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Ovarian Teratoma with Predominant Hemangiomatous Component in Early Childhood
Case Report Ovarian Teratoma with Predominant Hemangiomatous Component in Early Childhood Tip Pongsuvareeyakul MD 1, Jongkolnee Settakorn MD 1, Jiraporn Khorana MD 2, Surapan Khunamornpong MD 1,* 1 2
Department of Pathology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand Department of Surgery, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
a b s t r a c t Background: Ovarian teratoma with predominant hemangiomatous component in pediatric patients is very rare and its presentation may mimic that of malignancy. Case: A 2-year-old girl presented with proptosis of the right eye. A right ovarian mass was detected on the physical examination. The right orbital lesion was consistent with venolymphatic-type vascular malformation on magnetic resonance imaging. The abdominal computed tomographic scan revealed a solid ovarian mass that raised a suspicion for malignancy. A 5.5-cm right ovarian solid hemorrhagic mass was resected. Microscopically, the mass was almost exclusively composed of variable-sized blood vessels, with few microscopic mature teratomatous foci. Summary and Conclusion: Hemangiomatous lesion of the ovary may have a presentation mimicking a malignant neoplasm, and may be associated with multiple vascular lesions. Key Words: Ovary, Hemangioma, Teratoma, Early childhood
Introduction
Case
Ovarian masses are rare in pediatric patients. Ovarian operations account for only 0.2% of all pediatric surgeries.1 The majority of ovarian masses in pediatric patients occur in the late childhood or the adolescent period. In a large series of pediatric ovarian masses, patients in early childhood (between 1 and 8 years of age) accounted for approximately 10% of patients.2 Nevertheless, early childhood patients with ovarian masses had a higher risk of malignancy compared with the other pediatric age groups (ie, approximately 8-fold of those younger than 1 year and 2-3 fold of those aged 9 to 19 years).2 Vascular neoplasms of the ovary are rare, and are hemangiomas in most cases. When an ovarian hemangiomatous lesion contains a teratomatous component, it is regarded as a type of teratoma.3,4 The macroscopic and histologic appearance of the hemangiomatous component in ovarian teratoma is indistinguishable from that of a pure hemangioma of the ovary.4 Although teratoma is the most common type of ovarian neoplasm in either pediatric or adult patients, teratoma with predominant hemangiomatous component has been documented in only a small number of cases.4 Despite a benign nature, the presentation of ovarian hemangiomatous lesions may raise a clinical concern for malignant ovarian neoplasm. We report a case of ovarian teratoma with a predominant hemangiomatous component in an early childhood patient.
A 2-year-old girl presented with swelling in her right eye for 4 months. There was no remarkable past clinical history including ante- and perinatal events. She was the secondborn child in the family, with a healthy older sister. Her growth and development was normal, and she had regularly received vaccinations scheduled for age. There was no known history of familial illness. The physical examination revealed proptosis of the right eye and a right lower abdominal mass. The magnetic resonance imaging of the right orbit showed a 2.5 cm retrobulbar multiseptated cystic lesion in the medial orbital wall with encasement of the right ophthalmic artery, suggestive of venolymphatic-type vascular malformation. The abdominal ultrasonography showed a small amount of ascites and a 5.7-cm solid mass in the right side of the pelvis with scattered foci of calcification, which was suspicious for a malignant neoplasm. Abdominal computerized tomographic scan showed a 5.8 5.3 4.5 cm heterogeneous enhancing mass in the right ovary, which contained multiple enhancing internal septa and speckled calcifications, raising a suspicion for immature teratoma or malignant germ cell neoplasm. The mass pressed on the urinary bladder dome, but there was no evidence of intra-abdominal spread or lymphadenopathy. The serum levels of alpha-fetoprotein, b-human chorionic gonadotropin, and lactate dehydrogenase were not elevated. The patient underwent an exploratory laparotomy, through a right low transverse incision. Intraoperatively, a 5.5-cm right ovarian mass with smooth external surface was observed. There was 30 ml of serous ascites. A thorough intra-abdominal examination showed no peritoneal
The authors have indicated no conflicts of interest. * Address correspondence to: Surapan Khunamornpong, MD, Department of Pathology, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand; Phone: 66-53-945442; fax: 66-53-946727 E-mail address: [email protected] (S. Khunamornpong).
1083-3188/$ - see front matter Ó 2015 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpag.2014.02.013
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T. Pongsuvareeyakul et al. / J Pediatr Adolesc Gynecol 28 (2015) e1ee3
The follow-up visit at 8 months after surgery was uneventful. The orbital vascular lesion was not feasible for surgical excision and was under clinical surveillance. Summary and Conclusion
Fig. 1. Sectioned surface of the right ovarian mass shows lobules of spongy to solid hemorrhagic tissue.
adhesion or nodule and no palpable lymph nodes. The ascitic fluid was collected for cytologic examination, and right oophorectomy was performed. On macroscopic examination, the ovarian mass showed an intact capsule. The sectioned surface was composed of lobules of solid or spongy hemorrhagic tissue with intervening fibrous septa (Fig. 1). Microscopically, the mass was composed of numerous variable-sized blood vessels within collagenous stroma. The vessels ranged from dilated thin-walled (or cavernous-type) vascular channels to variable-sized veins with thick muscular walls, which were prominent in many areas (Fig. 2, A). No atypia of the endothelial lining cells was observed. Thrombotic occlusions with or without calcifications in the vascular lumen were occasionally seen. A few microscopic cystic foci were present within the vascular proliferation. These cysts were lined either with stratified squamous epithelium (Fig. 2, B) or mucin-containining glandular epithelium associated with smooth muscle that resembled intestinal structure (Fig. 2, C). Neither glial tissue nor immature components were identified. A narrow rim of residual normal ovarian tissue was observed at the periphery of the mass with a circumscribed interface. The ascites cytology was negative.
Several features at the time of presentation of ovarian masses in pediatric patients are associated with an increased malignancy risk including chief complaints of an abdominal mass or precocious puberty, age between 1 and 8 years, solid appearance on ultrasonography or CT scan, and imaging tumor size of 5 cm or larger.2 Based on these features, the pre-operative findings in our patients would raise a strong suspicion of malignant ovarian neoplasm. Although serologic tumor markers (alpha-fetoprotein, bhuman chorionic gonadotropin, CA125, or carcinoembryonic antigen) are of value in preoperative evaluation for malignant germ cell tumor, it should be noted that these tumor markers may not be elevated in up to 46% of pediatric ovarian malignant neoplasms.2 Intraoperative presence of ascites and the solid and hemorrhagic gross appearance of tumor may also appear worrisome for malignancy. In the present case, oophorectomy was justified based on the absence of radiologic and intraoperative evidence of neoplastic spread beyond the ovary. A teratoma with predominance of a single tissue type (or monodermal teratoma) that is composed of hemangiomatous component has been rarely described and appears to be less common than a pure ovarian hemangioma.3-5 The latter is classified as a mesenchymal neoplasm in the category of nonspecific group of primary ovarian tumors other than the 3 major groups (ie, epithelial-stromal, sex cordstromal, and germ cell tumors). The identification of teratoma foci is considered to be the feature that distinguishes a teratoma with predominant hemangiomatous component from an ovarian hemangioma.3,4 The hemangiomatous component should be distinguished from florid vascular proliferation, which is composed of immature-appearing small and thin-walled vessels and is typically associated with a neural tissue component in immature teratoma without a grossly circumscribed mass-like growth.6
Fig. 2. Microscopic appearance of the right ovarian mass (hematoxylin and eosin). (A) The predominant component is composed of variable-sized blood vessels with thin or thick walls (40). (B) A small cystic space lined with stratified squamous epithelium surrounded by blood vessels (100). (C) A focus of mucin-producing glandular epithelium among the proliferating vessels is partially surrounded with smooth muscle, imparting an intestinal structure (100).
T. Pongsuvareeyakul et al. / J Pediatr Adolesc Gynecol 28 (2015) e1ee3
To our knowledge, less than 10 well-documented cases of ovarian teratoma or germ cell neoplasm with hemangiomatous component have been reported, with only 2 of these occurring in pediatric patients.4,7,8 One patient was a 4-year-old girl presenting with progressive abdominal enlargement for a 2-month duration. Macroscopically, a 10cm right ovarian mass showed a solid sectioned surface with small hemorrhagic cysts and necrotic foci. Histologically, the mass was composed of cavernous hemangioma associated with a microscopic teratomatous component characterized by a squamous cyst.7 The other case was a 16-year-old girl with Turner syndrome who had a 15-cm right gonadal mass composed of cavernous hemangioma in half of the mass, merging with a mixed germ cell tumor which included a minor component of immature teratoma in the other half.8 The left gonad of this patient also contained a 1.2-cm gonadoblastoma.8 The histologic appearance of ovarian hemangiomatous lesions, either with or without teratomatous component, was described as thin-walled vascular channels of variable sizes (capillary or cavernous) in most previous reports.5 In our patient, large veins with thick muscular wall were also prominent in many areas and this resulted in an appearance similar to a venous hemangioma rather than a cavernous hemangioma. The presence of bilateral involvement or extra-ovarian vascular lesions has been reported in approximately 10% of patients with ovarian hemangioma.5 Bilateral involvement of ovarian teratoma with hemangiomatous component was also previously described in a 26-year-old patient.3 In our patient, there was a co-existent orbital vascular lesion that caused the presenting symptom which led to the detection of an ovarian mass on physical examination. Therefore the diagnosis of an ovarian hemangiomatous lesion, either associated with teratoma or not, may indicate a clinical evaluation for the presence of multiple vascular lesions. Our patient had no familial history of
e3
vascular lesions that might suggest certain hereditary syndromes associated with multiple vascular lesions. The clinical course of ovarian hemangioma is benign, as would be expected for that of a hemangiomatous lesion of teratomatous origin.3-5 Surgical excision would be an optimal treatment. Conservative surgery of enucleation of the mass should be considered when technically possible to preserve the ovarian function such as in a patient with a small lesion in the absence of clinically malignant characteristics. In a patient with a clinical suspicion for ovarian malignancy, intraoperative frozen section of the mass may help in making a decision for conservative surgery by a confirmation of the hemangiomatous nature. Acknowledgments
This study was supported by The National Research University Project under Thailand's Office of the Higher Education Commission. References 1. Cass DL, Hawkins E, Brandt ML, et al: Surgery for ovarian masses in infants, children, and adolescents: 102 consecutive patients treated in a 15-year period. J Pediatr Surg 2001; 36:693 2. Oltmann SC, Garcia N, Barber R, et al: Can we preoperatively risk stratify ovarian masses for malignancy? J Pediatr Surg 2010; 45:130 3. Feuerstein IM, Aronson BL, McCarthy EF: Bilateral ovarian cystic teratomata mimicking bilateral pure ovarian hemangiomata: case report. Int J Gynecol Pathol 1984; 3:393 4. Itoh H, Wada T, Michikata K, et al: Ovarian teratoma showing a predominant hemangiomatous element with stromal luteinization: report of a case and review of the literature. Pathol Int 2004; 54:279 lu H, Gu € ngo €r T, Oz M, et al: Primary ovarian hemangioma: case report 5. Bayramog and review of literature. Med J Islam World Acad Sci 2012; 20:106 6. Baker PM, Rosai J, Young RH: Ovarian teratomas with florid benign vascular proliferation: a distinctive finding associated with the neural component of teratomas that may be confused with a vascular neoplasm. Int J Gynecol Pathol 2002; 21:16 7. Gay RM, Janovski NA: Cavernous hemangioma of the ovary. Gynaecologia 1969; 168:248 8. Tanaka Y, Sasaki Y, Tachibana K, et al: Gonadal mixed germ cell tumor combined with a large hemangiomatous lesion in a patient with Turner's syndrome and 45,X/46,X, þmar karyotype. Arch Pathol Lab Med 1994; 118:1135
Department of Pathology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand Department of Surgery, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
a b s t r a c t Background: Ovarian teratoma with predominant hemangiomatous component in pediatric patients is very rare and its presentation may mimic that of malignancy. Case: A 2-year-old girl presented with proptosis of the right eye. A right ovarian mass was detected on the physical examination. The right orbital lesion was consistent with venolymphatic-type vascular malformation on magnetic resonance imaging. The abdominal computed tomographic scan revealed a solid ovarian mass that raised a suspicion for malignancy. A 5.5-cm right ovarian solid hemorrhagic mass was resected. Microscopically, the mass was almost exclusively composed of variable-sized blood vessels, with few microscopic mature teratomatous foci. Summary and Conclusion: Hemangiomatous lesion of the ovary may have a presentation mimicking a malignant neoplasm, and may be associated with multiple vascular lesions. Key Words: Ovary, Hemangioma, Teratoma, Early childhood
Introduction
Case
Ovarian masses are rare in pediatric patients. Ovarian operations account for only 0.2% of all pediatric surgeries.1 The majority of ovarian masses in pediatric patients occur in the late childhood or the adolescent period. In a large series of pediatric ovarian masses, patients in early childhood (between 1 and 8 years of age) accounted for approximately 10% of patients.2 Nevertheless, early childhood patients with ovarian masses had a higher risk of malignancy compared with the other pediatric age groups (ie, approximately 8-fold of those younger than 1 year and 2-3 fold of those aged 9 to 19 years).2 Vascular neoplasms of the ovary are rare, and are hemangiomas in most cases. When an ovarian hemangiomatous lesion contains a teratomatous component, it is regarded as a type of teratoma.3,4 The macroscopic and histologic appearance of the hemangiomatous component in ovarian teratoma is indistinguishable from that of a pure hemangioma of the ovary.4 Although teratoma is the most common type of ovarian neoplasm in either pediatric or adult patients, teratoma with predominant hemangiomatous component has been documented in only a small number of cases.4 Despite a benign nature, the presentation of ovarian hemangiomatous lesions may raise a clinical concern for malignant ovarian neoplasm. We report a case of ovarian teratoma with a predominant hemangiomatous component in an early childhood patient.
A 2-year-old girl presented with swelling in her right eye for 4 months. There was no remarkable past clinical history including ante- and perinatal events. She was the secondborn child in the family, with a healthy older sister. Her growth and development was normal, and she had regularly received vaccinations scheduled for age. There was no known history of familial illness. The physical examination revealed proptosis of the right eye and a right lower abdominal mass. The magnetic resonance imaging of the right orbit showed a 2.5 cm retrobulbar multiseptated cystic lesion in the medial orbital wall with encasement of the right ophthalmic artery, suggestive of venolymphatic-type vascular malformation. The abdominal ultrasonography showed a small amount of ascites and a 5.7-cm solid mass in the right side of the pelvis with scattered foci of calcification, which was suspicious for a malignant neoplasm. Abdominal computerized tomographic scan showed a 5.8 5.3 4.5 cm heterogeneous enhancing mass in the right ovary, which contained multiple enhancing internal septa and speckled calcifications, raising a suspicion for immature teratoma or malignant germ cell neoplasm. The mass pressed on the urinary bladder dome, but there was no evidence of intra-abdominal spread or lymphadenopathy. The serum levels of alpha-fetoprotein, b-human chorionic gonadotropin, and lactate dehydrogenase were not elevated. The patient underwent an exploratory laparotomy, through a right low transverse incision. Intraoperatively, a 5.5-cm right ovarian mass with smooth external surface was observed. There was 30 ml of serous ascites. A thorough intra-abdominal examination showed no peritoneal
The authors have indicated no conflicts of interest. * Address correspondence to: Surapan Khunamornpong, MD, Department of Pathology, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand; Phone: 66-53-945442; fax: 66-53-946727 E-mail address: [email protected] (S. Khunamornpong).
1083-3188/$ - see front matter Ó 2015 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. http://dx.doi.org/10.1016/j.jpag.2014.02.013
e2
T. Pongsuvareeyakul et al. / J Pediatr Adolesc Gynecol 28 (2015) e1ee3
The follow-up visit at 8 months after surgery was uneventful. The orbital vascular lesion was not feasible for surgical excision and was under clinical surveillance. Summary and Conclusion
Fig. 1. Sectioned surface of the right ovarian mass shows lobules of spongy to solid hemorrhagic tissue.
adhesion or nodule and no palpable lymph nodes. The ascitic fluid was collected for cytologic examination, and right oophorectomy was performed. On macroscopic examination, the ovarian mass showed an intact capsule. The sectioned surface was composed of lobules of solid or spongy hemorrhagic tissue with intervening fibrous septa (Fig. 1). Microscopically, the mass was composed of numerous variable-sized blood vessels within collagenous stroma. The vessels ranged from dilated thin-walled (or cavernous-type) vascular channels to variable-sized veins with thick muscular walls, which were prominent in many areas (Fig. 2, A). No atypia of the endothelial lining cells was observed. Thrombotic occlusions with or without calcifications in the vascular lumen were occasionally seen. A few microscopic cystic foci were present within the vascular proliferation. These cysts were lined either with stratified squamous epithelium (Fig. 2, B) or mucin-containining glandular epithelium associated with smooth muscle that resembled intestinal structure (Fig. 2, C). Neither glial tissue nor immature components were identified. A narrow rim of residual normal ovarian tissue was observed at the periphery of the mass with a circumscribed interface. The ascites cytology was negative.
Several features at the time of presentation of ovarian masses in pediatric patients are associated with an increased malignancy risk including chief complaints of an abdominal mass or precocious puberty, age between 1 and 8 years, solid appearance on ultrasonography or CT scan, and imaging tumor size of 5 cm or larger.2 Based on these features, the pre-operative findings in our patients would raise a strong suspicion of malignant ovarian neoplasm. Although serologic tumor markers (alpha-fetoprotein, bhuman chorionic gonadotropin, CA125, or carcinoembryonic antigen) are of value in preoperative evaluation for malignant germ cell tumor, it should be noted that these tumor markers may not be elevated in up to 46% of pediatric ovarian malignant neoplasms.2 Intraoperative presence of ascites and the solid and hemorrhagic gross appearance of tumor may also appear worrisome for malignancy. In the present case, oophorectomy was justified based on the absence of radiologic and intraoperative evidence of neoplastic spread beyond the ovary. A teratoma with predominance of a single tissue type (or monodermal teratoma) that is composed of hemangiomatous component has been rarely described and appears to be less common than a pure ovarian hemangioma.3-5 The latter is classified as a mesenchymal neoplasm in the category of nonspecific group of primary ovarian tumors other than the 3 major groups (ie, epithelial-stromal, sex cordstromal, and germ cell tumors). The identification of teratoma foci is considered to be the feature that distinguishes a teratoma with predominant hemangiomatous component from an ovarian hemangioma.3,4 The hemangiomatous component should be distinguished from florid vascular proliferation, which is composed of immature-appearing small and thin-walled vessels and is typically associated with a neural tissue component in immature teratoma without a grossly circumscribed mass-like growth.6
Fig. 2. Microscopic appearance of the right ovarian mass (hematoxylin and eosin). (A) The predominant component is composed of variable-sized blood vessels with thin or thick walls (40). (B) A small cystic space lined with stratified squamous epithelium surrounded by blood vessels (100). (C) A focus of mucin-producing glandular epithelium among the proliferating vessels is partially surrounded with smooth muscle, imparting an intestinal structure (100).
T. Pongsuvareeyakul et al. / J Pediatr Adolesc Gynecol 28 (2015) e1ee3
To our knowledge, less than 10 well-documented cases of ovarian teratoma or germ cell neoplasm with hemangiomatous component have been reported, with only 2 of these occurring in pediatric patients.4,7,8 One patient was a 4-year-old girl presenting with progressive abdominal enlargement for a 2-month duration. Macroscopically, a 10cm right ovarian mass showed a solid sectioned surface with small hemorrhagic cysts and necrotic foci. Histologically, the mass was composed of cavernous hemangioma associated with a microscopic teratomatous component characterized by a squamous cyst.7 The other case was a 16-year-old girl with Turner syndrome who had a 15-cm right gonadal mass composed of cavernous hemangioma in half of the mass, merging with a mixed germ cell tumor which included a minor component of immature teratoma in the other half.8 The left gonad of this patient also contained a 1.2-cm gonadoblastoma.8 The histologic appearance of ovarian hemangiomatous lesions, either with or without teratomatous component, was described as thin-walled vascular channels of variable sizes (capillary or cavernous) in most previous reports.5 In our patient, large veins with thick muscular wall were also prominent in many areas and this resulted in an appearance similar to a venous hemangioma rather than a cavernous hemangioma. The presence of bilateral involvement or extra-ovarian vascular lesions has been reported in approximately 10% of patients with ovarian hemangioma.5 Bilateral involvement of ovarian teratoma with hemangiomatous component was also previously described in a 26-year-old patient.3 In our patient, there was a co-existent orbital vascular lesion that caused the presenting symptom which led to the detection of an ovarian mass on physical examination. Therefore the diagnosis of an ovarian hemangiomatous lesion, either associated with teratoma or not, may indicate a clinical evaluation for the presence of multiple vascular lesions. Our patient had no familial history of
e3
vascular lesions that might suggest certain hereditary syndromes associated with multiple vascular lesions. The clinical course of ovarian hemangioma is benign, as would be expected for that of a hemangiomatous lesion of teratomatous origin.3-5 Surgical excision would be an optimal treatment. Conservative surgery of enucleation of the mass should be considered when technically possible to preserve the ovarian function such as in a patient with a small lesion in the absence of clinically malignant characteristics. In a patient with a clinical suspicion for ovarian malignancy, intraoperative frozen section of the mass may help in making a decision for conservative surgery by a confirmation of the hemangiomatous nature. Acknowledgments
This study was supported by The National Research University Project under Thailand's Office of the Higher Education Commission. References 1. Cass DL, Hawkins E, Brandt ML, et al: Surgery for ovarian masses in infants, children, and adolescents: 102 consecutive patients treated in a 15-year period. J Pediatr Surg 2001; 36:693 2. Oltmann SC, Garcia N, Barber R, et al: Can we preoperatively risk stratify ovarian masses for malignancy? J Pediatr Surg 2010; 45:130 3. Feuerstein IM, Aronson BL, McCarthy EF: Bilateral ovarian cystic teratomata mimicking bilateral pure ovarian hemangiomata: case report. Int J Gynecol Pathol 1984; 3:393 4. Itoh H, Wada T, Michikata K, et al: Ovarian teratoma showing a predominant hemangiomatous element with stromal luteinization: report of a case and review of the literature. Pathol Int 2004; 54:279 lu H, Gu € ngo €r T, Oz M, et al: Primary ovarian hemangioma: case report 5. Bayramog and review of literature. Med J Islam World Acad Sci 2012; 20:106 6. Baker PM, Rosai J, Young RH: Ovarian teratomas with florid benign vascular proliferation: a distinctive finding associated with the neural component of teratomas that may be confused with a vascular neoplasm. Int J Gynecol Pathol 2002; 21:16 7. Gay RM, Janovski NA: Cavernous hemangioma of the ovary. Gynaecologia 1969; 168:248 8. Tanaka Y, Sasaki Y, Tachibana K, et al: Gonadal mixed germ cell tumor combined with a large hemangiomatous lesion in a patient with Turner's syndrome and 45,X/46,X, þmar karyotype. Arch Pathol Lab Med 1994; 118:1135