Soft Tissue Masses In Children Research Articles

Lipoblastomas and liposarcomas in paediatric patients: A case series

Lipoblastomas and liposarcomas are rare causes of soft tissue masses in paediatric patients. In this retrospective clinical case series we identified 11 patients from our paediatric database (10 with a lipoblastoma and one with a liposarcoma) who had attended our hospital between 1998 and 2019. The median age of patients with lipoblastoma was 29 months. All lipoblastoma cases were managed with surgical excision and histological examination. The 18-year old patient with liposarcoma presented with a metastatic and unresectable tumour that was unresponsive to chemotherapy and radiation. Our experience demonstrates the importance of differentiating the type of soft tissue mass in children.

Quantitative peripheral computed tomography to measure muscle area and assess lean soft tissue mass in children

Background Skeletal muscle is one of the main components of lean soft tissue mass (LSTM). Low levels in children affect locomotion, posture, and increase the risk of metabolic syndrome. Aim (1) To evaluate the association between muscle cross-sectional area (MCSA) of the lower left leg measured by peripheral quantitative computed tomography (pQCT) and total LSTM; namely, total left leg and left lower leg LSTM assessed by dual-energy X-ray absorptiometry (DXA) in a group of children, (2) to examine if MCSA is a predictor of LSTM, (3) to determine the ability of pQCT to identify children with low LSTM. Subjects and methods Lower left leg MCSA and LSTM were measured using pQCT and DXA, respectively, in 396 children. Results Statistically significant positive correlations were found between the lower leg MCSA – total LSTM (r² = 0.789), total leg LSTM (r² = 0.79), and lower leg LSTM (r² = 0.791) (p < .01). MCSA explained 64–68% of the variance in LSTM. Receiver operator characteristic (ROC) curves determined the capacity of the lower left leg MCSA to identify low LSTM in girls (AUC 0.95) and boys (AUC 0.87). Conclusions Our results showed that lower left leg MCSA, measured using pQCT, could be a tool to predict low LSTM in children.

A Step-by-Step Sonographic Approach to Vascular Anomalies in the Pediatric Population: A Pictorial Essay.

Vascular anomalies are a common cause of soft-tissue masses in children and often referred for ultrasonographic (USG) evaluation. They are broadly classified as vascular tumors (hemangiomas, hemangioendotheliomas, and angiosarcomas) or vascular malformations (venous malformations, lymphatic malformations, and arteriovenous malformations). Findings on USG and Doppler imaging can be used to categorize vascular anomalies into high- or low-flow lesions, which forms the basis for further workup, diagnosis, and management. On careful evaluation of various sonographic features, in conjunction with clinical findings, an accurate clinicoradiological diagnosis can be made in most cases. Further imaging with magnetic resonance (MR) imaging or computed tomography (CT) helps in delineation of lesion extent, whereas MR or CT angiography is useful to map the vascular supply of high-flow lesions. We have illustrated and discussed a step-by-step approach to diagnose vascular anomalies using ultrasound and Doppler imaging.

A review of paediatric soft tissues masses referred to a tertiary musculoskeletal sarcoma centre.

To determine the differential diagnosis of musculoskeletal soft tissue masses in children referred to a specialist musculoskeletal oncology unit. All children (0-18 y) referred to a specialist musculoskeletal oncology unit over a 20-month period (September 2018-May 2020) were retrospectively reviewed. Demographic data and referral diagnoses were obtained from the electronic patient notes. MRI findings and histopathological results were recorded. The comparison of non-neoplastic, benign neoplastic and malignant diagnoses at the point of referral and final diagnosis was determined. 116 patients were included, 60 (51.7%) males and 56 (48.3%) females with mean age of 10.6 years (3 months-18 years). 69 (59.5%) patients were referred with a suspected sarcoma, 29 (25.0%) with a suspected benign tumour and 18 (15.5%) with a non-neoplastic lesion. A diagnosis was achieved by histological assessment in 61 (52.6%) cases, microbiological assessment in 3 (2.6%) or clinical and imaging assessment in 52 (44.8%). 67 (57.8%) cases had non-neoplastic pathology, 39 (33.6%) a benign tumour, 4 (3.4%) an intermediate-grade tumour, 6 (5.2%) a malignant tumour. Although over half of children referred to a specialist musculoskeletal oncology unit were suspected of having a soft tissue sarcoma at referral, only 5.2% were diagnosed with a malignant tumour. Approximately, 6 of 69 (8.7%) children referred to a specialist musculoskeletal oncology unit with a suspected soft tissue sarcoma will have a malignant lesion. Most paediatric soft tissue masses are non-neoplastic, the commonest diagnosis being a vascular malformation.

Pearls and Pitfalls in the Imaging of Soft-Tissue Masses in Children

There is a broad spectrum of soft-tissue masses in children that can be challenging to diagnose clinically and on imaging. This article reviews the typical clinical and imaging findings of the most common and relevant benign, intermediate and malignant pediatric soft-tissue tumors in the following categories of the 2013 World Health Organization (WHO) classification: adipocytic tumors (lipoma, lipoblastoma, and liposarcoma), fibroblastic/myofibroblastic tumors (nodular fasciitis, myositis ossificans, fibrous hamartoma of infancy, fibromatosis colli, desmoid-type fibromatosis, lipofibromatosis, and infantile fibrosarcoma), pericytic tumors (myofibroma/myofibromatosis), skeletal muscle tumor (rhabdomyosarcoma), nerve sheath tumors (neurofibroma, malignant peripheral nerve sheath tumor), and uncertain differentiation (synovial sarcoma). In general, ultrasound and magnetic resonance imaging are used as first- and second-line imaging modalities, with limited roles for plain radiographs, computed tomography, and fluorodeoxyglucose-positron emission tomography. Many of these tumors have nonspecific imaging findings although there are some key imaging clues that in conjunction with the clinical information allow a specific diagnosis or a narrow differential diagnosis. However, in many instances, histology is required for final diagnosis.

Imaging Approach to Suspected Vascular Anomalies in Children

The purpose of this review is to become familiar with the terminology and typical presentation of vascular anomalies in children and to present the imaging approach to these anomalies. Vascular anomalies are the most common cause of soft-tissue masses in children. Many physicians are not familiar with the correct terminology for vascular anomalies which results in errors in diagnosis, prognosis, and treatment. The updated International Society for the Study of Vascular Anomalies classification, based on the original Hamburg classification, divides vascular anomalies into two types: vascular tumours (haemangiomas) and vascular malformations (venous, lymphatic, arteriovenous). Grey-scale and Doppler ultrasound (US) are the initial imaging modalities in suspected vascular anomalies. Grey-scale US is highly nonspecific. Therefore, a diagnostic algorithm based on grey-scale and Doppler signs is proposed. Spectral Doppler analysis reveals the type of vascular flow present within the lesion: high-flow is typical for infantile haemangiomas and arteriovenous malformations, low-flow for venous and noflow for lymphatic malformations. Magnetic resonance imaging with angiography is indicated only when US has not been diagnostic, when the lesion is too big or lies too deeply, and when information about the accurate anatomic location and possible infiltration of surrounding structures is necessary. Digital subtraction angiography is performed when interventional treatment is considered. Conclusion − The new terminology of vascular anomalies should be used in everyday clinical practice to avoid misunderstandings. Grey-scale and Doppler US are the most important imaging modalities for the accurate diagnosis and follow-up of vascular anomalies in children.

Fat-containing soft-tissue masses in children.

The diagnosis of soft-tissue masses in children can be difficult because of the frequently nonspecific clinical and imaging characteristics of these lesions. However key findings on imaging can aid in diagnosis. The identification of macroscopic fat within a soft-tissue mass narrows the differential diagnosis considerably and suggests a high likelihood of a benign etiology in children. Fat can be difficult to detect with sonography because of the variable appearance of fat using this modality. Fat is easier to recognize using MRI, particularly with the aid of fat-suppression techniques. Although a large portion of fat-containing masses in children are adipocytic tumors, a variety of other tumors and mass-like conditions that contain fat should be considered by the radiologist confronted with a fat-containing mass in a child. In this article we review the sonographic and MRI findings in the most relevant fat-containing soft-tissue masses in the pediatric age group, including adipocytic tumors (lipoma, angiolipoma, lipomatosis, lipoblastoma, lipomatosis of nerve, and liposarcoma); fibroblastic/myofibroblastic tumors (fibrous hamartoma of infancy and lipofibromatosis); vascular anomalies (involuting hemangioma, intramuscular capillary hemangioma, phosphate and tensin homologue (PTEN) hamartoma of soft tissue, fibro-adipose vascular anomaly), and other miscellaneous entities, such as fat necrosis and epigastric hernia.

Sonographic diagnosis of intraosseous epidermoid cysts of the calvarium

Sonographic diagnosis of intraosseous epidermoid cysts of the calvarium

Juvenile Xanthogranuloma of the Hand: A Case Report

Introduction: Juvenile xanthogranuloma (JXG) is a non-Langerhans-cell histiocytosis, a group of histiocytic proliferative disorders, seen throughout the first two decades of life. Case Presentation: The studied case was a 14-year-old boy who presented a rapidly growing circumscribed brown papule on his left palm. The mass was surgically resected and histopathologic examination confirmed the diagnosis of JXG. Conclusions: Juvenile xanthogranuloma is a cutaneous fibrohistiocytic lesion, which is rarely reported on palms. It is recommended to excise any soft tissue masses in children due to their possible association with malignancy.

Malformation veineuse intramusculaire scapulaire thrombosée : à propos d’un cas chez un enfant

Venous malformation (VM) is a frequent soft tissue mass in children that should not be confused with vascular tumors. We report the case of a 10-year-old boy with a bulky thrombosed VM of the shoulder with functional disability. D-dimer levels were high. Pathognomonic phleboliths were demonstrated on conventional x-rays. Echo-doppler confirmed the venous nature of the lesion and areas without flow suspicious for thrombosis. MRI provides the deep extension and can show non-calcified thrombi that are difficult to see with ultrasound. Localized intravascular coagulopathy is often associated with thrombosis phenomena and has to be confirmed by measurement of D-dimer levels in order to initiate anticoagulation quickly with low-molecular-weight heparin. Follow-up includes measurement of D-dimer levels and potentially MRI to evaluate the anticoagulation efficacy and tailor treatment duration.

Pediatric nodular fasciitis in the head and neck: evaluation and management.

Nodular fasciitis is a rare benign tumor that can present in the head and neck in children. A better understanding of this rare condition is critical to optimize management. To review the presentation, evaluation, diagnosis, and management of pediatric nodular fasciitis of the head and neck. Retrospective review of all patients treated for nodular fasciitis of the head and neck over a 20-year period at a pediatric tertiary care center. Surgical excision. Clinical data, including age, presenting symptoms, anatomical site(s), evaluation, treatment, and complications. Fifteen children with pathologically confirmed nodular fasciitis of the head and neck were identified, including 8 boys and 7 girls. The median (range) age at diagnosis was 9.3 years (2 months to 18 years). Patients most commonly presented with a firm, enlarging soft-tissue mass. Two patients reported pain, and 1 patient presented with erythema. The most common location was the maxillofacial region (5 patients). Other locations included the scalp (3 patients), forehead (2 patients), neck (2 patients), mandible (1 patient), postauricular region (1 patient), and nasal dorsum (1 patient). One patient reported a preceding trauma, and 1 patient, a preceding infection. Presurgical imaging varied; imaging modalities used included computed tomography, magnetic resonance imaging, radiography, ultrasound, and sialography. All patients underwent surgical excision, which focused on excising the mass while preserving surrounding normal tissues. Mean (range) follow-up was 7.69 (0-46) months. Two minor complications were reported: 1 patient who underwent a near-total excisional biopsy experienced residual firmness and tenderness at the site of the lesion and another patient was left with an unfavorable cosmetic scar that necessitated intralesional steroid injection. No patient demonstrated recurrence at follow-up. Although an uncommon diagnosis, nodular fasciitis should be considered in the evaluation and treatment of head and neck soft-tissue masses in children. Preoperative imaging is nonspecific and variable. Pathological findings are necessary for diagnosis. Surgical excisional biopsy is curative, with no instances of recurrence in our series.

Masses des tissus mous d’allure tumorale de l’enfant et de l’adolescent

Pseudotumoral soft tissue masses in children and adolescents are a frequent reason for consultation and a diagnostic dilemma. Soft tissue malignancies are relatively uncommon, unlike the large number of benign lesions that may be seen in the superficial tissue and that can be diagnosed with clinical characteristics. This retrospective study concerns 161 children and adolescents less than 20 years old, referred for a soft tissue mass between 2007 and 2011. It describes their epidemiology, clinical characteristics, and course of care to validate a diagnostic strategy for such masses. Final diagnoses were malignant tumors (44%), benign tumors (32%), and pseudotumoral lesions (24%). Clinical features were similar between these three groups except for age and tumor location, with more benign thoracic masses in younger children. Clinical and radiological association led to an accurate diagnosis for 50% of benign masses and with cytological analysis contribution in 79% of benign tumors and 86% of pseudotumoral lesions. Malignant tumors were suspected in only 39% of cases with radiological exams and in 89% after fine-needle aspiration, an essential additional diagnostic tool. Final diagnoses were formally established through simple standard clinical and radiological evaluation in 19patients (11.8%; benign tumors, seven patients; malformations, eight patients; post-traumatic lesions, two patients; infection and inflammation, one patient each); ultrasound exam in five patients (3.1%; hemangioendotheliomas, two patients, fascial dehiscence, hemangioma, and vascular malformation, one patient each); MRI in four patients (2.5%; three vascular malformations and one lipoma); CT in two cases (1.2%; vascular malformation and myositis ossificans), and radiological examinations associated with cell aspiration in 15 cases (9.3%; ten benign tumors and five malignant tumors). A multidisciplinary approach should be requested from oncological, radiological, and pathologic experts to optimize soft tissue mass management as soon as initial investigations start. The authors advise a diagnostic strategy for children with pseudotumoral soft tissue masses.

Fibrous hamartoma of infancy

Fibrous hamartoma of infancy is a rare benign soft tissue tumor that usually occurs within the first 2 years of life. We report 3 cases of fibrous hamartoma of infancy seen over a 10-year period. Treatment is curative by local excision with a low local recurrence rate. Fibrous hamartoma of infancy should be considered in the differential diagnosis of soft tissue masses in children because it is always benign and subject to nonaggressive treatment.

Pediatric Soft-Tissue Tumors and Pseudotumors: MR Imaging Features with Pathologic Correlation

In the final part of this two-part review article on soft-tissue masses in children, the magnetic resonance (MR) imaging features, clinical findings, and pathologic findings in a wide variety of tumors, including those of fibroblastic/myofibroblastic origin, so-called fibrohistiocytic tumors, smooth-muscle tumors, skeletal-muscle tumors, tumors of uncertain differentiation, and lymphoma, are described. Other neoplasms that are not included in the World Health Organization classification of soft-tissue tumors but may be seen clinically as soft-tissue masses, specifically dermatofibrosarcoma protuberans, neurogenic tumors and pilomatricoma, are also included. In contrast to the tumors reviewed in Part 1 of this review, the MR imaging features and clinical findings of the tumors included here are largely nonspecific. However, MR imaging is useful in determining site of tumor origin, extent of disease, and relation of tumor to adjacent anatomic structures, and for follow-up after therapy. In some of these entities, the combination of findings may aid in narrowing the differential diagnosis, such as persistent low signal intensity on T1- and T2-weighted images in some fibroblastic lesions, identification of hemosiderin and a synovial origin in pigmented villonodular synovitis, or the presence of multiple target signs on T2-weighted images in deep plexiform neurofibroma. In a large number of cases, however, tissue biopsy is required for final diagnosis.

Pediatric Soft-Tissue Tumors and Pseudo-tumors: MR Imaging Features with Pathologic Correlation

A wide spectrum of entities may give rise to soft-tissue masses in children, including benign and malignant tumors, pseudotumors, and both neoplastic and nonneoplastic vascular lesions. Because of its excellent tissue contrast, multiplanar capability, and lack of ionizing radiation, magnetic resonance (MR) imaging has become the modality of choice in the evaluation of deep and large soft-tissue masses in children. In the vast majority of cases, however, accurate interpretation of the MR imaging findings requires correlation with the clinical findings. For example, in most posttraumatic and inflammatory pseudotumors, the clinical history is fundamental to establishing the diagnosis. In the evaluation of periarticular cysts, the location of the mass and its relationship to a joint are crucial for diagnosis, whereas in the evaluation of vascular lesions, including hemangiomas and vascular malformations, clinical findings combined with MR imaging findings are needed for accurate diagnosis in most cases. The identification of fat within adipocytic tumors is useful, but tissue biopsy may be required for final diagnosis. Nevertheless, MR imaging is useful in determining the origin and character of pediatric soft-tissue masses, defining their extent and their relationship to adjacent structures, and performing posttherapy follow-up.

Nodular fasciitis: a sarcomatous impersonator

Reports of nodular fasciitis among adults are common; however, this condition is relatively rare in the pediatric population. Its clinical and histologic characteristics are similar to malignancies such as sarcoma; thus, it is prudent for the clinician caring for children and adolescents to be aware of the possibility of its occurrence. Nodular fasciitis is a benign mesenchymal tumor. Often presenting as a rapidly enlarging soft tissue mass, clinically, it can easily be mistaken as a sarcoma or other malignancy during clinical evaluation. In addition, the pathologist may recognize its high cellularity, high mitotic index, and infiltrative borders, which, as a result, may lead to erroneous diagnosis as a malignancy. Although more frequently seen in adults, it does occur in the pediatric population and should be considered during evaluation and treatment of soft tissue masses in children and adolescents.

Imaging and diagnostic strategy of soft tissue tumors in children

The diagnosis of a soft tissue mass in children is a common clinical situation. Most of the lesions are benign and can be treated conservatively or by non-mutilating surgery. Nevertheless, the possibility of a malignant soft tissue tumor must be systematically considered. The most frequent benign soft tissue lesions in children are vascular lesions, fibrous and fibrohistiocytic tumors and pseudotumors, whereas rhabdomyosarcomas account for 50% of all soft tissue sarcomas. A child presenting an atypical soft tissue mass should be managed by a multidisciplinary centre, and primary resection must be proscribed until a definite diagnosis has been established. The role of imaging is essential either to confirm the benign nature of the mass or to give arguments to perform a diagnostic biopsy. Clinical examination, conventional radiography and ultrasound with Doppler represent the first-line examinations and are sometimes sufficient to assess a diagnosis. In all other situations, MRI is mandatory to establish the probable nature of the lesion and to assess local extension.

Doppler sonography of soft tissue masses in children

Doppler sonography of soft tissue masses in children

Is reexcision in pediatric nonrhabdomyosarcoma soft tissue sarcoma necessary after an initial unplanned resection?

Purpose: The aim of this study was to determine the importance of pretreatment reexcision (PRE) of pediatric nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) after initial unplanned resection. Methods: The authors retrospectively reviewed the records of 116 consecutive patients with surgically resected NRSTS treated at their institution between February 1978 and September 1999. Ninety-four (81.0%) patients had undergone unplanned resections before referral to their institution for further therapy. Demographic data, tumor characteristics, treatment administered, and outcomes were recorded. Results: Sixty-nine patients (73.4%) underwent PRE at a median interval after the initial unplanned resection of 29 days. Twenty-five patients were thought unsuitable for PRE because of the proximity to vital neurovascular bundles. Tumors deemed not feasible for PRE were more likely to be greater than 5 cm (P =.0094) and high grade (P =.0200). Tumor was found in 33 (47.8%) of the PRE specimens, and 24 of these patients (72.7%) were deemed disease free after achieving negative surgical margins. Residual tumor was more likely to be found after PRE in head and neck primary tumors than in trunk wall or extremity primary tumors (P =.0128). There were no local failures in the 60 PRE patients who achieved clear margins. The estimated 5-year event-free and 5-year overall survival rates in these 60 patients were 98.3% ± 2.0% and 98.2% ± 2.1%, respectively. Conclusions: Pretreatment reexcision should be performed whenever feasible in pediatric patients with NRSTS who had an initial unplanned resection. The proportion of patients with residual tumor in the PRE specimen is high, and negative microscopic margins can be achieved after PRE in most patients with residual tumor. Despite delay in obtaining a complete surgical resection, no local recurrences were seen. The possibility of NRSTS should be considered when resecting a soft tissue mass in children, and diagnostic incisional biopsy followed by wide local excision with negative microscopic margins should be the surgical goal. J Pediatr Surg 37:1424-1429. Copyright 2002, Elsevier Science (USA). All rights reserved.

Recurrent congenital haemangiopericytoma in a child

A five-day-old boy was referred with a soft-tissue mass in his right upper arm. Plain radiographs and ultrasound demonstrated a lesion extending from the axilla to the elbow on the posterolateral aspect of the humerus. Open biopsy confirmed the diagnosis of congenital haemangiopericytoma. After MRI and selective angiography, excision biopsy was carried out, but no adjuvant therapy was administered. At further examination, four years and ten months later, he was noted to have three small nodules at the site of the original tumour. Excision biopsy confirmed this to be a local recurrence, although the lesion was less cellular with no appreciable mitotic activity. Congenital haemangiopericytoma is a rare cause of a soft-tissue mass in children. Most tumours are benign, and recurrence is uncommon. The treatment is controversial, but most centres recommend the use of adjuvant chemotherapy, combined with complete excision. We recommend treatment with doxorubicin. Orthopaedic surgeons should be familiar with this tumour since 30% to 50% of cases occur in the limbs.