Germ cell tumor of ovary with plenty of sarcoid like granulomas: A diagnosis on fine needle aspiration cytology.

Germ cell tumors of ovary with elements of two or more subtypes are relatively uncommon. Sarcoid-like granulomas are seen in rare association with dysgerminomas, a subgroup of germ cell tumors of ovary, which are again less common. Fine-needle aspiration cytology (FNAC) of the gonadal germ cell tumor, though not performed frequently, is of help when a gonadal mass does not require resection as in lymphoma which can be treated by chemotherapy. FNAC helps to diagnose this tumor and to rule out other lesions, including germ cell tumors. It is also an effective, rapid and reliable procedure for the diagnosis of germ cell tumors.

Introduction

Germ cell tumors represent a group of neoplasms, presumably derived from the germ cells, which usually occur in ovary and testis, but may also occur in several extragonadal locations. World Health Organization (WHO) recognizes several subtypes of germ cell tumors containing elements of two or more subtypes.[1] Sarcoid-like granulomas are present in some of the cases of dysgerminomas, which form a group of germ cell tumors of the ovary.[2]

Several studies have demonstrated the usefulness of fine-needle aspiration cytology (FNAC) in the diagnosis of germ cell tumors. It helps to rule out non-germ cell tumors like malignant lymphoma, which do not require resection.[1]

Case Report

A 25-year-old female presented with pain abdomen in the right iliac fossa. On examination, there was a mass in the right iliac fossa near the umbilicus, measuring 7 × 6 cm, and was firm to hard in consistency. Clinically, ilieocecal kochs or carcinoma cecum was suspected. Ultrasonography showed bilateral solid adnexal mass, suspected to be an ovarian tumor.

FNAC of the right iliac fossa mass was performed. The smears showed moderately cellular aspirate comprising large number of tumor cells arranged singly and in loose clusters with granular eosinophilic to clear cytoplasm and round to oval nuclei [Figure 1]. Background showed plenty of sarcoid-like granulomas, epithelioid cells, few lymphocytes and plasma cells [Figure 1, inset]. Ziehl Neelsen stain for acid-fast bacilli was negative. A diagnosis of germ cell tumor of the ovary, possibly dysgerminoma, with plenty of sarcoid-like granulomas was offered.

Figure 1

Cytology smear showing tumor cells with granular eosinophilic cytoplasm and cells with clear cytoplasm (arrows) (Pap, ×400). Inset: Smear showing plenty of sarcoid-like granulomas (Pap, ×100)

Total abdominal hysterectomy with bilateral salphingoophorectomy was performed and the specimen was sent for histopathological examination. Grossly, bilateral ovarian masses were identified with the right- and left-sided masses measuring 8 × 6 × 3.5 cm and 7 × 5 × 2 cm, respectively. External surface of both the masses was nodular with cerebriform appearance, and the cut surface was solid, lobulated, grey white with yellowish areas. Right-sided mass showed areas of cystic and mucoid degeneration.

Microscopic examination confirmed the presence of dysgerminoma component with plenty of sarcoid-like granulomas in the stroma and revealed the presence of an additional minor component of yolk sac tumor involving both the ovaries [Figure 2]. Periodic acid Schiff stain showed both intracytoplasmic and extracellular hyaline droplets. A final diagnosis of mixed germ cell tumor with plenty of sarcoid-like granulomas was made.

Figure 2

Photomicrograph showing both dysgerminoma and yolk sac component in histology section (H and E, ×100)

Discussion

Germ cell tumors of ovary constitute about 20% of all ovarian tumors. Mixed germ cell tumors occur in about 8% of cases.[3] The usual combinations include teratoma with embryonal carcinoma or dysgerminoma with yolk sac tumor, although any combination of various elements may be seen.[13] Our case showed a combination of dysgerminoma with yolk sac tumor. A thorough sampling of the various parts of the tumor should be performed to identify relatively minor component and to make a diagnosis of mixed germ cell tumor on FNAC.[1] The present case showed only a component of dysgerminoma with sarcoid-like granulomas on FNAC, which is due to the predominance of this component as seen on histopathological examination.

Dysgerminoma constitutes less than 1% of all ovarian tumors and about 5% of malignant ovarian tumors.[3] Bilaterality is seen in 10–20% of cases. 60% are diagnosed in patients younger than 20 years.[4] About 20% of these cases are associated with sarcoid-like granulomas, which represent immunological response to the tumor.[25] Results suggest that γ/δ T cells accumulate in dysgerminoma and exhibit autologous tumor killing (ATK) activity through Vγ9/δ2 T cell receptor (TCR), and these γ/δ T cells also play a role in the formation of granulomatous inflammation associated with dysgerminoma.[6] Our case showed plenty of such granulomas which were dispersed throughout the cytology smear and within the stroma in histopathological section.

Distinction of germ cell tumors from other types of neoplastic and non-neoplastic lesions as well as distinction between the various subtypes of germ cell tumors may have an important therapeutic implication and may thus be crucial in planning proper management of the patient. FNAC of the gonadal germ cell tumor is frequently not performed as resection is usually required. However, occasionally when a gonadal mass does not require resection, as in lymphoma which can be treated by chemotherapy, FNAC is of help to diagnose this tumor and to rule out other lesions, including germ cell tumor.[1]

The present case was diagnosed on FNAC which is an effective, rapid and reliable procedure for the diagnosis of germ cell tumors. Presence of plenty of sarcoid-like granulomas in association with the tumor is a rare finding.