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Krukenberg tumor, also known as carcinoma mucocellulare, refers to the "signet ring" subtype of metastatic tumor to the ovary. The stomach followed by colon are the two most common primary tumors to result in ovarian metastases, pursued by the breast, lung, and contralateral ovary.
The tumors represent 5-10% of all ovarian tumors and up to 50% of all metastatic tumors to the ovary. The estimated incidence of Krukenberg tumor is at approximately 0.16/100000 per year. They tend to develop during the reproductive years 4-5.
Abdominal or pelvic pain, abdominal bloating, or pain during intercourse, may be the presenting symptom. Irregular bleeding may also be seen.
The median patient age at presentation is 45 years 8.
Krukenberg tumors are metastatic tumors to the ovary that contain well-defined histological characteristics (mucin-secreting “signet ring” cells) and usually originate in the gastrointestinal tract 4. The exact mechanism of primary tumor metastasize to the ovaries is unclear. However, several mechanisms have been proposed, such as through lymphatic channels, blood vessels, and peritoneal cavity 9.
The time from diagnosis of the primary neoplasm to the development of ovarian metastasis is variable and can range from several months to >10 years.
Cytologic examination often reveals mucoid degeneration and many large cells shaped like signet rings.
They can originate from 1:
stomach cancer (signet ring cells): most common
colorectal carcinoma: second most common
contralateral ovarian carcinoma
Most imaging features are non-specific, consisting of predominantly solid components or a mixture of cystic and solid areas. It is often difficult to differentiate from other ovarian neoplasms 1,4. There are a variety of metastatic carcinomas to the ovary that can mimic primary ovarian tumors 4.
These tumors are typically seen sonographically as:
bilateral, solid and sometimes cystic ovarian masses, with clear well-defined margins
an irregular hyper-echoic solid pattern and moth-eaten-like cyst formation are also considered characteristic features
CT appearances can be indistinguishable from primary ovarian carcinoma 2. Features will favor towards a Krukenberg tumor if a concurrent gastric or colic mural lesion is seen. There is some evidence that tumors originating from the stomach may be denser on contrast-enhanced CT than those originating from the colon 3.
The great majority of Krukenberg tumors are signet ring cell carcinomas arising in the stomach. Signet ring cells scatter in the ovarian stroma with abundant collagen formation or marked edema. Therefore, Krukenberg tumors can occasionally show low or high signal intensity on T2-weighted images 5.
Krukenberg tumors may demonstrate some distinctive findings on MRI, including:
bilateral complex masses with hypointense solid components (dense stromal reaction) 1,4
internal hyperintensity (mucin) on T1 and T2 weighted MR images 4
Strong contrast enhancement is usually seen in the solid component or the wall of the intratumoral cyst 5.
Treatment and prognosis
Differentiation between primary and metastatic ovarian carcinoma is of great importance with respect to treatment and prognosis but may be very difficult based on imaging findings solely.
It is a stage IV disease, and median overall survival is of the order of approximately 16 months 7,8. The breakdown by primary tumor location is as follows 7.
gastric: 11 months
colorectal: 21.5 months
breast: 31 months
other (appendix, gallbladder, small intestine, unknown): 19.5 months
Associated factors for poor prognosis and predictors of unfavorable outcomes include 7,8:
ovarian metastasis beyond the pelvis
History and etymology
It is named after the German pathologist Friedrich E Krukenberg (1871-1946) who first described them in 1896.
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