Bathija S*, Hatkar P**.
(*First year resident, ** Associate Professor, Department of Obstetrics and Gynecology, Seth G S Medical College & K E M Hospital, Mumbai, India.)
A 17 year old girl presented to the gynecology outpatient department with complaints of abdominal distension which was insidious in onset associated with lower abdominal pain for the past 3 months. There was no significant medical or surgical history. She had no complaints of weight loss, loss of appetite or menstrual complaints. On examination, her general condition was fair, she was thin built and weighed around 49 kg. Her general physical examination was unremarkable. On abdominal inspection, there was generalised distension and the skin over the abdomen was tense and shiny. On palpation, a cystic mass was felt arising from the pelvis and extending till the epigastrium and on both sides upto iliac fossae occupying the whole of the abdomen. The mass was firm in consistency with no appreciable solid areas. Per vaginal examination revealed a firm cystic mass with lower extent of the mass that caused forniceal fullness. Tumor markers were evaluated and showed elevated CA 125 level of 131 U/L. β-human chorionic gonadotropin, alpha feto protein, carcino embryonic antigen and lactate dehydrogenase were within normal limits. Mantoux test was negative. Complete hemogram, liver function test and coagulation profile were found normal. USG revealed right ovarian 25x15 cm unilocular cyst with one solid component measuring approximately 4x2 cm without vascularity. There was evidence of bilateral ureteric compression and bilateral hydronephrosis, right more than left with no ascites. Contrast enhanced CT scan of the abdomen and pelvis revealed a well-defined 25x5x23 cm cyst having few non-enhancing septa with foci of calcification within. Right ovary showed multiple follicles and left ovary was not seen separately. The mass was displacing the retroperitoneal structures posteriorly and hydronephrosis with hydroureter in upper one third of the right ureter was confirmed. There was no evidence of ascites or lymphadenopathy. Urinary bladder, visualised vessels, bones and sections of chest were normal. CT scan impression was that of a mature cystic teratoma of left ovary or a mesenteric cyst. Urological examination was done and DJ stenting of the right ureter was performed for the treatment of hydronephrosis. After obtaining a valid consent, she was posted for exploratory laparotomy with left ovarian cystectomy SOS left salphingo-oophorectomy. A vertical midline skin incision was taken. A left ovarian cyst measuring 25×20 cm was noted. [Fig. 1] Left fallopian tube was found stretched over the cyst. There was no evidence of ascites. The cyst was punctured inadvertently while opening the abdomen. There was minimal spillage of cyst fluid into the abdominal cavity. About six liters of mucinous fluid was aspirated from the cyst, which was sent for cytology examination for presence of malignant cells. The left ovarian complex cyst wall was excised and sent for frozen section. It showed presence of two solid areas both approximately 4x3cm. On the right side, a simple ovarian cyst approximately 3 cm in diameter was found. Right sided ovarian cystectomy and ovarian reconstruction was done. The cyst fluid was sent for cytology and cyst wall was sent for frozen section. Frozen section report of the left ovarian cyst showed atypical proliferative mucinous neoplasm suspicious of mucinous carcinoma and cytology report did not show malignant cells. Right ovarian cyst showed features of a benign cyst. Left salpingo-oophorectomy was done. There was no evident lymphadenopathy and uterus, right fallopian tube, pouch of Douglas, under surface of liver, omentum and peritoneum were grossly unremarkable. However, omental biopsy was done to rule out micrometastasis after noting frozen section report and counseling the parents and taking appropriate consent.
Final Histopathology report:
- Left ovarian cyst showed atypical proliferative mucinous neoplasm with intraepithelial carcinoma.
- Right ovarian cyst: benign nature of cyst.
- Omentum: mild lymphohistiocytic infiltrate. No evidence of metastasis.
Tumor markers and radiology in collaboration serve as a vital tool in the diagnosis of ovarian cancers. CA 125 has been widely used as a marker for epithelial ovarian tumors however its utility is debatable. Although elevated serum CA 125 levels (>35 U/mL) have been found in more than 90% of advanced ovarian cancer patients, only 50% of patients with stage I disease have elevated levels. Furthermore, CA 125 is also raised in approximately 1% of healthy individuals, in patients of liver cirrhosis, endometriosis, first-trimester pregnancy, pelvic inflammatory disease, pancreatitis and in other malignancies of lung, breast and pancreas. Therefore, its raised levels must always be interpreted with caution and in conjunction with symptomatology and radiology. It is believed that if levels are initially raised at the time of detection they can be used as a marker for identifying residual or recurrent disease later at follow up. Sometimes huge masses can create confusion as to the exact source of origin even on radiology. In this case too, the huge intra-abdominal mass on USG seemed to have originated from the right side, but a contrast enhanced CT suggested it to be a left ovarian cyst or mesenteric cyst. Elevated CA 125 levels in adjunct with the computed tomography scan report lead us to believe that it was an ovarian mass probably epithelial in origin and exploratory laparotomy was performed. The intraoperative examination of the other ovary and surrounding structures is crucial as it can often lead to escalation of the stage of the tumor if macroscopic deposits are visualized during surgery. Though the adult staging protocols dictate mandatory lymph node dissections and biopsies of peritoneal surfaces; these procedures are often omitted in pediatric and adolescent cases unless gross metastatic disease is present. Some surgeons perform a prophylactic wedge biopsy of the uninvolved ovary at the time of initial debulking surgery or biopsy from grossly suspicious foci ; but in our patient, the uterus, right fallopian tube, pouch of Douglas were unremarkable and the right ovary was grossly normal with a cyst of approximately 3 cm diameter. Thus, only left ovarian cyst wall and right ovarian cyst wall were sent for frozen section and no additional tissue samples were sent. When compared with primary ovarian serous carcinoma, primary ovarian mucinous carcinoma was more commonly associated with younger age, unilateral involvement of the ovary, unilateral tumour size >10 cm, less-advanced FIGO tumour stage (83% are stage 1), lower histological grade, higher completion rate of optimal primary surgery, more frequent use of unilateral salpingo-oophorectomy, lower volume of ascites and lower preoperative CA125 levels. Our patient too had similar findings. Since on CT scan and intraoperatively no evidence of metastasis was seen unilateral salpingo-oopherectomy with omental biopsy was performed in view of frozen section report suggestive of mucinous cystadenocarcinoma.
The diagnostic standard for distinguishing mucinous carcinoma from borderline tumours lies in the presence of stromal invasion. Two invasive patterns, ‘destructive’ and ‘expansile’, have been well recognized in primary ovarian carcinoma. An ‘expansile’ pattern is the most common pattern in primary ovarian mucinous carcinoma whereas a ‘destructive’ pattern is less common and should raise the concern for metastatic carcinoma. [10,13] However, it can often be challenging to report a carcinoma in a minor age group without definitive infiltration into the stroma, rendering an equivocal report as in this case. For stage I epithelial cancers in adults, surgery alone is adequate. Here surgery is used for both staging and therapeutic purposes. Surgery includes total abdominal hysterectomy, bilateral salpingo-oophorectomy and omentectomy along with tumor debulking or cytoreduction. Fertility sparing surgery may be preferred in young patients inspite of possible metastasis since fertility is an important issue. Furthermore, some studies have compared fertility sparing surgery with radical surgery in borderline ovarian tumors. In these studies, though the recurrence rate was slightly higher in the fertility sparing group as compared to radical group, these recurrences could be managed by subsequent surgeries. A study done by Aggarwal et al. pointed out that low malignant recurrences have been reported more than ten years after initial surgery even in an adult patient population. Therefore, like in adults young patients should also be kept under close follow up to monitor recurrence which should be treated with another salvage surgery.
Several adjuvant regimens ranging from single agent to multi agent regime have been tried for the treatment of malignant ovarian neoplasms. Our patient was referred to an oncology institute for further follow up and management. In conclusion, prognosis of ovarian cancers presenting at younger age remains variable and depends on the stage of presentation. Most of the cases of metastatic variant reported in the literature have had bad prognosis with almost all the patients dying within five years of detection of the lesion. In the mucinous variety, primary mucinous carcinomas have a favourable prognosis though it is less common than the metastatic variant. Although there are a no clear indications for ovarian cancer screening, testing is recommended for women at high risk such as those with a significant family history, but consultation with a genetic counsellor is recommended to discuss limitations and alternatives to genetic testing. Potential screening tests include for CA-125, other tumor markers and USG. However, these tests are neither sensitive nor specific for ovarian cancer. Since signs and symptoms are vague, patient often presents late and at an advanced stage, management of these cases differ from their adult counterparts. Instead of radical surgery, fertility sparing conservative approach should be preferred in these cases.
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Bathija S, Hatkar P. Malignant Ovarian Tumor In A Teenage Girl. JPGO 2019. Volume 6 No.5. Available from: https://www.jpgo.org/2019/05/malignant-ovarian-tumor-in-teenage-girl.html