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.
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.
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.
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
AuthorsEn Bee Cho, Jung Mi Byun, Dae Hoon Jeong, Hye Kyoung Yoon, Young Nam Kim, Moon Su Sung, Kyung Bok Lee, Ki Tae Kim
- • Accepted on 03/06/2015
- • Available online on 10/07/2015
- • Published online on 11/11/2016
This article is available as full text PDF.
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.
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 (
Computed tomography (CT) of the abdomen and pelvis.
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
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
- Cho, En Bee [PubMed] [Google Scholar] 1
- Byun, Jung Mi [PubMed] [Google Scholar] 1, 2, * Corresponding Author (email@example.com)
- Jeong, Dae Hoon [PubMed] [Google Scholar] 1, 2
- Yoon, Hye Kyoung [PubMed] [Google Scholar] 3
- Kim, Young Nam [PubMed] [Google Scholar] 1, 2
- Sung, Moon Su [PubMed] [Google Scholar] 1
- Lee, Kyung Bok [PubMed] [Google Scholar] 1
- Kim, Ki Tae [PubMed] [Google Scholar] 1, 2
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