Seminoma of the mediastinum

Definition

A germ cell tumour identical to the corresponding gonadal tumour.

Epidemiology

Primary mediastinal seminomas accounts for 8% of extragonadal germ cell tumours and 9% to 39% of mediastinal germ cell tumours and 3% to 4% of all mediastinal neoplasms. Mediastinal seminoma is almost exclusively found in males, with most cases occurring the in the third or fourth decade of life.

Clinical features

Presentation is usually secondary to the mass effect. Unlike non-seminomatous tumours, seminomas of the mediastinum are not associated with Klinefelter's syndrome.

Macroscopic appearances

Most tumours are circumscribed, without infiltration of adjacent structures. Occasional cases show cystic change, which possibly arises in the residual or hyperplastic thymic tissue2,3.

Histopathology

The morphology is similar to that of gonadal seminomas. They are composed uniform sheet of round or polygonal cells. The cytoplasm contains copious glycogen and often appear clear2. The nuclei show one or more large nucleoli. The cells are usually separated by fibrous septa2. There is often a background of lymphocytes, plasma cells, sometimes eosinophils. There is often granulomatous inflammation2. Occasionally, the inflammation may mask the seminoma. Syncytiotrophoblastic cells are occasionally present, but, in contrast to choriocarcinoma, there is no cytotrophoblastic component.

Seminoma may occur as a component of a mixed germ cell tumour. Spermatocytic seminoma has not been reported in the mediastinum.

Immunohistochemistry

 

OCT4

23/232, 11/118

 
 

PLAP

80-90%0, 10/232, 10/114, 40/505, 37/406

 

Vimentin

70%0

CD117

often positive0, 13/232, 3/87

pancytokeratin

70%0

AE1/AE3

10/232, 0/114, 35/505, 27/406

34bE12

9/232

Cam5.2

11/232, 0/114, 75%5, 32/406

CK7

3/232

CK20

0/232

EMA

2/232, 0/114, 0/505

bHCG

positive in any syncytiotrophoblastic cells0

CD30

0/232, 1/406

CEA

negative0

EMA

negative0

AFP

negative0

   
   

Mediastinal seminomas differ from testicular seminoma in more often expressing cytokeratins, PLAP and vimentin.

Cytogenetics

Abnormalities, particularly amplifications, of chromosome 12p are common2.

Molecular studies

Primary mediastinal seminoma often shows c-kit mutation, whereas testicular seminoma shows wild type c-kit.

 

Differential diagnosis

Management and Prognosis

Mediastinal seminoma is highly responsive to radiotherapy and cisplatin-based chemotherapy. Metastases occur to cervical and abdominal lymph nodes, lung, pleura, chest wall, liver, adrenals, bone and brain. The prognosis is better than for non-seminomatous mediastinal germ cell tumours. The five year survival is 90% with combination chemotherapy.

References

0 Tumours of the Lung, Pleura, Thymus and Heart. WHO Classification of Tumours. IARC Press 2004.

1 Sung MT, Maclennan GT, Lopez-Beltran A, et al. Primary mediastinal seminoma: a comprehensive assessment integrated with histology, immunohistochemistry, and fluorescence in situ hybridization for chromosome 12p abnormalities in 23 cases. Am J Surg Pathol 2008; 32:146-55

2 Sung MT, Maclennan GT, Lopez-Beltran A, et al. Primary mediastinal seminoma: a comprehensive assessment integrated with histology, immunohistochemistry, and fluorescence in situ hybridization for chromosome 12p abnormalities in 23 cases. Am J Surg Pathol 2008; 32:146-55

3 Moran CA,Suster S. Mediastinal seminomas with prominent cystic changes. A clinicopathologic study of 10 cases. Am J Surg Pathol 1995; 19:1047-53

4 Niehans GA, Manivel JC, Copland GT, et al. Immunohistochemistry of germ cell and trophoblastic neoplasms. Cancer 1988; 62:1113-23

5 Moran CA, Suster S, Przygodzki RM, et al. Primary germ cell tumors of the mediastinum: II. Mediastinal seminomas--a clinicopathologic and immunohistochemical study of 120 cases. Cancer 1997; 80:691-8 FULL TEXT

6 Suster S, Moran CA, Dominguez-Malagon H, et al. Germ cell tumors of the mediastinum and testis: a comparative immunohistochemical study of 120 cases. Hum Pathol 1998; 29:737-42

7 Przygodzki RM, Hubbs AE, Zhao FQ, et al. Primary mediastinal seminomas: evidence of single and multiple KIT mutations. Lab Invest 2002; 82:1369-75

8 Jung SM, Chu PH, Shiu TF, et al. Expression of OCT4 in the primary germ cell tumors and thymoma in the mediastinum. Appl Immunohistochem Mol Morphol 2006; 14:273-5

 

This page last revised 18.5.2008.

©SMUHT/PW Bishop