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ORIGINAL ARTICLE
Year : 2017  |  Volume : 54  |  Issue : 3  |  Page : 576-579
 

Clinicopathological spectrum of teratomas: An 8-year retrospective study from a tertiary care institute


Department of Pathology, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh, India

Date of Web Publication24-May-2018

Correspondence Address:
Dr. Garima Malpani
Department of Pathology, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijc.IJC_294_17

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 » Abstract 


BACKGROUND: Teratomas are tumors that contain tissues derived from three different germ cell layers and having a wider range of differentiation with different site and age at presentation. The aim of the present study was to know the frequency of teratomas in various sites and to analyze their clinicomorphological features. MATERIALS AND METHODS: The present study is a retrospective study conducted in tertiary care hospital of Central India. All the cases diagnosed as teratoma in the period of 8 years were included and studied with reference to age, sex, site, size, gross, and microscopic features. RESULTS: A total of 92 cases were retrieved. The most common teratoma was ovarian (78.26%) followed by intracranial/intraspinal and sacrococcygeal in frequency of 7.61% each. Out of 92 cases, 89 were mature and benign, 2 cases were immature teratoma each in ovary and in sacrococcyx, and 1 case of teratocarcinoma in testis. CONCLUSION: Teratomas have much diversity in their age at presentation, location, gross features, and in degree of differentiation. The prognosis and treatment also depends on the histological grading of teratomas. Thus, pathologists have an important role in establishing a reliable prognostic profile.


Keywords: Immature teratoma, mediastinal teratoma, ovarian teratoma, sacrococcygeal teratoma, teratoma


How to cite this article:
Varma AV, Malpani G, Agrawal P, Malukani K, Dosi S. Clinicopathological spectrum of teratomas: An 8-year retrospective study from a tertiary care institute. Indian J Cancer 2017;54:576-9

How to cite this URL:
Varma AV, Malpani G, Agrawal P, Malukani K, Dosi S. Clinicopathological spectrum of teratomas: An 8-year retrospective study from a tertiary care institute. Indian J Cancer [serial online] 2017 [cited 2019 Aug 19];54:576-9. Available from: http://www.indianjcancer.com/text.asp?2017/54/3/576/233142





 » Introduction Top


Teratoma is a special type of mixed tumor that contains recognizable mature and immature cells or tissues derived from one or more of the three primordial germ cell layers.[1] The word teratoma is derived from the Greek word “teras,” meaning monster or deformed and “oma” means tumor. Virchow coined the term “teratoma” in his book on tumors published in 1863.[2] Teratomas originate from totipotential germ cells such as those normally present in the ovary and testis and sometimes abnormally present in sequestered midline embryonic rests.[1] Thus, they are common in gonads and in midline or paraxial location. Teratomas are common in children but can occur in all ages. Teratomas display wide range of differentiation from most primitive elements on one end to highly organized axial and metamaric structures such as fetus-in-fetu at other end.[3] Many single case reports and series are published in literature, but very few studies mentioned all the teratomas of different sites in one study. Thus, we conducted the study on teratomas with special interest on age, sex, site, and histomorphological features.


 » Materials and Methods Top


This was a retrospective study conducted in Department of Pathology, Sri Aurobindo Medical College and PG Institute, Indore (Madhya Pradesh), from 2008 to 2015. Before conducting the study, approval was taken from the Institutional Ethical Committee. We included all the cases diagnosed as teratoma during this period. Clinical data (age, sex, site, and clinical features) and gross findings (size, appearance, consistency, hemorrhage, and necrosis) were obtained from the histopathology record section of the institute and hematoxylin- and eosin-stained slides were retrieved and reviewed. Where necessary, blocks were recut, stained, and reviewed.


 » Results Top


A total of 92 cases of teratomas were studied. There was a female dominance with a female:male ratio of 6.6:1. The most common site of teratoma was ovary followed by brain and spinal cord. Site-wise distribution of these cases is depicted in [Table 1]. The age range, presenting features, and gross findings of these teratomas are discussed separately.
Table 1: Distribution of teratomas according to site and sex

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Ovarian teratomas

Ovary was the most common site for teratoma in the present study. The age range was very wide ranging from 6 years to 66 years. The mean age was 33.6 years. Most of the cases (48 out of 72) were seen in 21–40 years of age group and rare below the 20 years of age and above 60 years of age. The most common presentation was pain in abdomen. Unilaterality was more common in ovarian teratomas representing 93% (67 cases) with more involvement of right ovary. A total of five cases of bilateral teratomas were observed in the study. The size ranged from 4 cm to 19 cm. All 72 cases predominantly cystic in nature contained hair tufts, teeth, and pultaceous material. Thirteen cases also showed foci of cartilage and bone along with hair tufts and pultaceous material [Figure 1]. Teeth were seen in five cases. One case of immature teratoma (IT) was found in 20 years female. The mass was 19 cm × 11 cm × 9 cm in size, predominantly solid with focal areas of hemorrhage and necrosis. Microscopic examination of most of the mature teratomas revealed squamous epithelium, adnexal structures and adipose tissue [Figure 2]. A total of 13 cases out of 72 cases showed mature cartilage [Figure 2] and bone. Respiratory epithelium was seen in total three cases. Glial tissue was observed in two cases. One case also showed cerebellar tissue. A single case of IT showed immature neural tissue, with neuroepithelial rosettes [Figure 3], immature mesenchymal tissue, and choroid plexus [Figure 4] in addition to the other adnexal structures. It was graded II according to the criteria of Norris et al.
Figure 1: Photograph showing cystic-solid cut section of teratoma with hair tufts and cartilaginous glistening area

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Figure 2: Photomicrograph showing skin, adnexal structures (sebaceous glands, hair follicles) and mature cartilage (H and E, ×100)

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Figure 3: Photomicrograph showing immature neural tissue with neuroepithelial rosettes (H and E, ×100)

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Figure 4: Photomicrograph showing choroid plexus (H and E, ×100)

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Brain and spinal cord teratomas

Four intracranial and three intraspinal teratomas were observed during the study period. All three intraspinal teratomas were seen in adults with female predominance, two were located in dorsal spine and one in lumbar spine [Table 2]. The mean age of diagnosis of intraspinal teratomas in our study was 31 years. The presenting features were tingling, numbness, sharp pain, and weakness in the extremities. None of the patients presented with urinary and bowel complaints. Among intracranial teratomas, three cases were seen in adults and one case in an 8-year-old boy. The clinical features were headache, vomiting, seizure, and gait disturbances according to their sites. All seven cases histologically were mature teratomas.
Table 2: Distribution of cranio-spinal teratomas

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Sacrococcygeal teratomas

We found total seven cases (7.61%) of sacrococcygeal teratomas (SCTs) in our study with male to female ratio 1.3:1. All the cases presented in neonatal life with exophytic mass in sacral region. None of the patients had any other congenital anomaly. The average size was 6 cm with size range 5–10 cm. On microscopic examination, three cases were diagnosed as mature teratoma containing mature tissues derivatives of ectoderm and mesoderm. One case of IT was found in 2-day-old female child having immature mesenchymal tissue and immature neuroepithelium [Figure 5] along with mature tissues.
Figure 5: Photomicrograph showing immature cartilage, immature neuroepithelial tissue with rosettes (H and E, ×100)

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Mediastinal teratomas

In our study, we found three cases (3.26%) of teratomas in mediastinum. All three cases were seen in males in the anterior mediastinum with a wide age range (18–80 years). One patient was incidentally diagnosed in chest xray and two patients presented with persistent cough and sternal pain more on deep breathing. The mean size of tumor was 3.5 cm. All three cases, on histological examination, were diagnosed as mature teratomas.

Testicular teratomas

Three cases of testicular teratoma were found in our study. All three cases were seen in postpubertal male patients with age of presentation being 19, 28, and 38 years. The mean size of testicular teratoma in our study was 4 cm with predominantly solid cut surface on gross examination. On microscopy, two cases were mature teratoma showing squamous epithelium, skin appendages, cartilage, skeletal muscles, and adipose tissue in varying proportion and in disordered arrangement. One case was diagnosed as teratocarcinoma in 28-year-aged male patient showed area of typical embryonal carcinoma with other teratomatous components.


 » Discussion Top


According to Willis, teratomas are composed of multiple tissues foreign to the parts, in which they arise both in benign and malignant forms.[4] Teratomas are classified on the basis of site into intragonadal (ovary and testis) and extragonadal (sacrococcygeal, mediastinum, retroperitoneum, nasopharyngeal, etc.), and histologically into mature, immature, and teratoma with malignant component.

There are three theories postulated for the origin of teratomas. The first theory is “gastrulation theory” which states that teratomas arise from remnants of the primitive streak or primitive node. SCT and pelvic teratomas can be explained by this theory. The second theory is “missed target theory” which states that teratomas originate from totipotent primordial germ cells. These cells develop among the endodermal cells of the yolk sac near the origin of the allantois and migrate to the gonadal ridges during weeks 4 and 5 of gestation. Some cells may miss their target destination and produce a teratoma. This theory explains the origin of gonadal and midline teratomas. The third theory is “incomplete twinning.”[5]

Ovary was the most common site of teratoma in the present study similar to Sushma et al.[6] and Partlow and Taybi.[7] However, in studies conducted in infants and children, ovary was the second most common site of teratoma after the sacrococcygeal region.[3],[8],[9] Recently, a large study from Hyderabad showed the dominance of sacrococcygeal and retroperitoneal teratomas in frequency of 43.3% and 25%, respectively.[10] Female dominance was seen in our study in accordance with other studies.[6],[8],[9]

In this study, ovarian teratomas have wider age distribution with a peak incidence in third and fourth decade constituting two-third of the total teratomas similar to Sushma et al.[6] In accordance with Deodhar et al.[11] and Ayhan et al.,[12] the most common complaint in our study was pain in abdomen followed by lump in abdomen. In accordance with Sushma et al.,[6] the unilaterality with dominance of right ovary was seen in the present study. However, size difference between mature and IT were not observed in this study, but tumor with larger size, predominantly solid and presenting at a younger age, were seen having more chances of IT or teratoma with malignant component.

Frequency of craniospinal axis teratomas (CST) in our study was 7.61%. A larger study by Tapper and Lack [3] reported 3.5% CST in 254 cases of teratomas of different sites. Partlow and Taybi [7] also showed lower (4.76%) prevalence of intracranial tumors. Out of seven cases, 4 (57.14%) were located in cranial cavity and 3 (42.86%) were intraspinal. A study on CSTs by Agrawal et al.[13] showed the dominance of intraspinal teratomas (57.14%) over intracranial teratomas (42.86%). CSTs were more common in females than males in our study in contrast to Agrawal et al.[13] Histologically, all teratomas showed mature tissues in our study similar to Agrawal et al.[13]

The frequency of SCT in our study was 7.61%, which was much lower as compared to the studies conducted in children.[3],[8],[9],[10] Most of the studies [8],[9],[14] showed the female predilection for SCT similar to the present study. All cases were diagnosed in neonatal life with the presence of sacral mass similar to other studies. The mean size of SCT was 6 cm in our study similar to Al-Salem et al.[8] whereas Sinha et al.[15] reported >10 cm size in more than half of the cases. In accordance with Al-Salem et al.[8] and Keslar et al.,[16] we did not find any correlation in the size and behavior of SCTs; however, Sinha et al.[15] found immature histology in larger sized tumors.

The frequency of mediastinal teratomas (3.26%) in our study was comparable to Tapper and Lack (4%).[3] The case series on 57 cases of mediastinal teratomas by Chang et al.[17] found female dominance with a median age of 27 years. All three cases of our study were males with a very wide age range of 18–80 years. All three cases of the present study were in the anterior mediastinum similar to Chang et al.[17] On microscopy of mediastinal teratomas, we found mature elements of all three germ layers; however, we did not find pancreatic tissue, gastrointestinal epithelium, and nerve tissue which was frequently reported in the study by Chang et al.[17]

The frequency of testicular teratomas (3.26%) in the present study was similar to Tapper and Lack (3%);[3] however, Partlow and Taybi [7] and Roshanzamir et al.[9] reported higher frequency (7%) of testicular teratomas. Prepubertal teratomas are usually pure and mature following benign course whereas postpubertal (adult) teratomas are mixed (components of other germ cell tumors) and malignant.[18] However, Porcaro et al.[19] published a case series of five cases of adult pure testicular teratomas, in which three cases (60%) were mature, one (20%) was IT, and one case (20%) was teratoma with malignant transformation.


 » Conclusion Top


Teratomas have much diversity in age at presentation, location, gross features, and presence of tissues as well as in degree of differentiation of these tissues. The prognosis and treatment mainly depends on the anatomical site of the teratoma and age of the patient along with clinical staging and histological grading of immature tissue. Thus, pathologists have a pivotal role in the assessment of histological grading and in establishing reliable prognostic profile.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
 » References Top

1.
Kumar V, Abbas AK, Aster JC. Robbins and Cotrans Pathologic Basis of Disease. 9th ed. Mumbai: Elsevier Inc., Neoplasia; 2014. p. 267.  Back to cited text no. 1
    
2.
Hamilton CA, Harris JE, editors. Cystic Teratoma Workup: Medscape Reference. Available from: http://www.emedicine.medscape.com/article/281850-overview. [Last accessed on 2017 Feb 22; Last updated on 2012 Jan 17].  Back to cited text no. 2
    
3.
Tapper D, Lack EE. Teratomas in infancy and childhood. A 54-year experience at the Children's Hospital Medical Center. Ann Surg 1983;198:398-410.  Back to cited text no. 3
    
4.
Kingsley DP, Elton A, Bennett MH. Malignant teratoma of the thyroid. Case report and a review of the literature. Br J Cancer 1968;22:7-11.  Back to cited text no. 4
    
5.
Keene DJ, Craigie RJ, Shabani A, Batra G, Hennayake S. Bipartite anterior extraperitoneal teratoma: Evidence for the embryological origins of teratomas? Case Rep Med 2011;2011:208940.  Back to cited text no. 5
    
6.
Sushma TA, Shashikala K, Sharmila PS, Sushan SJ, Mahantachar V, Raj JA, et al. Study of histomorphological changes of teratomas in tertiary care hospital. J Evol Med Dent Sci 2013;2:1771-8.  Back to cited text no. 6
    
7.
Partlow WF, Taybi H. Teratomas in infants and children. Am J Roentgenol Radium Ther Nucl Med 1971;112:155-66.  Back to cited text no. 7
    
8.
Al-Salem AH, Hamchou M, Nawaz A, Matta H. Teratoma in infants and children. J Pediatr Surg Specialities 2014;8:14-21.  Back to cited text no. 8
    
9.
Roshanzamir F, Ghoroubi J, Mirhashemi A, Khaleghnejad Tabari A, Rouzrokh M, Izadi M, et al. Teratoma in infants and children. Iran J Pediatr Surg 2015;1:76-9.  Back to cited text no. 9
    
10.
Sathyanarayana KV, Reddy PS. Teratomas in children: An institutional retrospective study. Asian Pac J Health Sci 2016;3:18-23.  Back to cited text no. 10
    
11.
Deodhar KK, Suryawanshi P, Shah M, Rekhi B, Chinoy RF. Immature teratoma of the ovary: A clinicopathological study of 28 cases. Indian J Pathol Microbiol 2011;54:730-5.  Back to cited text no. 11
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Ayhan A, Bukulmez O, Genc C, Karamursel BS, Ayhan A. Mature cystic teratomas of the ovary: Case series from one institution over 34 years. Eur J Obstet Gynecol Reprod Biol 2000;88:153-7.  Back to cited text no. 12
    
13.
Agrawal M, Uppin MS, Patibandla MR, Bhattacharjee S, Panigrahi MK, Saradhi V, et al. Teratomas in central nervous system: A clinico-morphological study with review of literature. Neurol India 2010;58:841-6.  Back to cited text no. 13
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14.
Chirdan LB, Uba AF, Pam SD, Edino ST, Mandong BM, Chirdan OO. Sacrococcygeal teratoma: Clinical characteristics and long-term outcome in Nigerian children. Ann Afr Med 2009;8:105-9.  Back to cited text no. 14
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Sinha S, Kumar Sarin Y, P Deshpande V. Neonatal sacrococcygeal teratoma: Our experience with 10 cases. J Neonatal Surg 2013;2:4.  Back to cited text no. 15
    
16.
Keslar PJ, Buck JL, Suarez ES. Germ cell tumors of the sacrococcygeal region: Radiologic-pathologic correlation. Radiographics 1994;14:607-20.  Back to cited text no. 16
    
17.
Chang CC, Chang YL, Lee JM, Chen JS, Hsu HH, Huang PM, et al. 18 years surgical experience with mediastinal mature teratoma. J Formos Med Assoc 2010;109:287-92.  Back to cited text no. 17
    
18.
Wetherell D, Weerakoon M, Williams D, Beharry BK, Sliwinski A, Ow D, et al. Mature and immature teratoma: A review of pathological characteristics and treatment options. Med Surg Urol 2014;3:124.  Back to cited text no. 18
    
19.
Porcaro AB, Antoniolli SZ, Martignoni G, Brunelli M, Curti P. Adult primary teratoma of the testis – Report on 5 cases in clinical stage I disease. Int Urol Nephrol 2001;33:657-9.  Back to cited text no. 19
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
 
 
    Tables

  [Table 1], [Table 2]



 

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