|Year : 2015 | Volume
| Issue : 4 | Page : 1024
Krukenberg tumor presenting with amenorrhea as the sole initial symptom: Case report and review of the literature
Suleyman Sahin, Fatih Karatas, B Hacioglu, A Aytekin, I Imamoglu, N Koseoglu, E Sari, M Altinbas
Department of Medical Oncology, Diskapi Yildirim Beyazid Education and Research Hospital, Ankara, Turkey
|Date of Web Publication||15-Feb-2016|
Irfan Bastug Street, Ulus/Diskapi, Ankara
Source of Support: None, Conflict of Interest: None
Krukenberg tumor (KT), mostly originates from gastric cancer, is the metastatic tumor of ovaries accounting for 1–2% of all ovarian cancer. Common presenting symptoms include abdominal pain, distension, and ascites. Rests of the patients have non-specific gastrointestinal symptoms including dyspepsia, weight loss, nausea and vomiting. Gynecologic symptoms such as virilization, menstrual bleeding or irregularity and amenorrhea are much less frequent in the literature cases. Here, we present an unusual case of KT presented with amenorrhea as the sole initial symptom.
Keywords: Amenorrhea, krukenberg, ovary
|How to cite this article:|
Sahin S, Karatas F, Hacioglu B, Aytekin A, Imamoglu I, Koseoglu N, Sari E, Altinbas M. Krukenberg tumor presenting with amenorrhea as the sole initial symptom: Case report and review of the literature. J Can Res Ther 2015;11:1024
|How to cite this URL:|
Sahin S, Karatas F, Hacioglu B, Aytekin A, Imamoglu I, Koseoglu N, Sari E, Altinbas M. Krukenberg tumor presenting with amenorrhea as the sole initial symptom: Case report and review of the literature. J Can Res Ther [serial online] 2015 [cited 2020 Feb 28];11:1024. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/1024/148679
| > Introduction|| |
Krukenberg tumor (KT) is an uncommon metastatic signet-ring cell adenocancer of the ovaries, which accounts for 1–2% of all ovarian tumors. This special type of carcinoma was first described in 1896 by Friedrich Ernst Krukenberg (1871-1946). In view of the literature, a signet-ring cell carcinoma, which produces mucin in the dense fibroblastic stroma of the ovary defined as KT. KT patients are generally young with an average age of 45 years. In 80% of the cases, tumor is bilateral. Much less frequently primary site of this tumor may be large intestine, breast, uterine cervix, appendix, or urinary bladder. Patients with KT mostly present with abdominal pain and distension. Some patients have non-specific gastrointestinal symptoms or are asymptomatic. Menstrual irregularities such as abnormal uterine bleeding or alterations of menstrual duration may be seen less frequently.
| > Case Report|| |
A 23-years-old female patient presented with the complaints of amenorrhea for about 4 months. On physical examination, abdominal distension and bilateral hard fixed masses were noted in the lower abdominal quadrant. Abdominal ultrasound identified severe ascites along with a big mass in the lower part of the abdomen. Toracoabdominal screening revealed an irregular tumoral wall thickness on the antro-pyloric level and a big mass size of 127 ± 122 mm filling all pelvic area [Figure 1]. Gastrointestinal endoscopy detected an ulcero-vegetating fragile mass lesion covered with spotted exudate on the small curvature of the stomach. Biopsy specimens were consistent with signet-ring cell adenocancer of the stomach. CA-125 level was 219.8 U/ml (six fold from normal range). Ovarian biopsy showed infiltration of signet-ring cell carcinoma stained positive for CK-7, focally positive for CK-20, intracytoplasmic positive for c-erb-B2 and positive for musicarmin that shows intracytoplasmic mucin in tumor cells. Histologically and morphologically, these findings were consistent with a diagnosis of gastric carcinoma with ovarian metastasis known as KT [Figure 2]̵[Figure 3] [Figure 4]. Due to patient's poor performance and disseminated disease, patient was not considered to be a candidate for chemotherapy or radiotherapy. So palliative best supportive care was performed. Unfortunately patient was lost of follow-up.
|Figure 1: A large mass extending to both ovary and filling completely all pelvic area|
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|Figure 3: Tumor cells are stained for neutrol mucin with mucicarmine (X200)|
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|Figure 4: Krukenberg tumor. Signet ring cells with cytoplasmic mucin vacuoles infiltrate ovarian stroma (H and E, x200)|
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| > Discussion|| |
KT mostly occurs in young women mostly in the fifth decade of their lives and common presenting symptoms are abdominal pain and distension. In most cases, primary tumor and its metastasis to ovary are diagnosed at the same time but in 20–30% cases, ovarian metastasis occurs after primary tumor resection. In our case, patient was 23-years-old, tumor was bilateral and primary tumor was diagnosed after ovarian metastasis. Although abdominal pain and distension were seen much more frequently at initial presentation, in our case the only initial symptom was amenorrhea. Surprisingly and rarely; abdominal pain, abdominal mass, ascites, nonspecific gastrointestinal symptoms and fatigue were absent at initial presentation. Three pathways have been reported for the possible metastasis mechanisms of this tumor; hematogenous, peritoneal seeding, and lymphatic route. Lymphatic route is the most common mechanism in metastasis.
Diagnosis of KT mostly depends on the light microscopic features such as dense fibroblastic stroma, which is diffusely infiltrated by malign signet-ring cells. Differential diagnosis must be made from the primary ovarian tumors. Immunochemical features may help to differentiate KT from primary ovarian cancers. CK7 and CK20 are commonly used two immuno parameters to distinguish metastatic ovarian neoplasias from primary ovarian tumors. Primary ovarian tumors usually tend to be positive for CK7 and negative for CK20 in contrast to metastatic gastric cancers. Metastatic gastric carcinomas tend to be positive for CK20 in the 70% of cases and much less frequently positive for CK7. In our case, immunophenotyping of the tumor was positive for CK7 and focally positive for CK20. Immunostaining for CK20 is expected to have more prominent positivity than CK7.
Up to day, the optimal treatment modality has not yet been established clearly. Surgery is the main treatment for the medically fit patients who have no metastasis. Chemotherapy and radiotherapy are found to have no significant effects on prognosis of KT. But in one study, Weiqi Lu et al., showed that use of postoperative aggressive chemotherapy is a favorable prognostic factor on survival of patients with Krukenberg. In another study Wei Peng et al., showed that if the primary tumor is resected and patient has no ascites then ovarian metastasectomy may prolong the survival time of the patients. Wei Peng et al., also recommend patients not to undergo ovarian metastasectomy if the primary tumor is unresected and the patient has ascites. It has been reported that serum levels of CA-125 may be helpful for the early diagnosis of Krukenberg disease. In our patient, level of CA-125 was 219.8 U/ml which was approximately 6-fold of the upper limit. KT has a poor prognosis with a median survival time of 14 months and patients die within 2 years.
| > Conclusion|| |
KT, known as the metastatic ovarian tumor, originating mostly from stomach has a very poor prognosis with an estimated survival of 14 months. Having resectable primary tumor and absence of residual metastatic lesion seem to be the most important prognostic factors for long-term survival. Yet the optimal treatment has not been established no curative treatment is currently available. Because of the extensive disease, it has a poor prognosis with a short median survival time. Chemotherapy and radiotherapy are palliative treatment options in advanced disease. We report this patient because of initially presenting only with amenorrhea for a few months duration without any other gastrointestinal symptoms like abdominal pain, discomfort, ascites or mass those are the most frequently presenting symptoms.
| > References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]