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Journal of Hematology & Oncology
Published in: Journal of Hematology & Oncology 1/2008

Open Access 01-12-2008 | Case report

Aggressive juvenile fibromatosis of the paranasal sinuses: case report and brief review

Authors: Shaheen E Lakhan, Robert M Eager, Lindsey Harle

Published in: Journal of Hematology & Oncology | Issue 1/2008

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Abstract

Desmoid fibromatoses are benign, slow growing fibroblastic neoplasms, arising from musculoaponeurotic stromal elements. Desmoids are characterized by local invasion, with a high rate of local recurrence and a tendency to destroy adjacent structures and organs. Desmoid fibromatoses are rare in children, and though they may occur in the head and neck region, are extremely rare in the paranasal sinuses. Here we report a case of extraabdominal desmoid fibromatosis in a seven-year-old boy involving the sphenoid sinus, one of only six published reports of desmoid fibromatosis of the paranasal sinuses. The expansile soft tissue mass eroded the walls of the sphenoid sinus as well as the posterior ethmoid air cells extending cephalad through the base of the skull. We discuss the clinicopathologic features of this lesion, including structural and ultrastructural characteristics, and we review the literature regarding treatment and outcome.
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Literature
1.
Mannan AA, Ray R, Sharma SC, Hatimota P: Infantile fibromatosis of the nose and paranasal sinuses: report of a rare case and brief review of the literature. Ear Nose Throat J. 2004, 83 (7): 481-484.PubMed
2.
Conley J, Healey WV, Stout AP: Fibromatosis of the head and neck. Am J Surg. 1966, 112 (4): 609-614. 10.1016/0002-9610(66)90330-8.CrossRefPubMed
3.
Fu YS, Perzin KH: Nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx. A clinicopathologic study. VI. Fibrous tissue tumors (fibroma, fibromatosis, fibrosarcoma). Cancer. 1976, 37 (6): 2912-2928. 10.1002/1097-0142(197606)37:6<2912::AID-CNCR2820370649>3.0.CO;2-L.CrossRefPubMed
4.
Maillard AA, Kountakis SE: Pediatric sino-orbital desmoid fibromatosis. Ann Otol Rhinol Laryngol. 1996, 105 (6): 463-466.CrossRefPubMed
5.
Naidu RK, Aviv JE, Lawson W, Biller HF: Aggressive juvenile fibromatosis involving the paranasal sinuses. Otolaryngol Head Neck Surg. 1991, 104 (4): 549-552.PubMed
6.
Thompson DH, Khan A, Gonzalez C, Auclair P: Juvenile aggressive fibromatosis: report of three cases and review of the literature. Ear Nose Throat J. 1991, 70 (7): 462-468.PubMed
7.
Reitamo JJ, Hayry P, Nykyri E, Saxen E: The desmoid tumor. I. Incidence, sex-, age- and anatomical distribution in the Finnish population. Am J Clin Pathol. 1982, 77 (6): 665-673.PubMed
8.
Enzinger FM, Weiss SW: Soft tissue tumors. 1995, St. Louis: Mosby, 3
9.
Hansmann A, Adolph C, Vogel T, Unger A, Moeslein G: High-dose tamoxifen and sulindac as first-line treatment for desmoid tumors. Cancer. 100 (3): 612-620. 10.1002/cncr.11937. Feb 1 2004
10.
De Wever I, Dal Cin P, Fletcher CD, Mandahl N, Mertens F, Mitelman F, Rosai J, Rydholm A, Sciot R, Tallini G, Berghe Van Den H, Vanni R, Willén H: Cytogenetic, clinical, and morphologic correlations in 78 cases of fibromatosis: a report from the CHAMP Study Group. CHromosomes And Morphology. Mod Pathol. 2000, 13 (10): 1080-1085. 10.1038/modpathol.3880200.CrossRefPubMed
11.
Bridge JA, Swarts SJ, Buresh C, Nelson M, Degenhardt JM, Spanier S, Maale G, Meloni A, Lynch JC, Neff JR: Trisomies 8 and 20 characterize a subgroup of benign fibrous lesions arising in both soft tissue and bone. Am J Pathol. 1999, 154 (3): 729-733.PubMedCentralCrossRefPubMed
12.
Fletcher JA, Naeem R, Xiao S, Corson JM: Chromosome aberrations in desmoid tumors. Trisomy 8 may be a predictor of recurrence. Cancer Genet Cytogenet. 1995, 79 (2): 139-143. 10.1016/0165-4608(94)00134-W.CrossRefPubMed
13.
Mertens F, Willén H, Rydholm A, Brosjö O, Carlén B, Mitelman F, Mandahl N: Trisomy 20 is a primary chromosome aberration in desmoid tumors. Int J Cancer. 63 (4): 527-529. 10.1002/ijc.2910630412. Nov 15 1995
14.
Qi H, Dal Cin P, Hernández JM, Garcia JL, Sciot R, Fletcher C, Van Eyken P, De Wever I, Berghe Van den H: Trisomies 8 and 20 in desmoid tumors. Cancer Genet Cytogenet. 1996, 92 (2): 147-149. 10.1016/S0165-4608(96)00170-7.CrossRefPubMed
15.
Ballo MT, Zagars GK, Pollack A, Pisters PW, Pollack RA: Desmoid tumor: prognostic factors and outcome after surgery, radiation therapy, or combined surgery and radiation therapy. J Clin Oncol. 1999, 17 (1): 158-167.PubMed
16.
Abbas AE, Deschamps C, Cassivi SD, Nichols FC, Allen MS, Schleck CD, Pairolero PC: Chest-wall desmoid tumors: results of surgical intervention. Ann Thorac Surg. 2004, 78 (4): 1219-1223. 10.1016/j.athoracsur.2004.03.015. discussion 1219–1223CrossRefPubMed
17.
Buitendijk S, Ven van de CP, Dumans TG, den Hollander JC, Nowak PJ, Tissing WJ, Pieters R, Heuvel-Eibrink van den MM: Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer. 104 (5): 1090-1099. 10.1002/cncr.21275. Sep 1 2005
18.
Faulkner LB, Hajdu SI, Kher U, La Quaglia M, Exelby PR, Heller G, Wollner N: Pediatric desmoid tumor: retrospective analysis of 63 cases. J Clin Oncol. 1995, 13 (11): 2813-2818.PubMed
19.
Merchant NB, Lewis JJ, Woodruff JM, Leung DH, Brennan MF: Extremity and trunk desmoid tumors: a multifactorial analysis of outcome. Cancer. 86 (10): 2045-2052. 10.1002/(SICI)1097-0142(19991115)86:10<2045::AID-CNCR23>3.0.CO;2-F. Nov 15 1999
20.
Lev D, Kotilingam D, Wei C, Ballo MT, Zagars GK, Pisters PW, Lazar AA, Patel SR, Benjamin RS, Pollock RE: Optimizing treatment of desmoid tumors. J Clin Oncol. 25 (13): 1785-1791. 10.1200/JCO.2006.10.5015. May 1 2007
21.
Gronchi A, Casali PG, Mariani L, Lo Vullo S, Colecchia M, Lozza L, Bertulli R, Fiore M, Olmi P, Santinami M, Rosai J: Quality of surgery and outcome in extra-abdominal aggressive fibromatosis: a series of patients surgically treated at a single institution. J Clin Oncol. 21 (7): 1390-1397. 10.1200/JCO.2003.05.150. Apr 1 2003
22.
Pignatti G, Barbanti-Bròdano G, Ferrari D, Gherlinzoni F, Bertoni F, Bacchini P, Barbieri E, Giunti A, Campanacci M: Extraabdominal desmoid tumor. A study of 83 cases. Clin Orthop Relat Res. 2000, 207-213. 10.1097/00003086-200006000-00025. 375
23.
Ballo MT, Zagars GK, Pollack A: Radiation therapy in the management of desmoid tumors. Int J Radiat Oncol Biol Phys. 42 (5): 1007-1014. Dec 1 1998
24.
Reitamo JJ: The desmoid tumor. IV. Choice of treatment, results, and complications. Arch Surg. 1983, 118 (11): 1318-1322.CrossRefPubMed
25.
Miralbell R, Suit HD, Mankin HJ, Zuckerberg LR, Stracher MA, Rosenberg AE: Fibromatoses: from postsurgical surveillance to combined surgery and radiation therapy. Int J Radiat Oncol Biol Phys. 1990, 18 (3): 535-540.CrossRefPubMed
26.
Posner MC, Shiu MH, Newsome JL, Hajdu SI, Gaynor JJ, Brennan MF: The desmoid tumor. Not a benign disease. Arch Surg. 1989, 124 (2): 191-196.CrossRefPubMed
27.
Spear MA, Jennings LC, Mankin HJ, Spiro IJ, Springfield DS, Gebhardt MC, Rosenberg AE, Efird JT, Suit HD: Individualizing management of aggressive fibromatoses. Int J Radiat Oncol Biol Phys. 40 (3): 637-645. Feb 1 1998
28.
Karakousis CP, Mayordomo J, Zografos GC, Driscoll DL: Desmoid tumors of the trunk and extremity. Cancer. 72 (5): 1637-1641. 10.1002/1097-0142(19930901)72:5<1637::AID-CNCR2820720524>3.0.CO;2-Y. Sep 1 1993
29.
Nuyttens JJ, Rust PF, Thomas CR, Turrisi AT: Surgery versus radiation therapy for patients with aggressive fibromatosis or desmoid tumors: A comparative review of 22 articles. Cancer. 88 (7): 1517-1523. 10.1002/(SICI)1097-0142(20000401)88:7<1517::AID-CNCR3>3.0.CO;2-9. Apr 1 2000
30.
Goy BW, Lee SP, Eilber F, Dorey F, Eckardt J, Fu YS, Juillard GJ, Selch MT: The role of adjuvant radiotherapy in the treatment of resectable desmoid tumors. Int J Radiat Oncol Biol Phys. 9 (3): 659-665. Oct 1 1997
31.
Zlotecki RA, Scarborough MT, Morris CG, Berrey BH, Lind DS, Enneking WF, Marcus RB: External beam radiotherapy for primary and adjuvant management of aggressive fibromatosis. Int J Radiat Oncol Biol Phys. 54 (1): 177-181. Sep 1 2002
32.
Janinis J, Patriki M, Vini L, Aravantinos G, Whelan JS: The pharmacological treatment of aggressive fibromatosis: a systematic review. Ann Oncol. 2003, 14 (2): 181-190. 10.1093/annonc/mdg064.CrossRefPubMed
33.
Sorensen A, Keller J, Nielsen OS, Jensen OM: Treatment of aggressive fibromatosis: a retrospective study of 72 patients followed for 1–27 years. Acta Orthop Scand. 2002, 73 (2): 213-219. 10.1080/000164702753671830.CrossRefPubMed
34.
Tsukada K, Church JM, Jagelman DG, Fazio VW, McGannon E, George CR, Schroeder T, Lavery I, Oakley J: Noncytotoxic drug therapy for intra-abdominal desmoid tumor in patients with familial adenomatous polyposis. Dis Colon Rectum. 1992, 35 (1): 29-33. 10.1007/BF02053335.CrossRefPubMed
35.
Izes JK, Zinman LN, Larsen CR: Regression of large pelvic desmoid tumor by tamoxifen and sulindac. Urology. 1996, 47 (5): 756-759. 10.1016/S0090-4295(96)00026-X.CrossRefPubMed
36.
Bauernhofer T, Stöger H, Schmid M, Smola M, Gürtl-Lackner B, Höfler G, Ranner G, Reisinger E, Samonigg H: Sequential treatment of recurrent mesenteric desmoid tumor. Cancer. 77 (6): 1061-1065. 10.1002/(SICI)1097-0142(19960315)77:6<1061::AID-CNCR9>3.0.CO;2-K. Mar 15 1996
37.
Lackner H, Urban C, Kerbl R, Schwinger W, Beham A: Noncytotoxic drug therapy in children with unresectable desmoid tumors. Cancer. 80 (2): 334-340. 10.1002/(SICI)1097-0142(19970715)80:2<334::AID-CNCR22>3.0.CO;2-U. Jul 15 1997
38.
Waddell WR, Kirsch WM: Testolactone, sulindac, warfarin, and vitamin K1 for unresectable desmoid tumors. Am J Surg. 1991, 161 (4): 416-421. 10.1016/0002-9610(91)91102-O.CrossRefPubMed
39.
Dominguez-Malagon HR, Alfeiran-Ruiz A, Chavarria-Xicotencatl P, Duran-Hernandez MS: Clinical and cellular effects of colchicine in fibromatosis. Cancer. 69 (10): 2478-2483. 10.1002/1097-0142(19920515)69:10<2478::AID-CNCR2820691016>3.0.CO;2-V. May 15 1992
40.
Brooks MD, Ebbs SR, Colletta AA, Baum M: Desmoid tumours treated with triphenylethylenes. Eur J Cancer. 1992, 28A (6–7): 1014-1018. 10.1016/0959-8049(92)90445-8.CrossRefPubMed
41.
Raguse JD, Gath HJ, Oettle H, Bier J: Interferon-induced remission of rapidly growing aggressive fibromatosis in the temporal fossa. Int J Oral Maxillofac Surg. 2004, 33 (6): 606-609. 10.1016/j.ijom.2004.01.011.CrossRefPubMed
42.
Fernberg JO, Brosjo O, Larsson O, Soderlund V, Strander H: Interferon-induced remission in aggressive fibromatosis of the lower extremity. Acta Oncol. 1999, 38 (7): 971-972. 10.1080/028418699432680.CrossRefPubMed
43.
Leithner A, Schnack B, Katterschafka T, Wiltschke C, Amann G, Windhager R, Kotz R, Zielinski CC: Treatment of extra-abdominal desmoid tumors with interferon-alpha with or without tretinoin. J Surg Oncol. 2000, 73 (1): 21-25. 10.1002/(SICI)1096-9098(200001)73:1<21::AID-JSO6>3.0.CO;2-G.CrossRefPubMed
44.
Plukker JT, van Oort I, Vermey A, Molenaar I, Hoekstra HJ, Panders AK, Dolsma WV, Koops HS: Aggressive fibromatosis (non-familial desmoid tumour): therapeutic problems and the role of adjuvant radiotherapy. Br J Surg. 1995, 82 (4): 510-514. 10.1002/bjs.1800820424.CrossRefPubMed
45.
Geurs F, Kok TC: Regression of a great abdominal desmoid tumor by interferon alpha. J Clin Gastroenterol. 1993, 16 (3): 264-265. 10.1097/00004836-199304000-00025.CrossRefPubMed
46.
Tjandra SS, Hsu C, Goh YI, Gurung A, Poon R, Nadesan P, Alman BA: IFN-{beta} signaling positively regulates tumorigenesis in aggressive fibromatosis, potentially by modulating mesenchymal progenitors. Cancer Res. 67 (15): 7124-7131. 10.1158/0008-5472.CAN-07-0686. Aug 1 2007
47.
Heinrich MC, McArthur GA, Demetri GD, Joensuu H, Bono P, Herrmann R, Hirte H, Cresta S, Koslin DB, Corless CL, Dirnhofer S, van Oosterom AT, Nikolova Z, Dimitrijevic S, Fletcher JA: Clinical and molecular studies of the effect of imatinib on advanced aggressive fibromatosis (desmoid tumor). J Clin Oncol. 24 (7): 1195-1203. 10.1200/JCO.2005.04.0717. Mar 1 2006
48.
Mace J, Sybil Biermann J, Sondak V, McGinn C, Hayes C, Thomas D, Baker L: Response of extraabdominal desmoid tumors to therapy with imatinib mesylate. Cancer. 95 (11): 2373-2379. 10.1002/cncr.11029. Dec 1 2002
49.
Azzarelli A, Gronchi A, Bertulli R, Tesoro JD, Baratti D, Pennacchioli E, Dileo P, Rasponi A, Ferrari A, Pilotti S, Casali PG: Low-dose chemotherapy with methotrexate and vinblastine for patients with advanced aggressive fibromatosis. Cancer. 92 (5): 1259-1264. 10.1002/1097-0142(20010901)92:5<1259::AID-CNCR1446>3.0.CO;2-Y. Sep 1 2001
50.
Weiss AJ, Lackman RD: Low-dose chemotherapy of desmoid tumors. Cancer. 64 (6): 1192-1194. 10.1002/1097-0142(19890915)64:6<1192::AID-CNCR2820640605>3.0.CO;2-J. Sep 15 1989
51.
Skapek SX, Ferguson WS, Granowetter L, Devidas M, Perez-Atayde AR, Dehner LP, Hoffer FA, Speights R, Gebhardt MC, Dahl GV, Grier HE, Pediatric Oncology Group: Vinblastine and methotrexate for desmoid fibromatosis in children: results of a Pediatric Oncology Group Phase II Trial. J Clin Oncol. 25 (5): 501-506. 10.1200/JCO.2006.08.2966. Feb 10 2007
52.
Reich S, Overberg-Schmidt US, Buhrer C, Henze G: Low-dose chemotherapy with vinblastine and methotrexate in childhood desmoid tumors. J Clin Oncol. 1999, 17 (3): 1086-PubMed
53.
Wehl G, Rossler J, Otten JE, Boehm N, Uhl M, Kontny U, Niemeyer C: Response of progressive fibromatosis to therapy with liposomal doxorubicin. Onkologie. 2004, 27 (6): 552-556. 10.1159/000081337.CrossRefPubMed
54.
Lynch HT, Fitzgibbons R, Chong S, Cavalieri J, Lynch J, Wallace F, Patel S: Use of doxorubicin and dacarbazine for the management of unresectable intra-abdominal desmoid tumors in Gardner's syndrome. Dis Colon Rectum. 1994, 37 (3): 260-267. 10.1007/BF02048164.CrossRefPubMed
55.
Patel SR, Evans HL, Benjamin RS: Combination chemotherapy in adult desmoid tumors. Cancer. 72 (11): 3244-3247. 10.1002/1097-0142(19931201)72:11<3244::AID-CNCR2820721118>3.0.CO;2-D. Dec 1 1993
56.
Pilz T, Pilgrim TB, Bisogno G, Knietig R, Koscielniak E, Carli M, Treuner J: [Chemotherapy in fibromatoses of childhood and adolescence: results from the Cooperative soft tissue sarcoma study (CWS) and the Italian Cooperative study group (ICG-AIEOP)]. Klin Padiatr. 1999, 211 (4): 291-295.CrossRefPubMed
57.
Okuno SH, Edmonson JH: Combination chemotherapy for desmoid tumors. Cancer. 97 (4): 1134-1135. 10.1002/cncr.11189. Feb 15 2003
Metadata
Title
Aggressive juvenile fibromatosis of the paranasal sinuses: case report and brief review
Authors
Shaheen E Lakhan
Robert M Eager
Lindsey Harle
Publication date
01-12-2008
Publisher
BioMed Central
Published in
Journal of Hematology & Oncology / Issue 1/2008
Electronic ISSN: 1756-8722
DOI
https://doi.org/10.1186/1756-8722-1-3

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