# Extraovarian Granulosa Cell Tumor of Mesentery: A Case Report

Authors: Manjiri R. Naniwadekar; N. J. Patil
Journal: Pathology Research International
 (2010)

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## Abstract

Extraovarian granulosa cell tumor (GCT) is a very uncommon tumor, assumed to arise from the ectopic gonadal tissue along the embryonal route of the genital ridge. A 54 years old female patient presented with a mass and acute pain in abdomen. Exploratory laparatomy revealed hemoperitoneum with a large mesenteric mass measuring  13 × 12 cm in size, showing extensive areas of  haemorrhages. Histopathological examination of the excised mass showed features of adult-type GCT. As the patient had a history of hysterectomy   with bilateral salpingo-oophorectomy 10 years ago for ‘‘leiomyoma’’ with no evidence of GCT of the ovary in the  histopathology report, a diagnosis of extraovarian GCT was made. A diagnosis of extraovarian GCT should be carried out after excluding any previous history of GCT of the ovary. Tumor rupture with haemoperitoneum is a well-known complication of GCT. Extraovarian GCT is a rare tumor with only  10 cases reported in literature. The case is presented for its rarity.


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## Body

## 1. Introduction

Granulosa cell tumors (GCT) are the most common malignant sex cord—stromal tumors of the ovary [1]. They can arise in locations other than the ovary and may be derived from the mesenchyme of the genital ridge [2]. Women who have undergone oophorectomy may have the potential to develop  GCT [2]. The primary extraovarian GCT is extremely rare tumor [3]. In the English literature only 10 cases have been reported to date [3, 4], including one case arising in a mullerian cyst of the broad ligament [5]. Ours is the eleventh case and third  case from India [4, 6].


## 2. Case Report

A 54 years old female presented with mass and acute pain in abdomen. She  gave a history of hysterectomy with bilateral salpingo-oophorectomy 10 years ago for uterine leiomyoma. Per abdominal examination revealed  a vague mass palpable in epigastric and periumbilical region.  On investigation, her hemoglobin was 8.6 gram%  and total WBC count was 15,500 /cumm with neutrophilia. RBC morphology was hypochromic and  microcytic on peripheral smear. Sonography of the abdomen showed a12×12 cm-sized solid heterogeneous mass  in the mesentery.  Exploratory laparatomy revealed hemoperitoneum with a large mesenteric mass measuring 13×12 cm in size showing extensive areas of haemorrhages. The mass was removed.Gross findings—the mass was grayish brown, soft, and nodular  with haemorrhagic areas measuring13×11 cm. Cut section  revealed solid homogenous grayish white tumor with small cystic areas and areas of  haemorrhages (Figure 1).grayish brown, soft, and nodular  mass with haemorrhagic areas measuring13×11 cm. Cut section  showed solid homogenous grayish white tumor with areas of  haemorrhages.
(a)(b)Microscopic findings—sections from the tumor showed small round to oval neoplastic  cells with predominantly diffuse and trabecular patterns. The cells showed scanty cytoplasm and round to oval nuclei with nuclear grooves—coffee bean nuclei (Figure2). With the typical histopathological features, a diagnosis of  GCT (adult type ) of mesentery was made. The patient having a history of hysterectomy for leiomyoma, histopathology  reports were reviewed which showed no evidence of GCT of ovary. Immunohistochemistry (IHC) for inhibin and EMA was done. The tumor  was positive for inhibin and negative for EMA, thus confirming the diagnosis of extraovarian GCT (adult type )of mesentery.Microphotogaph  showing a tumor composed of small round to oval neoplastic  cells with predominantly diffuse  patterns. The cells showed scanty cytoplasm and round to oval nuclei with nuclear grooves—coffee bean nuclei (H&E×100-(a) and ×400-(b)).
(a)(b)


## 3. Discussion

Granulosa cell tumors (GCT) are the malignant sex cord—stromal tumors of the ovary. They constitute 1-2% of all ovarian tumors [1]. They  can recur or metastatsize many years after initial treatment. Rarely GCT can develop at an extraovarian site. Possibility of metastasis has to be ruled out before diagnosis of extraovarian GCT.  Extraovarian granulosa cell tumor is a rare tumor with only ten  cases reported in English literature [4]. Two cases were reported from India [4, 6]. Extraovarian granulosa cell tumor can develop in retroperitoneum [4, 7], broad ligament [5], mesentery, omentum, liver, adrenals, and so forth, [4]. Histogenetic origin is thought to be from ectopic gonadal stromal tissue from the mesonephros [4].GCTs  vary in their gross appearance. Most are partly cystic and partly solid. Intracystic hemorrhage is common [1].  Microscopically, the tumor cells resemble normal granulosa cells. They are small with uniform round or oval hyperchromaic nuclei with finely granular chromatin  and  longitudinal nuclear grooves or folds [1]. They show  microfollicular, macrofollicular, trabecular or diffuse pattern [1]. Similar histologic findings were observed in the present case, with a predominantly diffuse and trabecular pattern.Extraovarian GCT should be differentiated from other metastatic carcinomas of ovary having similar morphology. Inhibin and EMA can help in differentiating these tumors. GCT is positive for inhibin and negative for EMA. GCT also has to be differentiated from other tumors such as small cell carcinoma, undifferentiated carcinoma, endometrial stromal sarcoma, carcinoid, and lymphoma [1]. These  tumors do not show positivity for inhibin. IHC for CK, EMA, LCA, CD99 and  Chromogranin can help in diagnosing and differentiating these tumors. GCT  does not show positivity for EMA, LCA, and  Chromogranin.In the present case, the patient had a history of hysterectomy, with bilateral salpingo-oophorectomy 10 years ago for “leiomyoma” with no evidence of GCT of the ovary in the previous histopathology report. The patient presented with mass in abdomen and pain due to tumor rupture with haemoperitoneum, which is a well-known complication of GCT. Hormonal studies were not done, as the diagnosis of GCT was not suspected.  The histopathology features of the present tumor are typical of GCT, viz-small pale, round to oval granulosa cells with diffuse histological pattern and characteristic “coffee-bean” nuclei. The tumor showed positivity for inhibin while EMA was negative, thus confirming the diagnosis of  GCT.The case is reported for its rarity and to describe its relevance to the histologic origin and in clinical practice.  Women with H/O oophorectomy may develop extraovarian  GCT. Diagnosis is made by characteristic histologic features and by excluding previous H/O GCT of ovary. Immunostains like inhibin help in definitive diagnosis.



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