Autopsy and Case Reports
Autopsy and Case Reports
Clinical Case Report and Review

Cervical Infantile Fibrosarcoma: a rare cause of paediatric parapharyngeal neck mass

Madhu Priya; Parvendra Singh; Manu Malhotra; Sumeet Angral; Saurabh Varshney; Abhishek Bhardwaj; Amit Kumar Tyagi; Amit Kumar; Manish Kumar Gupta

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ABSTRACT: Soft tissue tumors are not uncommon in childhood and comprise entities that range from common to very rare malignancies. Infantile fibrosarcoma (IFS) is a rare pediatric malignancy mainly seen in the first two years of life. The data about the incidence of infantile fibrosarcoma occurring in the neck in the Indian subcontinent is scarce. To the best of our knowledge, only one case of infant cervical IFS has been reported previously in the Indian subcontinent. We present another case of an eight-year-old male patient with a rapidly growing mass on the left side of the neck. He was successfully treated with a combined modality of surgery and chemotherapy with a good outcome. Among the soft tissue tumors of childhood, IFS is a rare entity. It has a good prognosis and lesser chance of distant metastasis as compared to adult fibrosarcoma. Though surgical excision is the mainstay of treatment, chemotherapy also has a significant role in the treatment of primary tumor and metastasis. We discuss the stated case to bring to the notice this uncommon cause, which can be considered as a differential diagnosis of upper cervical swellings. A better understanding of this entity would help in early diagnosis and aggressive treatment, reducing the overall morbidity and mortality.


Fibrosarcoma, Rhabdomyosarcoma, Soft Tissue Tumor


1 Lyos AT, Goepfert H, Luna MA, Jaffe N, Malpica A. Soft tissue sarcoma of the head and neck in children and adolescents. Cancer. 1996;77(1):193-200.<193::AID-CNCR31>3.0.CO;2-Z. PMid:8630930.

2 Carroll SJ, Nodit L. Spindle cell rhabdomyosarcoma: A brief diagnostic review and differential diagnosis. Arch Pathol Lab Med. 2013;137(8):1155-8. PMid:23899074.

3 Hashemi A, Tefagh S, Seifadini A, Moghimi M. Infantile fibrosarcoma in a child: A case report. Iran J Ped Hematol Oncol. 2013;3(3):135-7. PMid:24575285.

4 Coffin CM, Jaszcz W, O’Shea PA, Dehner LP. So-called congenital-infantile fibrosarcoma: does it exist and what is it? Pediatr Pathol. 1994;14(1):133-50. PMid:8159611.

5 Ries L, Smith M, Gurney J, et al. Cancer incidence and survival among children and adolescents: United States SEER program 1975-1995. Bethesda, MD: National Cancer Institute, SEER Program; 1999. (NIH Pub. No. 99-4649).

6 Weihkopf T, Blettner M, Dantonello T, et al. Incidence and time trends of soft tissue sarcomas In German children 1985-2004- A report from the population based German childhood cancer registry. Eur J Cancer. 2008;44(3):432-40. PMid:18077150.

7 Soule EH, Pritchard DJ. Fibrosarcoma in infants and children: A review of 110 cases. Cancer. 1977;40(4):1711-21.<1711::AID-CNCR2820400447>3.0.CO;2-9. PMid:561651.

8 Cofer BR, Vescio PJ, Wiener ES. Infantile fibrosarcoma: complete excision is the appropriate treatment. Ann Surg Oncol. 1996;3(2):159-61. PMid:8646516.

9 Gülhan B, Kupeli S, Yalcin B, Akyuz C, Buyukpamukcu M. An unusual presentation of infantile fibrosarcoma in a male newborn. Am J Perinatol. 2009;26(5):331-3. PMid:19067284.

10 Schmidt H, Taubert H, Wurl P, et al. Gains of 12q are the most frequent genomic imbalances in adult fibrosarcoma and are correlated with a poor outcome. Genes Chromosomes Cancer. 2002;34(1):69-77. PMid:11921284.

11 Sait SF, Danzer E, Ramirez D, LaQuaglia MP, Paul M. Spontaneous regression in a patient with infantile fibrosarcoma. J Pediatr Hematol Oncol. 2018;40(4):253-5. PMid:29200168.

12 Gupta A, Sharma S, Mathur S, Yadav DK, Gupta DK. Cervical congenital infantile fibrosarcoma: a case report. J Med Case Reports. 2019;13(1):41. PMid:30797242.

13 Fletcher CDM, Unni KK, Mertens F, editors. World Health Organization Classification of Tumors. Pathology and genetics of tumors of soft tissue and bone. IARC Press: Lyon 2002.

14 Ferrari A, Orbach D, Sultan I, Casanova M, Bisogno G. Neonatal soft tissue sarcomas. Semin Fetal Neonatal Med. 2012;17(4):231-8. PMid:22633289.

15 Brisse HJ, Orbach D, Klijanienko J. Soft tissue tumors: imaging strategy. Pediatr Radiol. 2010;40(6):1019-28. PMid:20432021.

16 Yan AC, Chamlin SL, Liang MG, et al. Congenital infantile fibrosarcoma: a masquerader of ulcerated hemangioma. Pediatr Dermatol. 2006;23(4):330-4. PMid:16918626.

17 Knezevich SR, Garnett MJ, Pysher TJ, Beckwith JB, Grundy PE, Sorensen PH. ETV6-NTRK3 gene fusions and trisomy 11 establish a histogenetic link between mesoblastic nephroma and congenital fibrosarcoma. Cancer Res. 1998;58(22):5046-8. PMid:9823307.

18 Bourgeois JM, Knezevich SR, Mathers JA, Sorensen PH. Molecular detection of the ETV6-NTRK3 gene fusion differentiates congenital fibrosarcoma from other childhood spindle cell tumors. Am J Surg Pathol. 2000;24(7):937-46. PMid:10895816.

19 Gadd S, Beezhold P, Jennings L, et al. Mediators of receptor tyrosine kinase activation in infantile fibrosarcoma: A Children’s Oncology Group study. J Pathol. 2012;228(1):119-30. PMid:22374738.

20 Orbach D, Rey A, Cecchetto G, et al. Infantile fibrosarcoma: management based on the European experience. J Clin Oncol. 2010;28(2):318-23. PMid:19917847.

21 Ferrari A, Casanova M. New concepts for the treatment of pediatric nonrhabdomyosarcoma soft tissue sarcomas. Expert Rev Anticancer Ther. 2005;5(2):307-18. PMid:15877527.

22 Nagasubramanian R, Wei J, Gordon P, Rastatter JC, Cox MC, Pappo A. Infantile fibrosarcoma with NTRK3-ETV6 fusion successfully treated with the tropomyosin-related kinase inhibitor LOXO-101. Pediatr Blood Cancer. 2016;63(8):1468-70. PMid:27093299.

23 Chug EB, Enzinger FM. Infantile fibrosarcoma. J Indian Med Assoc. 1997;95(6):198. PMid:9420407.

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