Management of Diaphragm Tumors.

Diaphragm tumors are very rare, with secondary tumors being more common than primary tumors. The most common benign primary tumors include lipomas and cysts, and malignant primary tumors include rhabdomyosarcoma and leiomyosarcoma. Endometriosis is the most common benign secondary tumor, followed by malignant tumors with localized spread of disease. In addition, widely metastatic disease has been described. Benign lipomas and cysts can be managed conservatively, but more complex or concerning disease typically requires resection. The diaphragm can often be repaired primarily, though any large defect or tension would indicate the need for mesh or an autologous reconstruction.

Unique Case of Rare Non-Neural Granular Cell Tumor of the Rectus Abdominis Muscle.

Background and Objectives: Our report contributes a unique case of a non-neural GCT occurring in an unusual location, with its development during pregnancy adding to its rarity. Materials and Methods: Granular cell tumors (GCTs), also known as Abrikossoff's tumors, are rare neoplasms of Schwann cell origin with predominantly benign behavior. We present a case of a 29-year-old female with a non-neural variant of a GCT discovered incidentally during a cesarean section, situated on the posterior surface of the rectus abdominis muscle. Results: Histologically, the tumor exhibited features consistent with a benign non-neural GCT, confirmed through an immunohistochemical analysis. Despite the atypical presentation and challenging surgical removal due to prior scarring, the patient experienced no postoperative complications and showed no signs of recurrence during follow-up. Conclusions: This case highlights the importance of considering GCTs in differential diagnoses, particularly in unusual anatomical locations, and underscores the favorable prognosis associated with timely surgical intervention.

Rapidly growing intramuscular lipoma: a unique entity of benign lipomas in children.

This report presents a case of an intramuscular lipoma observed in the left back of a healthy female toddler. It was resected after 3 months of observation because of rapid enlargement, raising suspicion of malignancy. Histopathological examination confirmed a diagnosis of intramuscular lipoma without malignant and blastemal components. Intramuscular lipomas are benign neoplasms that mostly appear as a rapidly growing tumour. Several hypotheses regarding the pathogenesis of this characteristic growth pattern have been proposed, including atrophy of the surrounding muscle, reactive adipocytic neoformation and multiple contractive interactions between the lipoma and the surrounding muscle.

Laparoscopic resection of a rare diaphragmatic haemangioma: a case report.

This case report describes the laparoscopic resection of a rare diaphragmatic haemangioma. A 45-year-old male patient was diagnosed incidentally with a left subphrenic mass by computed tomography. Laparoscopic left subphrenic mass excision was performed under general anaesthesia. A phrenic haemangioma was confirmed by postoperative pathology. Tumours originating in the diaphragm are rare, with only approximately 200 cases reported in the past century. The diaphragmatic tumour was determined to be primary because intraoperative imaging showed that the tumour was relatively isolated and had no obvious relationship with the surrounding tissues and organs.

Use of Neoadjuvant Chemotherapy in Elderly Patients With Muscle-Invasive Bladder Cancer: A Population-Based Study, 2006-2017.

Objectives/Introduction Neoadjuvant chemotherapy (NAC) prior to radical cystectomy (RC) for muscle-invasive bladder cancer (MIBC) is associated with survival benefit across patients of all ages, yet it is not offered to many elderly patients. We aimed to evaluate age-based disparity in treatment and outcomes of MIBC. Methods Using the National Cancer Database, we identified patients with MIBC from 2006 to 2017. Use of treatment modalities was compared between age groups. A second analysis compared perioperative outcomes and overall survival (OS) in elderly patients (70 years or older) undergoing RC with NAC vs no NAC. Propensity score weighting (PSW) was used for each analysis. Results In 70,911 patients, use of NAC with RC was lower in patients 70 years or older (7.2% vs 20.9%; P < .001). In patients 70 years or older undergoing RC, NAC was associated with shorter inpatient stay (8.5 vs 9.6 days; P < .001), decreased 30-day readmission (8.6% vs 10.6%; P <.001), and lower 30- and 90-day mortality (1.5% vs 3.1%; P = .01; and 4.9% vs 7.7%; P = .003, respectively). On weighted multivariate regressions, NAC predicted shorter length of stay and lower 30-and 90-day mortality. Elderly patients receiving NAC had improved OS compared with RC alone (P = .0011, 2010-2013; P < .001, 2014-2016). Conclusions Despite increased omission of NAC in patients 70 years or older, elderly patients receiving NAC and RC had improved perioperative outcomes and OS compared with those undergoing RC alone. There may be selection bias unaccounted for with our PSW; however, our results provide compelling evidence that NAC does not compromise surgical outcomes in appropriately selected elderly patients. Patients of advanced age who are candidates for RC should be offered NAC.

A case of intramuscular lumbar myxoma: Uncertainty in the preoperative diagnosis of a spinal soft tissue tumour.

We report an uncommon case of a 76-year-old woman who presented with lower back pain, an intermittent L5 radiculopathy and a right palpable paraspinal mass. Imaging studies revealed a 7-cm lumbar paraspinal pseudo-cystic soft tissue tumour developed in the paravertebral musculature, without a clear radiological diagnosis. Gross total surgical resection was performed, resulting in complete resolution of pain. Histopathological studies revealed an intramuscular (IM) myxoma. With a low positive predictive value of radiological work-up and a poor yield of percutaneous biopsies, surgery remains the mainstay treatment for these rare soft tissue tumours of the lumbar spine. Intramuscular myxomas show excellent postoperative results.

The impact of patient sex on characteristic-adjusted bladder cancer prognosis.

CONTEXT: Bladder cancer is one of the most common malignancies worldwide. Some studies noted sex differences in the prognosis of bladder cancer, but results are inconsistent. SUBJECTS AND METHODS: In this study, we assessed whether women with bladder cancer exhibit a worse prognosis, after adjustment for disease stage, age, and body mass index (BMI), using clinical data from The Cancer Genome Atlas. We used a Student's t-test to compare age and BMI in groups with different sexes. STATISTICAL ANALYSIS USED: The Kaplan-Meier method with log-rank test was used to determine clinical prognosis. RESULTS: The BMI (30.15 vs. 26.68, P = 0.0035) and age (67.54 years vs. 66.01 years, P = 0.045) of female patients with muscle-invasive bladder cancer (MIBC) were higher than those of male patients. The overall survival (OS) prognosis of female patients was worse than that of male patients. After grouping by disease characteristics, the disease-free survival (DFS) and OS prognoses of female patients under 60 years of age were worse than those of male patients. In the group with BMI >24, the OS prognosis of female patients was worse than that of male patients, but no difference was found in DFS prognosis. In the group with BMI ≤24, the DFS prognosis of female patients was worse than that of male patients, but no difference was found in OS prognosis. Compared to males, female patients with Stage III disease demonstrated a worse DFS prognosis and poorer OS prognosis, women with stage T3 demonstrated a worse DFS prognosis, and women with stage N0 demonstrated a poorer OS prognosis. No difference was found in prognosis between male and female patients in all other groups. CONCLUSIONS: In patients with MIBC, women tended to exhibit a worse prognosis than men. More specifically, we found a correlation between prognosis and sex after grouping patients by BMI.

Skeletal muscle metastasis from a gastrointestinal stromal tumor: A case report.

RATIONALE: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Common sites for metastasis are the liver and peritoneum, whereas skeletal muscle metastases are rare. PATIENT CONCERNS: A 59-year-old man with skeletal muscle metastasis was diagnosed during a period of adjuvant imatinib therapy following the recurrence of GIST of the small intestine. DIAGNOSIS: The patient was diagnosed with skeletal muscle metastasis of GIST based on immunohistochemistry and molecular pathology analysis results. INTERVENTION: Extensive resection of the left thigh tumor was performed. The patient underwent whole-exome sequencing of tissue examination. The results suggest that resistance to imatinib may have been developed, and the patient was therefore administered sunitinib instead. OUTCOMES: Complete remission was observed following sunitinib therapy. LESSONS: In cases of skeletal muscle metastasis diagnosed during a period of adjuvant imatinib therapy following the recurrence of a GIST of the small intestine, whole exome sequencing may be used to discover more gene variations.

Adjunct diagnostic strategies in improving diagnostic yields in image-guided biopsies of musculoskeletal neoplasms-A cost-effectiveness analysis.

BACKGROUND: Routine use of adjunct intraprocedural fresh frozen biopsy (FFP) or point-of-care (POC) cytology at the time of image-guided biopsy can improve diagnostic tissue yields for musculoskeletal neoplasms, but these are associated with increased costs. OBJECTIVE: This study aimed to ascertain the most cost-effective adjunctive test for image-guided biopsies of musculoskeletal neoplasms. METHODS: This expected value cost-effectiveness microsimulation compared the payoffs of cost (2020 United States dollars) and effectiveness (quality-adjusted life, in days) on each of the competing strategies. A literature review and institutional data were used to ascertain probabilities, diagnostic yields, utility values, and direct medical costs associated with each strategy. Payer and societal perspectives are presented. One- and two-way sensitivity analyses evaluated model uncertainties. RESULTS: The total cost and effectiveness for each of the strategies were $1248.98, $1414.09, $1980.53, and 80.31, 79.74, 79.69 days for the use of FFP, permanent pathology only, and POC cytology, respectively. The use of FFP dominated the competing strategies. Sensitivity analyses revealed FFP as the most cost-effective across all clinically plausible values. CONCLUSIONS: Adjunct FFP is most cost-effective in improving the diagnostic yield of image-guided biopsies for musculoskeletal neoplasms. These findings are robust to sensitivity analyses using clinically plausible probabilities.

High IL-22RA1 gene expression is associated with poor outcome in muscle invasive bladder cancer.

BACKGROUND: The cell surface interleukin 22 (IL-22) receptor complex is mainly expressed in epithelial and tissue cells like pancreatitis cells. Recent studies described that IL-22R was overexpressed in malignant diseases and was associated with a poor overall survival (OS). The role of IL-22RA1 gene expression in muscle invasive bladder cancer (MIBC) has not been investigated, yet. OBJECTIVES: The aim of this study was to analyze the role of IL-22RA1 gene expression in patients with MIBC. METHODS: In a cohort of 114 patients with MIBC who underwent radical cystectomy, IL-22RA1 gene expression was analyzed with qRT-PCR and correlated with clinical parameters. Furthermore, Kaplan-Meier and Cox regression analysis were performed. For validation, an in silico dataset (TCGA 2017, n=407) was reanalyzed. RESULTS: IL-22RA1 gene expression was independent of clinicopathological parameters like age (P=0.2681), T stage (P=0.2130), nodal status (P=0.3238) and lymph vascular invasion (LVI, P=0.5860) in patients with MIBC. A high expression of IL-22RA1 was associated with a shorter OS (P=0.0040) and disease-specific survival (P=0.0385). Furthermore, a shorter disease-free survival (DFS) was also associated with a high expression of IL-22RA1 (P=0.0102). In the multivariable analysis, IL-22RA1 expression was an independent prognostic predictors regarding OS (P=0.0096, HR=0.48). In the TCGA cohort, IL-22RA1 expression was independent regarding to OS and DFS. CONCLUSION: A high IL-22RA1 gene expression was associated with worse outcome. Furthermore, IL-22RA1 represented an independent predictor regarding OS in our cohort and therefore might be used for risk stratification in patients with MIBC.

Practice Patterns and Pain Outcomes for Targeted Muscle Reinnervation: An Informed Approach to Targeted Muscle Reinnervation Use in the Acute Amputation Setting.

BACKGROUND: Targeted muscle reinnervation (TMR) and regenerative peripheral nerve interface (RPNI) procedures have been shown to improve patient-reported outcomes for the treatment of symptomatic neuromas after amputation; however, the specific indications and comparative outcomes of each are unclear. The primary research questions were what complement of nerves most frequently requires secondary pain intervention after conventional amputation, whether this information can guide the focused application of TMR and RPNI to the primary amputation setting, and how the outcomes compare in both settings. METHODS: We performed a retrospective review of records for patients who had undergone lower-extremity TMR and/or RPNI at our institution. Eighty-seven procedures were performed: 59 for the secondary treatment of symptomatic neuroma pain after amputation and 28 for primary prophylaxis during amputation. We reviewed records for the amputation level, TMR and/or RPNI timing, pain scores, patient-reported resolution of nerve-related symptoms, and complications or revisions. We evaluated the relationship between the amputation level and the frequency with which each transected nerve required neurologic intervention for pain symptoms. RESULTS: The mean pain score decreased after delayed TMR or RPNI procedures from 4.3 points to 1.7 points (p < 0.001), and the mean final pain score (and standard deviation) was 1.0 ± 1.9 points at the time of follow-up for acute procedures. Symptom resolution was achieved in 92% of patients. The sciatic nerve most commonly required intervention for symptomatic neuroma above the knee, and the tibial nerve and common or superficial peroneal nerve were most problematic following transtibial amputation. None of our patients required a revision pain treatment procedure after primary TMR targeting these commonly symptomatic nerves. Failure to address the tibial nerve during a delayed procedure was associated with an increased risk of unsuccessful TMR, resulting in a revision surgical procedure (odds ratio, 26 [95% confidence interval, 1.8 to 368]; p = 0.02). CONCLUSIONS: There is a consistent pattern of symptomatic nerves that require secondary surgical intervention for the management of pain after amputation. TMR and RPNI were translated to the primary amputation setting by using this predictable pattern to devise a surgical strategy that prevents symptomatic neuroma pain. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Locoregional recurrence after cystectomy in muscle invasive bladder cancer: Implications for adjuvant radiotherapy.

PURPOSE: We report the patterns of locoregional recurrence (LRR) in muscle invasive bladder cancer (MIBC), and propose a risk stratification to predict LRR for optimizing the indication for adjuvant radiotherapy. MATERIALS AND METHODS: The study included patients of urothelial MIBC who underwent radical cystectomy with standard perioperative chemotherapy between 2013 and 2019. Recurrences were classified into local and/or cystectomy bed, regional, systemic, or mixed. For risk stratification modelling, T stage (T2, T3, T4), N stage (N0, N1/2, N3) and lymphovascular invasion (LVI positive or negative) were given differential weightage for each patient. The cohort was divided into low risk (LR), intermediate risk (IR) and high risk (HR) groups based on the cumulative score. RESULTS: Of the 317 patients screened, 188 were eligible for the study. Seventy patients (37.2%) received neoadjuvant chemotherapy (NACT) while 128 patients (68.1%) had T3/4 disease and 66 patients (35.1%) had N+ disease. Of the 55 patients (29%) who had a recurrence, 31 (16%) patients had a component of LRR (4% cystectomy bed, 11.5% regional 0.5% locoregional). The median time to LRR was 8.2 (IQR 3.3-18.8) months. The LR, IR and HR groups for LRR based on T, N and LVI had a cumulative incidence of 7.1%, 21.6%, and 35% LRR, respectively. The HR group was defined as T3, N3, LVI positive; T4 N1/2, LVI positive; and T4, N3, any LVI. The odds ratio for LRR was 3.37 (95% CI 1.16-9.73, P = 0.02) and 5.27 (95% CI 1.87-14.84, P = 0.002) for IR and HR respectively, with LR as reference. CONCLUSION: LRR is a significant problem post radical cystectomy with a cumulative incidence of 35% in the HR group. The proposed risk stratification model in our study can guide in tailoring adjuvant radiotherapy in MIBC.

Adipocyte-rich CTNNB1-mutated Intramuscular Gardner Fibroma Progressing to Desmoid Fibromatosis.

Gardner fibroma (GF) is a benign soft-tissue tumor that is associated with Gardner syndrome and can progress to, or co-occur with, desmoid fibromatosis (DF). Herein, we report a unique case of an 11-year-old boy who presented with a rapidly growing soft-tissue mass after biopsy of a stable fat-rich lesion present in the calf muscles since infancy, with Magnetic resonance imaging findings suggesting an intramuscular adipocytic tumor. The resection showed GF and DF. DF arising from a preexisting GF (the so-called "GF-DF sequence") is a well-documented phenomenon. Although immunohistochemistry was negative for nuclear ß-catenin expression, a CTTNB1 S45F mutation, which has been associated with aggressive behavior in DF, was identified in both components using a next-generation sequencing-based molecular assay. This is the first time a mutation in CTNNB1 has been identified in GF and the GF-DF sequence, thus expanding our knowledge of the molecular pathogenesis of the GF-DF sequence and highlighting the role of molecular testing in pediatric soft-tissue tumors. The histologic findings of an adipocyte-rich intramuscular GF also are unique, expanding the morphological spectrum of GF and adding GF to the differential diagnosis of intramuscular lesions with an adipocytic component.

Histiocyte-rich rhabdomyoblastic tumor: a report of two cases and a review of the differential diagnoses.

Histiocyte-rich rhabdomyoblastic tumor is a recently described skeletal muscle neoplasm of uncertain malignant potential, characterized by slow growth, a fibrous capsule containing peripheral lymphoid aggregates, spindle-to-epithelioid cells with a rhabdomyoblastic immunophenotype, and a dense histiocytic infiltrate. It most commonly arises within the muscles of the lower legs and trunk in young-to-middle-aged men, and initial reports suggest indolent behavior. In this paper, we present two additional cases of histiocyte-rich rhabdomyoblastic tumor with similar clinicopathologic features and discuss the differential diagnosis including its overlap with inflammatory leiomyosarcoma.

Oral intramuscular myxoma: case report and brief review of the literature.

OBJECTIVE: Myxomas are rare benign mesenchymal soft tissue tumors that seldom affect the head and neck area. The present report describes a rare case of intramuscular myxoma of the oral cavity and briefly reviews the literature regarding the occurrence of these lesions in the maxillofacial area. STUDY DESIGN: A 59-year-old patient was referred for the development of a slow-growing tumefaction in the right buccal mucosa in the previous 12 months. Magnetic resonance imaging was performed to characterize the lesion's dimensions and its relationship with the surrounding structures, and it revealed intramuscular localization in correspondence with the medial pterygoid muscle. Incisional biopsy was carried out to aid in the final diagnosis. RESULTS: Histology revealed the presence of an intramuscular myxoma, characterized by discontinuous margins and locally infiltrating muscular fibers. A second surgery was performed to totally remove the lesion. Follow-up at 6 months did not show any signs of recurrence. A literature search was performed to retrieve data on the incidence of oral intramuscular myxomas. CONCLUSIONS: To the best of our knowledge, this is the first case of intramuscular myxoma of the medial pterygoid muscle reported in the literature. Although rarely encountered, intramuscular myxomas should be considered when performing differential diagnoses of soft tissue masses at the level of the masticatory muscles.

The discovery of bacterial biofilm in patients with muscle invasive bladder cancer.

The carcinogenic effects of microorganisms have been discovered in multiple cancer types. In urology, the development of squamous cell carcinoma of the bladder due to the parasitic infection with Schistosoma Mansoni is widely accepted. The oncogenic potential of biofilms has been studied in colorectal cancer and experimental studies have shown that bacteria such as Escherichia coli drive the development of colorectal cancer. Notably, Escherichia coli is responsible for 80% of all urinary tract infections. Recent findings suggest an altered urinary microbiome in patients with bladder cancer compared to healthy subjects. In this case series, we demonstrate our findings of biofilm formation in human bladder cancer tissue. Tissue samples from ten patients that underwent routine Transurethral Resection of Bladder Tumor (TURBT) were obtained from the Danish National Biobank. Pathological tissue was examined for presence of bacterial aggregates by Fluorescence in situ Hybridization. In two of ten patients, analysis showed abundant bacterial aggregation on the surface epithelium. Both positive cases had pT2 urothelial bladder cancer. Our findings suggest that biofilm occurs in urothelial cancer tissue indicating an association between biofilm formation and bladder cancer.

Surgical excision of a malignant metastatic melanoma located in a skeletal muscle of the lateral thorax of a horse.

BACKGROUND: A 20-year-old grey Warmblood gelding that had history of dermal melanomatosis. OBJECTIVE: To report surgical treatment of a 6-cm large malignant metastatic melanoma located in the cutaneous trunci muscle of the left lateral thorax of a horse. METHODS: A 20-year-old grey Warmblood gelding was referred for evaluation of a large, rapidly growing, thoracic mass. Clinical examination revealed an ovoid, firm, non-painful, movable mass, approximately 6 cm in diameter, located in the cutaneous trunci muscle of the left lateral thorax. Multiple melanocytic nodules were also found at the perianal region and ventral tail. Rectal examination, ultrasonography and endoscopy of the respiratory tract revealed no melanomas internally. Haematological and biochemical values were within normal limits. Surgical excision of both the thoracic mass and perianal nodules was the treatment of choice. Histopathology of the distant thoracic mass confirmed the diagnosis of malignant metastatic melanoma secondary to the perineal lesions that were confirmed as dermal melanomatosis. RESULTS: The horse recovered uneventfully. Up until 3 years post surgery there was no tumour regrowth at the excision sites. CONCLUSION: Surgical excision of the metastatic melanoma was performed and considered successful, with no gross evidence of tumour reoccurrence. Despite the fact that surgical excision is considered a treatment option regarding equine dermal melanomatosis, there is no previously published study proposing surgical removal of distant malignant metastatic melanocytic masses. This is the first report of a successful surgical excision of a large (6 cm) metastatic melanocytic tumour from the skeletal muscle of the lateral thorax of a horse.

Desmoid tumour of the distal forearm involving the distal radioulnar joint.

A 32-year-old woman presented with progressive pain and swelling of the left wrist for 6 months. Physical examination revealed a firm, tender, oval swelling over the left wrist. X-rays showed a pressure effect on the distal radius and ulna. Magnetic Resonance Imaging (MRI) revealed a well-defined, asymmetrical, dumbbell-shaped soft-tissue lesion involving the interosseous region of the distal forearm and extending until the distal radioulnar joint (DRUJ). Core needle biopsy confirmed the diagnosis of desmoid tumour. Marginal excision of the tumour was done. At the 2-year follow-up, the patient was doing well and had painless and improved left wrist motion. Desmoid tumour involving the DRUJ has not been previously reported. We, through this case, report new observation and discuss the epidemiology, investigation of choice, treatment modalities, and the need for a regular follow-up for appendicular desmoid tumours.

[Abdominal wall repair after total resection of desmoid fibroma in a patient with diabetes mellitus type 1]. / Rekonstruktsiya perednei bryushnoi stenki posle radikal'noi rezektsii desmoidnoi fibromy na fone sakharnogo diabeta 1-go tipa.

Desmoid fibroma (DF) is a rare connective tissue tumor comprising about 0.03-0.13% of all neoplasms. DF has a low potential for malignant transformation, but it is characterized by aggressive course and unfavorable prognosis. The main contingent of patients consists of women of reproductive age. Despite the nearly two-century history of study, there are certain unsolved problems including endocrine problems associated with this disease. We report a 30-year-old female with DF and diabetes mellitus type 1. Total resection of the affected right rectus abdominis muscle was performed in a single block with aponeurotic sheath and peritoneum after normalization of carbohydrate metabolism. Muscular aponeurotic defect 27�10 cm was closed after separation of abdominal wall structures and implantation of polypropylene prosthesis. There are no X-ray and clinical signs of DF recurrence or postoperative hernia after 8 months.