Ovarian Sertoli-Leydig cell tumour with raised serum alpha fetoprotein

Summary A case of ovarian Sertoli-Leydig cell tumour with a raised serum alpha fetoprotein in reported. The patient first presented at the age of 27 years with a history of 6 years' amenorrhoea followed by 3 months irregular vaginal bleeding. A ovarian tumour was found and excised and shown microscopically to be a spindle cell malignant tumour. The patient was treated with chemotherapy and had a complete response. Thirty months after first presentation there was a recurrence in the pelvis which microscopically showed the typical features of a Sertoli-Leydig cell tumour. Six months later a second recurrence had the microscopic appearance of a lipid cell tumour. A raised serum alpha fetoprotein was found at the time of the second recurrence and immunohistochemistry showed this protein in the Leydig and luteinized cells of the recurrent tumours but not in the spindle cells of the original ovarian neoplasm.     

Alpha-fetoprotein and Sertoli-Leydig cell tumor

Gard GB, Mulvany N, Quinn MA. Alpha-fetoprotein and Sertoli-Leydig cell tumor. Int J Gynecol Cancer 1998; 8 : 499–503.
The association between a Sertoli-Leydig cell tumor and a raised serum alpha-fetoprotein (AFP) is an uncommon occurrence which has only been previously described in 20 patients. We report a 17-year-old patient who presented with a pelvic mass and a raised serum AFP. At laparotomy she had a FIGO stage IA moderately differentiated Sertoli-Leydig cell tumor which required no further treatment. We have reviewed the literature relating to AFP production by Sertoli-Leydig cell tumors. When serum AFP is raised at diagnosis it appears to be a reliable tumor marker, hence a baseline level should be documented on all patients with Sertoli-Leydig cell tumor.     

Ovarian Sertoli-Leydig cell tumor with heterologous gastrointestinal epithelium as a source of alpha-fetoprotein: a case report

Ovarian Sertoli-Leydig cell tumors (SLCT) are rare sex cord stromal neoplasms. To date there have been approximately 25 case reports of ovarian SLCT expressing alpha-fetoprotein (AFP). In such cases, AFP was immunohistochemically detected in the Sertoli cells, Leydig cells, or hepatocytes. This case report confirms heterologous gastrointestinal epithelium expression of AFP. A 20-year-old woman presented with complaints of abdominal enlargement and irregular menstrual cycles over one year. A right ovarian tumor was detected and the patient's serum AFP was elevated. A right salpingo-oophorectomy was performed. On microscopic examination, the tumor was composed of a fibrosarcoma-like area and a poorly differentiated SLCT area with heterologous gastrointestinal epithelium. Immunohistochemical analysis detected AFP in the gastrointestinal epithelium only. Postoperatively, serum AFP levels fell to normal. A recurrent tumor was discovered in the omentum after adjuvant chemotherapy, but serum AFP remained normal. A second laparotomy was performed and the recurrent tumor showed only fibrosarcoma-like features. The patient received second line chemotherapy and is currently in remission. This is the first case of AFP production by heterologous gastrointestinal epithelium in SLCT.     

Ovarian Sertoli-Leydig cell tumor: success with salvage therapy

A case of metastatic ovarian Sertoli-Leydig cell tumor is presented. The patient achieved a complete pathologic response with four courses of cisplatin, vinblastine and bleomycin chemotherapy. Recurrence of the tumor was detected with elevated serum alpha-fetoprotein. After surgical debulking, the recurrence was treated with intra-arterial cisplatin and intravenous etoposide with pelvic radiation therapy followed by intravenous doxorubicin and cyclophosphamide. The patient is alive and disease free 75 months after initial diagnosis, 4 years after salvage therapy.     

Virilising ovarian tumour: a case associating a Sertoli-Leydig cell tumour and a Brenner tumour

Ovarian Sertoli-Leydig cell tumours (SLCT), also termed arrhenoblastomas, are the most frequent virilising tumours in women of reproductive age. Very rare secretory Brenner tumours (BT) have been described, generally after the menopause. A 31-year-old woman sought medical advice for secondary amenorrhoea, progressive hirsutism and a 5-year history of virilisation syndrome with clitoromegaly. Testosterone was markedly high (285 ng/dl, N<85) with moderate elevation of delta 4-androstenedione (D4AD) (311 ng/dl, N <270), dehydroepiandrosterone sulfate (DHEAS) (366 μg/dl, N <340) and 17-hydroxyprogesterone (17OHP) (275 ng/dl). LH was 9 IU/l, FSH 4.3 IU/l, estradiol 60 pg/ml and progesterone 314 ng/100?ml. Cortisol was decreased (1.3 μg/dl) after the dexamethasone suppression test. Pelvic MRI showed a 5-cm right ovarian tumour with a 2.5?cm nodular component and cystic areas, and two nodules measuring 11?mm and 15?mm above the right and left ovaries. After right ovariectomy by laparoscopy, pathological examination concluded on a 3-cm SLCT and a 2-cm BT; the nodules above the ovaries were dysembryoplastic cysts. Postoperatively, testosterone level was normal after 24?h (26 ng/dl), estradiol and progesterone rapidly decreased, cyclic secretion then resumed and the patient menstruated at day 27. To our knowledge, this is the first report of an ovarian tumour associating a Sertoli-Leydig cell tumour and a Brenner tumour in a patient with virilisation syndrome which resolved after ovariectomy.     

A case of Sertoli-Leydig cell tumour of the ovary with a multilocular cystic appearance on CT and MR imaging

We present a case of Sertoli-Leydig cell tumour of the ovary in a 14-year-old girl who presented with abdominal distension. Ultrasonography showed a multilocular cystic lesion filled with finely echogenic fluid. Contrast-enhanced CT demonstrated a huge multilocular cystic mass with thickened septa. At MR imaging, the capsule of the cyst was focally thickened, showing intermediate signal intensity on T2-W images. Although extensive cyst formation of Sertoli-Leydig cell tumour is rare, this tumour should be considered in the differential diagnosis of a multilocular cystic ovarian tumour in a young female.     

Ovarian Sertoli-Leydig Cell Tumor with Rhabdomyosarcoma: An Ultrastructural Study

The clinical and light and electron microscopic findings of a moderately differentiated, virilizing, Sertoli-Leydig cell tumor (SLT) with pleomorphic rhabdomyosarcoma of the ovary are presented. The tumor recapitulates the primitive embryonal testis and rhab-domyogenesis, respectively. The natural history, including pathogenesis, of this peculiar and rare tumor is discussed in the light of the pertinent literature on SLT and ovarian rhabdomyosarcoma. It seems that when rhabdomyosarcoma is a significant or predominant component of SLT, as occurred in this case, the prognosis is poor and is that of rhabdomyosarcoma in general.     

Tumor de Sertoli-Leydig como hallazgo casual en quistectomía por endometriosis

Ovarian tumours are mostly casual findings in imaging tests performed for another reason. Symptomatic endometriosis is a common reason in young women attending clinics. Sertoli-Leydig tumours have an embryological origin and are very rare. In this case the diagnosis was made during surgery for an endometriosis and as a finding in the contralateral ovarian biopsy.