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Silent existence
Images in Gynecology
www. AJOG.org
Silent existence A retroperitoneal tumor had an unexpected origin Tsung-Jung Liang, MD; Shiuh-Inn Liu, MD, PhD; I-Shu Chen, MD
Case notes A 63-year-old woman was referred to our institute, because a lesion of the pancreatic tail, an incidental finding during a general health examination, was progressively enlarging. At the time of admission, she did not have abdominal pain, bowel discomfort, or weight loss. Her physical examination was unremarkable, except for a surgical scar located low on the abdomen. She had undergone hysterectomy and left salpingooophorectomy 9 years earlier because of postmenopausal vaginal bleeding. Pathologic examination of that surgical specimen showed uterine leiomyomas and a granulosa cell tumor (GCT) of the left ovary. Abdominal computed tomography disclosed a well-defined, heterogeneously-enhanced mass in the pancreatic tail region (Figure 1, arrow). Serum tumor marker levels, including cancer antigen (CA)-19-9, CEA, and CA-125, were all within reference ranges. The patient underwent laparoscopic tumor resection for a presumed pancreatic malignancy. A well-demarcated retroperitoneal tumor was found in the left upper abdomen (Figures 2-4). The tumor was close to the pancreas but was not adhered to it. Conclusions Microscopic examination of the resected specimen showed proliferation of ovoid and spindle cells with pale nuclei, small nucleoli, and occasional longitudinal nuclear grooving (Figure 5). Only a few mitotic figures were observed. The tumor cells stained positive for alpha-inhibin and negative for epithelial membrane antigen. On the basis of these features and the patient’s history, she was diagnosed with metastatic GCT. GCTs are rare neoplasms that comprise 2-5% of all ovarian cancers. They are usually hormonally-active and can produce steroid hormones, which leads to a typical tanyellow coloration in gross appearance and clinical symptoms related to hyperestrogenism, including vaginal bleeding, endometrial hyperplasia, and breast tenderness (Figure 4). These tumors have malignant potential and a tendency for late recurrence. The median time from initial diagnosis to relapse is approximately 4-6 years. Extremely late recurrences of more than 20 years have been documented in
FIGURE 1
Computed tomography of the abdomen disclosed a well-defined, heterogeneously-enhanced mass in the pancreatic tail region (arrow)
Liang. Silent existence. Am J Obstet Gynecol 2011.
several reports.1 Most commonly, the tumors recur in the pelvis, but the retroperitoneum, liver, spleen, bone, and lung parenchyma have also been reported as recurrence sites.2,3 FIGURE 2
A yellowish tumor was found after dissecting the omentum
From the Division of General Surgery, Department of Surgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan. The authors report no conflict of interest. Cite this article as: Liang T-J, Liu S-I, Chen I-S. Silent existence: a retroperitoneal tumor had an unexpected origin. Am J Obstet Gynecol 2011;205:160.e1-2. 0002-9378/$36.00 © 2011 Mosby, Inc. All rights reserved. doi: 10.1016/j.ajog.2011.06.050
160.e1
American Journal of Obstetrics & Gynecology AUGUST 2011
Liang. Silent existence. Am J Obstet Gynecol 2011.
Images in Gynecology
www.AJOG.org
FIGURE 3
FIGURE 5
Upon gross examination, the excised tumor was a soft encapsulated mass measuring up to 8 ⴛ 6.5 ⴛ 5 cm
A microscopic view of a tumor sample showed features compatible with granulosa cell tumor
Liang. Silent existence. Am J Obstet Gynecol 2011. Liang. Silent existence. Am J Obstet Gynecol 2011.
Currently, no standard approach exists for managing recurrent GCTs. Considering the poor prognosis of patients with residual tumors, complete resection should be attempted if the recurrent mass is localized and resectable.4 If complete tumor extirpation is not feasible, debulking surgery, followed by radiotherapy or chemotherapy, should be considered, since it has been associated with prolonged survival and better symptom FIGURE 4
The cut surface was homogenously tan-yellow in coloration
control in select cases.4,5 Platinum-based chemotherapy is currently the preferred regimen and may be superior to radiation for patients who have widespread disease or whose surgery resulted in suboptimal cytoreduction.3 The 10-year survival rate after recurrence is more than 50%; however, patients with advanced disease are difficult to treat and do not have durable remissions.4 Our patient had an indolent metastatic tumor that exhibited late recurrence. This may be attributed at least partially to the tumor being well-encapsulated, rather than widely metastatic. She underwent surgery and had an uneventful postoperative course. Our findings highlight the necessity of long-term follow-up for patients with GCTs. f REFERENCES
Liang. Silent existence. Am J Obstet Gynecol 2011.
1. Schumer ST, Cannistra SA. Granulosa cell tumor of the ovary. J Clin Oncol 2003;21:1180-9. 2. Paul PC, Chakraborty J, Chakrabarti S, Chattopadhyay B. Extraovarian granulosa cell tumor. Indian J Pathol Microbiol 2009;52: 231-3. 3. Abu-Rustum NR, Restivo A, Ivy J, et al. Retroperitoneal nodal metastasis in primary and recurrent granulosa cell tumors of the ovary. Gynecol Oncol 2006;103:31-4. 4. Sehouli J, Drescher FS, Mustea A, et al. Granulosa cell tumor of the ovary: 10 years follow-up data of 65 patients. Anticancer Res 2004;24:1223-9. 5. Pectasides D, Pectasides E, Psyrri A. Granulosa cell tumor of the ovary. Cancer Treat Rev 2008;34:1-12.
AUGUST 2011 American Journal of Obstetrics & Gynecology
160.e2
www. AJOG.org
Silent existence A retroperitoneal tumor had an unexpected origin Tsung-Jung Liang, MD; Shiuh-Inn Liu, MD, PhD; I-Shu Chen, MD
Case notes A 63-year-old woman was referred to our institute, because a lesion of the pancreatic tail, an incidental finding during a general health examination, was progressively enlarging. At the time of admission, she did not have abdominal pain, bowel discomfort, or weight loss. Her physical examination was unremarkable, except for a surgical scar located low on the abdomen. She had undergone hysterectomy and left salpingooophorectomy 9 years earlier because of postmenopausal vaginal bleeding. Pathologic examination of that surgical specimen showed uterine leiomyomas and a granulosa cell tumor (GCT) of the left ovary. Abdominal computed tomography disclosed a well-defined, heterogeneously-enhanced mass in the pancreatic tail region (Figure 1, arrow). Serum tumor marker levels, including cancer antigen (CA)-19-9, CEA, and CA-125, were all within reference ranges. The patient underwent laparoscopic tumor resection for a presumed pancreatic malignancy. A well-demarcated retroperitoneal tumor was found in the left upper abdomen (Figures 2-4). The tumor was close to the pancreas but was not adhered to it. Conclusions Microscopic examination of the resected specimen showed proliferation of ovoid and spindle cells with pale nuclei, small nucleoli, and occasional longitudinal nuclear grooving (Figure 5). Only a few mitotic figures were observed. The tumor cells stained positive for alpha-inhibin and negative for epithelial membrane antigen. On the basis of these features and the patient’s history, she was diagnosed with metastatic GCT. GCTs are rare neoplasms that comprise 2-5% of all ovarian cancers. They are usually hormonally-active and can produce steroid hormones, which leads to a typical tanyellow coloration in gross appearance and clinical symptoms related to hyperestrogenism, including vaginal bleeding, endometrial hyperplasia, and breast tenderness (Figure 4). These tumors have malignant potential and a tendency for late recurrence. The median time from initial diagnosis to relapse is approximately 4-6 years. Extremely late recurrences of more than 20 years have been documented in
FIGURE 1
Computed tomography of the abdomen disclosed a well-defined, heterogeneously-enhanced mass in the pancreatic tail region (arrow)
Liang. Silent existence. Am J Obstet Gynecol 2011.
several reports.1 Most commonly, the tumors recur in the pelvis, but the retroperitoneum, liver, spleen, bone, and lung parenchyma have also been reported as recurrence sites.2,3 FIGURE 2
A yellowish tumor was found after dissecting the omentum
From the Division of General Surgery, Department of Surgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan. The authors report no conflict of interest. Cite this article as: Liang T-J, Liu S-I, Chen I-S. Silent existence: a retroperitoneal tumor had an unexpected origin. Am J Obstet Gynecol 2011;205:160.e1-2. 0002-9378/$36.00 © 2011 Mosby, Inc. All rights reserved. doi: 10.1016/j.ajog.2011.06.050
160.e1
American Journal of Obstetrics & Gynecology AUGUST 2011
Liang. Silent existence. Am J Obstet Gynecol 2011.
Images in Gynecology
www.AJOG.org
FIGURE 3
FIGURE 5
Upon gross examination, the excised tumor was a soft encapsulated mass measuring up to 8 ⴛ 6.5 ⴛ 5 cm
A microscopic view of a tumor sample showed features compatible with granulosa cell tumor
Liang. Silent existence. Am J Obstet Gynecol 2011. Liang. Silent existence. Am J Obstet Gynecol 2011.
Currently, no standard approach exists for managing recurrent GCTs. Considering the poor prognosis of patients with residual tumors, complete resection should be attempted if the recurrent mass is localized and resectable.4 If complete tumor extirpation is not feasible, debulking surgery, followed by radiotherapy or chemotherapy, should be considered, since it has been associated with prolonged survival and better symptom FIGURE 4
The cut surface was homogenously tan-yellow in coloration
control in select cases.4,5 Platinum-based chemotherapy is currently the preferred regimen and may be superior to radiation for patients who have widespread disease or whose surgery resulted in suboptimal cytoreduction.3 The 10-year survival rate after recurrence is more than 50%; however, patients with advanced disease are difficult to treat and do not have durable remissions.4 Our patient had an indolent metastatic tumor that exhibited late recurrence. This may be attributed at least partially to the tumor being well-encapsulated, rather than widely metastatic. She underwent surgery and had an uneventful postoperative course. Our findings highlight the necessity of long-term follow-up for patients with GCTs. f REFERENCES
Liang. Silent existence. Am J Obstet Gynecol 2011.
1. Schumer ST, Cannistra SA. Granulosa cell tumor of the ovary. J Clin Oncol 2003;21:1180-9. 2. Paul PC, Chakraborty J, Chakrabarti S, Chattopadhyay B. Extraovarian granulosa cell tumor. Indian J Pathol Microbiol 2009;52: 231-3. 3. Abu-Rustum NR, Restivo A, Ivy J, et al. Retroperitoneal nodal metastasis in primary and recurrent granulosa cell tumors of the ovary. Gynecol Oncol 2006;103:31-4. 4. Sehouli J, Drescher FS, Mustea A, et al. Granulosa cell tumor of the ovary: 10 years follow-up data of 65 patients. Anticancer Res 2004;24:1223-9. 5. Pectasides D, Pectasides E, Psyrri A. Granulosa cell tumor of the ovary. Cancer Treat Rev 2008;34:1-12.
AUGUST 2011 American Journal of Obstetrics & Gynecology
160.e2