Laparoscopic Management of Ovarian Dysgerminoma Presenting with Acute Abdomen Caused by Adnexal Torsion in a 17-Year-Old Girl

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1 J Pediatr Adolesc Gynecol (2009) 22:e9ee13 Case Reports Laparoscopic Management of Ovarian Dysgerminoma Presenting with Acute Abdomen Caused by Adnexal Torsion in a 17-Year-Old Girl Akihiro Takeda, MD, Masahiko Mori, MD, Kotaro Sakai, MD, Takashi Mitsui, MD, and Hiromi Nakamura, MD Department of Obstetrics and Gynecology, Gifu Prefectural Tajimi Hospital, Tajimi, Gifu, Japan Abstract. Background: Ovarian dysgerminoma cases very rarely present with acute abdomen due to adnexal torsion and the successful laparoscopic management of such a case has not been reported previously. Case: A 17-year-old sexually inactive high school girl presenting with acute abdomen was referred to our emergency department. Transabdominal ultrasonography showed the presence of homogeneous tumor in the lower abdomen. Emergency laparoscopic surgery was performed and left ovarian tumor with adnexal torsion was identified. Left salpingo-oophorectomy was performed carefully to avoid damaging the tumor capsule and the excised tissue was removed after retrieval in an endobag. The histopathological diagnosis was pure ovarian dysgerminoma. The postoperative course was uneventful. Conclusion: Laparoscopic management with careful postoperative follow-up to monitor recurrence could be a treatment option in a young girl with dysgerminoma, if the disease condition is detected in the early stage. Key Words. Dysgerminoma Acute abdomen Ad nexal torsion Laparoscopic surgery Young girl Introduction Dysgerminoma is a rare ovarian tumor, accounting for 3e5% of all ovarian malignancies and for 30e50% of all ovarian germ cell malignancies. 1,2 Since dysgerminoma tends to occur in a young age group, 2 future fertility preservation should be considered when surgical intervention is performed for dysgerminoma detected at an early stage. 3 Address correspondence to: Akihiro Takeda, MD, Department of Obstetrics and Gynecology, Gifu Prefectural Tajimi Hospital, Maebata-cho 5-161, Tajimi, Gifu , Japan; gyendoscopy@gmail.com Ó 2009 North American Society for Pediatric and Adolescent Gynecology Published by Elsevier Inc. Adnexal torsion is an uncommon but significant cause of acute lower abdominal pain and morbidity. 4 Most cases of adnexal torsion in young girls are secondary to adnexal pathology and it has been reported that ovarian masses inducing torsion are usually benign. 5 There are few reports of adnexal torsion due to ovarian dysgerminoma in the literature. 6,7 With advances in laparoscopic surgical procedures and the development of surgical equipment, the advantages of laparoscopic treatment over laparotomy for adnexal diseases occurring in young girls have been reported. 8 This form of minimally invasive surgery provides potential benefits for improved cosmetic appearance, shorter hospital stay, reduced postoperative pain, and earlier postoperative return to daily activities. Therefore, the application of laparoscopic procedures for the treatment of gynecological disorders occurring in young girls would be highly desirable. 9,10 Furthermore, our previous report showed that selected solid adnexal tumors could be managed by laparoscopic surgery, if tumor spillage can be avoided by retrieving the tumor tissue in an endobag. 11 In the present report, successful laparoscopic management of ovarian dysgerminoma presenting with an acute abdominal emergency due to adnexal torsion is described. Case Presentation A 17-year-old sexually inactive high school girl (height: 162 cm, body weight: 57 kg) with no previous disease history complained of sudden severe left lower abdominal pain at 8:30 AM on December 30th, 2006 and was brought to a hospital at 10:00 AM. Because the presence of lower abdominal tumor was suspected by ultrasonography, she was transferred to /09/$34.00 doi: /j.jpag

2 e10 Takeda et al: Dysgerminoma with Adnexal Torsion Fig. 1. (A) Transabdominal sonography of dysgerminoma with adnexal torsion. Presence of homogeneous solid mass measuring 7 cm in diameter is noted in the lower abdomen. (B) Laparoscopic finding of twisted adnexa with ovarian dysgerminoma. Neither adhesion nor capsule rupture was noted. (C) After untwisting, left adnexal tissue was excised by LigaSure Atlas. (D) Excised tissue was retrieved in an endobag and extracted through the 12-mm port. the emergency department of our hospital at 11:00 AM. Transabdominal ultrasonography showed a homogeneous mass measuring 7 cm in diameter in the lower abdomen (Fig. 1A). Urinary human chorionic gonadotropin (hcg) test with a sensitivity of 25 IU/ L was weakly positive, suggesting a germ cell origin of this tumor. Under a diagnosis of solid adnexal tumor with torsion, she immediately underwent emergency laparoscopic surgery at 12:30 PM. Since the patient was assumed to have difficulty in understanding her condition due to severe pain, preoperative informed consent was obtained from her family, including a statement that laparotomy might be required if the mass could not be managed by laparoscopy due to advanced malignant condition of disease, or salpingo-oophorectomy might be required if malignant condition was highly suspected under laparoscopic observation. Gasless laparoscopic surgery was performed, according to the procedure previously described with some modifications. 9e11 Briefly, under endotracheal general anesthesia, the abdominal wall was lifted using a subcutaneous lift system (Mizuho Co., Tokyo, Japan) and a 5-mm Endopath XCEL trocar (Ethicon Japan, Tokyo, Japan) was placed at the superior edge of the umbilicus to introduce the 5-mm laparoscope (Olympus, Tokyo, Japan). Additional 5-mm (on the right side) and 12-mm (on the left side) Endopath XCEL trocars were placed under direct vision at the level of the umbilicus. Under laparoscopic observation, left adnexal tissue was found to be twisted 720 (Fig. 1B). Peritoneal washing cytology was negative for malignant tumor cells. After untwisting of the adnexal tissue, the grade of blood-flow resumption was assessed by the change in color. 9,10 Because ischemic change of the left adnexal tissue was assumed to be irreversible and positive urinary hcg test suggested suspicious germ cell origin of this solid ovarian tumor, it was assessed that preservation of the left ovarian tissue would be difficult. Left salpingo-oophorectomy was performed carefully using LigaSure Atlas (Tyco Health Care, Tokyo, Japan) to avoid damage to the tumor capsule (Fig. 1C) and the excised tissue was placed into an Endopouch retriever (Ethicon Japan, Tokyo, Japan)(Fig. 1D). Tissue was extracted through the 12-mm port after fragmentation with scissors in the retrieval bag. Because apparent enlargement of the right adnexa was not observed, there was no tissue sampling obtained from the right ovary. After extensive washing with saline,

3 Takeda et al: Dysgerminoma with Adnexal Torsion e11 the abdominal cavity was finally inspected for hemostasis and additional pathological condition. Neither peritoneal metastatic implants nor apparent pelvic lymph node enlargement were noted under laparoscopic observation. Surgical wound was closed by subcutaneous suturing and covered by Karayahesive hydrocolloidal dressing (Alcare, Tokyo, Japan). Excised tissue weight was 116 g. Surgical duration was 72 minutes and the estimated blood loss was 160 ml. There were no intraoperative or postoperative complications. Preoperative serum tumor marker values (alpha fetoprotein: 2.71 ng/ml, carcinoembryonic antigen: 0.39 ng/ml, CA19-9: 6.62 U/ml, CA125: U/ml, CA72-4: 3.0 U/ml, SCC: 0.5 ng/ml) were within the normal range; however, serum LDH: 261 IU/l (normal range: IU/L) and serum b-hcg: miu/ml (normal range: 0e6 miu/ ml) showed slight elevation. The patient was discharged 3 days after surgery. Postoperatively, serum LDH and b-hcg immediately decreased to the normal value. This tumor was histopathologically diagnosed as pure dysgerminoma of the left ovary (Fig. 2A) with the presence of placental alkaline phosphatase-positive cells by immunohistochemical staining (Fig. 2B). Despite slight elevation of serum hcg, an extensive search could not identify the hcg-positive cells in the immunohistochemically stained tissue sections. After a histological diagnosis was obtained, CT imaging was performed, but apparent metastatic foci were not detected. During discussions about a second surgery for surgical staging and adjuvant chemotherapy, the patient and her family expressed their preference for observation of the disease condition. There have been no signs of tumor recurrence for 8 months since surgery. Discussion Germ cell tumors are derived from the primordial germ cell of the ovary and are classified based on histologic similarity to embryonic or extraembryonic structures. 1 Histologically, dysgerminomas consist of large aggregates of uniform, round cells that have not differentiated into embryonic or extraembryonic structures. These tumors are also characterized by infiltrating channels of lymphocytes admixed with connective tissue stroma. The presence of placental alkaline phosphatase-positive cells demonstrated in tumor tissue by immunohistochemistry aids the diagnosis, 12 as in the present case. Typically, dysgerminomas are known to be associated with increased serum LDH, CA125 and hcg; however, serum AFP should be normal. 13 This elevated hcg is derived from the occasional presence of syncytiotrophoblasts in dysgerminoma tissue. However, approximately 10% of malignant germ cell tumors are mixed with other tissue types. 2 Since treatment and determination of prognosis of the patient with a mixed germ cell tumor should be based on the nondysgerminomatous component in the tumor tissue, 1 histological search for choriocarcinoma component is important to decide the postoperative treatment strategy in dysgerminoma case with elevated serum hcg. In the present case, preoperative urinary hcg test was weakly positive and the serum hcg showed slight elevation; however, the choriocarcinoma component could not be identified by immunohistochemical staining of histological sections. Thus, the final diagnosis was pure dysgerminoma without any mixed germ cell components. Since dysgerminomas tend to occur in a young age group with the peak incidence at 15e19 years of age, 2 Fig. 2. Histological findings of dysgerminoma with adnexal torsion. (A) Large aggregates of uniform, round cells existed with infiltrating channels of lymphocytes admixed with connective tissue stroma. (Hematoxylin-Eosin staining, scale bar: 200 mm) (B) Presence of placental alkaline phosphatase-positive cells was shown by immunohistochemical staining. (Scale bar: 200 mm).

4 e12 Takeda et al: Dysgerminoma with Adnexal Torsion preservation of fertility should be considered at surgical management for these young patients with dysgerminoma. 3 For this reason, unilateral salpingooophorectomy could be reasonable for dysgerminoma detected at the early stage by preoperative image diagnostic procedures and intraoperative findings, 11 instead of exploratory laparotomy to remove the mass and determine the extent of the disease by a thorough and systemic exploration of the abdomen and pelvis. Although bilateral involvement is noted in 10% to 20% of dysgerminoma cases, 7 biopsy of the contralateral ovary, which may cause adhesion formation, is not required 1 if the contralateral ovary appears normal as in the present case. After histological diagnosis of dysgerminoma is established, the decision regarding adjuvant therapy must be made after weighing the toxicity of treatment against the benefits, which include not only reducing the risk of recurrence but also increasing the chance for cure. Adjuvant chemotherapy is not routinely done for well-staged stage I patients with dysgerminoma despite a high recurrence rate (15e25%), since the literature has shown that early recurrence can be salvaged with chemotherapy. 13 In the present case, follow-up by observation of the disease condition was chosen after denying the presence of apparent metastatic foci by postoperative image diagnostic procedures and discussion with the patient and family about risks and benefits of additional treatments. Eight months after surgery, there has has been no sign of recurrence. However, careful clinical, radiologic, and serologic follow-up is required for an extended period of time, since most dysgerminomas recur within 2 years and, in addition, late recurrence has also been noted. 13 Adnexal torsion is an uncommon but significant cause of acute lower abdominal pain and morbidity. 4 Most cases of adnexal torsion are secondary to adnexal pathology and it has been found that ovarian masses undergoing torsion are usually benign. 5 There are few reports on adnexal torsion due to ovarian dysgerminoma in the literature. 6,7 However, since subacute abdominal pain and a palpable pelvic mass are the most common symptoms and sign in a girl or young women presenting with a germ cell tumor as a result of rupture, torsion or hemorrhage, 1 the presence of malignancy should be considered when a patient with solid adnexal tumor presents with acute abdomen. Laparotomic surgery is supposed to be the treatment of choice in the majority of cases similar to the present case, especially under emergency settings. However, with advances in laparoscopic surgical procedures and the development of surgical equipment, the advantages of laparoscopic treatment over laparotomy for adnexal diseases occurring in young girls have been well recognized, even in cases requiring emergency surgery. 9,10 In our department, a laparoscopic approach has been the primary surgical intervention selected for over 13 years, if the surgical indication fulfills our criteria for management of adnexal mass. 11 Briefly, in suspicious cases, patients with tumors!10e12 cm that could be retrieved in an endobag are chosen for laparoscopic surgery and the final decision to pursue laparoscopic surgery is made after finally confirming the disease condition, including presence of severe adhesion formation, rupture of the tumor capsule, and apparent metastatic foci under laparoscopic observation. When the laparoscopic procedure is performed for suspicious adnexal mass, spillage of tumor tissue should be minimized by careful manipulation of tumor tissue, because recurrent dysgerminoma was reported at the site of tumor removal after laparoscopic treatment by recovery of tumor tissue through posterior colpotomy without retrieval in an endobag. 14 Furthermore, even in a suspicious case detected at the early stage, sufficient information should be given to the patient and family about the risks of laparoscopic procedures for malignant ovarian tumors, because the laparoscopic management of ovarian cancer remains controversial and vertical midline laparotomy remains the gold standard. 15 Conclusions Ovarian dysgerminoma should be part of the differential diagnosis in a young girl presenting with acute abdomen when a solid adnexal mass is detected by ultrasonography. Laparoscopic management with careful postoperative follow-up to monitor recurrence could be a treatment option in a young girl if the disease condition is detected at the early stage. References 1. Lu KH, Gershenson DM: Update on the management of ovarian germ cell tumor. J Reprod Med 2005; 50: Smith HO, Berwick M, Verschraegen CF, et al: Incidence and survival rates for female malignant germ cell tumors. Obstet Gynecol 2006; 107: Casey AC, Bhodauria S, Shapter A, et al: Dysgerminoma: the role of conservative surgery. Gynecol Oncol 1996; 63: Bayer AI, Wiskind AK: Adnexal torsion: Can the adnexa be saved? Am J Obstet Gynecol 1994; 171: Kokoska ER, Keller MS, Weber TR: Acute ovarian torsion in children. Am J Surg 2000; 180: Tatekawa Y, Kemmotsu H, Mouri T, et al: A case of pediatric ovarian dysgerminoma associated with high serum levels and positive immunohistochemical staining of neuron-specific enolase. J Pediatr Surg 2004; 39: Varras M, Tsikini A, Polyzos D, et al: Internal hemorrhage caused by a twisted malignant ovarian dysgerminoma:

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