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|Year : 2023 | Volume
| Issue : 2 | Page : 102-104
A rare case of primary retroperitoneal extraovarian granulosa cell tumor
A Menon, L Singla, SG Pujari, CV Kantharia
Department of Surgical Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra, India
|Date of Submission||06-Jan-2022|
|Date of Decision||15-Jun-2022|
|Date of Acceptance||17-Jun-2022|
|Date of Web Publication||09-Dec-2022|
Dr. C V Kantharia
Department of Surgical Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
Extraovarian granulosa cell tumors (GCTs) are rare neoplasms. They arise from the ectopic gonadal tissue along the embryonal route of genital ridge. We present a case of an extraovarian tumor in an elderly female who presented with lump in the abdomen occupying the pelvic region. She was operated and the mass was excised with intact capsule. Histopathology revealed the mass to be an adult granulosa cell tumor. Extraovarian granulosa cell tumors are very rare with only 13 such cases reported in literature to date. The present case is the largest primary retroperitoneal adult granulosa cell tumor reported till date.
Keywords: Extraovarian granulosa cell tumors, primary extraovarian, retroperitoneal tumor
|How to cite this article:|
Menon A, Singla L, Pujari S G, Kantharia C V. A rare case of primary retroperitoneal extraovarian granulosa cell tumor. J Postgrad Med 2023;69:102-4
| :: Background|| |
Granulosa cell tumors (GCTs) constitute 2-5% of all ovarian tumors. They are the most common malignant sex cord stromal tumors of ovary. The cell of origin of these tumors is mesenchyme of genital ridge. There have been reports in literature of its unusual location such as broad ligament, mesentery, fallopian tube, and retroperitoneum. Of these, primary retroperitoneal adult GCTs are exceedingly rare with only nine cases reported in literature. We present a rare case of primary retroperitoneal adult GCT in an elderly female.
| :: Case Presentation|| |
A 66-year-old lady presented with a painless, progressively increasing lump in abdomen for two months. Patient had history of undergoing abdominal hysterectomy with bilateral oophorectomy 20 years prior for dysfunctional uterine bleeding. Clinical examination revealed a large non tender 21 x 20 cm lump, [Figure 1], arising from pelvis. It was occupying the umbilical, hypogastric, left lumbar, and left iliac regions. It was solid-cystic in consistency [Figure 1]. The lump had well-defined margins, but for at lower margin as it was arising from the pelvis.
Contrast-enhanced computed tomography (CT) scan done revealed, a large heterogenously enhancing solid cystic mass arising from the pelvis, occupying the entire lower abdomen, displacing and indenting bowel loops, and compressing the ureter on left side causing mild hydro-uretero-nephrosis. [Figure 2] and [Figure 3]. Tumor markers CEA and Ca- 125 were within normal limits.
|Figure 2: Contrast enhanced tomograph scan showing: (a) solid –cystic mass compressing bowel loops. (b) Mass indenting ureter|
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|Figure 3: (a) Mass dissected out during laparotomy. (b) Gross specimen following its excision|
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After optimizing, she was taken up for surgery. Intra-operatively a 21 cm x 20 cm well capsulated bosselated mass was found arising from the retroperitoneum. Small bowel loops and sigmoid colon were adherent to the mass. The feeding arteries from Mesenteric vessels were found to be supplying the mass [Figure 4]. The mass was compressing the ureter. The mass was dissected free of the bowels and ureter by meticulous adhesiolysis. The mass was excised in to with intact capsule. The tumor measured 21 × 20 cm [Figure 5] and weighed 4 kg. Cut surface showed multiple cystic spaces between solid areas with central necrotic areas.
|Figure 4: Cut surface of mass showing multiple solid- cystic areas with intervening areas of necrosis|
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|Figure 5: Histopathology showing small round to oval neoplastic cells with trabecular patterns, scanty cytoplasm and round to oval nuclei|
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Histopathology showed small round to oval neoplastic cells with diffuse and trabecular patterns. The cells showed scanty cytoplasm and round to oval nuclei with nuclear grooves and Call–Exner bodies. Immunohistochemistry staining performed, was found to be positive for Inhibin and negative for EMA. A diagnosis of adult GCT was made. The patient had an uneventful postoperative recovery. She was discharged on fifth postoperative day.
| :: Discussion|| |
GCTs are rare tumors of ovary. They are the commonest sex cord stromal type of tumors. They are classified into juvenile type or adult type, based on their clinical and histological features. The adult type usually manifests in peri-menopausal or postmenopausal women. Rarely, adult GCTs arise from extraovarian sites. Their origin is from the ectopic gonadal stromal tissue within the sex cord originating from mesonephros. Another theory postulates that both coelomic epithelium and mesonephros may contribute to the origin of pre-granulosa cells. As a result, sites of these extraovarian GCTs are usually in tissues that originate close to mesonephros like retroperitoneum, broad ligament, and adrenals. Adult GCTs are characterized by distinct histological features. They are usually solid-cystic in appearance. Cut section is yellow white or grey in color owing to its high lipid content. They are either well differentiated or poorly differentiated. Characteristic feature of well-differentiated variants is Call–Exner bodies, which are cystic degeneration of granulosa cells with nuclear debris seen in 30-50%. The granulosa nuclei are radially oriented around these structures. These nuclei are bland with hyperchromatism and have characteristic nuclear grooving giving a 'coffee bean' appearance. Immunohistochemistry plays an important role in confirming diagnosis as these tumors are histologically similar to tumors like endometrial stromal carcinoma or other retroperitoneal tumors. They are found to be positive to Inhibin-alpha, calretinin, progesterone receptors, and negative for EMA.
We reviewed the literature on extraovarian adult GCTs with keywords 'granulosa cell tumour', 'extra ovarian', 'retroperitoneal' and could find only 13 cases of extraovarian adult GCTs reported till date.,,,, Out of these, nine tumors had a retroperitoneal location. Our patient had a retroperitoneal GCT measuring 21 × 20 cm and is the largest primary retroperitoneal extraovarian adult GCT described in literature till date.
Declaration of patient consent
The authors certify that appropriate patient consent was obtained.
Department of Pathology for the histopathological diagnosis.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]