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A Huge Ovarian Mucinous Cystadenoma in a 14-Year-Old Premenarchal Girl: Review on Ovarian Mucinous Tumor in Premenarchal Girls
J Pediatr Adolesc Gynecol (2008) 21:41e44
Case Reports A Huge Ovarian Mucinous Cystadenoma in a 14-Year-Old Premenarchal Girl: Review on Ovarian Mucinous Tumor in Premenarchal Girls _ Aysxe Karaman, MD, Mu¨jdem Nur Azılı, MD, Esin Cengiz Bodurog˘lu, MD1, Ibrahim Karaman, MD, ¨ zden C Derya Erdog˘an, MD, Yusuf Hakan C¸avus xog˘lu, MD, Mustafa Kemal Aslan, MD, and O ¸ akmak, MD Dr. Sami Ulus Children’s Hospital, Department of Pediatric Surgery and Pathology1, Ankara, Turkey
Abstract. Superficial epithelial ovarian tumors are unusual in adolescent girls (when compared with adult women) and extremely rare before menarche. Mucinous cystadenoma (MCA) in children that is a rare form of epithelial tumor is a benign cystic ovarian neoplasm. To our knowledge, there are only eight cases of mucinous cystadenoma, three of borderline mucinous cystadenoma, and three of mucinous cystadenocarcinoma reported in the English-language literature. We present a 14-year-old premenarchal girl with a giant ovarian mucinous cystadenoma. This review is supported by the finding that epithelial ovarian neoplasms are extremely rare prior to puberty and that only 14 mucinous tumors have been reported prior to menarche.
Case Report
Address correspondence to: Aysxe Karaman, MD, Ko¨ylu¨ler Sokak, 15/2, 06590, Cebeci, Ankara, Turkey; E-mail: [email protected]
A 14-year-old premenarchal girl presented with 6month history of increasing abdominal distension (Fig. 1). Constipation was added to her complaints for the last week. A smooth and firm mass occupying the entire abdomen was palpable. Ultrasound demonstrated a giant multiloculated cystic mass arising from the pelvis reaching the level of the processus xiphoideus. CT showed an enormous mass occupying almost the entire abdomen. Tumor markers (AFP, CEA, hCG, CA-125, CA 19-9) were within the normal range. Laparotomy was performed by midline incision. At surgical exploration, the tumor was found to originate from the right ovary. The examination of the pelvis, abdominal walls, diaphragmatic surface, and peritoneum was not indicative of implants or metastases. Free fluid in the abdomen was sampled for cytological assessment. Partial omentectomy and wedge biopsy from the left ovary, which had surface abnormalities, were performed. Unilateral salpingo-oophorectomy with tumor removal was performed. The tumor measured 403020 cm and weighed 7.25 kg (Fig. 2). On gross examination, the cystic mass was filled and expanded with thick tenacious mucinous material and shown to be multiloculate with thin septa. Cysts had smooth surfaces without papillary formations. Cyst walls were quite thin; the thickest cyst walls measured only 0.3 cm. Microscopically, cysts were lined with single layered mucinous epithelium of tall columnar cells. Multiloculation due to ‘daughter-cyst’ formations was found only rarely (Fig. 3). Neither mitotic activity nor atypical changes were seen. Microscopic appearances were of a MCA showing no malign changes. On cytological examination, the peritoneal fluid was normal. Wedge biopsy of the left
Ó 2008 North American Society for Pediatric and Adolescent Gynecology Published by Elsevier Inc.
1083-3188/08/$34.00 doi:10.1016/j.jpag.2007.09.005
Key Words. Mucinous cystadenoma—Mucinous tumor—Epithelial ovarian tumor—Premenarchal— Child
Introduction Ovarian tumors represent less than 2% of all tumors in girls less than 16 years of age. Less than 20% of ovarian tumors are reported to arise from ovarian surface epithelial cells.1,2 Mucinous tumors of the ovary occur principally in middle adult life and are extremely rare prior to menarche.1,3 The following report describes a patient with a giant abdominal mass that was originated in the right ovary. By histology the tumor was diagnosed as MCA that was a very rare entity in childhood. This is the ninth case of mucinous cystadenoma in a premenarchal girl.
42
Karaman et al: Ovarian Mucinous Tumor in Premenarchal Girls
Fig. 3. Multiple cyst formations due to daughter cysts, HE. Fig. 1. Patient showing the abdominal distension.
ovary and biopsy from omentum both appeared nonspecific. The patient is followed up regularly; there is no evidence of recurrence at one year.
Discussion Ovarian masses are uncommon in children.1,2,4 Of these, up to 64% are malignant with majority arising from germ cells.1,4 Only 10e17% of them are epithelial tumors.5,6 All ovarian epithelial neoplasms are by far encountered most commonly in adult age groups but are also seen in young girls.1,7,8,9 The most common types of epithelial neoplasm encountered were benign cystadenoma, of which 75% were serous cystadenomas and 25% were MCAs. Mucinous tumors of the ovary occur principally in middle adult life and are extremely rare prior to menarche.1
Fig. 2. Macroscopy of the specimen.
Three histopathological groups of mucinous tumors are described1: (1) MCA, (2) mucinous tumor of uncertain malignant potential (borderline) and (3) mucinous carcinoma. Table 1 summarizes a review of 15 cases of ovarian mucinous tumors in premenarchal girls reported in the literature. MCA appears as a large cystic mass, often multiloculates and contains sticky gelatinous fluid. Microscopically, the tumor consists of cystic spaces lined by tall columnar epithelium with mucinous differentiation.1 Quint and Smith10 reported the first case of MCA in a premenarchal girl. In the literature review, we found only 8 previous cases of MCA of the ovary in premenarchal girls that were reported in the English-language literature.1,5,6,8-10 In general, ovarian MCAs tend to present with abdominal distension. An ultrasound scan would be the first line of investigation with a palpable mass. When there is a diagnostic doubt, either a CT or a MRI scan is performed. Generally, it is not possible to accomplish ovary sparing surgery in this tumor, which is too big despite its benign nature. The treatment for benign MCA usually has consisted of salpingo-oophorectomy and no further treatment is required. Mucinous tumor of uncertain malignant potential is also rare in the premenarchal period. Minor epithelial atypicality is compatible with the diagnosis of MCA but significant epithelial stratification, bridging, prominent papillary projections and also nuclear atypia are indicative of borderline malignancy.5 Mucinous borderline tumors have been subclassified into intestinal and endocervical subtypes, which differ both pathologically and clinically. Only three reported cases were found in the literature.3,7,8 There was no problem encountered in the follow-up period. In this tumor group, clinical follow-up is advised. Mucinous carcinoma is a rare malignant tumor in premenarchal girls. Hernandez et al11 reported the
Salpingo-oophorectomy
first case of invasive mucinous carcinoma in a 10year-old in 1982. After that two additional case reports were found in literature review.2,4 All of them received chemotherapy following salpingo-oophorectomy, and Hernandez et al11 reported the subject to have normal puberty. The subject in Shankar et al2 was determined to have right ovarian tumor recurrence 15 months later and she succumbed to metastatic disease 2 years after diagnosis. The vast majority of mucinous tumors are benign (75%), 10% borderline, and 15% carcinomas in adult series12; in our review it was found that in premenarchal girls the tumor is more likely to be borderline (20%) and malignant (20%). We did not find any discussion about the likelihood of subsequently developing a mucinous tumor in the contralateral ovary. We found only one case (6.6%), in a premenarchal girl who developed contralateral ovarian tumor recurrence, after a mucinous tumor operation. In differential diagnosis, physicians always keep in mind other intraabdominal masses and ovarian masses, especially ovarian germ cell tumors, which are predominant in children. Therefore, imaging techniques and serum tumor makers must be used to investigate preoperatively. Unilateral oophorectomy and unilateral salpingoophorectomy are the treatment of choice in these cases. In conclusion, in the pediatric population, an ovarian neoplasm presents both a diagnostic and therapeutic dilemma, and very often the diagnoses are missed or delayed. Epithelial neoplasms form a minority of childhood ovarian tumors, and tend to occur in postmenarchal adolescent girls. However, very little is known about the behavior of these neoplasms in premenarchal girls, because they are extremely rare. Management strategies are based on experiences gathered from treating similar tumors in postmenarchal adolescent girls and adults.
7.2 kg 630 g 6.8 kg
Salpingo-oophorectomy Salpingo-oophorectomy Salpingo-oophorectomy Oophorectomy Salpingo-oophorectomy Salpingo-oophorectomy Salpingo-oophorectomy Salpingo-oophorectomy Salpingo-oophorectomy Salpingo-oophorectomy
Invasive mucinous cystadenocarcinoma
Endocervical epithelium Endocervical type Cervical and intestinal epithelium
4 years 2.6 years 16.2 years 3.6 years 1 year 1 year 3 years 1.1 year Normal puberty 15 months later right nu¨x
? 1 year 30 months Not reported Endocervical epithelium Cervical type
Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Borderline mucinous cystadenoma Borderline mucinous cystadenoma Borderline mucinous cystadenoma Invasive mucinous cystadenocarcinoma Invasive mucinous cystadenocarcinoma
Not reported Left
Right Left Left Left Right Left Right Right
403020cm 16cm 402020cm
Salpingo-oophorectomy Salpingo-oophorectomy 1.8 kg 7 kg
43
References
Morowitz4
Our case Deprest7 Flotho3 Stankovic8 Hernandez11 Shankar2
Stankovic8
Quint10 Yazıcı5 Vizza9 Grapsa6 Sri Paran1
13y 13y 11y 13y 14y 13.3y 13.4y 14y 12y 15y 12.5y 10y 11y
Left Left
Not reported 211810cm 423629cm Not reported 341816cm 261414cm
Operation Weigh Size Age
Table 1. Mucinous tumors of the ovary in premenarchal girls
Diagnosis
Histopathological type
Follow-up
Karaman et al: Ovarian Mucinous Tumor in Premenarchal Girls
1. Sri Paran T, Mortell A, Devaney D, et al: Mucinous cystadenoma of the ovary in perimenarchal girls. Pediatr Surg Int 2006; 22:224 2. Shankar KR, Wakhlu A, Kokai GK, et al: Ovarian adenocarcinoma in premenarchal girls. J Pediatr Surg 2001; 36: 511 3. Flotho C, Ru¨ckauer K, Duffner U, et al: Mucinous cystadenoma of the ovary in a 15-year-old girl. J Pediatr Surg 2001; 36:E6 4. Morowitz M, Huff D, von Allmen D: Epithelial ovarian tumors in children: a retrospective analysis. J Pediatr Surg 2003; 38:331 5. Yazıcı M, Etensel B, Gu¨rsoy H, et al: Mucinous cystadenoma: a rare abdominal mass in childhood. Eur J Pediatr Surg 2002; 12:330
44
Karaman et al: Ovarian Mucinous Tumor in Premenarchal Girls
6. Grapsa D, Kairi-Vassilatou E, Hasiakos D, et al: Ovarian mucinous cystadenoma with extended calcification in an 11-year-old girl: case report and review of the literature. Clin Exp Obstet Gynecol 2006; 33:181 7. Deprest J, Moerman P, Corneillie P, et al: Ovarian borderline mucinous tumor in a premenarchal girl: review on ovarian epithelial cancer in young girls. Gynecol Oncol 1992; 45:219 8. Stankovic Z, Djuricic S, Djukic M, et al: Epithelial ovarian tumors and CA 125 in premenarchal girls. Eur J Gynaecol Oncol 2006; 27:597
9. Vizza E, Galati GM, Corrado G: Voluminous mucinous cystadenoma of the ovary in 13-year-old girl. J Pediatr Adolesc Gynecol 2005; 18:419 10. Quint EH, Smith YR: Ovarian surgery in premenarchal girls. J Pediatr Adolesc Gynecol 1999; 12:27 11. Hernandez E, Rosenshein NB, Parmley TH: Mucinous cystadenocarcinoma in a premenarchal girl. South Med J 1982; 75:1265 12. Rodrı´guez IM, Prat J: Mucinous tumors of the ovary. A clinicopathologic analysis of 75 borderline tumors (of intestinal type) and carcinomas. Am J Surg Pathol 2002; 26:139
Case Reports A Huge Ovarian Mucinous Cystadenoma in a 14-Year-Old Premenarchal Girl: Review on Ovarian Mucinous Tumor in Premenarchal Girls _ Aysxe Karaman, MD, Mu¨jdem Nur Azılı, MD, Esin Cengiz Bodurog˘lu, MD1, Ibrahim Karaman, MD, ¨ zden C Derya Erdog˘an, MD, Yusuf Hakan C¸avus xog˘lu, MD, Mustafa Kemal Aslan, MD, and O ¸ akmak, MD Dr. Sami Ulus Children’s Hospital, Department of Pediatric Surgery and Pathology1, Ankara, Turkey
Abstract. Superficial epithelial ovarian tumors are unusual in adolescent girls (when compared with adult women) and extremely rare before menarche. Mucinous cystadenoma (MCA) in children that is a rare form of epithelial tumor is a benign cystic ovarian neoplasm. To our knowledge, there are only eight cases of mucinous cystadenoma, three of borderline mucinous cystadenoma, and three of mucinous cystadenocarcinoma reported in the English-language literature. We present a 14-year-old premenarchal girl with a giant ovarian mucinous cystadenoma. This review is supported by the finding that epithelial ovarian neoplasms are extremely rare prior to puberty and that only 14 mucinous tumors have been reported prior to menarche.
Case Report
Address correspondence to: Aysxe Karaman, MD, Ko¨ylu¨ler Sokak, 15/2, 06590, Cebeci, Ankara, Turkey; E-mail: [email protected]
A 14-year-old premenarchal girl presented with 6month history of increasing abdominal distension (Fig. 1). Constipation was added to her complaints for the last week. A smooth and firm mass occupying the entire abdomen was palpable. Ultrasound demonstrated a giant multiloculated cystic mass arising from the pelvis reaching the level of the processus xiphoideus. CT showed an enormous mass occupying almost the entire abdomen. Tumor markers (AFP, CEA, hCG, CA-125, CA 19-9) were within the normal range. Laparotomy was performed by midline incision. At surgical exploration, the tumor was found to originate from the right ovary. The examination of the pelvis, abdominal walls, diaphragmatic surface, and peritoneum was not indicative of implants or metastases. Free fluid in the abdomen was sampled for cytological assessment. Partial omentectomy and wedge biopsy from the left ovary, which had surface abnormalities, were performed. Unilateral salpingo-oophorectomy with tumor removal was performed. The tumor measured 403020 cm and weighed 7.25 kg (Fig. 2). On gross examination, the cystic mass was filled and expanded with thick tenacious mucinous material and shown to be multiloculate with thin septa. Cysts had smooth surfaces without papillary formations. Cyst walls were quite thin; the thickest cyst walls measured only 0.3 cm. Microscopically, cysts were lined with single layered mucinous epithelium of tall columnar cells. Multiloculation due to ‘daughter-cyst’ formations was found only rarely (Fig. 3). Neither mitotic activity nor atypical changes were seen. Microscopic appearances were of a MCA showing no malign changes. On cytological examination, the peritoneal fluid was normal. Wedge biopsy of the left
Ó 2008 North American Society for Pediatric and Adolescent Gynecology Published by Elsevier Inc.
1083-3188/08/$34.00 doi:10.1016/j.jpag.2007.09.005
Key Words. Mucinous cystadenoma—Mucinous tumor—Epithelial ovarian tumor—Premenarchal— Child
Introduction Ovarian tumors represent less than 2% of all tumors in girls less than 16 years of age. Less than 20% of ovarian tumors are reported to arise from ovarian surface epithelial cells.1,2 Mucinous tumors of the ovary occur principally in middle adult life and are extremely rare prior to menarche.1,3 The following report describes a patient with a giant abdominal mass that was originated in the right ovary. By histology the tumor was diagnosed as MCA that was a very rare entity in childhood. This is the ninth case of mucinous cystadenoma in a premenarchal girl.
42
Karaman et al: Ovarian Mucinous Tumor in Premenarchal Girls
Fig. 3. Multiple cyst formations due to daughter cysts, HE. Fig. 1. Patient showing the abdominal distension.
ovary and biopsy from omentum both appeared nonspecific. The patient is followed up regularly; there is no evidence of recurrence at one year.
Discussion Ovarian masses are uncommon in children.1,2,4 Of these, up to 64% are malignant with majority arising from germ cells.1,4 Only 10e17% of them are epithelial tumors.5,6 All ovarian epithelial neoplasms are by far encountered most commonly in adult age groups but are also seen in young girls.1,7,8,9 The most common types of epithelial neoplasm encountered were benign cystadenoma, of which 75% were serous cystadenomas and 25% were MCAs. Mucinous tumors of the ovary occur principally in middle adult life and are extremely rare prior to menarche.1
Fig. 2. Macroscopy of the specimen.
Three histopathological groups of mucinous tumors are described1: (1) MCA, (2) mucinous tumor of uncertain malignant potential (borderline) and (3) mucinous carcinoma. Table 1 summarizes a review of 15 cases of ovarian mucinous tumors in premenarchal girls reported in the literature. MCA appears as a large cystic mass, often multiloculates and contains sticky gelatinous fluid. Microscopically, the tumor consists of cystic spaces lined by tall columnar epithelium with mucinous differentiation.1 Quint and Smith10 reported the first case of MCA in a premenarchal girl. In the literature review, we found only 8 previous cases of MCA of the ovary in premenarchal girls that were reported in the English-language literature.1,5,6,8-10 In general, ovarian MCAs tend to present with abdominal distension. An ultrasound scan would be the first line of investigation with a palpable mass. When there is a diagnostic doubt, either a CT or a MRI scan is performed. Generally, it is not possible to accomplish ovary sparing surgery in this tumor, which is too big despite its benign nature. The treatment for benign MCA usually has consisted of salpingo-oophorectomy and no further treatment is required. Mucinous tumor of uncertain malignant potential is also rare in the premenarchal period. Minor epithelial atypicality is compatible with the diagnosis of MCA but significant epithelial stratification, bridging, prominent papillary projections and also nuclear atypia are indicative of borderline malignancy.5 Mucinous borderline tumors have been subclassified into intestinal and endocervical subtypes, which differ both pathologically and clinically. Only three reported cases were found in the literature.3,7,8 There was no problem encountered in the follow-up period. In this tumor group, clinical follow-up is advised. Mucinous carcinoma is a rare malignant tumor in premenarchal girls. Hernandez et al11 reported the
Salpingo-oophorectomy
first case of invasive mucinous carcinoma in a 10year-old in 1982. After that two additional case reports were found in literature review.2,4 All of them received chemotherapy following salpingo-oophorectomy, and Hernandez et al11 reported the subject to have normal puberty. The subject in Shankar et al2 was determined to have right ovarian tumor recurrence 15 months later and she succumbed to metastatic disease 2 years after diagnosis. The vast majority of mucinous tumors are benign (75%), 10% borderline, and 15% carcinomas in adult series12; in our review it was found that in premenarchal girls the tumor is more likely to be borderline (20%) and malignant (20%). We did not find any discussion about the likelihood of subsequently developing a mucinous tumor in the contralateral ovary. We found only one case (6.6%), in a premenarchal girl who developed contralateral ovarian tumor recurrence, after a mucinous tumor operation. In differential diagnosis, physicians always keep in mind other intraabdominal masses and ovarian masses, especially ovarian germ cell tumors, which are predominant in children. Therefore, imaging techniques and serum tumor makers must be used to investigate preoperatively. Unilateral oophorectomy and unilateral salpingoophorectomy are the treatment of choice in these cases. In conclusion, in the pediatric population, an ovarian neoplasm presents both a diagnostic and therapeutic dilemma, and very often the diagnoses are missed or delayed. Epithelial neoplasms form a minority of childhood ovarian tumors, and tend to occur in postmenarchal adolescent girls. However, very little is known about the behavior of these neoplasms in premenarchal girls, because they are extremely rare. Management strategies are based on experiences gathered from treating similar tumors in postmenarchal adolescent girls and adults.
7.2 kg 630 g 6.8 kg
Salpingo-oophorectomy Salpingo-oophorectomy Salpingo-oophorectomy Oophorectomy Salpingo-oophorectomy Salpingo-oophorectomy Salpingo-oophorectomy Salpingo-oophorectomy Salpingo-oophorectomy Salpingo-oophorectomy
Invasive mucinous cystadenocarcinoma
Endocervical epithelium Endocervical type Cervical and intestinal epithelium
4 years 2.6 years 16.2 years 3.6 years 1 year 1 year 3 years 1.1 year Normal puberty 15 months later right nu¨x
? 1 year 30 months Not reported Endocervical epithelium Cervical type
Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Mucinous cystadenoma Borderline mucinous cystadenoma Borderline mucinous cystadenoma Borderline mucinous cystadenoma Invasive mucinous cystadenocarcinoma Invasive mucinous cystadenocarcinoma
Not reported Left
Right Left Left Left Right Left Right Right
403020cm 16cm 402020cm
Salpingo-oophorectomy Salpingo-oophorectomy 1.8 kg 7 kg
43
References
Morowitz4
Our case Deprest7 Flotho3 Stankovic8 Hernandez11 Shankar2
Stankovic8
Quint10 Yazıcı5 Vizza9 Grapsa6 Sri Paran1
13y 13y 11y 13y 14y 13.3y 13.4y 14y 12y 15y 12.5y 10y 11y
Left Left
Not reported 211810cm 423629cm Not reported 341816cm 261414cm
Operation Weigh Size Age
Table 1. Mucinous tumors of the ovary in premenarchal girls
Diagnosis
Histopathological type
Follow-up
Karaman et al: Ovarian Mucinous Tumor in Premenarchal Girls
1. Sri Paran T, Mortell A, Devaney D, et al: Mucinous cystadenoma of the ovary in perimenarchal girls. Pediatr Surg Int 2006; 22:224 2. Shankar KR, Wakhlu A, Kokai GK, et al: Ovarian adenocarcinoma in premenarchal girls. J Pediatr Surg 2001; 36: 511 3. Flotho C, Ru¨ckauer K, Duffner U, et al: Mucinous cystadenoma of the ovary in a 15-year-old girl. J Pediatr Surg 2001; 36:E6 4. Morowitz M, Huff D, von Allmen D: Epithelial ovarian tumors in children: a retrospective analysis. J Pediatr Surg 2003; 38:331 5. Yazıcı M, Etensel B, Gu¨rsoy H, et al: Mucinous cystadenoma: a rare abdominal mass in childhood. Eur J Pediatr Surg 2002; 12:330
44
Karaman et al: Ovarian Mucinous Tumor in Premenarchal Girls
6. Grapsa D, Kairi-Vassilatou E, Hasiakos D, et al: Ovarian mucinous cystadenoma with extended calcification in an 11-year-old girl: case report and review of the literature. Clin Exp Obstet Gynecol 2006; 33:181 7. Deprest J, Moerman P, Corneillie P, et al: Ovarian borderline mucinous tumor in a premenarchal girl: review on ovarian epithelial cancer in young girls. Gynecol Oncol 1992; 45:219 8. Stankovic Z, Djuricic S, Djukic M, et al: Epithelial ovarian tumors and CA 125 in premenarchal girls. Eur J Gynaecol Oncol 2006; 27:597
9. Vizza E, Galati GM, Corrado G: Voluminous mucinous cystadenoma of the ovary in 13-year-old girl. J Pediatr Adolesc Gynecol 2005; 18:419 10. Quint EH, Smith YR: Ovarian surgery in premenarchal girls. J Pediatr Adolesc Gynecol 1999; 12:27 11. Hernandez E, Rosenshein NB, Parmley TH: Mucinous cystadenocarcinoma in a premenarchal girl. South Med J 1982; 75:1265 12. Rodrı´guez IM, Prat J: Mucinous tumors of the ovary. A clinicopathologic analysis of 75 borderline tumors (of intestinal type) and carcinomas. Am J Surg Pathol 2002; 26:139