Advertisement

Metastatic choriocarcinoma as initial presentation of small bowel perforation in absence of primary uterine lesion: a case report

Abstract

Background

Choriocarcinoma is a highly malignant tumor of gestational trophoblastic neoplasia that characteristically spreads via the bloodstream. Small bowel metastasis is very rare, and a small number of cases of choriocarcinoma metastasis to small bowel have been reported.

Methods

We report a case of a 40-year-old woman presenting with acute abdominal pain and vaginal bleeding due to small bowel perforation secondary to jejunal metastasis. In our case, metastatic choriocarcinoma was present in the small bowel, lung, and liver, but no primary lesion was apparent.

Results

After resection and anastomosis of the perforated small bowel, chemotherapy was performed. The patient began chemotherapy with etoposide, methotrexate, actinomycin D, cyclophosphamide, and vincristine (EMA-CO) for 9 cycles. When the 4th cycle of chemotherapy was finished, β-human chorionic gonadotropin (hCG) level was normalized and follow-up computed tomography scans showed regression of the liver metastasis and small bowel lesion and significantly decreased lung metastasis. After 9 cycles of chemotherapy were completed, the patient showed complete response.

Conclusions

If patients have high β-hCG of unknown origin and suspected panperitonitis, which are suspicious of choriocarcinoma metastasis to small bowel, the pathologic findings are important. It is essential to confirm the histopathologic diagnosis postoperatively. Choriocarcinoma can spread to various organs and show aggressive manifestations. Therefore, we should be aware of possible metastatic sites and remain alert to its diagnosis.

Tumori 2016; 102(Suppl. 2): e87 - e89

Article Type: CASE REPORT

DOI:10.5301/tj.5000379

Authors

En Bee Cho, Jung Mi Byun, Dae Hoon Jeong, Hye Kyoung Yoon, Young Nam Kim, Moon Su Sung, Kyung Bok Lee, Ki Tae Kim

Article History

Disclosures

Financial support: None.
Conflict of interest: None.

This article is available as full text PDF.

Download any of the following attachments:

Introduction

Choriocarcinoma is an aggressive malignant tumor with a characteristic of hematogenous metastases to lung, vagina, central nervous system, liver, and gastrointestinal tract. Fewer than 5% of patients present with choriocarcinoma metastasis to the gastrointestinal system, and among them, metastatic choriocarcinoma of the small bowel is particularly rare (1). Choriocarcinoma most commonly arises from the uterus, but not all patients have a demonstrable lesion in the uterus. Therefore, symptoms related to the sites of metastasis may be the first presentation.

We report a rare case of metastatic choriocarcinoma presenting with acute abdominal pain and vaginal bleeding due to small bowel perforation secondary to jejunal metastasis in the absence of a primary uterine lesion.

Case report

A 40-year-old woman, gravida 4, para 2, abortion 2, was admitted to the emergency department with acute abdominal pain and vaginal bleeding similar to menstruation. Two years earlier, she had undergone dilation and curettage for hydatidiform mole and treated with a single dose of methotrexate. She remained asymptomatic for 2 years. One month prior to admission, she had melena and was diagnosed with anemia. Although esophagogastroduodenoscopy, colonofiberscopy, and capsule endoscopy were performed, no abnormal findings were found. One day prior to admission, she developed whole-abdominal pain and vaginal bleeding. On physical ­examination, she had direct and rebound tenderness of the whole abdomen, and the cervix and vaginal wall were intact. Computed tomography (CT) scanning of the abdomen-pelvis revealed jejunal perforation due to a mass, suspected to be lymphoma, and multiple metastatic masses in the lung and liver (Fig. 1). However, no intrauterine lesion was observed on CT scan or transvaginal ultrasonography. The initial serum β-human chorionic gonadotropin (β-hCG) level was 8,837 mIU/mL.

Computed tomography (CT) of the abdomen and pelvis. A) Contrast-enhanced CT shows irregular wall thickening with enhancement at the distal jejunum and proximal ileum. B) A 3.2-cm mass with central low attenuation in right lower lobe. C) Multiple hypodense lesions at both lobes of the liver.

The patient underwent segmental resection and end-to-end anastomosis to remove the ruptured mass of the jejunum. Diagnostic curettage was performed to evaluate vaginal bleeding with elevated β-hCG level and previous history of hydatidiform mole. The pathologic diagnosis was metastatic choriocarcinoma of the jejunum, but the results of curettage were not specific (Fig. 2). Postoperative β-hCG level was decreased to 4,089 mIU/mL. Seven days after the operation, β-hCG level increased to 9,906 mIU/mL, and the patient demonstrated hypotension and decreased hemoglobin, from 12.3 g/dL to 8.8 g/dL. A CT scan revealed hepatic rupture with active bleeding due to metastatic choriocarcinoma, and emergency embolization of the S6 segmental artery was performed using glue and Lipiodol. Two days after embolization, the patient was in a clinically stable condition. Therefore, she began chemotherapy with etoposide, methotrexate, actinomycin D, cyclophosphamide, and vincristine (EMA-CO) for 9 cycles. When the 4th cycle of chemotherapy was finished, β-hCG level was normalized and follow-up CT scans showed regression of the liver metastasis and small bowel lesion and significantly decreased lung metastasis. After 9 cycles of chemotherapy were completed, the patient showed complete response.

A) Small bowel mucosa shows an ulcerofungating mass, measuring 2.5 × 1.9 cm. B) The tumor cells consist of atypical cytoblasts and syncytiotrophoblasts, with occasional mitotic figures (×200).

Discussion

Choriocarcinoma is a rare and highly malignant form of gestational trophoblastic disease. Gestational choriocarcinoma is preceded by a pregnancy; 50% arise from hydatidiform mole, 25% from prior abortions, 22% from normal pregnancies, and 3% from ectopic pregnancies or teratomas (2). Two factors have consistently been associated with an increased risk of gestational trophoblastic disease: maternal age and history of hydatidiform mole (3). The risk associated with maternal age is bimodal, with increased risk both for mothers under 20 and over 35 years old (3). In our case, the patient had a history of hydatidiform mole and was 40 years old, thus demonstrating risk factors, but she did not receive regular follow-up for the previous hydatidiform mole for 2 years.

The symptoms of choriocarcinoma vary depending on the disease site. Primary gestational choriocarcinoma usually arises in the uterus with atypical vaginal bleeding, but gestational choriocarcinoma that primarily occurs outside the uterus is rare (4). The lung, liver, brain, vagina, and gastrointestinal tract are common sites of metastasis (5), but metastatic choriocarcinoma of the small bowel is rare (1). Only 16 cases of small intestinal choriocarcinoma have been reported (1, 6, 7), but it is difficult to be certain from these reports whether the small intestinal tumors were primary or metastatic (8). We could not detect genital tumors in our case, but the ­history of previous hydatidiform mole suggests a diagnosis of metastatic choriocarcinoma of the jejunum.

The majority of patients with choriocarcinoma respond to chemotherapy, but prognosis is worse for those patients presenting with small bowel metastasis. In our case, metastatic choriocarcinoma was present in the small bowel, lung, and liver, but no primary lesion was apparent. However, our patient demonstrated a good response to chemotherapy, and after 4 cycles, serum hCG level had normalized.

Diagnosis of metastatic choriocarcinoma of the small bowel requires suspicion and a multidisciplinary approach to its management in order to reduce patient morbidity and mortality. The imaging study that is carried out will depend on the type of presentation (hemorrhage, obstruction, or perforation).

Treatment varies according to the manifestation in patients and severity. Immediate surgical intervention by laparotomy may be life-saving in situations with suggested intestinal perforation, unexplained intestinal bleeding despite adequate imaging, or limitations in diagnostic facilities.

To our knowledge, this case is the first small bowel perforation secondary to jejunal metastasis of choriocarcinoma in the absence of a primary uterine lesion to be reported in South Korea. Therefore, the case is being reported for its rarity. If patients are treated for hydatidiform mole, regular follow-up could be warranted. Additionally, if patients have high β-hCG of unknown origin and suspected panperitonitis, which are suspicious of choriocarcinoma metastasis to small bowel, the pathologic finding is important. It is essential to confirm the postoperative histopathologic diagnosis. Choriocarcinoma can spread to various organs and show aggressive manifestations. Therefore, we should be aware of all possible metastatic sites and remain alert to diagnosing this phenomenon.

Disclosures

Financial support: None.
Conflict of interest: None.
References
  • 1. Iyomasa S Senda Y Mizuno K etal. Primary choriocarcinoma of the jejunum: report of a case. Surg Today 2003 33 12 948 951 Google Scholar
  • 2. Brewer JI Mazur MT Gestational choriocarcinoma. Its origin in the placenta during seemingly normal pregnancy. Am J Surg Pathol 1981 5 3 267 277 Google Scholar
  • 3. Altieri A Franceschi S Ferlay J Smith J La Vecchia C Epidemiology and aetiology of gestational trophoblastic diseases. Lancet Oncol 2003 4 11 670 678 Google Scholar
  • 4. Wan X Li J Xie X Extrauterine choriocarcinoma of the greater omentum after tubal pregnancy: case report. Int J Gynecol Cancer 2006 16 3 1476 1478 Google Scholar
  • 5. Berkowitz RS Goldstein DP Chorionic tumors. N Engl J Med 1996 335 23 1740 1748 Google Scholar
  • 6. Simpson L Sundaresan R Vohra M et al. Invasive choriocarcinoma involving the major duodenal papilla. Gastrointest Endosc 2005 61 7 926 928 Google Scholar
  • 7. Yokoi K Tanaka N Furukawa K et al. Male choriocarcinoma with metastasis to the jejunum: a case report and review of the literature. J Nippon Med Sch 2008 75 2 116 121 Google Scholar
  • 8. Suekane T Oshitani N Okazaki H Maeda K Ohsawa M Arakawa T A case of jejunal choriocarcinoma detected by capsule endoscopy and double-balloon endoscopy. Endoscopy 2010 42 Suppl 2 E52 E53 Google Scholar

Authors

Affiliations

  • Department of Obstetrics and Gynecology, Busan Paik Hospital, Inje University, Busan - Korea
  • Paik Institute for Clinical Research, Busan Paik Hospital, Inje University, Busan - Korea
  • Department of Pathology, Busan Paik Hospital, Inje University, Busan - Korea

Article usage statistics

The blue line displays unique views in the time frame indicated.
The yellow line displays unique downloads.
Views and downloads are counted only once per session.

No supplementary material is available for this article.