Primary small cell neuroendocrine carcinoma of larynx is a rare, aggressive type of malignancy. As only about 200 cases worldwide have been reported, any larger institutional experience is valuable. This study reports our experience in managing this rare entity.

Of the nine patients identified, three had metastatic cancer at the time of diagnosis. Four patients underwent radical treatment: one was managed surgically followed by adjuvant chemotherapy and consolidation radiotherapy; the remaining three were treated with neoadjuvant chemotherapy and definitive radiotherapy.

Of the 4 patients treated with radical intent, 1 was alive and disease free after 99 months, 2 died of metastatic disease after 22 and 26 months, and 1 was alive after 20 months with a diagnosis of recurrent disease.

Our relatively small number of patients confirms other centres' experiences. This cancer has a poorer prognosis than most other head and neck cancers. Although the logistics would be challenging, there is a need for international multicentre trials for this disease modelled on those performed for other cancers, as has been done for paediatric malignancies.

Small cell carcinoma has the worst prognosis of all laryngeal neoplasms. In order to further characterise this tumour, with a view to development of new therapeutic approaches, we report the results of KIT gene and platelet-derived growth factor receptor α gene expression analysis, for two extremely rare cases of primary small cell carcinoma of the larynx.

Case reports, including immunohistochemical study, and review of the literature.

We present two patients with laryngeal small cell carcinoma, who died from tumour metastasis to the lungs and brain despite aggressive treatment. Immunohistochemical studies revealed positive reactions for KIT gene expression and platelet-derived growth factor α gene expression in patient one, and for KIT gene expression in patient two. Molecular genetic analysis, using polymerase chain reaction direct sequencing, identified no mutations of the KIT or platelet-derived growth factor receptor α genes.

Although further investigation is necessary regarding KIT gene expression and platelet-derived growth factor receptor α gene expression in laryngeal small cell carcinoma, the reported results suggest that these genes may be significant in the development of molecular targeted therapy.

We present the first reported case of primary small cell carcinoma of the lacrimal sac.

A 67-year-old Japanese woman was referred to our department with a two-month history of left medial canthal swelling, epiphora and occasional nasal bleeding. Nasal endoscopy revealed a readily bleeding tumour in the left inferior meatus. Computed tomography and magnetic resonance imaging scans demonstrated that the tumour was mainly located in the left lacrimal sac. Histopathological studies of a biopsy specimen revealed small cell carcinoma. The patient was treated with four cycles of chemotherapy consisting of cisplatin and etoposide, in combination with radiotherapy. There was no evidence of recurrence or metastasis for five years.

Small cell carcinoma originating in the head and neck region has been reported to be highly aggressive and to have a poor prognosis. We report a case of primary small cell carcinoma of the lacrimal sac successfully treated with chemo-radiotherapy.