Krukenberg tumor

Krukenberg tumor
Krukenberg tumor
ICD-10	C56
ICD-9	183
ICD-O:	8490/6
DiseasesDB	30081
MeSH	C04.557.470.200.025.415.410

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Soujanya Thummathati, MBBS [2]

Synonyms and keywords: carcinoma mucocellulare; Synonym 2; Synonym 3

Overview

Krukenberg's tumor is a rare metastatic signet ring cell adenocarcinoma of the ovary.

Historical Perspective

• Krukenberg's tumor was first described as a new type of primary ovarian malignancy by Friedrich Ernst Krukenberg (1871–1946), a German gynecologist and pathologist, in 1896 which was later confirmed to be of metastatic gastrointestinal tract origin.

Pathophysiology

• The majority of Krukenberg’s tumors are bilateral.
• Stomach is the primary site in the majority of Krukenberg tumor cases (70%).
• The pathogenesis of krukenberg tumors is from metastasis of tumor cells from the stomach, appendix or colon to the ovaries.
  • Metastasis is more likely via the lymphatic spread. However, direct seeding across the abdominal cavity may also occur.
• On gross pathology, asymmetrically enlarged ovaries with a bosselated contour, usually solid, yellow or white cross sectioned surfaces, and the absence of adhesions or peritoneal deposits are characteristic findings of krukenberg tumors.
• On microscopic histopathological analysis, krukenberg tumors are characterized by the following features:
  • Tumor composed of two components:
    • Epithelial
      • Mucin-secreting signet ring cells with eccentric hyperchromatic nuclei
      • Cytoplasm may be eosinophilic and granular, pale and vacuolated, or a bull's eye (targetoid) appearance with a large vacuole with a central to paracentral eosinophilic body composed of a droplet of mucin
      • Signet ring cells may be single, clustered, nested, or arranged in tubules, acini, trabeculae, or cords
    • Stromal
      • Plump and spindle-shaped cells with minimal cytologic atypia or mitotic activity
      • Focal or diffuse stromal edema which may form pseudo cysts
      • Desmoplastic reaction may be present
• Stomach is the primary site in the majority of Krukenberg tumor cases (70%).

Causes

• Krukenberg's tumor may be caused by metastasis of the tumor cells from a primary cancer in the stomach, appendix, or the colon via:
  • Lymphatic spread; or
  • Direct seeding across the abdominal cavity

Differentiating [disease name] from other Diseases

• Krukenberg's tumor must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics

• The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
• In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

• Patients of all age groups may develop [disease name].

• Krukenberg's tumor is more commonly observed among patients aged [age range] years old.
• Krukenberg's tumor is more commonly observed among [elderly patients/young patients/children].

Gender

• Krukenberg's tumor affects men and women equally.

• [Gender 1] are more commonly affected with [disease name] than [gender 2].
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

• There is no racial predilection for [disease name].

• Krukenberg's tumor usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop [disease name].

Risk Factors

• Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally poor, and the 5 ­year survival rate of patients with krukenberg tumor is lower in patients in with a preoperative serum CA 125 levels greater than 75 U/mL when compared with patients with CA 125 levels less than 75 U/mL.

Diagnosis

Diagnostic Criteria

• The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• Krukenberg's tumor is usually asymptomatic.
• Symptoms of [disease name] may include the following:

• [symptom 1]
• [symptom 2]
• [symptom 3]
• [symptom 4]
• [symptom 5]
• [symptom 6]

Physical Examination

• Patients with [disease name] usually appear [general appearance].
• Physical examination may be remarkable for:

• [finding 1]
• [finding 2]
• [finding 3]
• [finding 4]
• [finding 5]
• [finding 6]

Laboratory Findings

• There are no specific laboratory findings associated with [disease name].

• A [positive/negative] [test name] is diagnostic of [disease name].
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
• Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

• There are no [imaging study] findings associated with [disease name].

• [Imaging study 1] is the imaging modality of choice for [disease name].
• On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

Immunohistochemistry

• Krukenberg's tumor may also be diagnosed using immunohistochemistry.
• Findings on immunohistochemistry include:
  • Cytokeratins (AE1/AE3) positive
  • Epithelial membrane antigen positive
  • Vimentin negative
  • Inhibin negative

=Serum CA-125

• Serum concentrations of CA 125 may be helpful for:
  • Post-operative follow-up of patients for evaluation of complete resection of the tumor
  • Follow-up of patients with a history of primary adenocarcinomas (particularly gastrointestinal) for early detection of ovarian metastasis

Treatment

Medical Therapy

• There is no treatment for [disease name]; the mainstay of therapy is supportive care.

• The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for [disease name].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

Case Studies

Case #1