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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 2  |  Issue : 1  |  Page : 54-56

A rare case of primary mediastinal yolk sac tumor


1 Department of Pulmonary Medicine, KLE University's J. N. Medical College, Belgaum, Karnataka, India
2 Department of Pathology, KLE University's J. N. Medical College, Belgaum, Karnataka, India
3 Department of Radiology, KLE University's J. N. Medical College, Belgaum, Karnataka, India

Date of Web Publication4-Jun-2014

Correspondence Address:
Vinay Mahishale
Department of Pulmonary Medicine, J. N. Medical College, Belgaum, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2321-4848.133812

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  Abstract 

Yolk sac tumor (endodermal sinus tumor) is a subtype of germ cell tumor and a highly malignant neoplasm. In addition to presenting in ovaries and testes, the tumor has been detected at several extragonadal sites, including the presacral area, the anterior mediastinum, face, uvula, and the pineal gland. Primary yolk sac tumor of the anterior mediastinum is rare and carries a grave prognosis, affecting mainly young men. Patients often present with advanced, bulky tumors that are unresectable. We present a 16-years-old young male patient with primary yolk sac tumor of mediastinum.

Keywords: Alpha-fetoprotein, endodermal sinus tumor, mediastinum, primary yolk sac tumor


How to cite this article:
Mahishale V, Malur PR, Hattiholi V, Avuthu S, Rathi A. A rare case of primary mediastinal yolk sac tumor. Arch Med Health Sci 2014;2:54-6

How to cite this URL:
Mahishale V, Malur PR, Hattiholi V, Avuthu S, Rathi A. A rare case of primary mediastinal yolk sac tumor. Arch Med Health Sci [serial online] 2014 [cited 2019 Dec 7];2:54-6. Available from: http://www.amhsjournal.org/text.asp?2014/2/1/54/133812


  Introduction Top


Yolk sac tumor (endodermal sinus tumor) is a subtype of germ cell tumor and a highly malignant neoplasm. [1] In addition to presenting in ovaries and testes, the tumor has been detected at several extragonadal sites, including the presacral area, the anterior mediastinum, face, uvula, and the pineal gland. Primary yolk sac tumor of the anterior mediastinum is rare and carries a grave prognosis, affecting mainly young men. Patients often present with advanced, bulky tumors that are unresectable. [2],[3],[4] Yolk sac tumor or endodermal sinus tumor is also known by a variety of other names such as juvenile embryonal carcinoma, orchidoblastoma or embryonal adenocarcinoma. Yolk sac tumor is defined as tumor characterized by numerous patterns that recapitulate the embryonal yolk sac, allantois, and extra embryonic mesenchyme. [5]


  Case Report Top


A 16-years-old young male patient was referred to outpatient department with history of high-grade fever, breathlessness, and severe chest pain. On examination, patient was febrile with blood pressure of 110/70 mmHg, pulse rate 76 beats/min, and respiratory rate of 22 breaths/min. No evidence of clubbing or lymphadenopathy was seen. Tenderness was noticed on anterior chest wall. Examination of abdomen and pelvis was normal. There was no abnormality noticed in the scrotal examination. Patient was admitted in hospital in view of high-grade fever. Chest radiograph showed mediastinal widening with a bulky lobulated mass lesion [Figure 1]. Patient was subjected to CT scan thorax plain and contrast. Plain chest CT scan showed a bulky anterior mediastinal mass, and contrast-enhanced chest CT scan showed central non-enhancing necrotic areas with peripherally enhancing solid component [Figure 2]. Patient subsequently underwent CT-guided biopsy of mediastinal mass using Cook coaxial biopsy gun (18G). Histopathology report suggested possibility of germ cell tumor of yolk sac origin [Figure 3]. Further immunohistochemistry and elevated serum alpha-fetoprotein levels confirmed the diagnosis of primary yolk sac tumor of the mediastinum. In immunohistochemistry, tumor cells expressed cytokeratin, gypican, and chromographin A (focal) and were immunonegative for EMA and Synaptophysin. Serum alpha-fetoprotein level was 250 ng/dl. CT scan of the abdomen and pelvis both plain and contrast were normal.
Figure 1: Chest radiograph showing bulky mediastenal mass

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Figure 2: CT-thorax -evidence of bulky anterior mediastinal mass with contrast enhancement

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Figure 3: HPR- highly malignant primitive gondal cells arranged in sheets

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  Discussion Top


Primary germinal tumors of the mediastinum probably result from the aberrant migration of germ cells during embryogenesis. The histology of these tumors is similar to the primary tumors of the gonads. The germ cell origin of YST was first recognized by Teilum. Because of the presence of structures that resemble immature glomeruli, they have been called "mesonephromas"(Shiller). These were later found similar to the intraplacental structures in the rat known as endodermal sinuses of Duval. These structures are called Shiller-Duval bodies. [6]

The origin of these tumors from the endoderm of yolk sac is further strengthened by the finding of alpha-fetoprotein in the serum of these patients. [7] The elevated serum alpha-fetoprotein level is useful in diagnosis and follow-up of these patients.

All the anterior mediastinal yolk sac tumor cases so far reported have been males between 13-49 years. Radiologically, anterior mediastinal tumors have no distinctive characteristics, and germ cell tumors should be always considered in the differential diagnosis. Tumors are usually bulky and lobulated. The presence of endodermal sinus tumor should especially be considered when patient is systemically ill, febrile with chest pain. A markedly elevated serum alpha-fetoprotein is almost virtually diagnostic. [1]

The present patient is male and 16 years of age, presented with high-grade fever with chest pain. Histopathologically, patient had highly malignant cells. Final diagnosis was achieved after immunohistochemistry and elevated alpha-fetoprotein in the serum of the patient. Careful examination of the abdomen, pelvis, and scrotal area was done to find primary lesions in these areas. No abnormality or primary lesion was noticed. CT scan of the abdomen and pelvis both plain and contrast were normal. Hence, we came to the conclusion of primary yolk sac tumor of the mediastinum in young male patient, which is rare and carries poor prognosis. Very few cases are reported in India.

 
  References Top

1.Fox MA, Vix VA. Endodermal sinus (yolk sac) tumour of the anterior mediastinum. AJR Am J Roentgenol 1980; 135:291-4.  Back to cited text no. 1
    
2.Kuzur ME, Cobleigh MA, Greco FA, Einhorn LH, Oldham RK. Endodermal sinus tumour of the mediastinum. Cancer 1982;50:766-74.  Back to cited text no. 2
    
3.Kundu S, Mandal S, Sengupta A, Dey A. A rare case of primary mediastinal non-seminomatous germ cell carcinomain a 18 year old boy. Lung India 2006;23:151-3.  Back to cited text no. 3
  Medknow Journal  
4.Tinica G, Butcovan D, Cimpeanu C, Tarcoveanu E. A mediastinal germ cell tumor of yolk SAC type- case report. Chirurgia (Bucur) 2010;105:831-4.  Back to cited text no. 4
    
5.Woodward PJ, Mostofi FK, Talerman A. Germ cell tumors. In: Eble JN, Sauter G, Epstein JI, Sesterhenn IA, editors. WHO classification of tumors. Pathology & Genetics. Tumors of the Urinary system and Male Genital Organs. Lyon: IARC Press; 2004. p. 237-40.  Back to cited text no. 5
    
6.Teilum G. Mesonephroma ovarii (Schiller): Extraembryonic mesoblastoma of germ cell origin in ovary and testis. Acta Pathol Microbiol Scand 1950;27:249-61.  Back to cited text no. 6
    
7.Malati T. Tumour markers: An overview. Inidan J Clin Biochem 2007;22:17-31.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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