Skeletal Muscle Metastases from Carcinomas: A Review of the Literature.

* Bones are a common site for metastases; however, muscle metastases recently have been more commonly reported, not only as a result of the multidisciplinary approach in the treatment of these patients but also because of more sensitive imaging modalities such as positron emission tomography-computed tomography (PET-CT) that identify these lesions in early stages.* The most common carcinoma is lung carcinoma, with a hematogenous route of spread mainly to the axial region of the body (the psoas muscle, the gluteal muscles, and the paravertebral muscles). * Clinically, skeletal muscle metastases from carcinomas frequently present as painful palpable masses with or without swelling and are commonly found before diagnosis of the primary carcinoma.* Multiple imaging modalities, including radiographs, CT, magnetic resonance imaging (MRI), and PET-CT, have been used for diagnosis and staging, but tissue sampling is needed for a final diagnosis. The most important differential diagnosis of skeletal muscle metastases is with soft-tissue sarcomas.* Treatment is mainly based on chemotherapy and/or radiation; surgery is performed in cases of symptomatic lesions that fail to respond to nonoperative treatment.

Spectrum of Biopsy Proven Extraocular Muscle Tumours of Non-Thyroid Origin.

OBJECTIVE: To describe different types of primary extraocular muscle (EOM) tumours based on the results of imaging studies, peroperative clinical picture and their histopathological diagnosis. STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: Al-Shifa Trust Eye Hospital, Rawalpindi, from July 2001 to January 2017. METHODOLOGY: A retrospective analysis of 640 diagnosed orbital tumours was carried out using non-randomised sampling technique, and the prevalence of primary EOM tumours was determined. Based on the results of imaging studies, the clinical picture observed during surgery (orbitotomy) and the histopathological diagnosis, primary EOM tumours were divided into different types, accordingly. RESULTS: Nineteen (n=19) primary EOM tumours (frequency of 2.96%) had 12 types of histopathological diagnoses, and were categorised into inflammatory tumours (n=8, 42%), vascular tumours (n=4, 21%), lymphoproliferative tumours (n=3, 16%), neurogenic tumours (n=2, 10.5%) and myogenic tumours (n=2, 10.5%). The recti were involved more frequently than obliques (n=15, 78.94% and n=4, 21.06%, respectively). All the patients presented with proptosis of varying degree with some degree of globe rotation and had surgical excision/appropriate management. Visual acuity was not affected in any of the patients. Four (n=4, 21.05%) tumours were malignant (NHL, ASPS, myeloid sarcoma and rhabdomyosarcoma) and these patients underwent chemotherapy and/or radiotherapy. CONCLUSION: Biopsy-proven primary EOM tumours were devisable into five broad categories. Patients with primary EOM tumours presented with proptosis and impaired ocular motiliy. The primary EOM tumours involved both the recti and the obliques and were excised surgically with favourable outcomes in most cases.

Prevalence and Clinical Features of Mazabraud Syndrome: A Multicenter European Study.

BACKGROUND: Mazabraud syndrome is a rare disorder, characterized by the presence of fibrous dysplasia (FD) with associated intramuscular myxomas. Data are scarce on the prevalence, clinical features, and natural history of this disorder and outcomes. In this multicenter study, we evaluated a series of patients from 6 European centers. METHODS: All centers affiliated with the European Musculo-Skeletal Oncology Society (EMSOS) were invited to include data on all patients with Mazabraud syndrome who were seen between 1980 and 2015. The study investigated the prevalence of Mazabraud syndrome, the type, severity, and localization of FD lesions in relation to myxomas, the histopathology of myxomas, and results of GNAS-mutation analysis, when available. RESULTS: Thirty-two patients (22 female) from 6 centers were included. The prevalence of Mazabraud syndrome was 2.2% in the combined cohort of 1,446 patients with FD, and the syndrome was diagnosed at a mean of 10.1 years after diagnosis of FD. The myxomas were predominantly localized in the upper leg. Excision was performed in 20 patients, recurrence occurred in 6 of these patients (30%) at a median of 8.5 years (range, 1.9 to 16.0 years), and revision surgery was necessary in 5 (25%). High cellularity of myxomas was associated with recurrence (p < 0.05). A GNAS mutation was identified in the myxoma tissue of 5 (83%) of 6 patients with GNAS-mutation analysis. CONCLUSIONS: This study is the first, to our knowledge, to provide data on the prevalence of Mazabraud syndrome in a relatively large cohort. Although the outcomes of surgical resection were good, a quarter of the patients required revision surgery despite clear resection margins. High cellularity of myxomas was associated with recurrence. GNAS mutations were identified in 83% (5 of 6), emphasizing the shared origin of FD and myxomas. Our data show that patients with FD who have disproportionate complaints, irrespective of FD type, extent, or severity, should be investigated for the possible presence of myxomas. LEVEL OF EVIDENCE: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.

[ypT0N0 after neoadjuvant chemotherapy and cystectomy for muscle-invasive bladder cancer: Incidence and prognosis. A review from the Bladder group of the French Committee of Oncology]. / Pièce opératoire ypT0N0 après séquence chimiothérapie néo-adjuvante ­ cystectomie pour TVIM : épidémiologie et impact pronostique. Une mise au point du CCAFU Vessie.

INTRODUCTION: Neoadjuvant chemotherapy (NAC) is recommended for localized muscle-invasive bladder cancer when patients are fit for cisplatin-based chemotherapy. A pathological complete response can be observed, corresponding to ypT0N0 stage on the radical cystectomy specimen. This review discusses the incidence, prognosis and potential therapeutic impact of complete response on pathological specimen in NAC treated patients. METHODS: A comprehensive review of the literature was conducted using Medline database, with no time frame. The articles were selected using the following keywords association: "Bladder cancer" (Mesh) AND "Neoadjuvant chemotherapy" (Mesh) AND "pT0" (Mesh). RESULTS: After NAC, ypT0N0 rates vary from 9 to 46% among the series, reported rates that are higher compared to those of pT0 without NAC administration. The incidence depends on the chemotherapy regimen (maximal local effect with cisplatin-based chemotherapy) and the pathological type of the disease (presence of variant histologies). Molecular analyses of bladder cancer could probably help in the near future to identify and predict NAC responders. Pathological complete response is associated with a favorable prognosis in terms of recurrence-free and overall survival. Nevertheless, disease recurrences are still observed in 10-15% of cases, which underlies the importance of local treatment and close follow-up even in these patients. CONCLUSION: ypT0N0 rate is approximately 25% after NAC, that is 4.3 higher than after bladder resection alone. The prognosis is better than that with residual tumor on specimen and is comparable to that of pT0 without NAC administration.

Accurate prediction of progression to muscle-invasive disease in patients with pT1G3 bladder cancer: A clinical decision-making tool.

PURPOSE: To improve current prognostic models for the selection of patients with T1G3 urothelial bladder cancer who are more likely to fail intravesical therapy and progress to muscle-invasive bladder cancer (MIBC). MATERIALS AND METHODS: We performed a retrospective analysis of 1,289 patients with pT1G3 urothelial bladder cancer who were treated with transurethral resection of the bladder (TURB) and adjuvant intravesical bacillus-Calmette-Guérin (BCG). Random-split sample data and competing-risk regression were used to identify the independent impact of lymphovascular invasion (LVI) and variant histology (VH) on progression to MIBC. We developed a nomogram for predicting patient-specific probability of disease progression at 2 and 5 years after TURB. Decision curve analysis (DCA) was performed to evaluate the clinical benefit associated with the use of our nomogram. RESULTS: In the development cohort, within a median follow-up of 51.6 months (IQR: 19.3-92.5), disease progression occurred in 89 patients (13.8%). A total of 84 (13%) patients were found to have VH and 57 (8.8%) with LVI at TURB. Both factors were independently associated with disease progression on multivariable competing-risk analysis (HR: 4.4; 95% CI: 2.8-6.9; P<0.001 and HR: 3.5; 95% CI: 2.1-5.8; P<0.001, respectively). DCA showed superior net benefits for the nomogram within a threshold probability of progression between 5% and 55%. Limitations are inherent to the retrospective design. CONCLUSIONS: We demonstrated the clinical value of the integration of LVI and VH in a prognostic model for the prediction of MIBC. Indeed, our tool provides superior individualized risk estimation of progression facilitating decision-making regarding early RC.

Skeletal muscle metastasis from the most common carcinomas orthopedic surgeons deal with. A systematic review of the literature.

INTRODUCTION: There is scarce information in the literature dealing with the clinical presentation, management and oncologic outcomes of skeletal muscle metastases (SMM). We sought to perform a systematic review of the literature to investigate: (1) tumor characteristics of SMM, (2) therapeutic approach, and (3) oncological outcomes. METHODS: A systematic review of the literature was performed using PubMed and EMBASE search engines. A total of 3231 references were reviewed and 49 studies were included. Demographic data, presentation characteristics, and oncological outcomes were recorded. Statistical analysis was performed using SPSS 22.0 software (IBM; Armonk, New York) and Comprehensive Meta-Analysis software version 3 (Biostat, Inc.), with p < 0.05 as statistically significant. RESULTS: A total of 231 patients were included. These tumors presented more commonly on males 58.4% (135/231), with a mean age of 60.08 ± 10.6 years, and in the axial area 39.6% (88/222). The most common carcinoma type was lung 41.1% (95/231). Resection of a single metastases did not change survival significantly (p = 0.992). LRR was higher within the group of patients that underwent WLE compared with non-WLE [31.3% (23/74) vs. 8.7% (2/23), p ≤ 0.001]. Kaplan-Meier survival analysis for the entire cohort showed an estimate of 15.3 months [95% confidence interval (CI) 11.6-19; standard error (SE) 0.432], with lung carcinoma carrying the worst prognosis 6.7 months (95% CI 5.4-8.07; SE 0.68). Patients with a single SMM showed a worse estimate mean survival time compared to patients with multiple metastases limited to muscles [8.6 months (95% CI 4.7-12.5; SE 2.0) vs 25.4 months (95% CI 19.8-31.05; SE 2.8; p ≤ 0.001)]. CONCLUSIONS: Overall survival is poor and is driven mainly by the type of carcinoma. An Increased LRR might be present due to the systemic nature of the condition, and degree of control of the primary carcinoma.

Neoadjuvant chemotherapy prior to radical cystectomy for muscle-invasive bladder cancer with variant histology.

BACKGROUND: Neoadjuvant chemotherapy in pure urothelial bladder cancer provides a significant survival benefit. However, to the authors' knowledge, it is unknown whether this benefit persists in histological variants. The objective of the current study was to assess the effect of neoadjuvant chemotherapy on the probability of non-organ-confined disease and overall survival after radical cystectomy (RC) in patients with histological variants. METHODS: Querying the National Cancer Data Base, the authors identified 2018 patients with histological variants who were undergoing RC for bladder cancer between 2003 and 2012. Variants were categorized as micropapillary or sarcomatoid differentiation, squamous cell carcinoma, adenocarcinoma, neuroendocrine tumors, and other histology. Logistic regression models estimated the odds of non-organ-confined disease at the time of RC for each histological variant, stratified by the receipt of neoadjuvant chemotherapy. Cox regression models were used to examine the effect of neoadjuvant chemotherapy on overall mortality in each variant subgroup. RESULTS: Patients with neuroendocrine tumors (odds ratio [OR], 0.16; 95% confidence interval [95% CI], 0.08-0.32 [P<.001]), micropapillary differentiation (OR, 0.30; 95% CI, 0.10-0.95 [P=.041]), sarcomatoid urothelial carcinoma (OR, 0.40; 95% CI, 0.17-0.94 [P=.035]), and adenocarcinoma (OR, 0.24; 95% CI, 0.06-0.91 [P=.035]) were less likely to harbor non-organ-confined disease at the time of RC when treated with neoadjuvant chemotherapy. An overall survival benefit for neoadjuvant chemotherapy was only found in patients with neuroendocrine tumors (hazard ratio, 0.49; 95% CI, 0.33-0.74 [P=.001]). CONCLUSIONS: Patients with neuroendocrine tumors benefit from neoadjuvant chemotherapy, as evidenced by better overall survival and lower rates of non-organ-confined disease at the time of RC. For tumors with micropapillary differentiation, sarcomatoid differentiation, or adenocarcinoma, neoadjuvant chemotherapy decreased the frequency of non-organ-confined disease at the time of RC. However, this favorable effect did not translate into a statistically significant overall survival benefit for these patients, potentially due to the aggressive tumor biology. Cancer 2017;123:4346-55. © 2017 American Cancer Society.

Treatment of Muscle-Invasive Bladder Cancer in Older Patients.

Treatment of muscle-invasive bladder cancer in older patients is challenging. Definitive therapy of localized disease requires either surgery or radiation therapy, ideally combined with systemic chemotherapy. However, current population data suggest that less than half of patients older than age 70 are offered such treatments. We will review tools available to assess the fitness of older patients for surgery, alternatives, and tips for perioperative patient treatment.

Primary synovial sarcoma of the abdominal wall: a case report and literature review.

Synovial sarcoma (SS) is the fourth most common type of soft tissue sarcoma, following malignant fibrous histiocytoma, liposarcoma, and rhabdomyosarcoma. It usually occurs in the extremities near the large joints of middle-aged patients. We describe a case of synovial sarcoma of the anterior abdominal wall (SSAW) in an adolescent girl and undertake a review of the literature.

Intramuscular capillary-type hemangioma: radiologic-pathologic correlation.

BACKGROUND: Infantile hemangiomas demonstrate a pattern of proliferative growth in infancy followed by a slow phase of involution. In contrast a rare type of vascular tumor, intramuscular capillary-type hemangioma, usually presents beyond the period of infancy with nonspecific symptoms and no evidence of involution. OBJECTIVE: The purpose of this study was to characterize the clinical, imaging, histopathological characteristics and management of intramuscular capillary-type hemangioma. MATERIALS AND METHODS: We performed a retrospective review of a 20-year period to identify children diagnosed with intramuscular capillary-type hemangioma. Patient demographics, imaging and histopathological findings were recorded. RESULTS: We included 18 children (10 boys, 8 girls) with histologically proven intramuscular capillary-type hemangioma - and adequate imaging. The mean age at presentation was 8.1 years (range 1 day to 19 years). Twelve lesions involved muscles of the extremities, 4 were located in the trunk and 2 were in the head and neck. MRI had been performed in all children and demonstrated a soft-tissue mass with flow voids, consistent with fast flow. The lesion was well-circumscribed in 16 children and intralesional fat was seen in 14. Doppler US demonstrated a heterogeneous lesion, predominantly isoechoic to surrounding muscle, with enlarged arterial feeders. Enlarged feeding arteries, inhomogeneous blush and lack of arteriovenous shunting were noted on angiography (n = 5). The most common histopathological findings were lobules of capillaries with plump endothelium and at least some adipose tissue. The lesions were excised in six children. Two children were lost to follow-up. In the remaining 10, follow-up MRI studies ranging from 3 months to 10 years showed that the lesion enlarged in proportion to the child (n = 7), demonstrated slow growth (n = 2) or remained stable (n = 1). There was no change in imaging characteristics on follow-up. CONCLUSION: Intramuscular capillary-type hemangioma is a rare benign vascular tumor of skeletal muscle. The most typical imaging features show a heterogeneous intramuscular mass with fast flow, and intralesional fat. Although the lesion is relatively stable in appearance over time, imaging does not obviate the need for a biopsy to rule out sarcoma. The diagnosis can usually be established by typical findings on histopathology.

Musculo-skeletal tumors incidence and surgical treatment - A single center 5-year retrospective.

OBJECTIVE: Muscle-skeletal tumors represent a challenging pathology for orthopedic surgeons worldwide. The extremely invasive character, the local destruction, the high recurrence rate, the high incidence in young patients and the unfavorable prognosis are all very well known. For these patients it is very important to produce an accelerated functional, social and psychological postoperative rehabilitation. We studied 121 cases of muscle-skeletal tumors which were treated in our hospital over a 5 years period. PATIENTS AND METHODS: We noticed a high prevalence in males and mainly between the 2nd-3rd and 5th-7th decades of their life. At our observation, most patients were suffering in advanced stages of malignant lesions. RESULTS: We try to manage amputations below 5%, with a significant reduction in introducing reconstructive surgical methods as a choice of treatment (10 prostheses and 12 cases of filling with acrylic cement or bone substituent increased with internal fixation). In this way we could observe an unusual ratio between benign and malignant tumors, probably caused by the patient's lack of concern for minimal symptoms. The rural citizen's addressability towards the medical system is alarmingly low, due to the big gap existing from diagnosis to treatment. CONCLUSIONS: There is still an hard effort to develop better reconstructive techniques for the treatment of muscle-skeletal tumors and more studies must be made in order to achieve this goal.

[Muscle invasive bladder cancer after radical cystectomy. Prognostic factors of adjuvant chemotherapy]. / Muskelinvasives Harnblasenkarzinom nach radikaler Zystektomie. Prognosefaktoren der adjuvanten Chemotherapie.

BACKGROUND: The indications to administer adjuvant systemic chemotherapy to patients with urothelial carcinoma of the bladder who underwent radical cystectomy is discussed controversially and all international guidelines are very restrictive with regard to its recommendation for routine daily practice due to the low scientific evidence concerning its therapeutic benefit. Currently, adjuvant systemic chemotherapy should be preferably administered within clinical trials. In the daily routine adjuvant treatment might be given in patients with lymph node positive disease. Clinical, pathohistological and molecular biomarkers which might be associated with a positive or negative treatment response have been evaluated sparsely in the past. RESULTS: The presence of hemangiosis/lymphangiosis carcinomatosa and/or extranodal expansion in patients with lymph node metastases appears to be associated with a poor outcome. The markers ERCC-1, XAF and anti-apoptotic proteins of the Bcl-2 family seem to represent the most promising biomarkers associated with response to adjuvant cisplatin-based chemotherapy.

Incidence and risk factors for pulmonary embolism after primary musculoskeletal tumor surgery.

BACKGROUND: Limited information is available regarding the incidence, risk factors, and optimal prophylaxis in orthopaedic oncology patients, although malignancy and major orthopaedic surgery are associated with an increased pulmonary embolism (PE) risk. QUESTIONS/PURPOSES: We aimed to investigate the incidence of PE after musculoskeletal tumor surgery in Japanese patients and analyze the potential risk factors for PE. METHODS: We retrospectively identified 3750 patients (1981 males, 1769 females) who underwent musculoskeletal tumor surgery during 2007 to 2010 using the Japanese Diagnostic Procedure Combination administrative database. Data collected included sex, age, primary diagnosis, type of surgery, duration of anesthesia, and comorbidities that may affect PE incidence. Univariate logistic regression analyses were performed to examine the relationship of each factor with PE occurrence. RESULTS: We identified 10 patients with PE during the survey period. A primary malignant bone tumor was associated with a significantly higher risk of PE than a primary malignant soft tissue tumor (odds ratio [OR], 5.58; 95% CI, 1.39-22.42). Bone tumor resection (OR, 7.94; 95% CI, 1.77-35.59) and prosthetic reconstruction (OR, 9.15; 95% CI, 1.52-55.07) were associated with a significantly higher risk of PE than soft tissue tumor resection. CONCLUSIONS: Malignant bone tumors and bone tumor resections have a higher risk of PE than malignant soft tissue neoplasms and soft tissue resections. Both populations might require PE prophylaxis as it is likely that the risk is greater than with other major orthopaedic surgery, but data accumulation should continue, and further investigation should be done to clarify details of the incidence, risk factors, and optimal prophylaxis for PE. LEVEL OF EVIDENCE: Level II, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.

Bladder cancer.

Bladder cancer is the fourth most common cancer in the United States. Urothelial carcinoma that originates from the urinary bladder is the most common subtype. These NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) provide recommendations on the diagnosis and management of non-muscle-invasive and muscle-invasive urothelial carcinoma of the bladder. This version of the guidelines provides extensive reorganization and updates on the principles of chemotherapy management.

Relevance of compartmental anatomic guidelines for biopsy of musculoskeletal tumors: retrospective review of 363 biopsies over a 6-year period.

PURPOSE: To retrospectively assess percutaneous core needle biopsies performed by radiologists and the association with tumor seeding along the biopsy tract when anatomic compartment guidelines are not consistently observed. MATERIALS AND METHODS: Retrospective data from computerized patient records and digital images from 363 consecutive computed tomography-guided biopsies of the lower extremity (thigh and leg) performed by radiologists at a single institution from August 2002 to August 2008 were analyzed for breaches of biopsy guidelines. RESULTS: Of the 363 biopsies, 243 (67%) were of soft tissue lesions and 120 (33%) were of bony lesions. There were 188 (52%) malignant and 175 (48%) benign lesions. The following biopsy breaches were observed: 13 (3.6%) of anatomic compartment, 42 (11.6%) of "vital structures," and 82 (68.3%) of needle path for bony tumors. Vital structures as defined by the literature included, but were not limited to, the following: knee joint capsule, greater trochanteric bursa, rectus femoris and vastus intermedius muscles, tibial tubercle, peroneus brevis and peroneus longus distal tendons, and neurovascular bundles. No cases of tumor recurrences could be attributed to needle seeding along a biopsy tract for any of these biopsy guideline breaches. CONCLUSIONS: The concern for needle tract seeding with musculoskeletal tumors is more widespread than the evidence supporting it as a significant or frequent complication. In this study, breaching anatomic compartment, vital structures (other than neurovascular structures), and suggested exact needle path guidelines were not associated with needle tract seeding in the lower extremity.

Skeletal muscle metastases: a three-part study of a not-so-rare entity.

OBJECTIVE: Our purposes were to explore the epidemiology of metastases to skeletal muscle and their detection on fused positron emission tomography and computed tomography. MATERIALS AND METHODS: We evaluated the epidemiology of skeletal muscle metastases in the literature and among cases from our hospital and studied the prevalence and appearance of skeletal muscle metastases among 433 patients undergoing fused positron emission tomography and computed tomography for non-small-cell lung cancer. RESULTS: We found 264 cases of skeletal muscle metastases in 151 articles. Mean age was 57.8 years with 67% men. At our hospital we studied 70 cases. Mean patient age was 55.7 years with 63% men. The most common source was lung cancer, and the most common site of involvement was the muscles of the trunk. Among our lung cancer patients undergoing fused positron emission tomography and computed tomography, we found 7 (1.6%) with skeletal muscle metastases. In only one of these seven patients was the metastasis first discovered by another imaging modality. In one patient discovery of the metastasis at fused positron emission tomography and computed tomography changed management. CONCLUSION: Skeletal muscle metastases are not rare. They may be more apparent at fused positron emission tomography and computed tomography than at other staging examinations, particularly contrast-enhanced CT scanning. Radiologists need to be alert to their presence when interpreting staging examinations in cancer patients.

Metastasis to deep obturator and para-aortic lymph nodes in 649 patients with cervical carcinoma.

OBJECTIVE: The extent of retroperitoneal lymph node dissection for early stage cervical cancer remains debatable. Our study aimed to investigate the incidence of metastases in deep obturator lymph nodes (DOLNs) and para-aortic lymph nodes (PALNs) and its association with clinicopathological factors in cervical carcinoma. METHOD: Six hundred and forty-nine patients with stage IB to IIB cervical cancer underwent radical hysterectomy, pelvic lymphadenectomy and/or para-aortic lymphadenectomy. The incidence and distribution of metastases in retroperitoneal lymph nodes and the associated factors were analyzed. RESULTS: Metastases in pelvic lymph nodes (PLNs) were found in 175 patients. Of the 175 cases with positive PLNs, 15 had DOLN metastases. Only two cases without PLN metastases had positive DOLNs. There was no significant correlation between DOLN metastasis and tumor histology, differentiation or capillary lymphatic space involvement. PALN metastasis occurred in 19 of 137 patients with cervical lesions larger than 2 cm in diameter. Eighteen of the 19 patients also had PLN involvement. Logistic regression analysis showed that PLN metastasis and larger tumors were independent risk factors for metastasis to PALNs. CONCLUSION: DOLN metastasis is not common and may be secondary to metastasis to other PLNs. Routine removal of DOLNs might be unnecessary when PLNs are negative. Para-aortic sampling or lymphadenectomy should be considered when the PLNs are positive or/and the cervical lesion is larger than 2 cm.