Soft Tissue Masses: Evaluation and Treatment.

Soft tissue masses are a common presentation in family physician offices. Although most lesions, including lipomas, fibromas, and epidermal and ganglion cysts, are benign, rare lesions such as soft tissue sarcomas may have serious consequences. Masses that are deep to the fascia, are 5 cm in diameter or larger, grow rapidly, or present suddenly without explanation should prompt further workup. Imaging for concerning lesions may include ultrasonography, radiography, and sometimes magnetic resonance imaging with contrast. Ultrasonography can be used to assess size, depth, solid or cystic nature, and associated vasculature. Magnetic resonance imaging with contrast provides spatial orientation and delineation of soft tissue sarcomas from surrounding tissues such as muscles. Although less commonly used, computed tomography is an alternative in the initial evaluation of concerning masses and can assist with staging of retroperitoneal and visceral sarcomas. Incisional biopsy of a concerning soft tissue mass can also be useful for establishing a diagnosis. Lipomas and epidermal cysts may be excised if they are painful or if there is concern for malignancy. Because of the high mortality rate of soft tissue sarcomas, evaluation of high-risk masses with magnetic resonance imaging with contrast should be expedited with a referral to orthopedic oncology.

Imaging of spindle cell lipoma.

AIM: To review the evaluation, diagnosis, and treatment of spindle cell lipoma (SCL) with emphasis on the location of these tumours and the spectrum of magnetic resonance imaging (MRI) and computed tomography (CT) appearances. MATERIALS AND METHODS: The MRI and CT findings of 27 histopathologically proven SCLs were evaluated retrospectively. Imaging features evaluated included margins, percentage visible fat, MRI signal characteristics, oedema, and contrast enhancement patterns. RESULTS: Patient ages ranged from 18 to 80 years with an average age of 56.5 years. Men were affected twice as frequently as women (M=18, F=9). SCLs ranged in size from 2 to 10 cm, with an average greatest dimension of 5.5 cm. Five lesions (19%) contained no visible fat on CT or MRI, and the leading differential diagnosis of high-grade soft-tissue sarcoma diagnosis was suggested by referring surgeons. Five lesions (19%) had <50% fatty areas, nine lesions (52%) demonstrated >50% but <90% fat at MRI or CT. Only three of 25 lesions (12%) had an appearance of a typical lipoma on unenhanced MRI sequences. All SCLs that were imaged with contrast medium (n = 18) demonstrated some degree of enhancement, with eight (44%) showing marked enhancement, four (22%) showing moderate, and six (33%) minimal enhancement. CONCLUSION: SCLs have considerably variable imaging appearances and may have minimal or no visible fat at MRI or CT. Imaging features may make it difficult to distinguish this benign tumour from a potentially higher-grade malignant tumour.

Tumors of the Ischiorectal Fossa: A Single-Institution Experience.

BACKGROUND: Ischiorectal fossa tumors are rare. OBJECTIVE: This study reviews a single institution's series of ischiorectal tumors with comparison against presacral tumors and assesses the utility of preoperative biopsy and angioembolization. DESIGN: This is a retrospective study. SETTINGS: This study was conducted at a quaternary referral center. PATIENTS: All patients with ischiorectal tumor treated between February 1995 and April 2017 were retrospectively reviewed. Tumors extending secondarily into the ischiorectal fossa and inflammatory pathologies were excluded. INTERVENTIONS: Preoperative biopsy, neoadjuvant therapy, angioembolization, and surgical excision of these tumors were reviewed. MAIN OUTCOME MEASURES: Demographic, perioperative, pathological, and oncologic outcomes were evaluated. RESULTS: Twenty-four patients (15 female; median age 54) were identified. Two-thirds were symptomatic. Forty-six percent had a palpable mass. All patients had CT and/or MRI. Fifty percent had a preoperative biopsy, of which 83% were diagnostic, and management was altered in 50%. All patients underwent surgical excision. Fifty-five percent had local excision, 38% had radical pelvic excision, and 8% had total mesorectal excision. Two patients had preoperative angioembolization. Both had successful R0 local excision. Morbidity occurred in 25%, with 1 major complication. There was no 30-day mortality. Histopathology demonstrated 17 soft tissue tumors (3 malignant), 2 GI stromal tumors, 1 neuroendocrine tumor, 1 Merkel cell carcinoma, 1 basaloid carcinoma, 1 epidermal cyst, and 1 lipoma. R0 resection was achieved in 75%. All patients were alive after a median follow-up of 33 months. Four patients developed recurrence at a median 10 months postoperatively. All recurrences were malignant, and 75% had had a R1 resection. LIMITATIONS: This study is limited by its small numbers. The quaternary institution source may introduce bias. CONCLUSIONS: Ischiorectal fossa tumors are heterogeneous and more likely to be malignant than presacral tumors. Biopsy can be useful if a malignant diagnosis is suspected and changes management in 50% of cases. Preoperative embolization may be useful for large vascular tumors. R0 resection is important to minimize recurrence. See Video Abstract at http://links.lww.com/DCR/A779.

Cystic bone tumors of the foot and ankle.

Bone tumors are relatively rare in the foot and ankle region. Many of them present as cystic lesions on plain films. Due to the relative rarity of these lesions and the complex anatomy of the foot and ankle region, identification of such lesions is often delayed or they get misdiagnosed and mismanaged. This review discusses the most common cystic tumors of the foot and ankle including their radiographic features and principles of management.

Conservative and surgical treatment of pediatric asymptomatic lumbosacral lipoma: a meta-analysis.

The aim of this study is to compare the outcomes of surgical and conservative treatments of pediatric asymptomatic lumbosacral lipomas, and to address whether the patients can benefit from prophylactic surgeries. The literature reports of surgical and conservative treatments of child asymptomatic lumbosacral lipomas were reviewed and collected, and a meta-analysis of the reports regarding the incidence of sphincter and lower limb dysfunctions was performed. A total of five literatures were collected, containing a total of 403 patients, among which 124 patients received conservative treatments with 32 (25.81%) cases developing neurological dysfunctions during follow-up, and 279 received prophylactic surgical treatments with 30 (10.75%) patients developing neurological dysfunctions in follow-up, the difference being statistically significant (P ≤ 0.05). For pediatric asymptomatic lumbosacral lipomas of the three major subtypes, the limited source of literature so far suggests that prophylactic surgery is superior to conservative strategy in preventing the patients from neurological deterioration. Larger patient cohorts, randomized studies, and longer length of follow-ups are needed for further corroboration.

Phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha (PIK3CA)-related overgrowth spectrum: A brief report.

A patient with extensive multisystem overgrowth caused by a somatic gain of function PIK3CA-mutation is described. This case is an example of the clinical diversity of the PIK3CA-Related Overgrowth Spectrum (PROS) as the patient had overlapping features of Congenital Lipomatous Overgrowth Vascular malformations Epidermal nevi and Skeletal abnormalities (CLOVES) syndrome and Megalencephaly-Capillary malformation Polymicrogyria (MCAP) syndrome and underlines the utility of this umbrella term.

Lipomatous tumors of the breast: A contemporary review.

Breast tumors with lipomatous or liposarcomatous components are infrequently encountered, but can be a source of diagnostic difficulty if the context of the fatty differentiation is not recognized. Among the true adipocytic tumors, lipoma is the most common lipomatous tumor arising in the breast. Several mammary spindle cell tumors may show adipocytic differentiation, including fibroepithelial tumors and myofibroblastoma. Liposarcomatous components most often arise in malignant phyllodes tumors, as opposed to primary liposarcomas of the breast which are believed to be uncommon. This article will review the spectrum fat-containing tumors of the breast with an emphasis on differential diagnosis and insights from recent molecular studies.

An intradural, subpial lipoma.

Introduction - patients: Intradural lipomas are rare congenital tumors. A case of intradural lipoma in the absence of any congenital spinal anomalies is reported. Patient presented with spinal cord compression syndrome. Methods - results - conclusions: Treatment of this disorder is still controversial.

The natural history and management of patients with congenital deficits associated with lumbosacral lipomas.

INTRODUCTION: Many patients with lumbosacral lipoma are asymptomatic; however, a significant proportion will have neurological deficits present at birth. Implication of these deficits with respect to natural history and management are not well understood. METHODS: A retrospective review of all infants with lumbosacral lipoma seen at BCCH between 1997 and 2013 was carried out. The study population was stratified on the presence of a congenital, non-progressive deficit and subdivided on treatment approach. The subsequent developments of deficits resulting in untethering procedures were recorded. RESULTS: Of the 44 infants in this study, 24 patients had no neurologic deficit while 20 patients had a fixed, non-progressive deficit evident at birth. Ten of 24 patients without a neurological deficit at birth underwent a prophylactic untethering with 3 eventually requiring repeat untethering after, on average, 62.7 months. Eleven of 14 asymptomatic, monitored patients required untethering for clinical deterioration. Two required a second untethering procedure after 48.7 months. Ten of 20 infants with congenital deficits present at birth underwent prophylactic untethering, and 4 required further surgery after 124 months. Ten patients underwent observation with 8 eventually requiring surgery. Two required repeat untethered after 154 months. The complication rates and operative burden for patients are similar whether prophylactic or delayed surgery is performed. CONCLUSION: The presence of congenital neurologic deficit does not affect the likelihood of deterioration in patients managed expectantly; prophylactic detethering of these patients did not prevent delayed neurologic deterioration. Comparing the need for repeat surgery in prophylactically untethered patients with initial untethering of patients operated upon at the time of deterioration, prophylactic untethering may confer a benefit with respect to subsequent symptomatic tethering if complication rates are low. However, in a setting with multidisciplinary follow-up, a period of observation for patients and intervention when patients become symptomatic is an acceptable approach for patients with or without congenital deficits.

[A clinical observation of cool-tip radiofrequency ablation assisted enucleation for giant renal angiomyolipoma].

OBJECTIVE: To initially explore the clinical effect of cool-tip radiofrequency ablation combined with enucleation for the giant hamartoma of kidney with narrow base and export-oriented way of growth. METHODS: The clinical date of 15 patients including 6 male and 9 female with special hamartoma of kidney underwent cool-tip radiofrequency ablation assisted enucleation from July 2011 to October 2014 were reviewed.The median age was 49 years (ranging from 35 to 71 years). There were 6 cases with left renal tumor, 8 cases with right renal tumor and 1 case with solitary kidney tumor.All patients were confirmed by B ultrasound or CT scan, the mean diameter of hamartoma of kidney was 9.7 cm(8.5-12.7)cm, all tumors were located distant from the collecting system and presents with a special way of growth.The preoperative hemoglobin was (129±18)g/L, SCr was (92±41)µmol/L, glomerular filtration rate (GFR) was (32±12)ml·min(-1)·1.73 m(-2). RESULTS: Cool-tip radiofrequency ablation assisted enucleation was technically successful in all patients.The mean operative duration was (115±31)minutes, and the average intraoperative bleeding was (72±21)ml with no blooding transfusion.The postoperative hospital stay was(7±2)days, and the postoperative hemoglobin was(129±18)g/L, SCr (92±41)µmol/L, GFR(30±15)ml·min(-1)·1.73 m(-2). No statistic change of hemoglobin and SCr or glomerular filtration rate after operations(all P>0.05). Postoperative pathology showed that all cases were hamartoma of kidney.During a mean follow-up period of 19.5 months, none of them had local tumor recurrence or chronic renal insufficiency. CONCLUSIONS: Cool-tip radiofrequency ablation assisted enucleation is both safe and effective in the treatment of huge hamartoma of kidney with a narrow base and export-oriented way of growth. The short-term follow-up shows a satisfactory therapeutic effect.

Clinical evaluation and management of benign soft tissue tumors of the extremities.

Benign lesions comprise a majority of soft tissue tumors. It has been estimated that their incidence outnumbers that of malignant tumors by a factor of at least 100 [1]. While history and physical examination can start the diagnostic process, imaging including the use of magnetic resonance imaging can be more helpful. Biopsy of these tumors is sometimes necessary and can be performed in a number of ways, often in conjunction with definitive treatment. Specific diagnostic and treatment strategies for a number of the more commonly encountered benign soft tissue tumors including lipomas, pigmented villonodular synovitis and hemangiomas are reviewed. An algorithm for the management of benign soft tissue tumors is discussed.

Lipofibromatous hamartoma of the median nerve: a comprehensive review and systematic approach to evaluation, diagnosis, and treatment.

Many modalities exist for diagnosing and treating lipofibromatous hamartoma (LFH), with no clear consensus. This is the first comprehensive study to review the existing literature on LFH of the median nerve and to suggest a systematic approach to its diagnosis and treatment. An electronic and manual search was conducted on Medline, Embase, Google Scholar, Current Contents, and Science Citation Index for original and review articles in English or French, from 1946 to November 2012. After 2 levels of screening, 106 references containing case reports were retained. Data extraction included patient demographics, clinical information, diagnostic modalities, treatment, and follow-up. A total of 180 cases were reported in the literature. One third of patients had associated macrodactyly (32%). Gender distribution is equal in LFH with or without macrodactyly, with most patients (71%) presenting before age 30 years. The main presenting symptom is an enlargement (88%) over the volar forearm, wrist, or hand, with or without digital hypertrophy, followed by paresthesia (39%). A soft, mobile, nontender, nonfluctuant mass with variable degree of compressive median neuropathy is found on physical examination. Biopsy, which reveals abundant mature fat cells and fibrous connective tissue infiltrating between nerve fascicles and the space between the epineurium and the perineurium, is not necessary because the pathognomonic features of the mass on magnetic resonance imaging offer an accurate diagnosis. Treatment of nerve compression symptoms and macrodactyly should be addressed separately. Carpal tunnel release is the mainstay of treatment for neuropathy, and ray or digital amputation, wedge osteotomy, middle phalangectomy with arthroplasty, and epiphysiodesis are suggested options in the management of macrodactyly. Based on our review of the literature, we propose an algorithm for the diagnosis and treatment of LFH of the median nerve with or without macrodactyly.

CLOVES syndrome.

A cohort of patients with overgrowth syndromes has been identified with congenital lipomatous overgrowth, dysregulated fat deposits, and mixed vascular malformations. The acronym CLOVES was given on a heuristic basis to stand for congenital lipomatous overgrowth (CLO), vascular malformation (V), epidermal nevi (E), and scoliosis and spinal deformities (S). These patients have upper limb anomalies with variable phenotypes. Although hand anomalies alone cannot make the diagnosis, the foot, truncal, cutaneous and spinal anomalies are particularly diagnostic. CLOVES syndrome has emerged as a distinct clinical entity diagnosed by clinical and radiographic examinations. The overgrowth pattern is now easily distinguished from other overgrowth syndromes.

[Rare solid pancreatic tumors]. / Rzadko wystepujace lite nowotwory trzustki.

The most common tumor of the pancreas is cancer, which constitutes 85% of all pancreatic neoplasms. Cystic pancreatic tumors comprise 10% of malignancies. No more than 5% of pancreatic tumors are rare solid tumors as: neuroendocrine tumors, gastrointestinal stromal tumors, solid pseudopapillary tumors, pecomas, lymphomas, granulocytic sarcomas, schwannomas, lipomas, liposarcomas and metastases to pancreas. Nowadays, these tumors are diagnosed more commonly due to the developement and accessibility of the diagnostic imaging techniques. Moreover, the treatment and management of rare solid pancreatic tumors often differs from the management in pancreatic cancer what makes the differential diagnosis difficult and responsible challenge. The main purpose of this article is to present an actual data of epidemiology, clinical presentation, management and treatment of rare solid pancreatic tumors according to recent literature and self experience.

[Endobronchial lipoma: report of 2 cases and review of the Chinese literature].

OBJECTIVE: To describe the epidemiology of endobronchial lipoma and to propose appropriate diagnostic and therapeutic policies for this tumor. METHODS: Two cases of endobronchial lipoma were presented and 38 cases reported in literatures published in mainland China were reviewed. RESULTS: Thirty-one male and 9 female patients were included, with a mean age of (53 ± 11) years. The overwhelming majority of the tumors (n = 37) were found in the trachea, left or right main bronchi and lobular bronchi. The main symptoms included cough, dyspnea, fever and hemoptysis. Chest CT showed fat or soft tissue density endobronchial masses in 75% (21/28) cases. Eight patients (22.2%) were diagnosed by histological study of the transbronchial biopsy specimen. Thirty-two patients underwent surgical resection. Bronchoscopic resection was carried out in 7 cases. CONCLUSION: CT and bronchoscope are highly useful diagnostic tools for endobronchial lipoma. Bronchoscopic resection should be considered as the first choice of treatment for endobronchial lipoma.

Therapeutic potential of small extracellular vesicles derived from lipoma tissue in adipose tissue regeneration-an in vitro and in vivo study.

OBJECTIVE: To explore the adipogenic effects of the small extracellular vesicles derived from the lipoma tissues (sEV-LT), and to find a new cell-free therapeutic approach for adipose tissue regeneration. METHODS: Adipose tissue-derived stem cells (ADSCs) and small extracellular vesicles derived from the adipose tissues (sEV-AT) were isolated from human adipose tissue, while sEV-LT were isolated from human lipomatous tissue. ADSCs were characterized by using flow cytometric analysis and adipogenic and osteogenic differentiation assays. sEV was identified by electron microscopy, nanoparticle tracking, and western blotting. ADSCs were treated with sEV-LT and sEV-AT, respectively. Fluorescence confocal microscopy was used to investigate whether sEV-LT and sEV-AT could be taken by ADSCs. The proliferation and migration abilities and adipogenic differentiation assay of ADSCs were evaluated by CCK-8 assays, scratch test, and oil red O staining test, and the expression levels of adipogenic-related genes C/EBP-δ, PPARγ2, and Adiponectin in ADSCs were assessed by real-time quantitative PCR (RT-PCR). The sEV-LT and sEV-AT transplantation tubes were implanted subcutaneously in SD rats, and the neotissues were qualitatively and histologically evaluated at 2, 4, 8, and 12 weeks after transplantation. Hematoxylin and eosin (H&E) staining was subsequently used to observe and compare the adipogenesis and angiogenesis in neotissues, while immunohistochemistry was used to examine the expression and the distribution of C/EBP-α, PPARγ, Adiponectin, and CD31 at the 4th week. RESULTS: The in vitro experiments showed that both sEV-LT and sEV-AT could be taken up by ADSCs via endocytosis. The scratch experiment and CCK-8 experiment showed that the migration area and proliferation number of ADSCs in sEV-LT group and sEV-AT group were significantly higher than those in the non-sEV group (p < 0.05). Compared with sEV-AT group, sEV-LT group had larger migration area and proliferation number of ADSCs (p < 0.05). Oil red O staining and RT-PCR experiments showed that, compared with the non-sEVs group, the lipid droplets and the mRNA expression levels of adipogenesis-related genes PPARγ2 and Adiponectin of ADSCs in sEV-LT group and sEV-AT group were significantly upregulated (p < 0.05); however, there was no statistical significance in the expression level of C/EBP-δ (p > 0.05). In addition, no significant difference in the amount of lipid droplets and adipogenesis-related genes between the sEV-LT groups and sEV-AT was seen (p > 0.05). At 2, 4, 8, and 12 weeks, the adipocyte area and the number of capillaries in neotissues in the sEV-LT groups and sEV-AT groups were significantly increased compared with the Matrigel group (p < 0.05); however, there was no dramatic difference between sEV-LT groups and sEV-AT groups (p > 0.05). At the 4th week, neotissues in the sEV-LT groups and sEV-AT groups all showed upregulated expression of C/EBP-α, PPARγ, Adiponectin, and CD31 protein, while neotissues in the Matrigel group only showed positive expression of CD31 protein. CONCLUSIONS: This study demonstrated that sEV-LT exerted promotion effects on adipose tissue regeneration by accelerating the proliferation, migration, and adipogenic differentiation of ADSCs in vitro and recruiting adipocytes and promoting angiogenesis in vivo. The sEV-LT could serve as an alternative cell-free therapeutic strategy for generating adipose tissue, thus providing a promising application prospect in tissue engineering.

Diagnostic and management challenges for MDM2-, CDK4-negative fatty tumors of the retroperitoneum.

BACKGROUND: Neoplasms of the retroperitoneum that contain a major fat component may represent either benign entities, such as lipomas or angiomyolipomas, or malignancy such as liposarcoma. Distinguishing these diagnoses has important implications for management. While liposarcomas often stain positively for MDM2 and CDK4 proteins, absence of these markers can lead to diagnostic and management challenges. METHODS: We examined three cases in our institution of fat-containing masses of the retroperitoneum that lacked MDM2 and CDK4 markers to highlight the challenges in diagnosing and managing these cases. A thorough review of the literature examining radiologic and histologic features that can be used to determine that diagnosis was conducted and summarized. RESULTS: The three cases we present represent the three main diagnostic entities that can be found in among fatty tumors of the retroperitoneum: lipoma, angiomyolipoma, and liposarcoma. While radiologic features and analysis of histology helped to inform management, these cases in conjunction with the literature also illustrate the limitations of the diagnostic work up and importance also factoring the biologic behavior of the tumor in its management. CONCLUSION: Fat-containing tumors of the retroperitoneum that do not stain for MDM2 or CDK4 can pose a diagnostic challenge. Assessing radiologic and pathologic features in conjunction with the biologic behavior of these tumors should inform their management.

Calcaneal Bone Tumors.

Bone tumors of the foot are an uncommon finding. Most tumors are found incidentally on imaging and are benign. Care must be taken although due to the aggressive nature of malignant bone tumors that can occur in the calcaneus. Malignant lesions will more commonly present with symptoms of pain and swelling. Often misdiagnosed as soft tissue injuries, it is critical to be able to diagnose and treat these lesions early. Imaging plays an important role with plain films and advanced imaging. Surgical treatments can range from curettage with grafting to amputation for more aggressive lesions.

Orthopedic issues in vascular anomalies.

Vascular anomalies impact the musculoskeletal system dependent on the tissue involved (skin, subcutis, muscle, cartilage, or bone), the extent of involvement, and the type of anomalous vessels (arteries, capillaries, veins, or lymphatics). These malformations can cause a multitude of musculoskeletal problems for the patient. Leg-length discrepancy, intra-articular involvement, muscular lesions, and primary or secondary scoliosis are amongst the issues that patients face. All of these problems can cause pain, deformity, and a range of functional limitations. Surgical and nonsurgical treatment plans have a role in patient care. Patients with vascular anomalies may also suffer from life-threatening cardiovascular and hematologic abnormalities. For those patients who undergo surgery, the thromboembolic risk is elevated, wound breakdown and infection are much more common, and bleeding risk continues well into the postoperative course. Because of the complex nature of these disorders, the clinician must have a full understanding of the types of lesions, their natural history, appropriate diagnostic studies, associated medical problems, indications for treatment, and treatment options. For severe malformations, especially syndromes such as CLOVES and Klippel- Trenaunay syndrome, interdisciplinary team management is essential for the best outcomes.