Indian Journal of Pathology and Microbiology

CASE REPORT
Year
: 2013  |  Volume : 56  |  Issue : 1  |  Page : 54--56

Alfa-fetoprotein secreting ovarian sex cord-stromal tumor


Kusum D Jashnani1, Chandrashekar V Hegde2, Shrutika P Munot1,  
1 Department of Pathology, T N Medical College and BYL Nair Ch. Hospital, Mumbai, Maharashtra, India
2 Department of Obstetrics and Gynecology, T N Medical College and BYL Nair Ch. Hospital, Mumbai, Maharashtra, India

Correspondence Address:
Kusum D Jashnani
8, Aashirvad, 1st Floor, Opposite Kakad Industrial Estate, L J Cross Rd 3, Mahim, Mumbai - 400 016, Maharashtra
India

Abstract

Ovarian sex cord-stromal tumors are relatively infrequent neoplasms that account for approximately 8% of all primary ovarian tumors. They are a heterogeneous group of neoplasms composed of cells derived from gonadal sex cords (granulosa and Sertoli cells), specialized gonadal stroma (theca and Leydig cells), and fibroblasts. They may show androgenic or estrogenic manifestations. We report such a tumor associated with markedly raised serum alpha-fetoprotein (AFP) levels in a young female presenting with a mass and defeminising symptoms. Serum AFP levels returned to normal on removal of tumor.



How to cite this article:
Jashnani KD, Hegde CV, Munot SP. Alfa-fetoprotein secreting ovarian sex cord-stromal tumor.Indian J Pathol Microbiol 2013;56:54-56


How to cite this URL:
Jashnani KD, Hegde CV, Munot SP. Alfa-fetoprotein secreting ovarian sex cord-stromal tumor. Indian J Pathol Microbiol [serial online] 2013 [cited 2020 Oct 29 ];56:54-56
Available from: https://www.ijpmonline.org/text.asp?2013/56/1/54/116152


Full Text

 Introduction



Ovarian sex cord-stromal tumor (SCST) is an uncommon tumor usually occurring in young adults, presenting with an abdominal mass and feminizing or virilising effect in 40% cases. [1] Although hormonally active, elevated serum alpha-fetoprotein (AFP) level is not a regular feature of these tumors. Approximately 30 cases of Sertoli-Leydig cell tumors secreting AFP have been published as yet. [2],[3],[4],[5],[6] We present a case of SCST with markedly raised serum AFP levels, clinically masquerading as a germ cell tumor. It is important for both pathologists and oncologists to be aware of such cases and the clinicopathological distinction from germ cell tumors, as the diagnosis would affect the management plan for the patient.

 Case Report



A 22-year-old nulligravida, married since 1 year, presented with pain and distension of abdomen and secondary amenorrhoea of 2 months duration. Physical examination revealed a 30 weeks pelvic mass. Her secondary sexual characters were well developed; no signs of virilisation were noted. Ultrasound showed a large iso-to hyperechoic heterogenous solid mass with small cystic areas arising from pelvis displacing the abdominal viscera. Ipsilateral ovary could not be identified separately. Computed tomography scan confirmed these findings. Serum AFP level was 2925 IU/ml, whilst β-Human Chorionic gonadotrophin, Carcinoma Embryonic Antigen (CEA), and Carbohydrate Antigen-125 (CA 125) levels were normal. Clinically, a germ cell tumor was suspected. A unilateral salpingo-oopherectomy was performed. The other ovary was normal intraoperatively.

Gross examination revealed a large, encapsulated solid mass measuring 22 × 20 × 10 cm with few cystic areas, a beefy red necrotic center and peripheral narrow viable rim showing yellow and firm white areas [Figure 1]. Microscopy revealed large areas of hemorrhagic necrosis. Tissue sections from the viable areas showed sheets of granulosa cells surrounded by fibrothecomatous stroma admixed with sertoli tubules. The granulosa cell component showed cellular islands punctuated by characteristic Call-Exner bodies giving rise to a microfollicular pattern. Nuclei were round to oval without grooves and the cytoplasm was moderate in amount. No abnormal mitiotic activity was found. The Sertoli tubules showed central lumen surrounded by low columnar cells on a basement membrane. Islands of cells with abundant eosinophillic cytoplasm and central nuclei were also evident [Figure 2]. These may be Leydig cells. These were not hepatocytic or hepatoid cells as these cells did not show well-defined cell borders. Luteinised granulosa cells may be another possibility. Extensive sampling of the viable yellow areas did not reveal germ cell tumor in the form of yolk sac tumor. Immunohistochemistry revealed strong positive staining for AFP in the large eosinophillic cells. Inhibin was focal positive in the granulosa cells. Calretinin was negative and Pan Cytokeratin (CK) was positive [Figure 3]. Diagnosis was given as SCST. A diagnosis of Gynandroblastoma was considered but then withheld due to uncertainty of the proportion of the two components as almost 80-85% of tumor showed hemorrhagic necrosis.{Figure 1}{Figure 2}{Figure 3}

Post-operative serum AFP levels dropped precipitously, returning to normal within 1 week of removal of tumor. The patient regained her menses within 2 months and conceived 4 months later. Serial follow-up revealed serum AFP levels within normal limits. Pelvic ultrasound did not show any tumor recurrence. She is disease free for the last 15 months.

 Discussion



We report this case for its association with unusually high AFP levels, masquerading as a germ cell tumor clinically. AFP is normally secreted by fetal liver, yolk sac, and to a minute degree in gastrointestinal tract. It is elevated in malignancies resembling these tissues, as well as in variety of ovarian tumors and non-germ cell tumors of the urological system [7] serving as a useful tumor marker in both diagnosis and follow-up. AFP production is also associated with Leydig cells, Sertoli cells and heterologous hepatocytic differentiation. [4],[5],[6],[8] Albeit rare, there are an increasing number of cases of Sertoli Leydig cell tumor (SLCT)s and granulosa cell tumor with raised AFP being reported. [9] Profound elevation in AFP levels, as in our case, can be attributed to a possible torsion of the tumor mass leading to extensive hemorrhagic necrosis leading to outpouring of AFP from Leydig or luteinised cells which are otherwise responsible for only modest rise in AFP. The possibility of germ cell tumor with yolk sac component was ruled out as extensive sampling from both viable and necrotic areas did not reveal any traces of germ cell component. The half-life of AFP being short (i.e., 5 days), level regresses quickly after tumor removal. Sustained normal AFP levels and ultrasound denotes absence of tumor recurrence.

Immunohistochemistry of SLCTs is characteristically positive for inhibin, calretinin, and in some cases for AE1/3, CD99, and vimentin. It is negative for EMA and chromogranin. [10] The differentiation between Leydig cells and hepatocytic cells may be difficult on H and E. The latter shows positive immunoreactivity with keratin cocktail and CAM 5.2 and negative with inhibin and calretinin. In our case, AFP was localized to large eosinophilic cells. The cells were focally positive for inhibin but negative for calretinin. This suggests that the two markers, inhibin and calretinin could play a complementary role in the diagnosis of ovarian SCSTs. Strong positivity was seen for pan CK.

AFP secreting SLCTs are now well recognized as a distinct sub-group. However, the exact histogenesis and prognosis of this entity remains to be elucidated. Loco-regional metastasis is an important indicator of malignancy. We report a case of ovarian SCST with elevated serum AFP, which may mimic a germ cell tumor clinically. Such a case has never been reported in literature.

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