Nasal sinus leiomyosarcoma in a patient with history of non-hereditary unilateral treated retinoblastoma.
Hereditary patients with a history of treated retinoblastoma (RB) have a greatly increased risk of a broad spectrum of secondary malignancies appearing many years later, with a high incidence in the head and neck region. Leiomyosarcomas (LMS) account for up to 58% of these tumors. LMS in the sinonasal region generally are uncommon and are associated with a locally aggressive course and have a poor prognosis. RB may occur in two forms. The hereditary form is generally bilateral but can present unilaterally with a positive family history and typically exhibits a germline mutation in the RB1 gene on chromosome 13. The non-hereditary form is usually unilateral but can show the same germline mutation in up to 10% of cases. Patients with hereditary RB have been shown to have a significantly higher cumulative risk of developing secondary malignancies than those with the non-hereditary form (28 vs. 1.44% respectively). Most reported cases of sinonasal LMS are in patients with a history of the bilateral hereditary form of treated RB. We report a case of LMS of the nasal sinus area in a 35-year-old African American male with a history of non-hereditary unilateral RB and radiation therapy. To the best of our knowledge, this is the first reported case of sinonasal LMS arising in a patient with a history of non-hereditary unilateral RB. The clinical history, radiology, and pathology are presented along with a brief discussion of the literature.
Fitzpatrick
Sarah G
SG
Department of Oral and Maxillofacial Diagnostic Sciences, University of Florida College of Dentistry, Gainesville, FL 32610, USA. sfitzpatrick@dental.ufl.edu
Woodworth
Bradford A
BA
Monteiro
Carmela
C
Makary
Raafat
R
D000328:Adult; D016371:Cranial Irradiation / Q000009:adverse effects; D006801:Humans; D007890:Leiomyosarcoma / Q000473:pathology*; D008297:Male; D009381:Neoplasms, Radiation-Induced / Q000473:pathology*; D016609:Neoplasms, Second Primary / Q000473:pathology*; D010255:Paranasal Sinus Neoplasms / Q000473:pathology*; D019572:Retinal Neoplasms / Q000473:pathology / Q000532:radiotherapy; D012175:Retinoblastoma / Q000473:pathology / Q000532:radiotherapy
Arch Pathol Lab Med. 2009 May;133(5):699-712
19415944
Arch Otolaryngol Head Neck Surg. 1990 Nov;116(11):1278-86
2242259
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2004 Jun;97(6):726-31
15184855
J Clin Oncol. 2005 Apr 1;23(10):2272-9
15800318
Ophthalmic Surg Lasers. 1999 Jul-Aug;30(7):579-83
10929985
J Natl Cancer Inst. 2008 Dec 17;100(24):1771-9
19066271
J Neurosurg. 2005 Dec;103(6):1077-80
16381195
J Natl Cancer Inst. 2004 Mar 3;96(5):357-63
14996857
Histopathology. 2002 Jun;40(6):518-25
12047762
Laryngoscope. 2005 Dec;115(12):2242-8
16369174
Head Neck. 2007 May;29(5):439-45
17163467
Indian J Cancer. 2008 Jul-Sep;45(3):123-5
19018117
Arch Ophthalmol. 1983 Oct;101(10):1562-5
6626010
Oncogene. 2006 Aug 28;25(38):5350-7
16936757
Br J Ophthalmol. 2009 Sep;93(9):1159-62
19704040
J Natl Cancer Inst. 2007 Jan 3;99(1):24-31
17202110
Med Pediatr Oncol. 1998 Jan;30(1):59-62
9371391
Arch Ophthalmol. 1983 Oct;101(10):1557-61
6626009
Skull Base. 2003 Aug;13(3):179-182
15912176
J Craniofac Surg. 2001 Sep;12(5):464-8
11572252
J Laryngol Otol. 1991 Dec;105(12):1068-71
1787362
Radiat Med. 1991 Sep-Oct;9(5):183-4
1771249
Orbit. 2008;27(4):285-91
18716966
Sarcoma. 1997;1(2):89-93
18521207