Rare Incidence of Three Consecutive Primary Tumors in the Maxillofacial Region: Retinoblastoma, Leiomyosarcoma, and Choriocarcinoma: Case Report

Abstract

Multiple primary tumors occur more commonly in the region of the head and neck than elsewhere in the body. The chance of this is particularly high in patients treated for retinoblastoma, in part because of a genetic predisposition, and in part because of the possibility of irradiation treatment. However, triple tumors occur in only 0.5% of multiple tumors. A rare case of a triple (metachronous) tumor is reported: 12 years after the treatment of bilateral retinoblastoma (enucleation and irradiation), secondary leiomyosarcoma developed in the maxillofacial region, followed 5 years later by choriocarcinoma. Surgery was performed on all three types of tumor. As a result, the female patient (currently 21 years old) is now free of complaints and has married. It is extremely rare for either leiomyosarcoma or choriocarcinoma (CHC) to appear in the maxillofacial region. The long-term, systematic control of such patients is absolutely necessary, for the multiple tumors tend to develop only after a long latency period of 10 to 20 years.

Soft tissue, pelvic, and urinary bladder leiomyosarcoma as second neoplasm following hereditary retinoblastoma

“…Leiomyosarcomas are comparatively rare with, as far as we are aware, only 18 reported cases. [4][5][6][7][8][9][10][11][12][13][14] Some of these tumours have occurred in soft tissues in the field of previous radiotherapy. However, there are case reports of leiomyosarcoma developing in sites outside the head and neck, including the femur, liver, and urinary bladder.…”

Nasal Sinus Leiomyosarcoma in a Patient with History of Non-Hereditary Unilateral Treated Retinoblastoma

“…The other large cohort study of MacCarthy et al [2] included patients with secondary tumors, but the locations were also not matched to secondary tumor type. In addition, ten cases in the literature of LMS arising in patients with a history of RB originated in areas adjacent to the sinonasal cavity (orbit, maxilla) for which it is possible the sinonasal tract was involved by extension but did not appear to be the original or significantly involved site [10,[15][16][17][18][19][20].…”

Sinonasal Leiomyosarcoma: Review of Literature and Case Report

“…At treatment and diagnosis their ages ranged from 10 to 87 (mean 46.22) years. The most common primary treatment was surgery alone in 35 cases 2,3,5–7,9,13,15–17,19,22,24,25,27–30,32–34,37 . Surgery plus radiation was performed in 16 cases; 2,9,10,14,17,20,21,26,31–33,35 surgery plus chemotherapy was performed in 3 cases; 5,11,36 radiation plus chemotherapy was performed in 2 cases; 12,26 and surgery plus radiation plus chemotherapy was performed in 2 patients, 32 including ours.…”

“…Of these 63 reported patients, 2–37 disease status at long‐term follow‐up was available in 50 patients (including our patient) 2,3,5–8,10–12,14,15,17,20–22,24–33,35 to determine prognosis. No follow‐up data were available for 12 patients, 9,13,16,18,19,23,32,34,36,37 who were therefore excluded from consideration.…”

“…Of these 256 tumors, only 6 (2.3%) were leiomyosarcomas. Since Dobben 3 reported the first case in 1958, 62 cases of primary leiomyosarcomas of the sinonasal territory and anterior skull base have been reported in the English literature 2–37 . We treated a 24‐year‐old man for leiomyosarcomas involving the sinonasal area and the anterior skull base and reviewed the related literature.…”