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| | | Year : 2009 | Volume : 52 | Issue : 3 | Page : 417-420 | | | Pure choriocarcinoma of ovary diagnosed by fine needle aspiration cytology | | | MR Naniwadekar, SR Desai, NS Kshirsagar, NN Angarkar, VD Dombale, SV Jagtap
Department of Pathology, Krishna Institute of Medical Sciences, Karad - 415 110, India
Click here for correspondence address and email | Date of Web Publication | 12-Aug-2009 | | |      | |  Abstract | | | Pure ovarian choriocarcinoma is extremely rare and can develop as a germ cell tumor or as a metastasis from uterine or tubal gestational choriocarcinoma or rarely from an ovarian pregnancy. The cytomorphologic findings have been reported previously in different sites. However, this is the first case of pure ovarian choriocarcinoma diagnosed on cytology to the best of our knowledge. The distinction between a gestational and nongestational choriocarcinoma is difficult. A 19-year-old female patient presented with an irregular per-vaginal bleeding and a mass in lower abdomen. Fine needle aspiration cytology smears of the mass were hypocellular and showed large, multinucleated giant cells and malignant mononucleated cells. Background was hemorrhagic. Serum β hCG level was 3,80,000 mIU/ml. A diagnosis of choriocarcinoma was offered which was later confirmed by histopathology. The diagnosis of choriocarcinoma on fine needle aspiration cytology is based on the presence of large, multinucleated giant cells and malignant mononucleated cells. A high index of suspicion should be maintained and estimation of serum β hCG plays a key role in supporting the diagnosis. Keywords: Choriocarcinoma, fine needle aspiration cytology, gestational choriocarcinoma, ovary, ovarian tumors How to cite this article:
Naniwadekar M R, Desai S R, Kshirsagar N S, Angarkar N N, Dombale V D, Jagtap S V. Pure choriocarcinoma of ovary diagnosed by fine needle aspiration cytology. Indian J Pathol Microbiol 2009;52:417-20 |
How to cite this URL:
Naniwadekar M R, Desai S R, Kshirsagar N S, Angarkar N N, Dombale V D, Jagtap S V. Pure choriocarcinoma of ovary diagnosed by fine needle aspiration cytology. Indian J Pathol Microbiol [serial online] 2009 [cited 2014 Mar 5];52:417-20. Available from: http://www.ijpmonline.org/text.asp?2009/52/3/417/55013 |
| Introduction | |  |
Choriocarcinoma is a rapidly invasive and widely metastasizing malignancy. As it responds well to chemotherapy, it is important to obtain early diagnosis. [1] Ovarian choriocarcinoma is quite a rare entity. [2] Most choriocarcinomas involving the ovary represent metastasis from uterine choriocarcinoma. It can develop from an ovarian pregnancy rarely and also as a germ cell tumor.
So far cytological findings in a case of choriocarcinoma of mediastinum, [3] breast [1],[4] and testis [5] have been reported. Diagnostic cytology findings are large giant cells having multiple, hyperchromatic nuclei, accompanied by large mononucleated cells having hyperchromatic, pleomorphic nuclei and moderate to ample amount of amphophilic or vacuolated cytoplasm.
We are reporting a case of pure choriocarcinoma of ovary diagnosed on fine needle aspiration cytology. This is the first case of choriocarcinoma of ovary diagnosed on fine needle aspiration cytology in the literature, to the best of our knowledge. Criteria and importance of assigning gestational or nongestational etiology are also discussed.
| Case Report | | |
A 19-year-old primipara was admitted with complaints of pain in abdomen and irregular per vaginal bleeding for four months and mass in lower abdomen for one month. The patient had delivered a female baby, one year back, who was on breast- feeding. Pallor was the only positive finding on general examination. On per-abdominal examination, a mass was palpable, which was arising from the pelvis, up to the umbilicus and was tense, cystic, minimally tender and fixed. On per- vaginal examination, a mass was felt through all the fornices, which was tense, tender and fixed. Uterus was not felt separately. On per-speculum examination, cervix and vagina appeared healthy and there was bleeding through cervix.
Clinical diagnosis was ovarian tumor. Laboratory investigations revealed 6.9 gm% hemoglobin and hypochromic, microcytic red cell morphology on peripheral smear examination. Radiograph of the chest was normal. Ultrasonography (USG) of pelvis showed a well-defined solid mass lesion arising in the left ovary measuring 18 ´ 16 ´ 7 cm, with lobulated margins and hyperechoic areas. The fat planes between the mass and the uterine wall could not be made out. Uterus measured 9 ´ 5 ´ 3 cm and was of normal shape and position. Endometrium appeared irregular and endometrial thickness was 0.8 cm. Right ovary measured 8 ´ 6 ´ 3 cm and showed multiple cysts with internal echoes. Fine needle aspiration was performed from right and left ovarian masses under USG guidance.
Cytology findings
The aspirate from the left ovarian mass was hemorrhagic. On microscopic examination, the smears were hypocellular. Multiple smears studied showed scattered large giant cells having multiple, large, hyperchromatic nuclei, accompanied by large, pleomorphic mononucleated cells having large, hyperchromatic, pleomorphic nuclei and moderate to ample amount of pale or vacuolated cytoplasm. Background was hemorrhagic [Figure 1].The aspirate from the right ovarian mass was hemorrhagic. Microscopic examination showed hemorrhagic and acellular smears.
With the fine needle aspiration cytology findings, choriocarcinoma was suspected and estimation of serum b hCG was advised. It was 3,80,000 mIU/ml. A diagnosis of high- grade malignancy, suggestive of choriocarcinoma was offered. The patient underwent exploratory laparotomy. Left ovarian mass was adherent to the sigmoid colon and omentum and was infiltrating retroperitoneally. Total hysterectomy with removal of bilateral ovarian masses with omentectomy was done. The postoperative period was uneventful.
Gross examination
The specimen consisted of panhysterectomy [Figure 2], the uterus and cervix measuring 9 ´ 5 ´ 3 cm. The endometrium measured 0.8 cm in its maximum thickness and appeared dark brown and hemorrhagic. A large, soft left ovarian mass measuring 18.5 ´ 16 ´ 7 cm was seen adherent to the uterine wall. External surface showed bosselations and marked congestion with hemorrhagic areas. Cut section showed dark brown solid appearance with extensive areas of necrosis and hemorrhages. Right ovarian mass measured 8.5 ´ 6 ´ 3 cm and showed bosselated surface. Cut section showed hemorrhagic cysts, the largest measuring 2 cm in diameter. The omentum measured 28 ´ 6 ´ 1 cm and showed areas of blackish discoloration.
Histopathological examination
Multiple sections of left ovarian mass studied showed a tumor with extensive areas of necrosis and hemorrhages and small foci of preserved tumor tissue, composed of clusters of cytotrophoblastic cells, separated by streaming masses of syncytiotrophoblasts. The cytotrophoblastic cells showed large, hyperchromatic nuclei with prominent nucleoli. Sections from the right ovarian mass showed theca lutein cysts. Multiple sections from the endomyometrium showed decidual reaction with no evidence of choriocarcinoma. The omentum was free of tumor. Patient received two cycles of chemotherapy comprising five days (EMA co-regime viz. etoposide, methotrexate, adriamycin, cyclophosphamide and vincristine). She was recovering well, however, was lost to follow-up after second course of chemotherapy.
| Discussion | | |
Pure choriocarcinoma is a very rare tumor. Cytological findings in case of choriocarcinoma in different sites such as testis, [5] mediastinum [3] and breast [1],[4] have been described. At these sites, cytological smears showing large, pleomorphic, mononucleated cells with indistinct cell borders, pale or vacuolated cytoplasm and round, hyperchromatic, moderately pleomorphic nuclei and large, multinucleated giant cells with hyperchromatic nuclei of varying sizes and abundant, amphophilic cytoplasm have been described, which correspond to cytotrophoblastic cells and syncytiotrophoblastic cells respectively.
Differential diagnosis includes epithelial malignancy with giant cell change. However, serum b hCG levels are helpful because they are markedly elevated with choriocarcinoma but are only minimally elevated with other neoplasms.
According to Choi et al ., [1] the cytological findings are quite characteristic to diagnose choriocarcinoma, especially if there is history of pregnancy or serum b hCG level is raised. [1]
In the present case, clinical history, serum b hCG levels and fine needle aspiration cytology findings suggested a diagnosis of choriocarcinoma, which was confirmed on histopathology. We did not come across fine needle aspiration cytology findings of choriocarcinoma of ovary in the literature.
Ovarian choriocarcinoma is quite a rare entity. [2] Choriocarcinoma of the ovary may be gestational or nongestational in origin. Gestational choriocarcinoma can be preceded by hydatidiform mole, partial mole, ectopic pregnancy, nonmolar intrauterine abortion or term pregnancy. In the latter instance, which is exceedingly rare, the tumor may appear as masses in an otherwise normal placenta or develop following delivery. [6] Gestational choriocarcinoma of ovary (GCO) can occur due to metastasis from uterine or tubal choriocarcinoma or from malignant change in an ovarian pregnancy. [7] The estimated incidence of GCO is as rare as one in 369 million. [2]
Nongestational choriocarcinoma of the ovary (NGCO) is extremely rare and the pure type is less frequent than the mixed type consisting of other germ cell tumors. Only 29 cases had been reported up to 2001. [8] The first case of NGCO was described in combination with teratoma in a nine-year-old girl by Pick in 1904. [2]
Distinction between a gestational and nongestational choriocarcinoma is difficult. Diagnosis of nongestational choriocarcinoma of the ovary is very difficult in the reproductive age group. [9] Serologically, NGCO does not present with serum b hCG values as high as those seen in the GCO. Histologically, multiple blocks and multiple sections need to be taken to identify the germ cell elements other than choriocarcinoma if any, to diagnose NGCO. It is important to differentiate GCO from NGCO, as NGCO has a worse prognosis and needs more aggressive therapy than GCO. [2] Pure NGCOs are often lethal.
The etiology of ovarian choriocarcinoma in a sexually active woman can be established definitely only by the demonstration of HLA typing for paternal antigens in trophoblastic elements. As these techniques are expensive and are not available at all medical centers, it is of limited application.
The presence of corpus luteum or theca lutein cysts in the same and/or other ovary have been considered as a tumor-induced phenomenon due to the excess production of human chorionic gonadotropin.
The present case being a woman in the reproductive age group, with history of pregnancy one year back, a very high level of serum b hCG, presence of pure choriocarcinoma in one ovary and theca lutein cysts in contralateral ovary point towards a diagnosis of GCO. Wheeler et al . [10] have come across a similar case showing a large necrotic ovarian choriocarcinoma, along with large theca lutein cysts. Falsely low serum b hCG values were found due to the high-dose "hook effect" in the double antibody immunoassay system. [10]
Choriocarcinoma is treated by chemotherapy with or without surgery. Treatment for ovarian choriocarcinoma consists of removal of the affected ovary and hysterectomy to debulk the pelvic tumor volume. [2] Surgery is also useful in controlling life-threatening hemorrhage from metastatic lesions. GCOs are treated with single-agent chemotherapy with methotrexate or actinomycin D, whereas GCOs with high-risk factors or extensive metastasis and NGCOs are treated with triple- agent chemotherapy [2] or multidrug regimes (viz. EMA- co) as administered in this patient. However, surgical staging of ovarian choriocarcinoma is clinically more useful in initiating specific therapy than determining the gestational or nongestational etiology of the tumor. [2]
The presence of any risk factor such as duration for more than four months, pretherapy level of b hCG in serum more than 40,000 IU ml, brain/liver metastasis and gestational trophoblastic disease, after term gestation, carry bad prognosis in metastatic gestational choriocarcinoma. [6]
In the present case, serum b hCG level of 3,80,000 mIU/ml, duration of disease for one year and antecedent full-term pregnancy were major risk factors. Therefore total hysterectomy with right oophorectomy and removal of left ovarian mass with omentectomy was done. Patient was treated with two cycles of multiagent chemotherapy. However, the patient was lost to follow-up.
In conclusion, ovarian choriocarcinoma is quite rare. Pure choriocarcinoma of the ovary has not been reported on fine needle aspiration cytology before. Though the smears were hemorrhagic and hypocellular, the mixed population of, large multinucleated giant cells and large, pleomorphic mononucleated cells aided the diagnosis. A high index of suspicion and adequate screening of multiple smears is essential for making the diagnosis of choriocarcinoma. To support the diagnosis, estimation of serum b hCG should be performed.
| References | | |
| 1. | Choi HJ, Park IA. FNAC of metastatic choriocarcinoma, presenting as a breast lump-case report. Acta Cytol 2004;48:91-4.  [PUBMED] | | 2. | Babu MK, Kini U. Choriocarcinoma of the ovary in a post menopausal woman. Indian J Cancer 1996;33:111-6. [PUBMED] | | 3. | Celeste N. Powers, Kristen A. Atkins. Aspiration of the mediastinum. In: Barbara F. Atkinson,editor. Atlas of Diagnostic Cytopathology. 2 nd ed. Philadelphia: Saunders; printed in China. 2004. p. 396. | | 4. | Kumar PV, Esfahani FN, Salimi A. Choriocarcinoma metastatic to the breast diagnosed by fine needle aspiration. Acta Cytol 1991;35:239-42. [PUBMED] | | 5. | Orell SR, Sterrett GF, Walters MN,Whitaker D, editors. Male and female genital organs. In: Manual and Atlas of fine needle aspiration cytology. 3 rd ed. London: Churchill Livingston;1999. p. 354-5. | | 6. | Rosai J. Female eproducive System. In: Rosai J, editor. Rosai and Ackerman's surgical pathology. Vol. 2. 9 th ed. New Delhi: Mosby; 2004. p.1745-6. | | 7. | Vance RP, Geisinger KR. Pure nongestational choriocarcinoma of the ovary. Report of a case. Cancer 1985;56:2321-5. | | 8. | Goswami D, Sharma K, Zutshi V, Tempe A, Nigam S. Nongestational pure ovar]ian choriocarcinoma with contralateral teratoma. Gynecol Oncol 2001;80:262-6. [PUBMED] [FULLTEXT] | | 9. | Simsek T, Trak B, Tunc M, Karaveli S, Uner M, Sonmez C. Primary pure choriocarcinoma of the ovary in reproductive ages: A case report. Eur J Gynaecol Oncol 1998;19:284-6. | | 10. | Wheeler CA, Davis S, Degefu S, Thorneycroft IH, O'Quinn AG. Ovarian choriocarcinoma: A difficult diagnosis of an unusual tumor and a review of the hook effect. Obstet Gynecol 1990;75:547-9. [PUBMED] |
Correspondence Address:
M R Naniwadekar
Naniwadekar Hospital, Market Yard, Shaniwar Peth, Karad - 415 110, Dist - Satara, Maharashtra
India
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DOI: 10.4103/0377-4929.55013 PMID: 19679980
[Figure 1], [Figure 2] | |
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