Clinical Characteristics and Outcome of Infantile Fibrosarcoma: A Retrospective Single-institution Review

Enas El Nadi; Mona Moustafa; Gehad Ahmed; Alaa Younes; Mohamed Saad Zaghloul; Naglaa ElKinaai; Amal Rafaat; Elham Khaled; Sayed Abdel Hamid

doi:doi:10.11648/j.crj.20200802.12

Clinical Characteristics and Outcome of Infantile Fibrosarcoma: A Retrospective Single-institution Review

Enas El Nadi, Mona Moustafa, Gehad Ahmed, Alaa Younes, Mohamed Saad Zaghloul, Naglaa ElKinaai, Amal Rafaat, Elham Khaled, Sayed Abdel Hamid

Published in Cancer Research Journal (Volume 8, Issue 2)

Received: 18 March 2020 Accepted: 9 April 2020 Published: 28 April 2020

Views: Downloads:

Download PDF

Share This Article

Twitter
Linked In
Facebook

Abstract

Analysis was done to the clinical characteristics and outcome of children with infantile Fibrosarcoma (IFS) who were treated at Children’s Cancer Hospital Egypt (CCHE). A Retrospective chart review of all pathologically confirmed IFS patients treated at CCHE from July 2011 till December 2016. Detailed demographic, pathological, and clinical data of 27 IFS patients were collected, analyzed and correlated with treatment outcome and survival. We enrolled 27 patients with a median age 0.23 years (range 0.03-3.8), 54% were females. Extremities were the commonest primary site (59.3%), followed by head and neck (22.2%). The tumor size was >5cm in 18 patients, <5cm in six and undetermined in three. It was resectable without preoperative chemotherapy in three patients. Twenty one received preoperative VAC (Vincristine, Actinomycin-D and Cyclophosphamide) of those nine cases received second line Ifosphamide and Adriamycin to improve respectability. Overall survival of the whole group of patients was 82.3%. We need to increase sample size and to get facility for proper diagnosis by genetic analysis in such difficult cases for better pathological diagnosis

Published in	Cancer Research Journal (Volume 8, Issue 2)
DOI	10.11648/j.crj.20200802.12
Page(s)	26-35
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2020. Published by Science Publishing Group

Keywords

Infantile Fibrosarcoma, Risk Factors, Survival

References

[1]	Gurney JG, Young JL, Roffers SD, Smith MA, Bunin GR. Soft tissue sarcomas. In: Gloecker Ries IA, Smith MA, Gurney JG, Linet M, Tamra T, Young JL, Bunin GR, editors. SEER pediatric monograph: cancer incidence and survival among children and adolescents, United States SEER program 1975–1995. National Cancer Institute; Bethesda: 1999. pp. 111–124.
[2]	Sultan I, Casanova M, Al-Jumaily U, Meazza C, Rodriguez-Galindo C, Ferrari A. Soft tissue sarcomas in the first year of life. Eur J Cancer. 2010; 46: 2449–2456. [PubMed].
[3]	Cecchetto G, Carli M, Alaggio R, Dall’Igna P, Bisogno G, Scarzello G, Zanetti I, Durante G, Inserra A, Siracusa F, Guglielmi M Italian Cooperative Group. Fibrosarcoma in pediatric patients: results of the Italian cooperative group studies (1979–1995) J Surg Oncol. 2001; 78: 225–231. [PubMed].
[4]	Ferguson WS. Advances in the adjuvant treatment of infantile fibrosarcoma. Expert Rev Anticancer Ther. 2003; 3: 185–191. [PubMed].
[5]	Frieden IJ, Rogers M, Garzon MC. Conditions masquerading as infantile haemangioma: part 2. Australas J Dermatol. 2009; 50: 153–168. [PubMed].
[6]	Adibe OO, Juang D, Valusek PA, Holcomb GW, 3rd Snyder CL. Infantile fibrosarcoma: two case reports and literature review. Eur J Pediatr Surg. 2011; 21: 200–202. [PubMed].
[7]	Exelby PR, Knapper WH, Huvos AG, Beattie EJ. Soft-tissue fibrosarcoma in children. J Pediatr Surg. 1973; 8: 415–420. [PubMed].
[8]	Sivasankar Jayakumar, Sanjana Venkateswaran, Sathish Manivel, Balamurali G Paraspinal Congenital Infantile Fibrosarcoma: A Case Report ournal of Neonatal Surgery 2019; 8: 13.
[9]	Veronica Samedi, Jumana Samara, Ipsita Roy Goswami, Essa Hamdan Al Awad, Rare Presenation of Infantile Fibrosarcoma, Conference: 4th International Neonatology Association ConferenceAt: Ghent, Belgium, Volume: INAC, June 2018, DOI: 10.13140/RG.2.2.36714.16323
[10]	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: 133–150. [PubMed].
[11]	Soule EH, Pritchard DJ. Fibrosarcoma in infants and children. A review of 110 cases. Cancer 1977; 40: 1711–1721. 2.
[12]	Chung EB, Enzinger FM. Infantile fibrosarcoma. Cancer 1976; 38: 729–739.
[13]	Kynaston JA, Malcolm AJ, Craft AW, et al. Chemotherapy in the management of infantile fibrosarcoma. Med Pediatr Oncol 1993; 21: 488–493.
[14]	Grier HE, Perez-Atayde AR, Weinstein HJ. Chemotherapy for inoperable infantile fibrosarcoma. Cancer 1985; 56: 1507–1510.
[15]	Ninane J, Grosseye S, Panteon E, et al. Congenital fibrosarcoma. Preoperative chemotherapy and conservative surgery. Cancer 1986; 58: 1400–1406.
[16]	Robinson W, Crawford AH. Infantile fibrosarcoma. Report of a case with long-term follow-up. J Bone Joint Surg 1990; 72-A: 291– 294.
[17]	Vinnicombe SJ, Hall CM. Infantile fibrosarcoma: radiological and clinical features. Skel Radiol 1994; 23: 337–341.
[18]	Brock P, Renard M, Smet M, et al. Infantile fibrosarcoma. Med Pediatr Oncol 1991; 19: 210.
[19]	Blocker S, Koenig J, Ternberg J. Congenital fibrosarcoma. J Pediatr Surg. 1987; 22: 665–670. [PubMed].
[20]	Tröbs R, Meier T, Bennek J, Heinrich S, Willnow U. Fibrosarcoma in infants and children: a retrospective analysis—overdiagnosis in earlier years. Pediatr Surg Int. 1999; 15: 123–128. [PubMed].
[21]	Loh ML, Ahn P, Perez-Atayde AR, Gebhardt MC, Shamberger RC, Grier HE. Treatment of infantile fibrosarcoma with chemotherapy and surgery: results from the Dana-Farber Cancer Institute and Children’s Hospital, Boston. J Pediatr Hematol Oncol. 2002; 24: 722–726. [PubMed].
[22]	Orbach D, Rey A, Cecchetto G, Oberlin O, Casanova M, Thebaud E, Scopinaro M, Bisogno G, Carli M, Ferrari A. Infantile fibrosarcoma: management based on the European experience. J. Clin Oncol. 2010; 28: 318–323. [PubMed].
[23]	Daniel Orbach a, *, Bernadette Brennan b, Angela De Paoli c, Soledad Gallego d, Peter Mudry e, Nadine Francotte f, Max van Noesel g, Anna Kelsey h, Rita Alaggio i, Dominique Ranche`re j, Gian Luca De Salvo c, Michela Casanova k, Christophe Bergeron l, Johannes H. M. Merks m, Meriel Jenney n, Michael C. G. Stevens o, Gianni Bisogno p, Andrea Ferrari Conservative strategy in infantile fibrosarcoma is possible: The European paediatric Soft tissue sarcoma Study Group experience, European Journal of Cancer 57 (2016) 1e9.
[24]	Kurkchubasche AG, Halvorson EG, Forman EN, Terek RM, Ferguson WS. The role of preoperative chemotherapy in the treatment of infantile fibrosarcoma. J Pediatr Surg. 2000; 35: 880–883. [PubMed].
[25]	Spunt SL, Poquette CA, Hurt YS, Cain AM, Rao BN, Merchant TE, Jenkins JJ, Santana VM, Pratt CB, Pappo AS. Prognostic factors for children and adolescents with surgically resected non-rhabdomyosarcoma soft tissue sarcoma: an analysis of 121 patients treated at St Jude Children’s Research Hospital. J Clin Oncol. 1999; 17: 3697–3705. [PubMed].
[26]	Lalit Parida, Israel Fernandez-Pineda, John K. Uffman, Andrew M. Davidoff, Matthew J. Krasin, Alberto Pappo, and Bhaskar N. Rao. Clinical management of infantile fibrosarcoma: a retrospective single-institution review. Pediatr Surg Int. 2013 Jul; 29 (7): 703–708.
[27]	Russell H, Hicks MJ, Bertuch AA, Chintagumpala M. Infantile fibrosarcoma: clinical and histologic responses to cytotoxic chemotherapy. Pediatr Blood Cancer. 2009; 53: 23–27. [PubMed].
[28]	Yan AC, Chamlin SL, Liang MG, Hoffman B, Attiyeh EF, Chang B, Honig PJ. Congenital infantile fibrosarcoma: a masquerader of ulcerated hemangioma. Pediatr Dermatol. 2006; 23: 330–334. [PubMed].
[29]	Pousti TJ, Upton J, Loh M, Grier H. Congenital fibrosarcoma of the upper extremity. Plast Reconstr Surg. 1998; 102: 1158–1162. [PubMed].
[30]	Cofer BR, Vescio PJ, Wiener ES. Infantile fibrosarcoma: complete excision is the appropriate treatment. Ann Surg Oncol. 1996; 3: 159–161. [PubMed].
[31]	Hays DM, Mirabal VQ, Karlan MS, Patel HR, Landing BH. Fibrosarcomas in infants and children. J Pediatr Surg. 1970; 5: 176–183. [PubMed].
[32]	DeComas AM, Heinrich SD, Craver R. Infantile fibrosarcoma successfully treated with chemotherapy, with occurrence of calcifying aponeurotic fibroma and pleomorphic/spindled celled lipoma at the site 12 years later. J Pediatr Hematol Oncol. 2009; 31: 448–452. [PubMed].
[33]	Congenital Infantile Fibrosarcoma Causing Intestinal Perforation in a Newborn, Margarita Kaiser, Bernadette Liegl-Atzwanger, EszterNagy, DanielaSperl, Georg Singer, and Holger Till, Hindawi Case Reports in Pediatrics Volume 2017, Article ID 2969473, 3 pages https://doi.org/10.1155/2017/2969473.
[34]	Assessing the prevalence of a novel oncogene, EML4-NTRK3 https://www.briarcliffschools.org/.../Portfolios_2015_Seniors/jordana_cohen_paper.pdf (Hashemi, et al., 2013).
[35]	AC Yan, SL Chamlin, MG Liang, etal: Congenital infantile fibrosarcoma: A masquerader of ulcerated hemangioma Pediatr Dermatol 23: 330– 334, 2006 Crossref, Medline, Google Scholar.
[36]	Enos T, Hosler GA, Uddin N, Mir A, Congenital infantile fibrosarcoma mimicking a cutaneous vascular lesion: a case report and review of the literature J Cutan Pathol. 2017 Feb; 44 (2): 193-200. doi: 10.1111/cup.12854. Epub 2016 Dec 2.
[37]	Jonathan Bender, Bailey Anderson, David A. Bloom, Raja Rabah, Rhonda McDougall, Pankaj Vats and Rajen Mody. Refractory and metastatic infantile fibrosarcoma harboring LMNA–NTRK1 fusion shows complete and durable response to crizotinib. Journal of precision medicine, April, 2020, (6) 2: doi: 10.1101/mcs.a003376Cold Spring Harb Mol Case Stud 5: a003376

Cite This Article

Plain Text BibTeX RIS

APA Style

Enas El Nadi, Mona Moustafa, Gehad Ahmed, Alaa Younes, Mohamed Saad Zaghloul, et al. (2020). Clinical Characteristics and Outcome of Infantile Fibrosarcoma: A Retrospective Single-institution Review. Cancer Research Journal, 8(2), 26-35. https://doi.org/10.11648/j.crj.20200802.12

Copy | Download

ACS Style

Enas El Nadi; Mona Moustafa; Gehad Ahmed; Alaa Younes; Mohamed Saad Zaghloul, et al. Clinical Characteristics and Outcome of Infantile Fibrosarcoma: A Retrospective Single-institution Review. Cancer Res. J. 2020, 8(2), 26-35. doi: 10.11648/j.crj.20200802.12

Copy | Download

AMA Style

Enas El Nadi, Mona Moustafa, Gehad Ahmed, Alaa Younes, Mohamed Saad Zaghloul, et al. Clinical Characteristics and Outcome of Infantile Fibrosarcoma: A Retrospective Single-institution Review. Cancer Res J. 2020;8(2):26-35. doi: 10.11648/j.crj.20200802.12

Copy | Download

@article{10.11648/j.crj.20200802.12,
  author = {Enas El Nadi and Mona Moustafa and Gehad Ahmed and Alaa Younes and Mohamed Saad Zaghloul and Naglaa ElKinaai and Amal Rafaat and Elham Khaled and Sayed Abdel Hamid},
  title = {Clinical Characteristics and Outcome of Infantile Fibrosarcoma: A Retrospective Single-institution Review},
  journal = {Cancer Research Journal},
  volume = {8},
  number = {2},
  pages = {26-35},
  doi = {10.11648/j.crj.20200802.12},
  url = {https://doi.org/10.11648/j.crj.20200802.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.crj.20200802.12},
  abstract = {Analysis was done to the clinical characteristics and outcome of children with infantile Fibrosarcoma (IFS) who were treated at Children’s Cancer Hospital Egypt (CCHE). A Retrospective chart review of all pathologically confirmed IFS patients treated at CCHE from July 2011 till December 2016. Detailed demographic, pathological, and clinical data of 27 IFS patients were collected, analyzed and correlated with treatment outcome and survival. We enrolled 27 patients with a median age 0.23 years (range 0.03-3.8), 54% were females. Extremities were the commonest primary site (59.3%), followed by head and neck (22.2%). The tumor size was >5cm in 18 patients, <5cm in six and undetermined in three. It was resectable without preoperative chemotherapy in three patients. Twenty one received preoperative VAC (Vincristine, Actinomycin-D and Cyclophosphamide) of those nine cases received second line Ifosphamide and Adriamycin to improve respectability. Overall survival of the whole group of patients was 82.3%. We need to increase sample size and to get facility for proper diagnosis by genetic analysis in such difficult cases for better pathological diagnosis},
 year = {2020}
}

Copy | Download

TY  - JOUR
T1  - Clinical Characteristics and Outcome of Infantile Fibrosarcoma: A Retrospective Single-institution Review
AU  - Enas El Nadi
AU  - Mona Moustafa
AU  - Gehad Ahmed
AU  - Alaa Younes
AU  - Mohamed Saad Zaghloul
AU  - Naglaa ElKinaai
AU  - Amal Rafaat
AU  - Elham Khaled
AU  - Sayed Abdel Hamid
Y1  - 2020/04/28
PY  - 2020
N1  - https://doi.org/10.11648/j.crj.20200802.12
DO  - 10.11648/j.crj.20200802.12
T2  - Cancer Research Journal
JF  - Cancer Research Journal
JO  - Cancer Research Journal
SP  - 26
EP  - 35
PB  - Science Publishing Group
SN  - 2330-8214
UR  - https://doi.org/10.11648/j.crj.20200802.12
AB  - Analysis was done to the clinical characteristics and outcome of children with infantile Fibrosarcoma (IFS) who were treated at Children’s Cancer Hospital Egypt (CCHE). A Retrospective chart review of all pathologically confirmed IFS patients treated at CCHE from July 2011 till December 2016. Detailed demographic, pathological, and clinical data of 27 IFS patients were collected, analyzed and correlated with treatment outcome and survival. We enrolled 27 patients with a median age 0.23 years (range 0.03-3.8), 54% were females. Extremities were the commonest primary site (59.3%), followed by head and neck (22.2%). The tumor size was >5cm in 18 patients, <5cm in six and undetermined in three. It was resectable without preoperative chemotherapy in three patients. Twenty one received preoperative VAC (Vincristine, Actinomycin-D and Cyclophosphamide) of those nine cases received second line Ifosphamide and Adriamycin to improve respectability. Overall survival of the whole group of patients was 82.3%. We need to increase sample size and to get facility for proper diagnosis by genetic analysis in such difficult cases for better pathological diagnosis
VL  - 8
IS  - 2
ER  -

Copy | Download

Author Information

Enas El Nadi

Department of Clinical Oncology, Faculty of Medicine, Beni - Suef University, Beni-Suef, Egypt
Mona Moustafa

Department of Pediatric Oncology, Children Cancer Hospital of Egypt (CCHE) Cairo, Egypt
Gehad Ahmed

Department of Surgery, Faculty of Medicine, Helwan University, Helwan, Egypt
Alaa Younes

Department of Surgical Oncology, Children Cancer Hospital of Egypt (CCHE) Cairo, Egypt
Mohamed Saad Zaghloul

Department of Radiotherapy, National Cancer Institute, Cairo University, Cairo, Egypt
Naglaa ElKinaai

Department of Surgical Pathology, National Cancer Institute, Cairo University, Cairo, Egypt
Amal Rafaat

Department of Radiodiagnosis, National Cancer Institute, Cairo University, Cairo, Egypt
Elham Khaled

Department of Clinical Research Children Cancer Hospital of Egypt (CCHE), Cairo, Egypt
Sayed Abdel Hamid

Department of Pediatric Oncology, Children Cancer Hospital of Egypt (CCHE) Cairo, Egypt

Download PDF

Sections

Plain Text BibTeX RIS

APA Style

Enas El Nadi, Mona Moustafa, Gehad Ahmed, Alaa Younes, Mohamed Saad Zaghloul, et al. (2020). Clinical Characteristics and Outcome of Infantile Fibrosarcoma: A Retrospective Single-institution Review. Cancer Research Journal, 8(2), 26-35. https://doi.org/10.11648/j.crj.20200802.12

Copy | Download

ACS Style

Enas El Nadi; Mona Moustafa; Gehad Ahmed; Alaa Younes; Mohamed Saad Zaghloul, et al. Clinical Characteristics and Outcome of Infantile Fibrosarcoma: A Retrospective Single-institution Review. Cancer Res. J. 2020, 8(2), 26-35. doi: 10.11648/j.crj.20200802.12

Copy | Download

AMA Style

Enas El Nadi, Mona Moustafa, Gehad Ahmed, Alaa Younes, Mohamed Saad Zaghloul, et al. Clinical Characteristics and Outcome of Infantile Fibrosarcoma: A Retrospective Single-institution Review. Cancer Res J. 2020;8(2):26-35. doi: 10.11648/j.crj.20200802.12

Copy | Download

@article{10.11648/j.crj.20200802.12,
  author = {Enas El Nadi and Mona Moustafa and Gehad Ahmed and Alaa Younes and Mohamed Saad Zaghloul and Naglaa ElKinaai and Amal Rafaat and Elham Khaled and Sayed Abdel Hamid},
  title = {Clinical Characteristics and Outcome of Infantile Fibrosarcoma: A Retrospective Single-institution Review},
  journal = {Cancer Research Journal},
  volume = {8},
  number = {2},
  pages = {26-35},
  doi = {10.11648/j.crj.20200802.12},
  url = {https://doi.org/10.11648/j.crj.20200802.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.crj.20200802.12},
  abstract = {Analysis was done to the clinical characteristics and outcome of children with infantile Fibrosarcoma (IFS) who were treated at Children’s Cancer Hospital Egypt (CCHE). A Retrospective chart review of all pathologically confirmed IFS patients treated at CCHE from July 2011 till December 2016. Detailed demographic, pathological, and clinical data of 27 IFS patients were collected, analyzed and correlated with treatment outcome and survival. We enrolled 27 patients with a median age 0.23 years (range 0.03-3.8), 54% were females. Extremities were the commonest primary site (59.3%), followed by head and neck (22.2%). The tumor size was >5cm in 18 patients, <5cm in six and undetermined in three. It was resectable without preoperative chemotherapy in three patients. Twenty one received preoperative VAC (Vincristine, Actinomycin-D and Cyclophosphamide) of those nine cases received second line Ifosphamide and Adriamycin to improve respectability. Overall survival of the whole group of patients was 82.3%. We need to increase sample size and to get facility for proper diagnosis by genetic analysis in such difficult cases for better pathological diagnosis},
 year = {2020}
}

Copy | Download

TY  - JOUR
T1  - Clinical Characteristics and Outcome of Infantile Fibrosarcoma: A Retrospective Single-institution Review
AU  - Enas El Nadi
AU  - Mona Moustafa
AU  - Gehad Ahmed
AU  - Alaa Younes
AU  - Mohamed Saad Zaghloul
AU  - Naglaa ElKinaai
AU  - Amal Rafaat
AU  - Elham Khaled
AU  - Sayed Abdel Hamid
Y1  - 2020/04/28
PY  - 2020
N1  - https://doi.org/10.11648/j.crj.20200802.12
DO  - 10.11648/j.crj.20200802.12
T2  - Cancer Research Journal
JF  - Cancer Research Journal
JO  - Cancer Research Journal
SP  - 26
EP  - 35
PB  - Science Publishing Group
SN  - 2330-8214
UR  - https://doi.org/10.11648/j.crj.20200802.12
AB  - Analysis was done to the clinical characteristics and outcome of children with infantile Fibrosarcoma (IFS) who were treated at Children’s Cancer Hospital Egypt (CCHE). A Retrospective chart review of all pathologically confirmed IFS patients treated at CCHE from July 2011 till December 2016. Detailed demographic, pathological, and clinical data of 27 IFS patients were collected, analyzed and correlated with treatment outcome and survival. We enrolled 27 patients with a median age 0.23 years (range 0.03-3.8), 54% were females. Extremities were the commonest primary site (59.3%), followed by head and neck (22.2%). The tumor size was >5cm in 18 patients, <5cm in six and undetermined in three. It was resectable without preoperative chemotherapy in three patients. Twenty one received preoperative VAC (Vincristine, Actinomycin-D and Cyclophosphamide) of those nine cases received second line Ifosphamide and Adriamycin to improve respectability. Overall survival of the whole group of patients was 82.3%. We need to increase sample size and to get facility for proper diagnosis by genetic analysis in such difficult cases for better pathological diagnosis
VL  - 8
IS  - 2
ER  -

Copy | Download