Release Date: 2024-06-10

Pediatric Soft Tissue Tumors

Fatih Ates (Author), Mehmet Sedat Durmaz (Author)

Release Date: 2024-06-10

Soft tissue masses are very common and have an extraordinarily wide range of possible etiologies in pediatric age group. The World Health Organization (WHO) has classified soft tissue tumors into nine distinct categories. Each category is further subdivided into benign, intermediate (locally aggressive), and malignant types. These categories are as follows: Adipocytic tumors; Muscle tissue [...]

Media Type
    Buy from

    Price may vary by retailers

    Work TypeBook Chapter
    Published inThe Radiology of Cancer
    First Page405
    Last Page450
    DOIhttps://doi.org/10.69860/nobel.9786053359364.27
    Page Count46
    Copyright HolderNobel Tıp Kitabevleri
    Licensehttps://nobelpub.com/publish-with-us/copyright-and-licensing
    Soft tissue masses are very common and have an extraordinarily wide range of possible etiologies in pediatric age group. The World Health Organization (WHO) has classified soft tissue tumors into nine distinct categories. Each category is further subdivided into benign, intermediate (locally aggressive), and malignant types. These categories are as follows: Adipocytic tumors; Muscle tissue tumors; Peripheral nerve sheath tumors; Fibrohistiocytic tumors; Fibroblastic - myofibroblastic tumors; Vascular tumors; Tumors of bone and cartilage; Ewing sarcoma family of tumors; Tumors of uncertain differentiation. Malignant soft tissue tumors (sarcomas) are relatively rare, representing less than 1% of all malignancies. Soft tissue sarcomas are associated with high mortality rates. For many soft tissue masses, it is quite difficult to distinguish between benign and malignant solely based on imaging. Some soft tissue sarcomas can appear deceptively well-defined, with smooth margins and homogeneous signal intensity. If a lesion does not have characteristics specific to a particular benign entity, it should be considered potentially malignant. Superficial soft tissue masses larger than 5 cm have a 10% chance of being sarcomas. Estimating the grade of a soft tissue sarcoma using imaging alone is unreliable. The presence of large, necrotic, and infiltrative masses supports a high-grade lesion. However, high-grade lesions can also appear small, homogeneous, and well-capsulated. Most soft tissue tumors exhibit non-specific, heterogeneous signal intensity characteristics on both T1-weighted (T1W) and fluid-sensitive MRI sequences. However, certain tissue signal types can help indicate a specific tumor type. Adipose tumors contain fatty tissue with high signal intensity on T1W images, and these fatty areas are seen as low signal intensity on fat-suppressed (FS) sequences. Fibroblastic tumors often contain areas with intermediate or low signal intensity on both T1W and fluid-sensitive MRI sequences. Fibrohistiocytic tumors frequently exhibit heterogeneous signal intensity characteristics. The presence of a mass in a typical location can also aid in suggesting a specific diagnosis.

    Fatih Ates (Author)
    MD, Radiologist, Konya City Hospital
    https://orcid.org/0000-0002-2693-4616
    3Fatih ATEŞ completed his medical education at Akdeniz University Faculty of Medicine. He completed his radiology specialization training at Selcuk University Radiology Department. Currently, he continues his professional career in the Radiology Department at Konya City Hospital. Mehmet Sedat DURMAZ completed both his medical education and radiology specialization training at Akdeniz University. He obtained the titles of Associate Professor and Professor at Selçuk University. He received scientific publication awards at Selçuk University for two consecutive years. He has over 50 research articles published in Pubmed database. He holds various significant diplomas and certificates, including the European Diploma in Radiology (EDIR). He continues his profession as a Professor of Radiology at Selçuk University Faculty of Medicine.

    Mehmet Sedat Durmaz (Author)
    Professor, Selcuk University
    https://orcid.org/0000-0002-1340-2477

    • Kallen, M.E. and J.L. Hornick, The 2020 WHO Classification: What’s New in Soft Tissue Tumor Pathology? Am J Surg Pathol, 2021. 45(1): p. e1-e23.

    • Li, C., et al., Ultrasound performance in pediatric deep soft-tissue tumor characterization. Sci Rep, 2023. 13(1): p. 22107.

    • Riley, G.M., et al., MRI of Soft-Tissue Tumors: What to Include in the Report. Radiographics, 2024. 44(6): p. e230086.

    • Coran, A., et al., Non Lipomatous Benign Lesions Mimicking Soft-tissue Sarcomas: A Pictorial Essay. In Vivo, 2018. 32(2): p. 221-229.

    • Brennan, M.F., C.R. Antonescu, and R.G. Maki, Management of soft tissue sarcoma. 2013: Springer.

    • Achar, S., J. Yamanaka, and J. Oberstar, Soft Tissue Masses: Evaluation and Treatment. Am Fam Physician, 2022. 105(6): p. 602-612.

    • Burt, A.M. and B.K. Huang, Imaging review of lipomatous musculoskeletal lesions. Sicot j, 2017. 3: p. 34.

    • Goyal, S., et al., MRI Evaluation of Soft Tissue Tumors and Tumor-Like Lesions of Extremities. Cureus, 2023. 15(4): p. e37047.

    • Bansal, A., et al., WHO classification of soft tissue tumours 2020: An update and simplified approach for radiologists. Eur J Radiol, 2021. 143: p. 109937.

    • Navarro, O.M., E.E. Laffan, and B.Y. Ngan, Pediatric soft-tissue tumors and pseudo-tumors: MR imaging features with pathologic correlation: part 1. Imaging approach, pseudotumors, vascular lesions, and adipocytic tumors. Radiographics, 2009. 29(3): p. 887-906.

    • Gaskin, C.M. and C.A. Helms, Lipomas, lipoma variants, and well-differentiated liposarcomas (atypical lipomas): results of MRI evaluations of 126 consecutive fatty masses. AJR Am J Roentgenol, 2004. 182(3): p. 733-9.

    • Nishida, J., et al., Imaging characteristics of deep-seated lipomatous tumors: intramuscular lipoma, intermuscular lipoma, and lipoma-like liposarcoma. J Orthop Sci, 2007. 12(6): p. 533-41.

    • DiDomenico, P. and W. Middleton, Sonographic evaluation of palpable superficial masses. Radiol Clin North Am, 2014. 52(6): p. 1295-305.

    • Ameloot, E., et al., Update of Pediatric Lipomatous Lesions: A Clinicopathological, Immunohistochemical and Molecular Overview. J Clin Med, 2022. 11(7).

    • Shekhani, H.N., T. Hanna, and J.O. Johnson, Lipofibromatous Hamartoma of the Median Nerve: A Case Report. J Radiol Case Rep, 2016. 10(11): p. 1-7.

    • Al-Jabri, T., S. Garg, and G.V. Mani, Lipofibromatous hamartoma of the median nerve. Journal of Orthopaedic Surgery and Research, 2010. 5(1): p. 71.

    • Prabhu, C.S., et al., Macrodystrophia Lipomatosa: A Single Large Radiological Study of a Rare Entity. J Clin Imaging Sci, 2019. 9: p. 4.

    • Oztürk, A., et al., [Macrodystrophia lipomatosa: a case report]. Acta Orthop Traumatol Turc, 2004. 38(3): 220-3.

    • Sudesh, P., et al., Macrodystrophia lipomatosa. Foot (Edinb), 2012. 22(3): p. 172-4.

    • Mujtaba, B., et al., Myxoid Liposarcoma With Skeletal Metastases: Pathophysiology and Imaging Characteristics. Curr Probl Diagn Radiol, 2021. 50(1): p. 66-73.

    • Encinas Tobajas, V.M., et al., Myxoid liposarcoma: MRI features with histological correlation. Radiologia (Engl Ed), 2023. 65 Suppl 2: p. S23-s32.

    • Huh, W.W., et al., Liposarcoma in children and young adults: a multi-institutional experience. Pediatr Blood Cancer, 2011. 57(7): p. 1142-6.

    • Giri, S., et al., Rare Case of Metastatic Pleomorphic Liposarcoma of Thigh Presenting as Ewing’s Sarcoma. J Nepal Health Res Counc, 2021. 19(1): p. 215-217.

    • Wannapanich, T. and P. Pratipanawat, Primary orbital pleomorphic liposarcoma in a child: A case report. Am J Ophthalmol Case Rep, 2022. 25: p. 101285.

    • Baday, Y.I., et al., Pediatric liposarcoma: A case series and literature review. Pediatr Blood Cancer, 2021. 68(12): p. e29327.

    • Jeong, J., Nodular Fasciitis in the Preauricular Area in Pediatric Patients. Ear Nose Throat J, 2023: p. 1455613231154032.

    • Pandian, T.K., et al., Nodular fasciitis in the pediatric population: a single center experience. J Pediatr Surg, 2013. 48(7): p. 1486-9.

    • Devaney, D., P. Livesley, and D. Shaw, Elastofibroma dorsi: MRI diagnosis in a young girl. Pediatr Radiol, 1995. 25(4): p. 282-3.

    • Bianchi, S., et al., Elastofibroma dorsi: sonographic findings. AJR Am J Roentgenol, 1997. 169(4): p. 1113-5.

    • Chandrasekar, C.R., et al., Elastofibroma dorsi: an uncommon benign pseudotumour. Sarcoma, 2008. 2008: p. 756565.

    • Ateş, F., et al., Magnetic resonance imaging findings of elastofibroma dorsi: A case report. Journal of Surgery and Medicine, 2019. 3(4): p. 341-342.

    • Kager, M., et al., Tenosynovial giant cell tumor. Folia Med Cracov, 2022. 62(2): p. 93-107.

    • He, Q.F., et al., Giant Cell Tumor of Tendon Sheath with Tarsal Bones and Intertarsal Joint Invasion: A Case Report. J Am Podiatr Med Assoc, 2020. 110(3).

    • Otero, S., et al., Desmoid-type fibromatosis. Clin Radiol, 2015. 70(9): p. 1038-45.

    • The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer, 2020. 127: p. 96-107.

    • Nduka, J.C., K. Lam, and C.R. Chandrasekar, Diagnosing plantar fibromas Beware of sarcomas. Foot (Edinb), 2021. 49: p. 101736.

    • McNally, E.G. and S. Shetty, Plantar fascia: imaging diagnosis and guided treatment. Semin Musculoskelet Radiol, 2010. 14(3): p. 334-43.

    • Anwander, H., et al., Recurrence Rate After Wide Resection of Plantar Fibromatosis: A Case Series and Systematic Literature Review. Foot Ankle Spec, 2024. 17(2): p. 109-116.

    • Davis, D.D., S.J. Shah, and S.M. Kane, Fibrosarcoma, in StatPearls. 2024, StatPearls Publishing Copyright © 2024, StatPearls Publishing LLC.: Treasure Island (FL).

    • Cano-Padilla, A., et al., Paediatric fibrosarcoma treatment. Ecancermedicalscience, 2023. 17: p. 1608.

    • Raza, A., et al., A Case of Locally Advanced Fibrosarcoma in a Young Male. Cureus, 2023. 15(8): p. e44095.

    • Brooks, J., G. Menon, and M.L. Ramsey, Dermatofibrosarcoma Protuberans, in StatPearls. 2024, StatPearls Publishing Copyright © 2024, StatPearls Publishing LLC.: Treasure Island (FL).

    • Wickramasinghe, V.C., et al., Dermatofibrosarcoma protuberans in a pre-adolescent child. Int J Surg Case Rep, 2023. 110: p. 108761.

    • Noguchi, T., et al., Retroperitoneal low-grade fibromyxoid sarcoma. IJU Case Rep, 2024. 7(3): p. 255-258.

    • Wang, L., et al., Low-grade Myofibroblastic sarcoma: clinical and imaging findings. BMC Med Imaging, 2019. 19(1): p. 36.

    • Weiler, E.J., et al., A rare case report of pancreatic low-grade fibromyxoid sarcoma (LGFMS). Surg Case Rep, 2023. 9(1): p. 123.

    • Falcone, M.O., G. Asmar, and R. Chassat, Subungual Glomus Tumor. Hand Surg Rehabil, 2023.

    • Dhami, A., et al., Comparing Ultrasound with Magnetic Resonance Imaging in the Evaluation of Subungual Glomus Tumors and Subungual Myxoid Cysts. Skin Appendage Disord, 2023. 9(4): p. 262-267.

    • Ferrari, T.A., et al., Multiple Glomus Tumor and Neurofibromatosis Type 1. Skin Appendage Disord, 2023. 9(5): p. 388-391.

    • Guedes, P.M., et al., Intramuscular Hemangioma: A Rare Cause of Omalgia. Rev Bras Ortop (Sao Paulo), 2023. 58(4): p. e676-e680.

    • Gupta, R., A. Bhandari, and O.M. Navarro, Pediatric Vascular Anomalies: A Clinical and Radiological Perspective. Indian J Radiol Imaging, 2024. 34(1): p. 103-127.

    • Almousa, H.M., et al., Intramuscular Hemangioma of the Sternocleidomastoid: A Rare Tumor in an Unusual Location. Ear Nose Throat J, 2023: p. 1455613231189148.

    • Zhang, L. and J. Wu, Multimodal imaging features of retroperitoneal anastomosing hemangioma: a case report and literature review. Front Oncol, 2023. 13: p. 1269631.

    • Mamlouk, M.D., Solid and Vascular Neck Masses in Children. Neuroimaging Clin N Am, 2023. 33(4): p. 607-621.

    • Naganathan, S. and P. Tadi, Klippel-Trenaunay-Weber Syndrome, in StatPearls. 2024, StatPearls Publishing Copyright © 2024, StatPearls Publishing LLC.: Treasure Island (FL).

    • Wang, Y.P., et al., A rare presentation of Maffucci syndrome: A case report and literature review. Exp Ther Med, 2023. 26(3): p. 435.

    • Lewis, D. and R. Vaidya, Kasabach-Merritt Syndrome, in StatPearls. 2024, StatPearls Publishing Copyright © 2024, StatPearls Publishing LLC.: Treasure Island (FL).

    • Daigle, H.J., I.A. Sakharuk, and R.M. Hatley, Venolymphatic Malformation : An Uncommon Pediatric Inguinal Mass. Am Surg, 2021. 87(1): p. 152-153.

    • Reis, J., 3rd, et al., Ultrasound Evaluation of Pediatric Slow-Flow Vascular Malformations: Practical Diagnostic Reporting to Guide Interventional Management. AJR Am J Roentgenol, 2021. 216(2): p. 494-506.

    • Dhagat, P.K., M. Jain, and A. Farooq, MRI evaluation of soft tissue vascular malformations. Indian J Radiol Imaging, 2020. 30(2): p. 184-189.

    • Zhang, Z., et al., Paediatric mesenteric cystic lymphangioma with eggshell calcification. Br J Hosp Med (Lond), 2023. 84(10): p. 1.

    • Nguyen, K., et al., Update on MR Imaging of cystic retroperitoneal masses. Abdom Radiol (NY), 2020. 45(10): p. 3172-3183.

    • Hindman, N., et al., MRI Evaluation of Uterine Masses for Risk of Leiomyosarcoma: A Consensus Statement. Radiology, 2023. 306(2): p. e211658.

    • Sun, S., et al., How to differentiate uterine leiomyosarcoma from leiomyoma with imaging. Diagn Interv Imaging, 2019. 100(10): p. 619-634.

    • Yoshizawa, K., et al., Primary leiomyosarcoma of the inferior vena cava in a pediatric case: a case report and literature review. Surg Case Rep, 2023. 9(1): p. 52.

    • Husain, M.A., J. Shahangian, and A. Dovigi, Primary Leiomyosarcoma of the Mandible in a Child. J Dent Child (Chic), 2020. 87(3): p. 171-174.

    • Makhoul, K., et al., Leiomyosarcoma: Lung Metastasis. Cureus, 2023. 15(1): p. e34373.

    • Andrade, C.R., et al., Rabdomyosarcoma of the Mandible: An Uncommon Clinical Presentation. J Craniofac Surg, 2018. 29(3): p. e221-e224.

    • de Vries, I.S.A., et al., Imaging in rhabdomyosarcoma: a patient journey. Pediatr Radiol, 2023. 53(4): p. 788-812.

    • Jawad, N. and K. McHugh, The clinical and radiologic features of paediatric rhabdomyosarcoma. Pediatr Radiol, 2019. 49(11): p. 1516-1523.

    • Weissferdt, A. and C.A. Moran, Epithelioid hemangioendothelioma of the bone: a review and update. Adv Anat Pathol, 2014. 21(4): p. 254-9.

    • Jang, J.K., et al., A Review of the Spectrum of Imaging Manifestations of Epithelioid Hemangioendothelioma. AJR Am J Roentgenol, 2020. 215(5): p. 1290-1298.

    • Orbach, D., et al., Epithelioid hemangioendothelioma in children: The European Pediatric Soft Tissue Sarcoma Study Group experience. Pediatr Blood Cancer, 2022. 69(10): p. e29882.

    • El-Sharkawy, F., et al., Angiosarcoma of the Pelvis in a 13-Year-Old Girl. Pediatr Dev Pathol, 2017. 20(2): p. 163-167.

    • Gaballah, A.H., et al., Angiosarcoma: clinical and imaging features from head to toe. Br J Radiol, 2017. 90(1075): p. 20170039.

    • Gazendam, A.M., et al., Synovial Sarcoma: A Clinical Review. Curr Oncol, 2021. 28(3): p. 1909-1920.

    • Cho, E.B., et al., Synovial Sarcoma in the Extremity: Diversity of Imaging Features for Diagnosis and Prognosis. Cancers (Basel), 2023. 15(19).

    • Harjpal, P.L. and R.K. Kovela, Classical neurofibroma. Pan Afr Med J, 2021. 39: p. 146.

    • Fisher, M.J., et al., Management of neurofibromatosis type 1-associated plexiform neurofibromas. Neuro Oncol, 2022. 24(11): p. 1827-1844.

    • Bakshi, S.S., Plexiform neurofibroma. Cleve Clin J Med, 2016. 83(11): p. 792.

    • Garner, H.W., et al., Epithelioid schwannoma: imaging findings on radiographs, MRI, and ultrasound. Skeletal Radiol, 2019. 48(11): p. 1815-1820.

    • Hao, X., et al., Schwannoma of Foot and Ankle: Seven Case Reports and Literature Review. Anticancer Res, 2019. 39(9): p. 5185-5194.

    • Stacy, G.S., et al., Soft-Tissue Tumors of the Hand-Imaging Features. Can Assoc Radiol J, 2020. 71(2): p. 161-173.

    • Llanos, D., et al., Imaging palpable tumors in pediatrics. Radiologia (Engl Ed), 2022. 64(6): p. 552-565.

    • Patmano, M., et al., Primary Soft Tissue Hydatid Cysts. Turkiye Parazitol Derg, 2022. 46(2): p. 145-149.

    • Orhan, Ö., et al., Don’t miss it: Extremity-located cyst hydatid may mimic soft tissue tumors. Jt Dis Relat Surg, 2023. 34(3): p. 687-693.

    Share This Chapter!