Satia MN*, Mali K**, Ganapathi T***.
(* Professor, ** Assistant Professor, *** Third Year Resident. Department of Obstetrics and Gynecology, Seth G S Medical College & KEM Hospital, Mumbai, India.)
Acute abdomen with hemoperitoneum in childbearing age group is usually due to obstetrical complications but its extremely rare for a woman with gynecological conditions. We report a rare case of ruptured granulosa tumor of the ovary. She presented with acute abdomen due to hemoperitoneum. Ruptured ovarian tumor should be one of the differential diagnosis as ovarian tumors are known to occur at any age and clinicians should remain alert and aware of such a presentation. Anticipation of these problems and availability of facilities for frozen section would be of utmost importance as it would avoid incomplete surgeries.
Granulosa cell tumors account for approximately 4.3% of all ovarian cancers. Granulosa cell tumors are functional ovarian tumors and hence are associated with endocrine manifestations. Approximately three fourth of the tumors are associated with hyperestrogenism but some may be virilizing. These tumors are rarely bilateral (< 5 %) and have low malignant potential (3-14 %) with high recurrence rate. Symptoms depend on degree of hormonal activity and age of the patient. Clinicians should be aware of occurrence of granulosa cell tumors at any age and their propensity to rupture as in our patient who presented with acute abdomen and hemoperitoneum. Acute abdomen with hemoperitoneum is a common obstetric emergency, but a very rare entity in gynecologic conditions. It is most commonly seen in non accident related hospital admissions. Gynecological conditions causing acute abdominal pain are ovarian torsion and ruptured corpus luteum hematoma in the child bearing age group. Our patient presented with hemoperitoneum and history of amenorrhea mimicking ruptured ectopic pregnancy or a ruptured corpus luteum cyst.
A 35 year old multiparous woman came to emergency room with severe abdominal pain and giddiness. On examination her general condition was fair, she was pale with clinical hemoglobin of 6 gm%, pulse of 110/minute and blood pressure of 90/60 mm of Hg. Abdominal examination was suggestive of signs of acute abdomen with guarding, tenderness, rigidity. She was markedly obese. During pelvic examination there was tenderness in both the fornices with bogginess, more in the right fornix. Her urine pregnancy test (UPT) was negative, but colpopuncture was positive. Sonography examination revealed 500 ml of hemoperitoneum and hence the clinical impression of ruptured corpus luteum cyst was made. Decision of immediate exploratory laparotomy was taken. Intra operative findings revealed 500 cc of blood stained fluid along with clots weighing 200 gms. An actively bleeding large mass of 10x8 cms arising from the right ovary was observed. Uterus along with both fallopian tubes were normal and her left ovary was normal.
Figure 1. Ruptured 10 x 8 cm size ovarian cyst.
Figure 2. Ovarian cyst surface with normal uterus (U).
Right salpingo-oophorectomy was performed and the specimen was sent for histopathological examination. Histopathology report diagnosed a well differentiated adult type granulosa cell tumor of the right ovary.
Figure 3. Intact capsule (black arrow) seen in the histopathology slide indicating benign nature of the lesion.
Figure 4. Adult type granulosa cells (black arrow) with few areas of hemorrhage (yellow arrow) seen.
Post operative period was uneventful and she was referred to the oncology center for further evaluation where the histopathology slides were reviewed. Immunohistochemistry was positive for MIC-2 (gene product that is a cell surface antigen), AE/AE-3 (screening marker antibody used for epithelial differentiation) and inhibin. Histopathology reported it to be a sex cord stromal; granulosa cell tumor. She was advised to follow up at 6 monthly intervals.
Granulosa stromal cell tumors are a type of sex cord stromal cell tumors (SCST). These tumors constitute 4.3% of all ovarian neoplasms. They are differentiated histologically by WHO. These tumors can occur in any age group that is 1 year to 90 years of age but they are most common in 50-55 years of age. The chances of hemoperitoneum with granulosa cell tumor has been reported in 5 to 20% of cases. Our patient presented with amenorrhea and acute onset of pain in abdomen. It is reported that tumor rupture occurred in 17.6% of patients prior to surgical intervention. However, in diagnosed cases of adult granulosa cell tumor patients’ have not presented with acute abdomen.
Other causes of hemoperitoneum may include patients on anticoagulant therapy or patients having platelet function defects or rarely retrograde bleeding due to cervical stenosis. Diagnosis is confirmed on laparotomy as in the present case where hemoperitoneum and active bleeding ovarian mass was noticed. Anticipation of these problems and timely actions like frozen sections of the suspicious lesions will help in preventing incomplete surgeries requiring repeat procedures. Clinicians should be alert to the possibility of propensity of granulosa cell tumors rupturing and causing hemoperitoneum. Twenty percent of emergency surgical services visits are by the elderly out of which 3-4 % are for acute abdominal pain. Mortality is 6-8 times high in older patients with abdominal pain as compared to younger patients. About one-half to two-thirds of these patients need to be admitted for medical line of treatment, while one-third require surgical intervention.. Management of granulosa tumors depends not only on the stage of the tumor but also the age of the patient. In adolescent or child bearing age group patients presenting with early stage disease, unilateral salpingo-oophorectomy and appropriate surgical staging is preferred in an attempt to preserve fertility. In fifth and sixth decade of life surgery consists of a total abdominal hysterectomy along with removal of both tubes and the ovaries and surgical staging which is the most important prognostic factor. The overall survival with stage I and stage II disease is 99%. Patients with large tumor size (≥ 10-15 cm), stage IC, poorly differentiated tumor, high mitotic index or tumor rupture are said to be high risk and adjuvant chemotherapy should be advised in view of increased risk of relapse. Patients with low risk stage I tumor should be kept on observation. In patients with recurrence or stage III- IV granulosa cell tumors postoperative chemotherapy is recommended but the survival benefit is still not known due to paucity of results and lack of randomized trials, so no treatment has been standardized. The lack of specific markers in the identification of granulosa cell tumors has lead to development of a panel of antibodies to identify inhibin, calretinin, epithelial membrane antigen, and low molecular weight keratin which is expressed on tumor cells, through immunohistochemistry. Immunohistochemistry not only helps in diagnosis but also is useful in administration of targeted chemotherapy against these tumors.
Rupture of ovarian tumors are one of the rare causes of acute abdomen and in women presenting with acute abdomen with non obstetric causes, a high index of suspicion for malignancy should always be maintained. With good clinical suspicion and proper management patients can be given good quality care.
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Satia MN, Mali K, Ganapathi T. Ruptured Granulosa Cell tumor of Ovary. JPGO 2016. Volume 3 No. 11. Available from: http://www.jpgo.org/2016/11/ruptured-granulosa-cell-tumor-of-ovary.html