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Rare growth of a psammomatous meningioma in a mature ovarian Teratoma: A case report
ARTICLE IN PRESS
Pathology – Research and Practice 206 (2010) 322–324 www.elsevier.de/prp
TEACHING CASE
Rare growth of a psammomatous meningioma in a mature ovarian Teratoma: A case report Shramana Mandal, Kajal Dhingra, Parul Gupta, Nita Khurana Maulana Azad Medical College, Department of Pathology, New Delhi, India Received 23 April 2009; received in revised form 14 May 2009; accepted 25 May 2009
Abstract The presence of meningothelial elements along with abundant psammoma bodies indicates the presence of a benign meningioma within a teratoma. Recognition of meningioma arising in the setting of a teratoma is of prognostic significance, depending on the nature of this component and its spread beyond the organ of origin. We report a case of psammomatous meningioma in which meningothelial cells and psammoma bodies were both abundant and diffuse, allowing for the diagnosis of a meningioma. r 2009 Elsevier GmbH. All rights reserved. Keywords: Mature teratoma; Psammomatous meningioma; Ovary
Introduction
Case report
Few foci of meningothelial elements in a mature benign teratoma can be seen, and these are of no clinical or pathological significance. However, outgrowth of the meningothelial elements in the presence of abundant psammoma bodies indicates the presence of a benign meningioma within a teratoma, which is of prognostic significance. It depends on the nature of this component and its spread beyond the organ of origin [1–7]. An extensive literature search has revealed only two such cases of meningothelial elements in a mature benign teratoma of the ovary. We report a case of psammomatous meningioma in which meningothelial cells and psammoma bodies were both abundant and diffuse, which justifies the diagnosis of a meningioma.
A 45-year-old female presented to the gynecological outpatient clinic for pain in her abdomen and distension for one month. On examination, a mass was found in the left lumbar and umbilical region, measuring 12 10 8.4 cm. Ultrasound of the abdomen revealed a left ovarian mass of heterogeneous architecture suggestive of a teratoma. Total abdominal hysterectomy with bilateral saplingo-ophorectomy was carried out. Grossly, the left ovary showed a multiloculated cystic mass measuring 12.4 11 9.5 cm in largest dimension, as well as gray brown solid areas measuring 4 3.2 3 cm. The uterus, cervix, bilateral tubes, and left ovary were unremarkable. Microscopic examination of the cystic areas showed skin with pilosebaceous units, fibroadipose tissue, and respiratory epithelium. Sections from the solid gray brown areas revealed florid proliferation of spindle cells in a whorled pattern with an abundance of psammoma bodies. These cells were monomorphic and showed a uniform, round to oval vesicular nucleus and indistinct cell outlines. There
Corresponding author.
E-mail address: [email protected] (S. Mandal). 0344-0338/$ - see front matter r 2009 Elsevier GmbH. All rights reserved. doi:10.1016/j.prp.2009.05.011
ARTICLE IN PRESS S. Mandal et al. / Pathology – Research and Practice 206 (2010) 322–324
323
Fig. 1. Sections from the solid gray brown areas revealed florid proliferation of spindle cells in whorled pattern with abundance of psammoma bodies.
was no evidence of pleomorphism, mitosis, and necrosis, suggesting a low grade psammomatous meningioma (Fig. 1). These cells were strongly immunoreactive for Epithelial Membrane Antigen (EMA) and were negative for S100 and Glial Fibrillary Acid Protein (GFAP). Thus a diagnosis of meningioma within a mature ovarian teratoma was made on the basis of morphology and immunohistochemistry.
Discussion Pure teratomas constitute 95% of all the ovarian germ cell tumors [6]. The ovarian teratomas are predominantly cystic (dermoid cyst). On microscopic examination, the mature ovarian teratomas show a wellorganized arrangement of tissues that often duplicates the relationships seen in normal organs, for instance pilosebaceous structures and sweat glands within squamous epithelial-lined ‘dermis’ or respiratory epithelium encircled by smooth muscle and cartilage. Furthermore, the elements within these ‘organoid’ tissues lack cytological atypia and have scant mitotic activity, which is mostly confined to the normal proliferative zones of the organs they replicate. Choroid plexus and thyroid tissue are common in ovarian teratomas, and pituitary tissue can also be seen occasionally, rarely giving rise to functioning prolactinomas. These tissue types are infrequent (choroid plexus) to rare (thyroid) to virtually non-existent (pituitary) in testicular teratomas [6]. Mature teratomas belong to the group of germ cell tumors of the ovary. They account for 27–44% of all
ovarian neoplasms and for up to 58% of all benign tumors of the ovary. In mature and immature teratomas, secondary tumors originating from the three embryonic tissue components may be found. Malignant transformation of mature cystic teratoma is a rare complication, accounting for 1–2% of all cases. These tumors can show a benign or malignant behavior [4]. Malignant degeneration of mature cystic teratomas consists of differentiated tissues giving rise to carcinoma or sarcoma. While any of the constituent tissues of a teratoma has the potential of undergoing malignant transformation, squamous cell carcinoma arising from the squamous lining of the cyst is the most common type of malignant degeneration and accounts for over 80% of cases. Rarely, other types such as malignant melanoma, adenocarcinoma, follicular, papillary carcinoma, carcinoid tumor, and various forms of sarcoma can be seen, accounting for 0.2–1.4% of mature ovarian teratomas [3,6]. An extensive literature search revealed only three such cases in testicular teratomas, but only two such cases with meningothelial elements have been reported in the ovary to date [1–5]. We present the third unusual case of a postmenopausal woman with psammomatous meningioma, in which meningothelial cells and psammoma bodies were both abundant and diffuse, so that a diagnosis of meningioma in a mature cystic teratoma was justified. Meninges are rarely found in testicular teratomas, with one reported case of a microcystic meningioma arising in a mixed germ cell tumor of the testis composed predominantly of mature and immature teratoma with elements of seminoma and embryonal carcinoma [2]. Shelekhova et al. [3] reported a case of mature adult teratoma, focusing on a meningothelial cell
ARTICLE IN PRESS 324
S. Mandal et al. / Pathology – Research and Practice 206 (2010) 322–324
proliferation containing psammoma bodies. To label such a proliferation as meningioma, it is imperative to find such a predominating composition. The meningothelial cells were immunohistochemically positive for EMA and claudin and negative for S-100-protein, cytokeratins (AE1–AE3, CAM5.2), desmin, and chromogranin A [3]. Michal [1] reported a case of mature adult teratoma with large areas of a meningiomatous proliferation in close proximity to a peripheral nerve, and glial tissue was seen. These meningiomatous proliferations were mostly seen in the peripheral parts of the teratoma surrounding the rest of teratomatous elements, and they were immunohistochemically EMApositive and S-100 protein- and cytokeratin-negative. Identical meningothelial proliferations are well-known in the skin and in adjacent soft tissues of the scalp, where they have variously been called sequestrated meningoceles, meningeal hamartoma, cutaneous meningiomas, rudimentary meningocele, hamartoma of the scalp with ectopic meningothelial elements, or cutaneous heterotopic meningeal nodules. Adams et al. [4] reported a case of meningothelial meningioma within a mature teratoma in a 32-year-old female. It showed immunohistochemical expression for epithelial membrane antigen and desmoplakin, which are diagnostic. Takeshima et al. [5] reported a case of microcystic meningioma arising in a mature cystic teratoma of the ovary in a 60-year-old woman. The tumor was located in the right ovary, and salpingo-oophorectomy was performed. Histologically, the cyst wall was composed of typical mature cystic teratoma. The mural nodule was composed of proliferating spindle- and polygonal-shaped cells positive for epithelial membrane antigen, and microcystic change was prominent. The arachnoidal cells around the mature brain tissue were thought to be the origin of this unusual tumor. Malignant transformation occurs in the 6th or 7th decade of life. It has an imaging appearance that indicates the presence of underlying mature cystic teratoma: a sebaceous lipid component and a heterogeneous solid component protruding into the cavity or extending transmurally into adjacent organs. Most of the mature cystic teratomas are asymptomatic. Abdominal pain or other nonspecific symptoms occur in the minority of patients. Mature cystic teratomas grow slowly at an average rate of 1.8 mm each year, prompting some investigators to advocate nonsurgical management of smaller (o6-cm) tumors. These tumors are bilateral in about 10% of the cases [7]. Ovarian teratomas with malignant elements and malignant transformation occur after the development
of the teratoma (‘post-teratomatous’ malignant transformation). This applies to those dermoid cysts that develop malignant somatic neoplasms. In these cases, the homozygous nature of the malignant elements, which is similar to the benign elements, supports derivation of the former from the latter, as do similar cytogenetic changes in the two components. In addition, immaturity in ovarian teratomas can be similarly regarded as representing clones of malignant neuroepithelium that develop within a pre-existing teratoma. Ovarian ependymoma shows perivascular pseudorosettes. Immaturity usually manifests as immature neuroepithelium but sometimes, rarely, only as cellular, mitotically active glia. Therefore, it is important to assess immaturity in ovarian teratomas. Semi quantification of the amount of neuroepithelium correlates with the survival in ovarian immature teratoma, at least in adult patients, and is the basis for the grading of these tumors [6]. To conclude, recognition of meningioma arising in a setting of a teratoma is of prognostic significance, depending on the nature of this component and its spread beyond the organ of origin. The characteristic histomorphology and immunohistochemical expression for epithelial membrane antigen will help the pathologist to render the diagnosis.
References [1] M. Michal, Meningeal nodules in teratoma of the testis, Virchows Arch. 438 (2001) 198–200. [2] A. Allen Elizabeth, C. Burger Peter, I. Epstein Jonathan, Microcystic meningioma arising in a mixed germ cell tumor of the testis: a case report, Am. J. Surg. Pathol. 23 (1999) 1131. [3] K.V. Shelekhova, D.V. Kazakov, M. Michal, Testicular teratoma with meningothelial elements, Arkh Patol. 67 (2005) 37–38. [4] H. Adams, P. Went, M. Tolnay, G. Sartorius, G. Singer, Meningothelial meningioma in a mature cystic teratoma of the ovary, Pathologe 28 (2007) 278–280. [5] Y. Takeshima, M. Kaneko, O. Furonaka, A.V. Jeet, K. Inai, Meningioma in mature cystic teratoma of the ovary, Pathol. Int. 54 (2004) 543–548. [6] T.M. Ulbright, Germ cell tumors of the gonads: a selective review emphasizing problems in differential diagnosis, newly appreciated and controversial issues, Mod. Pathol. 18 (2005) S61–S79. [7] E.K. Outwater, E.S. Siegelman, J.L. Hunt, Ovarian teratomas: tumor types and imaging characteristics, Radiographics 21 (2001) 475–490.
Pathology – Research and Practice 206 (2010) 322–324 www.elsevier.de/prp
TEACHING CASE
Rare growth of a psammomatous meningioma in a mature ovarian Teratoma: A case report Shramana Mandal, Kajal Dhingra, Parul Gupta, Nita Khurana Maulana Azad Medical College, Department of Pathology, New Delhi, India Received 23 April 2009; received in revised form 14 May 2009; accepted 25 May 2009
Abstract The presence of meningothelial elements along with abundant psammoma bodies indicates the presence of a benign meningioma within a teratoma. Recognition of meningioma arising in the setting of a teratoma is of prognostic significance, depending on the nature of this component and its spread beyond the organ of origin. We report a case of psammomatous meningioma in which meningothelial cells and psammoma bodies were both abundant and diffuse, allowing for the diagnosis of a meningioma. r 2009 Elsevier GmbH. All rights reserved. Keywords: Mature teratoma; Psammomatous meningioma; Ovary
Introduction
Case report
Few foci of meningothelial elements in a mature benign teratoma can be seen, and these are of no clinical or pathological significance. However, outgrowth of the meningothelial elements in the presence of abundant psammoma bodies indicates the presence of a benign meningioma within a teratoma, which is of prognostic significance. It depends on the nature of this component and its spread beyond the organ of origin [1–7]. An extensive literature search has revealed only two such cases of meningothelial elements in a mature benign teratoma of the ovary. We report a case of psammomatous meningioma in which meningothelial cells and psammoma bodies were both abundant and diffuse, which justifies the diagnosis of a meningioma.
A 45-year-old female presented to the gynecological outpatient clinic for pain in her abdomen and distension for one month. On examination, a mass was found in the left lumbar and umbilical region, measuring 12 10 8.4 cm. Ultrasound of the abdomen revealed a left ovarian mass of heterogeneous architecture suggestive of a teratoma. Total abdominal hysterectomy with bilateral saplingo-ophorectomy was carried out. Grossly, the left ovary showed a multiloculated cystic mass measuring 12.4 11 9.5 cm in largest dimension, as well as gray brown solid areas measuring 4 3.2 3 cm. The uterus, cervix, bilateral tubes, and left ovary were unremarkable. Microscopic examination of the cystic areas showed skin with pilosebaceous units, fibroadipose tissue, and respiratory epithelium. Sections from the solid gray brown areas revealed florid proliferation of spindle cells in a whorled pattern with an abundance of psammoma bodies. These cells were monomorphic and showed a uniform, round to oval vesicular nucleus and indistinct cell outlines. There
Corresponding author.
E-mail address: [email protected] (S. Mandal). 0344-0338/$ - see front matter r 2009 Elsevier GmbH. All rights reserved. doi:10.1016/j.prp.2009.05.011
ARTICLE IN PRESS S. Mandal et al. / Pathology – Research and Practice 206 (2010) 322–324
323
Fig. 1. Sections from the solid gray brown areas revealed florid proliferation of spindle cells in whorled pattern with abundance of psammoma bodies.
was no evidence of pleomorphism, mitosis, and necrosis, suggesting a low grade psammomatous meningioma (Fig. 1). These cells were strongly immunoreactive for Epithelial Membrane Antigen (EMA) and were negative for S100 and Glial Fibrillary Acid Protein (GFAP). Thus a diagnosis of meningioma within a mature ovarian teratoma was made on the basis of morphology and immunohistochemistry.
Discussion Pure teratomas constitute 95% of all the ovarian germ cell tumors [6]. The ovarian teratomas are predominantly cystic (dermoid cyst). On microscopic examination, the mature ovarian teratomas show a wellorganized arrangement of tissues that often duplicates the relationships seen in normal organs, for instance pilosebaceous structures and sweat glands within squamous epithelial-lined ‘dermis’ or respiratory epithelium encircled by smooth muscle and cartilage. Furthermore, the elements within these ‘organoid’ tissues lack cytological atypia and have scant mitotic activity, which is mostly confined to the normal proliferative zones of the organs they replicate. Choroid plexus and thyroid tissue are common in ovarian teratomas, and pituitary tissue can also be seen occasionally, rarely giving rise to functioning prolactinomas. These tissue types are infrequent (choroid plexus) to rare (thyroid) to virtually non-existent (pituitary) in testicular teratomas [6]. Mature teratomas belong to the group of germ cell tumors of the ovary. They account for 27–44% of all
ovarian neoplasms and for up to 58% of all benign tumors of the ovary. In mature and immature teratomas, secondary tumors originating from the three embryonic tissue components may be found. Malignant transformation of mature cystic teratoma is a rare complication, accounting for 1–2% of all cases. These tumors can show a benign or malignant behavior [4]. Malignant degeneration of mature cystic teratomas consists of differentiated tissues giving rise to carcinoma or sarcoma. While any of the constituent tissues of a teratoma has the potential of undergoing malignant transformation, squamous cell carcinoma arising from the squamous lining of the cyst is the most common type of malignant degeneration and accounts for over 80% of cases. Rarely, other types such as malignant melanoma, adenocarcinoma, follicular, papillary carcinoma, carcinoid tumor, and various forms of sarcoma can be seen, accounting for 0.2–1.4% of mature ovarian teratomas [3,6]. An extensive literature search revealed only three such cases in testicular teratomas, but only two such cases with meningothelial elements have been reported in the ovary to date [1–5]. We present the third unusual case of a postmenopausal woman with psammomatous meningioma, in which meningothelial cells and psammoma bodies were both abundant and diffuse, so that a diagnosis of meningioma in a mature cystic teratoma was justified. Meninges are rarely found in testicular teratomas, with one reported case of a microcystic meningioma arising in a mixed germ cell tumor of the testis composed predominantly of mature and immature teratoma with elements of seminoma and embryonal carcinoma [2]. Shelekhova et al. [3] reported a case of mature adult teratoma, focusing on a meningothelial cell
ARTICLE IN PRESS 324
S. Mandal et al. / Pathology – Research and Practice 206 (2010) 322–324
proliferation containing psammoma bodies. To label such a proliferation as meningioma, it is imperative to find such a predominating composition. The meningothelial cells were immunohistochemically positive for EMA and claudin and negative for S-100-protein, cytokeratins (AE1–AE3, CAM5.2), desmin, and chromogranin A [3]. Michal [1] reported a case of mature adult teratoma with large areas of a meningiomatous proliferation in close proximity to a peripheral nerve, and glial tissue was seen. These meningiomatous proliferations were mostly seen in the peripheral parts of the teratoma surrounding the rest of teratomatous elements, and they were immunohistochemically EMApositive and S-100 protein- and cytokeratin-negative. Identical meningothelial proliferations are well-known in the skin and in adjacent soft tissues of the scalp, where they have variously been called sequestrated meningoceles, meningeal hamartoma, cutaneous meningiomas, rudimentary meningocele, hamartoma of the scalp with ectopic meningothelial elements, or cutaneous heterotopic meningeal nodules. Adams et al. [4] reported a case of meningothelial meningioma within a mature teratoma in a 32-year-old female. It showed immunohistochemical expression for epithelial membrane antigen and desmoplakin, which are diagnostic. Takeshima et al. [5] reported a case of microcystic meningioma arising in a mature cystic teratoma of the ovary in a 60-year-old woman. The tumor was located in the right ovary, and salpingo-oophorectomy was performed. Histologically, the cyst wall was composed of typical mature cystic teratoma. The mural nodule was composed of proliferating spindle- and polygonal-shaped cells positive for epithelial membrane antigen, and microcystic change was prominent. The arachnoidal cells around the mature brain tissue were thought to be the origin of this unusual tumor. Malignant transformation occurs in the 6th or 7th decade of life. It has an imaging appearance that indicates the presence of underlying mature cystic teratoma: a sebaceous lipid component and a heterogeneous solid component protruding into the cavity or extending transmurally into adjacent organs. Most of the mature cystic teratomas are asymptomatic. Abdominal pain or other nonspecific symptoms occur in the minority of patients. Mature cystic teratomas grow slowly at an average rate of 1.8 mm each year, prompting some investigators to advocate nonsurgical management of smaller (o6-cm) tumors. These tumors are bilateral in about 10% of the cases [7]. Ovarian teratomas with malignant elements and malignant transformation occur after the development
of the teratoma (‘post-teratomatous’ malignant transformation). This applies to those dermoid cysts that develop malignant somatic neoplasms. In these cases, the homozygous nature of the malignant elements, which is similar to the benign elements, supports derivation of the former from the latter, as do similar cytogenetic changes in the two components. In addition, immaturity in ovarian teratomas can be similarly regarded as representing clones of malignant neuroepithelium that develop within a pre-existing teratoma. Ovarian ependymoma shows perivascular pseudorosettes. Immaturity usually manifests as immature neuroepithelium but sometimes, rarely, only as cellular, mitotically active glia. Therefore, it is important to assess immaturity in ovarian teratomas. Semi quantification of the amount of neuroepithelium correlates with the survival in ovarian immature teratoma, at least in adult patients, and is the basis for the grading of these tumors [6]. To conclude, recognition of meningioma arising in a setting of a teratoma is of prognostic significance, depending on the nature of this component and its spread beyond the organ of origin. The characteristic histomorphology and immunohistochemical expression for epithelial membrane antigen will help the pathologist to render the diagnosis.
References [1] M. Michal, Meningeal nodules in teratoma of the testis, Virchows Arch. 438 (2001) 198–200. [2] A. Allen Elizabeth, C. Burger Peter, I. Epstein Jonathan, Microcystic meningioma arising in a mixed germ cell tumor of the testis: a case report, Am. J. Surg. Pathol. 23 (1999) 1131. [3] K.V. Shelekhova, D.V. Kazakov, M. Michal, Testicular teratoma with meningothelial elements, Arkh Patol. 67 (2005) 37–38. [4] H. Adams, P. Went, M. Tolnay, G. Sartorius, G. Singer, Meningothelial meningioma in a mature cystic teratoma of the ovary, Pathologe 28 (2007) 278–280. [5] Y. Takeshima, M. Kaneko, O. Furonaka, A.V. Jeet, K. Inai, Meningioma in mature cystic teratoma of the ovary, Pathol. Int. 54 (2004) 543–548. [6] T.M. Ulbright, Germ cell tumors of the gonads: a selective review emphasizing problems in differential diagnosis, newly appreciated and controversial issues, Mod. Pathol. 18 (2005) S61–S79. [7] E.K. Outwater, E.S. Siegelman, J.L. Hunt, Ovarian teratomas: tumor types and imaging characteristics, Radiographics 21 (2001) 475–490.