Superficial Soft Tissue Tumors Research Articles

Soft tissue hemangioma of the right upper extremity with intraosseous extension and secondary intravascular papillary endothelial hyperplasia.

Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's tumor, is an uncommon exuberant form of organizing thrombus that may occur within a vessel, vascular tumor, or hematoma and may change the imaging appearance to mimic an aggressive process. It must be distinguished pathologically from angiosarcoma. They have been most commonly reported within superficial soft tissue tumors, and rapid growth and effect on bone are rarely described. We present a case of a patient with a soft tissue hemangioma with IPEH with intraosseous extension that presented with a pathologic fracture of her right humerus with an aggressive appearing osseous lesion. CT and MRI demonstrated a multifocal ill-defined soft tissue mass throughout the right upper extremity with underlying cortical tunneling and scalloping of the proximal humerus. Similar imaging findings were also present in the distal humerus and ipsilateral scapula and evolved during her hospitalization. Following percutaneous biopsy revealing hemangioma with features of papillary endothelial hyperplasia with intraosseous extension, the patient died in the ICU secondary to unrelated septic shock. Diagnosis was confirmed at autopsy. Primary and secondary IPEH have been generally characterized as well-defined solitary masses, most often in the superficial soft tissues. This case of a deep soft tissue hemangioma with type II IPEH, intraosseous extension, and imaging findings of regional multicompartmental involvement is very unusual. Reporting of this case in the literature should be beneficial for pathologic correlation with similar confounding masses as well as propose a possible mechanism for intraosseous extension of soft tissue hemangiomas.

Intra-Abdominal and Retroperitoneal Benign Lipomatous Tumors-An Extremely Rare Mimic of Liposarcoma and its Diagnostic Challenge.

Background. Lipomas are common superficial soft tissue tumors of mature adipocytes. In contrast, well-differentiated/dedifferentiated liposarcoma typically presents in the retroperitoneum as large masses. We provide clinicopathologic and follow-up details of 9 retroperitoneal/intra-abdominal benign lipomatous tumors (BLT) and discuss the utility of ancillary fluorescence in situ hybridization (FISH) in distinguishing from their malignant counterparts. Design. Clinicopathologic details and histology of 9 intra-abdominal and retroperitoneal lipomas were studied along with ancillary CD10 immunohistochemistry (IHC) and FISH for MDM2 and CDK4 amplification. Results. There were 6 females and 3 males. Median age at diagnosis was 52 years (range 36-81 years). Seven were identified incidentally and 2 presented with primary complaints. On imaging, 7 were considered suspicious for liposarcoma. Grossly, the tumors ranged from 3.4 to 41.2 cm (median 16.5 cm). Histologically, all cases showed well-differentiated BLT, further classified as lipoma (n = 7; 1 with metaplastic ossification, 2 with prominent vessels, and 4 ordinary lipomas) and lipoma-like hibernoma (n = 2)-the latter 2 showed intramuscular lesions with interspersed brown fat. CD10 IHC showed strong staining in the 2 hibernomas, whereas the staining was weak in the remaining. MDM2 and CDK4 amplification were negative by FISH in all. Follow-up (median 18 months) did not show recurrence on clinical or imaging evaluation. Conclusion. Retroperitoneal/intra-abdominal BLT are extremely rare and are indistinguishable clinically and radiographically from liposarcoma. This necessitates molecular confirmation even when the histology is convincingly benign, for a confident diagnosis. Our cohort shows that conservative excision without removal of abutted organs is sufficient in most cases.

How Is the Spectrum of Sarcoma Surgery Assessed?

To meet the challenges of the precision medicine era, quality assessment of shared sarcoma care becomes pivotal. The MDT approach is the most important parameter for a successful outcome. Of all MDT disciplines, surgery is the key step to rendering sarcoma patients disease free; therefore, defining its spectrum is critical. To the best of the authors' knowledge, a comprehensive interoperable digital platform to assess the scope of sarcoma surgery in its full complexity is lacking. An interoperable digital platform on sarcoma surgery has been created to assess the clinical exposure, tumor characteristics, and surgical settings and techniques applied for both resections and reconstructions of sarcomas. The surgical exposure of an individual surgeon over time served as a pilot. Over the study period of 10 years, there were 723 sarcoma board/MDT meetings discussing 3130 patients. A total of 1094 patients underwent 1250 surgical interventions on mesenchymal tumors by one single sarcoma surgeon. These included 615 deep soft tissue tumors (197 benign, 102 intermediate, 281 malignant, 27 simulator, 7 metastasis, 1 blood); 116 superficial soft tissue tumors (45 benign, 12 intermediate, 40 malignant, 18 simulator, 1 blood); and 519 bone tumors (129 benign, 112 intermediate, 182 malignant, 18 simulator, 46 metastasis, 14 blood, and 18 sequelae of first treatment). Detailed types of resections and reconstructions were analyzed. An interoperable digital data platform on sarcoma surgery with transparent real-time descriptive analytics is feasible and enables large-scale definition of the spectrum of sarcoma surgery to meet the challenges of sarcoma precision care in the future.

Myxolipoma of hand in a child: case report of a rare tumor

Lipomatous tumors account for less than 10% of tumors in the pediatric population. Myxolipomas (a subset of lipoma characterised by mature adipose tissue and abundant mucoid substance) are found to be even rarer. There are a few case reports in different body parts like heart, kidney, oral cavity, epiglottis, cervical and mediastinal regions. However, there are no case reports on the involvement of the hands in any age group. High resolution ultrasound is the imaging modality of choice for the initial evaluation of superficial soft tissue tumors, their site, nature and extent. In conjunction with clinical findings and age of presentation, it helps in narrowing down the differential diagnosis and planning the management. Hyperechoic fatty tumors in the pediatric hand are mostly benign and includes lipomas, lipoblastomas and fibrous hamartomas of infancy as the main differentials. A definitive diagnosis is based on a histo-pathological and molecular cytogenetic examination. This article presents a never before reported case of a rare, large, myxolipoma of the hand in a 22-month-old boy.

Superficial Soft-Tissue Masses of the Head and Neck: A Pictorial Review

The differential diagnosis of superficial masses in the head and neck is broad and encompasses both benign and malignant soft-tissue tumors. Certain superficial masses of dermal origin do not fall under the World Health Organization classification for soft-tissue tumors but, nonetheless, present similarly and should be considered in the differential. Although many of these superficial masses cannot be definitively diagnosed on imaging alone, recognizing certain imaging patterns and ancillary clinical features may help narrow the differential diagnosis and distinguish benign and malignant lesions. The present article does not aim to provide a comprehensive review of all superficial head and neck masses but rather helps to organize the more common masses by cellular origin and provides an overview of pertinent demographics or risk factors to aid in informed decision-making.Learning Objective: To generate a differential diagnosis of head and neck superficial soft-tissue tumors based on clinical history and imaging features

Accuracy of ultrasound in the characterization of superficial soft tissue tumors: a prospective study.

To prospectively evaluate the accuracy of ultrasound in defining the specific nature of superficial soft tissue masses as well as determining malignancy. Eight hundred twenty-three superficial soft tissue masses were prospectively evaluated with ultrasound by one of five experienced musculoskeletal radiologists. The radiologist at the time of examination provided one to three specific differential diagnoses and the perceived level of confidence with regard to each diagnosis. Clinical and ultrasound diagnoses were compared with the histological diagnosis to determine accuracy. Tumor malignancy was determined by histology or clinical/imaging follow-up. Histological correlation was present for 219 (26.6%) of the 823 masses. Compared with histology, the accuracy of clinical and ultrasound examination for determining specific tumor type was 25.6% and 81.2% respectively considering all differential diagnoses provided. Radiologists were "fully confident" with the ultrasound diagnosis in 585 (71.1%) of 823 masses overall. In this setting, when compared with histology, the diagnostic accuracy of ultrasound was 95.5%. When the radiologist was "not fully confident," accuracy was 41.3% for the first differential diagnosis and 60.9% for all differential diagnoses. Diagnostic accuracy improved with increasing radiologist experience. Sensitivity, specificity, positive predictive value, and negative predictive value of ultrasound for identifying malignant tumor were 93.3%, 97.9%, 45.2%, and 99.9% respectively. One can be "fully confident" at characterizing over two-thirds of superficial soft tissue masses based on ultrasound appearances and, in this setting, diagnostic accuracy is very high. Ultrasound examination is also highly accurate at discriminating benign from malignant superficial soft tissue masses.

Ultrasonography of Pediatric Superficial Soft Tissue Tumors and Tumor-Like Lesions.

Ultrasonography (US) is usually the first imaging examination performed to evaluate palpable or visible superficial soft tissue lesions that are common in children. Although clinical assessments, such as age at presentation, clinical course, and overlying skin discoloration, are important for the differentiation of pediatric soft tissue lesions, US allows a specific diagnosis of some typical benign lesions and helps in guiding further investigation since it provides detailed information about the lesion location, characterization including solid versus cystic, vascularity, and compressibility. Therefore, sufficient knowledge of the normal anatomy, proper ultrasonographic techniques, and the imaging findings of common and uncommon soft tissue lesions in children are crucial for accurate assessment and management of patients. In this article, we review the techniques and imaging findings focusing on the ultrasonographic features of a variety of superficial soft tissue lesions detected in children.

Elastography: Applications in Superficial Soft Tissue Tumors

Elastography: Applications in Superficial Soft Tissue Tumors

Recurrent NTRK1 Gene Fusions Define a Novel Subset of Locally Aggressive Lipofibromatosis-like Neural Tumors

The family of pediatric fibroblastic and myofibroblastic proliferations encompasses a wide spectrum of pathologic entities with overlapping morphologies and ill-defined genetic abnormalities. Among the superficial lesions, lipofibromatosis (LPF), composed of an admixture of adipose tissue and fibroblastic elements, in the past has been variously classified as infantile fibromatosis or fibrous hamartoma of infancy. In this regard, we have encountered a group of superficial soft tissue tumors occurring in children and young adults, with a notably infiltrative growth pattern reminiscent of LPF, variable cytologic atypia, and a distinct immunoprofile of S100 protein and CD34 reactivity, suggestive of neural differentiation. SOX10 and melanocytic markers were negative in all cases tested. In contrast, a control group of classic LPF displayed bland, monomorphic histology and lacked S100 protein immunoreactivity. To define the pathogenetic abnormalities in these seemingly distinctive groups, we performed RNA sequencing for fusion gene discovery in 2 cases each, followed by screening for any novel alterations identified in a larger cohort representing both entities. The 2 index LPF-like neural tumors (LPF-NT) showed TPR-NTRK1 and TPM3-NTRK1 gene fusions, which were further validated by fluorescence in situ hybridization (FISH) and reverse transcription polymerase chain reaction. Subsequent FISH screening of 14 LPF-NT identified recurrent NTRK1 gene rearrangements in 10 (71%) cases. Of the NTRK1-negative LPF-NT cases, 1 case each showed ROS1 and ALK gene rearrangements. In contrast, none of the 25 classic LPFs showed NTRK1 gene rearrangements, although regional abnormalities were noted in the 1q21-22 region by FISH in a majority of cases. Furthermore, NTRK1 immunostaining was positive only in NTRK1-rearranged S100-positive LPF-NT but negative in classic LPF. These results suggest that NTRK1 oncogenic activation through gene fusion defines a novel and distinct subset of soft tissue tumors resembling LPF, but displaying cytologic atypia and a neural immunophenotype, provisionally named LPF-like neural tumors.

Points to notice during the diagnosis of soft tissue tumors according to the "Clinical Practice Guideline on the Diagnosis and Treatment of Soft Tissue Tumors".

To adequately treat soft tissue tumors, an accurate initial diagnosis is essential. In this article, we describe the notable points during initial diagnoses as outlined by the "Clinical Practice Guideline on the Diagnosis and Treatment of Soft Tissue Tumors". Clinical findings including pain and tumor size, growth speed, and depth, may be important in the diagnosis. In general, malignant soft tissue tumors tend to be larger than 5cm in size, to occur in deep layers, and grow quickly. However, there are many exceptions. Magnetic resonance images can show characteristics of soft tissue tumors, but most of these are non-specific. The most reliable diagnostic procedure is histological examination. Therefore, a biopsy is mandatory. A core needle biopsy can be performed under local anesthesia at an outpatient clinic without the need for imaging guidance. In cases with deep-seated tumors, an image-guided biopsy is recommended. Incisional biopsies are also widely used procedures. In an incisional biopsy, the skin incision should be parallel to the long axis of the extremities. A meticulous hemostasis must be carried out to prevent post-biopsy hemorrhaging. If adequate hemostasis cannot be achieved, a drain is placed within the incision or at the closest possible site along the incision. Indications for an excisional biopsy are limited to superficial soft tissue tumors, less than 3cm in size, located at a suitable distance from the nearest vital structure. Furthermore, an excisional biopsy should only be carried out after imaging studies are complete.

Strain elastography features of epidermoid tumours in superficial soft tissue: differences from other benign soft-tissue tumours and malignant tumours.

We evaluated ultrasonographic features of superficial epidermoid tumour with a focus on strain elastography (SE) features that will help in the differential diagnosis of epidermoid tumour from other benign and malignant soft-tissue tumours. We retrospectively evaluated ultrasonographic and SE data of 103 surgically confirmed superficial soft-tissue tumours and tumour-like lesions: 29 cases of epidermoid tumour, 46 cases of other benign tumours and 28 cases of malignant tumour. SE and B-mode imaging were performed at the same time. SE characteristics were assigned into four grades (1-4) according to their elasticity. Interobserver agreement for the four SE scores between the two radiologists was analysed using kappa statistics. We classified each SE finding as a hard lesion (SE Score 3-4) or soft lesion (SE Score 1-2) and compared these findings using the χ(2) test to identify whether a significant difference in mass hardness existed among epidermoid tumour, other benign tumour and malignant tumour. Overall interobserver agreement according to the four SE scores was moderate (κ = 0.540), and overall agreement for the hardness [soft (Score 1-2) or hard (Score 3-4)] was almost perfect (κ = 0.825). Malignant tumours showed higher SE scores (3-4, hard nature) than did epidermoid tumour or other benign soft-tissue tumours. There were no differences in SE score between epidermoid tumour and other benign tumours. Superficial epidermoid tumour exhibits a softer nature than does malignant tumour but does not have a different SE pattern from other benign tumours. SE features of epidermoid tumour might be helpful in differentiating from other benign and malignant tumours.

Lipomatous tumours of the head and neck: a spectrum of biological behaviour.

Lipomatous tumours are rare in the head and neck region. Their biological behaviour varies greatly, from absolutely benign to histologically benign but locally infiltrative and, finally, invasive with metastatic potential. Each lipomatous tumour has to be treated accordingly. It is of paramount importance before eventual surgery is planned to perform adequate imaging, diagnostic biopsy and careful assessment. Only in small (<5 cm), superficial soft tissue tumours or when magnetic resonance imaging has demonstrated specific features of lipoma, may diagnostic biopsy be omitted. In these cases, expectant management or simple excision is appropriate. Adequate preoperative diagnosis is important to assure adequate tumour control as well as optimal functional and cosmetic outcome. The major problem in the treatment of lipomatous tumours of the head and neck region is the presence of nearby delicate structures. Especially, wide surgical excision of liposarcomas may be hindered by anatomic constraints and may result in impaired functional and cosmetic outcome. Neoadjuvant radiotherapy and specific systemic chemotherapy may be helpful in the treatment of liposarcoma, especially when unresectable or when primary surgery is expected to result in poor oncological, functional or cosmetic outcome. Greater emphasis placed on the underlying biology of individual sarcoma subtypes, development and evaluation of novel therapies and greater specificity in the selection of chemotherapy agents based on activity in individual histological subtypes are expected to lead to improved efficacy of systemic treatment.

Use of strain ratio in evaluating superficial soft tissue tumors on ultrasonic elastography.

The aim of this study was to describe the strain ratio in histopathologically confirmed superficial soft tissue tumors using ultrasonic elastography (USE). From March to August 2012, 94 soft tissue tumors in 79 consecutive patients (age range 19-70years) were examined using USE for palpable masses. Among them, 39 lesions in 36 patients were histopathologically confirmed. Thirty-four lesions in 31 patients with lipoma, ganglion, epidermal inclusion cyst, and pilomatricoma were included: 34 histopathologically confirmed mature lipomas (n=19), ganglia (n=6), epidermal inclusion cysts (n=5), and pilomatricomas (n=4). Ultrasonographic features of margins, echogenicity, walls, ultrasonic transmission, and vascular flow via Doppler ultrasonography were evaluated. The strain ratios were calculated from the USE strain measured within the lesion and in adjacent tissue. Intraclass correlation (ICC) with Cohen's kappa was used to determine intraobserver agreement, and the strain ratios of the three groups were compared using Mann-Whitney U tests. All statistical analyses were performed using R package statistical software, and p values <0.05 were considered to be statistically significant. On USE, repeated measurements of the strain ratios showed excellent intraobserver agreement [ICC value=0.952 (CI 0.905-0.977), p<0.05]. The strain ratios of lipomas (median±standard error 0.83±0.18) were statistically different (p<0.05) from those of ganglia, epidermal inclusion cysts, and pilomatricomas. The strain ratios of ganglia (2.78±0.48) were also statistically different (p<0.05) from those of epidermal inclusion cysts and pilomatricomas. The strain ratios of epidermal inclusion cysts (0.17±0.21) were not statistically different (p>0.05) from that of pilomatricomas (0.13±0.02). Strain ratios determined by USE help to differentiate lipomas and ganglia from other superficial soft tissue tumors. Our results suggest that USE could be used in addition to traditional ultrasound techniques to obtain diagnostic clues in the evaluation of superficial soft tissue tumors.

Tumors of fibrous tissue involving the skin

Fibroblastic and myofibroblastic tumors represent a relatively common group of soft tissue lesions that includes reactive, hamartomatous and neoplastic conditions. The neoplastic tumors range from benign to intermediate to fully malignant neoplasms, capable of metastasis leading to the death of patient. These tumors can be diagnostically challenging because of the morphologic and immunohistochemical overlap, despite the clinical and molecular differences. As a general rule, superficial soft tissue tumors, especially cutaneous tumors, are less aggressive than deep tumors. In this short overview, several fibroblastic and myofibroblastic tumor entities which can involve the skin will be discussed with emphasis on the clinicopathological features, differential diagnosis, and the rule of immunohistochemical and molecular studies in their diagnosis. Recent advances/developments in this subject will also be reviewed. Fibroblastic and myofibroblastic tumors represent a relatively common group of soft tissue lesions that includes reactive, hamartomatous and neoplastic conditions. The neoplastic tumors range from benign to intermediate to fully malignant neoplasms, capable of metastasis leading to the death of patient. These tumors can be diagnostically challenging because of the morphologic and immunohistochemical overlap, despite the clinical and molecular differences. As a general rule, superficial soft tissue tumors, especially cutaneous tumors, are less aggressive than deep tumors. In this short overview, several fibroblastic and myofibroblastic tumor entities which can involve the skin will be discussed with emphasis on the clinicopathological features, differential diagnosis, and the rule of immunohistochemical and molecular studies in their diagnosis. Recent advances/developments in this subject will also be reviewed.

Unusual myoid, perivascular, and postradiation lesions, with emphasis on atypical vascular lesion, postradiation cutaneous angiosarcoma, myoepithelial tumors, myopericytoma, and perivascular epithelioid cell tumor

In recent years, a number of new soft tissue tumor entities have been described that occur in the skin only, or that also occur in other sites but form clinically and pathologically distinct subsets when arising in the skin and subcutaneous tissue. These include a variety of mesenchymal lineages and have variable malignant potential, although superficial malignant soft tissue tumors generally have a more favorable outcome than their more deeply located counterparts. This article reviews the clinical and pathologic features and differential diagnoses of atypical vascular lesion, postradiation cutaneous angiosarcoma, myoepithelioma, myopericytoma, and perivascular epithelioid cell tumor.

Sonographic Appearances of Small Organizing Hematomas and Thrombi Mimicking Superficial Soft Tissue Tumors

We retrospectively reviewed 2 cases of organizing hematomas and 2 cases of intravascular organizing thrombi and investigated correlations between sonographic and pathologic findings. In all 4 cases, a well-defined hypoechoic heterogeneous mass with surrounding increased echogenicity was evident in the subcutaneous fat layer. Organizing hematomas and thrombi have sonographic features similar to those of benign-looking soft tissue tumors. These lesions should therefore be considered in the differential diagnosis of superficial soft tissue masses.

The Spectrum of Vascularized Superficial Soft-Tissue Tumors on Sonography With a Histopathologic Correlation: Part 2, Malignant Tumors and Their Look-Alikes

The objective of this article is to illustrate the sonographic findings of malignant, vascularized superficial soft-tissue masses and their look-alikes. Radiologists need to be aware of the sonographic findings of malignant, vascularized superficial soft-tissue tumor findings and their look-alikes for the differential diagnosis and determining an optimal treatment plan.

The Spectrum of Vascularized Superficial Soft-Tissue Tumors on Sonography With a Histopathologic Correlation: Part 1, Benign Tumors

The objective of this article is to illustrate the sonographic findings of benign, vascularized superficial soft-tissue tumors. Sonography can play an important role in the diagnosis of benign, vascularized superficial soft-tissue tumors. Awareness of these findings is important in establishing the correct diagnosis and determining the optimal treatment.

Imaging of Benign Pediatric Soft Tissue Tumors

There is a broad spectrum of benign soft tissue tumors in children, including, among others, vascular, fibroblastic/myofibroblastic, and adipocytic lesions as well as pseudotumors. The diagnosis of superficial soft tissue tumors is in many instances made clinically, whereas those with more equivocal presentation and those found in the deeper soft tissues often require imaging. Ultrasonography is the modality of choice for smaller and superficial lesions and is particularly useful for vascular tumors. Magnetic resonance imaging is the modality of choice for the larger and deeper lesions and for those in which ultrasonography is not adequate. Plain radiographs and computed tomography have a very limited role in the evaluation of soft tissue masses. Correlation with clinical history and findings on physical examination are imperative for an appropriate interpretation of the imaging findings. Although certain entities can be confidently diagnosed based on the combination of clinical and imaging findings, histology is frequently required for a definitive diagnosis. The differentiation of benign from malignant tumors is also challenging and often not possible based on the imaging findings. This article reviews the clinical features and imaging findings of the most common benign pediatric soft tissue tumors.

Surgical Outcome in Patients Treated for Hemangioma During Infancy, Childhood, and Adolescence

Hemangiomas are the most common tumors in infancy and childhood and account for 7% of benign soft tissue tumors. Diagnosis is usually made in infancy or childhood. There are only a few reports on the surgical treatment of these lesions, likely because the lesions are quite vascular, have a tendency to infiltrate into the muscle and other tissues, and the recurrence rate is quite high. We reviewed the outcome of surgical treatment on 44 consecutive children and adolescents with 47 surgically treated hemangiomas. Hemangiomas involving the face and the head were excluded. Eleven lesions were surgically treated before the age of 5 years, 20 lesions between the ages of 5 and 12 years, and 16 lesions were treated after the age of 12 years. Eleven lesions had intralesional resection, 33 lesions underwent marginal resection, 2 lesions were managed with wide resection, and 1 lesion underwent radical resection. The overall recurrence rate was 22.2%. In the pediatric population, it is desirable to perform definitive treatment whenever possible, thereby minimizing morbidity, both functional and psychological. Asymptomatic lesions should be monitored to confirm the diagnosis and to look for signs of progression. Symptomatic lesions can be treated with surgical excision if this does not result in significant functional impairment. A marginal resection may be used to treat most superficial soft tissue tumors. Intramuscular hemangiomas pose a more difficult therapeutic problem. When the lesion is limited in size, it may be treated with wide local excision. However, if the lesion is more diffusely spread into the muscle unit, the morbidity created by extensive muscle resection has to be weighed against the morbidity of the condition or alternative treatment methods. Whenever possible, a wide marginal resection should be carried out to minimize the risk of recurrence and avoid further surgical procedures. We recommend prolonged follow-up of these patients because 7% of the patients included in this study required late review due to multiple recurrences of these lesions. Level IV (case series).