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Journal of Peking University(Health Sciences) ›› 2019, Vol. 51 ›› Issue (6): 1091-1095. doi: 10.19723/j.issn.1671-167X.2019.06.020

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Diagnosis and treatment of seven primary mediastinal yolk sac tumors

Yi-xiang MA1,Jing-wei LIU1,Kang QI1,Ji-xin ZHANG2,Gang LIN1,Hai-bo LIU1,Xue-qian SHANG1,Jian LI1,△()   

  1. 1. Department of Thoracic Surgery, Peking University First Hospital, Beijing 100034, China
    2. Department of Pathology, Peking University First Hospital, Beijing 100034, China
  • Received:2019-06-12 Online:2019-12-18 Published:2019-12-19
  • Contact: Jian LI E-mail:pkufts@163.com

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Abstract:

Objective: Primary mediastinal yolk sac tumor, which is also known as endodermal sinus tumor, is a rare but lethal neoplasm and its prognosis is very dismal. The current treatment for this tumor is controversial, and chemotherapy combined with resection of residual lesions is adopted sometimes. We summarized the experience of seven primary mediastinal yolk sac tumors treated with platinum-based chemotherapy and extended resection in Peking University First Hospital.Methods: Clinicopathological data of the patients with primary mediastinal yolk sac tumor who received operation in Peking University First Hospital between August 2014 and August 2018 were collected and analyzed retrospectively.Results: We experienced seven primary mediastinal yolk sac tumors during this period. Computed tomography scan revealed an anterior mediastinal tumor in all the patients and all of them had markedly raised alphafetoprotein (AFP) and normal β-human chorion gonadotropin (β-HCG). Five patients underwent needle core biopsy before treatment, which showed a mediastinal yolk sac tumor. All of these patients received preoperative platinum-based chemotherapy and they all presented partial response according to computed tomography. Two other patients did not receive preoperative biopsy, so they directly underwent extended resection. All of the seven patients underwent operation successfully and two of them expe-rienced postoperative complications, including one with pneumonia and the other with atelectasis. R0 resection was achieved in six patients and R1 resection was achieved in the other patient. According to postoperative pathology, there were one microcyst subtype, one adenoid subtye, one giant capsule subtype and two hybrid subtypes. Surprisingly, there were no yolk sac tumor tissue in the other two patients after preoperative chemotherapy. All the patients received postoperative chemotherapy, excluded one patient who was unable to tolerate chemotherapy after operation. Three patients experienced postoperative pulmonary metastases within one year and two of them died soon. The other patient received chemotherapy and immunotherapy after recurrence and he was alive at the time of writing. Four other patients were alive without recurrence and metastasis.Conclusion: Primary mediastinal yolk sac tumor is rare and its prognosis is poor. A multimodality approach including adjuvant chemotherapy and resection of residual lesions is the optimal treatment and it may lead to long-term survival.

Key words: Primary mediastinal yolk sac tumor, Chemotherapy, Extended resection

CLC Number: 

  • R736

Figure 1

Chest CT of primary yolk yolk sac tumor (case 7) before and after chemotherapy A, before chemotherapy, the tumor located in right front mediastinum; B, after chemotherapy, the tumor shrinked significantly."

Table 1

The clinical manifestations of seven primary mediastinal yolk sac tumor patients"

Case Age/years Symptom AFP before
chemotherapy/
(μg/L)		 Preoperative
chemotherapy
(cycle, drug)		 AFP after
chemotherapy/
(μg/L)		 Surgical
approach		 Excision
extension		 Surgical
margin		 Subtype Postoperative
chemotherapy
(cycle, drug)		 Recurrence
location		 Status* Survival/
months
1 16 Chest distress fever 9 545.00 4×PEB 4.84 LMSAT Tumor+partial pericardium+
phrenic nerve		 R0 Not clear 2×PEB No Alive 48
2 17 Chest distress chest pain 285.50 4×EP 13.31 MS Tumor+partial pericardium+
partial pulmonary		 R0 Microcyst 2×PEB Pulmonary Alive 39
3 32 No 2 697.00 - - RMSAT Tumor+partial pericardium R0 Adenoid 4×PEB Pulmonary Dead 11
4 35 Chest distress SVCS 12 045.00 6×PEB 1 117.00 MS Tumor+partial pericardium and
pulmonary+superior vena cava		 R1 Hybird No Pulmonary Dead 5
5 14 Cough chest pain 32 105.00 6×PEB 10.91 RMSAT Tumor+thymoma R0 Giant capsule 2×PEB No Alive 23
6 22 Cough 1 210.00 - - LMSAT Tumor+ phrenic nerve R0 Hybird 4×EP No Alive 15
7 22 Fever chest pain 42 034.00 2×PEB 261.60 MS Tumor+partial pericardium and
pulmonary+superior vena cava		 R0 Not clear 4×PEB No Alive 13

Table 2

The immunohistochemistry of seven primary mediastinal sac tumor patients"

Case Vimentin CK(AE1/AE3) AFP SALL4 PLAP CD117 CK7 OCT4 Ki-67/%
1 - + + - - + - - 70
2 - + + - + - - - 50
3 - + + + + + - - 70
4 - + + + + - - - 70
5 - + + + - + + - 60
6 - + + + - + - - 60
7 - + + - - - - - 60

Figure 2

The histopathology of primary mediastinal yolk sac tumor A and B, HE staining; C, AFP(+); D, CK(+); E, SALL4(+); F, PLAR(+)."

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