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Year : 2011  |  Volume : 54  |  Issue : 1  |  Page : 199-200
Colonic-type adenocarcinoma (somatic-type malignancy) arising in a mediastinal mature cystic teratoma: A case report of a rare entity


Department of Pathology, Rajiv Gandhi Cancer Institute And Research Centre, Sector-5, Rohini, Delhi - 110 085, India

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Date of Web Publication7-Mar-2011
 

How to cite this article:
Khurana A, Mehta A, Kamboj M. Colonic-type adenocarcinoma (somatic-type malignancy) arising in a mediastinal mature cystic teratoma: A case report of a rare entity. Indian J Pathol Microbiol 2011;54:199-200

How to cite this URL:
Khurana A, Mehta A, Kamboj M. Colonic-type adenocarcinoma (somatic-type malignancy) arising in a mediastinal mature cystic teratoma: A case report of a rare entity. Indian J Pathol Microbiol [serial online] 2011 [cited 2019 Nov 21];54:199-200. Available from: http://www.ijpmonline.org/text.asp?2011/54/1/199/77405


We discuss a case of a 21-year-old asymptomatic male who was incidentally detected to have a huge anterior mediastinal mass on routine medical examination. Computed tomography (CT) of thorax showed a well-defined lobulated and cystic, heterogeneously enhancing mass in the anterior mediastinum measuring 12.0 × 9.8 × 9.3 cm with areas of fat attenuation and calcification, suggesting a possibility of a teratoma. Ultrasonographic examination of abdomen and testes was normal and the serum markers alpha feto-protein (AFP) and beta human chorionic gonadotropin (β-HCG) were within normal range. A CT-guided biopsy was performed which revealed teratomatous components comprising stratified epithelium, seromucinous glands and fibroadipose tissue. The patient underwent resection of the mass which showed a well-encapsulated tumor measuring 13 × 8 × 8 cm. On cut section, the mass showed a unilocular cyst filled with yellow pultaceous material and hair tufts [Figure 1]a. Extensive sampling was done from the entire tumor. Microscopy revealed various elements derived from the three germinal layers in the form of skin with appendageal structures, intestinal and respiratory epithelium, cartilage, and mature adipose tissue [Figure 1]b. Mature thymic tissue was noted in sections submitted from the periphery of the cyst wall. Also noted was a focus composed of fused glandular structures lined by atypical columnar epithelium with interspersed goblet cells exhibiting nuclear pleomorphism and mitoses [Figure 2]. Both intracellular and extracellular mucin was seen [Figure 3]. This focus of suspected malignancy spanned approximately five low-power fields and exhibited features of invasion into the surrounding teratomatous tissue. On immunohistochemistry (IHC), these cells showed nuclear positivity with p53 [Figure 4]a, indicating their neoplastic nature. CDX2 [Figure 4]b and CEA immunostaining confirmed their colonic phenotype. A final diagnosis of mature cystic teratoma of thymic origin with somatic-type malignancy (STM) (colonic-type adenocarcinoma) was rendered. The patient is on close follow-up and has been symptom free for the past 6 months post surgery.
Figure 1: (a) Gross photograph shows predominantly cystic tumor containing yellow cheesy material along with tuft of hair. (b) Photomicrograph showing mature teratomatous component (H and E, ×100)

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Figure 2: Photomicrograph showing fused irregular glands infiltrating the stroma (H and E, ×100). Inset shows a gland lined by pleomorphic cells exhibiting mitosis (H and E, ×200)

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Figure 3: Dilated and disrupted glands showing intracellular and extracellular mucin (H and E, ×100)

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Figure 4: (a) p53 immunostaining showing nuclear positivity in the glandular structures (IHC, ×200); (b) nuclear staining by CdX-2 immunostain (IHC, ×200)

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Mature cystic teratoma of the mediastinum associated with a STM is a rare entity with only a few cases reported in the literature. [1],[2] Mediastinal germ cell tumors are rare neoplasms, representing less than 1% of all malignancies and 3-4% of all germ cell tumors in both adults and children. [1] Recent World Health Organization (WHO) classification has introduced the term germ cell tumor (GCT) with STM, defined as a germ cell tumor accompanied with a somatic type malignant counterpart of sarcoma, carcinoma or both, more frequently associated with a mixed GCT (>75%) as compared to a teratoma (10-20%). Teratomas with STM occur more commonly in males, with most cases falling in the age group of 20-40 years. [1] Also, 30-59% of mediastinal mature teratomas are incidental. Classical radiologic appearance of a cystic teratoma is a lobulated, usually thin-walled, inhomogeneous cystic mass that contains varying combinations of heterogeneous tissue types such as soft tissue, calcium, fluid and fat. The presence of a locally thickened and solid wall with invasion of adjacent structures would suggest malignant transformation. [3] A minimum size of one low-power field has been suggested as the threshold for the diagnosis of STM in GCTs. [1] The accompanying non germ cell components are frequently sarcomas (63%) and less commonly carcinomas (37%). [1]

Surgical resection plays an essential role in therapy when the tumor is limited to a single site. [3] There is no response to chemotherapy used for treatment of GCTs; however, adapted chemotherapy based on histology (such as fluorouracil-based regimen for adenocarcinoma transformation) has recently been advocated, and it may help improve patient outcome. [4],[5] In a study of series of patients with mediastinal teratoma with malignant transformation, three patients were noted to have only 4-6 months of treatment response, despite comprehensive treatment of surgery, chemotherapy, or radiation. [5] A diagnosis of GCTs with STM confers a dismal prognosis with a high frequency of metastases and recurrence. The prognosis of mediastinal GCTs with STM is known to be extremely poor. Histology of the somatic malignancy does not have a major impact on prognosis. [1] Metastasis is a feature in majority of cases, with median survival for these patients being approximately 9 months. [1] In a case of mediastinal mature cystic teratoma in a 57-year-old male, the patient presented with malignant effusion 7 months post-surgery; review of the tumor and extensive sampling of the resected specimen showed a small focus of adenocarcinoma within the tumor. [2] To conclude, a thorough sampling of the entire tumor mass is warranted to hunt for an occult malignancy, as was performed in the indexed patient.

 
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1.Wick MR, Perlman EJ, Orazi A, Muller-Hermelink HK, Zettl A, Gobel U, et al. Germ cell tumours of the mediastinum. In: Travis WD, Brambilla E, Muller-Hermelink HK, Harris CC, editors. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Lung, Pleura, Thymus and Heart. Lyon: IARC Press; 2004. p. 198-220.  Back to cited text no. 1
    
2.Sakurai H, Miyashita Y, Oyama T. Adenocarcinoma arising in anterior mediastinal teratoma: Report of a case. Surg Today 2008;38:348-51.  Back to cited text no. 2
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3.Chen C, Zheng H, Jiang S. An unusual case of giant mediastinal teratoma with malignant transformation. Ann Thorac Surg 2008;86:302-4.  Back to cited text no. 3
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4.El Mesbahi O, Terrier-Lacombe MJ, Rebischung C, Theodore C, Vanel D, Fizazi K. Chemotherapy in patients with teratoma with malignant transformation. Eur Urol 2007;51:1306-12.  Back to cited text no. 4
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5.Donadio AC, Motzer RJ, Bajorin DF, Kantoff PW, Sheinfeld J, Houldsworth J, et al. Chemotherapy for teratoma with malignant transformation. J Clin Oncol 2003;21:4285-91.  Back to cited text no. 5
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Correspondence Address:
Anuj Khurana
98 Sfs flats Phase-4, Ashok vihar, Delhi - 110 052
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.77405

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