Undiagnosed Intra-articular Synovial Hemangioma: A Rare Cause of Knee Pain and Swelling.

INTRODUCTION: Synovial hemangioma is a benign soft-tissue tumor of vascular origin. Hemangioma only accounts for 1% of all bone lesions and is mostly an incidental finding among the primary skeleton tumors. A delay in diagnosis results in joint degeneration and osteoarthritic damage because of infiltrating tumor growth. CASE PRESENTATION: We presented a rare case of an intra-articular synovial hemangioma in a 13- year-old pediatric patient who was asymptomatic for 5 years. She attended orthopedics OPD at AIIMS, Mangalagiri. Surgical excision of the mass and partial synovectomy was done. Synovial hemangioma came out to be the diagnosis following a histologic study. CONCLUSION: As radiography has limited diagnostic ability, synovial hemangiomas are difficult and challenging to identify on an outpatient basis. Histological examination and magnetic resonance imaging are extremely helpful. To minimize the hemarthrosis risks, early complete excision can be used as the best treatment modality.

Synchronous carcinoma and sarcoma in a burn scar: a treatment conundrum.

Marjolijn's ulcer is a malignant ulcer in a burn scar. Types of malignancy are squamous cell carcinoma, basal cell carcinoma and malignant melanoma. Soft tissue sarcoma case reports indicate only one type of cancer. We present a patient in her 60s with a 10-year-old burn scar developing a biopsy-proven squamous cell carcinoma on the lateral aspect of the left thigh with metastatic superficial inguinal node. A wide excision and grafting of ulcer with ilioinguinal dissection done on left side. On the 12th postoperative day 2, subcutaneous swellings adjacent to the grafted area developed, on biopsy revealed to be pleomorphic sarcoma. PET CT scan revealed tumour deposits in the muscles of the left lower limb, liver and lung. There are no case reports of synchronous carcinoma and sarcoma in a burn scar. The case is reported for its rarity and the decision-making dilemma.

Explainable Model Using Shapley Additive Explanations Approach on Wound Infection after Wide Soft Tissue Sarcoma Resection: "Big Data" Analysis Based on Health Insurance Review and Assessment Service Hub.

Background and Objectives: Soft tissue sarcomas represent a heterogeneous group of malignant mesenchymal tissues. Despite their low prevalence, soft tissue sarcomas present clinical challenges for orthopedic surgeons owing to their aggressive nature, and perioperative wound infections. However, the low prevalence of soft tissue sarcomas has hindered the availability of large-scale studies. This study aimed to analyze wound infections after wide resection in patients with soft tissue sarcomas by employing big data analytics from the Hub of the Health Insurance Review and Assessment Service (HIRA). Materials and Methods: Patients who underwent wide excision of soft tissue sarcomas between 2010 and 2021 were included. Data were collected from the HIRA database of approximately 50 million individuals' information in the Republic of Korea. The data collected included demographic information, diagnoses, prescribed medications, and surgical procedures. Random forest has been used to analyze the major associated determinants. A total of 10,906 observations with complete data were divided into training and validation sets in an 80:20 ratio (8773 vs. 2193 cases). Random forest permutation importance was employed to identify the major predictors of infection and Shapley Additive Explanations (SHAP) values were derived to analyze the directions of associations with predictors. Results: A total of 10,969 patients who underwent wide excision of soft tissue sarcomas were included. Among the study population, 886 (8.08%) patients had post-operative infections requiring surgery. The overall transfusion rate for wide excision was 20.67% (2267 patients). Risk factors among the comorbidities of each patient with wound infection were analyzed and dependence plots of individual features were visualized. The transfusion dependence plot reveals a distinctive pattern, with SHAP values displaying a negative trend for individuals without blood transfusions and a positive trend for those who received blood transfusions, emphasizing the substantial impact of blood transfusions on the likelihood of wound infection. Conclusions: Using the machine learning random forest model and the SHAP values, the perioperative transfusion, male sex, old age, and low SES were important features of wound infection in soft-tissue sarcoma patients.

RAF1-rearranged Spindle Cell Mesenchymal Tumor With Calcification and Heterotopic Ossification: A Case Report and Review of Literature.

We report an exceptional case of a spindle cell mesenchymal tumor with S100 and CD34 co-reactivity, which harbored a SLMAP::RAF1 fusion. To the best of our knowledge, this is the second case of a spindle cell mesenchymal tumor with S100 and CD34 co-reactivity with this specific fusion. Remarkable is the presence of calcification and heterotopic ossification in the center of our lesion, a feature that, to our knowledge, has not been described yet in RAF1-rearranged spindle cell mesenchymal tumors.

Nodular cystic fat necrosis: a distinctive rare soft-tissue mass.

We report the case of a 34-year-old female who was evaluated for a right lower extremity soft-tissue mass, found to be a large cystic lesion bound by fibrous tissue containing innumerable, freely mobile nodules of fat. Her presentation suggested the diagnosis of nodular cystic fat necrosis (NCFN), a rare entity that likely represents a morphological subset of fat necrosis potentially caused by vascular insufficiency secondary to local trauma. Her lesion was best visualized using MRI, which revealed characteristic imaging features of NCFN including nodular lipid-signal foci that suppress on fat-saturated sequences, intralesional fluid with high signal intensity on T2-weighted imaging, and a contrast-enhancing outer capsule with low signal intensity on T1-weighted imaging. Ultrasound imaging offered the advantage of showing mobile hyperechogenic foci within the anechoic cystic structure, and the lesion was otherwise visualized on radiography as a nonspecific soft-tissue radiopacity. She was managed with complete surgical excision with pathologic evaluation demonstrating, similar to the radiologic features, innumerable free-floating, 1-5 mm, smooth, nearly uniform spherical nodules of mature fat with widespread necrosis contained within a thick fibrous pseudocapsule. Follow-up imaging revealed no evidence of remaining or recurrent disease on postoperative follow-up MRI. The differential diagnosis includes lipoma with fat necrosis, lipoma variant, atypical lipomatous tumor, and a Morel-Lavallée lesion. There is overlap in the imaging features between fat necrosis and both benign and malignant adipocytic tumors, occasionally making this distinction based solely on imaging findings challenging. To our knowledge, this is the largest example of NCFN ever reported.

[Surgical treatment of cervical neurovascular bundle tumors]. / Khirurgicheskoe lechenie bol'nykh s opukholyami sosudisto-nervnogo puchka shei.

OBJECTIVE: To determine the main principles of a patient-oriented individual approach to diagnosis and surgical treatment of cervical neurovascular bundle tumors considering the capabilities of neurosurgical hospital. MATERIAL AND METHODS: There were 92 patients with cervical soft tissue tumors affecting neurovascular bundle. Age of patients ranged from 9 to 81 years (mean 47). There were 65.1% women and 34.9% men. We found chemodectoma (47.4%), neurofibroma (15.8%), neurinoma (13.2%), papillary thyroid cancer (5.3%), salivary gland heterotopia (5.3%), salivary gland adenocarcinoma (5.3%), Hodgkin lymphoma (2.6%), hemangioendothelioma (2.6%) and cavernous lymphangioma (2.6%). Diagnostic algorithm included neurological examinations, Doppler ultrasound of supra-aortic arteries, transcranial ultrasound of cerebral vessels, MRI of cervical soft tissues, CT-AG, MR-AG, CT-perfusion, direct selective angiography. RESULTS: A total of 94 surgical interventions were performed. All surgeries were performed using surgical optics and neurophysiological monitoring of cranial nerves IX, X, XII. We chose resection technique depending on localization, histological features and blood supply of tumor. En-bloc resection was performed in 46 cases, removal of fragments - in 23 cases, intracapsular resection of tumor followed by resection of the capsule - in 26 cases. Total and subtotal resection was performed in 68 (72%) and 23 (24%) cases, respectively. Three (4%) patients underwent partial resection of infiltrative tumors for carotid artery decompression and histological analysis. In 76% of cases, baseline symptoms of disease regressed after surgery. Persistent moderate bulbar disorders were observed in 16 patients (17%). Ischemic complications with additional surgical interventions were observed in 2 cases. CONCLUSION: Patients with cervical soft tissue tumors require individual approach regarding choosing the optimal surgical treatment including possible preoperative embolization of tumor, en-bloc or intracapsular resection and carotid artery repair.

Don't miss it: Extremity-located cyst hydatid may mimic soft tissue tumors.

OBJECTIVES: In this study, we present our experience in patients with hydatid cysts located intramuscularly. PATIENTS AND METHODS: Between May 2018 and May 2023, a total of 11 patients (3 males, 8 females; mean age: 29.1±13.6 years; range, 8 to 56 years) with intramuscular hydatid cysts were retrospectively analyzed. Demographic data, laboratory values, serological test results, location and size of the cyst, radiological imaging findings, and complications were recorded. RESULTS: The mean follow-up was 44.3±17.3 (range, 5 to 60) months. The mean mass size at the time of admission was 5.4±3.3 (range, 2 to 14) cm. Serologic tests were positive in the majority of cases (72.7%). Eosinophilia was negative in 72.7% patients. The rate of isolated muscle involvement was 81.8%. The rate of lower extremity involvement was 72.7%. The most common involvement was leg (36.4%), thigh (18.2%), and shoulder (18.2%). One patient developed compartment syndrome after cyst rupture during neoadjuvant antihelmintic therapy. There was no recurrence in any of the patients. CONCLUSION: Hydatid cysts should be considered in the differential diagnosis of slowly growing, deeply located, painless soft tissue masses, particularly in endemic areas. Although it is a rare complication, compartment syndrome may develop after spontaneous cyst rupture. Neoadjuvant antihelmintic chemotherapy can reduce complications. The combination of total surgical excision and chemotherapy yields successful results in the treatment of hydatid cysts located in the muscle.

Analysis of Risk Factors and Impact of Tumor Size in Radical Soft Tissue Sarcoma Resection.

INTRODUCTION: There is relatively little current literature analyzing predictive factors of postoperative complications in radical soft tissue sarcoma (STS) resection. The goal was to analyze risk factors based on STS size ( < 5 cm vs. > 5 cm) with regard to STS resection in a large up-to-date, multi- center, population-based study. Additionally, we sought to determine any independent risk factors for the development of postoperative complications. METHODS: Our study was completed through a retrospec- tive analysis of 2005-2014 American College of Surgeons National Surgical Quality Improvement Program (ACS- NSQIP). Data were queried for patients undergoing radical resection for soft tissue tumor based on CPT code. Univari- ate analysis, t-test, and multivariate logistic regressions were employed adjusting for patient demographic, preoperative, and intraoperative variables in order to identify patient- and surgery-specific predictive factors for patients who devel- oped complications. RESULTS: Based on the 1,845 patients who met the inclu- sion criteria, 1,709 (92.62%) had a STS smaller than 5 cm and 136 (7.37%) had tumors larger than 5 cm. Results indicate that larger tumors yield greater risk and greater po- tential for wound complications. Specifically, adult patients who had radical resection of soft tissue tumors greater than 5 cm were more likely to have inpatient status, history of smoking, hypertension, disseminated cancer, chemotherapy and radiation, and were more likely to have longer length of stay in the hospital. CONCLUSION: The results indicate that larger tumors (> 5 cm) carry greater risk for complications. We hypothesize that this may be due to larger tumors being more invasive and requiring greater surgical manipulation. As such, it is important to provide appropriate counseling and proper preoperative planning for these patients.

Glomus Tumor Within the Tensor Fascia Lata: A Case Report.

BACKGROUND Glomus tumors are rare, benign, soft-tissue lesions, usually occurring in the hand, but they can occur in other regions of the body, such as the thigh. Most of the time, extradigital glomus tumors are difficult to diagnose, and symptoms can persist for a long time. The usual clinical presentations consist of pain, tenderness at the site of the tumor, and hypersensitivity to cold. CASE REPORT We report a case of a GT of the proximal thigh in a 39-year-old man with left thigh pain without palpable mass for several years, without clear diagnosis. He had pain and hyperesthesia exacerbated by running. The patient was diagnosed initially by ultrasound imaging, which revealed a round, solid, hypoechoic, homogeneous mass in the left upper thigh. Magnetic resonance imaging (MRI) with contrast showed a well-defined intramuscular lesion in the tensor fascia lata. A percutaneous biopsy was done through ultrasound guidance, followed by excisional biopsy and immediate pain relief. CONCLUSIONS Glomus tumors of the thigh are a rare neoplasm, especially in the proximal thigh; they are difficult to diagnose and are associated with morbidity. Diagnosis can be made through a systematic approach and simple investigation, such as via ultrasonography. A percutaneous biopsy can help in drawing up a management plan, and malignancy must be considered if the lesion is suspicious. Symptoms can persist in case of incomplete resection or unrecognized synchronous satellite lesions; thus, symptomatic neuroma should be considered.

Concomitant tuberculosis of the iliotibial band mimicking a soft tissue tumor and tuberculous trochanteric bursitis: a case report emphasizing on magnetic resonance imaging findings.

The risk of tuberculosis (TB) increases in immunocompromised patients. Multiple myeloma is considered a risk factor for TB and myeloma patients with TB have a higher mortality rate than those without TB. Herein, we report a case of concomitant TB of the iliotibial band mimicking a soft tissue tumor and tuberculous trochanteric bursitis in a patient with multiple myeloma. In this article, the characteristic magnetic resonance imaging (MRI) findings were low T2 signals in the cystic fluid lesion, a dark T2 signal rim, and peripheral rim enhancement. These results could help differentiate TB of the iliotibial band and trochanteric bursitis from other pathologies. If the abovementioned findings were observed in immunocompromised patients, extrapulmonary TB may be expected even if chest radiographs are normal.

Primitive Myxoid Mesenchymal Tumor of Infancy With Fatal Hemorrhage In Utero: A Case Report and Literature Review.

Primitive myxoid mesenchymal tumor of infancy (PMMTI) is a rare soft tissue sarcoma in childhood. We present the case of a newborn male who experienced a severe hemorrhage in utero from the tumor on the scalp. He died at the age of 24 hours owing to hemorrhagic shock. The tumor was posthumously diagnosed as PMMTI. A literature search indicated that cases of severe hemorrhage from soft tissue sarcomas in utero or at birth are limited to infantile fibrosarcoma. This is the first case of PMMTI with massive hemorrhage. Clinicians must be aware of hemorrhagic complications of PMMTI.

A 60-Year-Old Woman with Symptoms of Combined Carpal Tunnel Syndrome and Cubital Tunnel Syndrome Due to an Elastofibroma Causing Compression of the Median and Ulnar Nerves.

BACKGROUND Cubital tunnel syndrome results from pressure or stretching of the ulnar nerve, and carpal tunnel syndrome involves the median nerve. Elastofibroma is a rare, benign, slow-growing soft-tissue tumor that commonly occurs as a bilateral infrascapular tumor in elderly women. This report is of a 60-year-old woman who presented with combined carpal tunnel syndrome and cubital tunnel syndrome due to an elastofibroma causing compression of the median and ulnar nerves. CASE REPORT We report the case of a 66-year-old woman with left-hand numbness, tingling along the fingers, sleep disturbance, and weakness in pinching or holding objects for an extended period. The clinical examination and nerve conduction studies established the diagnosis of combined carpal tunnel syndrome (CTS) and cubital tunnel syndrome (CuTs) complicated by intrinsic muscle wasting. The patient underwent left carpal and cubital tunnels release surgery and end-to-side anterior interosseous nerve transfer to the motor component of the ulnar nerve. Pathologic evaluation of the entire specimen showed collagen bundles alternating with refractive cylinders stained with Verfoeff-van Gieson elastic stain. CONCLUSIONS This report is of a rare case of a histologically-confirmed single, peripheral, benign elastofibroma involving compression of the ulnar and median nerves. This case highlights the importance of histopathology in diagnosing rare soft-tissue tumors arising at an uncommon site and presenting with rare symptoms.

Multifocal Sarcoma Mimicking Superficial Vein Thrombosis of the Leg: A Case Report.

BACKGROUND Leiomyosarcoma is a common tumor found in soft tissue. In relation to the vascular system, leiomyosarcoma appears as the most common malignancy characterized by poor prognosis. Leiomyosarcomas of the leg large vessels often occur late, and their appearance can imitate vein thrombosis with symptoms such as soft tissue swelling or mild pain, and can be misdiagnosed. Peripheral vascular leiomyosarcomas are rare. Especially leiomyosarcomas of the great saphenous vein are uncommon. The tumors develop on the media basis and grow from endovascular to exovascular order. Distant metastasis can be identified and worsen prognosis. CASE REPORT We present a case of a 61-year-old female patient with varicose vein disease complicated by recurrent superficial vein thrombosis. After 2 months of conservative treatment, while waiting for admission to the department of surgery, she developed additional symptoms. Clinical examination on the day of admission revealed several tumors along and near the great saphenous vein on the left limb below the knee. The diagnosis of leiomyosarcoma was confirmed after the surgery, involving excision of the saphenous vein, including tumors formed on its course. Preoperative clinical and ultrasound findings did not suggest malignancy. CONCLUSIONS Leiomyosarcoma of the great saphenous vein is an extraordinarily rare tumor originating from the middle layer of the vessel, mimicking unspecific symptoms and complicating and delaying diagnosis. In every case of vascular or perivascular lesions, a detailed examination and diagnosis it is required, and even unlikely clinical scenarios should be considered.

Intracranial phosphaturic mesenchymal tumors. A case report and review of literature.

Most osteomalacia-inducing tumors (OITs) are phosphaturic mesenchymal tumors (PMTs) that secrete fibroblast growth factor 23 (FGF23). These tumors usually occur in the bone and soft tissues, and intracranial OITs are rare. Therefore, intracranial OIT is difficult to diagnose and treat. This paper presents a case of intracranial OIT and shows a review of previous cases. A 45-year-old man underwent nasal cavity biopsy and treatment with active vitamin D3 and neutral phosphate for hypophosphatemia. Amplification of FGF23 mRNA level within the tumor was detected. Subsequently, the surgical specimen was diagnosed with a PMT and was considered the cause of the patient's osteomalacia. The patient was referred to a neurosurgery department for the excision of the intracranial tumor extending to the nasal cavity. After tumor removal, the serum levels of FGF23 and phosphorus were normalized as compared to preoperative those. The patient remains disease-free, without additional treatment, approximately 10 years after surgery, with no tumor recurrence. As per the literature, intracranial OITs usually occur in patients aged 8-69 years. Bone and muscle pain are major complaints. Approximately 60% of the patients reported previously had symptoms because of intracranial tumors. In some cases, it took several years to diagnose OIT after the onset of the osteomalacia symptoms. Laboratory data in such cases show hypophosphatemia and elevated FGF23 levels. Because FGF23 levels are associated with the severity of osteomalacia symptoms, total tumor resection is recommended. PMT and hemangiopericytoma (HPC) are histologically similar, but on immunochemistry, PMT is negative for signal transducer and activator of transcription 6 (STAT6), whereas HPC is positive. FGF23 amplification is seen in PMTs but not in HPCs. Therefore, the analysis of FGF23 and STAT6 was helpful in distinguishing PMTs from HPCs. In cases of hypophosphatemia and osteomalacia without a history of metabolic, renal, or malabsorptive diseases, the possibility of oncogenic osteomalacia should be considered.

Real-life incidence of cardiotoxicity and associated risk factors in sarcoma patients receiving doxorubicin.

BACKGROUND: Anthracycline induced cardiotoxicity is well recognized but only few data exist in sarcoma patients. This study retrospectively aimed to analyze sequential Cadmium Zinc Telluride (CZT)-multigated equilibrium radionuclide angiography (ERNA) for monitoring left ventricular ejection fraction (LVEF) and to assess the real-life incidence of cardiotoxicity in sarcoma patients receiving doxorubicin based chemotherapy. MATERIALS AND METHODS: A retrospective analysis was performed on all sarcoma patients referred to Herlev University Hospital between 2012 and 2015. Cardiotoxicity was defined as a decline in LVEF of > 10% percentage point to a LVEF < 50% as compared to baseline. Early cardiotoxicity was defined as < 1 year and late cardiotoxicity as ≥ 1 year. Recovery of cardiotoxicity was defined as a LVEF ≥ 50%. RESULTS: A total of 149 patients were referred, 75 (50%) sarcoma patients were included. The main reason for exclusion was that only one CZT-ERNA had been performed in 50 (68%) of the patients. Twenty-three (31%) of the patients experienced cardiotoxicity, 11 (48%) were female, mean age was 56.9 years. Early cardiotoxicity was observed in 16 (70%) of the patients and 11 (48%) experienced clinical symptoms of cardiotoxicity at diagnosis. Recovery of LVEF was seen in 12 (55%) of the patients and persistent recovery in 10 (45%). The diastolic blood pressure at baseline was positively and significantly associated with a higher risk of developing cardiotoxicity (Relative Risk (RR): 1.039 (95% CI= 1.001 - 1.079; p = 0.042)). The median survival was 1.4 years (range 0.5 - 2.2 years) for patients with metastatic disease versus 3.9 years (range 1.5 - 6.4 years) (p = 0.009) for localized disease at baseline. CONCLUSION: Cardiotoxicity is a relative frequent complication in sarcoma patients treated with doxorubicin based chemotherapy and the diastolic blood pressure at baseline was significantly associated with a higher risk of developing cardiotoxicity.

The transformation of isolated gastric myeloid sarcoma into acute myeloid leukemia presenting with a complex karyotype and TLS-ERG gene fusion: A case report.

RATIONALE: Isolated myeloid sarcoma (MS) is characterized by the rapid proliferation of myeloblasts of acute myeloid leukemia (AML), without any blood or bone marrow involvement. This disease can manifest with extramedullary organ involvement, such as the skin, lymph nodes, bone, brain, breast cervix, and visceral organs, while the occurrence of myeloid sarcomas in the stomach is rare. Isolated MS has been associated with acute myeloid leukemia (AML), but the rapid progression of MS to acute myeloid leukemia with a complex karyotype and TLS-ERG fusion gene is even rarer. PATIENT CONCERNS: A 33-year-old woman suffered from persistent epigastric pain accompanied by two months of anorexia and nausea, as well as 1-week of melena. DIAGNOSIS: This patient was initially diagnosed with gastric MS that eventually transformed into AML with a complex karyotype and TLS-ERG fusion gene, 4 months later. INTERVENTIONS: Only palliative care, including nutrition support, antacids, blood transfusion, anti-infection methods were used on this patient to determine the cachexia status and the family's requirement. OUTCOMES: Routine follow-up results demonstrated this patient had died due to cerebral hemorrhage five months after the diagnosis of MS. LESSONS: Comprehensive integration of patient history, imaging features, mass and bone marrow biopsy, and molecular cytogenetic may provide insights that could help us avoid the misdiagnosis of gastric MS. Isolated gastric MS can rapidly progress to AML with a poor prognosis if the patient does not receive appropriate treatment.

Subsequent malignant neoplasms in the pediatric age in retinoblastoma survivors in Argentina.

BACKGROUND: Retinoblastoma survivors in low- and middle-income countries are exposed to high-intensity treatments that potentially place them at higher risk of early subsequent malignant neoplasms (SMNs). METHODS: We followed 714 (403 [56.4%] nonhereditary and 311 [43.5%] hereditary) retinoblastoma survivors diagnosed from August 1987 to December 2016, up to the age of 16 years. We quantified risk of SMNs with cumulative incidence (CI) and standardized incidence ratios (SIR) analysis. Multivariate regression Cox model was used to determine the association of treatments and risk of SMNs. RESULTS: Median follow-up was of 9 years (range: 0.18-16.9) and 24 survivors (3.36%) developed 25 SMNs (n = 22 hereditary, n = 2 nonhereditary). SMNs included sarcomas (osteosarcomas, Ewing sarcomas, rhabdomyosarcomas; n = 12), leukemias (n = 5), and central nervous system tumors (CNS; n = 3). All cases of acute myeloid leukemia (AML) and most of Ewing sarcomas occurred within 5 years of retinoblastoma diagnosis. The type of SMN was the main indicator of mortality (five of five patients with leukemias, six of 12 with sarcomas, and zero of three with CNS tumors died). Compared to the general population, radiation increased the risk of Ewing sarcoma in hereditary survivors by 700-fold (95% CI = 252-2422.6) and chemotherapy increased the risk of AML by 140-fold (95% CI = 45.3-436). The CI of SMNs for hereditary survivors was 13.7% (95% CI = 8.4-22.1) at 15 years. CONCLUSION: Retinoblastoma survivors from Argentina are at higher risk of developing SMNs early in life compared to the general Argentinean population, especially those treated with radiation plus chemotherapy. AML and Ewing sarcoma presented within 5 years of retinoblastoma diagnosis are associated with chemotherapy and radiation exposure.