Ovarian small cell carcinoma

Epidemiology

Small cell carcinoma of the ovary, hypercalcaemic type, is a tumour of young women.

Clinical features

Presentation is with abdominal swelling or pain. About two thirds of patients develop hypercalcaemia, resulting in fatigue, lethargy, polydipsia and polyuria.

Macroscopic appearances

Most patients have extra-ovarian tumour at presentation.

Histopathology

Small cell carcinoma of hypercalcaemic type is composed of sheets, nests or cords of small hyperchromatic cells with sparse cytoplasm. There is necrosis and a high mitotic rate. Follicle-like spaces contain eosinophilic or basophilic material. A large cell variant has interspersed large cells with vesicular nuclei.

Immunohistochemistry

Combined positivity with WT1 and EMA may be diagnostically helpful, not being seen in most of the tumours which form the differential diagnosis.

	p16	small cell carcinoma, hypercalcaemic type: 6/72
	p16	small cell carcinoma, pulmonary type: 2/22
	p53	15/151
	WT1	14/151
		small cell carcinoma, hypercalcaemic type: 6/72
		small cell carcinoma, pulmonary type: 0/22
	EMA	13/151
	Calretinin	11/151
	AE1/3	9/151
	CD56	4/151
	BerEP4	3/151
	Synaptophysin	2/151
	S-100	1/151
	CK5/6	0/151
	Chromogranin	0/151
	CD10	14/151
	CD99	0/151
	CD117	small cell carcinoma, hypercalcaemic type: 0/72
	CD117	small cell carcinoma, pulmonary type: 0/22
	NB84	0/151
	Desmin	0/151
	a-inhibin	0/151
	TTF-1	0/151
		small cell carcinoma, hypercalcaemic type: 0/72
		small cell carcinoma, pulmonary type: 1/22

Differential diagnosis

Metastatic small cell carcinoma
Germ cell tumours
Sex cord stromal tumours

	small cell carcinoma of ovary, hypercalcaemic type	small cell carcinoma of ovary, pulmonary type	small cell carcinoma of cervix	small cell carcinoma of lung
WT1	6/72	0/22	1/82	1/222
TTF-1	0/72	1/22	3/82	20/222
p16	6/72	2/22	7/82	21/222
CD117	0/72	0/22	2/82	16/222
OCT3/4	0/72	0/22	0/82	0/212
HPV by PCR	0/72		5/62	0/122

Prognosis

Small cell carcinoma is usually lethal.

References

1 McCluggage WG, Oliva E, Connolly LE, et al. An immunohistochemical analysis of ovarian small cell carcinoma of hypercalcemic type. Int J Gynecol Pathol 2004; 23:330-6 previously presented McCluggage WG et al. An immunohistochemical analysis of ovarian small cell carcinoma of hypercalcaemic type. Pathological Society July 2004, abstract 75.

2 Carlson JW, Nucci MR, Brodsky J, et al. Biomarker-assisted diagnosis of ovarian, cervical and pulmonary small cell carcinomas: the role of TTF-1, WT-1 and HPV analysis. Histopathology 2007; 51:305-12

This page last revised 30.11.20047