Krukenberg tumor from an appendiceal carcinoma presenting as a primary ovarian tumour - P.M. Tebeu, M.F. Pelte, J.L. Anguenot, A.T. Vlastos, R. Bazarbachi de Pury, K. Kinkel, E. Megevand, P. Schaefer

Krukenberg tumor from an appendiceal carcinoma

presenting as a primary ovarian tumour

P.M. Tebeu (1,4)), M.F. Pelte (2), J.L. Anguenot (1), A.T. Vlastos (1), R. Bazarbachi de Pury (1), K. Kinkel (3), E. Megevand (1), P. Schaefer (1).

(1) Départment of Obstetric & gynecology, (2) Department of clinical pathology, (3), Department of Radiology, all in University hospital, Geneva, Switzerland

Text Box: INTRODUCTION :
First described in 1895 by Friedrich Krukenberg, the so called Krukenberg tumor represents 1-2% of all ovarian tumors, and is nowadays defined as a secondary ovarian neoplasm with goblet cells component. The primary tumour is digestive in 90%, with gastric origin in 70%, colic in 14%, pancreatic or bile duct in 5%,and appendicular in 2%. Rare Krukenberg tumors from thyroid or breast cancer were described. The appendicular tumor is rare (2 to 150 per 100 000 appendicectomy samples), and its diagnosis is often done through operation, pathological or immunohistochemical findings. In certain cases, the tumor can be disseminated in the abdomen and present peritoneal metastases much as like implants from a primary ovarian tumor. In the case this patient the Krukenberg tumor mimics an adenocarcinoma of the ovary.

Text Box: CASE PRESENTATION :
A 55 years old woman, gravida II, para II, was admitted for an exploratory laparotomy for an abdominal mass felt by her since ten months. Prior examination revealed central pelvic mass of about 30 cm, mobile with irregular surface, and the presence of ascites that led to the suspicion of an ovarian tumour. Investigations were done with the following findings : 1. abdominal ultrasound and the CT-Scan revealed an ascite and a central pelvic mass of 25cm diameter; 2. biopsy of the mass revealed a signet-ring cell carcinoma; 3. the CK20 positive and CK7 negative immunostaining of the tumor cells;

Text Box: 4. A complete digestive endoscopy was done with biopsies of oesophagus, stomach, and colon, without any evidence of the tumour. The diagnosis of primary ovarian carcinoma was retained and the patient received a neo-adjuvant chemotherapy. The size of the tumour did not regress on CT-Scan. Laparotomy revealed an enlarged right ovary with a solid and cystic component, covering a large and long appendix. There were no adhesions, nor vegetations. The left ovary looked normal. There were peritoneal implants : vesical, right parietolateral, rectal, and subdiaphragmatic nodules. Total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, appendicectomy and resection of implants was carried out, except for the subdiaphragmatic nodules.

The macroscopy revealed a voluminous ovarian tumor measuring 25x15x15cm, which, on section surface, had an heterogenous yellow parenchyma with hard nodular zones and cysts.

Text Box: DISCUSSION
The symptomatology of this patient was poor. According to the literature only in rare cases abdominal pain, vaginal bleeding and the acute appendiceal syndrom were descibed. The diagnosis of the primary appendiceal tumor is often difficult even during laparotomy and in certain cases the diagnosis is made only after a second laparotomy with appendicectomy, and histologic analysis. This fact can be explained by a vermiform, and apparently normal appendix presentation. Therefore, during laparotomy if the ovarian tumor is suspected as metastatic, and the primary lesion is not found, it is useful to carry out an appendicectomy. The proximal portion apparently normal explain the false negative report of the colonoscopy.

Text Box: CONCLUSION
This case demonstrates the potential difficulties in the differential diagnosis of pelvic tumours. Laparotomy and the histologic report should direct the final diagnosis. In case of epithelial digestive tumor, the digestive surgeon should perform a full pelvic evaluation.

Text Box: Three main presentations of epithelial appendiceal tumor can be observad “pure carcinoids“ derived from from neuroendocrine sub epithelial cells; adenocarcinomas with “goblet cells” from the cryptic epithelium, and adenocarcinoid tumor associating both previous aspects. The signet-ring cells carcinoma is a peculiar type of mucinous adenocarcinoma characterized by a high percentage (>50%) of isolated mucinous cells so-called signet-ring cells. In our case, the patient presents an adenocarcinoma, which in its invasive form has a poor prognosis.
Inspite of the therapeutic efforts, the mean survival of 7 to 9 months remains almost unchanged, nevertheless few cases of 5 and 7 years survival have been published. We hypothesize that the poor survival of this pathology should be the result of precocious multifocal metastatic status at the moment of diagnosis. Radiotherapy and chemotherapy are not efficient. When surgery is performed, it improves the quality of life and at time the survival. In case of epithelial digestive tumor, the digestive surgeon must do a full pelvic evaluation, and bilateral salpingo oophorectomy is recommended if the patient is menopaused.

Text Box: REFERENCES
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