Non-extremity Site Research Articles

Analysis of Risk Factors for Kasabach Merritt Phenomenom in Children With Kaposiform Hemangioendothelioma

BackgroundThis study generalized and analyzed the clinical attributes observed in patients afflicted with Kaposiform hemangioendothelioma (KHE) with the aim of elucidating the risk factors contributing to the manifestation of Kasabach-Merritt phenomenon (KMP). MethodsWe retrospectively analyzed 96 pediatric cases diagnosed with KHE at the Children's Hospital of Fudan University from January 2013 to December 2021. Among them, 62 patients (65%) showed KMP (KHE + KMP group), while 34 patients (35%) did not (KHE-KMP group). The risk factors for KMP associated with KHE were analyzed using univariate analysis and binary logistic regression analysis, comparing the differences between KHE + KMP group and KHE-KMP group. ResultsUnivariate analysis indicated no statistically significant differences between the two groups in gender, prematurity, family history, or color of involved skin. However, statistically significant differences were observed in age of onset, lesion site, and lesion depth. Multivariate analysis revealed significant associations: children with onset age ≤1 month had a 51-fold increased risk of KMP compared to those with onset age >1 month (95% CI 5.238–501.663); non-extremity lesion sites exhibited a 21-fold higher risk of KMP compared to extremity sites (95% CI 3.970–105.958); deeper lesions conferred a 5-fold higher risk of KMP compared to superficial lesions (95% CI 1.073–21.005); lesions >60 mm carried a 17-fold higher risk of KMP compared to lesions ≤60 mm (95% CI 2.999–96.157).A comprehensive predictive model was developed using the fitting formula: Logit (P) = 3.937∗(age at onset) + 1.558∗(lesion depth) + 3.021∗(lesion site) + 2.832∗(lesion size), demonstrating an accuracy of 82.9%. Furthermore, a scoring system was established to assess the likelihood of KMP occurrence. Children diagnosed with KHE were likely to have KMP if their score exceeded 72.5, as determined by Receiver Operating Characteristic (ROC) curve analysis. ConclusionAge of onset ≤1 month, deeper lesions, non-extremity sites, and lesions ＞60 mm are independent risk factors for KMP in children with KHE. The cumulative presence of these factors escalates the likelihood of KMP development. Additionally, the identification of these factors allows for the early recognition of potential KMP cases among children with KHE, facilitating prompt therapeutic intervention. Category of the manuscriptClinical Research article. Level of evidenceLEVEL Ⅲ.

Triple Local Recurrence of Synovial Sarcoma: A Case Report

Background: Synovial Sarcoma (SS) hardly appears on non-extremity sites (20% of cases). Even rarer is a local recurrence, which occurs between 10-30% of cases. Here, we present a rare case of hip Synovial Sarcoma, which recurs three times.Case Report: A 33-year-old male came to our outpatient clinic with chief complaints of a sudden ingrowing mass on his left hip. The lump is the size of a chicken egg and doesn't get bigger over time. There was palpable tenderness felt whenever he touched the lump. The patient was diagnosed with SS of the left hip with IIB stage from additional radiographic and histopathologic examination. Surgical-wide resection had been done three years before the visit, and chemotherapy six times. The lump reappeared one year before the visit, and the patient underwent a second surgery. The mass re-surfaced within the same region on the current visit for the third time, and he underwent the same surgical treatment afterward. Two years after the third surgery and adjuvant chemotherapy, the patient had no complaint about a lump and planned a radiograph evaluation.Discussion: Detailed examination from history taking, physical examination, histopathologic, and radiological results is important to determine appropriate staging. With proper staging, physicians can better understand disease prognosis and risk of recurrence or metastases.Conclusions: The third recurrence may still happen on SS. Its unique location might contribute to this unique occurrence, and the size of the tumor makes the recurrence rate higher.

Concurrent Radiation and Immunotherapy: Survey of Practice Patterns in the United States

Rapid FDA approval of multiple immunotherapy (IT) drugs for a variety of advanced and metastatic cancers, coupled with a current void of published clinical data on toxicity and outcomes from concurrent radiation (RT) and immunotherapy (IT) (CRI), leave radiation oncologists (ROs) with uncertainty regarding their concurrent administration. A survey to determine current practice patterns, experiences, and perceptions of CRI may help guide ROs until more definitive data is available. A survey was emailed to ROs over a 1-week period in February 2018. Responses were collected on Survey Monkey and descriptive statistics were used to report results. CRI was defined as RT completed within 1 week prior to initial IT infusion through 4 weeks after the final IT infusion. Of the 304 respondents from 44 states, 87.5% had experience treating a patient with CRI, including 50% private and 48% academic physicians. Results from the 266 ROs with prior CRI experience are summarized below. The majority of respondents have had limited experience with CRI: 60%, 56%, and 50% of respondents treated 1-5 patients on nivolumab, pembrolizumab, or ipilimumab, respectively, while 11-21%, 6-11%, and 4-8% have treated 5-10, 10-20 and >20 patients, respectively. 59% used CRI off-study, including 20% who prescribed a definitive RT course with concurrent IT. RT dose mostly was not altered: 13-15% decreased RT dose if treating the C/A/P, while 4-9% decreased dose in the brain, H/N, or extremities. For palliative RT vs SBRT/SRS to non-extremity sites, 65-67% vs 57-60% would not delay initiation of RT from IT infusion, while 12% vs 17-19% would wait ≥2 weeks from last IT infusion. For palliative RT vs SBRT to extremities, 80% vs 70% would not delay RT, but 5 vs 14% would wait 2 weeks. The two most common reasons for not offering CRI were due to concerns of increased toxicity (51%) or waiting to see response from newly initiated IT (40%). Moderate and significant toxicities were rare for all treatment sites and RT doses (medical intervention 6%, hospitalization/death <1%). 98% of ROs have not routinely prescribed prophylactic steroids for CRI. 49-59% of respondents remained uncertain if CRI vs RT monotherapy was improving local control or increasing abscopal responses, yet more ROs believed CRI with SBRT/SRS vs palliative RT had better local control (35% vs 25%) and higher rates of abscopal responses (40% vs 26%). Amongst the 38 ROs without prior CRI experience, 60% were concerned about increased toxicity, 32% would wait >2 weeks from IT to start SBRT/SRS, and 25% would offer prophylactic steroids. Despite concerns for toxicity, ROs with CRI experience reported minimal toxicities regardless of treatment site or RT dose. Most ROs did not alter RT dose, use prophylactic steroids, or delay starting RT from last IT infusion. Uncertainty remains about improved local control outcomes and abscopal responses from CRI, yet there is a perception that concurrent SBRT offers better outcomes than palliative RT.

Clinicopathological analysis of 12 cases of acquired digital fibrokeratoma

Objective To investigate clinical and pathological characteristics of acquired digital fibrokeratoma (ADF) . Methods Clinical data were collected from 12 patients with ADF, and their clinical manifestations, histopathological features, treatment and prognosis were analyzed retrospectively. Results The mean age at onset of ADF was 39.75 ± 17.69 years (range, 17-64.5 years) , and the average disease duration was 4.83 ± 5.77 years (range, 0.5-20 years) . The ratio of male to female was 1.4∶1. Most skin lesions were polymorphic, and mainly involved fingers, toes and nail grooves. Sometimes non-extremity sites are also involved. Histopathological examination of skin lesions showed epidermal hyperkeratosis, acanthosis and broadened dendroid rete ridges. The dermis consisted of mature collagen bundles, fibroblasts and a large number of dilated capillaries. The characteristic manifestation was that most collagen bundles were vertical to the epidermis. Conclusions ADF, a rare benign fibrous tumor with unknown etiology, usually occurs in adults as a solitary lesion on fingers or toes, sometimes in non-extremity sites. Surgery and laser therapy are common treatments for ADF with low recurrence rate. Key words: Fingers; Toes; Retrospective studies; Fibrokeratoma, digital, acquired

Outcome and the effect of age and socioeconomic status in 1318 patients with synovial sarcoma in the English National Cancer Registry: 1985-2009.

BackgroundThe role of age as a prognostic factor has been examined in single institutional studies and in larger data sets from the SEER database, showing a survival advantage for younger versus adult patients with synovial sarcoma (SS). To further assess the role of age, socioeconomic status and other prognostic factors on outcome for SS, we analysed a contemporary all-age population-based cohort of patients with SS registered in England.MethodsThe data on 1318 synovial sarcomas diagnosed in England between 1985 and 2009 were retrospectively analysed for incidence, and the effect of age, patient characteristics and deprivation on outcome using both univariate and multivariate analysis.ResultsThe incidence of SS increased to 1.4 per million over the time period, the numbers diagnosed in patients under 10 years of age were small. The site or incidence of metastases did not vary between age groups. There were, however, significant differences (p < 0.05) in the 5-year relative survival rates between patients aged 0–19 years and those ≥20 years of age, 76 % and 53 % respectively. Survival was better in localised tumours at an extremity site. In multivariate analysis higher mortality occurred in older patients, non-extremity site, presence of metastases, female adults and a higher deprivation score.ConclusionsSynovial sarcoma in children/teenagers compared with adults, have a similar clinical presentation in this population-based series, but a superior outcome. The finding of socioeconomic deprivation affecting outcome in SS needs further exploration in a complete and contemporary dataset, where all prognostic variables are present.

Primary breast synovial sarcoma: a rare primary breast neoplasm

A primary synovial sarcoma based on the breast is rare. The usual tumours on the breast are carcinomas. Synovial sarcomas account for about 6-9% of soft tissue sarcomas and most commonly develop in the extremity of young adults (80%). The other 20% of synovial sarcomas can arise in non-extremity sites (trunk 8%, retroperitoneal/ abdominal 7%, head and neck 5%) but synovial sarcomas can develop in almost any other anatomical location. We report a case of a young woman who presented with a suspected common breast tumour and started treatment of this tumour with carcinoma neoadjuvant chemotherapy. We were surprised when the pathologist identified a synovial sarcoma in the histopathology study.

Factors associated with survival in the National Registry of Veterans with ALS

The clinical course of patients with ALS is highly variable. While the median survival time from symptom onset is 2-4 years, there are reports of survival ranging from less than a year to more than 40 years. Such variability makes planning difficult for patients and physicians, and complicates clinical trial design. We sought to validate previous predictors of survival and search for new ones using a large group of ALS patients in the National Registry of Veterans with ALS. We were especially interested in how various aspects of military service might affect survival. Subjects were those in the National Registry of Veterans with ALS who had probable or definite ALS (according to El Escorial criteria). A multivariable Cox proportional hazard regression model was used to examine variables for statistical association with ventilator-free survival time (determined from date of first diagnosis). Subjects who had not died or started ventilation by 31 October 2006 were censored. Our group of 1085 US military veterans with ALS was primarily male (98%) and white (94%), with mostly sporadic (95%) and extremity-onset (76%) ALS. Symptom onset occurred at a mean age of 59.3 years (60.6 years for diagnosis). Median survival time from symptom onset was 4.7 years (3.3 years from diagnosis). In our multivariable model, older age at diagnosis (HR 1.41 (95% CI 1.27-1.55) per 10-year increase), non-extremity site of onset (HR 1.55 (1.24-1.94)), and past deployment to Vietnam (HR 1.73 (1.36-2.19)) were all associated with shortened survival. A longer time to diagnosis was associated with better survival (HR 0.77 (0.70-0.84) per one year increase in diagnosis time). In this unique cohort of veterans with ALS, traditional factors of reduced survival remained important. In addition, past deployment to Vietnam was found to be associated with shortened survival as well. This finding could be due to a common exposure, a shared characteristic, an unmeasured confounder, or an enrollment bias. More research will be needed to understand the reasons behind this new finding.

Poorly differentiated synovial sarcoma of the vagina: A case report and a clinical literature review

Synovial sarcomas (SS) account for 5–10% of soft-tissue sarcomas and typically arise in the para-articular regions of adolescents and young adults. Nonetheless, SS can occasionally occur in other regions of the body. Here, we present a first clinical literature report of a patient with an SS arising from the vaginal wall. A 40-year-old patient who presented a necrotic polypoid lesion, measuring 50 mm and extending from the external urethral meatus to the middle part of the anterior vaginal wall. The biopsy showed a poorly differentiated SS with abundant necrosis and a SYT-SSX1 mutation. A staging CT scan was negative for distant metastases. The patient, prior to the radical surgery, received neoadjuvant chemotherapy (ifosfamide and epirubicin) for three cycles. She underwent post-operative external radiotherapy and brachytherapy (50 Gy) due to close margins (<1 mm) in the pathologic specimen. She relapsed 11 and 16 months later with lung metastases, which, both times, were successfully removed by surgical resection. At 24 months from diagnosis, the patient is alive without further evidence of disease. In summary, in the presence of unfavourable prognostic factors, neoadjuvant chemotherapy could be the primary approach to reduce the tumour size and the risk of distant micro-metastases allowing a less aggressive radical surgery if the tumour is located in a non-extremity site. Hence, a multidisciplinary approach, if not influencing overall survival and disease-free survival, may improve the quality of life. In fact, in our patient we obtained a complete clinical control in the pelvis, avoiding pelvic exenteration with neoadjuvant chemotherapy.

Why Do Patients with Low-Grade Soft Tissue Sarcoma Die?

The patterns of failure and mechanisms of sarcoma-specific death are poorly characterized among the minority of patients with low-grade soft tissue sarcoma (STS) who succumb to disease. Between 1982 and 2006, 2,041 patients aged >or=16 years with low-grade STS of all sites were treated with curative intent and prospectively followed at a single institution. Among this cohort, 181 (9%) died from disease (DOD). Overall, 105 patients (58%) died from locally recurrent disease (DOLR), and 59 (32%) died from distant disease (DODR). In 17 patients (9%), the mechanism of sarcoma-related death could not be verified. DOD occurred at a median of 62 months, while median disease-specific survival for the entire cohort was not reached. Median follow-up was 66 months (range 2-431 months). On multivariate analysis, DOD was associated with site, size, and less than R0 resection. For DOLR, site, size, positive margins, liposarcoma histology, and local recurrence (by definition) were significant factors. For DODR, site, histology, and positive margins were not significant factors, while size and local recurrence were. Of DOLR, 80% were retroperitoneal, 68% were liposarcoma, and only 2% were extremity. Conversely, of DODR, extremity (47%) and trunk (18%) were the most common sites, but histology was more variable [liposarcoma 35%, malignant fibrous histiocytoma (MFH) 20%, fibrosarcoma 12%, extraskeletal myxoid chondrosarcoma 10%]. High-grade recurrence rates were comparable between DOLR (27%) and DODR (25%). Among patients with low-grade STS, DOD occurs in approximately 9% of patients. Nonextremity site, larger size, and less than R0 resection are the most important risk factors for DOD, and distinct patterns of recurrence and death are predicted by primary tumor site.

Osteosarcoma as a second malignant neoplasm

Purpose: The aim of this study is to compare primary and secondary osteosarcomas using data from the National Cancer Institute's Surveillance, Epidemiology and End Results (SEER) program. Methods: Osteosarcoma cases were identified in the SEER Cancer Incidence Public-Use Database, 1973–96 (1511 primary and 133 secondary osteosarcomas). Secondary osteosarcomas were further classified as occurring within or outside the previously irradiated field. Comparisons among groups were performed by nominal logistic regression. Survival analyses were performed using Kaplan–Meir and proportional hazards regression. Results: Secondary osteosarcomas were more likely to have a non-extremity primary site and an older age at diagnosis (>40 years) (<0.0001 for both). Secondary osteosarcoma cases continued to be more likely to have a non-extremity site when excluding those occurring within the radiation field. Five-year overall survival was 50% lower for secondary osteosarcomas for both extremity and non-extremity sites. Primary malignancies associated with secondary osteosarcomas included 14 lymphomas/leukemias, six sarcomas, 54 carcinomas, and seven other cancers. Secondary osteosarcomas occurring within a field of radiation were more likely to occur at a younger age, have a malignancy with a primary morphology other than carcinoma, a non-limb site, and a longer duration between their primary malignant neoplasms and the development of osteosarcoma. No difference in the overall survival was noted between secondary osteosarcomas occurring within an irradiated field and those that did not. Conclusions: Secondary osteosarcomas are associated with carcinomas especially in the elderly. Although a 50% decreased 5-year survival was observed for secondary osteosarcomas in this study, this might not reflect the current outcomes with more aggressive therapy.