Ovarian tumours though a common cause of gynaecological tumours, are not amongst the common solid tumours of childhood. Steroid (lipid) cell tumours account for less than 0.1% of all the ovarian tumours and are rarely seen in prepubertal age group. Virilization occurs in nearly all the childhood cases although few cases with isosexual pseudoprecocity have been reported. Extensive literature search has shown that Steroid cell tumours are benign when they occur in prepubertal children. A case of an eight-year-old girl with virilizing malignant Steroid cell tumour, not otherwise specified is reported.

Introduction

Ovarian steroid cell tumours are rare neoplasm thought to arise from ovarian stroma, hilus or adrenal cortical rests. The cellular origin of a large subset cannot be determined on morphological feature, thereby designated as steroid cell tumours not otherwise specified (NOS).1 In the paediatric age group among the primary ovarian neoplasm, gram cell tumours are frequent. Virilization in childhood is more frequently due to inborn defect in adrenal corticoid biosynthesis. Although rare, steroid cell tumour of ovary should also be considered in differential diagnosis of childhood virilization.2

We present a case of ovarian steroid cell tumour in an 8-year old girl child with androgenic pseudoprecocity.

Case Report
An 8-year old girl child came with history of continuous dragging sensation and abdominal pain since 2 months. She had not yet attained menarche. A large abdominal lump was palpable which almost filled the entire abdominal cavity. Ultrasonography revealed left solid ovarian tumour. Serum hormonal levels of b-hCG and AFP done to rule out germ cell tumour were negative. Left oopherectomy was carried out. The right ovary appeared normal. Grossly the tumour measured 15 x 14 x 10 cms and had a bosseland external surface with intact capsule. The cut surface was solid, bulging, and homogenous yellow with few areas of haemorrhage and necrosis (Fig. 1). Microscopy showed solid islands and nests of tumour cells compressing adjacent capillary network. The individual tumour cells were large polygonal with vacuolated to granular eosinophilic cytoplasm, distinct cell borders and vesicular nuclei, nuclear atypia was of grade 2 (Fig. 2). Frozen fat demonstration done by Sudan III was weakly positive. There was evidence of mitotic activity (> 2/10 hpf), areas of haemorrhage and necrosis. Reinke crystalloids were not observed in the cytoplasm of tumour cells. Lymphovascular space invasion was not evident. A histopathological diagnosis of steroid (lipid) cell tumour (NOS) - malignant was given based on morphological features. Clinical examination of the patient on followup revealed signs of virilization in the form of clitoromegaly with clitoral index of 50 mm2 and pubic hairs. No breast enlargement was noted. Hormonal studies like 24-hour urinary excretion of 17-ketosteroids or 17-hydroxycorticosteroids, plasma testosterone and androstenedione levels were not available as the signs of virilization were noted only after the histopathological diagnosis.

Fig. 1 : Cut surface is solid, bulging and homogenous yellow with areas of necrosis and haemorrhage.	Fig. 2 : Diffuse sheets of large polygonal tumour cells with vacuolated cytoplasm and vesicular nuclei (400 X H and E).

Discussion

The histogenesis of lipid cell tumour of ovary is still a matter of controversy. Steroid cell tumour is a term (coined by Scully) more appropriate because of the morphologic features of the tumour, its propensity to secrete steroid hormones and because such lesions contain little or no lipid.3 Virilization is the most common presenting symptom, unusual clinical presentations include abdominal swelling, oestrogenic manifestations and Cushing’s syndrome.4 Virilization in prepubertal girls (hetero-sexual puberty) results from increased circulatory androgen levels. Its clinical effects include clitoral enlargement, acne, deepening of voice and increased muscular development. Androgenic tumours of the ovary are an uncommon source of clinical virilization, especially in children, in whom the most common cause of virilization is an inborn, defect in adrenal corticoid biosynthesis.2

Most of these ovarian neoplasms are benign and removal leads to complete or partial reversal of signs of virilization. Among the rare neoplasms of the ovary, that may occur in prepubertal age are Leydig cell tumour, Granulosa cell tumour and steroid cell tumour. A review of reported cases show most steroid cell tumours (NOS) to occur during reproductive and middle age years. Only a handful of cases have been cited in prepubertal children.1,2 Though previous studies have reported malignancy in steroid cell tumours of the ovary, none of them have occurred in children. The criteria for malignancy includes tumour size greater than 8 cm in diameter, mitotic rate (> 2/hpf) and the presence of necrosis, all of these features were seen in the present case. Steroid cell tumours although can occur at any age are diagnosed rarely before 20 years and even though malignancy has been reported, none occurred in children.

This case is reported for its rarity in this age group, its unusual presentation and histomorphological features of malignant steroid cell tumour. Since only few cases of steroid cell tumour of ovary cited in literature are in prepubertal age group, all are reported benign, close follow up is essential to study the course/outcome of the patient.

References

1. Hayes MC, Scully RE. Ovarian steroid cell tumours not otherwise specified. Am J Surg Pathol 1987; 11 : 835-45.
2. Haris AC, Wakely PE, Kaplowitz PB, Lovinger RD. Steroid cell tumour of Ovary in a child. Arch Pathol Lab Med 1991; 115 : 150-54.
3. Lin CJ, Jorge AL, Latronico AC, et al. Origin of an ovarian steroid cell tumour causing isosexual pseudoprecocious puberty demonstrated by the expression of Adrenal steroidogenic enzymes and adrenocorticotropin receptor. J Clin Endocrinol Metab 2000; 85 : 1211-13.
4. Taylor HB, Norris HJ. Lipid cell tumour of the ovary. Cancer 1967; 20 : 1953-61.