A Rare Case of Mediastinal Yolk Sac Tumor

Andhika Kesuma Putra

, Noni Novisari Soeroso

and Muhammad Zainul Akbar

Department of Pulmonology and Respiratory Medicine, Faculty of Medicine, Universitas Sumatera Utara,

Universitas Sumatera Utara Hospital, Jl. Dr. Mansyur No. 5 Medan 20155, Sumatera Utara, Indonesia

Keywords: Yolk sac, Germ cell tumor, Core biopsy

Abstract: Yolk sac tumor is a germinal cell tumor that commonly causes testicular malignancy. The incidence of these

tumors lies in 90-95% of all testicular malignancies and commonly affects children with a median age of 1.5

years. In mixed type in adults, these tumors present in the age group 25-30 years. We reported a case of a

63-year-old man, who suffered from shortness of breath, accompanied by weight loss. Chest x-ray showed a

homogeneous consolidation in the mediastinum. With suspicion of a mediastinal tumor, the patient

underwent a core biopsy of the mass in the mediastinum. On histopathology examination showed

pleomorphic tumor cell form, enlarged nucleus, hyperchromatic, eosinophilic cytoplasm. The tumor cells

appear to form Schiller-Duval bodies. This case was diagnosed as a yolk sac tumor and underwent

chemotherapy.

1 INTRODUCTION

Germ cell tumor is a group of benign and malignant

neoplasm originated from primitive germ cell during

early embryogenesis. Germ cell tumor frequently

occurred in the gonads, only 5-10 % happened in the

extra gonad. Extragonadal germ cell tumor is mostly

found in the anterosuperior mediastinal (Bokemeyer

et al., 2002).

Primary yolk sac tumor in the anterior

mediastinum is rare and has a vicious prognosis.

Patients often present with advanced stage tumors

that are bulky and unresectable. Like other germ cell

tumors, yolk sac tumor is predominantly a disease of

young adults. However, a few cases of gonadal and

extragonadal germ cell tumors have been reported in

elderly patients as well (Nakhla and Sundararajan,

2016). We present a very rare case of an elderly 63-

year-old male with primary yolk sac tumor of the

mediastinum.

2 CASE REPORT

A 63-year-old male was admitted with shortness of

breath that he had been suffering for the past 3

months. Shortness of breath was not related to

physical activity or weather changes. The patient

also complained of cough that he had experienced in

the previous 2 months without any sputum

production. Systemic complaints experienced by

patient included very rapid weight loss during the

last 6 months and sub-febrile fever during the last 1

month. His vital signs were as follows: blood

pressure 120/80 mmHg, pulse rate of 112 beats per

minute, respiratory rate of 28 breaths per minute,

and temperature of 37.4

C. Based on the laboratory

findings, Hemoglobin was 12.5 gr%, leukocytes

were 9100 / mm

, and platelets were 424,800/mm

respectively. Atrial blood gas results were pH 7.45,

pCO

39.7 mmHg, pO

110.2 mmHg, HCO

27.9

mmol/L, BE 2,9 SaO

98.8%. Chest x-ray showed

widening in the mediastinal segment. With suspicion

of a mediastinal tumor, the patient underwent a core

needle biopsy of the mass in the mediastinum guided

by thoracic CT scan (Figure 1-3). Histopathology

results showed pleomorphic tumor cell form,

enlarged nucleus, hyperchromatic, eosinophilic

cytoplasm (Figure 4). The tumor cells appear to

form Schiller-Duval bodies. This case was

diagnosed as a yolk sac tumor and the patient

underwent chemotherapy.

Putra, A., Soeroso, N. and Akbar, M.

A Rare Case of Mediastinal Yolk Sac Tumor.

DOI: 10.5220/0009841000090011

In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease (ICTROMI 2019), pages 9-11

ISBN: 978-989-758-469-5

Figure 1: Coronal slice of Thorax CT showed the mass in

the right upper lobe.



Figure 2: Thorax CT showed needle marker in the

insertion phase.

Figure 3: Thorax CT showed needle marker in the tumor

site.

Figure 4: (A) Histopathology examination showed

pleomorphic tumor cell form, enlarged nucleus,

hyperchromatic, eosinophilic cytoplasm. (B) The tumor

cells appear to form Schiller-Duval bodies.

3 DISCUSSIONS

Germ cell tumors mostly occur in the gonads.

Extragonadal germ cell tumors are rare and most can

arise in the pineal gland, retroperitoneum, and the

mediastinum. The mediastinum is the most common

site of extragonadal germ cell tumors. Malignant

germ cell tumors in the mediastinum account for 1–

6% of all mediastinal tumors. Primary extragonadal

germ cell tumors, especially primary mediastinal

tumors, are considered to have a poor prognosis.

Germ cell tumors are histologically categorized into

teratomas, teratocarcinomas, seminomas, and

nonseminomatous carcinoma, including

choriocarcinoma, embryonal carcinoma, yolk sac

carcinoma, and mixed type carcinoma. Greater than

90% of malignant extragonadal tumors of the

mediastinum occur in men (Bokemeyer et al., 2002;

Nakhla and Sundararajan, 2016). In a retrospective

study by Sakurai et al. (2004) with 48 patients of

extragonadal germ cell tumors, the median age at

presentation was 28.8 years.

Yolk sac tumors can occur in both men and

women, usually arising from germ cells in testes and

ovaries, respectively. Pure yolk sac tumors are

usually found in young children and mixed germ cell

tumors with yolk sac are found in the adult. Similar

to other nonseminomatous germ cell tumors, the

latter can be associated with hematologic

Klinefelter’s syndrome (up to 20%) and other

hematological malignancies such as acute leukemia

and myelodysplastic syndrome. In an international

study by Bokemeyer et al. (2001) with 381

mediastinal germ cell tumors, the most common

symptoms on presentation were dyspnea (25%),

chest pain (23%), cough (17%), fever (13%), night

sweat, or weight loss (11%). Night sweat, fatigue,

hemoptysis, and symptoms of superior vena cava

compression were seen in <10% of patients with

mediastinal germ cell tumors (Nakhla and

Sundararajan, 2016).

Histologically, extragonadal germ cell tumors

and mediastinal germ cell tumors have many

similarities. Schiller-Duval bodies are

pathognomonic and are helpful for identification.

Yolk sac tumors immunohistochemical testing is

positive for AFP, glypican-3, SALL4, and placental

alkaline phosphatase (Bokemeyer et al., 2002;

Sakurai et al., 2004).

The treatment regimens of extragonadal and

gonadal yolk sac tumors are similar since they share

histological patterns. Extragonadal

nonseminomatous germ cell tumors have a

considerably poorer prognosis. Chemotherapeutic

schemes based on cisplatin have shown significant

results with up to 50% of patients achieving long-

ICTROMI 2019 - The 2nd International Conference on Tropical Medicine and Infectious Disease

term survival (Sakurai et al., 2004).

Bleomycin-

Etoposide-Cisplatin (BEP) therapy or etoposide

(Vepesid), ifosfamide, and cisplatin (VIP), with at

least 4 cycles of chemotherapy, are widely accepted

regimens. VIP regimen may be preferred over BEP

since patients with mediastinal germ cell tumors

might need postchemotherapy thoracotomy for

removal of residual tumor and bleomycin-induced

pulmonary toxicities can be potentiated by surgery

(Giannis et al., 2009).

Surgical resection as the primary treatment

modality is not recommended in mediastinal germ

cell tumors because of the likelihood of early

metastasis. However, there is a definite role for

postchemotherapy adjuvant surgery to remove

residual lesions and a rising serum tumor marker

after completion of chemotherapy is not considered

as a contraindication for surgery. Complete

resolution of serum AFP marker occurs in less than

5% of patients. Survival rates have increased in

patients who have the serum of AFP decreased after

chemotherapy and in cases with residual tumor

surgical excision (Vuky et al., 2001).

Elderly patients with germ cell tumor generally

have worse clinical outcomes compared to younger

patients. A Surveillance, Epidemiology, and End

Results (SEER) database analysis of 12,811 patients

comparing the outcomes of testicular cancers in

young adults (age <50) versus older adults (age >50

years) found survival from both localized and

metastatic nonseminomatous germ cell tumors to be

much better in younger patients compared to elderly

(76.9% versus 57.0%) (Bokemeyer et al., 2002;

Sakurai et al., 2004). Our patient had an overall poor

prognosis due to multiple reasons such as his age,

tumor location, and tumor bulk.

FUNDING

No grant support or funding from public institutions

or private enterprises was received for this case

report.

ACKNOWLEDGMENTS

The researcher would like to thank Columbia Asia

Hospital and Universitas Sumatera Utara Hospital

which have allowed the retrieval of medical history

data.

REFERENCES

Bokemeyer, C. et al., 2002. Extragonadal germ cell

tumors of the mediastinum and retroperitoneum:

Results from an international analysis. Journal of

Clinical Oncology. 20(7): 1864–1873. doi:

10.1200/JCO.2002.07.062.

Giannis, M. et al., 2009. Cisplatin-based chemotherapy for

advanced seminoma: Report of 52 cases treated in two

institutions. Journal of Cancer Research and Clinical

Oncology. 135(11): 1495–1500. doi: 10.1007/s00432-

009-0596-2.

Nakhla, S. G. and Sundararajan, S., 2016. A Rare Case of

Primary Anterior Mediastinal Yolk Sac Tumor in an

Elderly Adult Male. Case Reports in Oncological

Medicine. 16(4): 8961-8965. doi:

10.1155/2016/8961486.

Sakurai, H. et al., 2004. Management of primary

malignant germ cell tumor of the mediastinum.

Japanese Journal of Clinical Oncology. 34(7): 386–

392 doi: 10.1093/jjco/hyh062.

Vuky, J. et al., 2001. Role of postchemotherapy adjunctive

surgery in the management of patients with

nonseminoma arising in front of the mediastinum.

Journal of Clinical Oncology. 19(3): 682–688. doi:

10.1200/JCO.2001.19.3.682.

A Rare Case of Mediastinal Yolk Sac Tumor