.

Desmoplastic small round cell tumor (DSRCT) is a rare undifferentiated malignant soft tissue tumor with a poor prognosis and a lack of consensus on treatment. This study's objective was to build a nomogram based on clinicopathologic factors and an online survival risk calculator to predict patient prognosis and support therapeutic decision-making. A retrospective cohort analysis of the Surveillance, Epidemiology and End Results (SEER) database was performed for patients diagnosed with DSRCT between 2000 and 2019. The least absolute shrinkage and selection operator (LASSO) Cox regression analysis was applied to identify the individual variables related to overall survival (OS) and cancer-specific survival (CSS), as well as to construct online survival risk calculators and nomogram survival models. The nomogram was employed to categorize patients into different risk groups, and the Kaplan-Meier method was utilized to determine the survival rate of each risk category. Propensity score matching (PSM) was used to assess survival with different therapeutic approaches. A total of 374 patients were included, and the median OS and CSS were 25 (interquartile range 21.9-28.1) months and 27 (interquartile range 23.6-30.3) months, respectively. The nomogram models demonstrated high predictive accuracy. PSM found that patients with triple-therapy had better CSS and OS than those who received surgery plus chemotherapy (median survival times 49 vs 34 months and 49 vs 35 months, respectively). The nomogram successfully predicted the DSRCT patients survival rate. This approach could assist doctors in evaluating prognoses, identifying high-risk populations, and implementing personalized therapy.

Establishment of predictive nomogram and web-based survival risk calculator for desmoplastic small round cell tumor: A propensity score-adjusted, population-based study.

Desmoplastic small round cell tumor (DSRCT) is a rare undifferentiated malignant soft tissue tumor with a poor prognosis and a lack of consensus on treatment. This study's objective was to build a nomogram based on clinicopathologic factors and an online survival risk calculator to predict patient prognosis and support therapeutic decision-making. A retrospective cohort analysis of the Surveillance, Epidemiology and End Results (SEER) database was performed for patients diagnosed with DSRCT between 2000 and 2019. The least absolute shrinkage and selection operator (LASSO) Cox regression analysis was applied to identify the individual variables related to overall survival (OS) and cancer-specific survival (CSS), as well as to construct online survival risk calculators and nomogram survival models. The nomogram was employed to categorize patients into different risk groups, and the Kaplan-Meier method was utilized to determine the survival rate of each risk category. Propensity score matching (PSM) was used to assess survival with different therapeutic approaches. A total of 374 patients were included, and the median OS and CSS were 25 (interquartile range 21.9-28.1) months and 27 (interquartile range 23.6-30.3) months, respectively. The nomogram models demonstrated high predictive accuracy. PSM found that patients with triple-therapy had better CSS and OS than those who received surgery plus chemotherapy (median survival times: 49 vs 34 months and 49 vs 35 months, respectively). The nomogram successfully predicted the DSRCT patients survival rate. This approach could assist doctors in evaluating prognoses, identifying high-risk populations, and implementing personalized therapy.

“NEGLECTED GIANT DERMATOFIBROSARCOMA PROTUBERANS”

Dermatobrosarcoma protuberans (DFSP) is a rare, slow growing, intermediate to low grade malignant soft tissue neoplasm originating from the dermal layer of the skin. We encountered a massive DFSP over the back which presented to us with acute bleeding and pus discharge. This was successfully managed by wide local excision of the tumor. This case is remarkable due to the large size of tumor presenting unusually as acute bleeding and its successful management with wide local excision as a lifesaving procedure.

A Rare Case of Extraosseous Osteosarcoma (EOS) of Parotid Gland.

Extraosseous osteosarcoma is a rare malignant soft tissue neoplasm, and extraosseous osteosarcoma of the parotid gland is very rare. It has a very aggressive course, and there are no standardized treatment guidelines.We report the case of a 20 year old male patient who presented with history of right neck swelling since 6 years for which he had undergone right parotid surgery 5 years ago. The final histopathological report indicated that the mass was a pleomorphic adenoma. One year after the first surgery, the patient experienced recurrence of swelling over the operated site, and the size of the swelling has been increasing gradually since then. He was evaluated clinically, and a large mass was noted over the upper aspect of the right upper neck, extending to the occipital and parotid regions. An MRI scan was done which showed a 12 × 10 × 8cm lesion centred in the right parotid gland, involving paraspinal muscles, C1-C2 vertebrae and extending into the parapharyngeal space. FNAC of the lesion showed features of pleomorphic adenoma. The patient underwent a complete excision of the tumour. The patient's post-operative period was uneventful. The final histopathological report of the patient was extraosseous osteosarcoma of the parotid gland. The patient was referred for adjuvant radiotherapy. He has been on regular follow-up for the past 6 months and has shown no sign of recurrence.EOS is an extremely rare tumour of the head and neck region which often requires extensive surgical resection with or without adjuvant radiotherapy. It has a high rate of local recurrence and a very low disease free survival. Such patients should be kept on a close follow-up.

A Case of Thromboembolism and Deep Venous Thrombosis after Transfemoral Amputation with Short Stump

An 82-year-old patient underwent a left transfemoral amputation due to a malignant soft tissue tumor. He developed symptoms of chest pain and hypoxia on the 32nd day after the operation. These symptoms were caused by deep venous thrombosis (DVT) of the stump and acute pulmonary thromboembolism (PTE), for which he was treated with anticoagulant therapy. Shortly after treatment he could resume a rehabilitation therapy. Patients with a lower extremity amputation have a higher risk of developing a DVT because of immobility and increased venous pooling in the residual limb. Even with a short stump as in this case, it is important to actively train the range of motion of the joint and try to prevent DVT.

Hematoma crónico expansivo

Chronic expansive hematoma (CEH) is a rare lesion, characterized by the persistence and increase in size of an hematoma for a period greater than one month since the initial hemorrhage. The clinical importance of this pathology is due to the fact that it can simulate malignant soft tissue neoplasms, both clinically as a result of its progressive growth and radiologically for its findings in imaging studies. This article will review 3 cases of CEH in different scenarios, explaining the radiological findings in different imaging techniques such as ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and PET-CT.

Distribution and Surgical Management of Visceral Ewing Sarcoma Among Children and Adolescents

IntroductionEwing sarcoma (EWS) is a highly malignant tumor of bone and soft tissue that occasionally arises from viscera. Visceral EWS (V-EWS) is challenging to manage given its varied organ distribution and often late-stage presentation. We aimed to characterize our institutional experience with V-EWS, focusing on its surgical management, and to compare V-EWS outcomes against those with osseous (O-EWS) and soft tissue EWS (ST-EWS). MethodsRetrospective review of all EWS patients ≤21 years presenting to a single institution between 2000 and 2022. Patient- and disease-specific characteristics were compared. Overall and relapse-free survival were estimated using Kaplan Meier methods and log-rank test. Results156 EWS patients were identified: 117 O-EWS, 20 ST-EWS, and 19 V-EWS. V-EWS arose in the kidney (n = 5), lung (n = 5), intestine (n = 2), esophagus (n = 1), liver (n = 1), pancreas (n = 1), adrenal gland (n = 1), vagina (n = 1), brain (n = 1), and spinal cord (n = 1). No significant demographic differences were detected between EWS groups. V-EWS was more frequently metastatic at presentation (63.2%; p = 0.005), yet no significant overall or relapse-free survival differences emerged between EWS groups, with similar follow-up intervals. While V-EWS required multiple unique operative strategies to gain primary control, no significant difference in treatment strategies appeared between groups. Surgery-only primary control was associated with improved overall and relapse-free survival in all groups. ConclusionsV-EWS presents unique management challenges in children and adolescents given its variable sites of origin. This large cohort is the first to describe the surgical management and outcomes of V-EWS, demonstrating more frequent metastatic presentation, while achieving similar survival across groups. Level of evidenceLevel 2 – Cohort Study.

Наиболее распространенные диагнозы в ортопедической онкологии: регион Юго-Восточной Анатолии, Турция

The purpose of this study: Musculoskeletal tumors are very rare, and their distribution varies regionally. We aim to share the musculoskeletal system tumors and tumor-like lesions that orthopedic surgeons in the region commonly encounter in their daily practice.Methods. In this retrospective study, 339 patients diagnosed with tumor and tumor-like lesions in the musculoskeletal system who consulted a single orthopedic surgeon between 2022 and 2023 in a tertiary hospital in the Southeast of the country were included. Age, gender, size, type of lesions, tumor diagnoses, and anatomical sites were examined.Results. Bone localization (62.8%) is more common than soft tissue. Benign lesions are most commonly seen in both bone and soft tissue. Osteochondroma (21.7%) and chondrosarcoma (54%) are the most common benign and malignant bone tumors, while osteomyelitis (47.6%) is the most common tumor-like lesion. Hemangioma (27%) and peripheral nerve sheath tumor (62.5%) are the most common benign and malignant soft tissue tumors, while ganglion cyst (63.8%) is the most common tumor-like lesion. Benign bone tumors are larger in males than females (p:0.008). Lower extremity located of musculoskeletal tumors and tumor-like lesions is more common than upper extremity.Conclusions. In the Southeastern Anatolia region, benign bone and soft tissue tumors (osteochondroma and hemangioma) are more likely. The most common malignant tumors are chondrosarcoma and peripheral nerve sheath tumors. Additionally, it should not be forgotten that musculoskeletal system tumors are often located in the lower extremities.Keywords: Benign tumors, bone tumors, malign tumors, musculoskeletal tumors, soft tissue tumors, tumor-like lesions.Corresponding author: Özlem Orhan, Assistant professor of the Department of Orthopedics and Traumatology, Medical Faculty, Harran University, Sanlıurfa, Turkey. Postal code: 63300Address: Turkey, Sanlıurfa, Osmanbey campusPhone: +905057475401Email: droorhan@gmail.comJ Trauma Ortho Kaz 2023; 3 (69): 12-18Recieved: 28-07-2023Accepted: 11-09-2023 This work is licensed under a Creative Commons Attribution 4.0 International License

EPIDEMIOLOGICAL PROFILE AND EVOLUTION IN MUSCULOSKELETAL TUMORS AT THE LEVEL OF THE ELBOW.

To present the epidemiological profile of bone and soft tissue tumors that affect the elbow region treated at an oncology referral center in Brazil. Retrospective observational case series study to evaluate the results of elbow cancer undergoing clinical and/or surgical treatment with the first visit from 1990 to 2020. The dependent variables were benign bone tumor, malignant bone tumor, benign soft tissue tumor, malignant soft tissue tumor. Independent variables were sex, age; presence of symptoms (pain/increase in local volume/fracture); diagnosis; treatment and recurrence. In total, 37 patients were included, 51.35% of whom were female, with a mean age at diagnosis of 33.5 years. Soft tissue neoplasms correspond to 51% of cases against 49% of bone tumors. Among the symptoms, the general prevalence of pain was 56.75%, the general increase in local volume occurred in 54.04% of the patients and the presence of fractures in 13.43%. Surgical treatment occurred in 75.67% of cases and recurrence in 16.21% of cases. The tumors that affect the elbow in our series correspond mostly to benign tumors, involving bone or soft tissues, with a higher occurrence in young adult patients. Level of Evidence IV, Case Series.

Dermatofibrosarcoma Protuberans at Unusual Sites: A Case Series

Dermatofibrosarcoma Protuberans (DFSP) is a rare, locally aggressive malignant subcutaneous soft tissue tumour that often goes undiagnosed due to its indolent nature and non protuberant growth. Recurrences after surgical excision are a common issue. While the trunk and extremities are commonly affected sites, rarer locations such as the genitalia and breast have been reported. The rarity of the lesion, its occasional unusual presentation sites, the indolent yet locally aggressive nature, and the diagnostic and therapeutic challenges are the distinctive features of this tumour. This study presents three unusual sites of DFSP: scrotum, vulva, and pubic region, one of which is an uncommon variant known as pigmented DFSP (Bednar tumour).

Childhood sarcomas: fine-needle aspiration cytopathology with an emphasis on the use of molecular studies.

In children and adolescents, most sarcoma subtypes have a simple karyotype with a single genetic alteration; cytologic findings combined with ancillary testing can lead to a specific diagnosis. The goal of this study was to review the use of fine-needle aspiration in conjunction with immunohistochemistry and molecular studies as a part of an integrated, multidisciplinary diagnostic workup for bone and soft tissue sarcomas in this population. We searched for cases aged ≤18 years old with a malignant bone or soft tissue tumor that had corresponding cytology specimens from 3 institutions. Clinical data, cytologic findings and diagnoses, histologic diagnoses, and ancillary testing were documented. Of 99 cases, 55% were male with a mean age of 12 years. Ninety-four cases (95%) had a specific histologic diagnosis, and 84 cases (85%) were primary neoplasms. Ninety-four cases (95%) had a malignant cytologic diagnosis, and 71 cases (72%) had a specific cytologic diagnosis concordant with the histologic diagnosis. Among primary tumors with a specific histologic diagnosis, a specific cytologic diagnosis was made in 63 cases (79%). After excluding osteosarcoma, 74% of the tumors (n = 50) had molecular studies. Specific genetic alterations supporting a definitive diagnosis were found in 42 cases (84%), the majority of which were demonstrated using Fluorescence In Situ Hybridization (n = 33, 79%). We found that fine-needle aspiration in conjunction with core needle biopsy, immunohistochemistry, and molecular studies allowed cytopathologists to accurately classify sarcomas in a pediatric age group.

Results of amputation after limb salvage surgery and primary amputation after diagnosis in malignant bone and soft tissue tumours

Results of amputation after limb salvage surgery and primary amputation after diagnosis in malignant bone and soft tissue tumours

Immunohistochemical Expression of p16 and CDK4 in Soft Tissue Tumors

Background: The large majority of soft tissue tumours are benign.Malignantmesenchymal neoplasms amount to less than 1% of the overall human burden of malignant tumours but they are life threatening and may pose a significant diagnostic and therapeutic challenge since there are more than 50 histological subtypes of STS, which are 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 one year from September 2018 to august 2019 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 musculoskeletal sarcomas, and many bone and soft-tissue malignant tumors showed amplification of various genes located in this region

Sinonasal Rhabdomyosarcoma in a Child: Case Report and Review of the Literature

Rhabdomyosarcoma is a malignant soft-tissue neoplasm of skeletal muscle origin, which predominantly affects the young population. Embryonal and alveolar are the most common histological subtypes of rhabdomyosarcoma. We present a case of a 3-year-old child with alveolar rhabdomyosarcoma arising from the left nasal cavity.

Molekularpathologie bei ausgewählten Weichgewebssarkomen: diagnostisch und therapeutisch relevante Aberrationen

The current World Health Organization (WHO) classification for soft tissue and bone sarcomas published in 2020 includes more than 110 benign, intermediate, and malignant soft tissue tumors, among them numerous novel entities which in the majority of cases are defined by novel molecular findings. These alterations may be of diagnostic, prognostic, and/or therapeutic relevance. Due to the increased use of RNA sequencing, increasing numbers of established but also novel fusion transcripts are being detected although the clinical relevance is not always known. This leads to an increase of novel tumor entities with novel defining criteria. At the same time, sarcomas without recurrent genomic alterations are decreasing in number. In a subgroup of sarcomas, identification of the driving alteration ideally results in a therapeutic target. In the following review, we present preferentially tumor entities in which potential therapeutic options may arise based on the molecular characteristics.

Sinonasal Synovial Sarcoma Masquerading as A Common Soft Tissue Tumor: A Diagnostic Challenge and Literature Review

Synovial sarcoma is a malignant soft tissue neoplasm which amounts to 7-10% of all sarcomas. Clinicopathological heterogeneity within this tumour creates a diagnostic challenge in sorting it out from its differential diagnoses. Here we report a 42-year-old female patient presenting with a mass in the right nasal cavity for two months. With an imaging impression of a right ethmoidal polyp with a remote possibility of malignant etiology, a final diagnosis of sinonasal synovial sarcoma was made with the help of a panel of IHC antibodies. A high degree of suspicion along with a detailed work up is required to accurately diagnose synovial sarcoma in unlikely locations such as nasal cavity. Keywords: Synovial sarcoma, Nasal cavity, TLE­1, BCL2, SOX 10

Small (≤5 cm) soft tissue tumors of the extremity and superficial trunk: MRI features and demographic characteristics associated with malignancy.

Although a substantial proportion of small soft tissue tumors are malignant, magnetic resonance imaging (MRI) features and demographic characteristics associated with these tumors have not been well described. To investigate the MRI features and demographic characteristics associated with small (≤5 cm) malignant soft tissue tumors, and to identify independent predictors that allow differentiation of small benign and malignant soft tissue tumors. This retrospective study evaluated patients who underwent surgical excision of small soft tissue tumors of the extremities and superficial trunk, and preoperative contrast-enhanced MRI. Seven MRI findings (tumor depth, tumor-fascia relationship, heterogeneity of signal intensity, necrosis, peritumoral edema, peritumoral enhancement, and margin) and two demographic parameters (age and sex) were included in univariate and multivariate logistic regression analyses to identify independent predictors of small malignant soft tissue tumors. A total of 221 patients (102 men; mean age=45.6 ± 17.6 years) with 72 malignant and 149 benign tumors were included. In the univariate analysis, peritumoral edema (odds ratio [OR] = 3.854; P < 0.001) and peritumoral enhancement (OR = 3.966; P < 0.001) and patient age (≥46 years) (OR = 2.154; P = 0.009) were significantly associated with malignancy. Multivariate analysis showed that peritumoral enhancement on MRI (OR = 3.728; P < 0.001) and patient age (≥46 years) (OR = 1.907; P = 0.036) were independent predictors of malignancy. The combination of these two parameters showed accuracy of 75.1%, sensitivity of 55.6%, and specificity of 84.6% to predict malignancy. Among several MRI and demographic features, the presence of peritumoral enhancement on MRI and patient age (≥46 years) were independent predictors of malignancy in small soft tissue tumors.

Novel reconstruction method by mega-prosthesis wrapped with vancomycin-containing cement after resection of malignancies.

To introduce wrapping vancomycin-containing cement around a mega-prosthesis (MP) as a novel method to prevent prosthetic joint infection after reconstruction surgery for malignant bone and soft tissue tumors. Five patients with malignant bone and soft tissue tumors treated at our hospital from April 2009 to December 2019 were included. The average age was 71.4 years. Four males and one female were included. Three patients had a bone tumor, and two had a soft tissue tumor. Three right thighs and two left femurs were affected. These tumors were identified histologically as undifferentiated pleomorphic sarcoma, spindle cell sarcoma, diffuse large cell B-cell lymphoma, metastasis of renal cancer, and metastasis of lung cancer. All patients underwent tumor resection and reconstruction with a MP. In all cases, vancomycin-containing cement (2 g/40 g) was wrapped around the implant at the extension. The average follow-up period was 30.4 months. We surveyed whether infection occurred after surgical treatment. We also investigated the Musculoskeletal Tumor Society score and clinical outcome. We observed no postoperative infection. One case of local recurrence was observed, and a hip dissection was performed. The Musculoskeletal Tumor Society score was 79.26 ± 1.26 (mean ± standard deviation) (range: 76-80.3). Three patients remained disease-free, one survived but with disease, and one died of disease. Wrapping vancomycin-containing cement around the MP may be a useful method of preventing postoperative joint infections.

Role of Histopathology in diagnosis of Soft Tissue Tumors at Tertiary Care Center

Introduction: Soft tissue tumors (STTs) are a diverse group of neoplasms. It varies from the most common benign tumors to some self-limited lesions and rare malignant soft tissue tumors. Because of the overlap in their clinical and radiological characteristics, histopathology is crucial in determining their diagnosis.&#x0D; Objectives: To study the relative frequencies of types of soft tissue tumors (benign, intermediate and malignant) as well as their histological pattern in relation to age, gender and site of distribution.&#x0D; Methodology: This was a retrospective study conducted over a period of three years from January 2018 to December 2020 in the Department of Pathology, Dhulikhel Hospital, Kavre Nepal. This study included STTs of all the anatomical sites excluding STTs of the female reproductive system. The tumors were classified as benign, intermediate, or malignant using the WHO 2013 classification of soft tissue and bone tumors. The sarcomas were further graded into grades 1, 2, and 3 using the FNCLCC (French Federation Nationale des Centres de Lutte Contre le Cancer) grading system. Immunohistochemistry (IHC) and special staining procedures were also performed as and when required. &#x0D; Result: A total of 198 STTs were studied, of which benign cases were 191 (96.45%), intermediate 03 (1.52%) and malignant 04 (2.03%) in number. The most common type of STTs was vascular tumors 77(38.89%) followed by adipocytic tumors 52(26.26%) and nerve sheath tumors 13(6.56%). STTs were most common in the third decade of life, with the head and neck region being the commonest site. All four malignant tumors were categorized using the FNCLCC grading system. There were three cases of Grade 2 sarcoma and one case of Grade 3 sarcoma.&#x0D; Conclusion: The most common benign tumors were vascular tumors followed by adipocytic tumors and nerve sheath tumors. STTs were most common in the third decade of life. Benign tumors were more common than malignant tumors. In comparison to malignant tumors, benign tumors were found at a younger age group. STTs were found mainly in the head and neck region followed by upper and lower limbs. Despite the fact that molecular diagnostics is gaining popularity in the field of soft tissue tumors, histopathology still remains the gold standard.