J. zyxwvutsrqpon faediatr. Child Health (1990) 26, 12-16 zyxwvuts Congenital teratomas of the neck and nasopharynx: A clinical and pathological study of 18 cases R. W. BYARD,’ C. L. JIMENEZ,’ B.F. CARPENTER’ and C. R. SMITH3 ’Department of Histopathology, Adelaide Children‘s Hospital, North Adelaide, Australia, ‘Department of Pathology, Children’s Hospital of €as tern Ontario, Ottawa, %epartment of Pathology, Hospital for Sick Children, Toronto, and the Canadian Reference Centre for Cancer fathology, Ottawa, Canada Abstract Congenital teratomas of the neck and nasopharynx are unusual tumours that seldom metastasize. Due to their rarity, reviews often have relied upon compilations of isolated case reports. We report our experience of the early outcome of 18 cases (14 cervical, four nasopharyngeal) to demonstrate the high morbidity and mortality that these benign but critically placed lesions have because of local mass effects. Six patients (33%) either were stillborn or died of disease within 2 days zy of birth; of the survivors, four (22%) had significant respiratory obstruction requiring surgery. Tumour location and size rather than histologic grading were the most significant features affecting the immediate clinical course. All but one of the patients who underwent early surgical resection had a favourable outcome. Key words: congenital; cervical; nasopharyngeal; teratoma. Congenital teratomas of the neck and nasopharynx are rare tumours that account for less than 5% of reported terat~mas.’-~ In 1988 Jordan and Gauderer reviewed the literature and found only 163 cases of congenital cervical teratomas, and Gnepp in 1985 located fewer than 30 cases involving the nasopharynx.’m6 Individual reports have tended to involve only small numbers of Because of the paucity of large series in the literature we felt that it was appropriate to report our experience with a total of 18 cases from three institutions, both to demon- strate the clinico-pathological features and also to emphasize the significant short-term problems, due to local mass effect, that may be presented by these critically placed neoplasms. METHODS A 25 year retrospective review of the files of the Hospital for Sick Children, Toronto, the Children’s Hospital of Eastern Ontario, Ottawa and the Canadian Reference Centre for Cancer Pathology, Ottawa. for the years 1963-88, was undertaken to locate all cases of congenital teratoma of the neck and nasopharynx. To be included in the series, a tumour had to have been present at birth, with tissues derived from all three germ cell layers (i.e. ectoderm, mesoderm and endoderm). Specifically, dermoid cysts with no endodermally derived tissue were excluded. Clinical details of the patient’s age, sex, presenting symptoms and signs, treatment and survival and any associated complications of pregnancy were tabulated. The pathology Correspondence: Dr Roger W. Byard, Department of Histopathology, Adelaide Children’s Hospital. North Adelaide, South Australia 5006, Australia R. W. Byard, MB, BS, FRCPC. Histopathologist. C. zyxwvutsr L. Jimenez, MD, FRCPC, Pathologist B. F. Carpenter, MD. FRCPC, Chief Pathologist. C. R Smith, MD, FRCPC, Pathologist. Accepted for publication 14 November 1989. reports were reviewed, the tumour size recorded and the original slides examined to confirm the histologic diagnosis and grading. Sampling of the tumours had been uniform in 11 cases, with at least one histologic section taken for each 10 mm of maximum diameter. The other seven cases had been consultation cases, with multiple representative sections chosen by the referring pathologists. Detailed autopsies had been performed on all stillbirths and neonatal deaths. RESULTS In total, 18 patients with congenital teratoma of the neck or nasopharynx were identified. The clinical and pathological features are sumrnarised in Table 1. In the group of patients with cervical lesions (cases 1-14), two were stillborn (cases 1 and 2) and three died of related problems within 2 days of birth (cases 3-5) (Fig. zyx l), giving a mortality rate of 36% (five of 14). In the patients born alive, a significant number (50° /o, 6 of 12) experienced respiratory impairment due to extrinsic tracheal compression that required surgical release (cases 3-8). One patient died of upper airway obstruction before surgery could be undertaken (case 3). The clinical impression at the time of attempted tracheostomy and resuscitation was of tracheal stenosis. At autopsy, the cause of the obstruction was found to be kinking and external compression of the trachea by the teratoma, rather than stenosis. Surgery was not attempted in one patient, who succumbed to respiratory problems resulting from a combination of pulmonary hypoplasia and prematurity (case 5). A similar picture emerged in the four patients with nasopharyngealtumours (cases 15-18). One (25%) was stillborn. A second (25%) experienced both respiratory distress and difficulty swallowing due to upper airway osbstruction; this was completely relieved by surgery. Overall there was a slight male predominance (M:F = 11:7). Premature delivery occurred in five cases, all of whom were dead