Presented at:
“An Update On Comprehensive Cardiovascular and Cancer Health Care Delivery Service”
A Collaboration Of Muhammadiyah University Of Yogyakarta And Münster University Of Germany
dr. Supriyatiningsih, M.Kes, Sp.OG
Department of Obstetrics and Gynecology Faculty Medicine and Health Sciences
Muhammadiyah University of Yogyakarta/Asri Medical Center
OVARIAN CANCER STAGE IIIC IN PATIENT WITH LYNCH SYNDROME
(Case Report)
OVARIAN CANCER STAGE IIIC IN PATIENT WITH LYNCH SYNDROME
(Case Report)
Ovarian carcinoma is the second most gynecologic malignancies encountered
Lynch Syndrome refer to Hereditary Non Polyposis Colorectal Cancer (HNPCC) which is related to a variety of other cancers, especially cancers of the endometrium, ovary, urogenital and various primary malignancies in the gastrointestinal system
HNPCC related with approximately 1% incidence of ovarian carcinoma.
A woman, unmarried, 22 years old, came to the hospital with a complaint of a mass rapidly enlarging with intermittent pain.
The patient had undergone intestinal surgery laparotomy 9 years ago, and Anatomical Pathology examination showed adeno carcinoma of the colon mukoides
Her grandmother died because of breast cancer. Physical examination: solid tumors, fixed to, the
3
size of 14x12x10 cm .
PET-CT imaging of the abdomen: the presence of a large vascular mass and heterogeneous in the pelvic cavity and lower abdomen urgent and pressing some organ vein, very suspicious of malignancy of the ovary.
Examination of Ca-125 is normal (3.83 mIU / ml), CEA increased (68.8 mIU / ml)
From the results obtained ascites surgery, two solid masses of ovarian carcinomatosis lesions
infiltrating the uterus, bladder, back wall of the vagina, pelvic wall and inguinal lymph nodes-pelvic adhesions and omentum and mesentery great on, and sigmoid colon tranversum.
Pathological Anatomy results showed goblet cell carcinoma.
Performed TAH-BSO (Total Abdominal Hysterectomy-Bisalphingo Ooforectomy). Patient received alkylating-based chemotherapy agent that is CAP (500 mg-50 mg-50 mg) that is Cisplatin, i.v., 50 mg/ m², day 1 - Cyclophospamid, i.v., 500 mg/ m² one day - Doxorubicin (Adreamycin), i.v., 50 mg/ m², one day - every three weeks, for 6 cycles and good patient response. Result of laboratory findings are Ca 125: 220,65 U/ml and CEA: 52,8 U/ml.
Introduction
Illustrative cases
right ovary left ovary
In this patient, it is detected that there is malignancy in the ovaries and other organs 9 years after patient undergoing bowel resection surgery (colon) approximately 25 cm for adeno carcinoma of the colon.
Presumption leading presence Lynch syndrome or HNPCC (Hereditary Non Polyposis Colorectal Cancer) because of the relatively young age of the patient, previous history of colorectal cancer, family history of cancer, high CEA and relaparatomy results that show the results of the PA in the ovaries and some organs are infiltrated with goblet cell carcinoma.
An interesting fact in this patient is the result of PA on the uterine organs were normal, because in most cases the extra colon cancer at a more frequent incidence of genital organs of the uterus (40%),
1,2,3.
compared to the ovaries (1%)
Malignancy experienced by these patient also occur at a relatively young age is 22 years, This happens very early compared to the
1,5
existing epidemiological
The diagnosis is then based on the patient's medical history, clinical symptoms, results of operations and Anatomical Pathology examination, while genetic testing has not been done since this patient is JAMKESMAS participants.
Until now, the risk of cancer and management of Lynch syndrome is very dependent on the specific mutation of DNA mismatch repair
4,6
genes that cause cancer
Surgery performed is TAH-BSO. Appendectomy, and lymphadenectomy omentectomy has been done on previous laparotomy.
Lynch syndrome with an extra organ metastasis to the colon can be found at a very young age. Metastasis to the reproductive organs can occur without metastases to the ovary to the uterus or endometrium.
Genetic testing and genetic counseling are the standard diagnostic procedures
Lynch syndrome is the main therapeutic surgery followed by chemotherapy.
Poor prognosis when found multiplicity, as it shows the spread in
1,2
excess of the colon, with a five year survival rate <10%
Dennis J Ahnen, Lisen Axell, Clinical features and diagnosis of Lynch syndrome (hereditary nonpolyposis colorectal cancer), UpToDate, January, 2009
Hampel, H, Stephens, JA, Pukkala, E, et al. Cancer risk in hereditary nonpolyposis colorectal cancer syndrome: later age of onset. Gastroenterology 2005;129-415
Lin, KM, Shashidharan, M, Ternent, CA, et al. Colorectal and extracolonic cancer variations in MLH1/MSH2 hereditary nonpolyposis colorectal kindreds, and the general population, Dis Colon Rectum 1998; 41:428
American Gastroenterological Association medical position statement: heredittary colorectal cancer and genetic testing, Gastroenterology 2001;121:195
Boland CR, Koi M, Chang DK, Carethers JM. The biochemical basis of microsatellite instability and abnormal immune histochemistry and clinical behavior in Lynch Syndrome: from bench to bedside. Familial Cancer epub 2007; DOI 10.1007/s10689-007-9145-9
Vasen HF, Watson P, Mecklin JP, Lynch HT. New clinical criteria for hereditary nonpolyposis colorectal cancer (HNPCC, Lynch syndrome) proposed by the International Collaborative group on HNPCC. Gastroenterology 1999;116:1453
1. 2. 3. 4. 5. 6.