Sinonasal adenocarcinoma: A rare second malignancy in long term retinoblastoma survivors

Abstract

Retinoblastoma is the most common primary cancer of the eye in children. The incidence of second tumors in survivors of bilateral retinoblastoma and in survivors of unilateral retinoblastoma who presumably carry a germline RB1 mutation is documented. This paper describes the previously unrecognized association of sinonasal adenocarcinoma as a second malignancy in retinoblastoma survivors. We present three cases who received radiation therapy as a part of their treatment and developed sinonasal adenocarcinoma as a second malignancy. Sinonasal adenocarcinoma should be considered as a second malignancy in retinoblastoma survivors who present with vague sinus symptoms.

Second cranio-facial malignancies in hereditary retinoblastoma survivors previously treated with radiation therapy: Clinic and radiologic characteristics and survival outcomes

“…Physici ans should realise that otherw ise innocen t sympt oms, such as a combination of chronic headache and sinus sympt oms, may be potentially indica tive for cranio-facial SPTs. 8 These (chronic) sympt oms in irradiat ed hereditary Rb-pa tients should alert physici ans to perform imag ing without delay in order to detect SPTs at a potentiall y curative stage .…”

“…[2][3][4][5][6] The incide nce of SPTs in hereditary Rb is 8.4% at 18-yea rs and 36% at 50-yea rs after diagn osis. 3,4,[6][7][8][9] External beam radiotherap y (EBRT) increa ses the risk for subsequen t malignant neo plasms, as up to 70% of SPTs in Rb patients develop insi de or at bounda ries of the irradiat ion field. 3,4,6,[10][11][12][13][14] The age of Rb-diagnosis and subsequent ly age of irra diation is an addition al risk facto r: patien ts irradiat ed during the first year oftheir-l ife develop two to eight times more SPTs than patien ts irradiat ed after one year.…”

“…4 Most common histologic al types of SPT in irradiat ion fields are osteosarcom a, rhab domyosar coma, leiom yosarcom a, other soft tissue sarcom as, mening ioma 12,13,18,19 and rarely carcin omas. 8,9 Progno sis of cran io-facia l SPTs in Rb survi vors is poor despit e agg ressive treatment . One major pro gnostic factor of these tumour s is the feasibil ity of complete resection of the SPT 20 and theref ore early diagn osis is crucia l.…”

OTX2 is a therapeutic target for retinoblastoma and may function as a common factor between C-MYC, CRX, and phosphorylated RB pathways

“…There are no current therapies that specifically target a unique molecular defect intrinsic to retinoblastoma. The non-specific radio-and chemotherapy treatments can have side effects including cognitive decline and secondary malignancy, which is especially worrisome in such a young susceptible patient population (5)(6)(7). Approximately 1% of patients with retinoblastoma in both eyes develop second non-ocular tumors each year and the cumulative probability of death from these secondary malignancies is 26% at 40 years after diagnosis (8,9).…”

Sinonasal Malignancy Following Cranial Irradiation: A Scoping Review and Case Report of Sinonasal Teratocarcinosarcoma

“…6,7 Previous studies have demonstrated an increased risk of subsequent central nervous system neoplasms following cranial irradiation, 8,9 and there are several reported instances of sinonasal neoplasm development following delivery of RT adjacent to the brain, such as for retinoblastoma in children. [10][11][12] Tumors of the nasal cavity and paranasal sinuses (sinonasal tumors) are a relatively rare entity, constituting < 5% of all head and neck neoplasms. 13 They have an incidence of < 1 in 100,000 in the United States.…”