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CASE REPORT  
Year : 2013  |  Volume : 56  |  Issue : 3  |  Page : 300-302
Placental site trophoblastic tumor in the pelvic wall: A case report and review of the literature


1 Department of Pathology, West China Second University Hospital, Sichuan University, Chengdu, China
2 Department of Ultrasound, Nanchong Central Hospital, Nanchong, Sichuan, China

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Date of Web Publication24-Oct-2013
 

   Abstract 

Extra-uterine placental site trophoblastic tumor (PSTT) is extremely rare. To our knowledge, PSTT that occurs in the pelvic wall has not yet been reported. A 29-year-old woman presented with amenorrhea and irregular vaginal bleeding of 1 month. A solid tumor mass was detected by ultrasonography in the right pelvic wall. The tumor was comprised of large, polygonal tumor cells, with brisk mitosis and obvious vascular invasion. Immunohistochemical staining demonstrated that tumor cells were positive for human placental lactogen, CD146, cytokeratin, placental alkaline phosphatase, human chorionic gonadotropin were positive, the Ki-67 proliferative index was about 80%. The pathological diagnosis was PSTT. After the operation, the patient was treated with six cycles of etoposide, methotrexate, actinomycin, cyclophosphamide, and vincristine. The patient was followed for 18 months without recurrence. The report shows that extra-uterine PSTT is extremely rare and may have a good prognosis; surgical resection and adjuvant chemotherapy are good options. However, further experience to diagnose and cure this rare tumor is warranted.

Keywords: Extra-uterine, pelvic, placental site trophoblastic tumor

How to cite this article:
Tang X, Yang F, Jia L, Yao XY, Yang KX. Placental site trophoblastic tumor in the pelvic wall: A case report and review of the literature . Indian J Pathol Microbiol 2013;56:300-2

How to cite this URL:
Tang X, Yang F, Jia L, Yao XY, Yang KX. Placental site trophoblastic tumor in the pelvic wall: A case report and review of the literature . Indian J Pathol Microbiol [serial online] 2013 [cited 2020 Jun 4];56:300-2. Available from: http://www.ijpmonline.org/text.asp?2013/56/3/300/120405



   Introduction Top


Placental site trophoblastic tumor (PSTT) is the rarest form of gestational trophoblastic disease that accounts for 1-2% of trophoblastic tumor, typically occurring in the uterus. [1] Extra-uterine PSTTs are extremely rare. Up to date, there have only been eight cases reported in the literature. The occurrence sites included fallopian tube, ovary, and the pouch of Douglas. [2] To the best of our knowledge, a PSTT that occurs in the pelvic wall has not been reported before. Here, we present this rare case with its clinicopathologic features, and relevant literatures are reviewed.


   Case Report Top


A 29-year-old woman, gravida 4, para 0, presented with amenorrhea and irregular vaginal bleeding for 1 month. Four years ago she had 1-month amenorrhoea, since then the menstrual periods were irregular (longer than 28 days). The serum β-human chorionic gonadotropin (hCG) level was persistently variable from 247 to 1722 mIU/ml (normal < 10 mIU/ml) during these years. Ultrasonography showed an irregular echogenic mass (3.8 × 3.0 cm) lesion in the right pelvic wall with increased vascularity [Figure 1]. Ultrasound examination of the uterus, hysteroscopy, and the chest X-ray of the lungs showed no abnormality. A laparotomy was performed. Intra-operatively, a dark-red, hard, 4.0 × 3.5 × 4.0 cm mass was located in the right pelvic wall, tightly adherent to the right broad ligament and parametrium. The uterus and bilateral tubes and ovaries were normal in appearance. The mass measured 4.0 × 2.5 × 2.2 cm in size. Grossly, the tumor was round, solid, with non-circumscribed border, and showed yellow fleshy cut surface, accompanied by foci of hemorrhage and necrosis. Microscopically, the tumor was comprised of large and polygonal tumor cells, hyperchromatic with vesicular nuclei and abundant eosinophilic cytoplasm [Figure 2]. Obviousvascular invasion and severe nuclear atypia were also observed [Figure 3]. The mean mitotic count was five mitoses per 10 high-power fields (HPFs). Inmunohistochemically, the tumor cells showed strong diffuse positivity for human placental lactogen (hPL), CD146, cytokeratin, and placental alkaline phosphatase (PLAP), focal moderate positivity for hCG, and α-inhibin, the Ki-67 proliferative index was about 80% [Figure 4]a-d. The pathological diagnosis was PSTT.
Figure 1: Ultrasonography revealed a cystic mass (2.6 × 2.3 cm) in the right ovary and a irregular echogenic mass (3.8 × 3.0 cm) in the right pelvic wall with increased vascularity, which was located in the lateral of right ovary

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Figure 2: Microscopic appearance of the tumor shows monophasic cellular pattern of intermediate trophoblast. (Hematoxylin– eosin, ×400)

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Figure 3: The tumor cells were invading the blood vessels (arrow). (Hematoxylin eosin, 400)

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Figure 4: The immunohistochemical stains show diff using positive for (a) hPL, (b) CK, (c) CD146, and (d) with a high Ki - 67 proliferati ve index (400)

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Postoperatively, the patient was treated with six cycles of standard EMA-CO (etoposide, methotrexate, actinomycin, cyclophosphamide, and vincristine) chemotherapy, the serum β-hCG level decreased to the normal range, and the patient has been free of disease for 18 months after the end of the treatment.


   Discussion Top


Extra-uterine PSTTs are extremely unusual. We summarized all the eight cases occurred outside the uterine and added an additional case of extra-uterine PSTT [Table 1]. [2],[3],[4],[5],[6],[7] Most of extra-uterine pelvic lesions of intermediate trophoblast were located in the fallopian tube. Gupta et al. presumed that they may originate from previous ectopic pregnancies. [6] However, in this series of cases, only two patients had the history of ectopic pregnancy, most of which occurred after the full-term normal delivery. Also in our case, the tumor was located in the pelvic wall. The patient had miscarriages for four times, and had a synchronous intrauterine pregnancy for 49 days, without history of ectopic pregnancy. The origin of PSTT was most likely related to spontaneous abortion. Thus, more cases are needed to investigate the pathogenesis of extra-uterine PSTT.
Table 1: Cases of extra-uterine PSTTs reported in the literature

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PSTT is often associated with low levels of serum hCG. Only three cases can observe the serum hCG level (37.5 mIU/ml, 581 IU/l, and 2602 mIU/ml). In our case, the serum β-hCG level was persistently variable from 247 to 1722 mIU/ml. Therefore, it has a predictive value to monitor the course of the disease, the response to treatment, and detects recurrence of the extra-uterine PSTTs.

Microscopically, the appearance of tumor cells were similar to the uterine PSTTs. Immunohistochemically, PSTT is diffuse positive for hPL, CD146, and cytokeratin, and only focally positive for hCG and PLAP. In our case, The Ki-67 proliferative index was about 80%, obviously higher than that in most typical PSTTs (14 ± 6.9%). [8] It was likely indicated a malignant behavior. However, it is not clear whether it has a significant prognostic value or not.

The FIGO stage is the most important prognostic factor. [9] However, it is not applied to these rare tumors. In our case, the tumor occurred outside the uterus, with no evidence of metastasis to other organs. In addition, some prognostic factors mentioned may predict a poor outcome, including the interval to the preceding pregnancy nearly 2 years ago; the serum β-hCG level was elevated to 1722 mIU/ml (>1000 mIU/ml); histologically, both the tumor cells and the nuclei showed pleomorphism-variation in the cell size and shape; mitotic figures were 5/10 HPFs; the Ki-67 proliferative index was about 80%. While the tumor located in the pelvic wall with no evidence of metastases, and all extra-uterine PSTTs reported so far were benign. Hence, more cases are needed to reliably estimate the behavior of these rare tumors.

PSTT is not very sensitive to chemotherapy. Surgical resection is the main therapy. Adjuvant chemotherapy like EMA-CO and EMA-EP (etoposide, methotrexate, actinomycin, and cisplatin) is also a good option for patients desiring future fertility. In this series of cases, only one case received the chemotherapy, the regimen was not in detail. In our case, the patient received six cycles of standard EMA-CO chemotherapyafter the tumor excision, the serum β-hCG level was within normal ranges. The patient has been free of disease for 18 months.We presume that extra-uterine PSTT may have a good prognosis, and surgical resection complicated with adjuvant chemotherapy is good option to this rare tumor.

In conclusion, extra-uterine PSTT is an extremely rare tumor. We presented that a previously unreported PSTT occurs in the pelvic wall. Further experience to diagnose and cure this rare tumor is warranted.

 
   References Top

1.Ajithkumar TV, Abraham EK, Rejnishkumar R, Minimole AL. Placental site trophoblastic tumor. Obstet Gynecol Surv 2003;58:484-8.  Back to cited text no. 1
    
2.Baergen RN, Rutgers J, Young RH. Extrauterine lesions of intermediate trophoblast. Int J Gynecol Pathol 2003;22:362-7.  Back to cited text no. 2
    
3.Kjer JJ, Iversen T, Haarstad I. Malignant trophoblastic cell tumor localized to the fallopian tube: Acase report. Eur J Obstet Gynecol Reprod Biol 1991;39:163-4.  Back to cited text no. 3
    
4.Su YN, Cheng WF, Chen CA, Lin TY, Hsieh FJ, Cheng SP, et al. Pregnancy with primary tubal placental site trophoblastic tumor - A case report and literature review. Gynecol Oncol1999;73:322-5.  Back to cited text no. 4
    
5.El Hag IA, Ramesh K, Kollur SM, Salem M. Extrauterine placental site trophoblastic tumour in association with a lithopedion. Histopathology 2002;41:446-9.  Back to cited text no. 5
    
6.Gupta N, Mittal S, Misra R, Vimala N, Das AK. Placental site trophoblastic tumor originating in a tubal ectopic pregnancy. Eur J Obstet Gynecol Reprod Biol 2006;129:92-4.  Back to cited text no. 6
    
7.Arroyo MR, Podda A, Cao D, Rodriguez MM. Placental site trophoblastic tumor in the ovary of a young child with isosexual precocious puberty. Pediatr Dev Pathol 2009;12:73-6.  Back to cited text no. 7
    
8.Shih IM, Kurman RJ. The pathology of intermediate trophoblastic tumors and tumor-like lesions. Int J Gynecol Pathol 2001;20:31-47.  Back to cited text no. 8
    
9.Chang YL, Chang TC, Hsueh S, Huang KG, Wang PN, Liu HP, et al. Prognostic factors and treatment for placental site trophoblastic tumor-report of 3 cases and analysis of 88 cases. Gynecol Oncol 1999;73:216-22.  Back to cited text no. 9
    

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Correspondence Address:
Kai-Xuan Yang
Department of Pathology, West China Second University Hospital, Sichuan University, Chengdu, Sichuan - 610 041
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.120405

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